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.

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.

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.

Controversies Regarding Management of Vesico-ureteric Reflux.

The primary goal in the management of a child with urinary tract infection (UTI) is to prevent recurrence of UTI and acquired renal damage. Approximately 15% of children develop renal scarring after a first episode of febrile UTI. Vesico-ureteric reflux (VUR) is diagnosed in 30-40% of children imaged after first febrile UTI. The 'top-down' approach involving ultrasound and dimercaptosuccinic acid scan (DMSA) first after an appropriate interval following UTI, can help in avoiding voiding cystourethrogram (VCUG), an invasive test with higher radiation exposure. The majority view remains that VCUG should be done after the second attack of UTI in girls and first attack of UTI in boys. Although the evidence in favour of antibiotic prophylaxis remains doubtful in preventing renal scars associated with VUR, it remains the first line treatment for high-grade reflux (grade 3-5) with an aim to prevent UTI and allow spontaneous resolution of VUR. Early identification and appropriate treatment of associated bowel bladder dysfunction is an essential part of successful medical management of VUR. Endoscopic treatment of VUR, using a bulking agent, is useful in grade 3 VUR. The main controversy regarding intervention (endoscopic/open surgical intervention) involves absence of strong evidence for these interventions in reducing renal scarring on randomized controlled trials. However, several recent trials have found the surgical interventions to be effective in reducing recurrent pyelonephritis and repeated hospital admissions.

Role of Ultrasound-Based Prenatal Prediction of Pulmonary Function in Congenital Diaphragmatic Hernia: Does It Have Prognostic Significance Postnatally?

BACKGROUND AND OBJECTIVES: The incidence of congenital diaphragmatic hernia (CDH) in India is 1 in 1000. About 60 % of these are isolated, and the survival prognosis in them depends upon the quantum of contralateral functional lung. Out of the various pulmonary and extrapulmonary sonological predictors, observed to expected lung-head ratio (O/E LHR) is an efficient gestation-independent predictor of pulmonary function. This study was carried out to see the correlation of this prenatal predictor with the postnatal outcome depending on the pulmonary function. METHODOLOGY: This study was carried out at Apollo Center of Fetal Medicine, New Delhi, from January 2009 to December 2015. A total of 14 fetuses with isolated left-sided CDH were included. The contralateral lung area was measured in 2D transverse view of the thorax at the level of four-chamber view of the heart by tracing method. The obtained value (square mm) was then divided by the expected mean lung area at that gestation and multiplied with 100 to express O/E LHR as percentage. These were then classified as severe (O/E LHR <25 %), moderate (25-45 %) or mild (>45 %) varieties of CDH. The parents to be were counselled for termination or continuation of pregnancy based on severity of CDH and total lung area. The patients were followed up for obstetrical and neonatal outcome till the time of first postoperative visit (diaphragmatic repair). RESULTS: The survival correlation in mild cases was 100 % (n = 5 out of 5) and 50 % in moderate cases (n = 2 out of 4), and both severe cases were terminated. There was a significant difference (p < 0.01) in the survival rate in the mild versus severe cases. CONCLUSIONS: The prenatal predictor for postnatal pulmonary function correlates well with the neonatal outcome and hence is an important tool in prenatal counseling and triaging those who require termination of pregnancy versus expectant management. An obstetrician who is a first point of contact to the pregnant women can understand this and use it for counseling and differentiating the patients who need termination with regard to CDH.

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.

Robotic approach to vaginal atresia repair in an adolescent girl.

A 9-year-old girl presented to us, after failed perineal approach for vaginal atresia, with abdominal pain. She was thoroughly evaluated and contrast enhanced computed tomographic was done, which revealed absence of lower 1/3(rd) of vagina with normal uterus, fallopian tubes and ovaries. There was no associated anomaly. She was successfully managed by a combined robotic and perineal approach. Follow-up after 6 and 12 months revealed large capacious vagina with healthy mucocutaneous junction.

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.