A man in his early 40s presented to our hospital with a complaint of bilateral visual impairment starting approximately 2 years prior. He had noticed this visual impairment because any glasses failed to correct his decimal visual acuity up to 1.0 at an eyeglass shop. The patient had no ocular or family histories. At the first examination in our clinic, his decimal best-corrected visual acuity (BCVA) was 0.7 (equivalent to 0.155 on logMAR visual acuity) with a spherical refractive error of −0.25 diopter (D) and cylinder –1.00 D Axis 170° and 0.6 (0.22 on logMAR) with a cylinder refractive error of–1.25 D Axis 5° in the right and left eyes, respectively. The ocular tension was within the normal range in both eyes. Slit-lamp examinations showed mildly senile cataracts bilaterally and no other abnormalities in the anterior and medial segments. Dilated fundus examinations showed no apparent abnormalities in either eye (). Optical coherence tomography (OCT) images showed sustained macular structure but diffuse thinning of the entire retinal layers in the macular area in both eyes (, Cirrus HD-OCT model 4000, Carl Zeiss, Germany). Fluorescein angiography (FA) revealed no apparent abnormalities (). A static visual field test using a Humphrey field analyzer (HFA II 750, Carl Zeiss, Germany) showed slight relative visual field defects in both eyes (). A kinetic visual field test using the Goldmann perimeter did not reveal any obvious abnormalities (). During several ophthalmic examinations, especially the indirect ophthalmic scope and FA, the patient experienced photophobia such that he was unable to keep his eyes open and complained of intolerable discomfort due to the bright light. Thus, we avoided examinations with bright light or reduced the luminance of the light source as much as possible. Reducing the luminance enabled us to perform these ophthalmic examinations. Some retinal diseases, including inherited retinal dystrophy, were suspected to be the cause of visual impairment and photophobia; however, a clear diagnosis was not possible as he was reluctant to undergo further examinations. There was no abnormality in the fundus or visual field; however, OCT demonstrated diffuse retinal thinning, which could be considered as the major cause for his visual impairment and photophobia. Follow-up ophthalmic examinations were performed every 6 months. His visual acuity and visual field were stable for over 10 years ().
Twelve years later, the patient reported multiple floaters in his left eye. A slit-lamp examination revealed multiple pigments in the vitreous of this eye. Dilated fundus examination showed partial rhegmatogenous retinal detachment that did not include the macula. The RD occurred in the superior retina with five atrophic holes and one retinal tear and in the inferior retina with lattice breaks. We first planned cataract surgery with phacoemulsification and intraocular lens implantation and vitreous surgery under local anesthesia with RBA. In the preparation for surgery using a surgical microscope (OPMI Lumera 700, Carl Zeiss, Germany), the patient reported severe photophobia with body motion. After RBA, the photophobia was reduced and cataract surgery was completed without any complications. However, after inserting the fiber optic light pipe into a cannula for vitrectomy with Constellation instruments (Alcon Laboratories, Fort Worth, TX, USA), with a light level set to 100% (approximately 3.2×105 cd/m2, at 40 cm by PR-655 Spectrascan®, Photo Research, Inc., CA, USA), the patient experienced intolerable photophobia with body motion (, ). Although we reduced the light dose to 60% (1.9 × 105 cd/m2) and administered pentazocine and hydroxyzine hydrochloride as analgesics, the intolerable photophobia persisted. After consultation with the patient, we interrupted the surgery and performed the second surgery under GA 7 days later. Retinal reattachment was achieved after vitrectomy, laser photocoagulation (LPC), and 20% SF6 gas tamponade.
Seventeen months after vitrectomy for his left eye, the patient reported multiple floaters in his right eye. His right BCVA was 0.8. In the right eye, slit lamp examination revealed multiple pigments in the vitreous. Dilated fundus examination showed partial RD in the superior retina with a giant retinal tear, which did not include the macula. Similar to his left eye, we performed cataract surgery, vitrectomy, LPC, and gas tamponade with 20% SF6 under GA, which resulted in retinal reattachment. However, the RD recurred in his right eye 16 days after the operation. For the next operation (the fourth operation in total), we proposed to him and obtained his agreement to use an HUS system with minimal illumination under RBA as the patient had felt nausea and complained of throat pain after GA. In case that the patient experienced the bright light from the light pipe to be intolerable, we requested anesthesiologists to be on standby during the operation. We performed vitrectomy, LPC, and gas tamponade with 12% C3F8 under RBA with the HUS system, which was consisted of an NGENUITY 3D® visualization system (Alcon Laboratories) attached to a VISU 210 microscope (Carl Zeiss Meditec, Jena, Germany). We set the brightness of the light pipe to 15% (4.8 x 104 cd/m2) and inserted the light pipe into a cannula, which provided excellent intraoperative visibility with minimal illumination (, ). The patient did not complain of photophobia during the operation, and retinal reattachment was achieved. Twenty-six days after the re-operation, the RD recurred. For the second RD recurrence, and the fifth operation in total, we performed vitrectomy, encircling, LPC, and gas tamponade with 12% C3F8 under RBA with the HUS system under the same conditions as the fourth operation. After approximately 60 min of encircling and 25 min of vitrectomy, we inadvertently set the brightness of the twin light chandelier to 100% (1.9 × 105 cd/m2) as the default brightness setting. Upon noticing the wrong setting, we immediately reduced the brightness to 30% (5.9 × 104 cd/m2) and asked the patient if he felt photophobia (, ). Interestingly, he did not experience photophobia. Retinal reattachment was achieved and was sustained until 18 months after surgery. His decimal BCVAs were 0.6 and 0.9 in the right and left eyes, respectively (0.22 and 0.046 on logMAR).


A 48-year-old man of African ethnicity came to our outpatient, complaining of disabling pain, even at rest, and functional impairment of his right lower extremity, associated with limping.
The patient reported high-energy trauma from a motor vehicle accident 5 years before, left hip dislocation with fracture of the anterior pillar of the pelvis and an open fracture-dislocation of the right knee (Gustilo 2).
The early treatment had been performed in another trauma center. It consisted of a total hip revision prosthesis and multiple interventions of his right knee, unfortunately not well reported by the patient.
Failure of the extensor apparatus, associated with a patella rise, was clinically remarkable.
The range of motion (ROM) was 45° with maximum passive and active flexion of 90° and passive and active extension lag of 45°.
There was a slight lateral instability.
X-rays performed showed nonunion of Hoffa fracture of the medial condyle and confirmed the rise of patella ().
Caton-Deschamps index was 2.24 ().
A CT scan with 3D reconstruction was performed for a more accurate pre-operative planning. ().
The operation was planned with four surgical steps:
Autologous bone graft harvesting from the iliac crest.
Reduction and fixation of Hoffa fracture of the medial femoral condyle after placement of bone graft.
Quadricipital Z-plasty and patella distalization.
Reconstruction of the patellar tendon with Achille’s tendon allograft (,).


A 12-year-old boy with his mother presented to our outpatient department with complaints of pain, and swelling at the right wrist since 1 year. Patient also had complaints of tingling numbness on and around palmar aspect of index and middle finger since 6 months. Patient had no history of trauma/fall on the affected wrist. Patient was apparently alright 1 year back, when he started complaining of pain and started noticing a firm swelling on the volar aspect of his right wrist. Pain was insidious in onset, dull aching in character, not associated with any other symptoms, aggravated by movements and reduced by rest. Swelling was initially the size of a small pea (1 cm × 1 cm), which later increased to the current size of 3 cm × 2 cm. Patient also companied of finding it difficult to hold a pencil/pen for long, as he would then start experiencing tingling numbness on and around palmar aspect of index and middle. Patient had no associated symptoms and family history was non-significant.
On examination of the right wrist joint, swelling (3 cm × 2 cm) was noted on the volar aspect of distal radius (). Skin over the swelling was normal and pinchable. Patient had localized tenderness over and around the swelling. Swelling was firm and bony in consistency. Swelling was non-translucent and non-mobile. Patient had a restricted range of flexion due to pain. Tinel’s sign was positive. Phalen’s test could not be elicited due to pain. Radial pulse was regular, normal in volume, rhythm, and consistency.
On investigating, his blood counts were within normal limits, erythrocyte sedimentation rate and C-reactive protein too were within normal limits. X-ray showed an exostosis of 3 cm × 2 cm on the volar aspect, arising from the metaphysio-diaphyseal area of the wrist (). Magnetic resonance imaging showed a well-defined, bony excrescence (3 cm × 2 cm × 2 cm) with a STIR hyperintense cartilaginous cap of maximum thickness of 4 mm involving the antero-medial aspect of radial diaphysis (). Growing away from the joint, indenting the posterior aspect of flexor digitorum superficialis and flexor carpi radialis. The lesion was also minimally displacing the neurovascular bundle on the radial side. The distance between the cartilaginous cap and the neurovascular bundle was around 1.5 mm. The findings were suggestive of osteochondroma. Electromyogram nerve conduction study revealed a normal study.
An excision biopsy was planned. The whole mass was excised leaving behind fresh healthy bone, which was sent for histopathology (). Carpal tunnel release and median nerve decompression were done. Post-surgery, the tumor was cleared off the bone (). Histopathology confirmed the diagnosis of osteochondroma. Post-operative management was as regular and the patient was discharged on post-operative day 5. Postoperatively wrist range of motion (ROM) was started on day 1 and continued throughout the stay. Patient’s symptoms of pain and tingling numbness disappeared after surgery. Patient was then followed up at 1 month with full wrist ROM, showing no recurrence.


A 61-year-old man with a history of type 2 diabetes and tobacco smoking who complained of dyspnoea and weight loss of 10 kg developing over the past 5 months was evaluated in the cardiology clinic. Physical examination was unremarkable; blood pressure was 135/90 mmHg, and heart rate was 72 b.p.m. The electrocardiogram showed low QRS voltage in the limb leads and a pseudo-infarct pattern with anteroseptal Q waves (). Echocardiography revealed moderate symmetric left ventricular hypertrophy with a speckled appearance of the myocardium and thickened mitral and tricuspid valves ( and Video 1). Left ventricular ejection fraction was within normal limits (57%) but GLS was markedly decreased (−9.4%) with an apical sparing pattern (). There was a restrictive left ventricular filling pattern (E/A ratio 4.20; E/e′ 24.5). The constellation of these findings strongly suggested cardiac amyloidosis. In addition, there was an echo dense sessile and irregular mobile mass (10 mm × 6 mm) consistent with PFE on the ventricular side of the right aortic coronary cusp ( and Video 2) without valvular obstruction or regurgitation. N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level was 2230 ng/L (normal <125 ng/L). Cardiac magnetic resonance imaging (MRI) confirmed left ventricular hypertrophy (left ventricular mass 196 g) and showed areas of extensive late gadolinium enhancement in both ventricles ( online, Figure
S1). Monoclonal protein assessment detected a monoclonal lambda light chain on serum protein immunofixation. Kappa serum free light chain (FLC) was 17.2 mg/L (normal range 3.3–19.4 mg/L) and lambda serum FLC was 249.6 mg/L (normal range 0.7–26 mg/L) with kappa/lambda ratio at 0.07 (normal range 0.26-1.65). There was no significant proteinuria on the 24-h urine collection. Subcutaneous fat biopsy was negative, but labial salivary gland biopsy was positive for Congo red stain ( online, Figure
S2) with characteristic apple-green birefringence in the amyloid deposits. Immunofluorescent histology demonstrated Lambda light chain restriction establishing the diagnosis of AL amyloidosis. We concluded to the diagnosis of lambda AL amyloidosis at Stage IV of the revised Mayo Clinic staging system. Therapy consisted of six 28 days cycles of daratumumab (weekly in cycle 1–2, every 2 weeks in cycles 3–6) and weekly bortezomib (1.3 mg/m2), cyclophosphamide (300 mg/m2), and dexamethasone (20 mg) followed by daratumumab every 4 weeks for a further six cycles. The treatment allowed a complete haematological response. Global longitudinal strain improved after 6 months () and was near normal (−17.3%, ) after 12 months. At that moment, despite the persistence of left ventricular hypertrophy with a restrictive pattern of mitral inflow by Doppler echocardiography (E/A and E/e′ ratio of 2.9 and 18.1, respectively), patient’s symptoms improved (New York Heart Association IIA) and NT-proBNP level dropped to 757 ng/L. Although there was no neurological deficit, the high incidence of neurological events reported with PFE led us to perform cerebral MRI, which showed bilateral lesions consistent with ischaemic sequelae (). No other cardiac or vascular source of emboli were detected, reinforcing the indication for surgical resection. Routine preoperative coronary angiography showed a significant stenosis of the middle portion of the left anterior descending artery (LAD). Surgery was performed and perioperative inspection revealed a sessile tumour on the ventricular side of right coronary cusp of the aortic valve (). Complete resection of the tumour with valve preservation was not feasible, leading to bioprosthetic valve replacement in addition to left internal mammary artery bypass graft on the LAD. Histopathology of the native valve confirmed the diagnosis of cardiac PFE (). In addition, staining with Congo red dye allowed identifying apple-green birefringent valvular deposits under polarized light, consistent with amyloid deposits. Postoperative follow-up, currently 4 months, is unremarkable.


A 48-year-old female presented 3 years after being thrown against a wall with a sequela of traumatic brain injury (TBI) with cerebral palsy, worsening myelopathy, and gait ataxia. She initially utilized a walker for ambulation, but progressed to wheelchair bound by the time of presentation [].
She underwent MRI studies of the brain and spine, plus EMG and NCV of the lower extremities. The thoracic MRI showed a ventral thecal sac irregularity with accompanying distortion of the ventral cord, consistent with “tethering;” cervical and lumbar studies were unremarkable []. The CT myelogram was suggestive of ventral dural tear and spinal cord herniation at the T7-8 levels and a diffuse ventral subdural fluid collection extending from T1 to T10 [].
We performed a T7-T8 laminectomy and unilateral facetectomy with removal of the right T7 pedicle and transverse process, exposing the T7-8 nerve roots []. After using ultrasounds to confirm the level, the right T7 nerve root was ligated. Using microsurgical technique, the dural defect was approached from the right side intra- and extradurally. Once the dural defect was identified, it was repaired with onlay and inlay DuraGen (Integra LifeSciences, Princeton, NJ, USA) and, after weighing the risk and benefits, we reinforced with DuraSeal (Integra LifeSciences, Princeton, NJ, USA) to minimize risk of postoperative CSF leak.
Postoperatively, the patient went to rehab and within 2 months, she was ambulating with assistance using a walker. Her strength continued to improve at her 4-month and 1-year neurosurgical follow-up visits []. She is now ambulating independently with a walker. On repeat, MRI at 4 months postoperatively demonstrates postsurgical changes with a well-circumscribed right paraspinal fluid collection with minimal mass effect on the spinal cord []. There was no clinical or radiographic evidence of recurrent herniation, and the patient did not experience clinical signs or symptoms of CSF leak.


The craniofacial dysmorphic features of a-22-month-old boy manifested the severe form of ISH included extensive and severe papulo-nodular skin lesions extending over the fronto-nasal areas, with massive swelling of the lower face and lips, gingival hypertrophy and the ears were massively involved [Figure and ].
Progressive contractures were noted in the same child with ISH at the age of 3. Attempts for passive movements of the joints causing painful reactions. Violaceous nodules over the sacral prominences of the same patient were associated with hypertrophy around the anus in the form of nodulous swellings (large tumors and nodules/polypoid lesions around the anus) [].


A-2-year-old girl presenting with severe manifestations of ISH, showed sparse hair, frontal haemangioma, a severely depressed nasal bridge, deep-seated eyes, hypertrophy and a congested nasal tip, long philtrum and minimal scattered papulo-nodular skin lesions around the nose were also noted [].
Dentinogenesis imperfecta overwhelmed by massive gingival fibromatosis [].


A mild type of ISH in a-14-month-old girl (cousin of case-2) showed mild Papulo-erythemaous rash over the nape of the neck [] and noticeable thickening and hyper-pigmentation of the skin over the elbows with mild joint contractures [].


A-4-year-old boy (older sibling of case-2), manifested multiple lipoma-like along the anterior abdominal wall [] and mild hyper-pigmentation over the sacral area associated with a small anal nodule [].


A-five-year-old-girl (cousin of case-2) presented with minimal skin lesions, normal intelligence, but with abnormal gait.
All children with severe and mild/moderate types underwent additional radiographic study (included, AP and lateral skull, AP spine, AP shoulder joint and AP hands).
Skull radiographs and three-dimensional (3D) reconstruction computed tomography (CT) scan have been organised. The two children with severe ISH showed abnormal craniofacial contour.
The Lateral skull radiograph of case-1, showed brachycephaly in connection with partial closure of coronals/lambdoid [].
The Lateral skull radiograph of case-2 showed scaphocephaly in correlation with sagittal suture synostosis [].
The 3D reconstruction CT of the skull of case-2 showed total synostosis of the sagittal suture
With mild plagiocephaly [].
AP spine and shoulder radiograph of case-1 showed severe osteolysis of the shoulder and hip joints were noted []. AP spine radiograph of case-4. Who manifested moderate type of ISH showed a less important malformation complex except for bilateral and symmetrical hypoplasia of the clavicles (arrows) [].
The skeletal phenotype of case-1 with severe type of ISH; AP radiographs of the shoulders, humero-radial and pelvis showed multiple dislocations secondary to massive osteolysis along the major joints of the shoulders, elbows and hips [Figure –].
AP pelvis radiograph of case-5 showed “shepherd's crook” deformity with severe form of coxa vara [].


A 65-year-old male patient, with a past history of sarcoidosis, presented with an AAA detected on a routine abdominal ultrasound. Several abdominal ultrasounds performed in the previous years had never detected any AAA. Along with sarcoidosis (histologically diagnosed on a cervical adenopathy biopsy), he had arterial hypertension and atrial fibrillation. The patient was compliant to telmisartan 40 mg q.d. and apixaban 5 mg b.i.d. No symptoms were apparent. The patient had an excellent functional status and lived an active lifestyle. No fever, chills, malaise, fatigue or loss of weight were to be reported. He reported no domestic or professional contact with animals.
A computed tomography angiography (CTA) confirmed the ultrasound finding, revealing a saccular infra-renal AAA with 51 mm of diameter ().
Blood cell counts and biochemistry surveys, including the erythrocyte sedimentation rate and C-reactive protein, were within normal values. Serologic studies were negative, except for Coxiella burnetii IgG antibodies at a 1:1,280 titer. A minimal burden of atherosclerosis throughout the thoracoabdominal aorta, along with the saccular morphology, prompted an investigation for aortitis etiologies. An 18fluorodeoxyglucose-positron emission tomography/computed tomography (18FDG-PET/CT) was performed but only para-aortic adenopathies had a significant uptake ().
Further indirect immunofluorescence (IIF) testing (performed at Instituto Nacional de Saude Doutor Ricardo Jorge) revealed high titers of both anti-phase II and anti-phase I IgG antibodies (1:3,200 and 1:1,600, respectively). Anti-phase II and anti-phase I IgM antibodies were slightly above the positivity cutoff. Polymerase chain reaction (PCR) detection of Coxiella burnetii was not performed. No cardiac valvular vegetations were detected in the transthoracic echocardiography.
The patient promptly started doxycycline 100 mg b.i.d. and hydroxychloroquine 200 mg t.i.d., and after 10 days he was submitted to an endovascular aortic repair (EVAR) with a bifurcated Excluder® endoprosthesis (W. L. Gore & Associates, Inc., Flagstaff, AZ, USA).
He had an uneventful recovery and was discharged at the third post-operative day.
The antibiotic therapy was maintained for 1 year with no side effects, and is intended to be administered for at least 18 months. During follow-up, a complete aneurysm thrombosis was noticed () and no signs of graft infection were present.


A 76-year-old male patient with Fitzpatrick skin type II consulted the outpatient clinic of the Department of Dermatology and Allergy, University Hospital of Munich (LMU), Germany, showing bilateral pigmented axillary and inguinal lesions accompanied by mild pruritus. He reported that the lesions were persistent for 4 months (shown in Fig. ). Neither he nor his family had previously suffered from any skin conditions or allergies. Sun exposure prior to the occurrence of the skin lesions was denied. The patient was in an overall good health and nutritional status with a prehistory of persistent atrial fibrillation and arterial hypertension. Three years ago, he underwent unilateral mastectomy due to breast cancer. Thereafter, he had regular oncologic follow-up visits. Chemotherapy, radiation, or hormone therapy had not been administered. His long-term medication included bisoprolol, digoxin, and rivaroxaban.
The clinical examination showed sharply defined brown to gray macules, papules, and plaques limited to the intertriginous areas. Mucous membranes were free of pathology (shown in Fig. ). Differential diagnoses included fixed drug eruption, contact dermatitis with postinflammatory lesions, acanthosis nigricans, or erythema dyschromicum perstans. Punch biopsies taken from the intertriginous lesions revealed band-like lichenoid inflammatory infiltrates with epidermal atrophy, hypergranulosis, and discreet orthohyperkeratosis. The dermis showed an accumulation of macrophages containing pigments (shown in Fig. ). The suspected clinical diagnosis of LPPI was confirmed. Predilection sites for lichen planus were not affected (shown in Fig. ). Routine laboratory tests, hepatitis serology, and a patch test performed showed no anomalies.
Topical tacrolimus 0.1% cream was prescribed as an off-label treatment once a day. Follow-up examinations were performed after 2 and 4 months. Over time, the intertriginous lesions became lighter and flatter, reduced in size, and symptoms of itching diminished (shown in Fig. ). No side effects were observed. We therefore suggest that topical tacrolimus 0.1% cream as a potential safe, effective, and simple therapeutic option for LPPI.


A healthy 25-year-old woman, with no history of contact lens wear, presented with several hours of pain, injection and tearing in the right eye. The patient reported accidental exposure to coral reef fluid (Zoanthids-Green Bay Packers, Fig. ) when cleaning her aquarium. She immediately irrigated the eye and continued using artificial tears every 3–4 h.
On examination, before instillation of topical anesthetic, testing of the pH with a Hydrion (Micro Essential Laboratory Inc., Brooklyn, NY, USA) pH strip in both upper and lower fornices revealed a normal pH of 7.0. The pH strip was applied into each fornix for 1 s, then instantly compared the resulting color with the matching pH color chart. Visual acuity was 20/20 in both eyes with normal intraocular pressures. She had upper greater-than-lower lid edema with minimal erythema and no induration. In addition, there was a significant inferior bulbar conjunctival epithelial defect with associated injection, subtle blanching of conjunctival vessels, chemosis, and patchy subconjunctival hemorrhage (Fig. ); there was no corneal epithelial defect (Fig. ). Conjunctival swabs were sent for bacterial culture and gram stain. She was started on topical moxifloxacin 0.5% and prednisolone acetate 1.0% 4× daily as well as preservative-free artificial tears every 2 h. The lid edema worsened over the next 24 h, but she remained afebrile with no changes on ocular exam indicating a low likelihood of a secondary bacterial cellulitis.
On follow up 3 days later, her symptoms had significantly improved; similarly, her exam revealed resolution of the lid edema as well as resolution of the conjunctival injection and chemosis. Gram stain and culture of the conjunctiva showed no neutrophils or organisms. At 2-week follow-up, her symptoms had resolved. Moxifloxacin was stopped and she was subsequently tapered off the prednisolone over a 4-week period by reducing 1 drop each week.


A 67-year-old man presented to the outpatient department with painful decreased vision in his left eye for the past 3 days. He had no history of trauma but did have a history of diabetes and hypertension, which were currently controlled with treatment. Ophthalmology examinations revealed a visual acuity of 20/20 and 2/60 in the right and left eyes, respectively. The IOP (as measured by the Topcon Medical Systems CT-80 noncontact computerized tonometer) and other structures in the right eye were normal with pseudophakic status, whereas we noted an extremely high IOP (>60 mm Hg) in the left eye. Anterior segment examination in the right eye was found pseudophakic with IOL in central position, other structures were normal. Gonioscopy on the right eye was open (trabecular meshwork seen in 4 quadrants). The left eye revealed ciliary injections, corneal edema, shallow AC (Van Herick grade 1), sluggish irregular pupil and thickened and forward displacement of the lens. The gonioscopic examination was unclear due to corneal edema. Anterior segment optical coherence tomography in the left eye exhibited a narrowed iridocorneal angle (shown in Fig. ).
Initial therapy consisted of a combination of β-blocker and corticosteroid topical medications as well as oral carbonic anhydrase inhibitor, which addressed the acute nature of the angle closure and successfully lowered the patient's IOP to 55 mm Hg with a visual acuity 20/60. However, the IOP rose again, and his visual acuity dropped to 1/60 3 days later. After ensuring normal renal function, intravenous mannitol 20% was given immediately, followed by laser peripheral iridotomy (shown in Fig. ). A filtering trabeculectomy with antimetabolites (5-fluorouracil for 5 min) was performed within 5 days, which successfully lowered the patient's IOP to 19 mm Hg, improved visual acuity to 20/60, and resulted in well-functioning bleb (shown in Fig. ) and minimal corneal edema, and deepened the AC. We scheduled lens extraction through phacoemulsification and implantation of intraocular lens (IOL); unfortunately, the AC was noted to be extremely shallow during the week after trabeculectomy. Anterior segment optical coherence tomography revealed lenticulo-irido-endothelial touch (shown in Fig. ) and thickened central corneal thickness (CCT) to 814 μm, and the patient's IOP was >60 mm Hg. This pathological condition was considered due to aqueous humor misdirection (malignant glaucoma) and led to an inability to accomplish phacoemulsification for cataract extraction, so a posterior approach should be considered as one technique for safe and reliable management.
The following day, a vitreoretinal surgeon performed posterior lensectomy using 3-port pars plana vitrectomy under general anesthesia (shown in Fig. ). The surgical technique involved making 3-port 23-gauge sclerotomies 4 mm from the cornea-scleral limbal, and core and complete vitrectomy resulted in a lower IOP, which allowed for the construction of a main port through a clear corneal incision, reformed the AC depth and released the lenticulo-irido-endothelial adhesion by an ophthalmic viscosurgical device. To avoid friction between the lens and corneal endothelium, we performed posterior lensectomy once the AC depth allowed for sufficient space. We paid careful attention while performing lensectomy manipulation to avoid further zonular dehiscence that was seen from 10 to 12 o'clock. Harder fragments of the nucleus were intentionally dropped into the vitreous cavity and then completely removed using a phacofragmatome. We observed obvious significant corneal clarity and deepened AC intraoperatively. The eye was left aphakic with adequate anterior capsule support for further secondary IOL implantation in the sulcus when the CCT reached the normal limit (shown in Fig. ).
The most recent ophthalmology examination after 8 months post-surgery revealed remarkable improvement in visual acuity of 20/50, with an IOP of 18 mm Hg, clear cornea, normal AC depth (Van Herick grade 4), and central IOL position. Based on the Indiana Bleb Appearance Grading Scale system, we observed a flat bleb and results of the OCT revealed fibrotic sclera. Subjectively, the patient also stated a significant postsurgical improvement in both visual acuity and relevant symptoms without glaucoma medication.


A 33-year-old man with a reported history of sickle cell anemia and a penicillin allergy
presented to the emergency department (ED) with an extensive ulcerative rash involving his
face, extremities, trunk, genitals, and back with sparing of the mucous membranes, eyes,
palms, and feet (). The
rash initially began 5 days prior to presenting at our hospital as painful, small
ulcerations localized to the left arm. The patient originally sought care at an outside ED
where he was diagnosed with severe dermatitis with suspected bacterial superinfection, but
the patient declined admission and was subsequently discharged with a prescription of
cephalexin. The rash progressively worsened and disseminated despite antibiotic treatment,
prompting the patient to present to our ED. He denied a history of eczema, asthma, allergic
rhinitis, recent exposure to a new environmental allergen, shortness of breath, headaches,
and visual disturbances. Cardiovascular, pulmonary, and neurologic examinations were
normal.
The patient’s social history is remarkable for a monogamous relationship with 1 male
partner. He endorsed consistent use of barrier contraception during intercourse. He denied a
history of sexually transmitted infections, prior blood transfusions, and intravenous (IV)
drug use.
Given the severity of the rash, recent antibiotic exposure, and history of serious
penicillin allergy, initial differential diagnoses were drug allergy and a disease on the
Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) spectrum. However, the latter
was ruled out by the burn team and dermatology (virtually) due to the absence of mucosal
involvement, negative nikolsky sign, and absence of skin sloughing.
Laboratory studies in the ED revealed a reactive HIV antigen/antibody screen with a
subsequently positive HIV-1 antibody and negative HIV-2 antibody. In addition, his labs were
significant for CD4 count of 259, white blood cell count (WBC) of 7.4 k/microL with 54%
neutrophils, 9% lymphocytes, 0% eosinophils, 0% basophils, and creatinine and sodium of 3.05
mg/dL and 119 mEq/L, respectively. These findings suggested an immune deficiency cause
rather than an allergic reaction, and dermatology subsequently recommended starting IV
acyclovir for possible disseminated HSV-1 infection. Infectious disease (ID) was consulted,
and he was admitted for further workup of the skin lesions.
Screening for hepatitis, syphilis, gonorrhea and chlamydia was negative. Further tests for
opportunistic infections, including coccidiomycosis antigen/antibody, cryptococcal antigen,
tuberculosis, varicella zoster virus (VZV), and cytomegalovirus (CMV) were also negative. In
addition, autoimmune disease screening with antinuclear antibodies (ANAs), antineutrophil
cytoplasmic antibodies (ANCAs), and serum complements were unremarkable. However, HSV
polymerase chain reaction (PCR) from the skin lesions was positive for HSV-1 and negative
for HSV-2. A positive repeat test further suggested a diagnosis of disseminated HSV-1, and
subsequent HSV antibody testing showed elevated IgM. A skin punch biopsy of the left upper
extremity revealed irregular psoriasiform hyperplasia with epidermal hypergranulosis,
hyperkeratosis, and superficial pigmentary incontinence. There was superficial perivascular
inflammation consisting of lymphocytes and histiocytes. No spongiosis and intraepidermal
vesicles were present. There was no evidence of viral morphologic changes.
The patient was continued on IV acyclovir and emollients with gradual improvement in the
lesion throughout the admission (). He was discharged with 2 weeks of oral valganciclovir followed by lifelong
daily acyclovir prophylaxis. He was scheduled to follow up with ID outpatient for initiation
of highly active antiretroviral therapy (HAART).


A 70-year-old man (170 cm, 48 kg, American Society of Anaesthesiologists status IV) had a history of HFrEF with dilated cardiomyopathy, diabetes mellitus, and chronic obstructive pulmonary disease. He was scheduled to undergo debridement of skin necrosis in the right leg. He had developed right leg weakness 4 months previously and was diagnosed with thrombotic occlusion of the right ilio-femoral artery. Skin necrosis appeared on the anterolateral side of the lower leg (). While being treated for the skin lesion, his HF was treated with spironolactone, ivabradine, sacubitril/valsartan, and warfarin (1.5 mg) to optimise cardiopulmonary function. He had also received an implanted defibrillator 3 months previously.
Despite inotropic administration, the patient’s systolic and diastolic blood pressure was 70 and 40 mmHg, respectively. His coagulation profile showed that the activated prothrombin time was 49.4 s and the prothrombin time/international normalised ratio was 1.63. However, anticoagulation therapy was continued because of the high risk of thromboembolism. The initial BNP level was 837 pg/mL, which decreased to 216 pg/mL just before surgery after treating the HF. Chest radiography showed pneumonic infiltration in the right lung (mainly in the right lower lobe) and diffuse emphysematous changes in both lungs (). Preoperative transthoracic echocardiography revealed global hypokinesia with severe left ventricular dysfunction, an ejection fraction of 11%, an enlarged left atrium and ventricular chamber, and grade II diastolic dysfunction.
Regional anaesthesia was considered safer than general anaesthesia because of the patient’s unstable cardiopulmonary function, but a neuraxial block was contraindicated owing to anticoagulation therapy. Therefore, we used a USG sciatic nerve and fascia iliaca compartment block to cover the operative field and avoid haemodynamic instability. However, the scheduled operation was postponed because the patient strongly requested only general anaesthesia and refused regional anaesthesia using a PNB. He had previously undergone wound dressing changes with local anaesthesia, and suffered from excruciating pain. Because of this previous experience, he was afraid that he might feel severe pain during the operation because of failure of the nerve block. Several counselling sessions were held to provide sufficient information to the patient, along with emotional support to overcome his fear and optimise the anaesthesia.
Standard monitoring, including non-invasive blood pressure monitoring, three-lead electrocardiography, and pulse oximetry, were applied before the nerve block, and the left brachial artery was cannulated for continuous blood pressure monitoring. Supplemental oxygen was administered through a facial mask at 5 L/minute. The patient was positioned laterally for the popliteal sciatic nerve block. A high-frequency linear transducer (HFL38x®; SonoSite, Bothell, WA, USA) was transversely placed on the posterior aspect of the popliteal fossa, and the sciatic nerve was located. Under real-time ultrasound (M-Turbo®; SonoSite) guidance, a 25 G needle was inserted 5 cm from the popliteal crease using the in-plane approach. Once the needle tip was near the distal sciatic nerve, 40 mL of 0.375% ropivacaine was injected around the sciatic nerve after confirming negative blood aspiration. Subsequently, the patient was placed in the supine position for the fascia iliaca compartment block. Using the same ultrasound transducer, the fascia lata and fascia iliaca were checked. A 20-mL aliquot of 0.25% ropivacaine was injected into the plane between the fascia iliaca and iliopsoas muscles after checking for negative aspiration (). A loss of sensation was confirmed in the surgical area after 30 minutes. The surgery commenced after confirming adequate sensory block. During the operation, the patient did not require any analgesics or rescue block, and haemodynamic parameters were stable. The patient was transferred to the intensive care unit after surgery for close monitoring because of his severely compromised cardiopulmonary function. Haemodynamic stability was confirmed and he was transferred to the general ward the next day. Thereafter, the patient underwent wound debridement twice more and finally received a skin autograft at the defect site. All of these surgical procedures were successfully performed using the same PNB techniques. After the skin graft, the patient was discharged from the hospital without any symptoms, such as dyspnoea or surgical site pain.
The reporting of this study conforms to the CARE guidelines.


A 27-year-old woman presented with pain and discomfort in her left mandible when brushing her teeth. She was systemically healthy, taking no medications, and was a non-smoker, with no financial issues or cultural beliefs that prevented her from receiving the reported treatments. She had received no previous periodontal treatment. Despite good plaque control, severe periodontitis was observed around the mandibular left first molar. There was positive bleeding on probing (BOP), a probing depth (PD) of 5 mm, clinical attachment level (CAL) of 7 mm, horizontal probing depth (HPD) of 5 mm, KMW of 0.5 mm, GR of 2 mm at the mid-buccal site, and KMW of 0 mm at the mesio-buccal site due to abnormalities in the buccal frenulum and enamel projection (). BOP using controlled insertion pressure is diagnostic of an inflammatory lesion. The clinical parameters used in this case were based on Becker et al. The BOP score was 5.2% and the patient was diagnosed with localized stage III grade B periodontitis. Radiographic examination revealed a radiolucent lesion in the furcation area at the mandibular left first molar (). There was no periodontitis in the anterior and pre-molar areas, and mild periodontitis was detected in relation to other molars.
At 6 weeks after non-surgical treatment, BOP and class II furcation involvement persisted at the mandibular left first molar with a PD of 4 mm, CAL of 7 mm, and HPD of 5 mm. This was because the patient had difficulty cleaning the buccal side of the mandibular left first molar owing to an insufficient KMW and VD and buccal frenulum abnormalities. The degree of furcation involvement was graded according to the classification system proposed by Hamp et al. The vertical furcation involvement was classified as grade A according to the criteria proposed by Tarnow and Fletcher.
An eCTG with a 2-mm band of epithelium with enamel matrix derivative (EMD) gel (Straumann Emdogain, Straumann, Basel, Switzerland) was planned using a bilaminar technique to improve the GR, KMW, KMT, and VD. After local anesthesia, a combination flap (full thickness in the coronal area and partial thickness in the apical area) with vertical incisions was elevated on the buccal side (). The granulation tissue and enamel projections were removed, and planing of the exposed root was performed (). An eCTG was harvested from the left palate at the first molar using a single-incision technique. The donor site was sutured using 4-0 silk (MANI Sutures Silk; Mani Inc., Tochigi, Japan) cross mattress sutures (). The root surfaces were conditioned with 24% ethylenediamine tetraacetic acid gel (PrefGel; Institut Straumann AG, Basel, Switzerland) for 2 minutes to remove the smear layer. After a saline rinse, EMD gel was applied to the root surfaces (). The eCTG, with a 2-mm band of epithelium, was placed at the level of the cemento-enamel junction of the recipient tooth and secured with 6-0 absorbable polyglactin (Vicryl; Johnson & Johnson, Sint-Stevens-Woluwe, Belgium) cross mattress and simple sutures (). The flap was repositioned at the original level to prevent the decrease of VD and to completely cover the CTG, except for the 2-mm band of epithelium. The flap was then sutured with 6-0 absorbable polyglactin simple sutures (). The patient was recalled 7 and 14 days later for suture removal and infection control using irrigation with 0.05% chlorhexidine gluconate (Concool F; Weltec, Osaka, Japan). Postoperative care included amoxicillin 750 mg/day for 3 days and 0.05% chlorhexidine gluconate rinses twice a day for 14 days. The patient was advised to refrain from mechanical oral hygiene measures in the treated areas for the first 2 postoperative weeks. The patient also returned for professional teeth cleaning every 3 months.
Approximately 6 months after the first surgery, a sufficient KMW (1.5 mm), KMT (1 mm), and VD were observed at the mesio-buccal site (). A second surgical treatment was then performed by applying an EMD gel combined with a bovine-derived xenograft (BDX) (Bio-Oss; Geistlich Biomaterials, Wolhusen, Switzerland). After the administration of local anesthesia, a buccal full-thickness flap was raised, granulation tissue was removed, and the exposed root was planed (). The root surfaces were conditioned and the EMD gel was applied in the same manner as in the first surgery. The BDX was placed in the furcation defect (). Finally, the flap was sutured in place with 5-0 non-resorbable expanded polytetrafluoroethylene modified vertical mattress sutures (). Amoxicillin was prescribed (750 mg/day for 3 days) postoperatively.
No periodontal or functional problems were observed at the mid-buccal site at the 6-month and 3-year follow-up visits. The clinical parameter scores were the same at both follow-up visits (PD: 2 mm, CAL: 2 mm, CAL gain: 5 mm, HPD: 1 mm, KMW gain: 1.5 mm, GR reduction: 2 mm) (). Additionally, radiographic bone fill was observed in the furcation area at the 6-month and 3-year follow-up visits. An amalgam filling was replaced with composite resin at the 1.5-year-follow-up visit. The patient was satisfied with the results of the treatment. Patient adherence was high throughout the treatment period.
We obtained written informed consent from the patient for treatment, but not for the publication of this case report. However, because we de-identified the patient, we did not require ethical approval and signed consent to publish this report. CARE guidelines were followed in the reporting of this case.


A 58-year-old woman visited our emergency center with a complaint of right-sided back pain. CT examination showed gallstones, a right ureteral stone, and a right abdominal tumor. She had a history of several surgeries: appendectomy for appendicitis, simple hysterectomy for uterine myoma, salpingo-oophorectomy for a left ovarian tumor, and clipping for cerebral aneurysm at 17, 47, 48, and 51 years of age, respectively. The current complaint occurred after the ureteral stone passed naturally. Preoperative inspection for gallstones and additional evaluation of the abdominal tumor were performed. Tumor markers, such as carcinoembryonic antigen, carbohydrate antigen 19-9, α-fetoprotein, and soluble interleukin-2 receptor concentrations, were normal, and gastrointestinal endoscopy showed no abnormalities. The right abdominal tumor appeared as a submucosal tumor at the terminal ileum, with no findings of lymph node enlargement on CT (Fig. ). There were also no accumulations of fluorine-18 fluorodeoxyglucose in the tumor with positron emission tomography imaging. We then decided to resect the tumor during cholecystectomy, to obtain a diagnosis. The surgery was performed via the 4-port laparoscopic-assisted method. After removing the gallbladder routinely, the abdominal cavity was examined. Preoperative imaging suggested that the tumor was present in the small-bowel mesentery; however, 2 tumors were actually present in the right distal greater omentum (Fig. ). We enlarged the umbilical camera port incision, which permitted extraction of the tumors (Fig. ). The tumors were well defined and were resected with sufficient margins (Fig. ). Pathological analysis revealed granulosa cells in the tumors (Fig. ), and the features were the same as for the left ovarian tumor resected 11 years earlier. The postoperative course was uneventful, and the patient was discharged 8 days after surgery.


A 52-year-old female patient, with uneventful past medical history, was admitted to the emergency department due to altered level of consciousness. At the time of admission, Glasgow Coma Score score was 14. Computed tomography (CT) scan revealed a right-sided hyperdensity in the posterior cranial fossa compatible with acute intraparenchymal hematoma adjacent to a venous ectatic aneurysm. CT angiography and digital subtraction angiography (DSA) demonstrated a tentorial dural AVF located at the confluence of torcula and straight sinus, with arterial feeders from the right occipital artery and the posterior branch of the right middle meningeal artery and retrograde tortuous bidirectional venous cortical drainage through a huge venous ectasia up to the proximal end of straight sinus [Figure -] The angiographic characteristics and the clinical presentation classified this DAVF as stage IV according to the Cognard classification.[]
Initially, a transarterial antegrade embolization procedure was attempted through the right external carotid artery (ECA) but failed due to the tortuous course of arterial feeders of the right middle meningeal artery as well as the right occipital artery. Subsequently, the right internal jugular vein (IJV) was directly catheterized under ultrasound (U/S) guidance in an attempt to approach and secure the ruptured venous ectasia transvenously. A 6F guiding catheter was placed at the ipsilateral transverse sinus, and a PX SLIM™ Delivery Microcatheter (Penumbra) was navigated over a Synchro-14 (Stryker Neurovascular, Fremont, California, United States) microwire retrograde through the straight sinus, into the superior cerebellar vein and then through the superior culminate vein (portion of the superior vermian vein) inside the venous ectasia [Figure -]. Eventually, PC400™ Penumbra coils (PC400 can achieve high packing densities using fewer coils due to its larger primary diameter) were deployed inside the huge venous ectatic aneurysm causing almost complete occlusion of the ruptured ectasia except the origin of the two draining veins to the straight sinus [Figure and ]. In this way, occlusion of the venous ectasia was a first-stage life-saving procedure by securing the point of rupture, without compromise of the venous drainage. Subsequently, the patient underwent stereotactic radiosurgery for the DAVF 4 months after embolization. The selective occlusion of the venous ectatic aneurysm relieved venous hypertension inside the aneurysm without prohibiting dAVF venous outflow. This allowed for stereotactic radiosurgery of the DAVF in due time. Angiographic control with DSA 2 years later, revealed total occlusion of the tentorial DAVF. The patient experienced complete neurological recovery.


Patient 3 was a 30-year-old Brazilian male whose parents were Portuguese, without a family history of consanguinity or ophthalmological disorders. He was first observed at our centre at the age of twenty-nine. He presented a history of nystagmus since he was 7 months old.
Symptoms of photophobia were present at an early age. He did not complain of night blindness and peripheral vision loss. Best-corrected VA was 20/200 in OU. He presented refractive error of −6.00 and −8.00 dioptres in the OD and OS, respectively. He had a 15 prism dioptre binocular exotropia.
The anterior segments were normal. The fundus showed a macular atrophy in the OD and a mild macular RPE changes in a “bull's eye” pattern in the OS. Optic disc, retinal vessels, and peripheral retina were normal (Fig. ).
FAF demonstrated a small, round, hypoautofluorescent macular lesion in the OD and a discrete hyperautofluorescent ring at the perifoveal region in OS. SD-OCT scans revealed in the OD an atrophy of both inner and outer subfoveal retina, with well-limited borders. The OS showed an atrophy of photoreceptor external segments. There was reduced retinal thickness at the fovea, with a mean CSF thickness 32 μm for the OD and 74 μm for the OS. FA demonstrated an early diffuse hyperfluorescent mottling in OU and in the OD a macular hyperfluorescence due to a window defect.
Goldman kinetic perimetry revealed a normal visual field. Farnsworth-Munsell 100 Hue Test results showed multiple errors in all 3 axes.
Also, an Ishihara colour test was performed. The patient recognized plates number 1 and 6–8 and partially plates 3 and 9 plates (such as patients with red-green deficiencies).
Electrophysiological test (Fig. ) performed by the age of 30 and revealed a normal DA 0.01 ERG and DA 3 ERG responses. The LA 3 ERG was unrecordable and LA 30-Hz flicker ERG was severely delayed. On pattern ERG P50 wave was undetectable. These results were compatible with cone system dysfunction associated with macular impairment.


Patient 4 is an 11-year-old boy that presented pendular nystagmus and intense photophobia since he was 3 months. His medical history was unremarkable, and there was no family history of serious ophthalmologic diseases or consanguinity.
At the age of 5, he presented a best-corrected VA of 20/50 bilaterally. He had a low hypermetropic refractive error (+1.00 and +1.50 in OD and OS, respectively). Examination showed a 5 prism diopter exotropia of the OS. Ocular fundus examination was entirely normal.
Over time VA decreased. Currently, he is 11 year old and his best-corrected VA is 20/200 in OU. The first electrophysiological test performed at the age of 3 revealed DA adapted ERGs within normal limits, but the LA 3.0 ERG and LA 30-Hz flicker ERG were nonrecordable (Fig. ). These results indicated a cone dysfunction and normal rod function.
Colour vision was tested. Farnsworth-Munsell 100 Hue Test presented multiple errors is the 3 axes. Additionally, colour vision was tested using Ishihara colour plates in standard conditions. The boy recognized plates number 1 and 14 and partially plates 5 and 7–8 plates (such as patients with red-green deficiencies).
Fundus colour photography, SD-OCT, FA, and FAF are not displayed, was unable to cooperate to perform these exams due to the intense photophobia, nystagmus, and the young age.


Patient A: A 21-year-old, before healthy female patient suffered a severe polytrauma in a motorcycle accident with an injury severity score (ISS) of 75. The main injuries were severe craniocerebral trauma, severe blunt abdominal trauma with liver rupture and a decollement in the area of the left thigh extending to gluteal. Primary surgical treatment included laparotomy and liver suturing. On the first post-traumatic day LiMAx-value was 305 µg/kg/h, while static liver parameters were massively increased. Hemicraniectomy was performed due to rapidly increasing brain edema. Persistently high intracranial pressure (ICP) values prompted to change the sedation regimen to an inhalative concept with isoflurane under continuous ICP control. While laboratory liver parameters quickly normalized, LiMAx test results were 2 and 3 µg/kg/h after 25 and 123 h. After the cessation of isoflurane, the LiMAx returned spontaneously to 180 µg/kg/h.


Patient B: A 65-year-old man who had undergone rectum resection for advanced rectal carcinoma developed anastomosis insufficiency with abdominal sepsis. The antimicrobial chemotherapy included the liver-toxic antibiotic linezolid. LiMAx was used to monitor a possible deterioration of liver function at an early stage. The first test result was 151 µg/kg/h. Since the respirator therapy had to be continued for longer than 7 days, sedation was switched to isoflurane. In the further course of treatment, LiMAx decreased to 2 and 10 µg/kg/h. After isoflurane was discontinued LiMAx increased to 254 µg/kg/h while transaminases also increased.


Patient C: A 49-year-old man had received thoracotomy for a suspicious pulmonary node. After an atypical resection of the right lower lobe, he was transferred to the intensive care unit. In the course of the first post-operative day, an endobronchial hemorrhage occurred with acute deterioration of gas exchange. The bleeding from the middle lobe segment 4 was stopped by epinephrine instillation and tamponades in repeated bronchoscopies. A re-thoracotomy could be avoided. Due to recurrent bronchospastic episodes, isoflurane sedation was established early on. LiMAx test was performed sequentially to assess liver function in perceived sepsis. After an initial reading of 376 µg/kg/h with propofol, LiMAx values of 33 and 77 µg/kg/h, respectively, were measured under isoflurane sedation. Clinical and laboratory assessment did not correlate with this decrease. After discontinuation of isoflurane LiMAx increased back up to 496 µg/kg/h, other parameters also indicated normal liver function. The patient's demographics and clinical data are listed in detail in Tables and , for LiMAx test results in correlation with standard liver parameter and isoflurane see Figure . The LiMAx tests were performed according to the manufacturer's specifications (Humedics Inc) and the clinical standard. To avoid a possible influence of the metabolism of the hydrophilic 13C-methacetin by the continuous renal replacement therapy (CRRT), the latter was paused directly before the start and during each measurement. CRRT was re-started immediately after the end of the measurement.


A 75-year-old woman with a diagnosis of pancreatic mass was admitted for surgery.
A 75-year-old Chinese woman was admitted because of paroxysmal exacerbation of acute abdominal pain for 1 d with no apparent cause. The pain was not responsive to changes in body position and did not radiate to the other side. The patient did not complain of nausea, sour regurgitation, or abdominal fullness but did note slight fatigue.
The patient had hypertension for the previous 10 years, which was well controlled by drug therapy.
The patient had a more than 10-year history of hypertension that was under fairly satisfactory control via medication.
Abdominal physical examination showed a flattened belly, normal borborygmus, and epigastric tenderness and rebound pain, with a negative Murphy’s sign.
Blood biochemistry tests revealed the following: Elevated blood amylase (742 U/L; chromatometry reference range: 35-135 U/L), undetectable blood lipase (chromatometry reference range: less than 79 U/L), low albumin (32.2 g/L; reference range: 40-55 g/L), low calcium (2.06 mmol/L; reference range: 2.25-2.75 mmol/L), low platelets (98 × 109/L; reference range: 100-300 × 109/L), and highly elevated CA242 (over 150 IU/mL; reference range: 0-20 IU/mL), CA50 (289.6 IU/mL; reference range: 0-20 IU/mL), and CA199 (627.4 IU/mL; reference range: 0-37 IU/mL).
A plain CT scan showed a mass in the body and tail of the pancreas, with little exudation (Figure ). The morphology of the pancreas was disrupted, indicating edema, hyperemia, and fat liquefaction. The stomach appeared normal (Figure ). Two days later, contrast-enhanced CT showed a cystic solid mass surrounding splenic vessels in the caudal pancreas, indicating a pancreatic malignant tumor (Figure ). Multiple hepatic cysts, cholecystitis, and cholecystic polypus were also detected. The gastric antral wall was found to be thickened (Figure ). Gastric endoscopy was performed 3 d later. The images showed chronic superficial and atrophic gastritis, a protruding lesion in the submucosal area of the antrum (Figure ), and no Helicobacter pylori infection. Contrast MRI was performed the following day and indicated PC (Figure ) and uniform thickening of the antral wall (Figure ). Due to the aforementioned imaging findings, we decided to perform operations to relieve PC and tried to figure out the nature of the gastric mass intraoperatively.
The gastric mass under pathological examination post operation was a gray, solid, and tough nodule measuring 2 cm × 2.2 cm × 1 cm in the pylorus. Immunohistochemical staining showed that the tumor cells were positive for CK8, partially positive for Ki-67, and negative for CD147. Micrography showed multiple multifocal heterotopic pancreatic acini (Figure ), mucus-excreting glands such as Brunner’s glands, and undifferentiated ducts surrounded by proliferative smooth muscles in the submucosal layer, which typically indicate GA with HP. The tissue in the other slice showed undifferentiated epithelial cells covered with thickened smooth muscle, indicating GA alone (Figure ), with no signs of malignancy. Regarding the 3.5 cm × 2 cm × 3 cm pancreatic mass, distinct dysplasia of the acini and cells arranged in a mass without polarity indicated cancer cells. The nuclei were deeply basophilic, with common karyokinesis, enlarged in size, and increased in number compared to those in normal tissue (Figure ).


A 32-year-old male patient presented due to a 5-year history of an enlarged left scrotum with increasing discomfort.
The patient had signs of discomfort, without abdominal pain, and swelling.
The patient was healthy and had no relevant history of disease.
The patient had no personal and family history of malignancy.
Physical examinations revealed a palpable subcutaneous mass measuring about 7.0 cm × 6.5 cm in the left scrotum, with tenderness (Figure ). The left scrotum was enlarged, containing an oval-shaped mass with a smooth surface and no tenderness. Using a light transmission test, the testes on both sides were found to be palpable.
Laboratory findings were within normal limits. Serum human chorionic gonadotropin and alpha-fetoprotein were in normal level.
Color Doppler ultrasound of the scrotum, bilateral testes, and epididymis was performed, which revealed a subcutaneous hyperechoic mass in the left scrotum with a size of approximately 72 mm × 64 mm × 41 mm. The mass had a clear boundary, and the internal echo was uneven with visible sinusoids and strip-shaped blood flow signals. The mass was not connected to the abdominal cavity (Figure ). No apparent abnormalities were observed in the other parts.


An 80-year-old man with past medical history of peripheral artery disease, hypertension and end stage renal disease (on peritoneal dialysis) was sent to the emergency department from a nursing home for evaluation of hypotension. He denied any chest pain, shortness of breath, fever, cough, palpitation, nausea, vomiting, abdominal pain and changes in urinary or bowel habits. On arrival, patient’s blood pressure was 63/29, pulse was 72/min and temperature 97.2 F. Laboratory studies showed the following values: WBC count 16.5 K/UL, Hgb 14.3 GM/DL, PLT 115 K, Creatinine 12.59 MG/DL, BUN 56 MG/DL, Sodium 133 MMOL/L, Potassium 5.4 MMOL/L, BNP 143 PG/ML, Troponin 0.05 NG/ML. An hour in the ED, EKG showed significant ST segment elevation in lead V3, V4 and V5 and Q wave in lead II, III and AVF []. A diagnosis of acute ST elevation MI was made and oral aspirin 325 mg, clopidogrel 300 mg, atorvastatin 80 mg and 3500 units/kg of heparin were administered. Emergent cardiac catheterization was performed. Coronary angiography showed chronic total right coronary artery occlusion with collaterals from left and patent left coronary arteries []. Left ventriculography revealed wall motion abnormalities with mid to apical akinesis and basal hyperkinesis []. Post catheterization, the patient was transferred to ICU for further management. In the ICU, the patient was started on levophed pressure support as the patient was hypotensive. Four hours after the admission, CK-MB and Troponin reached a maximum value of 11.6 ng/ml and 2.10, respectively. Six hours after the admission, EKG showed resolution of ST elevation in anterior leads. Given the patient had leukocytosis in the presence of hypotension, broad spectrum antibiotics including vancomycin and Zosyn were started considering a combination of septic and cardiogenic shock. Approximately 12 hours later, transthoracic echocardiography revealed left ventricular ejection fraction 25–30%, elevated left ventricular end diastolic pressure and akinesis of left and right ventricle except in the basal region []. Although the patient remained chest pain free, but continued to remain on levophed and neosynephrine for pressure support. Workup for sepsis was unremarkable. A day later, a repeat echocardiogram showed sessile LV thrombus and akinesis of both left and right ventricles except in the basal region []. Anticoagulation with heparin was started. Plan was to repeat the echo after 3–4 days to monitor the response to therapy. Patient remained hemodynamically unstable and, ultimately, died 72 hours after admission. In accordance with his family wishes, an autopsy was not performed.


The individual was a 24-year-old African American male inpatient at a state psychiatric facility. His psychiatric diagnoses included unspecified schizophrenia and psychosis as well as alcohol, cannabis, and nicotine use disorders. His somatic diagnoses included benign ethnic neutropenia and constipation. During the time of this report, his psychiatric medications included haloperidol decanoate 300 mg IM every 28 days in the deltoid and quetiapine 800 mg PO at 9 pm for the past 1.5 years. His somatic medications included polyethylene glycol, docusate, and benztropine. During a previous admission, the patient received daily PO haloperidol (maximum 40 mg/d) but did not receive decanoate. He had no known food or drug allergies. Both haloperidol decanoate and quetiapine were continued at these doses throughout the duration of this case report.
As part of the hospital's routine vital sign monitoring, monthly temperatures were recorded for the patient from hospital day (HD) 1-263. With the local onset of the COVID-19 pandemic, daily temperatures were recorded starting on HD 263. Because of the increased frequency in temperature monitoring, a pattern began to emerge in which the patient's temperature was noted to be elevated shortly after receiving his haloperidol decanoate injections (). Upon questioning, the patient reported feeling feverish after injections since the beginning of haloperidol decanoate therapy, but no fevers were recorded due to the infrequency of temperature monitoring prior to the COVID-19 pandemic. He also reported that there was usual injection site pain, swelling, and a “knot” feeling that resolved within a few days. During the time of the fever, muscle rigidity or mental status changes were not observed. Out of the 11 haloperidol injections after initiation of daily temperature monitoring, a temperature of greater than or equal to 100.0°F occurred after 7 injections, each within 2 or 3 days. After each of these occurrences, he received 1 to 2 doses of acetaminophen, and the temperature returned to baseline until the next injection. No other doses of acetaminophen were utilized until the administration of the last 2 recorded haloperidol decanoate doses (HD 520 and 548). At this point, ibuprofen and acetaminophen were given prior to the injection and for the 3 days following as a preventative measure. This was effective against fever and the other adverse reactions he previously experienced, including the knot and swelling at the injection site. Unfortunately, no haloperidol levels were drawn, preventing further evaluation of why this may have occurred.
Additionally, the patient was started on clozapine on HD 387, reaching a maximum dose of 200 mg/d and discontinued on HD 503 due to no significant improvement in behaviors or delusions since initiation. During this time, weekly CBC counts were drawn to monitor absolute neutrophil count as a routine part of clozapine therapy at initiation. Thus, WBC counts are available during this time period, allowing analysis during haloperidol decanoate therapy (). By tracking WBC levels, another pattern emerged of elevated counts within 5 days of medication administration with a subsequent decrease that trends slightly higher than the previous month's baseline. It is important to note that this patient had benign ethnic neutropenia, which accounts for his baseline low WBC.
Aside from fever and injection site reactions, there were generally no other somatic complaints besides 1 headache on HD 354. The patient tested negative for COVID-19 multiple times as well as negative for a COVID-19 serology test on HD 551. He tested negative for influenza A/B on HD 497. Additionally, C-reactive protein levels were found to be elevated twice (HD 355 and 363), but no additional levels are available after a normal level obtained on HD 378. Creatine phosphokinase levels were not obtained due to an absence of muscle rigidity.
One consideration was that this adverse reaction could be occurring with any intramuscular injection because this trend was not observed when he was receiving only oral haloperidol. However, the patient received his first COVID-19 vaccination on HD 565, denied any adverse reactions, did not develop a fever postvaccination, and no acetaminophen or ibuprofen was given.


A 92-year-old man received hospital care after suffering a left hip fracture. This led to prolonged bed rest of more than a month which led him to develop PI in the left heel. Even though he was treated weekly on an outpatient basis for five months, the PI did not heal. The patient was assessed by the wound clinic of a specialist hospital Bogotá-Colombia for the management of his PI. Norton scale was used to obtain the risk to developing a new PI and the result was 14/20 which corresponded to high risk. The patient had a history of blindness, and he had to use a wheelchair. For this reason, he is limited in his mobility and made him dependent for basic activities. He was considered a candidate for the use of PRP alongside conventional therapy (cleaning, application of zinc oxide, maintenance of dry skin and removal of pressure on the lesion).
This intervention complied with the Declaration of Helsinki, including approval by the institution’s Ethics Committee (approval number: FM-CIE-0448-18).
Researchers prepared an informed consent that was approved by the ethics committee of the Pontificia Universidad Javeriana. The patient was blind, we obtained a verbal informed consent from the patient and patient’s legal guardian sign the informed consent, for the case details and accompanying images to be published.
At the first evaluation, the patient had no coagulation abnormalities, no history of cancer, or the presence of signs of local or systemic infection. Within his paraclinical tests, he presented a hemogram with leukocytes 8,800/mm3, hemoglobin 14.8 g/dL, hematocrit 43.5% platelets 336,000/mm3, creatinine 0.57 mg/dL, albumin 39 g/dL, all within normal values. Upon admission to the study, a NUAP classification Phase II pressure ulcer with thick edges measuring 1.8×3.2 cm was observed (). After the evaluation of the PI, the preparation of autologous platelet-rich plasma (APRP) was carried out as follows: 50 mL of whole blood was extracted in tubes with sodium citrate, it was centrifuged at room temperature for 10 minutes at a speed of 1,500 rpm, according to the previously standardized protocol. 8 cm3 of PRP were obtained, which were placed in a Petri dish and 400 mg of calcium gluconate (50 micrograms for each cubic centimeter obtained from PRP), after 25 minutes it was polymerized and applied to the previously cleaned wound. It was covered with a transparent sterile paraffin dressing (Cuticell ®Classic) and the patient’s family member was instructed to remove it after 72 hours. The wound was evaluated at 7 days (); the same treatment was repeated once a week for a total of four sessions. During treatment researchers observed that after the first week there was a slow decrease in the percentage of closure of the PI, but that after 15 days a closure greater than 50% was achieved and after the third application and evaluation after 24 days, the closing was greater than 90% (). Finally, at the fifth week, the PI was evaluated, showing changes in the diameter of the wound of 0.5×1.2 cm, without signs of infection or discharge (). The wound continued to receive normal healing during the following weeks and after another month of treatment the wound closed completely.


A 78-year-old male patient presented with a primary chief complaint of pain over the right half of his face, especially the right forehead and upper cheek, since 2014. When the patient wraps his right face, the pain was triggered by the gusts of wind, eating, and triggered simultaneously when the patient does nothing. This pain has a characteristic of stabbing-like pain that lasts about 1 min, which occurs about three times a day with a frequency of more or <6 days a week. He already takes a combination of anticonvulsants for about 5 years, but the pain does not decrease. He took carbamazepine for the first time in 2014 with a dose of 100 mg twice a day for 3 years, but the intensity and duration of pain do not decrease. Later, he takes a combination of carbamazepine with gabapentin 300 mg twice a day for about 2 years, but the medications do not be effective anymore. The patient also complains of a twitching over the right eyelid and forehead 3 years prior. For a month, the frequency is about seven times a week. The patient and his family had noticed that the twitching on the right eyelid and forehead persists even when he fell asleep. The patient does not complain about weakness on the right side face, decreased hearing, dizziness, or loss of balance.
The patient's visual analog scale is eight, the Barrow Neurological Institute (BNI) Pain Intensity Scale is 4, and the physical examination had revealed increased sensitivity to light touch over the right side face innervated by the ophthalmic and maxillary distribution of the N.V. No abnormalities found over the mandibular distribution of the right N.V. There are no abnormalities found in the examination of facial nerve (N.VII), vestibulocochlear nerve (N.VIII), and the other cranial nerves. The noncontrast T1 and T2 magnetic resonance imaging (MRI) showed a neurovascular contact of the right superior cerebellar artery (SCA) with the right N.V root entry zone []. There is no compression found over the N.VII structure.
The patient was considered to take a surgical procedure to release the neurovascular contact. Before we performed the procedure, the patient was informed about the surgery step-by-step, advantages, side effects, and complications that may be developed during and after the surgery, especially in elderly populations. This procedure was done in a retrosigmoid approach that bordered by transverse sinus superiorly and the sigmoid sinus laterally. The cerebellar hemisphere is retracted, and the arachnoid membrane is dissected. The N.VII and N.VIII complex is encountered from the root entry zone until the distal part enters the meatus. Anterior inferior cerebellar artery (AICA) compresses this structure along the lateral side of the N.VII/VIII complex []. A shredded Teflon implant is interposed between the AICA and VII/VIII complex []. The operation moved into the right N.V structure. The adjacent vascular and arachnoid membrane is dissected off, and the N.V is seen clearly from the root entry zone to the distal part. The SCA is seen and compresses the axillar side of the N.V []. The shredded Teflon implant is interposed between the N.V and SCA complex [].
This patient is observed overnight in the intensive care unit (ICU) and given intravenous (IV) ceftriaxone 2 × 1 g; IV omeprazole 2 × 40 mg; IV ketorolac 2 × 30 mg; IV plasminex 3 × 500 mg; IV Vitamin K 3 × 40 mcg; and IV dexamethasone 3 × 5 mg. He does not complain of any pain and spasm after the operation, so the postoperative BNI pain intensity scale in this patient is one and it is considered a satisfactory outcome. He had a mild complication such as hypoesthesia over the right-half face that resolved until postoperative day 3. The patient does not complain of any dizziness, nausea, decreased hearing, and paralyzed over the right half face. After the patient is stabilized and the hypoesthesia is resolved, the patient is discharged on postoperative day 4. There is no recurrence in pain and spasm after 1-year follow-up in this patient.


In the first case, a 32-year-old male presented with a complaint of lower backache for 5 years, which progressively increased in intensity, radiating to the left leg. The patient had left lower limb weakness in the form of difficulty in walking. There was no sphincter involvement. On examination, the power of the left knee 4/5, ankle in dorsiflexion and plantar flexion 4/5, left extensor hallucis longus 3/5, and the left Babinski sign was positive. No history of previous spine intervention was present. Magnetic resonance imaging (MRI) of the spine revealed intramedullary lesion in the conus at L1–2. Lesion was hyperintense with areas of isointense on T1-weighted image and heterogenous on T2 weighted image. Margins were enhanced on contrast T1 weighted MRI []. The patient underwent D12–L2 laminectomy. The dura was opened and intramedullary cystic lesion was found at the conus. The lesion was thin-walled with visualization of intrinsic yellowish content. Posterior midline myelotomy was performed followed by evacuation of dirty yellowish pultaceous material suggestive of EC. The cyst wall was firmly adhered to the conus medullaris proximally and nerve roots distally. The cyst was evacuated, but the wall could not be excised to prevent injury to neural tissue; hence, epithelial lining of the cyst wall was biopsied and extensively electrocoagulated to prevent recurrence. On histopathology, the cyst was lined by keratinizing stratified squamous epithelium and contained degenerated lamellated keratinous material []. All these features were characteristic of intramedullary white EC. Immediately after the surgery, the patient worsened to Grade 3/5 power in the right lower limb with foot drop, which partially recovered over a period of the next 6 months. Left-sided weakness totally recovered after surgery.


A 42-year-old male, presented with a complaint of numbness over the left foot for 5–6 months. On examination, the power of the left ankle was 3/5, left extensor hallucis longus was 3/5, and 50% loss of sensation of touch, pain, and temperature at L3–L5 dermatome. Sphincter involvement was absent. History of previous spine surgery or lumbar puncture was absent. MRI spine revealed intramedullary lesion in the conus at D12–L1. Lesion was hyperintense with areas of isointense on T1-weighted image and heterogenous on T2 weighted image []. The patient was operated with D12–L1 laminectomy. The dura was opened in the midline and intramedullary cystic lesion was found at the conus. Posterior midline myelotomy was performed followed by evacuation of yellowish pultaceous material. The cyst wall was firmly adhered to the neural tissue of conus medullaris and could not be excised to prevent injury to neural tissue. The cyst was evacuated and the epithelial lining of the cyst wall was electrocoagulated although not extensively to prevent any new deficit, based on previous experience. On histopathology, the cyst was lined by stratified squamous epithelium and underlying fibro collagenous tissue showed hemorrhage and lymphomononuclear infiltrate []. All these features were characteristic of intramedullary white EC. Immediately after the surgery, the patient recovered completely with no new deficit. On 5 months, follow-up patient developed numbness with recurrence of the cyst at the same site. The patient was advised to repeat surgery, which he refused.


A 7-year-old male with a history of fall presented with left flank pain and hematuria. Focused assessment with sonography in trauma (FAST), followed by contrast-enhanced computed tomography (CECT) revealed Grade-4 laceration at the lower pole of the left kidney and a peri-nephric hematoma, which was confined within the Gerota's fascia. The patient was managed conservatively on antibiotics and pain-killers. During the follow-up, ultrasound scan revealed increase in the size of the peri-nephric collection (after ~1.5 months). Thereafter, the patient was referred to the department of nuclear medicine for assessment of the nature of this peri-nephric collection, in addition to the evaluation of residual functioning renal parenchyma. 99Tc-diethylenetriamine pentaacetate scintigraphy revealed a photopenic area in relation to the lower half of the left renal fossa []. The upper half of the left kidney showed preserved function. In late dynamic images, an area of progressively increasing radiotracer activity was noted in the infero-lateral aspect of the left renal fossa; which was still separated from the functioning left renal parenchyma by a photopenic area. Still, greater extravasation of radioactive urine was noted into this collection in serial static images acquired till 4 h [Figure -]. Single-photon emission computed tomography images were also acquired, which revealed a parenchymal laceration in the inferior pole of the left kidney [Figure -]. A large iso-dense hematoma was noted inferior to the inferior pole of the left kidney, which did not show any significant radiotracer uptake. A radioactive collection with fluid density was noted lateral to this hematoma, but still confined within the Gerota's fascia [Figure and ]. This fluid density was communicating with the pelvi-calyceal system (PCS) though the renal laceration [arrows in Figure , and ]. The collection was thus diagnosed as a urinoma and because it was communicating with the PCS, the clinicians decided on the conservative management. During follow-up, the swelling was seen to reduce in size in sequential ultrasounds.
Posttraumatic urinomas are very rare. Prerequisites for their formation are functioning renal parenchyma, injury to PCS and obstruction in the urinary tract.[] Many urinomas are small and resolve spontaneously, but large and expanding urinomas often require intervention.[] Intervention may also be required to accelerate healing in cases where renal urinomas separate the fragments of renal parenchyma.[] CECT is the preferred modality for the diagnosis, as extravasated contrast can be used to identify urinomas/urinary leaks.[] However, CECT cannot be used to measure renal function and associated risks with administration of IV contrast are a constant worry. In addition, in many patients with renal trauma, the renal function may be compromised. Although there were no such complications in the presented case, there were apprehensions related to repeat contrast administration after just 1 month of previous CECT as well as radiation-related concerns in a young child. The use of sequential CECTs is less than ideal in such a scenario as there was no urinary collection at initial presentation but progressively increasing collection was noted in follow-up, which necessitated serial imaging. Renal scintigraphy, on the other hand, is not associated with severe side effects such as allergic reactions (vs. IV contrast), it can be used in patients with compromised renal function and delivers much less radiation dose compared to CECT.[] This case shows the utility of this old and relatively inexpensive imaging modality in multi-faceted evaluation of cases with renal trauma, wherein it was able to diagnose preserved function in the residual renal parenchyma, identify the nature of peri-nephric collection, and establish the communication of this collection with PCS. All this allowed clinician to opt of conservative management in spite of increasing the size of collection and provided them with a low radiation alternative to serial CECTs in follow-up.
The authors certify that they have obtained allappropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Nil.
There are no conflicts of interest.


A 36-year-old female patient, with a history of posterior fossa ependymoma, was operated 1997 when she was 13 years old with hydrocephalus. She had prior a VP-Shunt on the right side. No other medical history. The evolution was good with no complications.
In 2020, 8 months ago before her admission, she presented thoracic pains followed by yellowish cough and sputum, without fever and headaches, everything evolving in a context of conservation of general condition.
She was seen prior by the thoracic surgeons, that suspected a hydatic cyst, they performed a thoracic X-ray, that showed a rounded left diaphragmatic collection. She had an abdominal computed tomography (CT) scan that revealed a rounded left diaphragmatic collection with some peripheric calcifications. The dimensions were 31.8 mm × 31.1 mm × 25.5 mm [].
She was turned to the operating room, by thoracic surgeons. She had a thoracotomy and the diaphragm was ruptured, with a mass that they punctured and it was a puriform collection, the wall had been opened and they found a VP Shunt catheter. Then they called the neurosurgeons to the operating room.
When we came, we saw the herniated catheter in the thorax surrounding the abscess that has been removed and drained. The abdominal tip of the VP Shunt was still permeable and the liquid was citrus then gradually thinned out [ and ].
At the same time, we removed the functional VP Shunt after a retro-auricular incision. The ventricular tip was still functional. We removed initially the reservoir and after the distal part in the thorax. We decided to watched her clinically and with a cerebral CT scan and physical examination because of the risk of acute hydrocephalus.
She remained clinically fine with a Glasgow Coma Scale of 15, post-operatively, without headache and vomiting. The biological analysis found a gram-negative bacterium Escherichia coli, and also Beta (2 transferrin a protein-specific of cerebrospinal fluid (CSF) and perilymph was found confirming The CSF Leak treated by Trimethoprim/Sulfamethoxazole and Carbapenem for 14 days and treated as meningitis. The CSF analysis showed a normal dose of glucose and protein but the leukocytes were found 30 cells. The postoperative CT scan was performed and no active dilatation has been observed. The evolution was good without intrathoracic collection and without hydrocephalus. We planned to evaluate her condition 1 month after the infection. Hence, if she remains stable she will not need a shunt again. But if she came with hypertension signs we will choose then to operate.


A 47-year-old male with no cardiovascular history presented to the emergency department with acute onset lower limb weakness. He had a 3-day history of being unwell with pyrexia, rigours, lethargy, supra-pubic discomfort, and bilateral flank pain. Upon arrival, he had a blood pressure of 147/68 mmHg and tachycardic at 125 beats per minute (b.p.m.). His was tachypnoeic at 24 breaths a minute and pyrexial with a temperature of 39.4°C. On examination, he had tenderness over the lumbar spine with reduction of power (2/5) in the proximal lower limbs. There was absence of anal tone on digital rectal examination and he failed a tug test when a catheter was inserted due to urinary retention. His heart sounds were normal with no murmurs appreciated. There was no evidence of splinter haemorrhages, Janeway lesions, or Osler nodes.
Bloods on admission showed raised inflammatory markers, hence a set of blood cultures were obtained. The electrocardiogram (ECG) obtained on admission () shows sinus tachycardia with a ventricular rate of 125 b.p.m. with a normal cardiac axis, PR interval, QT and QTc intervals, and QRS duration. Given the neurological findings, a magnetic resonance imaging spine () was performed for suspected cord compression. This revealed a collection in both the anterior and posterior epidural space causing compression of the thecal sac. Post-contrast imaging performed confirmed an enhancing epidural abscess extending from the skull base to the 7th thoracic vertebrae. The brain parenchyma was unremarkable with no identifiable abnormality.
An ECG performed prior to surgery () revealed complete atrioventricular (AV) dissociation (3rd degree AV block). However, the resulting ventricular rate was 75 b.p.m. and the QRS was narrow (<100 ms) in duration. As the ventricular rate was normal, no specific intra-operative precautions were taken at this point. The patient underwent urgent cervicothoracic decompression and washout of cervical vertebrae 6 to thoracic vertebrae 5. Pus obtained during surgery was sent for microscopy, culture, and sensitivity.
Post-spinal surgery, he was still in complete AV dissociation but now bradycardic () in the intensive care unit (ICU) and was started on noradrenaline. Due to this change, the cardiology team was contacted to review the patient. Our choice for chronotropic support would have been isoprenaline. However, our colleagues in the intensive care setting were not as familiar with this and felt more comfortable with noradrenaline titration. As the heart rate improved with low-dose noradrenaline and the blood pressure was not compromised, the cardiology and ICU team felt that a temporary pacing wire was not required at that point. The patient remained on a cardiac monitor in ICU with close monitoring from the cardiology team. Given the lack of cardiac history, the initial working diagnosis was autonomic dysreflexia (AD).
An infective haematogenous spread was suspected as Staphylococcus
aureus was grown from peripheral blood cultures and pus obtained during spinal surgery. There was no obvious bacterial entry point identified on inspection of the patient, however a formal dental check was not performed. Given the evidence of heart block pre- and post-surgery, the source of infection was suspected to be from the heart. A transoesophageal echocardiogram performed revealed evidence of GD associated with small vegetation measuring 4.0 × 7.0 mm (, Videos 1 and 2) Both atria were mildly dilated but bi-ventricular size and function were normal. There were no additional valve lesions observed and no evidence of an aortic root abscess.
The patient was discussed at the infective endocarditis multidisciplinary meeting, the decision was made to treat with IV antibiotics and not close the GD in the acute setting. At the time of discussion, the patient was recovering from surgery in ICU with significant paralysis of the lower limbs. Furthermore, the shunt size was small with no ventricular chamber enlargement or reduction in systolic function. A positron emission tomography–computed tomography was not performed as the patient had clear vegetations associated with the GD and no pacing wires inserted. However, it would have been beneficial if the source of infection was unclear. He received IV Vancomycin for 46 days while recovering from spinal surgery.
The vegetation was not seen on transthoracic echocardiogram (, Video 3) performed 35 days post-antibiotic initiation. There were no further positive blood cultures and no murmur was heard. Intermittent periods of sinus rhythm with normal AV conduction were noted whilst being monitored in ICU and this was confirmed and sustained during recovery. Electrocardiogram 2 months’ post-surgery demonstrated a sinus tachycardia with no further episodes of complete heart block noted.
The patient was discharged from hospital after 83 days and is currently receiving rehabilitation in the local spinal rehab unit due to persistent lower limb weakness.
Our patient remains severely debilitated and disabled. He has been referred to the regional adult congenital heart disease (ACHD) team for long-term follow-up to monitor the defect and if he develops any symptoms of heart failure. We have deferred the decision to close is defect pending his neurological recovery. If surgery were to be performed, he would almost certainly require permanent pacing as the conduction defect would likely be exacerbated by surgery.


A 46-year-old postmenopausal woman was admitted to our ED with complaints of massive abdominal distention which started gradually 30 days ago. She also complained of difficulty in breathing and ambulation. There were no other gastrointestinal, gynecological, or urinary symptoms. The woman had a significant past medical history of hypertension. Her family history is negative for ovarian and breast cancer in first-degree relatives. On physical examination, the patient’s abdomen was distended. Blood analysis revealed hemoglobin of 13.9 g/dL, 8280 leukocytes, and 226 000 platelets. All tumor markers were within normal limits. However, the ovarian cancer antigen (CA 125) level was 260.9 U/mL (normal range: 0–35 U/mL). Abdominal and pelvis MRI was performed, which revealed a huge pelvic-abdominal mass measuring approximately 22 x 15 x 12 cm (). As there was a strong suspicion of malignancy, the patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy. The enormous lobulated tumor weighing 6.7 kg had a thick wall with solid and cystic areas (). Her final histopathology report was suggestive of uterine leiomyoma. The patient had no intra-operative or postoperative complications. On day 5 after the surgery, the patient was discharged home. Three months following the surgery, the CA 125 levels had regressed to the normal range. The patient has since been symptom-free, and the CA 125 levels have also remained normal.


A 72-year-old Japanese man was diagnosed with splenic DLBCL (stage IV) in April 2018 at the Department of Hematology, Kita-Harima Medical Center, and 8 cycles of R-CHOP therapy (375 mg/m2 rituximab on day 1, 50 mg/m2 doxorubicin on day 2, 750 mg/m2 cyclophosphamide on day 2, 1.4 mg/m2 vincristine on day 2, and 100 mg prednisolone on days 2–6) every 3 weeks were planned.
The first cycle of chemotherapy was started in the hospital setting (Fig. ). Prophylactic daily subcutaneous administration of granulocyte colony-stimulating factor, filgrastim (75 μg/day), was performed from day 4 to day 12 to reduce the incidence of febrile neutropenia (FN). On day 6, the patient developed high fever (38.8°C), nausea, vomiting, sore throat, and loss of appetite. Laboratory investigations showed an elevated white blood cell (WBC) count of 23,780/mm3 (normal, 3,500–9,700/mm3), along with a marked increase in neutrophil and decrease in monocyte percentages to 95.8% (normal, 38–74%) and 0.3% (normal, 2–10%), respectively. The concentration of C-reactive protein (CRP) was within normal limits. Neither chest X-ray nor urine findings showed any abnormality. Treatment with intravenous piperacillin/tazobactam (PIPC/TAZ) (18 g/day) was given for 5 days from day 7, with marked improvement in clinical symptoms within 3 days. Intravenous PIPC/TAZ was changed to oral levofloxacin hydrate (LVFX) (500 mg/day for 3 days) on day 12. Blood culture performed on day 7 was negative after culturing the sample for 5 days. Blood culture was performed using an automatic blood culture system (BACTEC FX; Nippon Becton Dickinson Co., Ltd., Minato City, Japan) with aerobic and anaerobic resin bottles. Retrospectively, FN was ruled out since absolute neutrophil count did not decrease below 500 during the entire course of the chemotherapy cycle. However, the cause of the fever remained unknown.
The second cycle of chemotherapy was commenced from day 1 to day 3 of admission (Fig. ). Subcutaneous injection of granulocyte colony-stimulating factor, pegfilgrastim (3.6 mg), was administered on day 4. Nausea and chills and high fever (38.0°C), respectively, appeared on days 5 and 6. Laboratory investigations on day 6 showed an elevated WBC count of 16,550/mm3, along with marked increase in neutrophil and decrease in monocyte percentages to 99.7% and 0%, respectively. CRP was elevated to 3.06 mg/dL (normal, 0–0.3 mg/dL). Based on a suspicion of bacterial infection, oral LVFX (500 mg/day) was prescribed. Several asymptomatic erythematous lesions appeared on the trunk and upper extremities on day 8 (Fig. ). Intravenous PIPC/TAZ (18 g/day) therapy was started on the same day, while oral LVFX was discontinued. The antibiotic therapy promptly improved clinical symptoms. Furthermore, the erythematous lesions completely disappeared within 3 days. The patient was discharged after 6 days of treatment with intravenous PIPC/TAZ. Simultaneously, however, a Gram-negative spiral bacillus was isolated from the aerobic blood culture bottle after a 5-day culture, while anaerobic culture was negative. Subculture of the positive blood broth was performed on blood agar plates for 2 days in a microaerobic (5% O2, 5–10% H2) atmosphere at 35°C, resulting in the formation of thinly spread film-like colonies. A positive colony from the subculture was analyzed by matrix-assisted laser desorption/ionization time of flight mass spectrometry [, ] (Fig. ). The identification score was 1.93, strongly suggesting that the organism constituting the positive colonies was H. cinaedi. Later, the organism was further identified as H. cinaedi by polymerase chain reaction (PCR) (Fig. ), as previously described []. Based on these results, it was concluded that the fever and skin eruption were caused by H. cinaedi bacteremia.
Before starting the third cycle of chemotherapy, amoxicillin clavulanate (AMPC/CVA) (1,500 mg/day for 7 days) was prescribed against H. cinaedi, and blood culture was started from 6 days before day 1 (day 17 of the second cycle). After confirming that the blood culture was negative after a 6-day culture, the third cycle of chemotherapy with dose reduction (80%) was started (Fig. ). Subcutaneous injection of pegfilgrastim (3.6 mg) was performed on day 4. On day 6, the patient developed fever (39.1°C) and chills and was seen at the emergency department. Laboratory investigations showed an elevated WBC count of 23,620/mm3, along with marked increase in neutrophil and decrease in monocyte percentages to 98.9% and 0%, respectively. The CRP level was high, at 4.16 mg/dL. Referring to the history of fever during the previous 2 chemotherapy cycles, we administered intravenous PIPC/TAZ (18 g/day) after admitting the patient, resulting in rapid improvement of clinical symptoms within 2 days. In addition, blood culture was performed on the same day. However, blood culture performed before the start of the third cycle of chemotherapy yielded a Gram-negative spiral bacillus on day 7 of the third cycle after a 12-day culture, and the bacillus was suspected to be H. cinaedi based on the fact that similar bacillus detected by blood culture during the second cycle had been identified as H. cinaedi. Thus, the patient was diagnosed as having H. cinaedi bacteremia, and intravenous PIPC/TAZ was changed to intravenous sulbactuam/ampicillin (SBT/ABPC) (12 g/day) on day 9. Blood culture performed on day 6 also yielded a Gram-negative spiral bacillus after a 9-day culture. Intravenous SBT/ABPC therapy was administered for 13 days, followed by oral AMPC/CVA (1,500 mg/day) therapy for 7 days. Blood culture performed from day 12 was negative after a 14-day culture.
The fourth cycle of dose-attenuated (80%) R-CHOP chemotherapy was commenced on an inpatient basis (Fig. ). Oral minocycline hydrochloride (MINO) (100 mg/day) was prescribed from day 1 to prevent H. cinaedi bacteremia. Daily subcutaneous administration of filgrastim (75 μg/day) was performed from day 4 to day 10. This cycle could be finished without fever or skin eruptions.
The subsequent fifth and sixth cycle of dose-attenuated (80%) chemotherapy was performed. Oral MINO was continued. Subcutaneous injection of pegfilgrastim (3.6 mg) was administered. He had no fever due to H. cinaedi infection during the clinical course. Blood culture performed on day 9 of the fifth cycle of chemotherapy was negative after a 14-day culture. Since positron emission tomography-computed tomography performed during the sixth cycle of R-CHOP chemotherapy showed no abnormalities in the visceral organs, he was subsequently followed up without any treatment. No recurrence of DLBCL has been seen during the 2-year follow-up period.