Journal of Indian Association of Pediatric Surgeons最新文献

Background and objective: The prevalence of disorders of sex development (DSD) is estimated to affect approximately 1 in 4500-5500 newborns, yet there is a dearth of studies, particularly in Central India, on this topic. This study aims to identify prevalent types of DSD in this region, analyze clinical patterns, and explore associated factors to enhance medical services for DSD management.

Materials and methodology: A prospective observational study was conducted at a tertiary care center in Central India from February 2022 to June 2023. Children presenting with conditions such as hypospadias, micropenis, and female hernia were included in the study, while those over 18 years old and specific hypospadias cases were excluded from the study. Detailed patient histories and clinical examinations focusing on external genitalia and associated anomalies were recorded to estimate DSD prevalence, positivity rates, and phenotypic correlations.

Results: Among 40 patients meeting inclusion criteria (mean age: 5.38 years), 35% were diagnosed with DSD, predominantly 46 XY DSD. Five-alpha reductase deficiency was the leading cause in 46 XY DSD, while congenital adrenal hyperplasia predominated in 46 XY DSD. The study provides valuable epidemiological insights into DSD types prevalent in Central India, though limited by financial constraints precluding genetic analysis.

Conclusion: This study addresses the knowledge gap in Central India, which can contribute to improved understanding and management of DSD. The difference in the incidence of the common cause of DSD is different in our study compared to the existing literature because of broader inclusion criteria. This highlights the importance of evaluating children presenting with proximal penile hypospadias, undescended testes, and females with inguinal hernia for timely intervention.

Primary hyperparathyroidism leads to increased serum parathyroid hormone levels resulting in hypercalcemia. Ectopic intrathymic parathyroid adenomas account for 6%-16% of all parathyroid adenomas and are rare in children but should be considered in cases with hypercalcemia. Early and timely diagnosis can preserve end-organ damage and bone and renal function.

Introduction: Choledochal cyst (CDC) is a rare congenital anomaly requiring surgical excision and bilioenteric reconstruction. Laparoscopic techniques have gained traction for their superior visualization, minimal postoperative pain, and cosmetic benefits.

Materials and methods: A retrospective review of 41 pediatric CDC excision and repair performed laparoscopically at a tertiary care center in India from July 2018 to February 2024 was conducted. Patient data included demographics, Todani classification, surgical technique, intraoperative challenges, and conversion to open surgery.

Results: The median age at surgery was 6 years. Intraoperative challenges included dense adhesions (requiring conversion to open surgery in 17% of cases) and vascular injuries, notably two cases involving the right hepatic artery. Managing giant cysts required decompression to improve access. Anatomical anomalies, such as anomalous portal vein positioning, further complicated dissections. Despite these challenges, laparoscopic approaches resulted in favorable outcomes, with a median operative time of 240 min and minimal postoperative complications.

Conclusions: Laparoscopic excision of CDCs, while technically demanding, is a feasible and effective approach in experienced hands. Key factors for success include preoperative imaging for detailed anatomical understanding, meticulous dissection, and preparedness for intraoperative complications.

Firecracker thoracic injuries are rare but critical, often requiring multidisciplinary management. We report a case of a teenage male patient who presented with hemothorax and empyema due to a firecracker-related chest wall injury. Initial management involved tube thoracostomy; however, the patient subsequently required thoracoscopic intervention for the removal of intrathoracic firecracker residue and decortication. This case emphasizes the significance of early intervention, tailored antibiotics, residue removal for recovery, and preventive measures for such injuries.

Portal vein thrombosis is the most common cause of portal hypertension in children. Congenital portal vein anomalies are rare. We report the case of a 3-year-old with extrahepatic portal vein obstruction secondary to portal vein web. The child was treated successfully by portal venoplasty.

Background: Despite being a technically demanding procedure, the laparoscopic approach for choledochal cyst excision (LEC) has gained popularity in the last two decades. We conducted a study to evaluate the outcomes of the initial cohort of LEC and Roux-en-Y hepaticojejunostomy performed at our center.

Subjects and methods: A retrospective observational study was conducted. Medical records of patients who underwent LEC from July 2019 to April 2023 at our institute were retrieved and analyzed.

Results: Twenty-eight patients were included in our study, of which 68% (n = 19) were females. The median age of the cohort was 5.5 years. 26/28 patients (92.8%) were diagnosed with Type 1 Choledochal cyst (CC), and Type 4a CC was noted in two children (7.2%). Majority of our patients were symptomatic. Pain in the abdomen was the predominant symptom noted in 26/28 patients (93%), eight children (28.5%) presented with jaundice. Antenatal diagnosis of choledochal cyst (CC) was established in one child (3%). Anomalous pancreaticobiliary junction was noted in 10/28 patients (35%) on magnetic resonance cholangiopancreatography. Clavien-Dindo Grade 3A and 3B complications were noted in 7% of patients each, Grade 1 in 17.5%, and Grade 2 in 3.5%. The overall morbidity rate was 28%, and there was no mortality. No cases of malignancy were reported. On a 3-month follow-up, 27/28 patients (96.5%) underwent hepatobiliary iminodiacetic acid scan, which showed good tracer clearance.

Conclusions: Laparoscopic excision of CC in 28 pediatric patients demonstrated safety and efficacy. The average surgery duration was 240 min, with a low conversion rate to open surgery (3.5%). The study confirms that laparoscopic approach as a viable option, although it requires an experienced surgical team.

Prostatic utricle cysts (PUCs) in males with normal external genitalia are rare and often asymptomatic. Minimally invasive surgical techniques, such as laparoscopic excision, have demonstrated effectiveness for symptomatic cases. This report highlights the advantages of a three-dimensional (3D) laparoscopic approach in managing a large PUC in a 6-month-old boy. A 6-month-old male presenting with urinary obstruction and recurrent infections underwent diagnostic imaging and isotope renography, revealing a large PUC with associated nonfunctioning right ectopic kidney and ureter opening in the cyst. A 3D laparoscopic excision of the cyst and associated structures was performed, followed by orchiopexy for the right intra-abdominal testis. The surgery was completed successfully in 70 min without complications. Postoperative recovery was uneventful, with the patient symptom-free during 2 years of follow-up. 3D laparoscopy offers superior visualization and ergonomics compared to traditional two-dimensional laparoscopy, making it a preferred option for complex pediatric pelvic surgeries. This case underscores its advantages in safety, efficiency, and surgical outcomes, especially in centers where robotic surgery may not be available at present.

Introduction: The Meso-Rex bypass (MRB) provides a physiological cure for EHPVO by restoring hepatopetal flow, effectively reducing portal pressure. We share our experience with the MRB procedure, its outcomes, and challenges.

Aim: To describe the feasibility and effectiveness of MRB in children with EHPVO in our practice.

Materials and methods: This is a retrospective study of children with EHPVO who underwent MRB between January 2013 and May 2024. MRB was considered in patients with asymptomatic splenomegaly, moderate hypersplenism, and patent left portal vein either on Doppler or contrast-enhanced computed tomography. The internal jugular vein was used in all cases as jump graft. At follow-up, Doppler was done to note the patency of the shunt and size of the spleen.

Results: Eighty-two shunts of all types were performed. MRB was considered in 17 patients (21%) as per preoperative criteria. MRB was possible in only 10 (12%). Data were analyzed for these 10 children. The average age was 4.5 years (range 1-11 years), 7 were <5 years. Presentation included hematemesis (eight children) and splenomegaly (two children). Four had moderate hypersplenism. Two developed transient self-limiting ascites in early postoperative period. There were no other immediate perioperative complications. The average duration of follow-up was 67 months. One child had hematemesis 2 years after surgery due to stenosis at the proximal end of graft which was treated with endovascular dilatation. Hypersplenism resolved in all children.

Conclusion: MRB is the only curative option for EHPVO. It is feasible in a small subset of patients. Outcomes of MRB are good, especially in younger children.

Umbilical granuloma (UG) is an occasional sequel after umbilical cord separation in neonates with an incidence of 0.2% to 3.01% of live births. It is characterized by moist, fleshy tissue at the umbilicus and presents as umbilical discharge, redness or frank infection. Treatment options include topical application of silver nitrate, copper sulphate, steroids, and common salt. Silver nitrate, though effective, can cause skin burns and needs caution. Common salt is popular as it is cheap, easily available and relatively safe. In this meta-analysis, we intend to compare the efficacy and safety of common salt with other topical agents for treating umbilical granuloma in neonates and infants. This meta-analysis was done as per the PRISMA guidelines and randomized controlled trials (RCTs) comparing common salt with other topical agents were included. Databases like Cochrane, MEDLINE and Scopus were searched. Data was extracted by two independent reviewers and analysed using RevMan software. Outcomes measured were resolution of UG after application and adverse effects of treatment. We observed that common salt is effective and safe for treatment of umbilical granuloma and has a similar success rate when compared to other topical agents. There are no significant adverse effects with its use. It can even be safely applied by caregivers at home.

Background: The type VI choledochal cyst of the cystic duct (CC-CD) is not only a taxonomical conundrum but also a matter of research in terms of its etiopathogenesis and optimal therapeutic options. The role of anomalous pancreaticobiliary junction (PBJ) and pancreatic juice regurgitation in the etiopathogenesis of CC-CD and its association with biliary malignancies has not been elucidated yet.

Objective: To evaluate the role of anomalous PBJ in the etiopathogenesis of CC-CD and generate a scientific opinion through a systematic review of the literature.

Materials and methods: A literature review (PUBMED, PUBMED Central, PMC, Google Scholar, Google search engine) following the PRISMA guidelines revealed 32 articles of interest. Search terms included choledochal cyst, choledochal, cyst, type VI, type 6, cystic duct dilatation, dilated cystic duct and cystic anomaly. Two independent reviewers extracted the data; the third reviewer had been involved in case of discrepancy.

Results: The study-cohort comprised of 45 patients. Roughly, 30% and 60% of the patients were diagnosed in the first and first-two decades of life, respectively. Sex ratio was skewed in favor of females (M:F = 1:1.4). Although males presented earlier than females, females were twice as likely to be diagnosed in the first decade of life. Anomalous PBJ was found in only one-third of these patients and was more common in females. Patients with anomalous PBJ presented early with three-fourth presenting in the first two decades of life. Anomalous PBJ was twice as prevalent in patients with concomitant involvement of other parts of the biliary tract (Group II) as compared to those with isolated CC-CD (Group I). Besides, patients in Group II presented early when compared to Group I, within the group females presented earlier. No significant association of biliary malignancies with anomalous PBJ was seen; the incidence of biliary malignancies was higher in those with a normal PBJ.

Conclusions: Observations from the study-cohort fail to establish a strong association of anomalous pancreaticobiliary junction in the etiopathogenesis of CC-CD or the development of biliary malignancies in these patients. It is likely that there are additional or alternative mechanisms that are important in the context of CC-CD.