Successful Laparoscopic Management of Zinner Syndrome in a Pediatric Patient.

Zinner syndrome, a rare congenital anomaly affecting males, is characterized by atresia of the ejaculatory duct, seminal vesicle cysts, and ipsilateral renal agenesis. This case report details a 2-year-old boy successfully treated with laparoscopic excision of a dilated vas deferens and seminal vesicle cyst. The rarity of Zinner syndrome in pediatric patients underscores the importance of understanding its diagnosis and minimally invasive surgical management.

Differentiating mild papilledema and buried optic nerve head drusen using spectral domain optical coherence tomography.

PURPOSE: To evaluate the clinical utility of spectral domain optical coherence tomography (SD-OCT) in differentiating mild papilledema from buried optic nerve head drusen (ONHD). DESIGN: Comparative case series. PARTICIPANTS: Sixteen eyes of 9 patients with ultrasound-proven buried ONHD, 12 eyes of 6 patients with less than or equal to Frisén grade 2 papilledema owing to idiopathic intracranial hypertension. Two normal fellow eyes of patients with buried ONHD were included. METHODS: A raster scan of the optic nerve and analysis of the retinal nerve fiber layer (RNFL) thickness was performed on each eye using SD-OCT. Eight eyes underwent enhanced depth imaging SD-OCT. Images were assessed qualitatively and quantitatively to identify differentiating features between buried ONHD and papilledema. Five clinicians trained with a tutorial and masked to the underlying diagnosis independently reviewed the SD-OCT images of each eye to determine the diagnosis. MAIN OUTCOME MEASURES: Differences in RNFL thickness in each quadrant between the 2 groups and diagnostic accuracy of 5 independent clinicians based on the SD-OCT images alone. RESULTS: We found no difference in RNFL thickness between buried ONHD and papilledema in any of the 4 quadrants. Diagnostic accuracy among the readers was low and ranged from 50% to 64%. The kappa coefficient of agreement among the readers was 0.35 (95% confidence interval, 0.19-0.54). CONCLUSIONS: We found that SD-OCT is not clinically reliable in differentiating buried ONHD and mild papilledema.

Management and Outcomes of Children with Malignant Germ Cell Tumor.

OBJECTIVES: To assess the clinico-pathological features, management and outcomes, amongst extracranial malignant germ cell tumors (MGCTs) in children treated primarily at a tertiary care center in a resource-challenged nation. METHODS: The prospectively maintained data for children below 14 y of age treated for extracranial MGCT from May 1994 to January 2023 was analyzed for patient characteristics, management, event-free survival (EFS) and overall survival (OS) and factors effecting survival. Events was defined as death, recurrence and progression. Multivariate logistic regression analysis was performed to identify the factors independently predicting unfavorable outcomes. RESULTS: One hundred and seventy-seven children (37% males) with a median (IQR) age at presentation of 30 mo (range 2-168 mo) were included. The cohort consisted of 87 (49%) extra-gonadal and 90 (51%) gonadal cases. Disease was metastatic at presentation in 48 (27%) with lungs being the most common site. Neoadjuvant chemotherapy (NACT) was given to 119 (67%) and finally 162/177 (92%) had undergone resection of the primary tumor. Endodermal sinus tumor (EST) was the commonest histological subtype in 141 children (73%). Twenty-two (12%) patients had died giving a 5-y OS of 84.7% (95% CI 78.3- 91.1). Recurrence occurred in 25 patients, and an additional 5 patients had progression giving a 5-y EFS of 69.9% (95% CI 62.5- 77.3). Stage III (p = 0.05), Stage IV (p = 0.006) and extra-gonadal site (p = 0.05) were significantly associated with poorer EFS. CONCLUSIONS: Children with MGCT have a favorable outcome with 5-y OS of 84.7% and EFS of 69.9%. Stage III and IV disease and extra-gonadal sites were independent predictors of a poor outcome.

Untreated bilateral optic nerve sheath meningiomas observed for 27 years.

A 48-year-old woman noted progressive, painless visual loss in her left eye. She was diagnosed with bilateral optic nerve sheath meningiomas (ONSMs), extending across the planum sphenoidale. Radiation was offered, but the patient declined. She has been followed for more than 27 years with stable visual function and neuroimaging findings. Bilateral ONSMs, although usually described as aggressive in nature, may follow a stable clinical course.

Primary dural lymphoma masquerading as a meningioma.

A 39-year-old woman noted progressive blurred vision in the right eye for 1 year. The right eye had visual acuity of 20/25, an afferent pupillary defect, pale optic nerve, and cecocentral scotoma. Magnetic resonance imaging findings were consistent with en plaque meningioma of the planum sphenoidale, which encircled the right optic nerve at the optic canal. The tumor was internally debulked to preserve the optic nerve. Histopathologic and molecular analysis revealed a low-grade B-cell lymphoma. Further evaluation showed no evidence of systemic disease. Primary dural lymphomas are a distinct entity that may mimic meningioma and cause vision loss.

Bilateral oculomotor synkinesis following Miller Fisher syndrome.

A 64-year-old female presented with ptosis, ophthalmoplegia, ataxia, and weakness. She was diagnosed with Miller Fisher syndrome (MFS) and was treated with plasmapheresis. Six months later, she developed bilateral oculomotor synkinesis, suggesting aberrant regeneration. The pathogenesis of MFS is reviewed in light of this unusual finding.

Stapled vs Conventional Hand-sewn Gastrointestinal Anastomosis during Infancy: A Prospective Comparative Study from Central India.

BACKGROUND: The creation of a joint between two bowel ends in newborns and infants is one of the core surgical procedures in pediatric surgery. For a proper and perfect gastrointestinal (GI) anastomosis, the factors to be considered are intraoperative duration, restoration of normal GI function, effective hemostasis, reduction of tissue damage, and prevention of postoperative mortality and morbidity. The safety and efficacy of stapled GI tract anastomosis in adults have been extensively documented; however, available literature on the same is limited for infants. MATERIALS AND METHODS: Fifty-six patients were divided into two groups-stapled group and hand-sewn group. Patients operated on both emergency and elective basis were included in the study. Hand-sewn anastomosis was done by either end-to-end single-layer or double-layer anastomosis. Suture material used for the anastomosis was Vicryl 3-0 or Vicryl 4-0. Stapled anastomosis was done by 55 mm linear cutting GI stapler with side-to-side anastomosis. RESULTS: The present study included a total of 56 patients; there were 28 neonates and 28 infants; 37 of them were males. The most common clinical presentations were vomiting, abdominal distention, refusal to feed, and lethargy. The intraoperative duration in stapled GI anastomosis was less when compared to hand-sewn anastomosis, so was the return of bowel activity and consequently early initiation of feeds and shorter hospital stay. CONCLUSION: The present study favors stapled over hand-sewn GI anastomosis in infancy in view of decreased intraoperative duration, reduced blood loss, early return of peristalsis, early initiation of feeds, and shorter duration of hospital stay. However, a small number of patients and lack of matching are the shortcomings of this study. HOW TO CITE THIS ARTICLE: Mitra AS, Chandak U, Kulkarni KK, et al. Stapled vs Conventional Hand-sewn Gastrointestinal Anastomosis during Infancy: A Prospective Comparative Study from Central India. Euroasian J Hepato-Gastroenterol 2020;10(1):11-15.

Intravitreal triamcinolone acetonide preceding photodynamic therapy for exudative age-related macular degeneration.

BACKGROUND AND OBJECTIVE: To evaluate the efficacy of intravitreal triamcinolone acetonide 1 week before photodynamic therapy (PDT) with verteporfin in the treatment of exudative age-related macular degeneration (AMD). PATIENTS AND METHODS: Retrospective chart review of 70 eyes of 66 consecutive patients who were treated with intravitreal triamcinolone acetonide followed by standard PDT 1 week later. Group 1 consisted of 42 eyes that received one or more previous treatments with PDT alone and responded poorly. Group 2 consisted of 28 eyes that had never received PDT. RESULTS: No statistically significant change in mean visual acuity was seen during the follow-up period for either group. At 12 months, 90% of group 1 eyes and 80% of group 2 eyes lost less than 2 lines of Snellen visual acuity. A mean of 1.24 additional PDT sessions were required in group 1 eyes and 1.04 additional sessions in group 2 eyes during the follow-up period. CONCLUSION: In eyes with exudative AMD, intravitreal triamcinolone acetonide preceding PDT with verteporfin results in stabilization of visual acuity and a decreased need for re-treatments with PDT. This regimen may be superior to PDT monotherapy.

The temporal sequence of combined intravitreal triamcinolone acetonide and photodynamic therapy for exudative age-related macular degeneration.

BACKGROUND AND OBJECTIVE: To compare visual acuity results in patients with exudative age-related macular degeneration treated with two different temporal sequences of combination intravitreal triamcinolone acetonide and photodynamic therapy with verteporfin. PATIENTS AND METHODS: A retrospective, comparative, interventional case series was used. Thirty-one eyes received intravitreal triamcinolone acetonide 1 week prior to photodynamic therapy, and 30 eyes received intravitreal triamcinolone acetonide followed by photodynamic therapy the same day. RESULTS: There was no significant difference in visual acuity between the groups at baseline (P = .084), 6 to 12 weeks of follow-up (P = .085), or 1 year of follow-up (P= .093). When visual acuity outcomes were adjusted for baseline visual acuity, spot size, lesion type, age, and gender, there was no significant difference in visual acuity at 6 to 12 weeks (P = .44) or 1 year (P= .28). CONCLUSIONS: There appears to be no significant difference in visual outcomes in eyes with exudative age-related macular degeneration treated with intravitreal triamcinolone acetonide 1 week prior to photodynamic therapy or those treated with intravitreal triamcinolone acetonide on the same day as photodynamic therapy.

Ab externo technique for accurate haptic placement of transscleral sutured posterior chamber intraocular lenses.

A modified technique is presented for transscleral suture fixation of posterior chamber intraocular lenses for aphakic correction in cases with inadequate capsular support. An ab externo approach for suture externalization is described that minimizes intraocular manipulation and facilitates accurate placement of haptics in the ciliary sulcus, thereby reducing the risk of lens tilt and decentration. This technique eliminates intraocular suture knots and reduces the amount of time the globe is open during surgery by creating two intraocular suture loops in preparation for posterior chamber intraocular lens placement before creating a limbal incision.

Visual field staging systems in glaucoma and the activities of daily living.

PURPOSE: To compare 8 clinically relevant methods of staging visual field (VF) damage in glaucoma with a performance-based measure of the activities of daily living and self-reported quality of life. DESIGN: Prospective cross-sectional study. METHODS: One hundred ninety-two patients with various types of glaucoma were evaluated at the Wills Eye Institute using standard monocular and binocular VF testing, as well as an objective, performance-based measure of visual function (the Assessment of Disability Related to Vision), and a subjective, standardized measure of quality of life (the 25-item National Eye Institute Visual Function Questionnaire). Binocular VFs were scored according to the Esterman and Integrated VF Systems. Monocular VFs were scored according to the mean defect, pattern standard deviation, Hodapp-Parrish-Anderson method, glaucoma staging system, glaucoma staging system 2, and the field damage likelihood scale. Partial Spearman correlations between VF staging systems, Assessment of Disability Related to Vision scores, and 25-item National Eye Institute Visual Function Questionnaire scores were calculated. RESULTS: Assessment of Disability Related to Vision scores and 25-item National Eye Institute Visual Function Questionnaire scores were associated most closely with the VF score in the better eye and the binocular VF scoring systems. CONCLUSIONS: The amount of binocular VF loss and the status of the better eye most accurately predict functional ability and quality of life in glaucoma.

A new method of assessing ability to perform activities of daily living: design, methods and baseline data.

PURPOSE: To describe study protocol and baseline characteristics of initial subjects. METHODS: Prospective study of 194 glaucoma patients. After informed consent, patients were evaluated using a third-generation, performance-based measure, Assessment of Disability Related to Vision (ADREV), nine tasks to simulate daily living activities; including: reading in reduced illumination, recognizing facial expression, detecting motion, recognizing street signs, locating objects, ambulating, placing pegs into different sized holes, telephoning, and matching socks. All patients completed clinical tests including visual acuity (Early Treatment Diabetic Retinopathy Study [ETDRS]), monocular and binocular visual fields, contrast sensitivity, stereopsis, and National Eye Institute's Visual Functioning Questionnaire (NEI VFQ-25). Main outcome measures are scores on subtests, total scores of ADREV and NEI VFQ-25. RESULTS: Mean age was 67.1 years; 51% were female, 55% were of European extraction. Seventy-two percent had open-angle glaucoma. Visual acuity (logMAR) ranged from -0.18 to 2.20, visual field mean defect ranged from -32.68 to 2.62, Binocular field score ranged from 5 to 100 and contrast sensitivity score ranged from 0 to 1.8. While ADREV score ranges from 0 (total disability) to 63 (no disability), total ADREV score ranged from 3.0 to 61.7. Total NEI VFQ-25 score ranged from 17.8 (low score indicates incapable) to 100 (high score indicates not hindered). CONCLUSIONS: Methodology of this study should provide information about disability caused by visual loss, specifically glaucoma, how difficulties in performing activities relate to standard clinical tests, and how they correlate to quality of life from patient's point of view. Subsequent papers will delineate findings and discuss their application.

Current visual and anatomic outcomes of pneumatic retinopexy.

PURPOSE: To assess current visual and anatomic outcomes of pneumatic retinopexy (PR) for the repair of rhegmatogenous retinal detachment (RD). METHODS: Retrospective chart review of 150 patients who underwent PR for RD between January 2000 and February 2005. Patients with a history of scleral buckling, Lincoff balloon, or proliferative diabetic retinopathy in the same eye were excluded. RESULTS: Successful reattachment was achieved in 76.7% with a single procedure and 98.7% after additional procedures. A total of 17.3% underwent a subsequent procedure for a new or persistent tear. The success rate remained consistent in pseudophakic and aphakic eyes, and eyes with traumatic detachments, macular detachment, multiple breaks, poor preoperative visual acuity (VA) (<20/50), superior and inferior lattice degeneration, >or=6 clock hours of detachment, and prior pars plana vitrectomy. A total of 2.7% of eyes underwent a second additional procedure for RD. A total of 32% of males versus 15.3% of females underwent an additional procedure for RD (P = 0.07). Final VA >or=20/50 was achieved in 80.8% of macula-on and 73.6% of macula-off RDs. Of eyes that underwent an additional procedure, final VA >or=20/50 was achieved in 63.2% of macula-on and 43.8% of macula-off RDs, and those with macula-on RD lost a mean of 1.79 (+/-4.9) lines of Snellen VA, while those with macula-off RD gained a mean of 5.6 (+/-4.6) lines (P = 0.00006). CONCLUSIONS: : PR had a high anatomic success rate in eyes with a wide variety of complicated RDs. Male sex may be a risk factor for failed PR. Careful monitoring and treatment of postoperative tears is important in preventing redetachment.

A novel NAD-binding protein revealed by the crystal structure of 2,3-diketo-L-gulonate reductase (YiaK).

Escherichia coli YiaK catalyzes the reduction of 2,3-diketo-L-gulonate in the presence of NADH. It belongs to a large family of oxidoreductases that is conserved in archaea, bacteria, and eukaryotes but shows no sequence homology to other proteins. We report here the crystal structures at up to 2.0-A resolution of YiaK alone and in complex with NAD-tartrate. YiaK has a new polypeptide backbone fold and a novel mode of recognizing the NAD cofactor. In addition, NAD is bound in an unusual conformation, at the interface of a dimer of the enzyme. The crystallographic analysis unexpectedly revealed the binding of tartrate in the active site. Enzyme kinetics studies confirm that tartrate and the related D-malate are inhibitors of YiaK. In contrast to most other enzymes where substrate binding produces a more closed conformation, the binding of NAD-tartrate to YiaK produces a more open active site. The free enzyme conformation is incompatible with NAD binding. His(44) is likely the catalytic residue of the enzyme.