Varela P & Torre M. (2011) Thoracoscopic cartilage resection with partial perichondrium

Wada J, Ikeda K, Ishida T & Hasegawa T. (1970) Results of 271 funnel chest operations. *Ann*

Waldhausen JH, Redding GJ & Song KM. (2007) Vertical expandable prosthetic titanium rib

Waters P, Welch K, Micheli LJ, Shamberger R & Hall JE. (1989) Scoliosis in children with

Welch KJ. (1958) Satisfactory surgical correction of pectus excavatum deformity in

Williams AM & Crabbe DC. (2003) Pectus deformities of the anterior chest wall. *Pediatr*

Poland's syndrome. *J Pediatr Surg*, Vol. 24, No. 8, pp. 760-765. Shamberger R & Welch K. (1990) Sternal defects. Pediatr Surg Int Vol. 5, pp. 156-164.

pectus excavatum. *J Pediatr Surg*, Vol. 43, No. 8, pp. 1468-1473.

*Cardiovasc Surg*, Vol. 139, No. 5, pp. 1177-1182.

*Genet*, Vol. 125A, No. 2, pp. 211-212.

*Thorac Surg*, Vol. 10, No. 6, pp. 526-532.

*Respir Rev*, Vol. 4, No. 3, pp. 237-242.

*Surg*, Vol. 42, No. 1, pp. 76-80.

46, No. 1, pp. 263-6.

*J Cardiothorac Surg*. 2011 Jul 5. [Epub ahead of print]

childhood. *J Thorac Surg*, Vol. 36, No. 5, pp. 697-713.

496-500.

*Surg*, Vol. 13, No. 6, pp. 529-536.

43, No. 10, pp. 1776-1780.

addition to the management of pectus excavatum. *J Pediatr Surg*, Vol. 40, No. 3, pp.

Silastic implants for correction of pectus excavatum. A new technique. *Ann Thorac*

carinatum: the use of radiographic markers to predict success. *J Pediatr Surg*, Vol.

FM, Valle M & Calevo MG. (2010) Dextrocardia in patients with Poland syndrome: Phenotypic characterization provides insight into the pathogenesis. *J Thorac*

management of sternal cleft: literature review and presentation of a new series. *Eur*

preservation in unilateral pectus carinatum: preliminary results. *J Pediatr Surg*, Vol.

for thoracic insufficiency syndrome: a new method to treat an old problem. *J Pediatr*

pectus excavatum and pectus carinatum. *J Pediatr Orthop*, Vol. 9, No. 5, pp. 551-556.

#### **1.1 Epidemiology and etiology/pathogenesis**

Pectus excavatum (PE) is a most frequent deformity at the anterior chest wall. Its incidence is about 1:400 in live births. Male to female ratio is 4:1. Various theories have been proposed to explain pathogenesis of PE.

Brown (Brown, 1939) described that the force producing the depression is diaphragm acting primarily in the anteroposterior direction through its attachment to the sternum. He pointed out a membranous structure beneath the sternum, and denominated it substernal ligament.

On the other hand, Sweet (Sweet, 1944) thought that the substernal ligament is not important structure, and stated "it seems almost as though the sternum is pushed down against spine by unusually long, inward curving costal cartilages".

This idea evoked sympathy of many surgeons, and the overgrowth of cartilages theory for PE became the leading theory, because of significance of theoretical background for the surgeon's act to resect the overgrown cartilages.

However, recently, Nakaoka and colleagues (Nakaoka et al., 2009, 2010) measured the length of the 5th and 6th costal cartilages and ribs in PE patients from reconstructed images of 3-dimensional computed tomography (CT). He calculated the C/R ratio, defined as the quotient of the costal cartilage length divided by the adjacent rib length, and compared it between the PE patients and the healthy controls. As the result, he found that the C/R ratio in the PE patients were not longer than that in the healthy controls at any level, so concluded that the overgrowth of costal cartilages is not the pathogenesis of PE.

PE patients often show characteristic figure, such as lanky shape, and thin thorax, and associate sometimes Marfan syndrome or Noonan syndrome. These findings suggest hereditary origin of PE.

Creswick and colleagues (Creswick et al., 2006) analysed 34 families and assumed autosomal dominant inheritance in 14 families, autosomal recessive inheritance in 4 families, and X-chromosomal inheritance in 6 families. However, many family members had additional connective tissue traits, that is, systemic connective tissue diseases can not be ruled out.

Marfan syndrome occurs in 1:5,000 to 10,000 of population. It is genetic disease, which is caused by mutation in the Fibrillin 1 gene localized on the long arm of chromosome 15.

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 139

Nakahara and colleagues (Nakahara et al., 1987) set 3 indices; degree of depression, degree of asymmetry, and degree of flatness (Fig. 4). These indices serve to define the diagnosis, to

Masaoka and colleagues (Masaoka et al., 2011) proposed 3 other indices; steepness index, excavation volume index, and asymmetry index. These are defined as shown in Fig. 5. This

1. Three indices express the morphological characteristic in PE straight, respectively. Steepness index represents cup shape, excavation volume index saucer shape, and

2. Excavation volume index and asymmetry index are defined two-dimensionally, in

decide operative indication, and evaluate the effects of operation.

asymmetry index Grand Canyon shape configuration.

Fig. 2. Saucer shape depression

Fig. 3. Grand Canyon shape depression

system has properties described below.

order to quantify more exactly.

Fig. 4. Deformity indices (Nakahara et al., 1987)

On the other hand, Noonan syndrome is a common autosomal dominantly inherited disorder caused by mutations in various genes (PTPN 11, KRAS, SOS1, RAF1). Incidence is 1:1,000 to 2,500 (Kotzot & Schwabegger, 2009).

However, causative genes of isolated (non syndromic) PE are not yet determined. PE can be an isolated malformation or dysmorphic feature or only one symptom of a genetic syndrome. The research of responsible gene relating with PE is yet on the start line.

#### **1.2 Symptomatology**

PE is a deformity of the thorax characterized by depression of the sternum. The cephalic border of the depression situates usually at the conjunction of manubrium and sternal body. The sternal body leans sharply to the depth, deepest just before its junction with the xiphoid. The lower costal cartilages bend inward to form depression.

In infant, a paradoxical inward motion of the lower sternum is conspicuous on inspiration. PE becomes fixed at child age (2~3 years). Eguchi and colleagues (Eguchi et al., 1993) observed natural course of the PE deformity in 25 patients (average 2.5 years). Mean period of observation was 3.2 years. Ten patients (40%) progressed, 14 (56%) did not change, and one (4%) patient improved. In the progressed patients, 5 patients showed asymmetrical progression. These results suggest some clinical implications, i.e., 1) decision of operative indication should be delayed until schoolchild age at least, 2) asymmetry starts already at child age.

Usually, deformity is going to progress together with growth. At puberty, growth of the thorax spurts. The characteristic of progression in this term is reinforce of asymmetry, characterizing deeper depression at right side and torsion of the sternum. Such asymmetric deformity is considered to be resulted from existence of heart in left side.

PE deformity is not common in all patients. There are some types with typical different features. Nuss (Nuss, 2008) expressed as cup shape, saucer shape and Grand Canyon shape. Cup shape means a localized, narrow depression (Fig. 1). Saucer shape is a wide and flat depression (Fig. 2). Grand Canyon shape means asymmetric type with deepest at right side (Fig. 3). Moreover, each patient shows different grade. In order to express objectively the characteristic of each patient, various deformity indices of PE have been proposed.

Introduction of computerized tomography (CT) enhanced such trend. Haller and colleagues (Haller et al., 1987) set transverse diameter/anterior-posterior diameter as an index. The anterior-posterior diameter means a distance between sternum and spine at the deepest depression level. He stated that this index was 4.42±0.76 in PE patients and 2.56±0.35 in normal subjects, and useful in judgment of operative indication.

Fig. 1. Cup shape depression

Fig. 2. Saucer shape depression

On the other hand, Noonan syndrome is a common autosomal dominantly inherited disorder caused by mutations in various genes (PTPN 11, KRAS, SOS1, RAF1). Incidence is

However, causative genes of isolated (non syndromic) PE are not yet determined. PE can be an isolated malformation or dysmorphic feature or only one symptom of a genetic

PE is a deformity of the thorax characterized by depression of the sternum. The cephalic border of the depression situates usually at the conjunction of manubrium and sternal body. The sternal body leans sharply to the depth, deepest just before its junction with the xiphoid.

In infant, a paradoxical inward motion of the lower sternum is conspicuous on inspiration. PE becomes fixed at child age (2~3 years). Eguchi and colleagues (Eguchi et al., 1993) observed natural course of the PE deformity in 25 patients (average 2.5 years). Mean period of observation was 3.2 years. Ten patients (40%) progressed, 14 (56%) did not change, and one (4%) patient improved. In the progressed patients, 5 patients showed asymmetrical progression. These results suggest some clinical implications, i.e., 1) decision of operative indication should be delayed until schoolchild age at least, 2) asymmetry starts already at

Usually, deformity is going to progress together with growth. At puberty, growth of the thorax spurts. The characteristic of progression in this term is reinforce of asymmetry, characterizing deeper depression at right side and torsion of the sternum. Such asymmetric

PE deformity is not common in all patients. There are some types with typical different features. Nuss (Nuss, 2008) expressed as cup shape, saucer shape and Grand Canyon shape. Cup shape means a localized, narrow depression (Fig. 1). Saucer shape is a wide and flat depression (Fig. 2). Grand Canyon shape means asymmetric type with deepest at right side (Fig. 3). Moreover, each patient shows different grade. In order to express objectively the characteristic of each patient, various deformity indices of PE have been

Introduction of computerized tomography (CT) enhanced such trend. Haller and colleagues (Haller et al., 1987) set transverse diameter/anterior-posterior diameter as an index. The anterior-posterior diameter means a distance between sternum and spine at the deepest depression level. He stated that this index was 4.42±0.76 in PE patients and 2.56±0.35 in

deformity is considered to be resulted from existence of heart in left side.

normal subjects, and useful in judgment of operative indication.

syndrome. The research of responsible gene relating with PE is yet on the start line.

1:1,000 to 2,500 (Kotzot & Schwabegger, 2009).

The lower costal cartilages bend inward to form depression.

**1.2 Symptomatology**

child age.

proposed.

Fig. 1. Cup shape depression

#### Fig. 3. Grand Canyon shape depression

Nakahara and colleagues (Nakahara et al., 1987) set 3 indices; degree of depression, degree of asymmetry, and degree of flatness (Fig. 4). These indices serve to define the diagnosis, to decide operative indication, and evaluate the effects of operation.

Masaoka and colleagues (Masaoka et al., 2011) proposed 3 other indices; steepness index, excavation volume index, and asymmetry index. These are defined as shown in Fig. 5. This system has properties described below.

Fig. 4. Deformity indices (Nakahara et al., 1987)

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 141

PE causes compression of lung and heart, which might result in impairment of respiratory and circulatory function. The impairment of these functions is sometimes too modest to be evaluated by usual function tests preoperatively, in spite of existence of subjective symptoms. In order to recognize relationship between the results of the examinations and PE deformity, it is important to confirm, 1) difference between those of the PE patients and the healthy controls, 2) relationship with the severity of deformity, and 3) improvement by surgical repair. Clinically, the results of such examinations could be parameters to evaluate the

Since 1950's, many papers on this topic have been published. In early years, measurement of conventional standard pulmonary volumes by spirometer was only one tool. Thereafter, flow-volume curve by pneumotachograph, TLC (Table 2) and RV by nitrogen washout, or body plethysmograph, and various work load tests were adopted. However, there are various opinions concerning what is the representative parameter expressing decreased

**1.3 Physiology**

effects of surgeries.

**1.3.1 Respiratory function**

pulmonary function due to PE deformity.

MVV maximal ventilation volume FVC forced ventilatory capacity FEV forced expiratory volume

RVSD right ventricle short distance PWC physical work capacity

cardiovascular factor, but not ventilatory.

FEV1 forced expiratory volume in 1 second

FEF25%~75% forced expiratory flow from 25% exhalation to 75% exhalation

In early years, MVV was thought to be a most reliable parameter to assess the reduced pulmonary function. Haller and colleague (Haller & Loughlin, 2000) pointed out significant difference of TLC, FVC, and RV between the PE patients and the matched controls. Nuss and colleague (Nuss & Kelly, 2008) stated that FVC, FEV1, FEF25%-75% reduced in the PE patients, comparing with the age-, height-matched predictive values. Which spirometrical parameter is definitely different between the PE patients and the controls are not agreed in various papers. Castile and colleagues (Castile et al., 1982) reported that in the symptomatic PE patients, the measured oxygen uptake increasingly exceeded the predicted value as the work loads approached maximum, different from healthy controls. Haller and colleague (Haller & Loughlin, 2000) did not find difference of O2 pulse (O2 consumption/heart rate) after treadmill works between the PE patients and the controls. On the other hand, Malek and colleagues (Malek et al, 2003) reported that the O2 pulse on maximal exercise testing was significantly lower than the reference value, and furthermore, this limitation was caused by

As for the relationship between decreased pulmonary function and severity of deformity, Lawson and colleagues (Lawson et al., 2011), after investigation of 310 PE patients, reported that the percentages of the patients with abnormal FVC, FEV1, FEF25%-75%, and TLC data

TLC total lung capacity RV residual volume

RV right ventricle

SV stroke volume Table 2. List of abbreviations

Fig. 5. Deformity indices (Masaoka et al., in press) (Reprinted with permission from *Eur J Cardiothorac Surg* 2011, in press)

PE is a symptomatic disease. Kelly (Kelly, 2008) investigated medical history of 327 PE patients (Table 1). Various symptoms with high percentages are listed up. Representatives are exercise intolerance, lack of endurance, shortness of breath, chest pain etc. Marfan syndrome (4.6%) and Ehlers-Danlos syndrome (2.8%) are associated with PE. Additionally, association of various psychological disturbances is pointed out, such as self-consciousness about chest, withdrawal from social and sports activities, depression even to suicide.

*Abbreviations:* PC, pectus carinatum; URI, upper respiratory infection.

Table 1. Medical history of 327 patients (Kelly, 2008)

(Reprinted with permission from *Semin Pediatr Surg* 2008; 17;189)

#### **1.3 Physiology**

140 Topics in Thoracic Surgery

PE is a symptomatic disease. Kelly (Kelly, 2008) investigated medical history of 327 PE patients (Table 1). Various symptoms with high percentages are listed up. Representatives are exercise intolerance, lack of endurance, shortness of breath, chest pain etc. Marfan syndrome (4.6%) and Ehlers-Danlos syndrome (2.8%) are associated with PE. Additionally, association of various psychological disturbances is pointed out, such as self-consciousness

Condition Number Percentage

about chest, withdrawal from social and sports activities, depression even to suicide.

Exercise intolerance 211 64.5 Lack of endurance 205 62.7 Shortness of breath 203 62.1 Chest pain with exercise 167 51.1 Family history of PE 140 42.8 Chest pain without exercise 104 31.8 Asthma 70 21.4 Scoliosis 69 21.1 Cardiac abnormalities 65 19.9 Frequent or prolonged URI 44 13.5 Palpitations 37 11.3 Pneumonia 28 8.6 Fainting/dizziness 27 8.3 Marfan syndrome 15 4.6 Family history of PC 13 4.0 Ehlers-Danlos syndrome 9 2.8 Family history of Marfan syndrome 8 2.4 Patient adopted 4 1.2 Patient has identical twin 3 0.9 Family history of Ehlers-Danlos syndrome 2 0.6 Sprengel deformity 2 0.6

Fig. 5. Deformity indices (Masaoka et al., in press)

(Reprinted with permission from *Eur J Cardiothorac Surg* 2011, in press)

*Abbreviations:* PC, pectus carinatum; URI, upper respiratory infection.

(Reprinted with permission from *Semin Pediatr Surg* 2008; 17;189)

Table 1. Medical history of 327 patients (Kelly, 2008)

PE causes compression of lung and heart, which might result in impairment of respiratory and circulatory function. The impairment of these functions is sometimes too modest to be evaluated by usual function tests preoperatively, in spite of existence of subjective symptoms. In order to recognize relationship between the results of the examinations and PE deformity, it is important to confirm, 1) difference between those of the PE patients and the healthy controls, 2) relationship with the severity of deformity, and 3) improvement by surgical repair. Clinically, the results of such examinations could be parameters to evaluate the effects of surgeries.

## **1.3.1 Respiratory function**

Since 1950's, many papers on this topic have been published. In early years, measurement of conventional standard pulmonary volumes by spirometer was only one tool. Thereafter, flow-volume curve by pneumotachograph, TLC (Table 2) and RV by nitrogen washout, or body plethysmograph, and various work load tests were adopted. However, there are various opinions concerning what is the representative parameter expressing decreased pulmonary function due to PE deformity.

Table 2. List of abbreviations

In early years, MVV was thought to be a most reliable parameter to assess the reduced pulmonary function. Haller and colleague (Haller & Loughlin, 2000) pointed out significant difference of TLC, FVC, and RV between the PE patients and the matched controls. Nuss and colleague (Nuss & Kelly, 2008) stated that FVC, FEV1, FEF25%-75% reduced in the PE patients, comparing with the age-, height-matched predictive values. Which spirometrical parameter is definitely different between the PE patients and the controls are not agreed in various papers.

Castile and colleagues (Castile et al., 1982) reported that in the symptomatic PE patients, the measured oxygen uptake increasingly exceeded the predicted value as the work loads approached maximum, different from healthy controls. Haller and colleague (Haller & Loughlin, 2000) did not find difference of O2 pulse (O2 consumption/heart rate) after treadmill works between the PE patients and the controls. On the other hand, Malek and colleagues (Malek et al, 2003) reported that the O2 pulse on maximal exercise testing was significantly lower than the reference value, and furthermore, this limitation was caused by cardiovascular factor, but not ventilatory.

As for the relationship between decreased pulmonary function and severity of deformity, Lawson and colleagues (Lawson et al., 2011), after investigation of 310 PE patients, reported that the percentages of the patients with abnormal FVC, FEV1, FEF25%-75%, and TLC data

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 143

In summary, compression of the right heart leads to diminished stroke volume, and in accordance with the modest decrease of ventilatory activity, leads to diminished cardiopulmonary capacity in severe PE patients. Repair surgery for PE could improve

Surgical correction for PE started at early 20th century, but could not gain successful results. The pioneer to open the curtain to modern era of the corrective surgery for PE was Brown (Brown 1939). Observing inward retraction of the xiphoid in baby's crying, he thought that the retraction is caused by the traction of diaphragm, and speculated that the substance of it could be the substernal ligament and the transverse thoracic muscle which combine the diaphragm with the sternum. Furthermore, he speculated that delay of such condition in

So, he thought up two procedures. The 1st was indicated to infant or little child. The core of the procedure is to dissect the substernal ligament and/or the attachment to diaphragm under a small, vertical incision at level of the xiphoid. He expected that such procedure

The 2nd was indicated to elder child, adolescent and adult. The core of this procedure is 1) resection of 2cm costo-chondral segments of the 4th~7th ribs near the junction with the sternum, and 2) wedge resection of the sternum on the anterior table at level of junction of the sternal body and the manubrium. Elevation of the sternum is secured by the sutures with wire at the wedge resection site (Fig.6.). He added an external traction by fixation of a wire penetrated the caudal part of the sternum and the 5th costal cartilage with a ladder

cardiorespiratory function in the patients with impaired function.

infancy could lead to the definite thoracic deformity in adult.

could prevent to proceed to the definitive PE deformity in adult.

**2. Corrective surgery for pectus excavatum**

**2.1 Overview**

placed on the anterior chest wall.

Fig. 6. The 2nd procedure of Brown (Brown, 1939)

increased with increasing Haller index. He speculated that the large tidal volume required by exercise causes some of the symptoms by the effects of deformity on rib motion and bellows function.

Comparison of the results of pulmonary function tests in preoperative and postoperative period was performed Cahill and colleagues (Cahill et al., 1984). He pointed out significant improvement of MVV, and improvement in exercise performance as quantified by maximal O2 consumption. Haller and colleague (Haller & Loughlin, 2000) recognized a higher O2 pulse in exercising tests in postoperative patients. Nuss and colleague (Nuss & Kelly, 2008) pointed out normalization of FEV1, TLC, and diffusing lung capacity. Lawson and colleagues (Lawson et al., 2005) found that FVC, FEV1, and FEF25%-75% were lower than normal preoperatively, and small but significant improvement of these 3 parameters after the Nuss surgeries and bar removals.

The above-described data about relationship between pulmonary function and PE deformity from many articles are summarized to be unfixed, because conduct of spirometrical test is difficult in child, and comparison of preoperative and postoperative data is difficult due to growth.

#### **1.3.2 Cardiovascular function**

Assessment of the cardiovascular function in PE began with introduction of cardiac catheterization. The hemodynamics measured at rest revealed normal pattern in majority of the early papers. However, Fabricius and colleagues (Fabricius et al., 1957) recognized abnormal high right atrial pressure in 3, and increased right ventricular pressure in one, after examinations of 26 patients.

Introduction of angiocardiography clarified dysmorphology of heart by dislocation or compression, i.e., anterior rotation of right atrium and compression on right lateral wall. CT scan substituted the angiocardiography, and verified noninvasively mitral valve prolapse and sternal imprint on the anterior wall of the right ventricle (Shamberger et al., 1987).

Radioisotope angiography appeared as a substitute of cardiac catheterization or angiography with contrast material. Shamberger and Welch performed this examination in 25 cases, and pointed out enlarged right atrium in 15, enlarged right ventricle in 15, dilated pulmonary artery in 10, delayed pulmonary artery transit time in 11, decreased left ventricular volume in 9, and compression defect in the right ventricle in 8 (Shamberger & Welch, 1988).

On the other hand, cardiac catheterization on exercise added new informations. Bevegard (Bevegard, 1962) performed right heart catheterization in 16 patients and found that the patients with severe PE showed 20% decrease of PWC (Table 2) from the supine position to the sitting position, similar to that of the normal subjects. But, increase of SV from rest to exercise was only 18.5%, much less than the 51% increase in the normal subjects. This finding could be an evidence of the exercise-induced symptoms in the PE patients.

Peterson and colleagues (Peterson et al., 1985) assessed cardiac volume and output with radionuclide angiography in 13 patients, and compared those data in preoperative and postoperative period. He found that the right ventricular ejection fraction at rest decreased after repair, which could be attributed to the increase of the right ventricular and diastolic volume index after operation. However, cardiac index did not increase significantly after operation at rest or during exercise.

Recently, cardiovascular resonance imaging was introduced to this field. Saleh and colleagues (Saleh et al., 2010) examined this test in 30 patients, and found reduction of RV ejection fraction and RVSD both at end diastole and systole.

In summary, compression of the right heart leads to diminished stroke volume, and in accordance with the modest decrease of ventilatory activity, leads to diminished cardiopulmonary capacity in severe PE patients. Repair surgery for PE could improve cardiorespiratory function in the patients with impaired function.

## **2. Corrective surgery for pectus excavatum**

## **2.1 Overview**

142 Topics in Thoracic Surgery

increased with increasing Haller index. He speculated that the large tidal volume required by exercise causes some of the symptoms by the effects of deformity on rib motion and

Comparison of the results of pulmonary function tests in preoperative and postoperative period was performed Cahill and colleagues (Cahill et al., 1984). He pointed out significant improvement of MVV, and improvement in exercise performance as quantified by maximal O2 consumption. Haller and colleague (Haller & Loughlin, 2000) recognized a higher O2 pulse in exercising tests in postoperative patients. Nuss and colleague (Nuss & Kelly, 2008) pointed out normalization of FEV1, TLC, and diffusing lung capacity. Lawson and colleagues (Lawson et al., 2005) found that FVC, FEV1, and FEF25%-75% were lower than normal preoperatively, and small but significant improvement of these 3 parameters after

The above-described data about relationship between pulmonary function and PE deformity from many articles are summarized to be unfixed, because conduct of spirometrical test is difficult in child, and comparison of preoperative and postoperative data is difficult due to

Assessment of the cardiovascular function in PE began with introduction of cardiac catheterization. The hemodynamics measured at rest revealed normal pattern in majority of the early papers. However, Fabricius and colleagues (Fabricius et al., 1957) recognized abnormal high right atrial pressure in 3, and increased right ventricular pressure in one,

Introduction of angiocardiography clarified dysmorphology of heart by dislocation or compression, i.e., anterior rotation of right atrium and compression on right lateral wall. CT scan substituted the angiocardiography, and verified noninvasively mitral valve prolapse and sternal imprint on the anterior wall of the right ventricle (Shamberger et al., 1987). Radioisotope angiography appeared as a substitute of cardiac catheterization or angiography with contrast material. Shamberger and Welch performed this examination in 25 cases, and pointed out enlarged right atrium in 15, enlarged right ventricle in 15, dilated pulmonary artery in 10, delayed pulmonary artery transit time in 11, decreased left ventricular volume in

On the other hand, cardiac catheterization on exercise added new informations. Bevegard (Bevegard, 1962) performed right heart catheterization in 16 patients and found that the patients with severe PE showed 20% decrease of PWC (Table 2) from the supine position to the sitting position, similar to that of the normal subjects. But, increase of SV from rest to exercise was only 18.5%, much less than the 51% increase in the normal subjects. This

Peterson and colleagues (Peterson et al., 1985) assessed cardiac volume and output with radionuclide angiography in 13 patients, and compared those data in preoperative and postoperative period. He found that the right ventricular ejection fraction at rest decreased after repair, which could be attributed to the increase of the right ventricular and diastolic volume index after operation. However, cardiac index did not increase significantly after

Recently, cardiovascular resonance imaging was introduced to this field. Saleh and colleagues (Saleh et al., 2010) examined this test in 30 patients, and found reduction of RV

9, and compression defect in the right ventricle in 8 (Shamberger & Welch, 1988).

finding could be an evidence of the exercise-induced symptoms in the PE patients.

bellows function.

growth.

the Nuss surgeries and bar removals.

**1.3.2 Cardiovascular function**

after examinations of 26 patients.

operation at rest or during exercise.

ejection fraction and RVSD both at end diastole and systole.

Surgical correction for PE started at early 20th century, but could not gain successful results. The pioneer to open the curtain to modern era of the corrective surgery for PE was Brown (Brown 1939). Observing inward retraction of the xiphoid in baby's crying, he thought that the retraction is caused by the traction of diaphragm, and speculated that the substance of it could be the substernal ligament and the transverse thoracic muscle which combine the diaphragm with the sternum. Furthermore, he speculated that delay of such condition in infancy could lead to the definite thoracic deformity in adult.

So, he thought up two procedures. The 1st was indicated to infant or little child. The core of the procedure is to dissect the substernal ligament and/or the attachment to diaphragm under a small, vertical incision at level of the xiphoid. He expected that such procedure could prevent to proceed to the definitive PE deformity in adult.

The 2nd was indicated to elder child, adolescent and adult. The core of this procedure is 1) resection of 2cm costo-chondral segments of the 4th~7th ribs near the junction with the sternum, and 2) wedge resection of the sternum on the anterior table at level of junction of the sternal body and the manubrium. Elevation of the sternum is secured by the sutures with wire at the wedge resection site (Fig.6.). He added an external traction by fixation of a wire penetrated the caudal part of the sternum and the 5th costal cartilage with a ladder placed on the anterior chest wall.

Fig. 6. The 2nd procedure of Brown (Brown, 1939)

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 145

Proposals from plastic surgeons are various. In 1970, Masson and colleagues (Masson et al., 1970) implanted a preformed silastic implant, with Dacron mesh patches on it, through a transverse incision just below the xiphoid. Although 40 years passed since then, the accumulated cases were not so many. Snel and colleagues (Snel et al., 2009) summed up 75 cases from 5 papers, and showed the long term outcome results of his own 16 patients. Complications occurred in 7 patients (43%); prolonged seroma in 5, in whom explantation of the implant were performed in one, reoperation and repositioning in 2 patients. Two patients underwent explantation of the implants due to pain and discomfort. Satisfactions of

the patients were evaluated by 4 grades; excellent 1, good 8, mediocre 3, and poor 1.

of foreign body do not always provide good results.

Fig. 9. Magnetic Mini-Mover Procedure (Harrison et al., 2010) (Reprinted with permission from *J Pediatr Surg* 2010;45:187)

description will appear in the 2.3 chapter.

were not yet gained.

Michlits and colleagues (Michlits et al., 2009) performed free fasciocutaneous infragluteal flap in 6 patients. He stated that the patients were satisfied on shape of the corrected PE. However, these plastic surgeries can not release the hearts and lungs from compression. Accordingly, they could not be indicated for symptomatic patients. Moreover, implantations

In 1998, Nuss and colleagues (Nuss et al., 1998) advocated a new procedure "minimally invasive technique for PE". This proposal invited revolution of corrective surgery for PE. The minimally invasive operation was reinforced with various modifications, and subsequently increased the followers worldwide. Concerning this procedure, detailed

Some new trials originating from new ideas appeared. Harrison and colleagues (Harrison et al., 2010) proposed Magnetic Mini-Mover Procedure (3MP). The 3MP uses a magnetic implant coupled with an external magnet to generate force sufficient to gradually remodel PE deformities. The magnimplant is set at surface of the sternum, and the magnatract is set on the rear table of the external brace, which is worn by the patient (Fig. 9.). The principle of this procedure is that the magnetic gravitation between the magnimplant and the magnatract lifts up the sternum. He performed this procedure in 10 patients, but outcome results were not yet gained. Schier and colleagues (Schier et al., 2005) proposed the vacuum chest wall lifter, which is set at anterior chest wall. Suction with this apparatus is conducted from 30 minutes to 5 hours daily, and repeated for 2 year (Fig. 10.). Reliable outcome results

The Brown's procedure was took over by Sweet (Sweet, 1944), and led to the Ravitch's procedure, which will be described in the next chapter.

A variety of corrective procedures have appeared, based on various ideas. The sternal turnover method was performed by Wada and colleagues (Wada et al., 1965) in Japan. He made an isolated plastron from the anterior chest wall, and turned it over, and sutured it back in place (Fig. 7.). This procedure had been performed in many cases in Japan, but not in other countries, because it had risk of necrosis of plastron. Laituri and colleagues (Laituri et al., 2010) described that osteo-necrosis or fistula formation occurred in 46% of the patients over 15 years of age in Wada's series. And such complications prompted Taguchi to preserve the internal mammary vessels to maintain the blood flow to the sternum (cited from Laituri et al.). However, despite of such modification, sternal turnover procedure became to be performed infrequently. Recently, Ninkovic and colleagues (Ninkovic et al., 2003) planned revival of the sternal turnover procedure, using technique of vascular surgery to anastomose the internal mammary vessels, i.e., the right internal mammary vessels to the inverted left mammary vessels (Fig. 8.).

Fig. 7. "Sternoturnover" procedure (Wada et al., 1965)

Fig. 8. Ninkovic's modification of "Sternoturnover" (Ninkovic et al., 2003) (Reprinted with permission from *Plast Reconst Surg* 2003;112:1356)

The Brown's procedure was took over by Sweet (Sweet, 1944), and led to the Ravitch's

A variety of corrective procedures have appeared, based on various ideas. The sternal turnover method was performed by Wada and colleagues (Wada et al., 1965) in Japan. He made an isolated plastron from the anterior chest wall, and turned it over, and sutured it back in place (Fig. 7.). This procedure had been performed in many cases in Japan, but not in other countries, because it had risk of necrosis of plastron. Laituri and colleagues (Laituri et al., 2010) described that osteo-necrosis or fistula formation occurred in 46% of the patients over 15 years of age in Wada's series. And such complications prompted Taguchi to preserve the internal mammary vessels to maintain the blood flow to the sternum (cited from Laituri et al.). However, despite of such modification, sternal turnover procedure became to be performed infrequently. Recently, Ninkovic and colleagues (Ninkovic et al., 2003) planned revival of the sternal turnover procedure, using technique of vascular surgery to anastomose the internal mammary vessels, i.e., the right internal mammary vessels to the

procedure, which will be described in the next chapter.

inverted left mammary vessels (Fig. 8.).

Fig. 7. "Sternoturnover" procedure (Wada et al., 1965)

Fig. 8. Ninkovic's modification of "Sternoturnover" (Ninkovic et al., 2003)

(Reprinted with permission from *Plast Reconst Surg* 2003;112:1356)

Proposals from plastic surgeons are various. In 1970, Masson and colleagues (Masson et al., 1970) implanted a preformed silastic implant, with Dacron mesh patches on it, through a transverse incision just below the xiphoid. Although 40 years passed since then, the accumulated cases were not so many. Snel and colleagues (Snel et al., 2009) summed up 75 cases from 5 papers, and showed the long term outcome results of his own 16 patients. Complications occurred in 7 patients (43%); prolonged seroma in 5, in whom explantation of the implant were performed in one, reoperation and repositioning in 2 patients. Two patients underwent explantation of the implants due to pain and discomfort. Satisfactions of the patients were evaluated by 4 grades; excellent 1, good 8, mediocre 3, and poor 1.

Michlits and colleagues (Michlits et al., 2009) performed free fasciocutaneous infragluteal flap in 6 patients. He stated that the patients were satisfied on shape of the corrected PE. However, these plastic surgeries can not release the hearts and lungs from compression. Accordingly, they could not be indicated for symptomatic patients. Moreover, implantations of foreign body do not always provide good results.

In 1998, Nuss and colleagues (Nuss et al., 1998) advocated a new procedure "minimally invasive technique for PE". This proposal invited revolution of corrective surgery for PE. The minimally invasive operation was reinforced with various modifications, and subsequently increased the followers worldwide. Concerning this procedure, detailed description will appear in the 2.3 chapter.

Some new trials originating from new ideas appeared. Harrison and colleagues (Harrison et al., 2010) proposed Magnetic Mini-Mover Procedure (3MP). The 3MP uses a magnetic implant coupled with an external magnet to generate force sufficient to gradually remodel PE deformities. The magnimplant is set at surface of the sternum, and the magnatract is set on the rear table of the external brace, which is worn by the patient (Fig. 9.). The principle of this procedure is that the magnetic gravitation between the magnimplant and the magnatract lifts up the sternum. He performed this procedure in 10 patients, but outcome results were not yet gained. Schier and colleagues (Schier et al., 2005) proposed the vacuum chest wall lifter, which is set at anterior chest wall. Suction with this apparatus is conducted from 30 minutes to 5 hours daily, and repeated for 2 year (Fig. 10.). Reliable outcome results were not yet gained.

Fig. 9. Magnetic Mini-Mover Procedure (Harrison et al., 2010) (Reprinted with permission from *J Pediatr Surg* 2010;45:187)

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 147

In 1965, Ravitch revised his original procedure; 1) oblique chondrotomy at 2nd or 3rd costal cartilages and overlap suture (Fig. 12 a.), and 2) sternal osteotomy on the rear table of the sternum, instead of the anterior table in the original procedure, and insertion of a small bone graft in the sternal opening (Fig. 12 b.). Such modifications were done, in order to elevate the sternum more effectively and prevent recurrence of depression. At present, the procedure with the above-stated modifications i.e., 3points fixation, is denominated "Ravitch procedure".

a)

Fig. 12. Modified Ravitch procedure by himself

procedure as a much less extensive repair.

(Reprinted with permission from *Kokyukigeka 1st ed.*, p. 378, Nanzan-do)

The most faithful successor to Ravitch is Alex Haller Jr. Although he took over the Ravitch procedure in general, he pointed out 2 important problems. The 1st is a fact that support of the anterior chest wall by the Ravitch procedure is not sufficient for children older than 10 years of age, teenagers, and adults. So, he performed placement of a temporary stainless steel strut beneath the sternum, and the strut is anchored bilaterally to the 5th or 6th ribs (Haller et al., 1989). The strut is removed on an outpatient basis, 6~9 months after the primary repair. The 2nd is a suggestion, that too extensive and too early operation could induce chest wall constriction. The total resections of the deformed costal cartilages in Ravitch procedure resulted to remove growth center activity of costal cartilages. He recognized such severe complication in 12 children, and denominated "aquired Jeune's syndrome" (Haller et al., 1996). He corrected his procedure as follows; 1) exclusion of the children below 4 years of age from indication of the repair, 2) shortening of the resected costal cartilages by 2.5cm. Fonkalsrud remarked that after the removal of deformed costal cartilages in the Ravitch's procedure, the regenerated cartilages are thin, irregular, and commonly rigid with calcification, even if the perichondrial sheaths are preserved. So, he removed short chips (3~8mm) of the costal cartilages on the medial and the lateral ends of the deformed cartilages (Fonkalsrud, 2004) (Fig. 13.). The remaining costal cartilages are reattached to the sternum and the ribs. Following to a transverse wedge osteotomy on the anterior table of the sternum and fixation of a thin stainless steel Adkins strut was placed posterior to both the sternum and the costal cartilages to elevate the sternum (Fig. 14). He evaluates this

b)

Fig. 10. The vacuum chest wall lifter (Schier, 2005) (Reprinted with permission from *J Pediatr Surg* 2005;40:497)

#### **2.2 Ravitch's procedure (open repair) 2.2.1 Procedure**

In 1949, Ravitch proposed his original procedure for PE. He intended to make free the sternum from all restrictions. In order to carry out his idea, he felt it necessary to divide the xiphi-sternal articulation and substernal ligament and to resect all the deformed costal cartilages for the length of their deformity. Furthermore, to elevate the sternum, he performed a transverse cuneiform osteotomy at the sterno-manubriul junction and sutured it, to maintain the corrected position (Fig. 11.). He did not use the traction apparatus that Brown used. The extensive defect resulting from excision of all of the deformed cartilages was left without sutures of any tissues.

Fig. 11. Original Ravitch procedure (Ravitch, 1949) (Reprinted with permission from Ann Surg 1949;129:434)

In 1949, Ravitch proposed his original procedure for PE. He intended to make free the sternum from all restrictions. In order to carry out his idea, he felt it necessary to divide the xiphi-sternal articulation and substernal ligament and to resect all the deformed costal cartilages for the length of their deformity. Furthermore, to elevate the sternum, he performed a transverse cuneiform osteotomy at the sterno-manubriul junction and sutured it, to maintain the corrected position (Fig. 11.). He did not use the traction apparatus that Brown used. The extensive defect resulting from excision of all of the deformed cartilages

Fig. 10. The vacuum chest wall lifter (Schier, 2005)

**2.2 Ravitch's procedure (open repair)**

was left without sutures of any tissues.

Fig. 11. Original Ravitch procedure (Ravitch, 1949) (Reprinted with permission from Ann Surg 1949;129:434)

**2.2.1 Procedure**

(Reprinted with permission from *J Pediatr Surg* 2005;40:497)

In 1965, Ravitch revised his original procedure; 1) oblique chondrotomy at 2nd or 3rd costal cartilages and overlap suture (Fig. 12 a.), and 2) sternal osteotomy on the rear table of the sternum, instead of the anterior table in the original procedure, and insertion of a small bone graft in the sternal opening (Fig. 12 b.). Such modifications were done, in order to elevate the sternum more effectively and prevent recurrence of depression. At present, the procedure with the above-stated modifications i.e., 3points fixation, is denominated "Ravitch procedure".

Fig. 12. Modified Ravitch procedure by himself (Reprinted with permission from *Kokyukigeka 1st ed.*, p. 378, Nanzan-do)

The most faithful successor to Ravitch is Alex Haller Jr. Although he took over the Ravitch procedure in general, he pointed out 2 important problems. The 1st is a fact that support of the anterior chest wall by the Ravitch procedure is not sufficient for children older than 10 years of age, teenagers, and adults. So, he performed placement of a temporary stainless steel strut beneath the sternum, and the strut is anchored bilaterally to the 5th or 6th ribs (Haller et al., 1989). The strut is removed on an outpatient basis, 6~9 months after the primary repair.

The 2nd is a suggestion, that too extensive and too early operation could induce chest wall constriction. The total resections of the deformed costal cartilages in Ravitch procedure resulted to remove growth center activity of costal cartilages. He recognized such severe complication in 12 children, and denominated "aquired Jeune's syndrome" (Haller et al., 1996). He corrected his procedure as follows; 1) exclusion of the children below 4 years of age from indication of the repair, 2) shortening of the resected costal cartilages by 2.5cm.

Fonkalsrud remarked that after the removal of deformed costal cartilages in the Ravitch's procedure, the regenerated cartilages are thin, irregular, and commonly rigid with calcification, even if the perichondrial sheaths are preserved. So, he removed short chips (3~8mm) of the costal cartilages on the medial and the lateral ends of the deformed cartilages (Fonkalsrud, 2004) (Fig. 13.). The remaining costal cartilages are reattached to the sternum and the ribs. Following to a transverse wedge osteotomy on the anterior table of the sternum and fixation of a thin stainless steel Adkins strut was placed posterior to both the sternum and the costal cartilages to elevate the sternum (Fig. 14). He evaluates this procedure as a much less extensive repair.

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 149

Robicsek hated metal materials supporting the elevated sternum, because they have the potential to leave their original position and wander all over the body. Even if the metal support remains in place, it has possibility to destroy the surrounding organ. So, he thought up a new supporting system using Marlex mesh. After wedge resection of the sternum and resection of the deformed cartilages and detachment of the xiphoid, the sternum is maintained in its corrected position by suturing a sheath of Marlex mesh taut under it (Robicsek, 2000) (Fig. 15.). Concerning the destiny of the implanted material, Robicsek stated that in 2 reoperating cases due to recurrence of depression, he found the area where the mesh was inserted to be a fibrocartilaginous plate with no identifiable traces of the mesh

Saxena and Willital (Saxena & Willital, 2007) reported 1,264 open-repair patients with the Willital-Hegemann procedure, which uses 3 struts. The 1st is passed transsternally, with its edges resting anteriorly on the ribs. The other 2 struts are set parasternally with the points of fixation being the 2nd ribs and the lowest ends of the rib cage (Fig. 16.). These struts are removed after 24~36 months. Saxena and colleagues (Saxena et al., 2007) performed the Nuss procedure in 160 patients from 2000~2006 too, and stated the open repair with

Hu and colleagues (Hu et al., 2008) performed open repair in 398 PE patients. His procedure consists of the resections of the deformed costal cartilages and fixation by "arch-shaped"

Willital–Hegemann procedure should be indicated to the severe deformed patients.

Fig. 15. Robicsek's procedure (Marlex mesh hammock) (Robicsek, 2000)

steal strut, which is removed 1 year after the operation.

(Robicsek, 2009).

Fig. 13. Fonkalsrud's procedure (I) (Fonalsrud, 2004) (Reprinted with permission from *Ann Surg* 2004;240:232)

Fig. 14. Fonkalsrud's procedure (II) (Fonalsrud, 2004) (Reprinted with permission from *Ann Surg* 2004;240:233)

Fig. 13. Fonkalsrud's procedure (I) (Fonalsrud, 2004) (Reprinted with permission from *Ann Surg* 2004;240:232)

Fig. 14. Fonkalsrud's procedure (II) (Fonalsrud, 2004) (Reprinted with permission from *Ann Surg* 2004;240:233) Robicsek hated metal materials supporting the elevated sternum, because they have the potential to leave their original position and wander all over the body. Even if the metal support remains in place, it has possibility to destroy the surrounding organ. So, he thought up a new supporting system using Marlex mesh. After wedge resection of the sternum and resection of the deformed cartilages and detachment of the xiphoid, the sternum is maintained in its corrected position by suturing a sheath of Marlex mesh taut under it (Robicsek, 2000) (Fig. 15.). Concerning the destiny of the implanted material, Robicsek stated that in 2 reoperating cases due to recurrence of depression, he found the area where the mesh was inserted to be a fibrocartilaginous plate with no identifiable traces of the mesh (Robicsek, 2009).

Saxena and Willital (Saxena & Willital, 2007) reported 1,264 open-repair patients with the Willital-Hegemann procedure, which uses 3 struts. The 1st is passed transsternally, with its edges resting anteriorly on the ribs. The other 2 struts are set parasternally with the points of fixation being the 2nd ribs and the lowest ends of the rib cage (Fig. 16.). These struts are removed after 24~36 months. Saxena and colleagues (Saxena et al., 2007) performed the Nuss procedure in 160 patients from 2000~2006 too, and stated the open repair with Willital–Hegemann procedure should be indicated to the severe deformed patients.

Hu and colleagues (Hu et al., 2008) performed open repair in 398 PE patients. His procedure consists of the resections of the deformed costal cartilages and fixation by "arch-shaped" steal strut, which is removed 1 year after the operation.

Fig. 15. Robicsek's procedure (Marlex mesh hammock) (Robicsek, 2000)

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 151

Hu and colleagues (Hu et al., 2008) had to perform early removal of metal bar due to its dislodge in 4 patients, and noticed recurrent depression in 3 patients (0.75%), and protrusion of 2nd or 3rd costal cartilages in 5 patients (1.26%). As the final results, normal contour of the costal cage was constructed in 98.74% (393/398). Cardiac function recovered to the

Fonkalsrud (Fonkalsrud, 2009) showed 8% of complication rate. Recurrence occurred in 5 patients from series I (17%), 15 patients from series II (4.3%), 7 patients from series III (3%), and 4 patients from series IV (1.3%), and total 22 patients (2.4%) underwent reoperations. The reoperation rates have decreased in the learning curve. Satisfaction of the patients or

Complications in our 307 patients of early series before the current procedure, which will be described in the 2.5 chapter, were 34 (11.0%). The list of each complication is shown in Table 4.

cence 2 <sup>1</sup>Pneumonia

1

(n=307) 5 6 9 2 5 7 <sup>34</sup>

In 1998, Nuss et al. proposed a new procedure — minimally invasive technique —, and assessed their 10 years results. The essence of this procedure is correction of thoracic cage by a metal bar inserted posterior to the sternum without incision or resection of costal cartilages.

Pleural effusion

2 1 Atelectasis

<sup>4</sup><sup>4</sup>

Pulmonary complications

1

1

1

Pulmonary edema 1 Atelectasis 1

Others Total

Paradoxical breathing 1

Ventilatory insufficiency

1 Early recurrent of

PE 1

2

Sudden death 1 Respiratory insufficiency

Pericardial effusion 1

(%)

7 (25.9)

4 (17.4)

> 8 (6.8)

11 (21.1)

(4.5)

(11.0)

parents was evaluated as very good or excellent by 94.2% of all patients.

Pneumothorax

(n=23) 1 2 Atelectasis

Wound complications

Wound necrosis 1

Wound infection 1 Osteomyelitis of sternum 1 Bleeding

1

Table 4. Complications in early series of us

healthy level of the same age.

Series Seroma

(n=27) <sup>2</sup>Dehis-

1

I

II

III (n=117) <sup>1</sup>

IV adult type (n=52)

IV child type (n=88)

Total

**2.3 Nuss procedure 2.3.1 Procedure**

Fig. 16. Saxena & Willital's procedure (Saxena and Willital, 2007) (Reprinted with permission from *J Thorac Cardiovasc Surg* 2007;134:874)

#### **2.2.2 Complications and outcome results**

Table 3 shows large series of the Ravitch repair. However, the complications and the outcome results in them are not completely clear, because these reviews deal not always their complications and outcome results, besides, even if such descriptions are involved, not in uniform style. Ravitch described about 2 cases with staphylococcal infection, one of whom underwent removal of sequester, and stated no other complications except them (Ravitch, 1965).

Haller and colleagues (Haller et al., 1989) stated the morbidity rate was less than 5%, and mean hospital stay was 7 days. Concerning of the outcome results, in early series during evolution of the surgical technique, reoperation occurred in 10% of the patients. However, recent series, revealed only 3 poor results in 352 patients. He stated that, therefore, the operative results with the current technique were quite satisfactory with greater than 95% of the patients having good to excellent results.

Table 3. Large series of Ravitch repair

Robicsek (Robicsek, 2000) stated that mean hospital stay was 3 days, and the important postoperative complications were bleeding from the internal mammary artery, infection, and seroma. However, he did not mention their frequency, as same as outcome results. Saxena and Willital (Saxena & Willital., 2007) stated that the complication rate was 5.7%, and hospital stay decreased from 20.5 days in early series to 6.2 days in recent series. They referred to late results, and indicated 1.4% of major recurrence and 3.6% of mild recurrence. Subjective complaints of the patients before the surgeries were eliminated in 97% of the patients.

Table 3 shows large series of the Ravitch repair. However, the complications and the outcome results in them are not completely clear, because these reviews deal not always their complications and outcome results, besides, even if such descriptions are involved, not in uniform style. Ravitch described about 2 cases with staphylococcal infection, one of whom underwent removal of sequester, and stated no other complications except them

Haller and colleagues (Haller et al., 1989) stated the morbidity rate was less than 5%, and mean hospital stay was 7 days. Concerning of the outcome results, in early series during evolution of the surgical technique, reoperation occurred in 10% of the patients. However, recent series, revealed only 3 poor results in 352 patients. He stated that, therefore, the operative results with the current technique were quite satisfactory with greater than 95% of

Author Publish Cases City (Nation) Remarks Ravitch 1965 164 Baltimore (USA) Original Ravitch

Robicsek 2000 800 Charlotte (USA) Marlex-mesh hammock

Willital 2007 1262 Graz (Austria) Wittel-Hegeman procedure

Robicsek (Robicsek, 2000) stated that mean hospital stay was 3 days, and the important postoperative complications were bleeding from the internal mammary artery, infection, and seroma. However, he did not mention their frequency, as same as outcome results. Saxena and Willital (Saxena & Willital., 2007) stated that the complication rate was 5.7%, and hospital stay decreased from 20.5 days in early series to 6.2 days in recent series. They referred to late results, and indicated 1.4% of major recurrence and 3.6% of mild recurrence. Subjective

complaints of the patients before the surgeries were eliminated in 97% of the patients.

(3 struts)

Haller 1989 664 Baltimore (USA) Strut

Hu 2008 398 Sichuan (China) Strut Fonkalsrud 2009 912 Los Angeles (USA) Strut Masaoka 2011 307 Nagoya (Japan) Metal-free

Fig. 16. Saxena & Willital's procedure (Saxena and Willital, 2007) (Reprinted with permission from *J Thorac Cardiovasc Surg* 2007;134:874)

**2.2.2 Complications and outcome results**

the patients having good to excellent results.

Table 3. Large series of Ravitch repair

(Ravitch, 1965).

Saxena and

Hu and colleagues (Hu et al., 2008) had to perform early removal of metal bar due to its dislodge in 4 patients, and noticed recurrent depression in 3 patients (0.75%), and protrusion of 2nd or 3rd costal cartilages in 5 patients (1.26%). As the final results, normal contour of the costal cage was constructed in 98.74% (393/398). Cardiac function recovered to the healthy level of the same age.

Fonkalsrud (Fonkalsrud, 2009) showed 8% of complication rate. Recurrence occurred in 5 patients from series I (17%), 15 patients from series II (4.3%), 7 patients from series III (3%), and 4 patients from series IV (1.3%), and total 22 patients (2.4%) underwent reoperations. The reoperation rates have decreased in the learning curve. Satisfaction of the patients or parents was evaluated as very good or excellent by 94.2% of all patients.

Complications in our 307 patients of early series before the current procedure, which will be described in the 2.5 chapter, were 34 (11.0%). The list of each complication is shown in Table 4.

Table 4. Complications in early series of us

## **2.3 Nuss procedure**

#### **2.3.1 Procedure**

In 1998, Nuss et al. proposed a new procedure — minimally invasive technique —, and assessed their 10 years results. The essence of this procedure is correction of thoracic cage by a metal bar inserted posterior to the sternum without incision or resection of costal cartilages.

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 153

Thereafter, he modificated the original procedure, in order to avoid the injuries to vessels and bar shifts. Modifications are as follows: 1) use of thoracoscopy to secure the insertion of introducer, 2) use of introducer (large metal rod) to secure the tunnel making, 3) fixation of the bar with stabilizer and fixing suture (Fig. 18.). The bar is stabilized by wiring a stabilizer to the left end of the bar and by placing sutures of polydioxanone (PDS) around the bar and underlying ribs on the right end. 4) Use of two or more bars in the patients with

Park and colleagues (Park et al., 2010) added 3 modifications; one of those is the crane technique. This technique involves elevating the depressed sternum before introducer insertion and lifting the wire suture along with the sternum by an operating table-mounted retractor system (Fig. 19.). This technique serves to alleviate the pressure on the bar, and to prevent internal organ injury during the passage of the introducer. The other is the morphology-tailored bar shaping system named "terrain contour matching". Each patient has distinctive terrain characteristics. Park planned to shape the bar as matched to the patient's thorax, and to compress the crest of the depression by the negative momentum of

unacceptable configuration after use of one bar.

the lever action, during rotation of the bar (Fig. 20.).

Fig. 18. Modification of Nuss procedure by Nuss (Nuss, 2005)

(Reprinted with permission from *Jpn J Thorac Cardiovasc Surg* 2005;53:339)

Fig. 19. Park's modification — crane technique — (Park et al., 2010) (Reprinted with permission from *J Thorac Cardiovasc Surg* 2010;139:381)

His idea was originated from observation of the thoracic deformity in the adult patients with chronic emphysematous lungs — barrel shaped thorax. He thought that, if this configuration of chest wall can occur long after their skeleton has matured and calcified, it should be possible to remodel the chest wall in children whose ribs and cartilages are still soft and pliable without having to resort to rib cartilage incision, resection, or sternal osteotomy. Detail of this procedure is as follows.

Fig. 17. Nuss procedure (Nuss et al., 1998) (Reprinted with permission from *J Pediatr Surg* 1998;33:548)

His idea was originated from observation of the thoracic deformity in the adult patients with chronic emphysematous lungs — barrel shaped thorax. He thought that, if this configuration of chest wall can occur long after their skeleton has matured and calcified, it should be possible to remodel the chest wall in children whose ribs and cartilages are still soft and pliable without having to resort to rib cartilage incision, resection, or sternal

1. Preoperatively, a proper length meal bar is selected, and bent into proper convexity. 2. A transverse incision 2.5cm long was made in each lateral chest wall between the

3. A skin tunnel was raised anteriorly, and 50cm long Kelly clamp is inserted along an intercostal space, and advanced slowly across the mediastinum immediately under the

4. Two strands of umbilical tape were pulled through the track (Fig. 17B.). One strand

5. When the track was deemed wide enough, a steel bar is pulled beneath the sternum using the umbilical tape for traction with the convexity facing posteriorly (Fig. 17C.). 6. When the bar is in position, it is turned over with a vice grip so that the convexity faces

osteotomy. Detail of this procedure is as follows.

anteriorly (Fig. 17D.).

Fig. 17. Nuss procedure (Nuss et al., 1998)

(Reprinted with permission from *J Pediatr Surg* 1998;33:548)

anterior axillary and posterior axillary lines.

sternum until it emerged on the opposite side (Fig. 17A.).

7. The bar is secured with heavy sutures to the lateral chest wall muscles.

8. If the bar is unstable, a 2~4cm cross bar is attached to one or both ends of the bar.

serves to guide the Kelly clamp from another side.

Thereafter, he modificated the original procedure, in order to avoid the injuries to vessels and bar shifts. Modifications are as follows: 1) use of thoracoscopy to secure the insertion of introducer, 2) use of introducer (large metal rod) to secure the tunnel making, 3) fixation of the bar with stabilizer and fixing suture (Fig. 18.). The bar is stabilized by wiring a stabilizer to the left end of the bar and by placing sutures of polydioxanone (PDS) around the bar and underlying ribs on the right end. 4) Use of two or more bars in the patients with unacceptable configuration after use of one bar.

Park and colleagues (Park et al., 2010) added 3 modifications; one of those is the crane technique. This technique involves elevating the depressed sternum before introducer insertion and lifting the wire suture along with the sternum by an operating table-mounted retractor system (Fig. 19.). This technique serves to alleviate the pressure on the bar, and to prevent internal organ injury during the passage of the introducer. The other is the morphology-tailored bar shaping system named "terrain contour matching". Each patient has distinctive terrain characteristics. Park planned to shape the bar as matched to the patient's thorax, and to compress the crest of the depression by the negative momentum of the lever action, during rotation of the bar (Fig. 20.).

Fig. 18. Modification of Nuss procedure by Nuss (Nuss, 2005) (Reprinted with permission from *Jpn J Thorac Cardiovasc Surg* 2005;53:339)

Fig. 19. Park's modification — crane technique — (Park et al., 2010) (Reprinted with permission from *J Thorac Cardiovasc Surg* 2010;139:381)

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 155

complications, the majority is mild one, but hemothorax includes injury of internal mammary vessels. In the late complications, the majority is one concerning with the bar. The bar displacements occurred in 64 patients (5.7%), in whom 45 patients required repositioning. Kelly and colleagues (Kelly et al., 2010) stated that the bar displacement rate was 12% in the patients without stabilizer, 6% in the patients with wired stabilizer, and with the addition of pericostal sutures placed around the bar and underlying ribs, it dropped to

Author Publish Cases City (Nation) Remark Dzielicki 2006 461 Gliwice (Poland) Modified Nuss Olbrecht 2008 244 Baltimore (USA) Modified Nuss Pilegaard 2009 507 Aorhus (Denmark) Modified Nuss

Park 2010 1170 Ansan (Korea) Park modification Kelly (Nuss) 2010 1215 Norfolk (USA) Modified Nuss

Pneumothorax with spontaneous resolution 64.7% (n=727) Pneumothorax with chest tube 4.0% (n=45) Horner's syndrome 15.5% (n=174) Drug reaction 3.2% (n=36) Suture site infection 1.0% (n=11) Pneumonia 0.5% (n=6) Hemothorax 0.5% (n=6) Pericarditis 0.5% (n=5) Pleural effusion (requiring drainage) 0.3% (n=3) Death 0% Cardiac perforation 0%

Table 6. Early postoperative complications of primary surgical patients (Kelly et al., 2010)

Bar displacements—total 64/1123 (5.7%) Bar displacements requiring revision 45/1123 (4.0%) Overcorrection (none required surgery) 41/1123 (3.7%) Bar allergy (3 required bar removal) 35/1123 (3.1%) Recurrence 11/1123 (1.0%) Bar infection—total 6/1123 (0.5%) Bar infection—required early removal 3/1123 (0.3%) Hemothorax (post-traumatic) 4/1123 (0.4%) Lactosorb stabilizer inflammation 4/1123 (0.4%)

(Reprinted with permission from *Ann Surg* 2010;252:1076)

Table 7. Late postoperative complications (Kelly et al., 2010) (Reprinted with permission from *Ann Surg* 2010;252:1076)

(Absorbable stabilizer)

2%. It should be paid attention that bar allergy is found in 3.1%.

Table 5. Large series of Nuss repair

Fig. 20. Park's modification — terrain contour matching — (Park et al., 2010) (Reprinted with permission from *J Thorac Cardiovasc Surg* 2010;139:382)

The 3rd is the multipoint fixation of the bar. This technique consisted of fixation of the bar, not only at end of the bar, but also at hinge point. The fixation is performed by "through-the-skin" technique, i.e., the needle stick is made directly through the overlying skin, passed around the rib, and passed back through the skin. The sutured wires are grabbed via a subcutaneous dissection. The retrieved sutures are passed through the endhole of the bar and tied. This technique was developed, to prevent displacement of the bar.

Pilegaard and Licht (Pilegaard & Licht, 2008) proposed to use absorbable stabilizer in order to avoid chronic pain and necessity of removal. However, as it break easier than the one of metal, further research is needed.

#### **2.3.2 Complications and outcome results**

Table 5 shows large series of the Nuss repair.

The latest report about postoperative complications after the Nuss repair in the Nuss's institute is shown in Table 6 and Table 7 (Kelly et al., 2010). In the line-up of the early complications, the majority is mild one, but hemothorax includes injury of internal mammary vessels. In the late complications, the majority is one concerning with the bar. The bar displacements occurred in 64 patients (5.7%), in whom 45 patients required repositioning. Kelly and colleagues (Kelly et al., 2010) stated that the bar displacement rate was 12% in the patients without stabilizer, 6% in the patients with wired stabilizer, and with the addition of pericostal sutures placed around the bar and underlying ribs, it dropped to 2%. It should be paid attention that bar allergy is found in 3.1%.

Table 5. Large series of Nuss repair

154 Topics in Thoracic Surgery

Fig. 20. Park's modification — terrain contour matching — (Park et al., 2010) (Reprinted with permission from *J Thorac Cardiovasc Surg* 2010;139:382)

bar.

metal, further research is needed.

**2.3.2 Complications and outcome results**  Table 5 shows large series of the Nuss repair.

The 3rd is the multipoint fixation of the bar. This technique consisted of fixation of the bar, not only at end of the bar, but also at hinge point. The fixation is performed by "through-the-skin" technique, i.e., the needle stick is made directly through the overlying skin, passed around the rib, and passed back through the skin. The sutured wires are grabbed via a subcutaneous dissection. The retrieved sutures are passed through the endhole of the bar and tied. This technique was developed, to prevent displacement of the

Pilegaard and Licht (Pilegaard & Licht, 2008) proposed to use absorbable stabilizer in order to avoid chronic pain and necessity of removal. However, as it break easier than the one of

The latest report about postoperative complications after the Nuss repair in the Nuss's institute is shown in Table 6 and Table 7 (Kelly et al., 2010). In the line-up of the early

Table 6. Early postoperative complications of primary surgical patients (Kelly et al., 2010) (Reprinted with permission from *Ann Surg* 2010;252:1076)

Table 7. Late postoperative complications (Kelly et al., 2010) (Reprinted with permission from *Ann Surg* 2010;252:1076)

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 157

The Nuss procedure has merits, such as minimal operative wound, lesser operation time, and lesser blood loss, but simultaneously some demerits, such as frequent occurrence of complications, burden of bar(s) for a long time, and necessity of operation for removal of the

On the other hand, the open procedure has merits, such as safety and certainly of manipulation under direct vision, and demerits, such as longer operative time, and larger

However, there are only few papers comparing the results of both procedures, because the institutes performing both procedures are limited. Fonkalsrud and Hebra (Fonkalsrud et al., 2002) compared 68 Nuss and 139 open repairs in UCLA and South Carolina University, and concluded that the open repair needed longer operating time, but decreased hospital stay,

Nasr and colleagues (Nasr et al., 2010) reviewed 9 papers performing both procedures, and showed no difference with respect to overall complication and patient's satisfaction.

On the other hand, Robicsek argued against the Nuss procedure (Robicsek & Hebra, 2009). He asserted, how the two 3~4cm cuts (plus the hole for the videoscope) are shorter than the wound of the open repair, and how the Nuss repair is called "minimally invasive", in which a metal bar (or bars) is driven through both pleural cavities, passed by the width of a hair between the heart and the sternum and left there for long time, and then 2 years later the same procedure is performed in "reverse". Furthermore, he was worried, how the Nuss bars

Hebra argued that the "minimally invasive" implies achievement of correction without the large incisions and the removal of bone or cartilage, and frequent occurrence of the complications was related to early experience, so the Nuss procedure has eliminated

As described above, all institutes of the Nuss repair and almost institutes of the Ravitch repair use metal bar. Certainly, it can keep configuration of the thorax more rigidly than the

However, it has some shortcomings; 1) possibility to injure the surrounding organs by compression, 2) displacement requiring reoperation, 3) necessity of removal, 4) pain due to long time burden, 5) growth inhibition of the thorax, 6) metal allergy and 7) possibility of

The bar is left at the thoracic wall during 2~4 years in the Nuss procedure, and during 6~12 months in the Ravitch procedure. The longer the duration of bar burden is, the higher the

Accordingly, the metal-free procedures for PE have been tried in some institutes. Hayashi and Maruyama (Hayashi & Maruyama, 1992) used autologous rib with preserved anterior intercostalis branch of the internal mammary artery in 3 cases. The sternoturnover procedure has been performed in minimal cases of limited institutes (Iida et al., 2010;

On the other hand, trials using artificial materials to support the elevated sternum have been performed, too. Lane-Smith and colleagues (Lane-Smith et al., 1994) used tubular

**2.4 Pros and cons for both procedures**

complication rates, and use of pain medications.

may affect the costal cartilages of the growing child?

majority of complications by refinement of the procedure.

However, the rate of reoperation was higher in the Nuss Procedure.

bar(s).

operation wound.

**2.5 Metal-free procedure**

recurrence of depression after removal.

incidence of complications increases.

other materials.

Ninkovic et al., 2003).

Park and colleagues (Park et al., 2010) reported decrease of the complications, comparing the results of 1999~2002 and 2006~2008, as follows: total complications (57/335, 17.0% vs 33/394, 7.5%) pneumothorax (25/335, 7.5% vs 3/394, 0.8%), bar displacement (13/335, 3.8% vs 2/394, 0.5%). The reoperation rate also decreased (17/335, 5.1% vs 3/394, 0.8%).

Pilegaard and colleagues (Pilegaard et al., 2008) noticed in his series of 383 patients, pneumothorax in 178 (49%), bleeding in 2, pleural effusion in 4, seroma in 11, and deep infection in 8. Seven patients were reoperated because of the bar dislocation (1.8%). In another 13 patients, the stabilizer was removed because of pain.

However, the most important and characteristic complication in the Nuss procedure is massive hemorrhage due to injury of heart or great vessels (Bouchard et al., 2009; Aydemir et al., 2011). This complication is sometimes life-threatening and requires an emergency operation. It might occur during the initial operation, or in the late period of the postoperative course, or during bar removal (Jemielity et al., 2011; Haecker et al., 2009). Despite of various measures, this complication has occurred still.

Mean hospital stay is about 3 days, and the bar is removed after 2~4years, in most institutes performing the Nuss procedure.

The outcome results in the latest paper of the Nuss group are shown in Table 8. Evaluation was made, based on grades of satisfaction of the patients or the parents. Excellent results were gained in 85.3% (Kelly et al., 2010). They compared the preoperative and the postoperative pulmonary function data, and pointed out improvement in FVC (88% to 92%, p<0.001), FEV1 (83% to 88%, p=0.01), and FEF25%-75% (81% to 87%, p=not significant). They performed echocardiogram in the preoperative patients, and found mitral valve prolapse (MVP) in 216 patients (18%). Forty-four patients with preoperative MVP underwent echocardiogram after surgery, and 20 (44%) had resolution of the MVP.

Park and colleagues (Park et al., 2010) evaluated the operative results by CT indices: The Haller index changed from 6.05±11.82 to 2.76±0.49 (p<0.001); the depression index changed from 1.95±1.71 to 1 (p<0.001); and the asymmetry index changed from 1.08±0.05 to 1.02±0.02 (p<0.001).

Table 8. Results after bar removal median follow-up 854 days post bar removal (Kelly et al., 2010)

(Reprinted with permission from *Ann Surg* 2010;252:1076)

## **2.4 Pros and cons for both procedures**

156 Topics in Thoracic Surgery

Park and colleagues (Park et al., 2010) reported decrease of the complications, comparing the results of 1999~2002 and 2006~2008, as follows: total complications (57/335, 17.0% vs 33/394, 7.5%) pneumothorax (25/335, 7.5% vs 3/394, 0.8%), bar displacement (13/335, 3.8%

Pilegaard and colleagues (Pilegaard et al., 2008) noticed in his series of 383 patients, pneumothorax in 178 (49%), bleeding in 2, pleural effusion in 4, seroma in 11, and deep infection in 8. Seven patients were reoperated because of the bar dislocation (1.8%). In

However, the most important and characteristic complication in the Nuss procedure is massive hemorrhage due to injury of heart or great vessels (Bouchard et al., 2009; Aydemir et al., 2011). This complication is sometimes life-threatening and requires an emergency operation. It might occur during the initial operation, or in the late period of the postoperative course, or during bar removal (Jemielity et al., 2011; Haecker et al., 2009).

Mean hospital stay is about 3 days, and the bar is removed after 2~4years, in most institutes

The outcome results in the latest paper of the Nuss group are shown in Table 8. Evaluation was made, based on grades of satisfaction of the patients or the parents. Excellent results were gained in 85.3% (Kelly et al., 2010). They compared the preoperative and the postoperative pulmonary function data, and pointed out improvement in FVC (88% to 92%, p<0.001), FEV1 (83% to 88%, p=0.01), and FEF25%-75% (81% to 87%, p=not significant). They performed echocardiogram in the preoperative patients, and found mitral valve prolapse (MVP) in 216 patients (18%). Forty-four patients with preoperative MVP underwent echocardiogram after surgery, and 20 (44%) had

Park and colleagues (Park et al., 2010) evaluated the operative results by CT indices: The Haller index changed from 6.05±11.82 to 2.76±0.49 (p<0.001); the depression index changed from 1.95±1.71 to 1 (p<0.001); and the asymmetry index changed from 1.08±0.05 to 1.02±0.02

Table 8. Results after bar removal median follow-up 854 days post bar removal (Kelly et al.,

(Reprinted with permission from *Ann Surg* 2010;252:1076)

Total number of primary patients 1123 Total number with bar removed 790 • Excellent result 674 (85.3%) • Good result 83 (10.5%) • Fair result 11 (1.4%) • Poor result 6 (0.8%) • Recurrence requiring re-do operation 11 (1.4%) • No return 5 (0.6%)

vs 2/394, 0.5%). The reoperation rate also decreased (17/335, 5.1% vs 3/394, 0.8%).

another 13 patients, the stabilizer was removed because of pain.

Despite of various measures, this complication has occurred still.

performing the Nuss procedure.

resolution of the MVP.

(p<0.001).

2010)

The Nuss procedure has merits, such as minimal operative wound, lesser operation time, and lesser blood loss, but simultaneously some demerits, such as frequent occurrence of complications, burden of bar(s) for a long time, and necessity of operation for removal of the bar(s).

On the other hand, the open procedure has merits, such as safety and certainly of manipulation under direct vision, and demerits, such as longer operative time, and larger operation wound.

However, there are only few papers comparing the results of both procedures, because the institutes performing both procedures are limited. Fonkalsrud and Hebra (Fonkalsrud et al., 2002) compared 68 Nuss and 139 open repairs in UCLA and South Carolina University, and concluded that the open repair needed longer operating time, but decreased hospital stay, complication rates, and use of pain medications.

Nasr and colleagues (Nasr et al., 2010) reviewed 9 papers performing both procedures, and showed no difference with respect to overall complication and patient's satisfaction. However, the rate of reoperation was higher in the Nuss Procedure.

On the other hand, Robicsek argued against the Nuss procedure (Robicsek & Hebra, 2009). He asserted, how the two 3~4cm cuts (plus the hole for the videoscope) are shorter than the wound of the open repair, and how the Nuss repair is called "minimally invasive", in which a metal bar (or bars) is driven through both pleural cavities, passed by the width of a hair between the heart and the sternum and left there for long time, and then 2 years later the same procedure is performed in "reverse". Furthermore, he was worried, how the Nuss bars may affect the costal cartilages of the growing child?

Hebra argued that the "minimally invasive" implies achievement of correction without the large incisions and the removal of bone or cartilage, and frequent occurrence of the complications was related to early experience, so the Nuss procedure has eliminated majority of complications by refinement of the procedure.

#### **2.5 Metal-free procedure**

As described above, all institutes of the Nuss repair and almost institutes of the Ravitch repair use metal bar. Certainly, it can keep configuration of the thorax more rigidly than the other materials.

However, it has some shortcomings; 1) possibility to injure the surrounding organs by compression, 2) displacement requiring reoperation, 3) necessity of removal, 4) pain due to long time burden, 5) growth inhibition of the thorax, 6) metal allergy and 7) possibility of recurrence of depression after removal.

The bar is left at the thoracic wall during 2~4 years in the Nuss procedure, and during 6~12 months in the Ravitch procedure. The longer the duration of bar burden is, the higher the incidence of complications increases.

Accordingly, the metal-free procedures for PE have been tried in some institutes. Hayashi and Maruyama (Hayashi & Maruyama, 1992) used autologous rib with preserved anterior intercostalis branch of the internal mammary artery in 3 cases. The sternoturnover procedure has been performed in minimal cases of limited institutes (Iida et al., 2010; Ninkovic et al., 2003).

On the other hand, trials using artificial materials to support the elevated sternum have been performed, too. Lane-Smith and colleagues (Lane-Smith et al., 1994) used tubular

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 159

Fig. 22. shows the resected area. This procedure was performed in 181 child patients. About 23 years follow-up revealed complication rate of 2.8% (5/181), reoperation rate of 0.5% (1/181). Mean operation time is 180.6±40.6 min, and mean blood loss is 133.6±84.8 ml. Mean hospital stay is 11.47±2.2 days. Patient can go to school 2 weeks after operation. Participation in sporting activity is allowed after 1 month, and competitive sports are resumed after 3

Fig. 21. Metal-free procedure (Masaoka et al., in press)

Fig. 22. Metal-free procedure. The black area is resected.

postoperative data. All indices improve strikingly.

(Reprinted with permission from *Eur J Cardiothorac Surg* 2011, in press)

Evaluation of correction in this operation was done by the deformity indices; Haller's, steepness, excavation volume, and asymmetry index. Table 9 shows preoperative and

months.

Dacron vascular graft, which usually 10mm in width, was sutured to the 4th or 5th ribs. Robicsek and colleagues (Robicsek et al., 2009) used Marlex sheet as a hammock, as described earlier. Although Robicsek and colleagues performed this procedure in many patients, outcome results are not shown. Länsman and colleagues (Länsman et al., 2002) tried bioabsorbable polylactide plate as a strut, but this procedure was not performed continuously.

Masaoka and colleagues (Masaoka et al., 2011) developed a new procedure without metal bar, which reconstructs the bony thorax with support of the bridge constructed by autologous costal cartilages. Core of this procedure is as follows.

Dacron vascular graft, which usually 10mm in width, was sutured to the 4th or 5th ribs. Robicsek and colleagues (Robicsek et al., 2009) used Marlex sheet as a hammock, as described earlier. Although Robicsek and colleagues performed this procedure in many patients, outcome results are not shown. Länsman and colleagues (Länsman et al., 2002) tried bioabsorbable polylactide plate as a strut, but this procedure was not performed

Masaoka and colleagues (Masaoka et al., 2011) developed a new procedure without metal bar, which reconstructs the bony thorax with support of the bridge constructed by

1. Subperichondrial transection of abnormally deformed 3rd~7th costal cartilages at 1cm

2. Removal of about 1cm long deformed cartilages lateral to the cut points. The 8th cartilages are shortened by several centimeters, in order to prevent protrusion of costal

5. Isolation of the sternum from the mediastinal structures and pleura. At that time, attention should be paid to preservation of the cranial parts of the internal thoracic

7. Transverse osteotomy on the rear table of the sternum at the 2nd rib level, and gentle

8. The caudal part of the sternum is detached from the 5th to 7th intercostal muscle

9. The caudal part of the sternum is resected transversely in width corresponding to the

10. The 4th or 5th costal cartilage is attached to the contralateral one at midline, in somewhat tense condition. This bridge contributes as a strut supporting the sternum. Selection of the 4th or 5th costal cartilage depends on the level of the sternum having

11. Anteriorly to the bridge, both ends of the sternum are sutured by five 1-0 absorbable

12. a) In cases, in which the bridge is constructed with the 5th costal cartilage, the 3rd, 4th and 6th costal cartilages on vertebral side are trimmed up in suitable manner, and

13. b) In cases, in which the bridge is constructed with the 4th costal cartilages, the 5th costal cartilages on vertebral side sutured to the 6th costal cartilages on sternal side. Similarly, the 6th costal cartilages on vertebral side are sutured to the 7th costal

14. The 7th costal cartilages are resected 2-3cm and sutured to the sternum. Thus, the bridge of the 4th or 5th cartilages supports the caudal part of the sternum and the 6th

15. The separated bundles of the 2nd, 3rd, 4th intercostal muscles are sutured to the same

16. The 6th and 7th intercostal muscles and perichondria are sutured to the reconstructed

sutured to the same cartilages on sternal side by two 1-0 absorbable threads.

3. Transverse transection of the sternum at level of the lower border of the 4th rib.

4. Ligations and transections of the bilateral aa. et vv. thoracia interna.

5th costal cartilage, in order to shorten the overlong sternum.

contact with the bridge, i.e., caudal part of sternum.

autologous costal cartilages. Core of this procedure is as follows.

lateral to the sternum bilaterally.

6. Transection of 4th to 2nd intercostal muscles.

bundles just at the margin of the sternum.

fracture to sternal elevation.

cartilages on sternal side (Fig. 21.)

and 7th costal cartilages.

bundles again.

sternum.

continuously.

arch.

vessels.

threads.

Fig. 22. shows the resected area. This procedure was performed in 181 child patients. About 23 years follow-up revealed complication rate of 2.8% (5/181), reoperation rate of 0.5% (1/181). Mean operation time is 180.6±40.6 min, and mean blood loss is 133.6±84.8 ml. Mean hospital stay is 11.47±2.2 days. Patient can go to school 2 weeks after operation. Participation in sporting activity is allowed after 1 month, and competitive sports are resumed after 3 months.

Fig. 21. Metal-free procedure (Masaoka et al., in press) (Reprinted with permission from *Eur J Cardiothorac Surg* 2011, in press)

Fig. 22. Metal-free procedure. The black area is resected.

Evaluation of correction in this operation was done by the deformity indices; Haller's, steepness, excavation volume, and asymmetry index. Table 9 shows preoperative and postoperative data. All indices improve strikingly.

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 161

Brown AL. Pectus excavatum (funnel chest) anatomic basis; surgical treatment of the

Cahill JL, Lees GM, Robertson HT. A summary of preoperative and postoperative

Castile RG, Staats BA, Westbrook PR. Symptomatic pectus deformities of the chest. *Am Rev*

Creswick HA, Stacey MW, Kelly RE Jr, Gustin T, Nuss D, Harvey H, Goretsky MJ, Vasser E,

Eguchi T, Sasaki S, Hara F, Kondo S, Masaoka A. Natural course of thoracic deformity in

Fabricius J, Davidsen HG, Hansen AT. Cardiac function in funnel chest; twenty-six patients investigated by cardiac catheterization. *Dan Med Bull*. 1957 Dec;4(8):251-7. Fonkalsrud EW, Beanes S, Hebra A, Adamson W, Tagge E. Comparison of minimally

Fonkalsrud EW. Open repair of pectus excavatum with minimal cartilage resection. *Ann*

Fonkalsrud EW. 912 open pectus excavatum repairs: changing trends, lessons learned: one

Haecker FM, Berberich T, Mayr J, Gambazzi F. Near-fatal bleeding after transmyocardial

Haller JA Jr, Kramer SS, Lietman SA. Use of CT scans in selection of patients for pectus excavatum surgery: a preliminary report. *J Pediatr Surg*. 1987 Oct;22(10):904-6. Haller JA Jr, Scherer LR, Turner CS, Colombani PM. Evolving management of pectus

Haller JA Jr, Colombani PM, Humphries CT, Azizkhan RG, Loughlin GM. Chest wall

Haller JA Jr, Loughlin GM. Cardiorespiratory function is significantly improved following

Harrison MR, Curran PF, Jamshidi R, Christensen D, Bratton BJ, Fechter R, Hirose S.

Hayashi A, Maruyama Y. Vascularized rib strut technique for repair of pectus excavatum.

Hu TZ, Li Y, Liu WY, Wu XD, Feng JX. Surgical treatment of pectus excavatum: 30 years 398

patients of experiences. *J Pediatr Surg*. 2008 Jul;43(7):1270-4.

stage. *J Thorac Surg*. 1939;9:164-184.

*Respir Dis*. 1982 Sep;126(3):564-8.

Mar;37(3):413-7.

discussion 191-2.

*Surg*. 2004 Aug;240(2):231-5.

*Cardiovasc Surg*. 2009 Nov;138(5):1240-1.

May;209(5):578-82; discussion 582-3.

carinatum repair. *J Pediatr Surg*. 1984 Aug;19(4):430-3.

excavatum. *J Pediatr Surg*. 2006 Oct;41(10):1699-703.

pectus excavatum. *Shonika* 1993;34:61-65. (In Japanese)

surgeon's experience. *World J Surg.* 2009 Feb;33(2):180-90.

*Thorac Surg*. 1996 Jun;61(6):1618-24; discussion 1625.

*Cardiovasc Surg (Torino)*. 2000 Feb;41(1):125-30.

*Ann Thorac Surg*. 1992 Feb;53(2):346-8.

incipient stage in infancy; and correction of the deformity in the fully developed

cardiorespiratory performance in patients undergoing pectus excavatum and

Welch JC, Mitchell K, Proud VK. Family study of the inheritance of pectus

invasive and modified Ravitch pectus excavatum repair. *J Pediatr Surg*. 2002

ventricle lesion during removal of the pectus bar after the Nuss procedure. *J Thorac*

excavatum based on a single institutional experience of 664 patients. *Ann Surg*. 1989

constriction after too extensive and too early operations for pectus excavatum. *Ann*

corrective surgery for severe pectus excavatum. Proposed treatment guidelines. *J*

Magnetic Mini-Mover Procedure for pectus excavatum II: initial findings of a food and drug administration-sponsored trial. *J Pediatr Surg*. 2010 Jan;45(1):185-91;

Table 9. Comparison of preoperative and postoperative indices (Masaoka et al., in print) (Reprinted with permission from *Eur J Thorac Cardiovasc Surg* 2011, in press)

The cartilages grow together with the growth of bony thorax, and continue to support sternum. The reconstructed thoracic configurations have been kept satisfactorily during 111±51.7 months after operations in 133 patients, excluding early postoperative (<3 years) and drop-out patients.

This procedure is indicated only to children (<15 years), because of malleable character of their thorax.

Longer operation time, more bleeding and longer hospital stay are shortcomings of this procedure. However, abandon of metal bar could provide benefits that would overcome these drawbacks.

## **3. Conclusion**

Throughout the history of corrective surgery for pectus excavatum, numerous procedures have been proposed, in whom 2 procedures survived and have been performed world-wide with some modifications — Ravitch and Nuss procedure—. Almost all institutes, whether Ravitch or Nuss, use metal bar to maintain configuration of the thorax. The use of metal bar has many drawbacks, i.e., frequent complications, pain, necessity of removal of bar, growth inhibition, metal allergy and possibility of recurrence of depression after removal.

A new metal-free procedure was proposed, in order to eliminate these shortcomings. Correction of thoracic configuration by this procedure was evaluated as excellent. This procedure is recommended as a standard operation for PE of children.

## **4. Acknowledgement**

We appreciated the careful secretarial work by Ms Mitsuko Tenpaku in preparing this manuscript.

#### **5. References**

The cartilages grow together with the growth of bony thorax, and continue to support sternum. The reconstructed thoracic configurations have been kept satisfactorily during 111±51.7 months after operations in 133 patients, excluding early postoperative (<3 years)

This procedure is indicated only to children (<15 years), because of malleable character of

Longer operation time, more bleeding and longer hospital stay are shortcomings of this procedure. However, abandon of metal bar could provide benefits that would overcome

Throughout the history of corrective surgery for pectus excavatum, numerous procedures have been proposed, in whom 2 procedures survived and have been performed world-wide with some modifications — Ravitch and Nuss procedure—. Almost all institutes, whether Ravitch or Nuss, use metal bar to maintain configuration of the thorax. The use of metal bar has many drawbacks, i.e., frequent complications, pain, necessity of removal of bar, growth

A new metal-free procedure was proposed, in order to eliminate these shortcomings. Correction of thoracic configuration by this procedure was evaluated as excellent. This

We appreciated the careful secretarial work by Ms Mitsuko Tenpaku in preparing this

Aydemir B, Sokullu O, Hastaoglu O, Bilgen F, Celik M, Dogusoy I. Aorta-to-right

Bevegard S. Postural circulatory changes at rest and during exercise in patients with funnel

Bouchard S, Hong AR, Gilchrist BF, Kuenzler KA. Catastrophic cardiac injuries encountered

ventricular fistula due to pectus bar migration. *Thorac Cardiovasc Surg.* 2011

chest, with special reference to factors affecting the stroke volume. *Acta Med Scand*.

during the minimally invasive repair of pectus excavatum. *Semin Pediatr Surg*. 2009

inhibition, metal allergy and possibility of recurrence of depression after removal.

procedure is recommended as a standard operation for PE of children.

Haller's 5.547±1.835 4.090±1.410 0.0000 Steepness 0.208±0.048 0.064±0.056 0.0000 Excavation volume 0.098±0.031 0.038±0.036 0.0000 Asymmetry 1.105±0.171 1.053±0.126 0.0001 Table 9. Comparison of preoperative and postoperative indices (Masaoka et al., in print)

(Reprinted with permission from *Eur J Thorac Cardiovasc Surg* 2011, in press)

and drop-out patients.

their thorax.

these drawbacks.

**3. Conclusion**

**4. Acknowledgement**

Feb;59(1):51-2.

1962 Jun;171:695-713.

May;18(2):66-72.

manuscript.

**5. References**

Index Preoperative Postoperative *P*-value

Pectus Excavatum: A Historical Perspective and a New Metal-Free Procedure 163

Nasr A, Fecteau A, Wales PW. Comparison of the Nuss and the Ravitch procedure for pectus excavatum repair: a meta-analysis. *J Pediatr Surg*. 2010 May;45(5):880-6. Ninkovic M, Schwabegger A, Gardetto A, Moser-Rummer A, Rieger M, Ninkovic M, Rainer

Nuss D, Kelly RE Jr, Croitoru DP, Katz ME. A 10-year review of a minimally invasive

Nuss D. Recent experiences with minimally invasive pectus excavatum repair "Nuss

Nuss D. Minimally invasive surgical repair of pectus excavatum. *Semin Pediatr Surg*. 2008

Nuss D, Kelly RE Jr. Minimally invasive surgical correction of chest wall deformities in

Park HJ, Jeong JY, Jo WM, Shin JS, Lee IS, Kim KT, Choi YH. Minimally invasive repair of

Peterson RJ, Young WG Jr, Godwin JD, Sabiston DC Jr, Jones RH. Noninvasive assessment

Pilegaard HK, Licht PB. Early results following the Nuss operation for pectus excavatum--a

Pilegaard HK, Licht PB. Can absorbable stabilizers be used routinely in the Nuss procedure?

Ravitch MM. The Operative Treatment of Pectus Excavatum. *Ann Surg*. 1949 Apr;129(4):429-

Ravitch MM. Technical problems in the operative correction of pectus excavatum. *Ann Surg*.

Robicsek F. Surgical treatment of pectus excavatum. *Chest Surg Clin N Am*. 2000

Robicsek F, Watts LT, Fokin AA. Surgical repair of pectus excavatum and carinatum. *Semin*

Robicsek F, Hebra A. To Nuss or not to Nuss? Two opposing views. *Semin Thorac Cardiovasc*

Saleh RS, Finn JP, Fenchel M, Moghadam AN, Krishnam M, Abrazado M, Ton A, Habibi R,

Saxena AK, Castellani C, Höllwarth ME. Surgical aspects of thoracoscopy and efficacy of

Saxena AK, Willital GH. Valuable lessons from two decades of pectus repair with the Willital-Hegemann procedure. *J Thorac Cardiovasc Surg*. 2007 Oct;134(4):871-6.

Fonkalsrud EW, Cooper CB. Cardiovascular magnetic resonance in patients with pectus excavatum compared with normal controls. *J Cardiovasc Magn Reson*. 2010

right thoracoscopy in minimally invasive repair of pectus excavatum. *J Thorac*

pectus excavatum: a novel morphology-tailored, patient-specific approach. *J Thorac*

of exercise cardiac function before and after pectus excavatum repair. *J Thorac*

single-institution experience of 383 patients. *Interact Cardiovasc Thorac Surg*. 2008

procedure". *Jpn J Thorac Cardiovasc Surg*. 2005 Jul;53(7):338-44.

children (Nuss procedure). *Adv Pediatr.* 2008;55:395-410.

*Reconstr Surg*. 2003 Oct;112(5):1355-61.

*Cardiovasc Surg*. 2010 Feb;139(2):379-86.

*Cardiovasc Surg*. 1985 Aug;90(2):251-60.

*Eur J Cardiothorac Surg*. 2009 Apr;35(4):561-4.

*Thorac Cardiovasc Sur*g. 2009 Spring;21(1):64-75.

*Cardiovasc Surg*. 2007 May;133(5):1201-5.

52.

Aug;17(3):209-17.

Feb;7(1):54-7.

1965 Jul;162:29-33.

May;10(2):277-96.

Dec 13;12:73.

*Surg*. 2009 Spring;21(1):85-8.

44.

C. Free sternum turnover flap for correction of pectus excavatum deformity. *Plast*

technique for the correction of pectus excavatum. *J Pediatr Surg*. 1998 Apr;33(4):545-

Iida H, Sunazawa T, Ishida K, Doi A. Surgical repair of pectus excavatum not requiring exogenous implants in 113 patients. *Eur J Cardiothorac Surg*. 2010 Feb;37(2):316-21. Jemielity M, Pawlak K, Piwkowski C, Dyszkiewicz W. Life-threatening aortic hemorrhage

Kelly RE Jr. Pectus excavatum: historical background, clinical picture, preoperative evaluation and criteria for operation. *Semin Pediatr Surg*. 2008 Aug;17(3):181-93. Kelly RE Jr, Goretsky MJ, Obermeyer R, Kuhn MA, Redlinger R, Haney TS, Moskowitz A,

Kotzot D, Schwabegger AH. Etiology of chest wall deformities--a genetic review for the

Laituri CA, Garey CL, St Peter SD. Review of the technical variants in the repair of pectus

Lane-Smith DM, Gillis DA, Roy PD. Repair of pectus excavatum using a Dacron vascular

Länsman S, Serlo W, Linna O, Pohjonen T, Törmälä P, Waris T, Ashammakhi N. Treatment

Lawson ML, Mellins RB, Tabangin M, Kelly RE Jr, Croitoru DP, Goretsky MJ, Nuss D.

Lawson ML, Mellins RB, Paulson JF, Shamberger RC, Oldham K, Azizkhan RG, Hebra AV,

Malek MH, Fonkalsrud EW, Cooper CB. Ventilatory and cardiovascular responses to exercise in patients with pectus excavatum. *Chest*. 2003 Sep;124(3):870-82. Masaoka A, Kondo S, Sasaki S, Hara F, Mizuno T, Yamakawa Y, Kobayashi T, Fujii Y. Thirty

Masson JK, Payne WS, Gonzalez JB. Pectus excavatum: use of preformed prosthesis for correction in the adult. Case report. *Plast Reconstr Surg*. 1970 Oct;46(4):399-402. Michlits W, Windhofer C, Papp C. Pectus excavatum and free fasciocutaneous infragluteal

Nakahara K, Ohno K, Miyoshi S, Maeda H, Monden Y, Kawashima Y. An evaluation of

of pectus excavatum with bioabsorbable polylactide plates: preliminary results. *J*

Impact of pectus excavatum on pulmonary function before and after repair with

Nuss D, Goretsky MJ, Sharp RJ, Holcomb GW 3rd, Shim WK, Megison SM, Moss RL, Fecteau AH, Colombani PM, Moskowitz AB, Hill J, Kelly RE Jr. Increasing severity of pectus excavatum is associated with reduced pulmonary function. *J*

years experience of open repair surgery for pectus excavatum −Development of a

flap: a new technique for the correction of congenital asymptomatic chest wall

operative outcome in patients with funnel chest diagnosed by means of the

costal cartilage cause pectus excavatum? A study on the lengths of ribs and costal

Nuss D. Twenty-one years of experience with minimally invasive repair of pectus excavatum by the Nuss procedure in 1215 patients. *Ann Surg*. 2010 Dec;252(6):1072-

during pectus bar removal. *Ann Thorac Surg*. 2011 Feb;91(2):593-5.

treating physician. *J Pediatr Surg*. 2009 Oct;44(10):2004-11.

the Nuss procedure. *J Pediatr Surg*. 2005 Jan;40(1):174-80.

metal-free procedure−. *Eur J Cardiovasc Surg* 2011 (in press)

deformities in adults. *Plast Reconstr Surg*. 2009 Nov;124(5):1520-8.

computed tomogram. *J Thorac Cardiovasc Surg*. 1987 Apr;93(4):577-82. Nakaoka T, Uemura S, Yano T, Nakagawa Y, Tanimoto T, Suehiro S. Does overgrowth of

cartilages in asymmetric patients. *J Pediatr Surg.* 2009 Jul;44(7):1333-6. Nakaoka T, Uemura S, Yoshida T, Tanimoto T, Miyake H. Overgrowth of costal cartilage is not the etiology of pectus excavatum. *J Pediatr Surg*. 2010 Oct;45(10):2015-8.

excavatum. *Eur J Pediatr Surg*. 2010 Jul;20(4):217-21.

graft strut. *J Pediatr Surg*. 1994 Sep;29(9):1179-82.

*Pediatr Surg*. 2002 Sep;37(9):1281-6.

*Pediatr*. 2011 Aug; 159(2):256-61.e2.

81.

**10**

*Germany*

 **Surgical Management of**

Geesche Somuncuoğlu

**Primary Upper Limb Hyperhidrosis – A Review**

 *Department of Thoracic Surgery, Schillerhoehe Hospital, Gerlingen,*

Sweating is a physiological and vital condition to control thermoregulation of the skin (Rajesh et al., 2003). Hyperhidrosis is defined as an excess of sweating beyond the amount

Primary hyperhidrosis as a disease seems trivial to general public because of its falsely perceived rarity (Eisenach et al., 2005). Furthermore, although not life-threatening (Reisfeld et al., 2002), it is evident that it can lead to severe psychologic, social and occupational dysfunction (Shargall et al., 2008). Nowadays primary hyperhidrosis is being recognized increasingly and its treatment options are gaining widespread attention (Eisenach et al., 2005). Although medical therapies have been the main treatment options for many years, surgical interventions have recently been proven to be an important therapeutic alternative. This shift has corresponded with the evolution of minimally invasive surgical techniques

Hyperhidrosis can be classified either by pathogenesis in a primary (idiopathic) and a secondary (symptomatic) form or by localisation and extension in a localised and a

needed to cool down elevated body temperature (Kreyden & Burg, 2000).

(Grondin, 2008), the main topic of this issue.

generalised form (Fig. 1).

**2. Classification and causes of hyperhidrosis**

Fig. 1. Classification of hyperhidrosis by localisation and extension

**1. Introduction**

## **Surgical Management of Primary Upper Limb Hyperhidrosis – A Review**

 Geesche Somuncuoğlu  *Department of Thoracic Surgery, Schillerhoehe Hospital, Gerlingen, Germany*

## **1. Introduction**

164 Topics in Thoracic Surgery

Schier F, Bahr M, Klobe E. The vacuum chest wall lifter: an innovative, nonsurgical addition to the management of pectus excavatum. *J Pediatr Surg.* 2005 Mar;40(3):496-500. Shamberger RC, Welch KJ, Sanders SP. Mitral valve prolapse associated with pectus

Shamberger RC, Welch KJ. Cardiopulmonary function in pectus excavatum. *Surg Gynecol*

Snel BJ, Spronk CA, Werker PM, van der Lei B. Pectus excavatum reconstruction with

Sweet RH. Pectus excavatum: Report of two cases successfully operated upon. *Ann Surg*.

Wada J, Ikeda T, Iwa T, Ikeda K. Sternoturnover: An advanced new surgical method to

correct funnel chest deformity. *J Int Coll Surg*. 1965 Jul;44:69-76.

silicone implants: long-term results and a review of the English-language literature.

excavatum. *J Pediatr*. 1987 Sep;111(3):404-7.

*Obstet*. 1988 Apr;166(4):383-91.

1944 Jun;119(6):922-34.

*Ann Plast Surg*. 2009 Feb;62(2):205-9.

Sweating is a physiological and vital condition to control thermoregulation of the skin (Rajesh et al., 2003). Hyperhidrosis is defined as an excess of sweating beyond the amount needed to cool down elevated body temperature (Kreyden & Burg, 2000).

Primary hyperhidrosis as a disease seems trivial to general public because of its falsely perceived rarity (Eisenach et al., 2005). Furthermore, although not life-threatening (Reisfeld et al., 2002), it is evident that it can lead to severe psychologic, social and occupational dysfunction (Shargall et al., 2008). Nowadays primary hyperhidrosis is being recognized increasingly and its treatment options are gaining widespread attention (Eisenach et al., 2005). Although medical therapies have been the main treatment options for many years, surgical interventions have recently been proven to be an important therapeutic alternative. This shift has corresponded with the evolution of minimally invasive surgical techniques (Grondin, 2008), the main topic of this issue.

## **2. Classification and causes of hyperhidrosis**

Hyperhidrosis can be classified either by pathogenesis in a primary (idiopathic) and a secondary (symptomatic) form or by localisation and extension in a localised and a generalised form (Fig. 1).

Fig. 1. Classification of hyperhidrosis by localisation and extension

Surgical Management of Primary Upper Limb Hyperhidrosis – A Review 167

A substantial part of the diagnosis of hyperhidrosis can be achieved by obtaining a patients history, followed by a physical examination. Once secondary causes of hyperhidrosis have been ruled out by additional laboratory tests, dermatologists have several techniques to stratify the severity of sweating like gravimetric testing, the Minor starch-iodine test and the ninhydrin test. Quality-of-life assessments may support the categorization of primary hyperhidrosis as a serious medical condition (Eisenach et al.,

Primary hyperhidrosis is treated symptomatically. There are lots of therapeutic options classified in nonsurgical and surgical treatment. Generally, therapy should be particular to each individual patient and chosen based on disease location, disease severity and expectations for improvement (Gee & Yamauchi, 2008). Dermatologists suggest following a graduated scheme like the guidelines elaborated by the German Society of Dermatology

The initial treatment for primary hyperhidrosis should always be nonsurgical. It includes topical treatments such as aluminium chloride, iontophoresis, oral medications such as anticholinergics and botulinum toxine (Reisfeld & Berliner, 2008). Unfortunately, a lot of cases do not respond sufficiently to these treatment regimes and effects are usually transient (T.S. Lin, 2001). Nonsurgical therapy options are mostly practised by dermatologists, they

Example Indication Mechanism/Specifics

Generalised adjuvant

Axillary

plantar

palmar plantar

Generalised

duct

Psychovegetative damping avoids activation of the reduced threshold of the eccrine glands

Anticholinergic, chemical psychovegetative damping Caution: Side effects

of the eccrine duct

cholinergic synapse

months

Blocking the lumen of the eccrine

Exact mechanism unknown: Ionic current causes a temporary block

Caution: Effect decreases within 6

Chemical block: Inhibits the release of acetylcholine at the

**3. Diagnosis and patient evaluation**

**4. Treatment of primary hyperhidrosis**

Autogenic training, hypnosis,

Antiperspirants (aluminium chloride hexahydrate)

Anticholinergics, psychotropics,

Iontophoresis Palmar

Local intradermal injection Axillary

psychotherapy

betablockers

Table 2. Nonsurgical therapy options

2005; Solish et al., 2008).

(Wörle et al, 2007).

**4.1 Nonsurgical treatment**

are listed below (Table 2).

Therapy option

Psychovegetative influence

Medications: - Topical

Physical therapy

Botulinum toxin (BTX)

Secondary hyperhidrosis appears to be more generalised and is triggered by an underlying disease process like infectious, endocrine or neurologic disorders (Eisenach et al., 2005; Kreyden & Burg, 2000; Shargall et al., 2008) (Table 1). Therapy of choice is treating the basic disease.

Table 1. Causes of secondary hyperhidrosis

The cause of primary hyperhidrosis, mostly affecting local parts of the body, is still unknown (Duarte & Kux, 2008). There seems to be a genetic predisposition in autosomal dominant fashion with variable penetrance, in 25-50% of cases a positive family history can be detected (Eisenach et al., 2005). The disease tends to begin in early childhood and becomes worse at puberty. It affects males and females equally (Shargall et al, 2008). The exact pathophysiology also remains unknown. There appears to be an overactive response of the eccrine glands to both heat and emotional stimuli, mediated through the sympathetic nervous system (Lee et al., 1999; Shargall et al., 2008) (Fig. 2). Therefore mostly affected areas are where eccrine glands are concentrated like the palms, the axillae, the face and the soles. Nearly half of diseased people suffer from an axillary manifestation (Schlereth et al., 2009). Overall the estimated prevalence of primary hyperhidrosis might be as high as 0,6-1% of the Western population, 2,8% of the US population and even 3% of the Asian population. Asia is also considered as an endemic area (Eisenach et al., 2005).

Fig. 2. Innervation of the eccrine glands by the sympathetic nervous system

## **3. Diagnosis and patient evaluation**

166 Topics in Thoracic Surgery

Secondary hyperhidrosis appears to be more generalised and is triggered by an underlying disease process like infectious, endocrine or neurologic disorders (Eisenach et al., 2005; Kreyden & Burg, 2000; Shargall et al., 2008) (Table 1). Therapy of choice is treating the basic

Hyperthyroidism, diabetes, menopause, obesity

The cause of primary hyperhidrosis, mostly affecting local parts of the body, is still unknown (Duarte & Kux, 2008). There seems to be a genetic predisposition in autosomal dominant fashion with variable penetrance, in 25-50% of cases a positive family history can be detected (Eisenach et al., 2005). The disease tends to begin in early childhood and becomes worse at puberty. It affects males and females equally (Shargall et al, 2008). The exact pathophysiology also remains unknown. There appears to be an overactive response of the eccrine glands to both heat and emotional stimuli, mediated through the sympathetic nervous system (Lee et al., 1999; Shargall et al., 2008) (Fig. 2). Therefore mostly affected areas are where eccrine glands are concentrated like the palms, the axillae, the face and the soles. Nearly half of diseased people suffer from an axillary manifestation (Schlereth et al., 2009). Overall the estimated prevalence of primary hyperhidrosis might be as high as 0,6-1% of the Western population, 2,8% of the US population and even 3% of the Asian population.

Spinal cord injury, parkinson´s disease Corticosteroids, antibiotics, antidepressants

Influenza, tuberculosis,

Leukemia, lymphoma

Asia is also considered as an endemic area (Eisenach et al., 2005).

Fig. 2. Innervation of the eccrine glands by the sympathetic nervous system

Panic disorder, stress, pain

disease.

Infectious Endocrine Malignancy Neurologic Drugs Psychogenic

Category Disorders

Table 1. Causes of secondary hyperhidrosis

A substantial part of the diagnosis of hyperhidrosis can be achieved by obtaining a patients history, followed by a physical examination. Once secondary causes of hyperhidrosis have been ruled out by additional laboratory tests, dermatologists have several techniques to stratify the severity of sweating like gravimetric testing, the Minor starch-iodine test and the ninhydrin test. Quality-of-life assessments may support the categorization of primary hyperhidrosis as a serious medical condition (Eisenach et al., 2005; Solish et al., 2008).

## **4. Treatment of primary hyperhidrosis**

Primary hyperhidrosis is treated symptomatically. There are lots of therapeutic options classified in nonsurgical and surgical treatment. Generally, therapy should be particular to each individual patient and chosen based on disease location, disease severity and expectations for improvement (Gee & Yamauchi, 2008). Dermatologists suggest following a graduated scheme like the guidelines elaborated by the German Society of Dermatology (Wörle et al, 2007).

### **4.1 Nonsurgical treatment**

The initial treatment for primary hyperhidrosis should always be nonsurgical. It includes topical treatments such as aluminium chloride, iontophoresis, oral medications such as anticholinergics and botulinum toxine (Reisfeld & Berliner, 2008). Unfortunately, a lot of cases do not respond sufficiently to these treatment regimes and effects are usually transient (T.S. Lin, 2001). Nonsurgical therapy options are mostly practised by dermatologists, they are listed below (Table 2).

Table 2. Nonsurgical therapy options

Surgical Management of Primary Upper Limb Hyperhidrosis – A Review 169

The first open surgical approaches occurred nearly a century ago (Baumgartner, 2008). These aggressive approaches were associated with significant patient morbidity and a protracted recovery period (Dewey et al., 2006). They often required a moderate to large sized incision in the chest which demanded cutting muscles and separating ribs to expose the sympathetic chain (Naunheim, 2000). Over the past decade, endoscopic sympathectomy, requiring three or two small thoracal incisions, replaced open procedures (Fig. 4). Today magnification and high resolution, attained with videoassisted thoracoscopic surgery, allows a detailed representation of anatomical structures which reduces risk of complications (Zacherl et al., 1999) (Fig. 5). Meanwhile further advances, utilizing microinstrumentation called needlescopic surgery or using a uniportal access, enable procedures done on an outpatient basis with minimal risk of surgical trauma and excellent

(a) (b)

First open cervical sympathectomies for epilepsy by Alexander and Ionnesco

Further thoracoscopic sympathectomies for different pathologies by Kux and

First videoassisted thoracoscopic surgery for posttraumatic pain syndrome by

Further videoassisted thoracoscopic surgery for hyperhidrosis by Claes and Drott presented at the First International Symposium on Thoracoscopic

A short overview of the historical development of sympathetic surgery and its application

First open thoracal sympathectomies for hyperhidrosis by Kotzareff First thoracoscopic sympathectomies for different pathologies by Hughes Further thoracoscopic sympathectomies for different pathologies by Goetz and

Table 4. Milestones in the history of sympathetic surgery and the therapy of hyperhidrosis

Fig. 4. (a) Example for a biportal access. (b) View via videothoracoscope (R=rib)

as to primary hyperhidrosis is listed in the table below (Table 4).

Sympathectomy, Boras, Sweden

Year History

Marr

Wittmoser

Chandler

1954

1992

1993

**<IP_ADDRESS> Surgical approaches and techniques**

cosmetic results (Dewey et al., 2006; Lee et al., 2000).

## **4.2 Surgical treatment**

Surgery should be reserved to severe hyperhidrosis and should only be contemplated when less invasive nonsurgical options have failed to provide adequate treatment (Naunheim, 2000). It includes local surgical axillary procedures such as excision, curettage or liposuction of the sweat glands and thoracoscopic sympathectomy (Baumgartner, 2008) (Table 3).

Table 3. Surgical therapy options

## **4.2.1 Thoracoscopic sympathectomy**

The rationale for sympathectomy in the management of primary hyperhidrosis is based on interrupting the transmission of impulses from the sympathetic nervous system to the eccrine sweat glands (Reisfeld et al., 2002). Object of surgery is the sympathetic trunk, a series of ganglia which are located in a line lateral and parallel to the vertebral bodies of the spinal column. The thoracic portion of the sympathetic trunk contains 12 ganglia, where the input is switched over to the effector (Naunheim, 2000). Sweat glands are innervated segmentally, that means a certain ganglion level can be ascribed to a certain localisation of hyperhidrosis (C.C. Lin & Telaranta, 2001).

Fig. 3. Anatomy of the sympathetic trunk and applied surgical procedure

#### **<IP_ADDRESS> Surgical approaches and techniques**

168 Topics in Thoracic Surgery

Surgery should be reserved to severe hyperhidrosis and should only be contemplated when less invasive nonsurgical options have failed to provide adequate treatment (Naunheim, 2000). It includes local surgical axillary procedures such as excision, curettage or liposuction of the sweat glands and thoracoscopic sympathectomy (Baumgartner, 2008) (Table 3).

Axillary

Axillary

therapy-refractory

therapy-refractory

Caution: Scarring, contractures

Caution: Hematoma,

infection

Therapy option Procedure Indication Specifics

Sympathetic block Thoracoscopic sympathectomy: Detailed description follows below

Fig. 3. Anatomy of the sympathetic trunk and applied surgical procedure

The rationale for sympathectomy in the management of primary hyperhidrosis is based on interrupting the transmission of impulses from the sympathetic nervous system to the eccrine sweat glands (Reisfeld et al., 2002). Object of surgery is the sympathetic trunk, a series of ganglia which are located in a line lateral and parallel to the vertebral bodies of the spinal column. The thoracic portion of the sympathetic trunk contains 12 ganglia, where the input is switched over to the effector (Naunheim, 2000). Sweat glands are innervated segmentally, that means a certain ganglion level can be ascribed to a certain localisation of hyperhidrosis (C.C. Lin & Telaranta, 2001).

Subcutaneous Excision of sweat glands (limited resection via tiny incisions)

Table 3. Surgical therapy options

**4.2.1 Thoracoscopic sympathectomy**

The first open surgical approaches occurred nearly a century ago (Baumgartner, 2008). These aggressive approaches were associated with significant patient morbidity and a protracted recovery period (Dewey et al., 2006). They often required a moderate to large sized incision in the chest which demanded cutting muscles and separating ribs to expose the sympathetic chain (Naunheim, 2000). Over the past decade, endoscopic sympathectomy, requiring three or two small thoracal incisions, replaced open procedures (Fig. 4). Today magnification and high resolution, attained with videoassisted thoracoscopic surgery, allows a detailed representation of anatomical structures which reduces risk of complications (Zacherl et al., 1999) (Fig. 5). Meanwhile further advances, utilizing microinstrumentation called needlescopic surgery or using a uniportal access, enable procedures done on an outpatient basis with minimal risk of surgical trauma and excellent cosmetic results (Dewey et al., 2006; Lee et al., 2000).

Fig. 4. (a) Example for a biportal access. (b) View via videothoracoscope (R=rib)

Table 4. Milestones in the history of sympathetic surgery and the therapy of hyperhidrosis

Surgical Management of Primary Upper Limb Hyperhidrosis – A Review 171

At which level sympathectomy should be performed and what is the correct extent of the procedure? So far, there is no consensus among surgeons, but there seems to be a correlation to postoperative effect and occurrence of side effects. During a long period of time extensive resections from level T2 to T5 were commonly performed (Dewey et al., 2006; T.S. Lin & Fang, 1999; Schmidt et al., 2006). But investigators demonstrated: reducing the extent of sympathectomy leads to a lower incidence of CS (Dumont, 2008). Today surgeons limit the extent of sympathectomy, there is a trend to two-level to the point of single-level surgery. The elected level of intervention is conditioned on the segmentally innervated localisation of hyperhidrosis and is shown in Table 5, based on a review of Krasna in 2008 (Krasna, 2008)

Table 5. Localisation of primary hyperhidrosis and related level of intervention

T1 sympathectomy for facial hyperhidrosis is abandoned long time ago due to high risk of Horner´s syndrome. For some time level of choice is T2 ganglia. Although T2 is also seen as centre point of neurologic impulse transmission through the brachial plexus to the hand (Hsia et al. 1999), some investigators suggest preparing lower levels like T3 (Dewey et al. 2006; Kwong et al., 2008; X. Li et al., 2008), because T2 is close to stellate ganglion with a risk of Horner´s Syndrome non-negligible and the fact that higher levels can increase rate of CS (Schmidt et al., 2006). T4 still remains level of choice for axillary hyerphidrosis (Lee et al. 1999; Licht et al. 2005). Isolated plantar hyperhidrosis occurs rarely but rather in combination with palmar or axillary type. As combined manifestation it is often treated by sympathectomy for upper limb hyperhidrosis and leads in 85% of cases to an improvement of plantar symptoms, shown by Reisfeld and colleagues (Reisfeld et al., 2002). Causes of improvement are unknown. Treating plantar hyperhidrosis by preparing lower thoracic levels is no longer practised (Kwong et al., 2008). Today isolated plantar hyperhidrosis is treated at level L2 and L3 by endoscopic lumbar sympathectomy (Loureiro et al., 2008).

Complications in hyperhidrosis surgery are rare and exceptional. Some can be avoided by experience or by technical improvement of the surgeon, others are unforeseeable. However any complications are less acceptable than for other sorts of thoracic operations because sympathectomy is a functional surgery for young patients in good health (Dumont, 2008). In 2004 Ojimba and Cameron did a Medline search using the term thoracoscopic sympathectomy and analysed all publications for reported complications (Ojimba & Cameron, 2004) (Table 6): No death has ever been reported in any published series but there are anecdotal reports of nine deaths following thoracoscopic sympathectomy. Five patients died from excessive haemorrhage, three due to incidents of narcosis and one death remained unexplained. Nevertheless, mortality associated with thoracoscopic sympathectomy is a rare condition compared to the high number of surgical procedures. The most common complication is pneumothorax. Up to 75% of patients have some residual

L2, L3 (today via endoscopic lumbar extraperitoneal sympathectomy)

(Table 5).

Facial T2 Palmar T2, T3 Axillary T3, T4

Localisation Level of intervention

Plantar >T4 (obsolete)

**<IP_ADDRESS> Complications and side effects**

Today much controversy and unanswered questions remain concerning the ideal thoracoscopic sympathetic operation (Baumgartner, 2008). What is the best technique of intervention: Should the sympathetic chain or ganglion be resected (sympathectomy), transected (sympathicotomy) or should only the rami communicantes be divided (selective sympathicotomy or ramicotomy)? Sympathectomy represents an aggressive approach, inducing a high rate of compensatory sweating (CS) (Lee et al., 1999). This unrequested side effect, specified below, could be considerably reduced by the nowadays commonly used sympathicotomy (Fig. 6). Another decrease of CS could be ascribed to ramicotomy, a limited technique first described by Wittmoser (Wittmoser, 1992) (Fig. 7). Due to the high rate of recurrence of preoperative symptoms, this approach is actually no longer used (Gossot et al., 2003).

Fig. 5. (a) Sympathicotomy. (b) Ramicotomy

Which instrument should be utilised to dissect the sympathetic trunk: Harmonic scalpel, ultrasonic, laser or thermocoagulation? All of them yield similar results (Inan et al., 2008), but due to electrocautery, care should be exercised to avoid heat damage to the adjoining structures (Krasna, 2008). Another recently upcoming procedure is the clamping method, published by Lin and colleagues in 1998 (C.C. Lin et al., 1998) with the potential advantage of reversibility in those patients unhappy with the outcome (Reisfeld et al., 2002) (Fig. 6). But so far, there have been only a mere handful of such reversals done with the surgical clamping technique and these reported reversals met with varied success (Kwong et al., 2008). Neural cell death is supposed to be responsible, when the clips are not removed relatively soon postoperatively (Dewey et al., 2006).

Fig. 6. Clamping method with two clips above and below the third rib

Today much controversy and unanswered questions remain concerning the ideal thoracoscopic sympathetic operation (Baumgartner, 2008). What is the best technique of intervention: Should the sympathetic chain or ganglion be resected (sympathectomy), transected (sympathicotomy) or should only the rami communicantes be divided (selective sympathicotomy or ramicotomy)? Sympathectomy represents an aggressive approach, inducing a high rate of compensatory sweating (CS) (Lee et al., 1999). This unrequested side effect, specified below, could be considerably reduced by the nowadays commonly used sympathicotomy (Fig. 6). Another decrease of CS could be ascribed to ramicotomy, a limited technique first described by Wittmoser (Wittmoser, 1992) (Fig. 7). Due to the high rate of recurrence of preoperative symptoms, this approach is actually no longer used (Gossot et al.,

(a) (b)

Which instrument should be utilised to dissect the sympathetic trunk: Harmonic scalpel, ultrasonic, laser or thermocoagulation? All of them yield similar results (Inan et al., 2008), but due to electrocautery, care should be exercised to avoid heat damage to the adjoining structures (Krasna, 2008). Another recently upcoming procedure is the clamping method, published by Lin and colleagues in 1998 (C.C. Lin et al., 1998) with the potential advantage of reversibility in those patients unhappy with the outcome (Reisfeld et al., 2002) (Fig. 6). But so far, there have been only a mere handful of such reversals done with the surgical clamping technique and these reported reversals met with varied success (Kwong et al., 2008). Neural cell death is supposed to be responsible, when the clips are not removed

2003).

Fig. 5. (a) Sympathicotomy. (b) Ramicotomy

relatively soon postoperatively (Dewey et al., 2006).

Fig. 6. Clamping method with two clips above and below the third rib

At which level sympathectomy should be performed and what is the correct extent of the procedure? So far, there is no consensus among surgeons, but there seems to be a correlation to postoperative effect and occurrence of side effects. During a long period of time extensive resections from level T2 to T5 were commonly performed (Dewey et al., 2006; T.S. Lin & Fang, 1999; Schmidt et al., 2006). But investigators demonstrated: reducing the extent of sympathectomy leads to a lower incidence of CS (Dumont, 2008). Today surgeons limit the extent of sympathectomy, there is a trend to two-level to the point of single-level surgery. The elected level of intervention is conditioned on the segmentally innervated localisation of hyperhidrosis and is shown in Table 5, based on a review of Krasna in 2008 (Krasna, 2008) (Table 5).

Table 5. Localisation of primary hyperhidrosis and related level of intervention