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Background: There is an ongoing pursuit to decrease complications of hypospadias surgery. We studied a modification to the double onlay preputial island flap, whereby an additional tunica vaginalis layer was used. Aims: We aim to study the efficacy, complication rates and outcomes of the modified double onlay urethroplasty technique. Materials and Methods: This study adopts an observational consecutive study design. From 2014 to 2019 the modified preputial double onlay urethroplasty technique was used in 30 patients. Selection criteria was severe hypospadias operated on using the technique studied in a single stage procedure. We excluded perineal hypospadias, cripple hypospadias, and combined procedures. The operative technique used involved a layer of preputial flap for the neourethra followed by a layer of tunica vaginalis followed by another preputial flap layer over the ventral penile skin defect. Results: Patients were followed up for a mean of 2.04 years. There were 11 (36.67%) complications. Urethrocutaneous fistulas occurred in 3 patients (10%). There were 3 cases (10%) of glanular dehiscence and 1 (3.3%) diverticulum. No correlation was found between operative age, hypospadias type, or chordee approach and complications. Short second surgeries were needed in 7 patients (23.3%). Conclusion: The modified technique used in this study yielded good cosmetic and functional results. Complication rates were in line with the lower ranges reported by the literature for the standard double onlay preputial flap technique.

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Surgical options for coarctation of aorta (CoA) with atrioventricular septal defect (AVSD) include single-stage repair vs. staged approach with neonatal CoA repair and delayed AVSD repair. The durability of left atrioventricular valve (LAVV) function after neonatal repair is questioned, and the optimal approach remains controversial. Eighteen CoA-AVSD patients who underwent single-stage repair 2005-2015 by a single surgeon were retrospectively analyzed. Fifteen patients had complete and three had partial AVSD. Birth weight was 3.19 kg (2.17-4.08). Age at surgery was 16 days (6-127). One- and ten-year survival were 80% and 69%. Freedom from reintervention was 60% and 40% at one and ten-year respectively. Reinterventions included relief of left ventricular outflow tract obstruction (LVOTO) (n = 4), repair of cleft LAVV (n = 3), and LAVV and aortic valve replacement (n = 1). Freedom from LAVV reintervention was 85.6% and 66% at 1 and 10 years respectively. There were four deaths: two post-operative and two following hospital discharge. Mortality was due to sepsis in three patients, and heart failure related to LVOTO and LAVV insufficiency in one. At 68-month (0.6-144) follow-up the majority had mild or less LAVV regurgitation, and all had normal LV dimension and systolic function. There was no recurrent arch obstruction. Single-stage surgical repair of CoA-AVSD is feasible and reasonable. Survival and freedom from reintervention in our cohort approximate those outcomes of two-stage repair with durable left AV valve function and no recurrent arch obstruction. These patients are frequently syndromic and demonstrate mortality risk from non-cardiac causes. Consideration of a single-staged approach is warranted for appropriate patients with CoA-AVSD.

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INTRODUCTION AND OBJECTIVE: The objective of the study was to evaluate the functional and cosmetic outcome of single-stage modified partial penile disassembly repair in isolated male epispadias. MATERIALS AND METHODS: A retrospective analysis of 15 cases of primary epispadias repair, from June 2015 to December 2018, was performed. Patients were classified by the type of epispadias, urinary incontinence, chordee, and rotation. SURGICAL TECHNIQUE: Penile degloving with the mobilization of the urethral plate from the ventral to the dorsal aspect with the preservation of blood supply at both ends, distally up to the level of mid-glans and proximally up to the pubic symphysis is done. Tubularization of urethral plate followed by spongioplasty, corporoplasty with medial rotation of corporeal bodies, and glanuloplasty with meatoplasty was done to bring the meatus ventrally. The skin cover is done by the rotation of the ventral flaps and the z-plasty whenever required. RESULTS: Age of the patients varied from 4 months to 21 years with a mean of 11 years. Thirteen patients had excellent cosmetic outcome while two patients had minimal residual chordee but did not require any surgery in a follow-up. Five patients with partial incontinence in the study group achieved continence after surgery. None of the patients developed complications such as fistula or stricture. All five male patients in the post-pubertal group reported normal erections and successful ejaculations after the surgery. Follow-up ranged from 3 months to 18 months. CONCLUSIONS: Modified partial penile disassembly incorporates all the benefits of Cantwell Ransley repair and needs less extensive dissection than total penile disassembly. Both functional and cosmetic results are good with a low complication rate.

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We present an unusual case of atrial septal defect and ventricular septal defect with a vascular ring formed by a right-sided aortic arch with an aberrant left subclavian artery that gave rise to a patent ductus arteriosus connecting to the main pulmonary artery. We performed a single-stage repair of the intracardiac defects and division of vascular ring with a sternotomy instead of the traditionally practiced dual approach. This included division and reimplantation of the aberrant left subclavian artery to the left carotid artery after transection. This approach has not been described so far.

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OBJECTIVE: To evaluate the long-term outcomes of hypospadias repair using an onlay preputial graft. PATIENTS AND METHODS: Patient records from 1989 to 2013 were retrospectively reviewed. One surgeon performed all cases and surgical technique was the same for all patients. RESULTS: There were 62 patients in the cohort, with a mean (range) follow-up of 47.4 (1-185) months. The meatal location was separated into distal (one patient), midshaft (19) and proximal (42). In all, 22 (35.5%) patients had complications. There were three main types of complications, including meatal stenosis in three (4.8%), stricture in three (4.8%), and fistula in 21 (33.9%). The mean (range) timing of presentation with a complication after surgery was 24.9 (1-127) months. In all, 54.5% of the patients with complications presented at ≥1 year after the initial surgery and 31.8% presented at ≥3 years. On univariable analysis age at the time of surgery, length of the graft, presence of chordee or meatal location (proximal or midshaft) did not predict a complication. The width of the graft was associated with a complication, with each 1 mm increase in width decreasing the odds of a complication by 56%. On multivariable analysis width remained statistically significant (odds ratio 0.44, 95% confidence interval 0.230-0.840; P = 0.013) for predicting a complication. CONCLUSION: Hypospadias repair with onlay preputial graft is an option for single-stage repair, especially in cases of proximal hypospadias or where the urethral plate width and/or the glanular groove is insufficient for other types of repair. Compared with flaps, the use of grafts may decrease the risk of penile torsion and prevent less bulk around the urethra, improving skin and glans closure.

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PURPOSE: The aim of the study is to assess the characteristics and outcome of anorectal malformation (ARM) patients who underwent single-stage repair of perineal fistula without colostomy according to the Krickenbeck classification. METHODS: From 2002 to 2013, twenty-eight males and four females with perineal fistula who underwent single-stage repair without colostomy in our institute were included in this study. Patients with perineal fistula who underwent staged repair were excluded. Demographics, associated anomalies, and operative complications were recorded. The type of surgical procedures and functional outcome were assessed using the Krickenbeck classification. RESULTS: Six patients had associated anomalies, including two patients with renal, two with cardiac, one with vertebral, and one with limb abnormalities. Thirteen patients underwent perineal operation, and fourteen patients underwent anterior sagittal approach in the neonatal period. One patient underwent anterior sagittal approach, and four patients underwent PSARP beyond the neonatal period. One patient had an intra-operative urethral injury and one a vaginal injury. Complications were not associated with the type of surgical procedure (p=0.345). All perineal wounds healed without infection. By using the Krickenbeck assessment score, all sixteen children older than five years of age had voluntary control. One patient had grade 1 soiling, and no patient had constipation. CONCLUSIONS: Single-stage operation without colostomy was safe with good outcomes in patients with perineal fistula. The use of Krickenbeck classification allows standardization in assessment on the surgical approach and on functional outcome in ARM patients.

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We report a 27 years old male who presented with a combination of both congenital and acquired cardiac defects. This syndrome complex includes congenital bicuspid aortic valve, Seller's grade II aortic regurgitation, juxta- subclavian coarctation, stenosis of ostium of left subclavian artery and ruptured sinus of Valsalva aneurysm without any evidence of infective endocarditis. This type of constellation is extremely rare. Neither coarctation of aorta with left subclavian artery stenosis nor the rupture of sinus Valsalva had a favorable pathology for percutaneus intervention. Taking account into morbidity associated with repeated surgery and anesthesia patient underwent a single stage surgical repair of both the defects by two surgical incisions. The approaches include median sternotomy for rupture of sinus of Valsalva and lateral thoracotomy for coarctation with left subclavian artery stenosis. The surgery was uneventful. After three months follow up echocardiography showed mild residual gradient across the repaired coarctation segment, mild aortic regurgitation and no residual left to right shunt. This patient is under follow up. This is an extremely rare case of single stage successful repair of coarctation and rupture of sinus of Valsalva associated with congenital bicuspid aortic valve.

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To evaluate the efficacy of single stage repair of hypospadias, the results of 76 patients were compared to those of 45 patients who had staged repair during the period of the last 22 years. Cases of single stage repair were tremendously increased in recent years (13 patients before 1987, 63 patients after 1987). The success rate was much better than that of staged repair (single stage: 63.1 %, staged: 28.9 %). Among 45 patients who had staged repair. 17 patients (53.1 %) underwent multiple surgical corrections due to troublesome stricture, fistula and remnant chordee, and 9 patients (20 %) required additional meatal advancement due to coronal or subcoronal neomeatus. In conclusion, single stage hypospadias repair could be applicable in most cases of hypospadias and the results were superior to those of staged repair. The complications and morbidity were minimal.

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