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).

Nephron-sparing Surgery for Syndromic Wilms' Tumor: Robotic Approach.

The current standard treatment for stage I Wilms' tumor is open radical nephrectomy. Patients with WAGR syndrome and Wilms' tumor have risk of contralateral tumor and are a group of patients benefitted by nephron-sparing surgery (NSS). Whereas laparoscopic NSS has been attempted in such patients, due to the inherent technical limitations it has failed to gain popularity. Robotic approach for NSS overcomes limitations of movement and dexterity occurring with laparoscopic approach. However, the existing literature in robotic NSS in children is very limited. We present the first report of robotic approach for NSS in a child with WAGR syndrome.

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.

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.

Single-stage repair of vestibular and perineal fistulae without colostomy.

PURPOSE: This retrospective study was undertaken to evaluate the feasibility of primary anorectoplasty without a covering colostomy using the anterior sagittal anorectoplasty (ASARP) or posterior sagittal anorectoplasty (PSARP) technique in patients having vestibular and perineal fistulae, its complications, results, and remote outcome in our institute. METHODS: From January 2000 to June 2007, patients with vestibular and perineal fistulae subjected to single-stage surgical correction at our institute were reviewed retrospectively from the data available in hospital records and follow-up complaints of patients and their parents in the outpatient department. Patients who had undergone a staged repair were excluded from the study. All patients were assessed for immediate and delayed complications including continence of the neorectum. RESULTS: From January 2000 to June 2007, 123 patients having vestibular (94) and perineal fistulae (29), age range from 28 days to 10 years, were subjected to primary repair either by the ASARP (34) or PSARP (89) technique. Follow-up period ranged from 3 months to 7 years. Mortality was nil. Constipation (25.68%) was the major long-term problem. Incontinence occurred in 1 patient (1.85%), who also had associated sacral agenesis. A total of 98.15% of patients were continent with stool frequency of 1 to 4 per day. Recurrence of fistula (0.81%), anal stenosis (6.76%), mucosal prolapse (2.70%), and anterior migration of the neoanus (1.35%) were the other major problems. Other minor problems like wound infection, superficial wound dehiscence, transient constipation, and diarrhea, etc, were successfully managed by local wound care, antibiotics, laxatives, enema, anal dilatation, and dietary changes. CONCLUSION: Primary anorectoplasty either by PSARP or ASARP is feasible in vestibular and perineal fistulae without covering colostomy. Associated sacral agenesis/hypoplasia, redundant rectosigmoid or pouch colon, and wound infections with dehiscence are the major confounding factors affecting overall outcome. Better outcome in terms of continence can be achieved by careful surgical technique and follow-up along with proper toilet training. Complication rate was greater in cases of vestibular fistula than of perineal fistula, regardless of technique used. Some sort of laxatives and enema are often required. Dilatation of the neoanus for varying periods is also needed.

Unusual variant of Cantrell's pentalogy?

A 12-hour-old male infant presented with prolapsed abdominal content through a defect on left side of chest wall with respiratory distress. A thorough clinical examination suggested absence of ectopia cordis, abdominal wall defect, and any bony anomaly. The child expired after 6 hours of admission because of respiratory distress and electrolyte imbalance. Is congenital defect of chest wall associated with diaphragmatic hernia without ectopia cordis and omphalocele, an unusual variant of Cantrell's pentalogy?