Extrarenal Teratoid Wilms Tumor.

Teratoid Wilms tumor (TWT) is a variant of Wilms tumor. 30 cases have been reported so far. The extrarenal TWT variant has only been 5 in number. We are reporting a case of retroperitoneal TWT in a 6-year-old female child with diagnostic and therapeutic challenges.

Abdominal Manifestations of Multisystem Inflammatory Syndrome in Children: A Single-Center Experience.

OBJECTIVES: We reviewed the cases of probable multisystem inflammatory syndrome in children (MIS-C) to identify those cases that mimicked surgical emergencies. METHODS: Records of children managed for MIS-C during a 15-month period between March, 2020 and April, 2021 were retrieved. Data on clinical presentation, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) RT-PCR report, SARS-CoV-2 antibody status, blood investigations, radiological investigations and management were collected. RESULTS: A total of 28 out of 83 children with probable MIS-C had acute abdominal symptoms and signs. Fifteen children had mild features like diffuse abdominal pain or non-bilious vomiting, and the remaining 13 (46.2%) had severe abdominal signs or bilious vomiting. Four children worsened with conservative treatment for MIS-C and were detected with perforated appendicitis. Two more children developed recurrent appendicitis on follow up. One child with appendicitis who underwent laparoscopic appendectomy, later manifested with MIS-C. CONCLUSION: Surgical abdominal emergencies may be confused with or occur concurrently in children with MIS-C that should be identified with a high index of suspicion.

Jejunal Intramural Hematoma: Masquerading as a Mass Lesion.

Jejunal intramural hematoma (JIH) is rare. A 5-year-old girl, a known attention-deficit hyperactivity disorder child with paraneoplastic symptoms, on imaging showed a suspicion of Jejunal intramural mass with internal bleeding. Laparotomy showed a JIH. Evacuation of hematoma and Histopathological Examination (HPE) ruled out tumor. Possible explanation of the manifestation is suggested.

Postnatal management of bilateral Grade 3-4 ureteropelvic junction obstruction.

INTRODUCTION: Bilateral hydronephrosis on prenatal ultrasound can be managed expectantly or with surgical intervention. The treatment strategies and outcomes are not clearly defined. METHODS: We conducted a retrospectively audit of outcomes of management of prenatally detected severe bilateral ureteropelvic junction obstruction (UPJO) in our institution.Patients with bilateral Grade 3-4 hydronephrosis were included. Those with complications like rupture, underwent bilateral intervention within 4 weeks; in the remaining, unilateral pyeloplasty was performed at 4-12 weeks. The contralateral renal unit was re-evaluated at a later date for further improvement or deterioration. All the patients were followed up with ultrasonography and renogram at 3 months, 6 months, and 1-year post operatively. The case records were analyzed for the resolution of antero-posterior diameter (APD) or the improvement in single-kidney glomerular filtration rate (s-GFR) in the operated units. RESULTS: Over 15 years, 28 patients (56 renal units) had bilateral UPJO (male-to-female ratio = 13:1). Twelve units underwent neonatal intervention to tackle the complications (6 bilateral pyeloplasty), 17 units underwent early pyeloplasty, and 15 underwent late pyeloplasty. Twelve of the twenty-two (54%) contralateral units, which were stented/observed, resolved spontaneously. Receiver operating characteristics analysis revealed that those with initial APD <25 mm and initial s-GFR >35 ml/m were more likely to improve during the observation. Ten of the forty-four operated units (22%) failed to show an improvement. Units with initial s-GFR <10 ml/m had poor chance of postoperative functional recovery. CONCLUSIONS: In neonates with bilateral UPJO, the worse affected kidney is operated first, as it still has the potential to recover. The contralateral milder UPJO unit is known to recover spontaneously following unilateral pyeloplasty. In those with bilateral Grade 4 UPJO and mass, bilateral pyeloplasty is feasible. Alternatively, unilateral pyeloplasty + contralateral cystoscopic retrograde stenting may prevent rupture or functional deterioration in the opposite kidney.

Age Is Not a Criterion in Patient Selection for Kasai Portoenterostomy.

AIMS: The aim of our study was to compare the outcome of Kasai portoenterostomy (KPE) in children with biliary atresia (BA) older than 90 days to children less than 90 days and to study its safety and efficacy in children older than 90 days. SUBJECTS AND METHODS: Relevant data were collected from our prospectively maintained database of all children with BA who underwent KPE over a 5-year period. Children were divided into two groups: Group 1 ≤90 days and Group 2 >90 days. Data analyzed and compared included total and direct bilirubin, aspartate aminotransferase-to-platelet ratio index (APRI), and the outcome of procedure which was defined as a serum direct bilirubin <2 mg/dl within 6 months after surgery. Standard statistical tests were used for analysis. RESULTS: Out of 62 children, 45 children were in Group 1 and 17 children were in Group 2. Children in Group 2 had similar total and direct bilirubin compared to children in Group 1. APRI, an indicator of fibrosis, was significantly increased in Group 2 (P = 0.08). About 47% of children in Group 2 had Stage III fibrosis on liver histology compared to 22% of children in Group 1. None of the children in Group 2 had synthetic liver failure (refractory ascites, hypoalbuminemia, or coagulopathy unresponsive to Vitamin K) or portal hypertension. KPE was successful in 29.4% of children in Group 2 and 44% in children in Group 1. There was no perioperative mortality in our group. CONCLUSIONS: KPE was successful in a third of children over 90 days of age and can be safely performed in this group. In the absence of synthetic liver failure, age should not be a disqualification for performing KPE.

Early Cholangitis after Portoenterostomy in Children with Biliary Atresia.

AIMS AND OBJECTIVES: Biliary atresia (BA) is a cholangiodestructive disease of the biliary tree. The first line of treatment is a Kasai portoenterostomy (PE) following which patients may develop cholangitis. We studied the effect of early cholangitis on the outcome of PE, namely jaundice clearance and early native liver survival (NLS). METHODS: We reviewed the data of all children who developed cholangitis after PE from our prospectively maintained database of children with BA. The standardized treatment of all children in the database is described. The frequency and nature of these episodes were characterized, and the outcome of PE and NLS 1 year after PE was calculated. RESULTS: Of 62 children who underwent PE in our institutions, 27 developed cholangitis. All episodes of cholangitis occurred within 14 months of PE. Of 25 children who cleared jaundice in the overall series, 19 had cholangitis. The incidence of cholangitis was significantly higher in children who cleared jaundice. Nine children who had cholangitis are alive with native livers for more than 1 year after PE. Twelve children had intractable cholangitis. Three of these children are alive with native liver 1 year after PE. CONCLUSION: In our series, cholangitis occurred in most children who cleared jaundice. Furthermore, the 1-year NLS of children who developed cholangitis was 33%.

α-Smooth muscle actin expression predicts the outcome of Kasai portoenterostomy in biliary atresia.

BACKGROUND/AIMS: Biliary atresia (BA) is a cholangio-destructive disease of the infant liver presenting with features of obstructive cholangiopathy. The Kasai portoenterostomy (KPE) is the first line of management. The aim of our study was to identify the characteristic features of liver histology in BA that impact the outcome of KPE. PATIENTS AND METHODS: Data from 30 consecutive children was retrieved from our prospectively maintained database of children undergoing KPE. This included basic demographics, laboratory values and histopathological data from liver biopsy. The stages of fibrosis, presence of ductal plate malformation (DPM), giant cell transformation, extramedullary hematopoiesis and area percentage of α-SMA (α-smooth muscle actin) expression was correlated with jaundice clearance after KPE using standard statistical tests. Native liver survival was computed. RESULTS: Overall, 13 (43%) children cleared jaundice in this series and 10 (33%) are alive with native liver. Lower area percent expression of α-SMA correlated with increased probability of jaundice clearance after KPE (P < 0.001). There was no correlation between stage of fibrosis and jaundice clearance (P = 0.52). DPM, giant cell transformation and extramedullary hematopoiesis did not correlate with outcome. All children who are alive with native liver had lower expression of α-SMA. CONCLUSION: α-SMA expression may be a potential predictor of jaundice clearance and native liver survival after KPE.