Dipygus is a complete caudal duplication deformity in its severest form. The structures derived from the embryonic cloaca and notochords are duplicated to various extent. We report a male baby who presented to us with complete somatic and visceral duplication below the umbilical level associated with gastroschisis and imperforated anus. Staged surgical corrections were suggested and three out of the four stages were performed successfully.

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Objective: The aim was to study the outcome of posterior urethral valve (PUV) cases treated by stepladder protocol and the prognostic factors affecting the outcome. Materials and Methods: Hospital records of all PUV patients treated by stepladder protocol between January 1992 and December 2013 were reviewed. The studied parameters were: Age at presentation, serum creatinine, types of surgical intervention, vesicoureteral reflux (VUR) on initial voiding cystourethrogram (VCUG), renal cortical scars, plasma renin activity (PRA), and glomerular filtration rate (GFR). Results: Of 396 PUV patients treated during the study period, 152 satisfied study criteria. The age at presentation ranged from 2 days to 15 years (mean 31.3 months). The mean follow-up period was 5 years (range: 2-18 years). Primary endoscopic valve ablation was the most common initial procedure. Chronic renal failure was seen in 42.7% patients at the last follow-up. Serum creatinine at presentation, initial PRA levels, initial GFR, and PRA levels at last follow-up were significant predictors of final renal outcome. Age at presentation (<1 vs. >1 year), presence/absence of VUR on initial VCUG and renal cortical scars had no significant correlation with ultimate renal function. Conclusion: Our study confirms the high prognostic significance of initial serum creatinine, PRA levels and GFR in cases with PUV. PRA also holds promise in long-term follow-up of these patients as a marker of progressive renal damage.

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Medium chain triglyceride (MCT)-based diet, total parenteral nutrition (TPN) and repeated paracentesis are considered as supportive management for congenital chylous ascites (CCA). TPN is considered where therapy with oral MCT is poorly tolerated by the patient especially young infant with unstable hemodynamic. Surgery is recommended when medical therapy fails. Herein, we report a 2΍-month-old infant with CCA, treated successfully with octreotide intravenous infusion after the initial failure to response to conventional conservative therapy with MCT-enriched formula and paracentesis.

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We seek to provide a background of the current state of pediatric urologic surgery including a brief history, procedural outcomes, cost considerations, future directions, and the state of robotic surgery in India. Pediatric robotic urology has been shown to be safe and effective in cases ranging from pyeloplasty to bladder augmentation with continent urinary diversion. Complication rates are in line with other methods of performing the same procedures. The cost of robotic surgery continues to decrease, but setting up pediatric robotic urology programs can be costly in terms of both monetary investment and the training of robotic surgeons. The future directions of robot surgery include instrument and system refinements, augmented reality and haptics, and telesurgery. Given the large number of children in India, there is huge potential for growth of pediatric robotic urology in India. Pediatric robotic urologic surgery has been established as safe and effective, and it will be an important tool in the future of pediatric urologic surgery worldwide.

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The presentation and management of ureterocele has been rarely reported from India and is limited to an odd case report. They can be detected antenatally, may have incidental diagnosis or present with consequences of obstructive uropathy. They always present with secondary complications in adulthood, if the diagnosis and treatment is missed in early years of life. The natural history may be particularly disastrous in cases of bilateral obstructing ureterocele. The complications secondary to obstructive ureterocele can be urinary retention, pyonephrosis, urosepsis, stones and even chronic renal failure. Each of these patients needs an individualized treatment plan. In the period 2003-2013, we managed 36 consecutive patients with varied presentation of this entity. All were managed on the basis of a fixed protocol of investigation and operative intervention. Ultrasound, micturating cystourethrogram, isotope renogram were done preoperatively in all the babies. Those with asymptomatic, unobstructed ureteroceles were left without any intervention. Simple or complex intravesical ureteroceles underwent examination under anesthesia and endoscopic deroofing and DJ stenting for 3 weeks. Of a total of 36 children, 6 were presented with acute complications of ureterocele. They have been managed on an individualized optimum management plan. Their management approach with follow-up is being reported as there is no previous reported series on ureterocele in children from our country.

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Background: Idiopathic urethritis [IU] in children is of unknown etiology and treatment options are limited. We propose a classification for IU based on cystourethroscopy findings and symptoms (Grade 1 - 4) and report our experience with use of topical and oral steroids in IU. Materials and Methods: Retrospective data collection of all male children (0-16 years) diagnosed with IU over a period of 8 years between 2005 and 2012 at our institution. Data was collected on patient demographics, laboratory and radiological investigations, cystourethroscopy findings, management and outcomes. Results: A total of 19 male children were diagnosed with IU. The median age of the patients was 13(7-16) years. Presenting symptoms included dysuria in 12; hematuria in 9; loin pain in 6; and scrotal pain in 2 patients. Both patients with scrotal pain had previous left scrotal exploration that revealed epididymitis. Serum C-reactive protein and Full blood count was tested in 15 patients and was within normal limits in all of them. Cystourethroscopy revealed urethritis of grade-I in 2; grade-II in 11; and grade-III in 3 patients. There were 3 patients with systemic symptoms from extra-urethral extension of inflammation (grade-IV). Mean follow up was 18.9(1-74) months. All patients had steroid instillation at the time of cystourethroscopy. Three patients with IU grade IV required oral steroids (prednisolone) in view of exacerbation of symptoms and signs despite steroid instillation. Complete resolution of symptoms and signs occurred in 18(94.7%) patients. Significant improvement in symptoms and signs was noted in 1(5.3%) patient who is still undergoing treatment. Conclusions: IU in male children can be successfully managed with steroid instillation, especially in grade I and II. Grade III, will need steroid instillation but treatment of scarring and stricture will necessitate longer duration of treatment. In children with IU and extra-urethral symptoms (grade IV), oral steroids may be required.

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Neuropathic bladder in children is most commonly secondary to spina bifida. The management starts early in life. The modalities of treatment vary depending on the severity of the symptoms. A proportion of children inspite of adequate medical management need augmentation ileocystoplasty later in life. The open surgery has proven safety and success over many decades. Earlier attempts to perform augmentation cystoplasty by the laparoscopic approach were limited by steep learning curve, long operating times, and technical difficulties in intracorporeal anastomosis. The emergence of robotic technology has revived the interest in minimally invasive approach for complex pediatric urological reconstructions. In the recent times, there has been only one reported case report and small series of pediatric robotic augmentation cystoplasty from Chicago. We report the first minimally invasive robotic reconstruction in a child with neuropathic bladder and early renal decompensation despite appropriate medical treatment, from our country.

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Megalourethra is a rare congenital disorder of anterior urethra and erectile tissue of penis. It mainly appears in two types-a milder scaphoid type and severe fusiform type. Fusiform type is commonly associated with congenital anomalies of various systems of the body. Isolated megalourethra without other congenital anomalies is extremely rare. We report one such case which was detected postnatally and successfully treated by reduction urethroplasty.

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Aim: Our study aims to find various enzymatic and biochemical components of bile and their clinical or prognostic correlation with regard to progression and severity of hepatobiliary diseases. Materials and Methods: It was a cross-sectional study where all the patients suffering from choledochal cyst (CDC), extrahepatic portal venous obstruction (EHPVO), and infantile obstructive cholangiopathy undergoing diagnostic preoperative cholangiogram; and patients with history of total parenteral nutrition (TPN) undergoing surgery for some other condition were included in the study. Intraoperatively, bile was collected from the gallbladder and sent for estimation of amylase, lipase, sodium, potassium, calcium, chloride, bicarbonate, total bilirubin, pH, cholesterol, triglycerides, and total bile acid. Results: A total of 80 patients were included in the study (20 in each of the four disease-based groups). Amylase, lipase, and pH were significantly different among the patients of CDC when compared with the presence or absence of dilated intrahepatic biliary radicals. Similarly, amylase, lipase, and pH were also significantly different among the patients of EHPVO when compared with presence or absence of biliopathy. Levels of cholesterol and bile acid were significantly higher in patients who were evaluated after 1 year following TPN than those who were evaluated before 1 year. The patients of infantile cholangiopathy, who had history of fever, had significantly higher level of calcium. Conclusion: The components of bile show close correlation with various clinical and prognostic markers, there is a very close correlation between these parameters and the clinical severity, disease progression, and final outcome.

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Introduction: Isolated obstruction of the ureteropelvic junction and the vesico-ureteric junction are the two most common causes of hydronephrosis in a pediatric population. ^[1] They do not pose diagnostic difficulties when are present alone but when together can be difficult to diagnose. Here, we discuss the problems we faced when we encountered these two anomalies in the same ureter and the way in which we managed them. Aim: To assess the difficulties in diagnosis of pediatric patients who present with both ureteropelvic junction obstruction (UPJO) and vesico-ureteric junction obstruction (VUJO) in the ipsilateral ureter and their management protocol. Materials and Methods: This is a retrospective study. The study period is from 1 January 2004 to 31 December 2011. Out of 254 children who were diagnosed to have hydronephrosis due to UPJO in our institute, 5 patients (in the age range of 5 to 10 years) had both UPJO and VUJO in the ipsilateral ureter. The problems we faced in diagnosing the two conditions are mentioned with a literature review. Results: Operative intervention was used in four out of five patients; none of the patients had an accurate diagnosis before surgery. All patients were suspected of having double obstruction during pyeloplasty when appropriate size double J stent could not be negotiated through the vesicoureteric junction into the bladder. Postoperative nephrostogram confirmed the diagnosis in all patients. Conclusion: Children with double obstruction of the ipsilateral ureter present as a diagnostic dilemma. Because of the rarity of this condition it can escape the eye of even an astute clinician. Early diagnosis can be made if this condition is kept in mind while treating any hydronephrosis due to UPJO or UVJO.

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Aim: To report our experience of Pediatric ureterorenoscopy for ureteric calculi from two tertiary Pediatric urology centers at Apollo Children's Hospital, Chennai and Indraprastha Apollo Hospital, New Delhi. Material and methods: All children who presented with symptomatic ureteric stones greater than 6 mm were entered into the study. All children less than 12 months and more than 18 years of age and those who underwent ureterorenoscopy for indications other than the stones were excluded from the study. The children were managed on a fixed investigative and treatment protocol. The data from the Apollo Hospital New Delhi and Apollo Children's Hospital Chennai was analysed. Results: There were a total of thirty eight children, twenty in Chennai and eighteen in the New Delhi study. The mean age was 10.4 years and 8.5 years and the youngest child was14 months and 24 months in the Chennai and New Delhi group respectively. There was one conversion to open surgery in either group. Pneumatic lithotripter was used in majority of cases and holmium laser in select children. Conclusion: This is the largest Indian series of ureterorenoscopy for ureteric calculi in children. This study over nearly a decade confirms the safety and efficacy of this technique even in young children. In children less than five years, prestenting and delayed ureterorenoscopy allows safe endoscopic treatment of ureteric calculi.

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Insertion of a ventriculo-peritoneal (VP) shunt is the most common operative procedure for the treatment of hydrocephalus in children. Of the several causes of shunt malfunction, cerebrospinal fluid (CSF) pseudocyst is relatively uncommon. There are several modalities to treat a CSF pseudocyst. Laparotomy is required, at times, more than once. We managed a patient of CSF pseudocyst with two-port laparoscopy, by deroofing the psuedocyst and repositioning of the shunt. This minimally invasive technique avoids morbidity associated with laparotomy and aids in early recovery.

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We report the case of a 6-month-old infant who presented with a complete duplication of the large intestine, debuting clinically with acute abdomen and severe metabolic disorders. We discuss the pathogenesis and morphology of the lesions, diagnostic difficulties and peculiarities of surgical treatment.

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Choledochoduodenal fistula (CDF) is an unusual clinical entity and a diagnostic challenge in children. We herein present CDF as an unusual cause of recurrent cholangitis in a 6-year-old child. To the best of our knowledge, this is the youngest patient reported until date. In this paper, we also highlight possible etiologic factors, presenting symptoms, diagnostic methods, and treatment modalities of CDF.

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Introduction: Non-operative management (NOM) of splenic trauma in children is currently the treatment of choice. Purpose: We report a series of 83 cases in order to compare our results with literature data. Patients and Methods: For this, we conducted a retrospective study of 13 years and collected 83 cases of children with splenic trauma contusion, managed at Lapeyronie Montpellier Hospital in Visceral Pediatric Surgery Department. The studied parameters were age, sex, circumstances, the blood pressure (BP), hematology, imaging, associated injuries, transfusion requirements, treatment, duration of hospital stay, physical activity restriction and evolution. Results: NOM was successful in 98.7% of cases. We noted 4 complications including 3 pseudo aneurysms (PSA) of splenic artery and 1 pseudocyst spleen with a good prognosis. There was no mortality in our series. Conclusion: NOM is the treatment of choice for splenic trauma in children with a success rate of over 90%. Complications are rare and are dominated by the PSA of splenic artery.

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