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.

Neoadjuvant Peptide Receptor Radionuclide Therapy in a Rare Case of Pediatric Primary Hepatic Gastrinoma.

ABSTRACT: Gastrinomas with predilection for the adult male population are located in the gastrinoma triangle (>90%). Primary hepatic gastrinoma especially in pediatric population is very rare. Peptide receptor radionuclide therapy has shown benefit in metastatic gastroenteropancreatic neuroendocrine tumors (NETs) with an increasing interest in expanding its role as neoadjuvant treatment modality to improve the surgical candidature in inoperable NETs. There is currently no literature supporting its role in the pediatric NET patients. We present a rare case of a young boy with primary hepatic gastrinoma where 177Lu-based peptide receptor radionuclide therapy in the neoadjuvant setting contributed to his final disease-free status.

Goal-directed fluid therapy guided by plethysmographic variability index versus conventional liberal fluid therapy in neonates undergoing abdominal surgery: A prospective randomized controlled trial.

BACKGROUND: Intraoperative fluid therapy maintains normovolemia, normal tissue perfusion, normal metabolic function, normal electrolytes, and acid-base status. Plethysmographic variability index has been shown to predict fluid responsiveness but its role in guiding intraoperative fluid therapy is still elusive. AIMS: The aim of the present study was to compare intraoperative goal-directed fluid therapy based on plethysmographic variability index with liberal fluid therapy in term neonates undergoing abdominal surgeries. METHODS: A prospective randomized controlled study was conducted in a tertiary care centre, over a period of 18 months. A total of 30 neonates completed the study out of 132 neonates screened. Neonates with tracheoesophageal fistula, congenital diaphragmatic hernia, congenital heart disease, respiratory disorders, creatinine clearance <90 mL/min and who were hemodynamically unstable were excluded. Neonates were randomized to goal-directed fluid therapy group where the plethysmographic variability index was targeted at <18 or liberal fluid therapy group. Primary outcome was comparison of total amount of fluid infused intraoperatively in both the groups. Secondary outcomes included intraoperative and postoperative arterial blood gas parameters, biochemical parameters, use of vasopressors, number of fluid boluses, complications and duration of hospital stay. RESULTS: There was no significant difference in total intraoperative fluid infused [90 (84-117.5 mL) in goal-directed fluid therapy and 105 (85.5-144.5 mL) in liberal fluid therapy group (p = .406)], median difference (95% CI) -15 (-49.1 to 19.1). There was a decrease in serum lactate levels in both groups from preoperative to postoperative 24 h. The amount of fluid infused before dopamine administration was significantly higher in liberal fluid therapy group (58 [50.25-65 mL]) compared to goal-directed fluid therapy group (36 [22-44 mL], p = .008), median difference (95% CI) -22 (-46 to 2). In postoperative period, the total amount of fluid intake over 24 h was comparable in two groups (222 [204-253 mL] in goal-directed fluid therapy group and 224 [179.5-289.5 mL] in liberal fluid therapy group, p = .917) median difference (95% CI) cutoff -2 (-65.3 to 61.2). CONCLUSION: Intraoperative plethysmographic variability index-guided goal-directed fluid therapy was comparable to liberal fluid therapy in terms of total volume of fluid infused in neonates during perioperative period. More randomized controlled trials with higher sample size are required. TRIAL REGISTRATION: Central Trial Registry of India (CTRI/2020/02/023561).

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.

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.

Isolated Fetal Ventriculomegaly- Postnatal Outcomes and Proposed New Prognostication Classification.

Background: Outcome of Isolated ventriculomegaly diagnosed antenatally depends on size of ventricles and associated malformations. There is scarcity of literature on the guidelines for prognostication based on outcomes as per the ventricle size. Aim: The aim of this work was to study outcome of antenatally detected isolated ventriculomegaly in terms of medical termination, postnatal neuro-developmental milestones, and mortality; and also to propose a new prognostication classification. Methods: Prospective and retrospective observational study on antenatal mothers with isolated ventriculomegaly diagnosed in fetus. Outcomes in terms of termination of pregnancy, postnatal mortality, need of surgery, and morbidity were recorded. Patients were categorized into four groups: Group 1--ventricle size <10 mm, II--11-15 mm, III--16-20 mm, and IV > 20 mm and neuro-developmental milestones were co-related. Association with chromosomal anomalies, congenital heart disease, and maternal infection were also analyzed. Results: 521 antenatal females were referred with fetal anomalies with 163 having CNS malformations. Isolated ventriculomegaly was seen in only 44. Patients of groups 1 and 2 had 100% normal neuro-developmental milestones without any intervention. Group 3 patients had normal neurodevelopmental milestones in 60% only while shunt surgery was required in 40% of patients. All patients of group 4 had poor outcome with only 50% survival. No association with chromosomal anomalies and heart disease was found. Conclusions: Prognosis of isolated ventriculomegaly depends upon size of ventricles and its progressive increase on serial ultrasounds. New proposed classification is simple and would be useful for the treating surgeons to explain the prognosis to parents so as to relieve them of anxiety.

Factors affecting stress levels in parents of surgical neonates: A prospective observational study.

BACKGROUND: There is a paucity of research focusing on the stress levels in parents of newborns undergoing surgery. Resource challenged systems have to deal with overcrowding, a shortage of workforce along with demographic and socioeconomic issues like delayed presentations and out of pocket expenses. The primary objective of this study was to understand the factors associated with stress in the parents of these congenitally malformed neonates. METHODOLOGY: This was a prospective cohort study, which was conducted in a neonatal surgical ICU of a tertiary care teaching hospital. Factors affecting stress levels in parents of surgical neonates were studied in 100 participants. A multi-dimensional questionnaire - The PSS: NICU score was utilized in the study. The parents were interviewed on Day 3-5 after surgery. RESULT: 59% of the respondents were fathers. The majority of the parents were in the age bracket of 24 to 35 years. The mean scores for the subscales sights and sounds, looks and behavior and alteration in the parental role were 3.24±0.8, 3.52±0.63, 3.55±0.8 and 2.8 ± 0.9 respectively. The highest level of stress was found in the domains of alteration of parental role and infant appearance and behavior. Comparisons showed significantly higher maternal scores in all the domains. Overall stress scores were highest for abdominal wall defects. CONCLUSION: Parents of neonates undergoing surgery suffer from significant stress levels and appropriate counseling targeted towards specific stressors is required to allay this important parental issue.

Anorectal Malformations: The Earlier the Diagnosis, the Better the Outcome.

OBJECTIVE: To estimate the impact of delayed presentation of anorectal malformation (ARM) in neonates and to compare the presenting characteristics and outcomes of early versus delayed presentation. METHODS: This is a prospective observational study of all neonates (age < 28 d) with ARM over 2 y. Delayed presentation was defined as presentation beyond 48 h of birth. Various presenting features and their early postoperative outcomes were compared. RESULTS: Nearly half (26, 48%) of the 54 neonates with ARM had delayed presentation. Early and late presenters did not differ in terms of gender, gestational age, birth weight, place of delivery, and type of ARM (p > 0.05 for all). Delayed group had lower weight at presentation (p = 0.008), higher incidence of severe abdominal distension (p = 0.05), and sepsis (p = 0.171) and required longer time for resuscitation (p = 0.007) and more inotropes (p = 0.015), preoperatively. Early postoperative outcomes including time for stoma to function, initiate feeds and time to reach full feeds were significantly delayed in late presenters. They also had more wound infections, longer hospital stay and higher mortality. CONCLUSIONS: Delayed diagnosis of ARM is associated with significantly higher morbidity and mortality. Adequate awareness and training of health workers for early identification of ARM by careful perineal examination of all newborns at birth is the need of the hour.

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.

Efficacy of dexmedetomidine as an adjunct to ropivacaine in transversus abdominis plane block for paediatric laparoscopic surgeries: A double-blinded randomised trial.

BACKGROUND AND AIMS: α2 agonists have been utilised in regional blocks, but very little data is available for their use in transversus abdominis plane (TAP) block in paediatric laparoscopic (LAP) surgeries. This study investigated the analgesic effect of ropivacaine alone versus its combination with dexmedetomidine for TAP block in children undergoing LAP surgery. METHODS: A randomised, double-blind trial was conducted in 50 American Society of Anesthesiologists (ASA) 1 and 2 children of 2-8 years undergoing LAP abdominal surgery. Children were randomised to receive a total volume of 0.5 ml/kg of 0.2% ropivacaine (LA group) or 0.2% ropivacaine with 1 µg/kg dexmedetomidine (LAD group) for performing ultrasound-guided bilateral TAP block postoperatively (PO). Patients were monitored PO for vital signs, pain, sedation, time to first rescue analgesic and total analgesic consumption for 24 h. Time to first rescue analgesic was expressed as mean ± standard deviation (SD) and analysed using Kaplan-Meier survival analysis. Pain and sedation scores were expressed as median [interquartile range (IQR)] and analysed using Mann-Whitney U test. RESULTS: First rescue analgesic demand was significantly longer (P = 0.001) in LAD (474.8 min) versus LA group (240.9 min) but total analgesics consumption in first 24 h was comparable. Pain scores were significantly lower (P < 0.05) in LAD compared to LA group at all times PO. Each group had comparable but significantly lower sedation scores up to 24 h PO. CONCLUSION: Addition of dexmedetomidine to ropivacaine in TAP block prolongs the time to first analgesic requirement without a difference in the total analgesic consumption.

Epidemiology of Acute Gastroenteritis Caused by Rotavirus among Children Less than Five Years Old Admitted in Hospital, in North India.

OBJECTIVES: To report the data of burden of rotaviral acute gastroenteritis in under-five children from two states post-introduction of the vaccine. METHODS: Children under 5 y of age hospitalized with diarrhea from the states of Haryana and Himachal Pradesh in north India were recruited in the study. Commercially available ELISA kits were used for testing rotavirus in the collected stool samples. Genotyping of the positive samples was done by reverse-transcription polymerase chain reaction. RESULTS: Out of 345 samples collected, 69 (20%) were found to be positive for rotavirus by ELISA. Genotyping was done and G3P[8] (31.3%), G1P[8] (13.4%), G2P[4] (13.4%) were found to be prevalent strains. Mixed strains were also found in 19.4% stool samples. CONCLUSIONS: The study highlighted the high burden of rotavirus associated diarrhea in north Indian states. The data is helpful for evaluating the impact of vaccine on the severity of acute gastroenteritis and the changing strains after the introduction of rotavirus vaccine in the Universal Immunization Program.

Epidemiology of Hospitalized Intussusception Cases from Northern States in India.

OBJECTIVE: To describe epidemiology of intussusception post-introduction of the rotavirus vaccine. METHODS: Hospital-based active surveillance system was set up in three tertiary care hospitals in Chandigarh and Haryana, India, to enroll children <2 y of age admitted with intussusception as per Brighton Collaboration Level-I criteria. The clinical characteristics, treatment modalities, seasonal trends, and outcome of the illness episodes were described. RESULTS: A total of 224 cases were reported. Majority were males (71%) and infants (69.5%). Number of intussusception was more in summer season. Location of intussusception was ileo-colic in 85% of the cases. Nearly 54% cases were treated conservatively and 46% needed surgical intervention. CONCLUSION: Surveillance data provided the epidemiological description of intussusception cases post-introduction of the rotavirus vaccine in northern India. This data could be used to assess the impact of vaccine and safety with a special focus on intussusception.

Role of urodynamics in male patients of high-anorectal malformations: a prospective study.

PURPOSE: Association of spinal or vertebral anomalies and the iatrogenic denervation during surgical correction of anorectal malformation patients especially in boys can lead to neurogenic bladder inthese subset of patients. The paucity of literature with regard to urodynamic studies focusing exclusively in male children with high-anorectal malformations (HARM) lead us to analyze the urodynamic changes. The objective was to study urodynamic profile in male patients who have undergone surgery for anorectal malformation. METHODS: Male high-anorectal malformation patients who had completed all the stages of repair were prospective studied. Following the basic work up, all patients based on the urodynamics were categorized into 2 groups as safe or unsafe bladders. Unsafe bladder was defined as detrusor pressure > 40 cm (high detrusor pressure) or pressure variability of 15 cm of water (detrusor overactivity) or significant post-void residue. MRI was limited to patients with only abnormal urodynamics to rule out spinal causes of neurogenic bladder and due to financial constraints, it could not be offered to all patients. RESULTS: 41 HARM meet the exclusion criteria. All patients were asymptomatic with none having history of urinary tract infections. Ultrasound showed bladder wall thickening in 31.7% patients. UDS revealed reduction in bladder capacity and compliance was noted in 31.7% and 30% patients, respectively. Elevated detrusor pressures (> 40 cm of water) were noted in 10% (4/41), detrusor overactivity with concomitant elevated detrusor pressures in 19.5% (8/41) and normal UDS in 70% (29/41). 13 patients (31.7%) had abnormal cystometric parameters with 12(30%) having unsafe bladders. MRI confirmed sacral agenesis in 1 patient with unsafe bladder. CONCLUSION: Urodynamics can demonstrate occult neurovesical dysfunction in patients with HARM. This would help in early renal protective therapy and prevent the burden of long-term sequelae of neurovesical dysfunction in HARM patients.