Right Phrenic Nerve Palsy following Long-gap Esophageal Atresia and Tracheoesophageal Fistula Repair.

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are surgically correctable congenital anomalies with reported surgical common complications such as anastomotic leaks, recurrent TEF, and esophageal strictures; however, phrenic nerve injury (PNI) is a very rare but possible complication which we have highlighted in our case report. Here, we report a baby girl operated for long-gap EA and TEF having respiratory distress and failed attempts to wean off oxygen support. Serial chest X-rays showed elevated right hemidiaphragm, whereas ultrasound thorax confirmed our diagnosis of right PNI causing diaphragmatic palsy. Conservative management with the hope of spontaneous recovery failed, so diaphragmatic plication was done at 5 weeks from index surgery. Postplication, the baby was weaned off oxygen and pressure support the very 1st day and had improved respiratory physiology.

Bilateral Synchronous Robotic-Assisted Adrenalectomies in a Patient of Bilateral Pheochromocytoma and Von Hippel-Lindau Disease - A Rewarding Approach.

Hypertensive crisis is rare in children. Among the rarest causes leading to this acute crisis, is bilateral adrenal tumour as a part of a syndrome, Von Hippel Lindau syndrome. The treatment is based on the excision of the adrenal tumour followed by long term surviellence. The authors present a case where conventional imaging with ultrasound and contrast enhanced CT scan demonstrated a right side adrenal tumour with raised catecholamines. The small left side tumour got missed on conventional imaging and got picked up on DOTA scan. The persistence of hypertension in post operative period can be related to such hidden functioning tumours. In view of the size of tumour & raised dopamine associated with high incidence of malignancy, robotic surgery was used for bilateral adrenal tumour excision which confirmed the diagnosis of Pheochromocytoma (PCC).

Frozen Section Biopsy and Nephron-Sparing Surgery for a Multiloculated Cyst of the Kidney; Is this the Ideal Approach?

Multiloculated cystic renal masses are uncommon in the pediatric population. The presentation may be as an asymptomatic incidental finding on imaging, abdominal mass, abdominal pain, or urinary tract infection. The differentiation between benign and malignant causes of a cystic lesion by clinical and radiological examination is difficult. Tru-cut biopsy is not recommended due to fear of upgrading a malignant tumor. A definitive diagnosis is confirmed histopathologically only after surgery. Based on certain imaging characteristics, benign nature can be suspected and a conservative approach to surgery can be contemplated to save the kidney. Frozen section biopsy is useful in ruling out malignancy while doing nephron-sparing surgery (NSS) in these patients. NSS may be done by an open or minimally invasive approach. After histological confirmation of cystic nephroma, no other adjuvant treatment is necessary, but long-term surveillance is strongly recommended.

Minimally Invasive Management for Vesicoureteral Reflux in Infants and Young Children.

Minimally invasive ureteral reimplantation is an attractive and useful tool in the armamentarium for the management of complicated vesicoureteral reflux (VUR). Subureteric dextranomer/hyaluronic acid injection, laparoscopic extravesical ureteric reimplantation and pneumovesicoscopic intravesical ureteral reimplantation with or without robotic assistance are established minimally invasive approaches to management of VUR. The high cost and the limited availability of robotics have restricted accessibility to these approaches. Laparoscopic and/or robotic ureteral reimplantation continues to evolve and will have a significant bearing on the management of complicated VUR in infants and young children.

Ureterocele in newborns, infants and children: Ten year prospective study with primary endoscopic deroofing and double J (DJ) stenting.

AIM: To evaluate the success of endoscopic deroofing with double J (DJ) stenting as a primary treatment modality of uncomplicated ureterocele. PATIENTS AND METHODS: All babies with uncomplicated ureterocele referred to us between 2005 and 2015 were to be prospectively recruited into the study without any exception. The preoperative management would include ultrasound KUB on a fixed protocol, micturating cystourethrogram, DTPA scan and MRU (magnetic resonance urography) or IVP (intravenous pyelography). Those who had non-obstructing ureterocele were to be followed conservatively. The indications for operative intervention were to be obstructed ureterocele, bladder outlet obstruction, recurrent UTI or progressive renal scarring. They were then to be taken for cystoscopy in diuretic phase for endoscopic deroofing and DJ stenting with 9F resectoscope. Four weeks later, the DJ stent was to be removed after examining the deroofing in each patient. They were then followed up for future development of symptoms and need for secondary surgery with anatomical and functional imaging as necessary. RESULTS: Forty three consecutive patients (47 ureterocele units) with ureterocele were recruited into the study between 2005 and 2015. Thirty four patients were managed by endoscopic first approach with ureterocele deroofing and DJ stenting. Four babies were asymptomatic and did not show obstructed drainage pattern on DTPA and hence were managed conservatively and kept on follow-up. Four patients underwent primary open surgery and reconstruction because of presentation with complications. ECTOPIC URETEROCELE: There were 17 patients with ectopic ureterocele. Thirteen underwent primary endoscopic deroofing. Three underwent open surgery and one underwent robotic bilateral ureterocele excision and ureteric reimplantation. Eight children out of thirteen are completely asymptomatic after the endoscopic incision. On ultrasound evaluation 8/13 have resolution of the hydronephrosis and 5/13 have stable hydronephrosis with no increase in dilatation. None of these 13 children have required a second endoscopic procedure for inadequate decompression of the ureterocele. None of the 13 patients have any voiding difficulty or incontinence. 2/13 cases with recurrent UTI and persistent symptoms, required secondary surgery. This constituted 15% secondary surgery in the ectopic ureterocele category. ORTHOTOPIC URETEROCELE: There were 21 patients out of 22 with orthotopic ureterocele who underwent endoscopic deroofing. Six out of these 21 patients had episodes of UTI, of which three had resolution of the reflux and two had downgrading of reflux. One child continued to have recurrent UTI and needed a laparoscopic nephroureterectomy. Eighteen children are completely asymptomatic after the initial endoscopic deroofing. The rate of secondary surgery was 4.7% in orthotopic ureterocele group, where nearly 50% have more than five years follow-up. None of our patients who are more than five years, have had any urinary incontinence or voiding dysfunction. All patients remain on follow-up from 6months to 10years (median 5years). CONCLUSION: At this stage of our study with median follow-up of 5years (6months-10years), we suggest that primary endoscopic deroofing with DJ stenting for obstructing ureterocele is the best initial approach for adequate decompression and reducing the rate of secondary surgery.

Unusual postoperative complication of minimally invasive transhiatal esophagectomy and esophageal substitution for absolute dysphagia in a child with corrosive esophageal stricture.

Esophageal substitution in children is a rare and challenging surgery. The minimally invasive approach for esophageal substitution is novel and reported from a few centers worldwide. While detailed report on the various complications of this approach has been discussed in adult literature, the pediatric experience is rather limited. We report the laparoscopic management of a rare complication which developed after laparoscopic esophagectomy and esophageal substitution. The timely recognition and management by the minimally invasive approach have been highlighted.

Endoscopic esophageal substitution for pure esophageal atresia and wide gap esophageal atresia: A report of five cases with minimum follow-up of twelve months.

AIM: The aim of the study is to report feasibility and safety of endoscopic esophageal substitution in infants with pure esophageal atresia and wide gap tracheoesophageal fistula with a minimum one year follow-up. MATERIALS AND METHODS: This prospective study was conducted from January 2012 for twenty four consecutive months at Apollo Hospital, New Delhi. All babies either followed up or referred for esophageal substitution without any history of mediastinitis or associated major congenital anomaly and weighing greater than 6kg were to be included in the study. The indication, intraoperative details, operative approach, conversion to open, esophageal substitute, postoperative ventilation, ICU and hospital stay, time to solid foods, morbidity and mortality were recorded. Informed consent was obtained from all the parents and ethical clearance was obtained for the study from the hospital ethical committee. Postoperatively babies were followed up monthly for first six months, 3 monthly for next six months and annually thereafter. RESULTS: Between January 2012 and December 2013, in the two year period six infants were admitted for laparoscopic gastric transposition. In five patients the procedure was completed by the laparoscopic approach and one required conversion to open surgery owing to dense adhesions. The age range at the time of surgery was from 8months to 12months with a mean age of 10months. Four patients had pure esophageal atresia (type A) and two had wide gap esophageal atresia with distal tracheoesophageal atresia (type C). Five had primary esophagostomy and gastrostomy as a newborn, the sixth had postoperative anastomotic leak and required subsequent diversion. The mean operating time was 194minutes (range 170-210minutes). The mean stay in ICU was 7days with a range of 4-12days. All patients were ventilated in the postoperative period for an average of 5days with a range of 4-7days. One patient had prolonged gastric ileus which delayed the oral feeds by 14days. The mean time to start the oral feeds was 8days with a range of 6-14days. The mean hospital stay was 19.6days (range 16-23days). Early complications were pneumonia and pleural effusion in one patient. One patient developed anastomotic stricture which was amenable to dilatation. One patient had leak from esophagogastric anastomosis which healed spontaneously. All children are now orally fed, swallow without difficulty, and parents report an excellent cosmetic outcome. The follow-up ranges from 12 to 36months. CONCLUSION: The initial results of endoscopic esophageal substitution are encouraging and easily comparable to the outcome of open surgery with all the attendant benefits of minimally invasive approach.

Rigid ureteroscopy in children: Our experience.

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.

Management of complicated ureteroceles: Different modalities of treatment and long-term outcome.

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.

Robotic augmentation ileocystoplasty with bilateral ureteric reimplantation in a young child with neuropathic bladder.

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.

Indiana pouch in children: a 15-year experience.

OBJECTIVE: We report our experience with the Indiana pouch (continent urinary reservoir) in 12 consecutive children over the last 15 years and report their follow-up. MATERIAL AND METHODS: Twelve consecutive children, who underwent the continent urinary reservoir procedure in the form of an Indiana pouch, were prospectively enrolled in the study. All consecutive children who were referred to our service with multiple failed surgeries for exstrophy-epispadias repair, cloacal exstrophy, genitourinary rhabdomyosarcoma with residual disease in the trigonal area not amenable to partial cystectomy, and neuropathic bladder with nephrogenic metaplasia were included over the period 1997-2012. All these children were offered the same form of bladder substitution (Indiana pouch) as described by the Indiana group many years ago. Postoperative care was on a fixed protocol, and follow-up details recorded over the years. They were followed up for dry interval with clean intermittent catheterisation, social acceptance, and early and late complications. RESULTS: Out of these 12 patients (7 males and 5 females), eight patients had exstrophy-epispadias with multiple failed operations carried out elsewhere, cloacal exstrophy (2), residual rhabdomyosarcoma in the trigonal area with incontinence following chemotherapy (1), and neuropathic bladder with recurrent diffuse neoplastic polyposis (1). In the follow-up period of 1-15 years (median 24 months) all the patients had a dry interval of 4 h or more with clean intermittent catheterisation. One patient had wound dehiscence during the postoperative period and another required stomal revision 1 year after initial surgery. CONCLUSIONS: The Indiana pouch is a reliable, safe, and effective form of bladder substitution. It can be reconstructed in a wide range of lower urinary tract disorders. In the vast majority of children with multiple failed surgical procedures for exstrophy-epispadias, the Indiana pouch is a safe, reliable, and reproducible procedure to provide a socially acceptable quality of life with a dry interval of 4 h.

Mitra shunt (spleen preserving, side to side lieno-renal shunt) for portal hypertension with hypersplenism in early infancy.

Extrahepatic portal vein obstruction (EHPVO) is the commonest cause of portal hypertension presenting with gastrointestinal bleeding and splenomegaly. Medical management of this condition may provide relief, but involves repeated hospital visits and endoscopic procedures. Surgery is an effective curative solution by lowering portal venous pressure with effective shunting of venous blood from splanchnic to systemic circulation. Shunt surgery for such a small baby has not been previously reported and splenectomy has its own problems. Similarly, banding or sclerotherapy in such babies is not without risk. Among the various shunt options, Mitra shunt (spleen preserving, side-to-side lienorenal shunt), developed and standardized in our own country, stands out as the most optimum surgical treatment for EHPVO in early infancy. We report a 4-month-old baby, youngest recipient of Mitra shunt reported in literature with successful outcome.

Intestinal obstruction in a premature baby: Endoscopic diagnosis and management by minimal access surgery.

Neonatal intestinal obstruction is the most common surgical emergency in a newborn. Although, large numbers of newborns are operated in our country, limited published literature is available on advances in diagnosis, and management of this problem with outcome analysis in newborns. We report a premature (32 weeks) newborn who developed acute onset symptoms of small bowel obstruction in 3(rd)week of life, and discuss the approach to diagnosis and management with the minimal access surgery and successful outcome.

Laparoscopic and thoracoscopic gastric pull-up for pure esophageal atresia in early infancy.

In the developing countries, the babies with pure esophageal atresia undergo an esophagostomy and feeding gastrostomy at birth. It assists in early discharge from hospital. Esophageal substitution in these babies around six months is recommended. We report the first laparoscopic and thoracoscopic gastric pull up in early infancy from India.

Wilms tumor: five-year tumor-free survival on a modified SIOP protocol from an Indian university hospital.

BACKGROUND AND PURPOSE: Wilms tumor is one of the most common solid malignancies of childhood. The long-term result of the surgical treatment of this tumor with adjuvant chemotherapy and radiation has been reported from many western centers. However, there is paucity of literature on the long-term outcome of treatment for this tumor from the developing world. We planned a prospective study that started in 1999 to evaluate the 5-year tumor-free survival of Wilms tumor treated on a fixed protocol from a university hospital in India. METHODS: All consecutive children with Wilms tumor who were seen by any of the 3 investigators (2 pediatric oncologists, 1 pediatric surgeon) between 1999 and 2003 at the Advanced Pediatric Centre were included in the study. Each patient was entered into a database. Their demographic profile and events during the treatment and follow-up were recorded. This was later analyzed. CONCLUSIONS: Seventy-five percent 5-year tumor-free survival has been achieved within the limitations of treatment in the developing world. The morbidity during therapy was 10% and the mortality 5%. This was based on the treatment of this common childhood malignancy at Chandigarh on a modified International Society of Pediatric Oncology protocol.

Cholecystoappendicostomy for progressive familial intrahepatic cholestasis.

We report a rare case of progressive familial intrahepatic cholestasis type 2 from India. The diagnosis was confirmed on the basis of gene mutation analysis. The child had intense pruritus refractory to conventional medical management. As liver biopsy did not reveal any cirrhosis, partial external biliary diversion was considered as an alternative to liver transplant. We performed cholecystoappendicostomy rather than the conventional method of using an ileal loop as a conduit between the gall bladder and abdominal wall. Child recovered completely.