Delphi Method Analysis and Consensus of Prevalent Distinctive Practices for Biliary Atresia Management after Kasai Portoenterostomy.

Background: Extrahepatic biliary atresia (BA) is seen in infants, with an incidence of 1 in 15,000 live births. The presentation is progressive jaundice, dark-colored urine, and clay-colored stools. Kasai portoenterostomy (KPE) is the commonly performed surgical procedure in these patients. Postoperatively, phenobarbitone, ursodeoxycholic acid (UDCA), steroids, and other drugs are given to improve bile drainage and prevent inflammation and fibrosis. However, a definitive protocol regarding the need for different drugs, dosage, and duration varies across individual surgeons and centers. No universally accepted protocol exists for postoperative management after KPE. Aim: The aim of this study was to know the prevailing postoperative management of BA by subject experts and use the Delphi process to know if the experts want to change their practice based on the results from the survey. Material and Methods: A questionnaire was made after discussing with two experts in the field of BA. The questionnaire was mailed to 25 subject experts. The first survey data were analyzed and shared with all responders. In the second survey, change in the management based on the results from the first survey was assessed. Results: The Delphi questionnaire was answered by 17 experts. Postoperatively, prophylactic antibiotics are prescribed for 6-12 weeks by around 40% and >12 weeks by 30% of respondents. Phenobarbitone is prescribed for <3 months by nearly 50%. UDCA is prescribed for <3 months, ≤6 months, and 6 months-1 year by 47.1%, 23.5%, and 23.5% responders, respectively. Nearly 50% prescribe steroids (mostly prednisolone), and among them, two-thirds prescribe it for 6-12 weeks. Approximately 60% give antiviral drugs to children who are cytomegalovirus immunoglobulin M positive. In our survey, 50% of experts perform 5-10 KPE per year, and 25% each perform 10-15 and >15 KPE per year. The second survey noted that a significant percentage of responders want to change their practice according to consensus. Conclusion: From our Delphi survey, an overview of the postoperative management of BA could be made. However, multicentric studies are required for uniform protocol on the postoperative management of BA.

A Randomized Control Trial to Evaluate the Effect of Local Instillation of Mitomycin-C at the Porta after Kasai Portoenterostomy in Patients of Biliary Atresia.

Background: Kasai portoenterostomy (KPE) is the initial treatment for biliary atresia (BA). Even after initial jaundice clearance, a significant number of children presented with the reappearance of symptoms due to ongoing fibrosis involving porta and intrahepatic ducts. Mitomycin-C (MMC) is an antifibrotic agent, and the study hypothesized that local application of MMC at porta can decrease fibrosis, which can improve jaundice clearance and lead to better native liver survival (NLS). Materials and Methods: This prospective randomized control trial included children with BA, who were allocated to groups A or B. The patients in both groups underwent standard KPE; in addition, a 5 French infant feeding tube (IFT) was placed near the porta through the Roux limb in Group B children. During the postoperative period, MMC was locally instilled over the porta in Group B children through IFT. Postoperative jaundice clearance and NLS were assessed and compared. Results: A total of 27 children were enrolled in the study, 16 in Group A and 11 in Group B. Both groups were comparable preoperatively. Although the NLS was not statistically significant in Group B, the survival was quite higher, that was 91%, 81%, and 73% at 6 months, 1 year, and 2 years, respectively, compared to 63%, 50%, and 38% in Group A. Conclusion: Children in Group B clinically showed an early jaundice clearance and a better trend of serial bilirubin levels as well as longer NLS than Group A, but it was not statistically significant. The procedure was technically easy, and no complication was encountered related to surgical technique or MMC instillation.

Pelviureteric Junction Obstruction in Ectopic Kidneys with Extrarenal Calyces: A Rare Renal Anomaly.

Extrarenal calyces (ERC) is a rare renal anomaly where calyces lie outside the renal parenchyma and are connected to pelvis by draining infundibulum. Its presentation with pelviureteric junction obstruction presents a confusing intraoperative finding. We report two cases of ERC with pelviureteric junction obstruction in the ectopic kidney. Emphasis is laid on the defining the anatomy so that reader can identify the condition if he comes across similar situation. The two patients were managed by minimal access approach. Management of these cases and review of pediatric cases reported in literature are discussed.

Bronchoscopic Airway Foreign Body Extraction without Using Optical Forceps.

AIM: Airway foreign body (FB) is a very common pediatric surgical emergency. On certain difficult occasions, the extraction of FB with optical forceps (OFs) is difficult. These conditions create a life-threatening scenario. We present some of the alternative methods of FB extraction for these situations. PATIENTS AND METHODS: This is a retrospective case review of patients presenting to pediatric emergency with airway FB. All patients had a chest X-ray done before the procedure. Rigid bronchoscopy was performed in all patients for retrieval. RESULTS: A total of 20 patients (6 females) were successfully scoped. The age ranged from 1.5 years to 12 years (mean 3.5 years). Bronchoscopic removal was aided by dormia basket (DB) in 13, Fogarty's catheter (FC) in 6, and magnet aided removal in 1. Complete removal was achieved in all patients. CONCLUSIONS: We recommend that DB, FC, and a magnet should form an indispensable part of bronchoscopy set. The use of these alternative methods is successful and allows ventilation during the removal in contrast to removal by OFs. These should be kept as an option if retrieval is difficult.

Retrocaval Ureter: Clinical Images.

Retrocaval ureter (RCU) is a rare congenital anomaly with the ureter looping around inferior vena cava (IVC). We present the case of 10-year-old boy. Diagnosis was confirmed with ultrasound and CT. He underwent retroperitoneoscopic uretero-ureterostomy.

Anorectal Malformations in Males: Pros and Cons of Neonatal versus Staged Reconstruction for High and Intermediate Varieties.

BACKGROUND: High and intermediate types of anorectal malformations (ARMs) in male neonates may be managed either by primary neonatal reconstruction without colostomy cover or by traditional policy of staged reconstruction after neonatal colostomy. Posterior sagittal anorectoplasty (PSARP) is the current widely practiced reconstructive technique with varied results. AIM: To assess our functional results of PSARP without colostomy in male neonates with high and intermediate ARMs compared to 3-stage (neonatal colostomy - PSARP - colostomy closure) methodology in a high volume tertiary care institution of a developing country. PATIENTS AND METHODS: The number of colostomies performed for male high/intermediate anomalies and the number of babies who completed 3-stage reconstruction during a 10-year period is analyzed. The outcome of primary neonatal PSARPs during the same period was analyzed. Eighty primary PSARPs were compared to 81 staged reconstructions for outcome analysis, using Kelly score. RESULTS: A total of 453 colostomies were performed, but only 253 of them completed all stages of reconstruction (52%). Good continence was achieved in 45% of cases of primary PSARP versus 26% in 3-staged surgery. CONCLUSIONS: Primary PSARP in neonatal period without colostomy is a good option for high and intermediate ARMs in males if the treating surgeon is reasonably skilled in neonatal surgery and PSARP procedures.

Laparoscopic retrieval of unusual intra-abdominal foreign bodies in children.

Foreign body (FB) ingestion is a common problem among the pediatric population. On numerous occasions, the FB is left to pass out spontaneously without expecting any harm. There are instances when the FB is either to dangerous to be left alone, that is a button battery or a sharp object. There may be FB, which is either stuck in GIT or have migrated. The situation in these instances demand active intervention. The traditional option has been laparotomy and retrieval. We present here three cases where two needles and a belt buckle were removed by laparoscopy thus avoiding a laparotomy. These cases prove that laparoscopy should be the first choice for such kind of retrieval. These cases had a successful outcome with full recovery.

Thoracoscopic repair of esophageal atresia with tracheoesophageal fistula: Basics of technique and its nuances.

AIM: To review the technique of thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (TREAT) and results reported in literature and with authors' experience. PATIENTS AND METHODS: The technique of TREAT was reviewed in detail with evaluation in patients treated at authors' institution. The patients were selected based on selection criteria and were followed postoperatively. The results available in literature were also reviewed. RESULTS: A total of 29 patients (8 females) were operated by TREAT. Mean age was 2.8 days (range 2-6 days). Mean weight was 2.6 kg (range 1.8-3.2 kg). There was a leak in four patients, and two patients had to be diverted. They are now awaiting definitive repair. Twenty-one patients have completed a mean follow-up of 1.5 years and are doing well except for two patients who had a stricture and underwent serial esophageal dilatations. The results from current literature are provided in tabulated form. CONCLUSIONS: TREAT is now a well-established procedure and currently is the preferred approach wherever feasible. The avoidance of thoracotomy is a major advantage to the newborn and is proven to benefit the recovery in the postoperative patient. The technique demonstrated, and the tweaks reported make the procedure easy and is helpful to beginners. The outcome is very much comparable to the open repair as proven by various series. Various parameters like leak rate, anastomotic stricture are the same. The outcome is comparable if you TREAT these babies well.

Multiple neural tube defects may not be very rare.

BACKGROUND: Multiple neural tube defects (MNTDs) are a rare occurrence. Although the clinical incidence is small, MNTDs raise some interesting embryological queries. AIM: This study aims to investigate the morphological and clinical variations observed in neonates presenting with multiple neural tube defects and associated central nervous system anomalies. MATERIALS AND METHODS: This is a prospective study carried out at our institute to assess clinical and morphological variation in patients presenting with multiple neural tube defects. RESULTS: Among the 263 patients with a neural tube defect, who presented to our outpatient department and emergency departments, only 10 cases of MNTDs were identified. Thus, incidence of MNTDs in the cohort of patients affected with NTD was 0.038 %. Among the 10 patients, 9 had double neural tube defects and 1 patient had three neural tube defects. CONCLUSIONS: Multiple neural tube defects may not be very rare in the general population, especially the populations with high incidence of neural tube defects. Multisite closure theory has the versatility to explain various combinations of neural tube defects, but better insights into the molecular pathways governing this tightly regulated process can provide us the missing link in establishing the pathogenesis of multiple NTDs. It can also provide us with an opportunity to prevent NTDs or treat them in utero by pharmacological modulation of these signaling pathways.

Polymeric immunoglobulin receptor and galectin-3-binding protein are raised in biliary atresia: Reveals a proteomic-based study.

To identify and evaluate differentially expressed plasma proteins in biliary atresia (BA), we performed plasma proteome profiling using liquid chromatography with tandem mass spectrometry (LC-MS/MS) in 20 patients with BA and 10 control children. Serological assays validated the most significant and highly upregulated proteins in a cohort of 45 patients and 15 controls. Bioinformatics tools were used for functional classification and protein-protein interactions of differentially expressed proteins (DEPs). Of 405 proteins detected in patients and 360 in controls, 242 proteins, each with ≥2 unique peptides (total of 3230 peptides), were common in both groups. Compared to controls, 90 proteins in patients were differentially expressed and were dysregulated. Twenty-five were significantly upregulated with polymeric immunoglobulin receptor (PIgR), galectin-3-binding protein (Gal-3BP), complement C2, the most prominent, and 15 had low expression. The bioinformatic analysis revealed functional interaction between DEPs and their role in an inflammatory immune response. Enzyme immunoassay for PIgR and Gal-3BP in patients' plasma showed their levels raised significantly (p = 0.0021 and p = 0.0369, respectively). The PIgR and Gal-3BP are novel proteins upregulated in BA and may be tested further for their utility as potential circulating disease biomarker(s). SIGNIFICANCE: The study shows that plasma PIgR and GAL-3BP levels are significantly raised in infants with BA within the first 3 months of life. If tested in a larger cohort, these proteins may be found to have their diagnostic potential and utility as disease biomarkers. The study also provides valuable information on the involvement of several DEPs in innate immune response, chronic inflammation, and fibrosis. This strengthens the hypothesis that the immune-mediated inflammatory processes are responsible for the progressive nature of BA.

Robotic Excision of Choledochal Cyst with Hepaticoduodenostomy (HD): Report of HD Technique, Initial Experience, and Early Outcome.

INTRODUCTION: Minimal access surgical approach to choledochal cyst (CC) is becoming a standard of care in pediatric age group. Robotic-assisted excision of CC is increasingly being practiced at centers which have access to the system. We present our experience and technique of hepaticoduodenostomy (HD). Over all initial experience, short-term outcomes and complications are also presented and discussed. MATERIALS AND METHODS: Patients with CC and undergoing robotic excision were retrospectively studied. Patients with active cholangitis, liver dysfunction, and perforated CC were excluded for robotic procedures. All included patients were preoperatively evaluated as per the defined protocol. They underwent excision of CC with HD. The duodenal anastomosis was done after limited mobilization and emphasis was laid on anastomosing the distal D2 part to the common hepatic duct. This prevents bile reflux into stomach. The follow-up evaluation was done for these patients. Hepatobiliary iminodiacetic acid (HIDA) scan for duodenogastric reflux (DGR) was done only if patients reported symptoms related to it. RESULTS: A total of 19 patients (10 females) were studied. The mean age was 84 months. Type 1b was present in 12 patients and the rest were type IVb. Complete cyst excision with HD was done in all patients except conversion to open in one patient. The mean surgical time was 170 ± 40 minutes with console time of 140 ± 20 minutes. Median follow-up duration is 2.5 years (range: 3.5-0.5 years). HIDA scan was done in five patients who had reported epigastric pain. Of these five, one patient had a positive DGR. He is on conservative management. CONCLUSION: Robot-assisted CC excision with HD is feasible as proven by the outcome of 19 patients presented in this series. HD is to be done away from pylorus in distal part of down curving D2. This particular step prevents DGR and is the most important point of technique in doing HD. The presented series is the first report of robotic excision of CC with HD. The robot is a facilitator for complex and difficult operations as CC excision and HD.

Dorsal enteric sinus with spina bifida: a rare form of split notochord syndrome.

Split notochord syndrome is a rare congenital malformation that results in spinal anomalies associated with anomalies of the gastrointestinal tract and central nervous system. This report presents an infrequently described variant of this syndrome. A 9-month-old female presented with a dorsal midline mass since birth which was partially covered by skin, and part of the lesion gave a gross appearance of intestinal mucosa. MRI was suggestive of partial sacral agenesis with spinal dysraphism with tethered cord. Excision of the mass with repair of the spinal defect was done. Histopathology confirmed the presence of gastrointestinal mucosa.

Retrocaval ureter with stone in the retrocaval segment of the ureter.

Retrocaval ureter is abnormal looping of the proximal ureter behind the inferior vena cava. The aberrant anatomy results in the compression of the ureter causing hydronephrosis. This is a very infrequent cause of hydronephrosis in children. Association of retrocaval ureter with a stone in the looping segment of the ureter is extremely rare. We report one such pediatric case which was diagnosed preoperatively with a review of pediatric-only cases reported in last 5 years.

Idiopathic simultaneous intussusceptions in a neonate.

Multiple simultaneous intussusception is a peculiar variety of intussusception. The condition is reported in older children and adults. The occurrence in neonates is an interesting situation. We present one such neonate who had a simultaneous ileo-colic and ileo-ileal intussusception without any specific cause. A review of existing literature revealed only three neonates. The terminology and various aspects of the condition are discussed.

Successful surgical management of combined presence of congenital diaphragmatic hernia, ventricular septal defect, and patent ductus arteriosus in a child with allergy to frusemide-a case report.

A 6-week-old male child presented with a right-sided congenital diaphragmatic hernia (CDH), large perimembranous (PM) ventricular septal defect (VSD) and multiple muscular VSDs, and a patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension (PAH). The child was found to be allergic to frusemide. The VSDs were closed, the PDA was ligated, and the CDH was repaired through a right subcostal incision. Postoperatively, there were recurrent episodes of right lung lobar collapse, but the child responded to intravenous antibiotics and nasal continuous positive airway pressure (CPAP). The child is medically stable and on outpatient follow-up. We present this rare combination of diseases in view of a successful simultaneous surgical repair.

Invasive Gastrointestinal Mucormycosis: A Master Masquerader.

We report 3 previously healthy children of postneonatal age who developed fatal form of gastrointestinal mucormycosis after systemic inflammatory response syndrome, shock and metabolic acidosis. Abdominal distension and peritonitis were secondary complications. The study highlights the importance of clinical suspicion of gastrointestinal mucormycosis when the triad of shock requiring vasoactive drugs, metabolic acidosis and abdominal distension is present.