Evaluation of Hepatic Shear Wave Elastography to Assess Liver Fibrosis in Biliary Atresia Patients and Its Correlation with Liver Histology and Surgical Outcomes: A Prospective Observational Study.

Introduction The native liver survival in biliary atresia (BA) depends on various factors, and one of the crucial factors is the rate of progression of liver fibrosis after portoenterostomy, but there is no reliable investigation to assess it. This study evaluated shear wave elastography (SWE) to detect liver fibrosis in BA patients and assess its utility during follow-up. Materials and Methods This was an observational study; SWE was done preoperatively and postoperatively at 3 and 6 months. The SWE values were analyzed to determine their correlations with preoperative liver histology as well as with postoperative SWE variation between different postoperative outcomes. Results Twenty-one patients were included in the study; the preoperative SWE values were strongly correlated with liver biopsy grading ( p < 0.001). At the 3 months postoperatively, SWE was done for 18 children: 12 in group A (patent bilioenteric drainage on hepatobiliary iminodiacetic acid scan) and 6 (nonpatent) in group B; mean SWE value was 12.8 and 17.3 kPa, respectively ( p < 0.001). Ten children from group A underwent SWE 6 months postoperatively, and the mean value was 13.23 kPa. Conclusion The SWE values correlate with liver histology grading, suggesting a reliable alternative to biopsy. Additionally, the baseline SWE values and their trend during follow-up can provide information on the disease's progression.

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.

Anesthetic Management of a Massive Cystic Hygroma of the Neck in a Neonate.

Cystic hygroma of the neck, a congenital benign tumor of the lymphatic system, is a potential cause of neonatal airway obstruction leading to stridor. Meticulous airway evaluation, case appropriate preparation, and use of advanced technology, including videolaryngoscope and ultrasonography, can facilitate the safe management of the difficult airway.

Comparison of the Surgical Outcomes in Neonates with Left-sided Congenital Diaphragmatic Hernia with Only Skin Closure versus Abdominal Muscle Closure.

Aim: This study aims to compare the outcome of neonatal left congenital diaphragmatic hernia (CDH, Bochdalek type) repair through laparotomy with and without abdominal muscle closure. Materials and Methods: This retrospective study was conducted between January 2012 and May 2021 at a neonatal surgical unit of a Tertiary Care Center. Demographic details, preoperative management, Two-dimensional-echo, intra-operative findings, postoperative course, and follow-up data were collected and analyzed. Results: The study group comprised 50 neonates with a mean standard deviation (SD) age at admission: 4.44 (5.12) days, male: female ratio of 3:2, and mean (SD) weight: 2.73 (0.51) kg. Following repair of the diaphragmatic defect through laparotomy, 26 (52%) underwent skin closure alone, whereas 24 (48%) underwent abdominal muscle closure. Postoperatively, there was a significant fall in the level of platelets (P = 0.021), increase in pressure support by at least 4-5 cm H2O (P = 0.027), and increase in the blood urea (P < 0.001), creatinine (P = 0.005), lactate (P = 0.019), and acidosis (P = 0.048) in the muscle closure group. Although not statistically significant, there was a fall in the urine output and blood pressure in this group. There was no significant difference in the duration of inotropes. Mortality was 8 (32%) in the skin closure group, and 14 (61%) in the muscle closure group (P = 0.05). Conclusions: Neonates undergoing left CDH repair through the abdominal route with skin closure alone, had better survival, as well as hematological, renal, and ventilatory parameters than those who underwent muscle closure. It is a useful surgical modification to improve outcome in centers with limited facilities.

Randomized controlled trial to evaluate the rate of successful neonatal endotracheal intubation performed with a stylet versus without a stylet.

INTRODUCTION: Neonates in intensive care units often require endotracheal intubation and mechanical ventilation. During this intubation procedure, a stylet is frequently used along with an endotracheal tube. Despite the widespread use of a stylet, it is still not known whether its use increases the intubation success rate. This study examined the association between stylet use and the intubation success rate in surgical neonates. METHODOLOGY: This single-center study was conducted between December 2021 and December 2022 in the Neonatal surgical intensive care unit of a tertiary care center in Northern India. Infants were randomized to have the endotracheal intubation procedure performed using either an endotracheal tube alone or with a stylet. The primary outcome of the study was to assess the successful first-attempt neonatal endotracheal intubation rate with and without using a stylet. Apart from the rate of successful intubation, the duration of the intubation and complications during the intubation procedures as measured by bradycardia, desaturation episodes, and local trauma were also recorded. Both groups were thus compared on above mentioned outcomes. RESULTS: The total number of neonates enrolled were 200, and the overall success rate (81% in the stylet group vs. 73% in the non-stylet group) was not statistically significant. Intubation time was however less, when stylet was used (16.2 ± 4.3 vs. 17.5 ± 5.0 s, p = .046). When the endotracheal tube size was 3 or less, the success rate was substantially higher in the stylet group (80%) than the non-stylet group (63%), p = .03. No statistical difference was recorded for bleeding and local trauma, though the esophageal intubation rate was higher when intubation was attempted without the stylet. CONCLUSION: Endotracheal intubation using a stylet did not significantly improve the success rate of the procedure, however, intubation time significantly varied between groups and in different conditions. The rigidity and curvature provided by the stylet may facilitate the process of intubation when smaller caliber endotracheal tubes are used.

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.

Colonic Atresia Associated with Biliary Atresia.

Colonic atresia (CA) is an uncommon type of intestinal atresia commonly associated with other anomalies, while biliary atresia (BA) is also rare but usually an isolated anomaly. The pathogenesis for either of the anomalies is unclear. The co-occurrence of both pathologies has not been mentioned in the literature. We here discuss the management of CA with BA and the review of pertinent literature.

Association between intraoperative anatomical variation and histopathological parameters in cases of ureteropelvic junction obstruction in children: A cross-sectional study.

Introduction: The intraoperative anatomical findings (IOAF) of all ureteropelvic junction obstruction (UPJO) cases are not identical. Moreover, there is also controversy in the literature regarding histopathological (HP) findings in cases of UPJO. In the present study, we evaluated different IOAF and assessed their association with specific HP parameters. Materials and Methods: This was a cross-sectional study set-up, which was carried out in a tertiary care centre. Children with UPJO who underwent surgery between 2017 and 2020 were enrolled. The following IOAF were noted: Type of pelvis (extrarenal or intrarenal), insertion of the ureter (high or normal), presence of lower pole crossing vessel (CV), negotiation of UPJ segment with double J stent (3 Fr) and length of internal narrowing (LIN) at UPJ. The resected segment of UPJ was assessed at three levels (pelvis, UPJ and ureter) for various HP parameters including fibrosis, oedema, inflammation and smooth muscle hypertrophy (SMH). Results: Thirty-nine children were included in the study with a mean age of 31 months. The summary statistics of IOAF were intrarenal pelvis in 5 cases, high insertion of the ureter (HIU) in 9, CV in 6, negotiable UPJ in 23, and 16 cases showed LIN >1 cm. All cases showed SMH at the pelvis region and SMH with fibrosis at the UPJ region. At the pelvis region, there was an association between (1) HIU with oedema and chronic inflammation (CIF), (2) CV with CIF and (3) LIN with CIF and SMH. At the UPJ region, there was an association between (1) CV and negotiable UPJ with less fibrosis and (2) LIN with SMH. At the ureteric end, CV showed an association with less fibrosis and more CIF. Conclusion: All UPJO cases have some common HP findings. Although, some particular IOAF, i.e., presence of CV, negotiable UPJ, HIU and LIN showed association with specific HP parameters.

Tracheoesophageal Fistula with Bilateral Facial Cleft: A Rare Occurrence.

Commissural or lateral facial cleft (macrosomia), classified at Tessier number 7 craniofacial clefts, is a rare congenital anomaly usually associated with deformities of other structures developed from the first and second branchial arches. It affects the esthetics and functional aspect of the oral cavity. Bilateral transverse cleft occurring alone is uncommon and it's with tracheoesophageal fistula (TEF) has not been reported to the best of our knowledge. We report a case of esophageal atresia (EA) and TEF with macrosomia. EA was repaired, and the patient was discharged on full feeds. He is awaiting cleft repair.

Unusual manifestations of Peutz-Jegher's syndrome in children.

PeutzJegher's syndrome (PJS) is a rare, autosomal dominant disease, characterized by gastrointestinal (GI) polyps and perioral hyperpigmentation along with the increased risk of certain malignancies. In children, the most common presentation is recurrent intussusception due to polyps. These polyps can involve any part of the GI tract and can present with a variety of clinical presentations. Usually, these polyps can be removed endoscopically but often require surgical excision also. In this report, we discuss two children of PJS with uncommon presentations, one presented with retrograde intussusception and another with gastric outlet obstruction. The first child underwent laparoscopy and another required open surgery with intraoperative enteroscopy.

Primary Repair of Pediatric Posttraumatic Complete Bladder Neck Horizontal Transection: Our Experience.

Complete transverse transection just below the bladder neck is extremely rare. We present two such cases with associated pelvic fracture following trauma. Both underwent early primary vesicourethral anastomosis with no postoperative complications and are continent in the follow-up.

Duodenal and Pyloric Web in Children: Clinical Presentation and Management.

Background: Duodenal and pyloric web (DW/PW) can present at any age, symptoms depend upon the location of the web along with the presence and size of the opening in the web. The surgical management is not straightforward always. Here, in this study, we aim to assess clinical characteristics, management, and outcome of children with DW/PW. Materials and Methodology: This was a retrospective study from 2005 to 2019, and data were collected from record registers. All children of DW/PW presented between this duration were included in this study. Results: A total of 45 patients (age range = 1 day to 11 years) included in the study, 40 had DW while 5 had PW. Seven patients were diagnosed antenatally and 20 patients had associated congenital anomalies. Most patients presented with vomiting either bilious or nonbilious. Plain X-ray was sufficient for the diagnosis in 60% of patients, the rest diagnosed on contrast study. The web excision and pyloroplasty were done for PW. The web excision and Heineke-Mikulicz type enteroplasty was the preferred surgery for DW but some patients were required Kimura's duodeno-duodenostomy. For postoperative nutrition, enteral feeding was established through the placement of a feeding tube beyond anastomosis. Ten patients died due to septicemia and associated anomalies. Four patients had a minor leak which was managed by conservative means. Four patients required redo surgery, adhesive obstruction was the most common indication. During follow-up, all 35 patients were doing well with no major complaints. Conclusion: DW/PW has different presentations as compared to other intestinal atresia and can present at any age. A contrast study confirms the diagnosis when plain X-ray is inconclusive. Associated anomalies and septicemia are the poor prognostic indicators. Postoperative enteral feeding helps in maintaining adequate nutrition and improves the outcome even in children with a minor anastomotic leak.

External and internal parasitic conjoined twinning: Diverse presentation and different surgical challenges.

Background: Parasitic conjoined twin is a rare but well-known entity with unclear embryopathogenesis. Abnormal conjoined twinning can result in an externally attached parasitic twin (PT), an enclosed foetus in foetu, or a mature teratoma. The treatment requires complete excision and reconstruction of local anatomy which is not always straightforward. Materials and Methods: PT cases presenting over 12 years were analysed. Patients with complete data, histopathological diagnosis and follow-up were included in the study. During follow-up, specific complications and related investigations were considered. Results: A total of five patients at four different sites were identified: two retroperitoneal foetus in foetu and three externally attached PTs which were located in the lumbar region, sacrococcygeal area and on the lower anterior abdominal wall. All patients underwent complete surgical excision. In foetus in foetu cases, the blood supply was directly from the aorta with a short stump while the three externally located ones required meticulous and careful dissection with the reconstruction of local anatomy. Conclusion: Parasitic conjoined twinning can present at different sites and surgical challenges vary accordingly. For surface lesions, reconstruction may be as complicated as excision. Prognosis can be affected by the excellence of anatomical restoration. Long-term follow-up is essential to address problems specific to the site of lesion and method of surgical reconstruction.

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.

Abdominal Wall Yolk Sac Tumor in a Child.

Midline vascular abdominal wall lesions are likely to be mistaken for vascular malformations in young children. We report a case of large yolk sac tumor located in the anterior abdominal wall just below xiphisternum in a 20-month-old girl diagnosed by raised serum alpha fetoprotein levels and fine-needle aspiration cytology. Preoperative chemotherapy helped in reducing its size allowing wide resection and primary wound closure. This case is reported for the unusual location and role of chemotherapy in management.

Benign Renal Tumors in Pediatric Age Group: Retrospective Analysis.

BACKGROUND: Benign renal tumors are extremely rare and were studied here. This series also includes a renal teratoma in a horseshoe kidney, probably only the second in the pediatric literature. MATERIALS AND METHODS: Retrospective review of children with benign renal tumors operated between 2006 and 2018 at one center. RESULTS: Twelve patients (M:F ratio 10:2), age range 3 weeks (31-week gestation) to 13 years presented with large palpable renal swelling (n = 12) and hematuria (n = 3). Computed tomography (CT) scan showed features typical of the tumor. Final histopathology (age group [mean]) showed: multilocular cystic nephroma (MLCN) - n = 5 (41.7%), (11-16 months [13.6]); congenital mesoblastic nephroma (CMN) - n = 4 (33.3%) (classic 1, cellular 3) (0.75-5 months [2.125]); mature cystic teratoma - n = 1 (8.3%): (48 months, in a horseshoe kidney), and angiomyolipoma (AML) - n = 2 (16.7%) (144 months [sporadic] and 156 months [tuberous sclerosis]) One patient with cystic teratoma with no calcification on CT scan received pre-operative chemotherapy as fine-needle aspiration cytology (FNAC) reported malignant small blue cell tumor. Nephroureterectomy with Gerota's fascia could be done easily in all without intraoperative complications. Delay in presentation in MLCN and CMN led to increased symptoms and CT scan changes. All patients did well in 1.5-12 years (median 3 years) follow-up including cellular mesoblastic nephroma. CONCLUSIONS: Benign renal tumors often occur in specific age groups but may overlap that of Wilms tumor. Proper interpretation of clinical presentation, CT scan, and FNAC findings help in avoiding preoperative chemotherapy. Upfront nephroureterectomy is curative. Histopathological findings decide further treatment. Children with AML and tuberous sclerosis need lifelong follow-up.

Congenital Parotid Fistula: Surgical Management in Two Infants and Review of Literature.

Congenital salivary gland fistulas are uncommon. They develop as a result of abnormalities of the first and second branchial arches. Operative and nonoperative methods of management have been described. We report two rare cases of congenital parotid fistula presenting to us in infancy that were managed surgically.

Clinical Presentation, Treatment, and Challenges in the Management of Child Sexual Assault at a Tertiary Care Referral Center in India.

BACKGROUND: Child Sexual Assault (CSA) is not an uncommon but an under-reported crime. Along with social and psychological critical issues, there are multiple challenges faced by the surgical team for the treatment of complex perineal injuries associated with CSA. This study was conducted to find clinical presentation and management of CSA along with its problems and challenges encountered by the pediatric surgical team. MATERIALS AND METHODS: This was a retrospective study from 2010 to 2019, conducted in the department of pediatric surgery at a tertiary referral center. All-female patients with a definitive history of sexual assault were included in the study. RESULTS: Seven patients fulfilled the inclusion criteria and the mean age was 5.3 years. After a primary survey, all patients were taken up for examination under anesthesia (EUA). Three patients were managed by the primary repair of the wound and did well during follow-up. Four patients had grade 4 perineal injury and required stage reconstruction. As a first stage, repair of rectal tear, vaginal tear, and the perineal body reconstruction was done along with diversion colostomy. One patient required redo repair of the perineal body and one had developed a rectovaginal fistula. Three patients completed all stages and they are fully continent. CONCLUSION: The spectrum of injuries varies widely in CSA and more chances of high-grade perineal injuries in children due to distinctive local anatomy. EUA is crucial to assess the extent of the injury and to decide the course of management. Meticulous anatomical repair and diversion stoma is the key for successful complex repair and excellent long-term outcomes in terms of continence for the severe grade of perineal injuries.

Nutritional Status of Children with Biliary Atresia and the Role of Portoenterostomy to Improve Nutrition.

BACKGROUND: Nutritional depletion and growth stunting are present in patients with biliary atresia; "normal" nutrient and vitamin supplementation fail to correct these deficiencies. Children with this condition form the largest group for possible liver transplantation in the future; hence, stress should be laid on close attention to their nutrition. METHODS: Twenty-five patients with biliary atresia as cases and 25 age-matched children as controls were enrolled in the study from November 2010 to June 2012. Preoperatively, patients underwent standard investigations and anthropometric measurement (weight, height, and head circumference) assessment. Nutritional status (assessed with standard growth chart) was compared with control population, and children were divided into poor nutritional status and good nutritional status. Kasai's portoenterostomy was performed in all patients, and comparison was done between preoperative nutritional status with postoperative status of children and also between hepatic iminodiacetic acid (HIDA) scan-positive (patent bilioenteric pathway) children with HIDA scan-negative children. Postoperatively, after 12 weeks, the same anthropometric measurements were taken again, growth velocity (GV) was assessed, and children were divided into poor, average, and good GV. RESULTS: Nutritional status of children with biliary atresia was significantly poor than that of control group. Postoperatively, children had better nutritional status than preoperative nutritional status, especially in HIDA scan-positive children. GV was also significantly better in those children in whom postoperative HIDA scan was positive. CONCLUSION: Children with biliary atresia have poor nutritional status in comparison to normal population and require multifaceted approach to achieve adequate nutrition. Establishment of a patent bilioenteric pathway in these children improves their nutritional status and GV.