Cloward, R.B. (1952). The treatment of ruptured lumbar intervertebral disc by vertebral body fusion. III. Methods and use of banked bone. *Ann Surg*, 136, 987–992 Cloward, R.B. (1953). The treatment of ruptured lumbar intervertebra1discs by vertebral body fusion. I. Indications, operative technique,after care. *J Neurosurg,*10, 154-168. Cloward, R.B. (1981). Spondylolisthesis, treatment by laminectomy and PLIF. Clin Orthop.

Cloward, R.B. (1985). The treatment of ruptured lumbar intervertebral disk by vertebral

Faubert, C. & Caspar, W. (1991). Lumbar percxtaneous discectomy. Initial experience in 28

Fischgrund, J.S.; MacKay, M.; Herkowitz, H.N., et al. (1997). Degenerative lumbar

spondylolisthesis with spinal stenosis: A prospective, randomized study

instrumentation in the treatment of degenerative spondylolisthesis with spinal

two-year clinical results in the first 26 patients. *Spine*, 18, 2106-2117.

invasive approaches can achieve good-to-excellent clinical results..

utilizing dowel graft. *J Neurol Ortop Surg*, 2, 171-175.

stenosis. *J Spinal Disord,* 6,461-472

body fusion. *Clin Orthop,* 193, 5-154.

cases. Neuroradiology, 33, 407–410.

Preoperative MR imaging demonstrated mild central canal stenosis secondary to degenerative spondylolisthesis L4-L5 (A,B). Postoperative MR imaging did not show cauda equina compression 2 year after surgery (C,D). Restration of disc hight and lordosis also demonstrated .

Fig. 10. Post-operative plain radiographs [anteroposterior view (A) ,lateral view(B)] and coronal reformatted CT (C).

This patient underwent a minimally invasive PLIF via a Casper retractor placed from a leftsided approach. The interbody fusion was supplemented with percutaneous pedicle screw and rods. The patient's surgery was uneventful. She was discharged on postoperative day 14 and returned to her previous job in 3 months after this operation. After 24 months of follow-up, she had no evidence of low back pain but slightly numbness requiring no medication persisted. The X-ray showed the bone union of PLIF without collapse at 2 years after operation. Frontal view (Fig.10A) and coronal CT scan (Fig.10C) showed bone union in PEEK interbody spacers. Lateral view showed solid fusion (Fig.10B). MR imaging did not show cauda equine compression (Fig9C,D).

#### **5. Complications of minimally invasive arthrodesis procedure**

The goal of lumbar interbody fusion is to stabilize the spinal segment and decompress the neural elements. PLIF can completely remove the pathological focus by the naked-eye or using an operating microscope. However, the PLIF requires significant retraction on the dural sac and nerve roots. As a result, it is associated with higher risks of cerebrospinal fluid leakage, dysesthetic nerve root pain syndrome, nerve root injury, and epidural fibrosis (Lin, 1985, Fritzell, et al., 2002, Scaduto, et al., 2003). These complications can be avoided by microscope visualization of the working space during surgery.

Transforaminal lumbar interbody fusion (TILF) is originally described by Blume and Rojas (1981) and Harms and Rolinger (1982). The TILF approach was popularized by Harms et al (1997). TILF was reported to be an effective surgical technique for the treatment of various degenerative lumbar diseases because it allows lateral access to the neural canal. This incision is two fingerbreadths off of the midline and allows for a paraspinal muscle splitting (Wiltse) approach to be performed (Wiltse & Spencer, 1988). The procedure involves less retraction of the dural sac and nerve roots resulting in low complication rate, and produces clinical outcomes and fusion rates similar to those of other techniques. However, the ideal indication for a TILF is a grade 1 or grade 2 spondylolisthesis without neurologic deficit or with a deficit on one side only (Moskowitz, 2002). This approach cannot completely remove the pathological focus with central canal stenosis. Although offering certain advantages, minimally invasive arthrodesis procedure has several limitations and potential complications. There is a learning curve associated with the development of technical skills necessary to perform these procedure.

### **6. Conclusions**

40 Orthopedic Surgery

Preoperative MR imaging demonstrated mild central canal stenosis secondary to degenerative spondylolisthesis L4-L5 (A,B). Postoperative MR imaging did not show cauda equina compression 2 year after surgery (C,D). Restration of disc hight and lordosis also

Fig. 10. Post-operative plain radiographs [anteroposterior view (A) ,lateral view(B)] and

**5. Complications of minimally invasive arthrodesis procedure**

microscope visualization of the working space during surgery.

This patient underwent a minimally invasive PLIF via a Casper retractor placed from a leftsided approach. The interbody fusion was supplemented with percutaneous pedicle screw and rods. The patient's surgery was uneventful. She was discharged on postoperative day 14 and returned to her previous job in 3 months after this operation. After 24 months of follow-up, she had no evidence of low back pain but slightly numbness requiring no medication persisted. The X-ray showed the bone union of PLIF without collapse at 2 years after operation. Frontal view (Fig.10A) and coronal CT scan (Fig.10C) showed bone union in PEEK interbody spacers. Lateral view showed solid fusion (Fig.10B). MR imaging did not

The goal of lumbar interbody fusion is to stabilize the spinal segment and decompress the neural elements. PLIF can completely remove the pathological focus by the naked-eye or using an operating microscope. However, the PLIF requires significant retraction on the dural sac and nerve roots. As a result, it is associated with higher risks of cerebrospinal fluid leakage, dysesthetic nerve root pain syndrome, nerve root injury, and epidural fibrosis (Lin, 1985, Fritzell, et al., 2002, Scaduto, et al., 2003). These complications can be avoided by

Transforaminal lumbar interbody fusion (TILF) is originally described by Blume and Rojas (1981) and Harms and Rolinger (1982). The TILF approach was popularized by Harms et al (1997). TILF was reported to be an effective surgical technique for the treatment of various degenerative lumbar diseases because it allows lateral access to the neural canal. This

demonstrated .

coronal reformatted CT (C).

show cauda equine compression (Fig9C,D).

Minimal invasive spinal surgery is an expanding technique and percutaneous pedicle screws are often used to minimize muscle injuries. However, there is no proof that spinal outcomes are better with minimally invasive spinal surgery than with conventional spinal surgery, which remains the gold standard against which new techniqus should be evaluated. The PLIF procedure has come into a fair amount of disfavor because of perceived and actual complications related to the procedure. These complication include bleeding, dural laceration, nerve root injuries, graft migration and pseudarthrosis. High fusion rates with good clinical outcomes can be achieved with few complications using microscope and minimal invasive technique. Our clinical experience using the microscope has showed excellent visualization, appropriate safety control, and low complication rate. Skilled surgeons familiar with the technique, anatomy, and instrumentation involved in minimally invasive approaches can achieve good-to-excellent clinical results..

#### **7. References**

**Part 2**

**Upper Extremity**

comparing decompressive and arthrodesis with and without spinal instrumentation. *Spine*, 22, 2807-2812.

## **Part 2**

**Upper Extremity**

42 Orthopedic Surgery

Foley KT, Gupta SK, Justis JR, et al. (2001). Percutaneous pedicle screw fixation of the

Fritzell, P.; Hagg, O.; Wessberg, P., et al. (2002). Chronic low back pain and fusion. A

Harms, J. & Rolinger, H. (1982). A one-stage procedure in operative treatment of

Harms, J.; Jeszenszky, D.; Stolze, D., et al. (1997). True spondylolisthesis reduction and more

Lin,P.M. (1985). Posterior lumbar interbody fusion technique: complications and pitfalls.

Magerl, F. (1982) External skeletal fixation of the lower thoracic and lumbar spine, in

Meyerding, H.W. (1932). Spondylolisthesis: surgical treatment and results. *Surg Gynecol*

Moskowitz, A. (2002). Transforaminal lumbar interbody fusion. Orthop Clin North Am, 33,

Park, P. & Foley, K.T. (2008) Minimally invasive transforaminal interbody fusion with

Scaduto, A.A.; Gamradt, S.C.; Yu,W.D., et al. (2003). Perioperative complications of tgreaded

Takeno, K., Kobayashi, S., Miyazaki, S., et al. (2010). Microsurgical excision of hematoma of

Taillard, W. (1954). Le spondylolisthesis chez l' enfant et l'adolescent (Etude de 50 cas). *Acta*

Wiltse, L.L. & Spencer, C.W. (1988). New uses and refinements of the paraspinal approach

Yamamoto, M.; Kadowaki, T.; Ota, N. et al. (1990). PLIF for lumbar degenerative

Young, S.; Veerapen, R.; O'Laoire, S.A.. (1988.): Relief of lumbar canal stenosis using

Yvon, H.A.; Garfin, S.R.; Dickman, C.A. et al. (1994). A historical comfort study of pedicle

Zdeblick, T.A. (1993). A prospective, randomized study of lumbar fusion. Spine, 18, 983-991.

multilevel subarticular fenestrations as an alternative to wide laminectomy:

screw fixation in thoracic, lumbar and sacral spinal fusions. *Spine*, 19(Suppl), 2279S-

the lumbar ligamentum flavum. *Joint Bone Spine,* 77, 351-354.

study from the Swedish Lumbar Spine Group. *Spine*, 27, 1131-1141.

instrumentation. *Spine*, 22, 2807-2812.

lumbar spine. *Neurosurg Focus*, 10, E10

Philadelphia: lipponcott-raven, 1337-1347.

New York: Springer-Verlag, pp 353–366.

years' follow-up. *Neurosurg Focus,* 25,E16.

to the lumbar spine. *Spine*, 13, 696–706.

spondylolisthesis. *Rinsho Seikeigeka,* 25, 487-494.

preliminary report.. *Neurosurgery,* 23, 628–633.

*Spinal Disord Tech*, 16, 502-507.

*Orthop Scand,*24, 115-144.

*Grenzgeb,* 120, 343–347

*Clin Orthop,* 193, 90-102.

*Obstet* 54:371–377.

359–366.

2296S.

comparing decompressive and arthrodesis with and without spinal

comparison of three surgical techniques: a prospective multicenter randomized

spondylolisthesis: dorsal traction-reposition and anterior fusion. *Z Orthop Ihre*

segmental fusion in spondylolisyjesis. In: *The Textbook of Spinal Surgery.* 2nd Ed.

Uhthoff, H.K. & Stahl, E. (eds.): Current Concepts of External Fixation of Fractures.

reduction of spondylolisthesis: technique and outcomes after a minimum of 2

cylindrical lumbar interbody fusion devices: anterior versus posterior approach. *J*

**3**

**The Distal Forearm Region – Ultrasonographic**

The distal forearm, particularly the distal radius, the radio-carpal joint and surrounding soft tissues are commonly affected by acute and chronic disorders. Ultrasonography has gained increasing importance in both the evaluation of acute injuries and chronic disorders in adults and in the diagnosis and follow-up of fractures in children and adolescents1-4. In children, the use of ultrasonography allows the chondral parts of the epiphyseal region to be better

The main advantage of sonography over CT and MRI is the possibility of performing dynamic examinations, resulting in exact clinical functional evaluation of the muscles, tendons and joints in question3,5,6. Furthermore, the contralateral limb can be examined in

It is the aim of this study, to demonstrate the normal ultrasonographic findings in the distal forearm region in children and adolescents, as this area is frequently involved in injuries.

We studied 100 children and adolescents 2 months - 18 years old (mean ± standard deviation [SD] = 7.1 ± 4.5 years) and 25 healthy adults aged between 20 and 60 years. Children were recruited from our institution's paediatric surgical outpatient clinics. The patients had been admitted for unrelated disorders, requiring surgery, and the ultrasound study of the distal forearm was obtained together with the ultrasound study of the abdomen, urinary tract or soft tissue small parts. The area of the distal forearm was used first to demonstrate the painlessness

evaluated without exposure to radiation than using standard radiographic techniques.

direct comparison when initial findings are uncertain.

**2. Probands and methods**

\*Corresponding Author

**1. Introduction**

**Anatomy in Children and Adolescents**

Andreas Fette3, Andreas H. Weiglein4 and Sergio Sesia1

*4Department of Anatomy, Medical University of Graz, Graz*

*2Medical University of Graz, Graz, Department of Traumatology 3Medical University of Graz, Department of Pediatric Surgery*

> *1Switzerland 2,3,4Austria*

Johannes M. Mayr1,\*, Wolfgang Grechenig2, Ursula Seebacher3,

*1Department of Pediatric Surgery, University Children's Hospital Basel, Basel,*

## **The Distal Forearm Region – Ultrasonographic Anatomy in Children and Adolescents**

Johannes M. Mayr1,\*, Wolfgang Grechenig2, Ursula Seebacher3, Andreas Fette3, Andreas H. Weiglein4 and Sergio Sesia1 *1Department of Pediatric Surgery, University Children's Hospital Basel, Basel, 2Medical University of Graz, Graz, Department of Traumatology 3Medical University of Graz, Department of Pediatric Surgery 4Department of Anatomy, Medical University of Graz, Graz 1Switzerland 2,3,4Austria*

#### **1. Introduction**

The distal forearm, particularly the distal radius, the radio-carpal joint and surrounding soft tissues are commonly affected by acute and chronic disorders. Ultrasonography has gained increasing importance in both the evaluation of acute injuries and chronic disorders in adults and in the diagnosis and follow-up of fractures in children and adolescents1-4. In children, the use of ultrasonography allows the chondral parts of the epiphyseal region to be better evaluated without exposure to radiation than using standard radiographic techniques.

The main advantage of sonography over CT and MRI is the possibility of performing dynamic examinations, resulting in exact clinical functional evaluation of the muscles, tendons and joints in question3,5,6. Furthermore, the contralateral limb can be examined in direct comparison when initial findings are uncertain.

It is the aim of this study, to demonstrate the normal ultrasonographic findings in the distal forearm region in children and adolescents, as this area is frequently involved in injuries.

#### **2. Probands and methods**

We studied 100 children and adolescents 2 months - 18 years old (mean ± standard deviation [SD] = 7.1 ± 4.5 years) and 25 healthy adults aged between 20 and 60 years. Children were recruited from our institution's paediatric surgical outpatient clinics. The patients had been admitted for unrelated disorders, requiring surgery, and the ultrasound study of the distal forearm was obtained together with the ultrasound study of the abdomen, urinary tract or soft tissue small parts. The area of the distal forearm was used first to demonstrate the painlessness

<sup>\*</sup>Corresponding Author

Distal Forearm Sonography 47

The median nerve was visualized during static and dynamic ultrasound investigation. No standoff pad was used. Instead, a liberal amount of coupling gel was applied. For young children pre-warmed contact gel was used. The contra-lateral limb was also investigated to

For evaluation of age-related changes, the patients were divided into 4 groups (25 children per group). Group 1 included infant and toddler children from 2 months - 3 years old; group 2, young children 4 - 6 years old; group 3, pre-adolescent children 7 - 11 years old; and

A dorsal, radial and volar longitudinal plane and corresponding transverse sections were investigated. The radius was defined as the leading structure for longitudinal scans. By moving the probe distally, the cavity of the radiocarpal joint was localised and positioned in the centre of the image. From this standard position, the probe was moved in both a radial and ulnar direction, maintaining an "orthograde" probe position to avoid hypo-echogenicity

In the dorsal transverse plane, the scan began in the distal forearm region, identifying the

The dorsal longitudinal scans were used to measure the width and investigate the echogenicity of the epiphyseal growth plate cartilage and to document the ossification of the secondary ossification centre of the distal epiphysis of the radius. From the dorsal radio-ulnar position, the probe was moved distally along the distal forearm, the wrist

The musculo-tendinous junction of the dorsal forearm muscles and the course of the

In order to evaluate the tendons, the probe was placed in an orthograde position and slow active and/or passive movement of the tendons and muscles was performed to demonstrate

The volar region of the distal forearm and wrist was examined using a similar technique.

To identify the median nerve the probe was placed in the transverse volar position and the focus was adjusted to a position just beneath the level of the skin. The median nerve was identified in its course running between the palmaris longus tendon and the flexor carpi

The volar longitudinal scans were used to search for branches of the radial artery supplying

In all planes, the osseous structures were shown as bright echogenic lines with dorsal acoustic shadows, due to the difference in impedance of bone when compared to the surrounding soft tissue. The radiocarpal joint was identified as an echo-free gap between the

group 4, adolescent and teenage children 12 - 18 years old.

of tendons caused by a non-orthograde transducer position7.

radius and ulna as osseous leading structures.

the function of the forearm muscles and their tendons.

**4. Ultrasonographic findings in adult probands (n = 25)**

**3. Technique of examination / standard planes**

allow comparison.

joint and the carpus.

radialis tendon.

extensor tendons was examined.

the distal epiphysis of the radius.

of the ultrasound examination to the child and the parents. Thereafter the linear ultrasound scanning probe was adjusted at the beginning of the ultrasound study, and the sonography of the distal forearm together with the ultrasound examination of the abdomen, retroperitoneum or small part region of interest was carried out. Consent was obtained from the parents, or in the case of older children, from both the children and parents. Patients with a history of forearm trauma or pathology were excluded from this investigation. However, a limited number of these patients are shown to demonstrate different pathological ultrasound findings (like fractures and osteomyelitis) in a separate chapter.

A 12-5 MHz linear probe ( Philips-ATL®, HDI 5000, Philips®, Bothell, WA, USA ) and high frequency probes ( 7.5 - 12 MHz) (Siemens® Acuson® and Elegra®, Siemens®, Erlangen, FRG) were used for static and dynamic examination of the distal forearm region concentrating on the distal radius, the radiocarpal joint and surrounding soft tissues.

All 100 children and adolescents were examined in 3 longitudinal and 2 transverse standard planes and Doppler colour sonography was used to demonstrate vessels running within the cartilage of the distal radius supplying the epiphysis of the distal radius(Fig. 1).

Fig. 1. Palmar-radial longitudinal colour coded duplex sonography scan in a boy aged 3 years showing the radial artery and an epiphyseal vessel. The ossification centre shows an echogenic reflex with dorsal acoustic shadow; the pronator quadratus muscle is visualised transversely beneath the radial artery.

of the ultrasound examination to the child and the parents. Thereafter the linear ultrasound scanning probe was adjusted at the beginning of the ultrasound study, and the sonography of the distal forearm together with the ultrasound examination of the abdomen, retroperitoneum or small part region of interest was carried out. Consent was obtained from the parents, or in the case of older children, from both the children and parents. Patients with a history of forearm trauma or pathology were excluded from this investigation. However, a limited number of these patients are shown to demonstrate different pathological ultrasound findings

A 12-5 MHz linear probe ( Philips-ATL®, HDI 5000, Philips®, Bothell, WA, USA ) and high frequency probes ( 7.5 - 12 MHz) (Siemens® Acuson® and Elegra®, Siemens®, Erlangen, FRG) were used for static and dynamic examination of the distal forearm region

All 100 children and adolescents were examined in 3 longitudinal and 2 transverse standard planes and Doppler colour sonography was used to demonstrate vessels running within the cartilage of the distal radius supplying the epiphysis of the distal radius(Fig. 1). The wrist joint was examined in neutral position with the child sitting in front of a table either on a

concentrating on the distal radius, the radiocarpal joint and surrounding soft tissues.

Fig. 1. Palmar-radial longitudinal colour coded duplex sonography scan in a boy aged 3 years showing the radial artery and an epiphyseal vessel. The ossification centre shows an echogenic reflex with dorsal acoustic shadow; the pronator quadratus muscle is visualised

(like fractures and osteomyelitis) in a separate chapter.

chair or on a parent's lap.

transversely beneath the radial artery.

The median nerve was visualized during static and dynamic ultrasound investigation. No standoff pad was used. Instead, a liberal amount of coupling gel was applied. For young children pre-warmed contact gel was used. The contra-lateral limb was also investigated to allow comparison.

For evaluation of age-related changes, the patients were divided into 4 groups (25 children per group). Group 1 included infant and toddler children from 2 months - 3 years old; group 2, young children 4 - 6 years old; group 3, pre-adolescent children 7 - 11 years old; and group 4, adolescent and teenage children 12 - 18 years old.

#### **3. Technique of examination / standard planes**

A dorsal, radial and volar longitudinal plane and corresponding transverse sections were investigated. The radius was defined as the leading structure for longitudinal scans. By moving the probe distally, the cavity of the radiocarpal joint was localised and positioned in the centre of the image. From this standard position, the probe was moved in both a radial and ulnar direction, maintaining an "orthograde" probe position to avoid hypo-echogenicity of tendons caused by a non-orthograde transducer position7.

In the dorsal transverse plane, the scan began in the distal forearm region, identifying the radius and ulna as osseous leading structures.

The dorsal longitudinal scans were used to measure the width and investigate the echogenicity of the epiphyseal growth plate cartilage and to document the ossification of the secondary ossification centre of the distal epiphysis of the radius. From the dorsal radio-ulnar position, the probe was moved distally along the distal forearm, the wrist joint and the carpus.

The musculo-tendinous junction of the dorsal forearm muscles and the course of the extensor tendons was examined.

In order to evaluate the tendons, the probe was placed in an orthograde position and slow active and/or passive movement of the tendons and muscles was performed to demonstrate the function of the forearm muscles and their tendons.

The volar region of the distal forearm and wrist was examined using a similar technique.

To identify the median nerve the probe was placed in the transverse volar position and the focus was adjusted to a position just beneath the level of the skin. The median nerve was identified in its course running between the palmaris longus tendon and the flexor carpi radialis tendon.

The volar longitudinal scans were used to search for branches of the radial artery supplying the distal epiphysis of the radius.

#### **4. Ultrasonographic findings in adult probands (n = 25)**

In all planes, the osseous structures were shown as bright echogenic lines with dorsal acoustic shadows, due to the difference in impedance of bone when compared to the surrounding soft tissue. The radiocarpal joint was identified as an echo-free gap between the

Distal Forearm Sonography 49

structures) the median nerve was documented by ultrasound in only 18 of 25 children (72 %). Other sonographic characteristics of the distal forearm region are presented in Table 1.

> Group 2 (4 - 6 years)

2.65 ± 0.49 (1.8 - 3.8)

5/25 (20 %) 0/25 0/25 0/25

15/25 (60 %) 23/25 (92 %) 24/25 (96 %) 16/25 (64 %)

8/25 (32 %) 0/25 1/25(4%) 0/25

25/25 (100 %) 25/25 (100 %) 25/25 (100 %) 25/25 (100 %)

growth plate fused 0/25 0/25 0/25 5/25 (20 %)

median nerve 18/25 (72 %) 25/25 (100 %) 25/25 (100 %) 25/25 (100 %)

Table 1. Sonographic characteristics of the distal forearm region in children and adolescents

Group 3 (7 - 11 years)

2.19 ± 0.47 (1.4 - 3.5)

Group 4 (12 - 18 years)

1.39 ± 0.86 (0.1 - 3.0) (growth plate fused in 5 children)

Group 1

Proximodistal diameter of the distal radial growth

Absent ossification of the secondary ossification centre of the distal radius

plate; mm1

Sonographic characteristics of the distal radius growth plate and epiphyseal cartilage

within the

Presence of vessels

epiphyseal cartilage

Visualisation of the

\* data are for n = 100 children (25 per group)

according to age groups\*.

1 mean ± standard deviation; range are shown in parenthesis.

Identification of the radial neurovascular bundle (grey scale and Doppler sonography)

(2 months - 3 years)

4.16 ± 1.16 (2.8 - 6.0) (n = 20 / 25)

radius and the carpal bones. The proportion of articular cartilage of the carpal bones that could be visualized was dependent on the functional position of the hand. This should be considered when anechoic formations or structures (e.g. ganglia; intraarticular effusions) are evaluated.

Dynamic examination in a dorsal radio-ulnar plane allows the course of the extensor tendons to be accurately evaluated. Special attention has to be paid to the course of the extensor pollicis longus tendon in the region of Lister's tubercle, as the tendon crosses the underlying tendons of the extensor carpi radialis longus and brevis muscles. In the region of the musculo-tendinous junctions of the forearm extensor muscles, the tendons are occasionally surrounded by a thin hypoechoic muscular layer. Ultrasonographically, this small hypoechoic area must not be confused with extensor tenosynovitis3.

The radial neurovascular bundle on the volar aspect of the distal forearm was identified by its pulsation by grey-scale ultrasound and confirmed by its flow characteristics by colour Doppler sonography in all probands. The course of the radial artery and its distribution can be depicted by distal movement of the probe. Due to the width of the soft tissue coverage on the volar aspect, identification of a single tendon is easier when compared to the dorsal distal forearm region. By functional evaluation, the superficial and deep flexor tendons were depicted in the longitudinal plane. Both the carpal joint and the median nerve were clearly identified. The flexor retinaculum is difficult to separate from the surrounding tissue8. The flexor retinaculum was identified in 13 of 25 patients (52%), suspected in 3 patients (12%) and not seen in 9 patients (36%). The main problem encountered with the identification of the flexor retinaculum was its hypoechogenicity, which was similar to the echogenicity of the overlying subcutaneous fat. The median nerve is characterised by its lower echogenicity when compared to its neighbouring tendons and its course between the superficial and deep flexor tendons is easily followed in a proximal direction3,4,6. Whilst the proband is moving his or her fingers, a characteristic transposition and change in the transverse shape of the median nerve can be noted. The median nerve is easily detected as it runs between the tendons of the flexor sublimis and flexor carpi radialis, and rather towards the radial side of the tendon of the palmaris longus. The palmaris longus tendon shows no longitudinal displacement during finger movements. The interosseous membrane and the pronator quadratus muscle on the volar aspect were identified easily in the transverse plane in all patients.

#### **5. Ultrasonographic findings in children**

Depending on the age of children various sonographic findings of the distal forearm region were observed.

In group 1 children (2 months - 3 years old), no ossification of the distal radial ossification centre was seen in 5 infants (aged up to 6 months). The echogenicity of the cartilage at the region of the distal radial growth plate was either anechoic with weak reticular echogenic pattern (5 children, aged from 0 - 3 months) or anechoic with weak echogenic spots (10 children). In 8 of 25 children (32 %) colour Doppler identified at least one small vessel within the cartilage of the volar distal radial epiphysis (Fig.1). Due to investigator problems (like coupling problems, more frequent forearm movements of infants, and very small

radius and the carpal bones. The proportion of articular cartilage of the carpal bones that could be visualized was dependent on the functional position of the hand. This should be considered when anechoic formations or structures (e.g. ganglia; intraarticular effusions) are

Dynamic examination in a dorsal radio-ulnar plane allows the course of the extensor tendons to be accurately evaluated. Special attention has to be paid to the course of the extensor pollicis longus tendon in the region of Lister's tubercle, as the tendon crosses the underlying tendons of the extensor carpi radialis longus and brevis muscles. In the region of the musculo-tendinous junctions of the forearm extensor muscles, the tendons are occasionally surrounded by a thin hypoechoic muscular layer. Ultrasonographically, this

The radial neurovascular bundle on the volar aspect of the distal forearm was identified by its pulsation by grey-scale ultrasound and confirmed by its flow characteristics by colour Doppler sonography in all probands. The course of the radial artery and its distribution can be depicted by distal movement of the probe. Due to the width of the soft tissue coverage on the volar aspect, identification of a single tendon is easier when compared to the dorsal distal forearm region. By functional evaluation, the superficial and deep flexor tendons were depicted in the longitudinal plane. Both the carpal joint and the median nerve were clearly identified. The flexor retinaculum is difficult to separate from the surrounding tissue8. The flexor retinaculum was identified in 13 of 25 patients (52%), suspected in 3 patients (12%) and not seen in 9 patients (36%). The main problem encountered with the identification of the flexor retinaculum was its hypoechogenicity, which was similar to the echogenicity of the overlying subcutaneous fat. The median nerve is characterised by its lower echogenicity when compared to its neighbouring tendons and its course between the superficial and deep flexor tendons is easily followed in a proximal direction3,4,6. Whilst the proband is moving his or her fingers, a characteristic transposition and change in the transverse shape of the median nerve can be noted. The median nerve is easily detected as it runs between the tendons of the flexor sublimis and flexor carpi radialis, and rather towards the radial side of the tendon of the palmaris longus. The palmaris longus tendon shows no longitudinal displacement during finger movements. The interosseous membrane and the pronator quadratus muscle on the volar aspect were identified easily

Depending on the age of children various sonographic findings of the distal forearm region

In group 1 children (2 months - 3 years old), no ossification of the distal radial ossification centre was seen in 5 infants (aged up to 6 months). The echogenicity of the cartilage at the region of the distal radial growth plate was either anechoic with weak reticular echogenic pattern (5 children, aged from 0 - 3 months) or anechoic with weak echogenic spots (10 children). In 8 of 25 children (32 %) colour Doppler identified at least one small vessel within the cartilage of the volar distal radial epiphysis (Fig.1). Due to investigator problems (like coupling problems, more frequent forearm movements of infants, and very small

small hypoechoic area must not be confused with extensor tenosynovitis3.

in the transverse plane in all patients.

were observed.

**5. Ultrasonographic findings in children**

evaluated.

Group 1 (2 months - 3 years) Group 2 (4 - 6 years) Group 3 (7 - 11 years) Group 4 (12 - 18 years) Proximodistal diameter of the distal radial growth plate; mm1 4.16 ± 1.16 (2.8 - 6.0) (n = 20 / 25) 2.65 ± 0.49 (1.8 - 3.8) 2.19 ± 0.47 (1.4 - 3.5) 1.39 ± 0.86 (0.1 - 3.0) (growth plate fused in 5 children) Absent ossification of the secondary ossification centre of the distal radius 5/25 (20 %) 0/25 0/25 0/25 Sonographic characteristics of the distal radius growth plate and epiphyseal cartilage - Anechoic 10/25 (40 %) 2/25 (8 %) 1/25 (4 %) 4/25 (16 %) - Mixed type (echogenic spots / bands / reticular pattern) within anechoic cartilage 15/25 (60 %) 23/25 (92 %) 24/25 (96 %) 16/25 (64 %) - Epiphyseal growth plate fused 0/25 0/25 0/25 5/25 (20 %) Presence of vessels within the epiphyseal cartilage 8/25 (32 %) 0/25 1/25(4%) 0/25 Visualisation of the median nerve 18/25 (72 %) 25/25 (100 %) 25/25 (100 %) 25/25 (100 %) Identification of the radial neurovascular bundle (grey scale and Doppler sonography) 25/25 (100 %) 25/25 (100 %) 25/25 (100 %) 25/25 (100 %)

structures) the median nerve was documented by ultrasound in only 18 of 25 children (72 %). Other sonographic characteristics of the distal forearm region are presented in Table 1.

\* data are for n = 100 children (25 per group)

1 mean ± standard deviation; range are shown in parenthesis.

Table 1. Sonographic characteristics of the distal forearm region in children and adolescents according to age groups\*.

Distal Forearm Sonography 51

Fig. 3.  The linear 12-5 MHz

Fig. 4.  Linear 12-5 MHz probe is placed along the distal end of the radius. Colour coded duplex ultrasound study shows

vessel supplying the secondary ossification centre of the epiphysis.

probe is placed along the distal radius.

The majority of children in group 2 (4 - 6 years old) showed weak punctuated echoes (76 %) or weak echogenic bands (16 %) within an anechoic growth plate cartilage(Fig.2). The median nerve was seen in all children on grey scale sonography. On colour Doppler sonography, no vascularisation was noted in the cartilage of the distal radial epiphysis or growth plate.

Fig. 2. Boy, 5 years of age; palmar longitudinal transducer position. Linear 12-5 MHz probe is placed along the distal radius. Anechoic cartilage of the growth plate and epiphysis of the radius shows small hypoechoic band-like spot within the growth plate cartilage (solid arrow).

In group 3 children the proximal-distal diameter of the distal radial growth plate was smaller when compared to group 2 (mean values 2,19 mm; 2,65 mm respectively). The appearance of the distal radial growth plate cartilage and epiphyseal cartilage in group 3 children (7 - 11 years old) resembled the appearance in group 2 children (4 - 6 years old)(Fig. 3-8). No problems were encountered during visualisation of the median nerve in group 3 children. On colour Doppler sonography, no vascularization was found in the cartilage of the growth plate. However, in one of these children a vessel was visualized entering the cartilage of the epiphysis from the palmar aspect of the epiphysis of the distal radius(Fig. 4).

The majority of children in group 2 (4 - 6 years old) showed weak punctuated echoes (76 %) or weak echogenic bands (16 %) within an anechoic growth plate cartilage(Fig.2). The median nerve was seen in all children on grey scale sonography. On colour Doppler sonography, no vascularisation was noted in the cartilage of the distal radial epiphysis or

Fig. 2. Boy, 5 years of age; palmar longitudinal transducer position. Linear 12-5 MHz probe is placed along the distal radius. Anechoic cartilage of the growth plate and epiphysis of the radius shows small hypoechoic band-like spot within the growth plate cartilage (solid

In group 3 children the proximal-distal diameter of the distal radial growth plate was smaller when compared to group 2 (mean values 2,19 mm; 2,65 mm respectively). The appearance of the distal radial growth plate cartilage and epiphyseal cartilage in group 3 children (7 - 11 years old) resembled the appearance in group 2 children (4 - 6 years old)(Fig. 3-8). No problems were encountered during visualisation of the median nerve in group 3 children. On colour Doppler sonography, no vascularization was found in the cartilage of the growth plate. However, in one of these children a vessel was visualized entering the cartilage of the epiphysis from the palmar aspect of the epiphysis of the distal

growth plate.

arrow).

radius(Fig. 4).

Fig. 3.  The linear 12-5 MHz probe is placed along the distal radius.

Fig. 4.  Linear 12-5 MHz probe is placed along the distal end of the radius. Colour coded duplex ultrasound study shows vessel supplying the secondary ossification centre of the epiphysis.

Distal Forearm Sonography 53

Fig. 7.  Linear 12-5 MHz probe is placed along the distal radius. The epiphyseal and growth plate cartilage of the distal epiphysis of the radius appears anechoic. Extensor tendons are overlying the dorsal contour

Fig. 8. Boy, 10 years of age; dorsal transverse transducer position. Linear 12-5 MHz probe is

placed across Lister`s tubercle. The extensor tendons are shown.

of the distal radius.

Fig. 5.  Linear 12- 5 MHz probe is placed across the region of the growth plate of the distal radius. The cartilage of the growth plate of the radius appears anechoic.

Fig. 6.  Linear 12-5 MHz probe is placed across the metaphyseal area of the distal forearm. The extensor tendons are shown.

Fig. 5.  Linear 12-

Fig. 6.  Linear 12-5 MHz probe is placed across the metaphyseal area of the distal forearm. The extensor tendons are shown.

5 MHz probe is placed across the region of the growth plate of the distal radius. The

cartilage of the growth plate of the radius appears anechoic.

Fig. 7.  Linear 12-5 MHz probe is placed along the distal radius. The epiphyseal and growth plate cartilage of the distal epiphysis of the radius appears anechoic. Extensor tendons are overlying the dorsal contour of the distal radius.

Fig. 8. Boy, 10 years of age; dorsal transverse transducer position. Linear 12-5 MHz probe is placed across Lister`s tubercle. The extensor tendons are shown.

Distal Forearm Sonography 55

Fig. 10. Girl, 13 years of age; palmar transverse transducer position. Linear 12-5 MHz probe is placed across the distal metaphyseal-shaft junction of the forearm. Note the transverse muscle fibers of the pronator quadratus muscle. The median nerve and the flexor tendons

The distal growth plate of the radius can not be differentiated from the cartilage of the epiphysis ultrasonographically, unless the secondary ossification centre is already discernable within the distal radial epiphysis (Fig. 1). The sonomorphologic characteristics of the distal radial growth plate are shown in Table 1. Its width gradually decreases during further development (Tab.1). With increasing age of the child, the chondro-osseous junction bends towards the epiphysis or appears slightly interdentated. Approaching the age of fusion of the distal radial epiphysis, it is represented by a narrow gap in the cortical bone and repetitive echoes can be seen posterior to the surface of the growth plate (Fig. 11).

are visualized.

In 5 children of group 4 (12 - 18 years old) the distal radial epiphyseal growth plate was fused (20 %). In the remaining children, the epiphyseal growth plate cartilage was either anechoic (16 %)(Fig. 9) or anechoic with weak echogenic spots within the cartilage (64 %). The median nerve was identified by ultrasound in all children of group 4(Fig. 10). No vessels were noted penetrating the epiphyseal or growth plate cartilage at colour Doppler sonography.

During the growth period, characteristic morphologic changes occur within the distal part of the radius which can be sonographically observed. The secondary ossification centre within the distal radial epiphysis appears on plain x-ray images as late as three to eighteen months post partum. However, morphologic changes during ossification are clearly visible: firstly, towards the end of the first trimester, the epiphyseal centre shows an increasing echogenicity. Subsequently, a small epiphyseal area with high echogenity and dorsal ultrasound extinction develops.

Using colour coded duplex sonography, vessels supplying the epiphyseal cartilage during the first and second year of life can be visualised frequently(Fig.1). Ultrasonographically, the periosteum is visible as a hyperechoic thin band-like structure, separated from the cortical reflex by a thin hypoechoic line.

Fig. 9. Girl, 13 years of age; palmar longitudinal transducer position. Linear 12-5 MHz probe is placed along the distal radius. The cartilage interface sign is visible between the distal radius and the scaphoid articular cartilage.

In 5 children of group 4 (12 - 18 years old) the distal radial epiphyseal growth plate was fused (20 %). In the remaining children, the epiphyseal growth plate cartilage was either anechoic (16 %)(Fig. 9) or anechoic with weak echogenic spots within the cartilage (64 %). The median nerve was identified by ultrasound in all children of group 4(Fig. 10). No vessels were noted penetrating the epiphyseal or growth plate cartilage at colour Doppler

During the growth period, characteristic morphologic changes occur within the distal part of the radius which can be sonographically observed. The secondary ossification centre within the distal radial epiphysis appears on plain x-ray images as late as three to eighteen months post partum. However, morphologic changes during ossification are clearly visible: firstly, towards the end of the first trimester, the epiphyseal centre shows an increasing echogenicity. Subsequently, a small epiphyseal area with high echogenity and dorsal

Using colour coded duplex sonography, vessels supplying the epiphyseal cartilage during the first and second year of life can be visualised frequently(Fig.1). Ultrasonographically, the periosteum is visible as a hyperechoic thin band-like structure, separated from the cortical

Fig. 9. Girl, 13 years of age; palmar longitudinal transducer position. Linear 12-5 MHz probe is placed along the distal radius. The cartilage interface sign is visible between the distal

sonography.

ultrasound extinction develops.

reflex by a thin hypoechoic line.

radius and the scaphoid articular cartilage.

Fig. 10. Girl, 13 years of age; palmar transverse transducer position. Linear 12-5 MHz probe is placed across the distal metaphyseal-shaft junction of the forearm. Note the transverse muscle fibers of the pronator quadratus muscle. The median nerve and the flexor tendons are visualized.

The distal growth plate of the radius can not be differentiated from the cartilage of the epiphysis ultrasonographically, unless the secondary ossification centre is already discernable within the distal radial epiphysis (Fig. 1). The sonomorphologic characteristics of the distal radial growth plate are shown in Table 1. Its width gradually decreases during further development (Tab.1). With increasing age of the child, the chondro-osseous junction bends towards the epiphysis or appears slightly interdentated. Approaching the age of fusion of the distal radial epiphysis, it is represented by a narrow gap in the cortical bone and repetitive echoes can be seen posterior to the surface of the growth plate (Fig. 11).

Distal Forearm Sonography 57

In the hands of an experienced clinician using high frequency linear probes (more than 7 MHz), and adequate equipment, ultrasonography is a suitable technique for the investigation of cortical structures and surrounding soft tissues3-6. Furthermore, there is the substantial advantage of dynamic examination, direct comparison to the contralateral limb and the lack of exposure to radiation5-7. As in most joints, the use of linear probes is also preferable in the distal radius region7. As the soft tissue coverage is relatively thin, high resolution and low penetration is preferable for this examination. Depending on the resolution capacity, the use of an echo-free standoff pad to increase the distance to the tissue

In the longitudinal plane, tendons are depicted as longitudinal structures of high echogenicity with parallel echos3,5-7. In the horizontal plane, a punctuated reticular, hyperechoic tendon fibre structure is seen3,5-7. The examiner must take into account that the probe is aligned parallel to the tendon and the sonographic impulse is directed orthograde to the tendon7. When high frequency probes are used, deviations of 15° from the orthograde transducer position can change the echogenicity of tendons and nerves. Because of these physical phenomena, smooth circular surfaces of small dimension create a reflex only in that area, where the sound impulse arrives perpendicularly. Furthermore, if there is no "orthograde" alignment, the whole tendon diameter shows lower echogenicity7. Thus, not all tendons are clearly depicted in a transverse plane and areas of low echogenicity are found. These artefacts have to be kept in mind especially in the evaluation of pathologic changes of the peritendinous tissue and fluid accumulation within the tendon sheaths and the carpal canal, respectively7. However, even tendon aplasia in children can be accurately diagnosed

Furthermore, it is very important to use an "orthograde" position of the probe towards the evaluated structure (bone, tendon) (Fig. 5,7). In most cases, it is impossible to find this

Ultrasonography can be considered as the gold standard for evaluation of tendinous disorders3,5-7, localization and differentiation of fluid accumulations (possibility of sonographically guided punction)3, diagnosis of intraarticular effusions10, diagnosis of soft

It is especially important in children to consider the radiation exposure during radiographic diagnosis and follow-up of fractures. At ultrasonography the metaphyseal cortex is easily identified as a marked hyperechoic line with a dorsal acoustic shadow. Subsequently, fractures can be clearly evaluated, as well(Fig.11). So, ultrasonography is a reliable tool for the follow-up of distal fractures of the radius in children. In addition to the evaluation of the fracture gap, the course of fracture consolidation supplemented by stability tests and dynamic examination can also be evaluated and estimated14. So the follow-up of fractures could be a special field of interest for ultrasonography in the children in the future. However, primary radiologic diagnosis will not be replaced by sonography, especially in fractures of the distal forearm region. It must be kept in mind, that obtaining an ultrasound study is more time consuming compared to obtaining X-rays. Ultrasonography is especially

**6. Ultrasound applications in the region of the distal forearm**

can be helpful7.

using this ultrasonographic technique9.

position both for tendons and bones within one singular plane.

tissue tumors (ganglia, cysts, neuromas)3,4,11 and foreign bodies5,12,13.

Fig. 11. Girl, 13 years of age; undisplaced Salter-Harris type II epiphysiolysis; This fracture is considered stable because there is no break in the dorsal metaphyseal corticalis of the radius. Longitudinal dorsal transducer position. Repetitive echoes are visible within the central part of the growth plate( marked by solid arrow).

Depending mainly on the gender of the individual, fusion of the distal radial epiphysis occurs between 15 and 18 years of age. During fusion of the distal radial physis towards the end of skeletal growth the gap like anechoic remnant of the physis was found to be wider on the dorsal side when compared to the palmar side of the radius.

*General findings:* At dynamic investigation using limited, slow movements of the wrist joint a characteristic change in shape and position of the median nerve was noted, whereas the palmaris longus tendon and flexor carpi radialis tendon showed longitudinal movements and nearly no change in shape. In contrast to the fine reticular fibrillary pattern of the flexor tendons the median nerve showed a coarse-grained fibrillary pattern.

After the occurrence of the hyperechoic secondary ossification centre of the distal radius the width of the dorsal epiphyseal cartilage was 1 - 2 mm wider when compared to the width of the volar epiphyseal cartilage (measurements taken between the dorsal contour of the secondary ossification centre and dorsal boarder of the epiphyseal cartilage); and the volar contour of the secondary ossification centre and volar contour of the epiphyseal cartilage (respectively).

Fig. 11. Girl, 13 years of age; undisplaced Salter-Harris type II epiphysiolysis; This fracture is considered stable because there is no break in the dorsal metaphyseal corticalis of the radius. Longitudinal dorsal transducer position. Repetitive echoes are visible within the central part

Depending mainly on the gender of the individual, fusion of the distal radial epiphysis occurs between 15 and 18 years of age. During fusion of the distal radial physis towards the end of skeletal growth the gap like anechoic remnant of the physis was found to be wider on

*General findings:* At dynamic investigation using limited, slow movements of the wrist joint a characteristic change in shape and position of the median nerve was noted, whereas the palmaris longus tendon and flexor carpi radialis tendon showed longitudinal movements and nearly no change in shape. In contrast to the fine reticular fibrillary pattern of the flexor

After the occurrence of the hyperechoic secondary ossification centre of the distal radius the width of the dorsal epiphyseal cartilage was 1 - 2 mm wider when compared to the width of the volar epiphyseal cartilage (measurements taken between the dorsal contour of the secondary ossification centre and dorsal boarder of the epiphyseal cartilage); and the volar contour of the secondary ossification centre and volar contour of the epiphyseal cartilage

of the growth plate( marked by solid arrow).

(respectively).

the dorsal side when compared to the palmar side of the radius.

tendons the median nerve showed a coarse-grained fibrillary pattern.

#### **6. Ultrasound applications in the region of the distal forearm**

In the hands of an experienced clinician using high frequency linear probes (more than 7 MHz), and adequate equipment, ultrasonography is a suitable technique for the investigation of cortical structures and surrounding soft tissues3-6. Furthermore, there is the substantial advantage of dynamic examination, direct comparison to the contralateral limb and the lack of exposure to radiation5-7. As in most joints, the use of linear probes is also preferable in the distal radius region7. As the soft tissue coverage is relatively thin, high resolution and low penetration is preferable for this examination. Depending on the resolution capacity, the use of an echo-free standoff pad to increase the distance to the tissue can be helpful7.

In the longitudinal plane, tendons are depicted as longitudinal structures of high echogenicity with parallel echos3,5-7. In the horizontal plane, a punctuated reticular, hyperechoic tendon fibre structure is seen3,5-7. The examiner must take into account that the probe is aligned parallel to the tendon and the sonographic impulse is directed orthograde to the tendon7. When high frequency probes are used, deviations of 15° from the orthograde transducer position can change the echogenicity of tendons and nerves. Because of these physical phenomena, smooth circular surfaces of small dimension create a reflex only in that area, where the sound impulse arrives perpendicularly. Furthermore, if there is no "orthograde" alignment, the whole tendon diameter shows lower echogenicity7. Thus, not all tendons are clearly depicted in a transverse plane and areas of low echogenicity are found. These artefacts have to be kept in mind especially in the evaluation of pathologic changes of the peritendinous tissue and fluid accumulation within the tendon sheaths and the carpal canal, respectively7. However, even tendon aplasia in children can be accurately diagnosed using this ultrasonographic technique9.

Furthermore, it is very important to use an "orthograde" position of the probe towards the evaluated structure (bone, tendon) (Fig. 5,7). In most cases, it is impossible to find this position both for tendons and bones within one singular plane.

Ultrasonography can be considered as the gold standard for evaluation of tendinous disorders3,5-7, localization and differentiation of fluid accumulations (possibility of sonographically guided punction)3, diagnosis of intraarticular effusions10, diagnosis of soft tissue tumors (ganglia, cysts, neuromas)3,4,11 and foreign bodies5,12,13.

It is especially important in children to consider the radiation exposure during radiographic diagnosis and follow-up of fractures. At ultrasonography the metaphyseal cortex is easily identified as a marked hyperechoic line with a dorsal acoustic shadow. Subsequently, fractures can be clearly evaluated, as well(Fig.11). So, ultrasonography is a reliable tool for the follow-up of distal fractures of the radius in children. In addition to the evaluation of the fracture gap, the course of fracture consolidation supplemented by stability tests and dynamic examination can also be evaluated and estimated14. So the follow-up of fractures could be a special field of interest for ultrasonography in the children in the future. However, primary radiologic diagnosis will not be replaced by sonography, especially in fractures of the distal forearm region. It must be kept in mind, that obtaining an ultrasound study is more time consuming compared to obtaining X-rays. Ultrasonography is especially

Distal Forearm Sonography 59

High frequency ultrasonography can yield reliable information about the distal radial epiphysis and growth plate, the median nerve and the forearm tendons. The sonographic features of the normal distal forearm during growth described herein may hopefully

The authors want to thank Mrs. Helen Parker, Nottingham, GB for assistance in the

No financial support for this investigation was given to any of the authors or their

[1] Cohen E, Blankstein A, Rosenstock M, Atou D. Greenstick fractures of distal radius

[2] Hübner U, Schlicht W, Outzen S, Barthel M, Halsband H. Ultrasound in the diagnosis of

[4] Fornage BD. Peripheral nerves of the extremities: imaging with US. Radiology

[5] Fornage BD, Schernberg FL, Rifkin MD. Ultrasound of the hand. Radiology

[6] Fornage BD, Rifkin MD. Ultrasound examination of the hand and foot. Radiol Clin

[7] Fornage BD: The hypoechoic normal tendon – a pitfall. J Ultrasound Med 1987;6:19-

[8] Nakamichi K, Tachibana S. Transverse sliding of the median nerve beneath the flexor

[9] Grechenig W, Mayr J, Clement H, Peicha G. Aplasia of the flexor tendon of the fifth

[10] Fornage BD. Soft-tissue changes in the hand in rheumatoid arthritis: evaluation with

[11] Cardinal E, Buckwalter KA, Braunstein EM, Mik AD. Occult dorsal carpal ganglion: comparison of ultrasound and MR imaging. Radiology 1994;193: 259-262 [12] Jacobson JA, Powell A, Craig J, Bouffard J, Van Holsbeeck M. Wooden foreign bodies in

[13] Fornage BD, Schernberg FL. Sonographic preoperative localization of a foreign body in

finger in a new born child – sonographic diagnosis. Eur Surg 2002; Suppl. 184 : 114-

[3] Ferrara Ma, Marcelis S. Ultrasound of the wrist. J Belge Radiol 1997;80:78-80

followed-up by ultrasound - a prospective study. Akt Traumatol 2000; 30:227-

contribute to a more widespread use of ultrasound in this anatomic region.

fractures in children. JBJS 2000; 82B:1170 –3

retinaculum. J Hand Surg 1992; 17B: 213-216

soft-tissue: detection at US. Radiology 1998; 206: 45-48

US. Radiology 1989; 173: 735-737

the hand. J Ultrasound Med 1987;6:217-9

**7. Conclusion**

institutions.

**9. References**

229

22

115.

1988;167:179-18

1985;155:785-8

North Am 1988;26:109-29

**8. Acknowledgement**

translation of the manuscript.

suitable for the follow-up of fractures in children and adolescents, when there is no closed plaster cast applied. The examiner has to pay special attention to the investigation of the periosteum, as the width of the hypoechoic line between the periosteum and bone surface is of outstanding clinical relevance in the diagnosis of subperiosteal fluid accumulation, for example in acute osteomyelitis(Fig. 12). Therefore, it should only be evaluated in comparison to the contralateral limb. Due to the stable affixation of periosteum / perichondrium at the region of the growth plate, compared to the diaphyseal and metaphyseal region, subperiosteal fluid accumulations are only found in the metaphyseal and diaphyseal region (Fig. 12).

Fig. 12. Longitudinal distal dorsal-radial ultrasound scan in a boy aged 9 years suffering from acute osteomyelitis of the distal radius. The periosteum is detached from the corticalis of the distal radius, there is subperiosteal hypoechoic fluid accumulation with some low level echos. At operation acute purulent osteomyelitis of the distal radius complicated by subperiostal abscess formation and phlegmonous infiltration of the intermuscular spaces between the forearm extensor muscles was confirmed.

However, difficulties might arise during investigation of intra- and extraarticular pathologic changes of low echogenicity. For example, the differentiation of purely chondral articulating surfaces is facilitated only by the depiction of the "cartilage interface sign", a smooth, hypoechoic borderline reflex at the region of the articular gap(Fig.9).

#### **7. Conclusion**

58 Orthopedic Surgery

suitable for the follow-up of fractures in children and adolescents, when there is no closed plaster cast applied. The examiner has to pay special attention to the investigation of the periosteum, as the width of the hypoechoic line between the periosteum and bone surface is of outstanding clinical relevance in the diagnosis of subperiosteal fluid accumulation, for example in acute osteomyelitis(Fig. 12). Therefore, it should only be evaluated in comparison to the contralateral limb. Due to the stable affixation of periosteum / perichondrium at the region of the growth plate, compared to the diaphyseal and metaphyseal region, subperiosteal fluid accumulations are only found in the metaphyseal

Fig. 12. Longitudinal distal dorsal-radial ultrasound scan in a boy aged 9 years suffering from acute osteomyelitis of the distal radius. The periosteum is detached from the corticalis of the distal radius, there is subperiosteal hypoechoic fluid accumulation with some low level echos. At operation acute purulent osteomyelitis of the distal radius complicated by subperiostal abscess formation and phlegmonous infiltration of the intermuscular spaces

However, difficulties might arise during investigation of intra- and extraarticular pathologic changes of low echogenicity. For example, the differentiation of purely chondral articulating surfaces is facilitated only by the depiction of the "cartilage interface sign", a smooth,

between the forearm extensor muscles was confirmed.

hypoechoic borderline reflex at the region of the articular gap(Fig.9).

and diaphyseal region (Fig. 12).

High frequency ultrasonography can yield reliable information about the distal radial epiphysis and growth plate, the median nerve and the forearm tendons. The sonographic features of the normal distal forearm during growth described herein may hopefully contribute to a more widespread use of ultrasound in this anatomic region.

#### **8. Acknowledgement**

The authors want to thank Mrs. Helen Parker, Nottingham, GB for assistance in the translation of the manuscript.

No financial support for this investigation was given to any of the authors or their institutions.

#### **9. References**

**4**

*Poland*

**Limited Hand Surgery in Epidermolysis Bullosa**

Epidermolysis bullosa (EB) is the name given to a group of rare congenital diseases with similar clinical symptoms. Their common feature is the susceptibility of the epithelia and skin to injury. Even minor shear forces from the sideways stretching of the skin contribute to epidermal tearing with blister formation. These blisters turn into wounds. In the most severe forms of the disease, wounds dominate on the skin and mucosa, leading to early death or

Four main types of EB are distinguished based on the level of the ultrastructural defect in the area of the basement membrane zone (BMZ) (Fine, 2010). In epidermolysis bullosa simplex (EBS), the epithelium separates at the level of the basal cells, most commonly as a result of damage to keratin 5 and 14. In junctional epidermolysis bullosa (JEB), the level of the epithelial separation is in the lamina lucida and results from laminin -332, collagen XVII or integrin 64 defects. In dystrophic epidermolysis bullosa (DEB), the epithelium is damaged below the lamina densa as a result of an incorrectly structured collagen VII building the anchoring fibrils. In Kindler syndrome, protein damage may affect several layers simultaneously. Apart from the epidermis, any basement membrane epithelia may become damaged. Most typically affected are the oral cavity and the esophageal and upper respiratory tract mucosa. The disease may also affect the tissues not covered with

The extent of symptoms involving the skin is varied and not completely characteristic. In the localized EBS form (EBS – loc) they manifest mainly as blisters on the hands and feet. In both generalized forms of EBS the blisters are located throughout the whole body. The risk of pseudosyndactyly in Dowling – Meara (EBS-DM) is estimated at 3% at or after the age of 3 (Fine et al., 2005). In the, non-Dowling-Meara (EBS-nDM), the blisters, although

Among the several different subtypes of junctional epidermolysis bullosa (JEB), two are generalized. The highest risk of death accompanies the Herlitz (JEB-H) form. In these patients, death stems from severe infectious complications or multi-organ failure. The predominating symptoms in this type are non-healing wounds covered with granulation tissue. It appears in the first months of life and can involve the upper respiratory tract. In the

epithelium, such as tooth enamel, which is damaged in all the JEB forms.

generalized, tend to spare the hands and feet (Fine, 2010).

**1. Introduction**

**1.1 Types of EB**

deformations from scars and contractures.

Bartlomiej Noszczyk and Joanna Jutkiewicz-Sypniewska

*Medical Center for Postgraduate Education*

[14] Dias JJ, Hui ACW, Lamont AC. Real time ultrasonography in the assessment of movement at the site of a scaphoid fracture non-union. J Hand Surg 1994;19B: 498- 504

## **Limited Hand Surgery in Epidermolysis Bullosa**

Bartlomiej Noszczyk and Joanna Jutkiewicz-Sypniewska *Medical Center for Postgraduate Education Poland*

#### **1. Introduction**

60 Orthopedic Surgery

[14] Dias JJ, Hui ACW, Lamont AC. Real time ultrasonography in the assessment of

504

movement at the site of a scaphoid fracture non-union. J Hand Surg 1994;19B: 498-

Epidermolysis bullosa (EB) is the name given to a group of rare congenital diseases with similar clinical symptoms. Their common feature is the susceptibility of the epithelia and skin to injury. Even minor shear forces from the sideways stretching of the skin contribute to epidermal tearing with blister formation. These blisters turn into wounds. In the most severe forms of the disease, wounds dominate on the skin and mucosa, leading to early death or deformations from scars and contractures.

#### **1.1 Types of EB**

Four main types of EB are distinguished based on the level of the ultrastructural defect in the area of the basement membrane zone (BMZ) (Fine, 2010). In epidermolysis bullosa simplex (EBS), the epithelium separates at the level of the basal cells, most commonly as a result of damage to keratin 5 and 14. In junctional epidermolysis bullosa (JEB), the level of the epithelial separation is in the lamina lucida and results from laminin -332, collagen XVII or integrin 64 defects. In dystrophic epidermolysis bullosa (DEB), the epithelium is damaged below the lamina densa as a result of an incorrectly structured collagen VII building the anchoring fibrils. In Kindler syndrome, protein damage may affect several layers simultaneously. Apart from the epidermis, any basement membrane epithelia may become damaged. Most typically affected are the oral cavity and the esophageal and upper respiratory tract mucosa. The disease may also affect the tissues not covered with epithelium, such as tooth enamel, which is damaged in all the JEB forms.

The extent of symptoms involving the skin is varied and not completely characteristic. In the localized EBS form (EBS – loc) they manifest mainly as blisters on the hands and feet. In both generalized forms of EBS the blisters are located throughout the whole body. The risk of pseudosyndactyly in Dowling – Meara (EBS-DM) is estimated at 3% at or after the age of 3 (Fine et al., 2005). In the, non-Dowling-Meara (EBS-nDM), the blisters, although generalized, tend to spare the hands and feet (Fine, 2010).

Among the several different subtypes of junctional epidermolysis bullosa (JEB), two are generalized. The highest risk of death accompanies the Herlitz (JEB-H) form. In these patients, death stems from severe infectious complications or multi-organ failure. The predominating symptoms in this type are non-healing wounds covered with granulation tissue. It appears in the first months of life and can involve the upper respiratory tract. In the

Limited Hand Surgery in Epidermolysis Bullosa 63

Despite many attempts to use modern technology or to change surgical techniques, the recurrence of deformation is unavoidable and commonplace. Because every epidermal cell in the body has the same genetic defect, local therapy may result in only temporary improvement. Hand procedures may become necessary in 61% of RDEB patients. In this group, 5 or more operations are presumed to be the norm (Fine et al., 2005). It is estimated that recurrences appear, on average, every 2.4 years (1 to 5 years) (Terrill et al., 1992). In the experience of many surgeons, this time interval is even shorter. Furthermore, the time required for full wound healing after surgery for advanced deformations may reach 6 to 8 weeks (Fivenson et al., 2003). It is frequently observed, therefore, that patients spend a considerable period of potentially good hand efficiency in the hospital receiving dressing

In order to support patients' independence and psychomotoric development, operations could be recommended early, when deformations may no longer be prevented by physiotherapy and splinting. In practice, however, patients typically present to the hospital when they or their parents are aware that surgery may no longer be postponed. If the deformations are advanced, the extent of the needed surgery influences the postoperative course. Patients then become discouraged by repeated hospitalizations and less likely to

Removal of the thick epidermis that creates the cocoon is ineffective. In the beginning of the operation however, the hand may be "de-cocooned" using epidermal degloving technique. It outlines the area where direct force may be applied and saves intact epidermis beyond surgical manipulation (Ladd et al., 1996). This maneuver helps to apply manual traction to the tips of the affected fingers, which may be extended after a sharp release on the palmar side of the contracted joints. Other authors prefer to dispense with degloving, emphasizing however the need to apply some force in order to extend the contracted digits (Ciccarelli et al., 1995). Although such forceful manipulation destroys epidermis, the same principle applies to the first web space, where pseudosyndactyly often involves the adductor fascia and the first dorsal interosseous muscles (Ladd et al. 1996). The abduction and extension of the fingers is then maintained by Kirshner wires. Others have suggested the continuous use of acrylic gloves for three months following surgery (Terrill et al., 1992). Today modern splints from thermplastic materials are recommended, that may be easily remodeled or

The most thorough review and practical evaluation of surgical methods in RDEB was presented recently (Bernardis & Box, 2010). Currently, most authors prefer to avoid using force during joint extension to prevent neurovascular bundle injury and subluxation. It has also been suggested that the full release of contractures may be not useful or necessary. This procedure creates extensive defects, leaving a bare dermal surface and uncovered neurovascular bundles, which require grafting. Full thickness skin grafts (FTSGs) are often used in deeper defects. The amount of full-thickness dermis suitable for harvesting is limited, however. Therefore, other solutions have been proposed for the remaining surface of superficial defects. Various opinions exist over the use of split skin grafts (SSGs). The

**1.4 Repeated hand surgery – The problem in RDEB**

replacements. Most of these procedures require general anesthesia.

cooperate in the future.

**1.5 Common methods in RDEB surgery**

discarded after they become polluted.

more common non-Herlitz (JEB-nH) form, the blisters are generalized and occur on the hands as well, often accompanied by atrophic scars and atrophic nails. The risk of pseudosyndactyly increases with advanced age.

The dystrophic type of epidermolysis bullosa (DEB) is divided into two main subtypes, depending on the mode of inheritance. In both, the generalized blisters lead to the formation of wounds, which typically heal and scar. The autosomal dominant form (generalized dominant dystrophic - DDEB) is characterized by symptoms of a slightly lower intensity. The risk of pseudosyndactyly is just 3% by the age of 40 (Fine et al., 2005). The forms with autosomal recessive inheritance have a more severe course. The most severe one, described as generalized recessive dystrophic epidermolysis bullosa (RDEB), occurs in 2 out of one million births (Pfendner et al., 2001). The disease activity in RDEB is extremely high in nearly every anatomic site (Devries et al., 2004).

#### **1.2 Hand deformations in RDEB**

The characteristic features of generalized RDEB are intensifying contractures and pseudosyndactyly affecting the hands and feet. Their risk by the age of 20 is estimated at 98% (Fine et al., 2005). In time, they lead the hands to contract into a cocoon. If such a severe disability appears in early childhood, it may hinder or preclude normal development. The mechanism of pseudosyndactyly, finger contractures and the development of the characteristic mitten deformities is not completely clear. The gradual web creep in children not undergoing surgery is probably facilitated by fine blisters and recurrent epidermal injuries. In the early postoperative period in operated patients, this complication typically stems from the adhesion of contacting wound surfaces. However, in all patients, this process also develops on the hands and feet under the influence of unknown causes. Observations suggest that the epithelium has a natural tendency towards migration "with shortcuts". It gradually retreats from the indented web spaces, leaving non-separated surfaces of the dermis. As a result, this retreat leads the dermal layers to stick under the surface of the forming cocoon. The contacting layers usually do not fully adhere. Hence, the pseudosyndactyly results. Initially, despite the adhesions, the fingers remain extended. During this period, the epithelium forming the cocoon continues to overgrow, forming a thick stratum corneum. Its structure at this time resembles paper, and it quickly becomes too rigid to allow movement. This rigidity leads to habitual finger bending and further cocoon overgrowth, which soon involves the entire hand.

#### **1.3 Other complications of RDEB**

Effective intervention for RDEB patients requires multidisciplinary treatment. Apart from dermatological care, the majority of patients require pediatric care due to multifactorial anemia and malnutrition. In some patients, kidney failure and cardiomyopathy develop, both of which may increase the risk of death. Among the other symptoms of note are corneal injuries requiring ophthalmologic treatment and esophageal stenosis, which can develop in early childhood. Sometimes, these are accompanied by microstomy and ankyloglossia. Difficulties with swallowing quickly contribute to malnutrition and growth retardation. The treatment requires esophageal dilation or gastrostomy. Some RDEB patients are diagnosed with squamous cell carcinoma by the age of 20. This cancer is a common cause of death in adults. The risk of this neoplasm is estimated at 7.5% by the age of 20 and 67.8% after the age of 35 (Fine & Mellerio, 2009a).

more common non-Herlitz (JEB-nH) form, the blisters are generalized and occur on the hands as well, often accompanied by atrophic scars and atrophic nails. The risk of

The dystrophic type of epidermolysis bullosa (DEB) is divided into two main subtypes, depending on the mode of inheritance. In both, the generalized blisters lead to the formation of wounds, which typically heal and scar. The autosomal dominant form (generalized dominant dystrophic - DDEB) is characterized by symptoms of a slightly lower intensity. The risk of pseudosyndactyly is just 3% by the age of 40 (Fine et al., 2005). The forms with autosomal recessive inheritance have a more severe course. The most severe one, described as generalized recessive dystrophic epidermolysis bullosa (RDEB), occurs in 2 out of one million births (Pfendner et al., 2001). The disease activity in RDEB is extremely high in

The characteristic features of generalized RDEB are intensifying contractures and pseudosyndactyly affecting the hands and feet. Their risk by the age of 20 is estimated at 98% (Fine et al., 2005). In time, they lead the hands to contract into a cocoon. If such a severe disability appears in early childhood, it may hinder or preclude normal development. The mechanism of pseudosyndactyly, finger contractures and the development of the characteristic mitten deformities is not completely clear. The gradual web creep in children not undergoing surgery is probably facilitated by fine blisters and recurrent epidermal injuries. In the early postoperative period in operated patients, this complication typically stems from the adhesion of contacting wound surfaces. However, in all patients, this process also develops on the hands and feet under the influence of unknown causes. Observations suggest that the epithelium has a natural tendency towards migration "with shortcuts". It gradually retreats from the indented web spaces, leaving non-separated surfaces of the dermis. As a result, this retreat leads the dermal layers to stick under the surface of the forming cocoon. The contacting layers usually do not fully adhere. Hence, the pseudosyndactyly results. Initially, despite the adhesions, the fingers remain extended. During this period, the epithelium forming the cocoon continues to overgrow, forming a thick stratum corneum. Its structure at this time resembles paper, and it quickly becomes too rigid to allow movement. This rigidity leads to habitual

finger bending and further cocoon overgrowth, which soon involves the entire hand.

Effective intervention for RDEB patients requires multidisciplinary treatment. Apart from dermatological care, the majority of patients require pediatric care due to multifactorial anemia and malnutrition. In some patients, kidney failure and cardiomyopathy develop, both of which may increase the risk of death. Among the other symptoms of note are corneal injuries requiring ophthalmologic treatment and esophageal stenosis, which can develop in early childhood. Sometimes, these are accompanied by microstomy and ankyloglossia. Difficulties with swallowing quickly contribute to malnutrition and growth retardation. The treatment requires esophageal dilation or gastrostomy. Some RDEB patients are diagnosed with squamous cell carcinoma by the age of 20. This cancer is a common cause of death in adults. The risk of this neoplasm is estimated at 7.5% by the age

pseudosyndactyly increases with advanced age.

nearly every anatomic site (Devries et al., 2004).

**1.2 Hand deformations in RDEB**

**1.3 Other complications of RDEB**

of 20 and 67.8% after the age of 35 (Fine & Mellerio, 2009a).

#### **1.4 Repeated hand surgery – The problem in RDEB**

Despite many attempts to use modern technology or to change surgical techniques, the recurrence of deformation is unavoidable and commonplace. Because every epidermal cell in the body has the same genetic defect, local therapy may result in only temporary improvement. Hand procedures may become necessary in 61% of RDEB patients. In this group, 5 or more operations are presumed to be the norm (Fine et al., 2005). It is estimated that recurrences appear, on average, every 2.4 years (1 to 5 years) (Terrill et al., 1992). In the experience of many surgeons, this time interval is even shorter. Furthermore, the time required for full wound healing after surgery for advanced deformations may reach 6 to 8 weeks (Fivenson et al., 2003). It is frequently observed, therefore, that patients spend a considerable period of potentially good hand efficiency in the hospital receiving dressing replacements. Most of these procedures require general anesthesia.

In order to support patients' independence and psychomotoric development, operations could be recommended early, when deformations may no longer be prevented by physiotherapy and splinting. In practice, however, patients typically present to the hospital when they or their parents are aware that surgery may no longer be postponed. If the deformations are advanced, the extent of the needed surgery influences the postoperative course. Patients then become discouraged by repeated hospitalizations and less likely to cooperate in the future.

#### **1.5 Common methods in RDEB surgery**

Removal of the thick epidermis that creates the cocoon is ineffective. In the beginning of the operation however, the hand may be "de-cocooned" using epidermal degloving technique. It outlines the area where direct force may be applied and saves intact epidermis beyond surgical manipulation (Ladd et al., 1996). This maneuver helps to apply manual traction to the tips of the affected fingers, which may be extended after a sharp release on the palmar side of the contracted joints. Other authors prefer to dispense with degloving, emphasizing however the need to apply some force in order to extend the contracted digits (Ciccarelli et al., 1995). Although such forceful manipulation destroys epidermis, the same principle applies to the first web space, where pseudosyndactyly often involves the adductor fascia and the first dorsal interosseous muscles (Ladd et al. 1996). The abduction and extension of the fingers is then maintained by Kirshner wires. Others have suggested the continuous use of acrylic gloves for three months following surgery (Terrill et al., 1992). Today modern splints from thermplastic materials are recommended, that may be easily remodeled or discarded after they become polluted.

The most thorough review and practical evaluation of surgical methods in RDEB was presented recently (Bernardis & Box, 2010). Currently, most authors prefer to avoid using force during joint extension to prevent neurovascular bundle injury and subluxation. It has also been suggested that the full release of contractures may be not useful or necessary. This procedure creates extensive defects, leaving a bare dermal surface and uncovered neurovascular bundles, which require grafting. Full thickness skin grafts (FTSGs) are often used in deeper defects. The amount of full-thickness dermis suitable for harvesting is limited, however. Therefore, other solutions have been proposed for the remaining surface of superficial defects. Various opinions exist over the use of split skin grafts (SSGs). The

Limited Hand Surgery in Epidermolysis Bullosa 65

intestinal epithelium, and insufficient food supply caused by blisters in the mouth and esophageal stenosis. The progressing stenosis hinders the intake of liquids and the swallowing of saliva. Attempts to insert gastric tubes are often associated with skin and mucosal injuries. Confirmed stenosis should rather be an indication for esophageal dilation. The recommended method is contrast X-ray or fluoroscopic guidance balloon dilation. In the population of patients with RDEB, approximately one-third require at least one such procedure. In many patients, the stenosis recurs. The mean time between subsequent

In greater deficiencies or independently of esophageal dilation, gastrostomy should be considered. In particular, during the preoperative period, it enables easy supplementation of food taken orally. It should be emphasized that attempts to insert percutaneous endoscopic gastrostomy (PEG) tubes. These expose the patient to unnecessary risk. In the authors' center, laparotomy is performed under general anesthesia, without intubation. Such a choice

Apart from food deficiencies, anemia also requires management. Many patients with RDEB are hospitalized with very low hemoglobin values. This is, to a large extent, caused by iron deficiency, associated with both insufficient supply in food and poor absorption in the intestine. Anemia in these patients also results from chronic inflammation associated with healing (Fine & Mellerio, 2009a). In a large portion of patients, despite the treatment with oral preparations, the hemoglobin level remains below 8 g/dl. With these levels, modern intravenous preparations are believed to be more effective. In the authors' center, Venofer is administered. This substitute has, thus far, prevented the need for treatment with blood, even at hemoglobin values below 6 g/dl. However, it should be emphasized that such a low hemoglobin is recognized as an indication for transfusion. Recently, erythropoietin has also

been used, although there have yet been no studies indicating its clear advantage.

In preparation for surgery, local infections accompanying the non-healing wounds should also be considered for treatment. The patients admitted for hand surgery often have old wounds in other body regions. It is emphasized that the distinction between the colonization of such wounds and infection should be made based on the clinical picture and not the culture results. Systemic treatment is rarely required. However, the presence of group A streptococcus may constitute an indication for antibiotic use (Brandling-Bennett & Morel, 2010). The growth of beta – hemolytic streptococcus is also a contraindication for hand surgery (Bernardis & Box, 2010). The majority of the other microorganisms present in non-healing wounds do not affect the planning of surgery. Fungal infections in the mouth do not constitute a contraindication either. *Candida albicans* and *Candida parapsilosis* are both common florae of non-healing wounds in patients with EB (Brandling-Bennett &

Before elective surgery, it is important to assess comorbid conditions. In over 75% of patients with RDEB, gastroesophageal reflux disease (GERD) is observed (Fine & Mellerio, 2009b). This condition increases the risk of choking during intubation. In the majority of

dilations is estimated at 1 to 2 years (Fine & Mellerio, 2009b).

also rules out laparoscopic gastrostomy.

**2.1.3 Skin infections**

Morel, 2010).

**2.2 Preparation for anesthesia**

harvesting of SSGs is associated with the creation of dermal defects in the donor area that often become difficult-to-heal wounds. In addition, the use of dermatome can deepithelize the skin adjacent to the planned donor site and can destroy the graft epidermis, usually rendering it useless (Bernardis & Box, 2010). In such cases, leaving the wounds on the lateral surfaces of the fingers and on the palmar surface of the hand without any biological dressings may be an equally good solution. Some authors claim that this technique does not prolong the healing time similarly compared with any other methods (Ciccarelli et al., 1995).

Attempts to use allogeneic dermal substitutes have not produced clear results. Allogeneic culture skin substitutes (Eisenberg & Lleewelyn, 1998) and Apligraf (Fivenson et al., 2003) have been used. Although the time to recurrence was increased in allogeneic skin patients, the results for Apligraf were unclear. Some modern dressings were also used, but these did not reduce the duration of healing or the number of weekly dressing changes performed under general anesthesia (Jutkiewicz et al., 2010). All these methods, however, enabled the avoidance of dermatome use on the fragile epidermal surface.

#### **2. Limited hand surgery in RDEB**

#### **2.1 Preparation for surgery**

#### **2.1.1 Basic recommendations**

In the modern treatment of EB, a great degree of significance is placed on the establishment of centers that enable contact with a team of specialists experienced in managing this rare disease (Pohla-Gubo & Hintner, 2010). Pediatric surgeons unfamiliar with EB may make errors stemming from a lack of knowledge of basic care principles. The following recommendations are designed to help practitioners avoid iatrogenic skin injuries:

#### **2.1.2 Metabolic disorders**

Metabolic disorders in RDEB may require treatment in the preparation for surgery. These stem from increased energy expenditure, impaired absorption due to defects of the small

harvesting of SSGs is associated with the creation of dermal defects in the donor area that often become difficult-to-heal wounds. In addition, the use of dermatome can deepithelize the skin adjacent to the planned donor site and can destroy the graft epidermis, usually rendering it useless (Bernardis & Box, 2010). In such cases, leaving the wounds on the lateral surfaces of the fingers and on the palmar surface of the hand without any biological dressings may be an equally good solution. Some authors claim that this technique does not prolong the healing time similarly compared with any other methods (Ciccarelli et al., 1995). Attempts to use allogeneic dermal substitutes have not produced clear results. Allogeneic culture skin substitutes (Eisenberg & Lleewelyn, 1998) and Apligraf (Fivenson et al., 2003) have been used. Although the time to recurrence was increased in allogeneic skin patients, the results for Apligraf were unclear. Some modern dressings were also used, but these did not reduce the duration of healing or the number of weekly dressing changes performed under general anesthesia (Jutkiewicz et al., 2010). All these methods, however, enabled the

In the modern treatment of EB, a great degree of significance is placed on the establishment of centers that enable contact with a team of specialists experienced in managing this rare disease (Pohla-Gubo & Hintner, 2010). Pediatric surgeons unfamiliar with EB may make errors stemming from a lack of knowledge of basic care principles. The following

 Children should not be lifted up independently. In particular, grabbing by the armpits easily causes wounds. Patient transfers should be assisted by the parents. Heavier

 In the case of disposable cloths, any elastics or belts should be removed from. Also, buttons or zippers in the bedclothes may cause blisters, particularly in anesthetized patients. Disposable clothes or wristbands should not adhere to skin when moist. Do not pull by the naked skin. In particular, do not restrain a child who has broken free. Grabbing by the wrists or by the fingers when attempting to stretch them causes epidermal tearing. When swabbing the skin before an injection, do not rub it with the

 Band-aids should not be applied to the skin. Wounds may be caused by a band-aid used for fixing the vein needle or the intubation tube. Remember not to affix disposable electrodes to the skin during the preparation for surgery, particularly ECG and

Ensure that information on the proper procedure is provided at level of the department

Metabolic disorders in RDEB may require treatment in the preparation for surgery. These stem from increased energy expenditure, impaired absorption due to defects of the small

recommendations are designed to help practitioners avoid iatrogenic skin injuries:

avoidance of dermatome use on the fragile epidermal surface.

children should be transferred on a blanket.

diathermy or self-adhesive draping in the operating field.

**2. Limited hand surgery in RDEB**

**2.1 Preparation for surgery 2.1.1 Basic recommendations**

swab.

and the operating suite.

**2.1.2 Metabolic disorders**

intestinal epithelium, and insufficient food supply caused by blisters in the mouth and esophageal stenosis. The progressing stenosis hinders the intake of liquids and the swallowing of saliva. Attempts to insert gastric tubes are often associated with skin and mucosal injuries. Confirmed stenosis should rather be an indication for esophageal dilation. The recommended method is contrast X-ray or fluoroscopic guidance balloon dilation. In the population of patients with RDEB, approximately one-third require at least one such procedure. In many patients, the stenosis recurs. The mean time between subsequent dilations is estimated at 1 to 2 years (Fine & Mellerio, 2009b).

In greater deficiencies or independently of esophageal dilation, gastrostomy should be considered. In particular, during the preoperative period, it enables easy supplementation of food taken orally. It should be emphasized that attempts to insert percutaneous endoscopic gastrostomy (PEG) tubes. These expose the patient to unnecessary risk. In the authors' center, laparotomy is performed under general anesthesia, without intubation. Such a choice also rules out laparoscopic gastrostomy.

Apart from food deficiencies, anemia also requires management. Many patients with RDEB are hospitalized with very low hemoglobin values. This is, to a large extent, caused by iron deficiency, associated with both insufficient supply in food and poor absorption in the intestine. Anemia in these patients also results from chronic inflammation associated with healing (Fine & Mellerio, 2009a). In a large portion of patients, despite the treatment with oral preparations, the hemoglobin level remains below 8 g/dl. With these levels, modern intravenous preparations are believed to be more effective. In the authors' center, Venofer is administered. This substitute has, thus far, prevented the need for treatment with blood, even at hemoglobin values below 6 g/dl. However, it should be emphasized that such a low hemoglobin is recognized as an indication for transfusion. Recently, erythropoietin has also been used, although there have yet been no studies indicating its clear advantage.

#### **2.1.3 Skin infections**

In preparation for surgery, local infections accompanying the non-healing wounds should also be considered for treatment. The patients admitted for hand surgery often have old wounds in other body regions. It is emphasized that the distinction between the colonization of such wounds and infection should be made based on the clinical picture and not the culture results. Systemic treatment is rarely required. However, the presence of group A streptococcus may constitute an indication for antibiotic use (Brandling-Bennett & Morel, 2010). The growth of beta – hemolytic streptococcus is also a contraindication for hand surgery (Bernardis & Box, 2010). The majority of the other microorganisms present in non-healing wounds do not affect the planning of surgery. Fungal infections in the mouth do not constitute a contraindication either. *Candida albicans* and *Candida parapsilosis* are both common florae of non-healing wounds in patients with EB (Brandling-Bennett & Morel, 2010).

#### **2.2 Preparation for anesthesia**

Before elective surgery, it is important to assess comorbid conditions. In over 75% of patients with RDEB, gastroesophageal reflux disease (GERD) is observed (Fine & Mellerio, 2009b). This condition increases the risk of choking during intubation. In the majority of

Limited Hand Surgery in Epidermolysis Bullosa 67

The treatment of early lesions is also beneficial in view of the higher probability of maintaining long-term hand function. Postponing the decision about the next surgery and delaying the treatment reduces the total duration of functionality. In the authors' center, the following assumptions have recently been made: (1) the treatment of early lesions permits the reduction of the scope of the surgery, (2) the reduction of the scope of the surgery lowers the number of dressing changes, and (3) giving up the aim of full finger extension may have little effect on the time to recurrence. These assumptions have enabled the formulation of the surgery objectives as pinch grip and grasp and independent finger movement.

Assuming that the patients present for regular visits, it is possible to adequately notice the progression of lesions early and make a timely decision with regards to surgery. With less frequent visits, the patients often present with palmar finger contractures secondary to pseudosyndactyly (Fig. 1a, 1b). Indications for surgery had occurred in those patients prior to presentation. Isolated flexion contractures without advanced pseudosyndactyly are less common. These are only a relative indication for treatment because they may be extended

Fig. 1. (a) Pseudosyndactyly of the first web space. (b) Palmar contracture and

Sometimes, discouraged by previous experiences with protracted healing, patients do not return for years. This avoidance leads to total deformations of the mitten type (Fig. 2a). This

pseudosyndactyly of fingers II-V. Explanation in the text. Patient AA.

Therefore, indications for surgery may be defined as follows:

and maintained in such a state only for short periods.

Pseudosyndactyly of fingers II-V

Pseudosyndactyly of the first web space reaching the IP joint,

children, continued treatment with proton pump inhibitors should be considered. Of note, the risk of cardiomyopathy may also constitute a relative contraindication for surgery. In patients with RDEB, this risk stands at 4.5% by the age of 20 (Fine & Mellerio, 2009a). To rule out the disease, echocardiography is required. The integrity of the respiratory tract should also be assessed. In view of the possibility of microstomy, ankyloglossia, and fixation of the epiglottis, intubation may prove difficult.

During the immediate preparation, the operating room should be supplied with appropriate dressings, and the operating table should be adapted. Adhesives should be removed from all needles and electrodes affixed to the skin. A good alternative for band-aids are silicone tapes with limited adhesiveness that are recommended for patients with EB (e.g. Mepitac). Fixing the intubation tube requires the use of a bandage or silicone tape. For the closing of the eyelids, moisturizing gels with methylcellulose are used.

Two techniques for the safe use of anesthesiological masks are recommended. The first consists of the intensive moisturizing of the skin and mask edge with a moisturizing agent. The second uses silicone tapes such as Mepitac. After either product is affixed, it protects the facial skin while at the same time maintaining air-tightness with the mask (Goldschneider et al., 2010). In patients with RDEB, anesthesia may be considered without intubation, using a mask alone. The common finding of ankyloglossia hinders the tongue from falling back, protecting against the closure of the airway. In the authors' center, anesthesia without intubation is primarily used. The mask is secured with a common cotton swab or a hydrogel wound dressing. However, the depth of such anesthesia is harder to control. Therefore, the method requires an experienced team and good communication between the surgeon and anesthesiologist.

#### **2.3 Indications for surgery**

The long-term skin symptoms of RDEB in the hands, including pseudosyndactyly and scars, lead to the establishment of contractures in the joints. Some authors introduce precise angle measurements, describing the extension and flexion deficits in the metacarpophalangeal (MCP) and interphalangeal (IP) joints (Terrill et al., 1992). Based on similar criteria, precise indications for surgery have been proposed (Ciccarelli et al., 1995). These were the following: (1) palmar contracture, (2) contracture of the proximal interphalangeal (PIP) joint greater than 30 degrees, (3) significant involvement of the small finger, (4) pseudosyndactyly extending to the PIP joint, or (5) impairment of activities of daily living.

Many authors emphasize, however, that irrespectively of deformity grade lesions should be treated early enough to prevent irreversible consequences (Azizkhan et al., 2007). Surgery is typically thought to be indicated when the loss of function compromises the patient's independence and impairs his/her appearance (Bernardis & Box, 2010). Because thumb mobility contributes to 50% of hand functionality, the release of the first web space alone results in a significant improvement. Therefore the aim of surgery is to provide simple pinch grip and grasp, by releasing the first web space and flexion contractures; independent finger movement, by releasing pseudosyndactyly; and improved appearance of the hand (Bernardis & Box, 2010).

children, continued treatment with proton pump inhibitors should be considered. Of note, the risk of cardiomyopathy may also constitute a relative contraindication for surgery. In patients with RDEB, this risk stands at 4.5% by the age of 20 (Fine & Mellerio, 2009a). To rule out the disease, echocardiography is required. The integrity of the respiratory tract should also be assessed. In view of the possibility of microstomy, ankyloglossia, and fixation

During the immediate preparation, the operating room should be supplied with appropriate dressings, and the operating table should be adapted. Adhesives should be removed from all needles and electrodes affixed to the skin. A good alternative for band-aids are silicone tapes with limited adhesiveness that are recommended for patients with EB (e.g. Mepitac). Fixing the intubation tube requires the use of a bandage or silicone tape. For the closing of the eyelids, moisturizing gels with methylcellulose are used. Petroleum-based products are difficult to rinse off post surgery and cause instinctive rubbing of the eyes (Goldschneider et

Two techniques for the safe use of anesthesiological masks are recommended. The first consists of the intensive moisturizing of the skin and mask edge with a moisturizing agent. The second uses silicone tapes such as Mepitac. After either product is affixed, it protects the facial skin while at the same time maintaining air-tightness with the mask (Goldschneider et al., 2010). In patients with RDEB, anesthesia may be considered without intubation, using a mask alone. The common finding of ankyloglossia hinders the tongue from falling back, protecting against the closure of the airway. In the authors' center, anesthesia without intubation is primarily used. The mask is secured with a common cotton swab or a hydrogel wound dressing. However, the depth of such anesthesia is harder to control. Therefore, the method requires an experienced team and good communication between the surgeon and

The long-term skin symptoms of RDEB in the hands, including pseudosyndactyly and scars, lead to the establishment of contractures in the joints. Some authors introduce precise angle measurements, describing the extension and flexion deficits in the metacarpophalangeal (MCP) and interphalangeal (IP) joints (Terrill et al., 1992). Based on similar criteria, precise indications for surgery have been proposed (Ciccarelli et al., 1995). These were the following: (1) palmar contracture, (2) contracture of the proximal interphalangeal (PIP) joint greater than 30 degrees, (3) significant involvement of the small finger, (4) pseudosyndactyly extending to

Many authors emphasize, however, that irrespectively of deformity grade lesions should be treated early enough to prevent irreversible consequences (Azizkhan et al., 2007). Surgery is typically thought to be indicated when the loss of function compromises the patient's independence and impairs his/her appearance (Bernardis & Box, 2010). Because thumb mobility contributes to 50% of hand functionality, the release of the first web space alone results in a significant improvement. Therefore the aim of surgery is to provide simple pinch grip and grasp, by releasing the first web space and flexion contractures; independent finger movement, by releasing pseudosyndactyly; and improved appearance of the hand

of the epiglottis, intubation may prove difficult.

al., 2010).

anesthesiologist.

**2.3 Indications for surgery**

(Bernardis & Box, 2010).

the PIP joint, or (5) impairment of activities of daily living.

The treatment of early lesions is also beneficial in view of the higher probability of maintaining long-term hand function. Postponing the decision about the next surgery and delaying the treatment reduces the total duration of functionality. In the authors' center, the following assumptions have recently been made: (1) the treatment of early lesions permits the reduction of the scope of the surgery, (2) the reduction of the scope of the surgery lowers the number of dressing changes, and (3) giving up the aim of full finger extension may have little effect on the time to recurrence. These assumptions have enabled the formulation of the surgery objectives as pinch grip and grasp and independent finger movement. Therefore, indications for surgery may be defined as follows:

Assuming that the patients present for regular visits, it is possible to adequately notice the progression of lesions early and make a timely decision with regards to surgery. With less frequent visits, the patients often present with palmar finger contractures secondary to pseudosyndactyly (Fig. 1a, 1b). Indications for surgery had occurred in those patients prior to presentation. Isolated flexion contractures without advanced pseudosyndactyly are less common. These are only a relative indication for treatment because they may be extended and maintained in such a state only for short periods.

Fig. 1. (a) Pseudosyndactyly of the first web space. (b) Palmar contracture and pseudosyndactyly of fingers II-V. Explanation in the text. Patient AA.

Sometimes, discouraged by previous experiences with protracted healing, patients do not return for years. This avoidance leads to total deformations of the mitten type (Fig. 2a). This

Limited Hand Surgery in Epidermolysis Bullosa 69

the use of scissors or a scalpel is necessary. Attention should be paid to the neurovascular bundles. If possible, they should not be exposed, and flexor sheaths should not be reached.

Fig. 2. (a) Mitten hand deformation, (b) Separated pseudosyndactyly. (c) Released

leads to extensive epidermal injuries and complete exposure of the flexor sheaths.

Fig. 3. (a) Joints extended to the right angle, (b) Separated thumb pseudosyndactyly.

In most cases, the extension in the PIP joints does not have to exceed a right angle (Fig. 3a). Further extension would require the grabbing of the finger by the surgeon's hand and its forced pulling. Such a maneuver is used in more invasive surgical techniques; however, it

The thumb is separated in a similar manner. The epidermis is cut on the dorsal side (Fig. 3a). Next, the surgeon moves to the palmar side, dissecting to the depth of the muscle fibers. Both the dermal contractures and some of the adductor pollicis fibers may require sharp separation. However, if possible, it is better to stretch the muscle and joints, gently pulling the suture on the thumb tip (Fig. 3b). This facilitates the protection of the neurovascular

contracture. Explanation in the text. Patient BB.

bundles near the flexor muscle sheath.

Explanation in the text. Patient BB.

situation is still an indication for surgery, although the patients have to be aware of the poor prognosis concerning mobility in the IP joints. Such advanced lesions prolong healing and reduce the efficacy of subsequent rehabilitation.

#### **2.4 Surgical treatment**

#### **2.4.1 Features of a limited approach**

An important principle and advantage of the presented treatment is the possibility of avoiding direct contact with the patient's skin. This protection minimizes the extent of iatrogenic epidermal injury and, consequently, facilitates healing. The disadvantage of this treatment is that it eliminates the possibility of full forced extension of the fingers during surgery. Both factors differentiate the suggested approach from other methods.

#### **2.4.2 Hand preparation in the operating room**

Only one hand is operated on during each hospital admission. This does not hinder the patient's functioning and reduces the associated stress and discomfort of healing. Half an hour before the patient is brought into the operating suite, the hand may be soaked in a bowl with Betadine solution. Washing at the suite, immediately before the surgery, is limited to patting with a web swab. Energetic rubbing would lead to epidermal injuries.

The authors do not use a tourniquet. We believe that it is not necessary in the described technique. However, the majority of surgeons feel comfortable operating without a blood supply. If a tourniquet is used, it should be placed on an arm wrapped with a thick layer of cotton wool. Prior to pumping, the limb is raised for 2 minutes. It must not be wrapped with an elastic bandage or a rubber band to remove the blood. Prior to placing the limb on the table, a thick pad of sterile cotton wool should be placed under the elbow.

Before starting the dissection, thick sutures, e.g. Vicryl 3.0 or 4.0, are placed on the fingertips. Their ends are left long and are tightened with an instrument. They are then used to pull away the fingers during the contracture release. The assistant holds the sutures during the surgery, which avoids the direct handling of the patient skin. The surgeon holds in his/her left hand only the suture of the finger that is currently extended. As a result, the right hand is free.

#### **2.4.3 Separation of pseudosyndactyly and contractures**

In cases where the hand is curled in a complete cocoon (Fig. 2a), the fingertips are difficult to release without an initial dissection. A small, circular incision around the fingertips enables them to be detached and lever with blunt scissors. Only then can the suture be placed. In such advanced deformities, the epidermis is retained only on the dorsal surfaces of the fingers. Therefore, the surgery is continued by cutting the epidermis on the dorsal side. During cutting, it is clear that the dermal layers between the fingers remain "glued" rather than grown together into one whole (Fig. 2b). It is easy to find the web space by parting it with scissors. Separation of the fingers may be made easier by pulling them slightly sideways. On the dorsal side, the epidermis is cut to the MP joints level. On the palmar side, the fingers are separated to only half the length of the proximal phalanges. However, to reach this level, the fingers must be simultaneously released from contractures (Fig. 2c). This release usually requires sharp dissection. Particularly on the palmar side of the PIP joints,

situation is still an indication for surgery, although the patients have to be aware of the poor prognosis concerning mobility in the IP joints. Such advanced lesions prolong healing and

An important principle and advantage of the presented treatment is the possibility of avoiding direct contact with the patient's skin. This protection minimizes the extent of iatrogenic epidermal injury and, consequently, facilitates healing. The disadvantage of this treatment is that it eliminates the possibility of full forced extension of the fingers during

Only one hand is operated on during each hospital admission. This does not hinder the patient's functioning and reduces the associated stress and discomfort of healing. Half an hour before the patient is brought into the operating suite, the hand may be soaked in a bowl with Betadine solution. Washing at the suite, immediately before the surgery, is limited to patting with a web swab. Energetic rubbing would lead to epidermal injuries.

The authors do not use a tourniquet. We believe that it is not necessary in the described technique. However, the majority of surgeons feel comfortable operating without a blood supply. If a tourniquet is used, it should be placed on an arm wrapped with a thick layer of cotton wool. Prior to pumping, the limb is raised for 2 minutes. It must not be wrapped with an elastic bandage or a rubber band to remove the blood. Prior to placing the limb on the

Before starting the dissection, thick sutures, e.g. Vicryl 3.0 or 4.0, are placed on the fingertips. Their ends are left long and are tightened with an instrument. They are then used to pull away the fingers during the contracture release. The assistant holds the sutures during the surgery, which avoids the direct handling of the patient skin. The surgeon holds in his/her left hand only the suture of the finger that is currently extended. As a result, the right hand is free.

In cases where the hand is curled in a complete cocoon (Fig. 2a), the fingertips are difficult to release without an initial dissection. A small, circular incision around the fingertips enables them to be detached and lever with blunt scissors. Only then can the suture be placed. In such advanced deformities, the epidermis is retained only on the dorsal surfaces of the fingers. Therefore, the surgery is continued by cutting the epidermis on the dorsal side. During cutting, it is clear that the dermal layers between the fingers remain "glued" rather than grown together into one whole (Fig. 2b). It is easy to find the web space by parting it with scissors. Separation of the fingers may be made easier by pulling them slightly sideways. On the dorsal side, the epidermis is cut to the MP joints level. On the palmar side, the fingers are separated to only half the length of the proximal phalanges. However, to reach this level, the fingers must be simultaneously released from contractures (Fig. 2c). This release usually requires sharp dissection. Particularly on the palmar side of the PIP joints,

surgery. Both factors differentiate the suggested approach from other methods.

table, a thick pad of sterile cotton wool should be placed under the elbow.

**2.4.3 Separation of pseudosyndactyly and contractures**

reduce the efficacy of subsequent rehabilitation.

**2.4.2 Hand preparation in the operating room**

**2.4.1 Features of a limited approach**

**2.4 Surgical treatment**

the use of scissors or a scalpel is necessary. Attention should be paid to the neurovascular bundles. If possible, they should not be exposed, and flexor sheaths should not be reached.

Fig. 2. (a) Mitten hand deformation, (b) Separated pseudosyndactyly. (c) Released contracture. Explanation in the text. Patient BB.

In most cases, the extension in the PIP joints does not have to exceed a right angle (Fig. 3a). Further extension would require the grabbing of the finger by the surgeon's hand and its forced pulling. Such a maneuver is used in more invasive surgical techniques; however, it leads to extensive epidermal injuries and complete exposure of the flexor sheaths.

The thumb is separated in a similar manner. The epidermis is cut on the dorsal side (Fig. 3a). Next, the surgeon moves to the palmar side, dissecting to the depth of the muscle fibers. Both the dermal contractures and some of the adductor pollicis fibers may require sharp separation. However, if possible, it is better to stretch the muscle and joints, gently pulling the suture on the thumb tip (Fig. 3b). This facilitates the protection of the neurovascular bundles near the flexor muscle sheath.

Fig. 3. (a) Joints extended to the right angle, (b) Separated thumb pseudosyndactyly. Explanation in the text. Patient BB.

Limited Hand Surgery in Epidermolysis Bullosa 71

or from another surface on which blisters are not found. It should be collected with excess to ensure the thorough covering of all wounds. Bleeding from the collection site is minimal. Thus, the area does not require prompt compression dressing. If necessary the next graft can

The graft margins should be marked with a felt-tip pen and cut superficially with a lancet (Fig.6a). Next, using the elevator, the epidermal edge is slipped off. Gently, it may be separated from the skin without any damage (Fig.6b). After complete layer separation, the outer surface of the graft is covered with a greased tulle, which facilitates the transfer of the epidermal layer (Fig.6c) and its splitting into appropriate fragments (Fig.6d). At the donor

Fig. 6. (a) Markings on the thigh. (b) Harvesting the split-off graft. (c) Graft transfer. (d)

The grafts, transferred to the recipient site may be implanted and fixed with sutures or applied to the wound with the transportation tulle, which then constitutes a part of the dressing (Fig.7a). The "split-off" epidermis may be combined, if necessary, with full-

be harvested in immediate vicinity.

site, a typical tulle dressing is applied.

Graft splitting. Explanation in the text.

thickness grafts (Fig.7b).

In less advanced contractures (Fig. 1a, 1b), the described method allows for the nearly complete preservation of the thumb (Fig. 4a) and palmar epidermis (Fig. 4b). It also facilitates healing and limits the dressing changes to one or two subsequent hospitalizations.

Fig. 4. (a) Minor palmar wound, (b) Epidermis on the fingers is preserved. Explanation in the text. Patient AA.

In some patients, the need for the release of contractures of fingers II-V should be carefully considered. This question concerns especially those whose multiple prior operations deterred from surgery (Fig 5a). The reduction of the extent of subsequent surgeries for the separation of the first web space (Fig. 5b) may sufficiently improve hand functionality.

Fig. 5. (a) Recurrent mitten deformation, (b) Isolated thumb release. Explanation in the text. Patient CC.

#### **2.4.4 Skin grafts**

At sites where flexor muscle sheaths or neurovascular bundles are exposed covering with FTSGs may be required. These grafts usually undergo epidermal separation during manipulation associated with the defatting of the undersurface and placing. Despite the above, they usually heal well. On the other hand, it is not known whether FTSGs tendency to resist contracture delays the recurrence in patients with EB.

Due to the limited supply of FTSGs and the instability of SSGs, an intermediate solution was suggested over 30 years ago (Cuono & Finsteth, 1978). Since this technique facilitates healing, it is particularly suitable for surgeries of limited scope. It involves the acquisition of isolated epidermis in a technique called the "split-off". The graft is acquired from the thighs

In less advanced contractures (Fig. 1a, 1b), the described method allows for the nearly complete preservation of the thumb (Fig. 4a) and palmar epidermis (Fig. 4b). It also facilitates healing and limits the dressing changes to one or two subsequent hospitalizations.

Fig. 4. (a) Minor palmar wound, (b) Epidermis on the fingers is preserved. Explanation in

In some patients, the need for the release of contractures of fingers II-V should be carefully considered. This question concerns especially those whose multiple prior operations deterred from surgery (Fig 5a). The reduction of the extent of subsequent surgeries for the separation of the first web space (Fig. 5b) may sufficiently improve hand functionality.

Fig. 5. (a) Recurrent mitten deformation, (b) Isolated thumb release. Explanation in the text.

At sites where flexor muscle sheaths or neurovascular bundles are exposed covering with FTSGs may be required. These grafts usually undergo epidermal separation during manipulation associated with the defatting of the undersurface and placing. Despite the above, they usually heal well. On the other hand, it is not known whether FTSGs tendency

Due to the limited supply of FTSGs and the instability of SSGs, an intermediate solution was suggested over 30 years ago (Cuono & Finsteth, 1978). Since this technique facilitates healing, it is particularly suitable for surgeries of limited scope. It involves the acquisition of isolated epidermis in a technique called the "split-off". The graft is acquired from the thighs

to resist contracture delays the recurrence in patients with EB.

the text. Patient AA.

Patient CC.

**2.4.4 Skin grafts**

or from another surface on which blisters are not found. It should be collected with excess to ensure the thorough covering of all wounds. Bleeding from the collection site is minimal. Thus, the area does not require prompt compression dressing. If necessary the next graft can be harvested in immediate vicinity.

The graft margins should be marked with a felt-tip pen and cut superficially with a lancet (Fig.6a). Next, using the elevator, the epidermal edge is slipped off. Gently, it may be separated from the skin without any damage (Fig.6b). After complete layer separation, the outer surface of the graft is covered with a greased tulle, which facilitates the transfer of the epidermal layer (Fig.6c) and its splitting into appropriate fragments (Fig.6d). At the donor site, a typical tulle dressing is applied.

Fig. 6. (a) Markings on the thigh. (b) Harvesting the split-off graft. (c) Graft transfer. (d) Graft splitting. Explanation in the text.

The grafts, transferred to the recipient site may be implanted and fixed with sutures or applied to the wound with the transportation tulle, which then constitutes a part of the dressing (Fig.7a). The "split-off" epidermis may be combined, if necessary, with fullthickness grafts (Fig.7b).

Limited Hand Surgery in Epidermolysis Bullosa 73

soaked in Betadine solution. The non-healed, and in some areas, macerated epidermis separates easily from its base (Fig. 9a). Only after it dries up does it adhere fully to the wound (Fig. 9b). The spot bleeding appearing locally may be blocked by touching with a moist swab with 40% solution of silver nitrate (Fig. 9c). The donor area of the collected

Fig. 9. One week after the operation. (a) Macerated epidermis. (b) Healing epidermis, (c) Minor bleeding, (d) Healing donor area. Explanation in the text. Patients BB, CC and AA respectively.

Both the donor area and the hand require new dressings that are made as described

Fig. 10. One week after the operation. (a) Tulle dressing. (b) Silver tulle. Explanation in the

text. Patients BB and AA respectively.

previously, using the greased (Fig. 10a) or silver impregnated tulles (Fig. 10b).

"split-off" graft does not heal during this time (Fig. 9d).

Fig. 7. (a) Grafts left with tulle. (b) Grafts sutured to their beds. Explanation in the text. Patients BB and CC respectively.

Prior to the application of further dressings, a polyurethane sponge is wrapped around the wrist (Fig. 8a). The subsequent dressings inserted into the web spaces are sutured to it (Fig.8b). The hand and fingers may be immobilized with an additional layer of thermoplastic splint applied on the palmar side.

Fig. 8. (a) A sponge band wrapped around a wrist. (b) Dressings fixed to wrist band. Explanation in the text. Patient AA.

#### **2.4.5 Postoperative dressings**

The first dressing change after a week, requires the reservation of the operating room and general anesthesia. Before the removal of each dressing layer, it should be thoroughly

Fig. 7. (a) Grafts left with tulle. (b) Grafts sutured to their beds. Explanation in the text.

Fig. 8. (a) A sponge band wrapped around a wrist. (b) Dressings fixed to wrist band.

The first dressing change after a week, requires the reservation of the operating room and general anesthesia. Before the removal of each dressing layer, it should be thoroughly

Prior to the application of further dressings, a polyurethane sponge is wrapped around the wrist (Fig. 8a). The subsequent dressings inserted into the web spaces are sutured to it (Fig.8b). The hand and fingers may be immobilized with an additional layer of

Patients BB and CC respectively.

Explanation in the text. Patient AA.

**2.4.5 Postoperative dressings**

thermoplastic splint applied on the palmar side.

soaked in Betadine solution. The non-healed, and in some areas, macerated epidermis separates easily from its base (Fig. 9a). Only after it dries up does it adhere fully to the wound (Fig. 9b). The spot bleeding appearing locally may be blocked by touching with a moist swab with 40% solution of silver nitrate (Fig. 9c). The donor area of the collected "split-off" graft does not heal during this time (Fig. 9d).

Fig. 9. One week after the operation. (a) Macerated epidermis. (b) Healing epidermis, (c) Minor bleeding, (d) Healing donor area. Explanation in the text. Patients BB, CC and AA respectively.

Both the donor area and the hand require new dressings that are made as described previously, using the greased (Fig. 10a) or silver impregnated tulles (Fig. 10b).

Fig. 10. One week after the operation. (a) Tulle dressing. (b) Silver tulle. Explanation in the text. Patients BB and AA respectively.

Limited Hand Surgery in Epidermolysis Bullosa 75

Fig. 12. Twenty one and twenty four days after the operation. (a) Accidental trauma. (b – d)

In the authors' center, the results of 10 hands operated with the described limited approach currently exceed one year (Fig. 13a-13b). No early recurrence was noted within

Fig. 13. (a) Left thumb six month after the operation. (b) Right thumb one year after the

Wounds healed. Explanation in the text. Patients BB.

this time.

operation. Patient CC.

The dressing procedures at two weeks also require anesthesia in the operating room. The grafts, despite local maceration, are already clearly healed (Fig.11a). The dried up and macerated epidermis is not removed to avoid bleeding (Fig. 11b). Upon the application of new dressings, the hand is immobilized on the palmar side, with the use of a splint with thumb support (Fig. 11c). A properly adjusted thermoplastic splint further supports the extension of the fingers, even if they were left partly flexed during the operation (Fig. 11b). The "split-off" graft donor area is, by this time, already healed and does not require any dressing (Fig. 11d).

Fig. 11. Dressings at two weeks. (a) Local maceration. (b) Minor wounds. (c) Splint. (d) Healed donor area. Explanation in the text. Patients AA, BB and AA respectively.

After three weeks, most of the dressings may be changed in the dressing room without anesthesia. Minor wounds are present at that time only in patients in whom a decision was made to undertake a more extensive approach. These wounds are often associated with secondary injuries occurring during the removal of old dressings (Fig. 12a). The removal of subsequent dressing layers should therefore be preceded by the soaking of the hand in Betadine solution. From the third week post surgery, despite the presence of minor wounds (Fig. 12b – 12d), daily hygiene procedures should be performed in a home setting. Parents should continuously collaborate with the rehabilitation specialist. The dorsal thermoplastic hand splints with bands on the fingers are used from the third week until the end of the third month during the day and night. The daily application of the thermoplastic splint after dressing replacement may require an ongoing adjustment of its setting. Without parental cooperation, this adjustment would be impossible.

The dressing procedures at two weeks also require anesthesia in the operating room. The grafts, despite local maceration, are already clearly healed (Fig.11a). The dried up and macerated epidermis is not removed to avoid bleeding (Fig. 11b). Upon the application of new dressings, the hand is immobilized on the palmar side, with the use of a splint with thumb support (Fig. 11c). A properly adjusted thermoplastic splint further supports the extension of the fingers, even if they were left partly flexed during the operation (Fig. 11b). The "split-off" graft donor area is, by this time, already healed and does not require any

Fig. 11. Dressings at two weeks. (a) Local maceration. (b) Minor wounds. (c) Splint. (d) Healed donor area. Explanation in the text. Patients AA, BB and AA respectively.

cooperation, this adjustment would be impossible.

After three weeks, most of the dressings may be changed in the dressing room without anesthesia. Minor wounds are present at that time only in patients in whom a decision was made to undertake a more extensive approach. These wounds are often associated with secondary injuries occurring during the removal of old dressings (Fig. 12a). The removal of subsequent dressing layers should therefore be preceded by the soaking of the hand in Betadine solution. From the third week post surgery, despite the presence of minor wounds (Fig. 12b – 12d), daily hygiene procedures should be performed in a home setting. Parents should continuously collaborate with the rehabilitation specialist. The dorsal thermoplastic hand splints with bands on the fingers are used from the third week until the end of the third month during the day and night. The daily application of the thermoplastic splint after dressing replacement may require an ongoing adjustment of its setting. Without parental

dressing (Fig. 11d).

Fig. 12. Twenty one and twenty four days after the operation. (a) Accidental trauma. (b – d) Wounds healed. Explanation in the text. Patients BB.

In the authors' center, the results of 10 hands operated with the described limited approach currently exceed one year (Fig. 13a-13b). No early recurrence was noted within this time.

Fig. 13. (a) Left thumb six month after the operation. (b) Right thumb one year after the operation. Patient CC.

Limited Hand Surgery in Epidermolysis Bullosa 77

Azizkhan RG, Denyer JE, Mellerio JE, González R, Bacigalupo M, Kantor A, Passalacqua G,

Bernardis C, Box R. Surgery of the hand in recessive dystrophic epidermolysis bullosa.

Brandling-Bennett HA, Morel KD. Common wound colonizers in patients with

Ciccarelli AO, Rothaus KO, Carter DM, Lin AN. Plastic and reconstructive surgery in

Cuono C, Finseth F. Epidermolysis bullosa: current concepts and management of the

Devries DT, Johnson LB, Weiner M, Fine JD. Relative extent of skin involvement in inherited

Eisenberg M, Llewelyn D. Surgical management of hands in children with recessive

Fine JD, Johnson LB, Weiner M, Stein A, Cash S, Deleoz J, Devries DT, Suchindran C.