Minimally Invasive Open Cystogastrostomy for Giant Pancreatic Pseudocyst in Pediatric Patients.

Open cystogastrostomy is the standard treatment for the operative management of pancreatic pseudocysts. We describe our technique of minimally invasive open cystogastrostomy for giant pediatric pancreatic pseudocyst. Preoperative incision marking on the most prominent part of the pseudocyst was done by ultrasound guidance. A transverse incision of approximately 3-4 cm was made, and a minilaparotomy was performed. Stay sutures were applied on the anterior wall of the stomach. The anterior wall was exteriorized; transverse gastrotomy was performed, and superior and inferior flaps were made. Deaver's retractor was placed inside the lumen, and cystogastrostomy was completed. We employed this technique in five male patients without any complications. All patients were allowed clear liquids on postoperative day 4 or 5; and gradually shifted to a soft diet. The mean duration of postoperative stay was 7 days. The size of the scar ranged from 3 to 5 cm. All patients were doing well on follow-up. Our technique of minimally invasive open cystogastrostomy is a viable option for pancreatic pseudocyst in pediatric patients.

Gastrointestinal Duplications: A Decade's Experience.

Context: Gastrointestinal (GI) duplications are rare congenital malformations with diverse presentations. They usually present in the pediatric age, especially in the first 2 years of life. Aims: To present our experience with GI duplication (cysts) at a pediatric surgery tertiary care teaching institute. Settings and Design: It is a retrospective observational study undertaken in the department of pediatric surgery at our center between 2012 and 2022 for GI duplications. Materials and Methods: All children were analyzed for their age, sex, presentation, radiological evaluation, operative management, and outcomes. Results: Thirty-two patients were diagnosed with GI duplication. Slight male predominance was present in the series (M: F ≈ 4:3). Fifteen (46.88%) patients presented in the neonatal age group; 26 (81.25%) patients were under 2 years. In the majority of cases (n = 23, 71.88%), the presentation was acute onset. Double duplication cysts on opposite sides of the diaphragm were present in one case. The most common location was ileum (n = 17), followed by gallbladder (n = 6), appendix (n = 3), gastric (n = 1), jejunum (n = 1), esophagus (n = 1), ileocecal junction (n = 1), duodenum (n = 1), sigmoid (n = 1), and anal canal (n = 1). Multiple associations (malformations/surgical pathologies) were present. Intussusception (n = 6) was the most common, followed by intestinal atresia (n = 5), anorectal malformation (n = 3), abdominal wall defect (n = 3), hemorrhagic cyst (n = 1), Meckel's diverticulum (n = 1), and sacrococcygeal teratoma (n = 1). Four cases were associated with intestinal volvulus, three cases with intestinal adhesions, and two with intestinal perforation. Favorable outcomes were present in 75% of cases. Conclusion: GI duplications have varied presentations depending on site, size, type, local mass effect, mucosal pattern, and associated complications. The importance of clinical suspicion and radiology cannot be underrated. Early diagnosis is required to prevent postoperative complications. Management is individualized as per the type of duplication anomaly and its relation with the involved GI tract.

Muscle-Sparing Skin Crease Incision Posterolateral Thoracotomies in Pediatric Patients: Our Experience.

Context: Standard posterolateral muscle cutting thoracotomies in pediatric patients are associated with morbidities in terms of poor motor and esthetic outcomes and also more postoperative analgesia requirement, when compared with muscle-sparing technique. Aims: The aim of this study is to evaluate the outcomes of muscle-sparing skin crease incision posterolateral thoracotomies in pediatric patients in terms of abovementioned variables. Settings and Design: This prospective observational study was conducted over a period from January 2016 to July 2020 in a tertiary care teaching institute. Materials and Methods: All patients of stage 3 empyema and pulmonary hydatidosis were included in the study. Results: Thirty-nine patients fulfilled the inclusion criteria. There were 38 children with organized (Stage 3) empyema and one patient with bilateral lung hydatid. There were 24 males and 15 females; age ranging from 6 months to 15 years. Right thoracotomy was performed in 28, left in 10, and bilateral in one patient with lung hydatid. Adequate exposure was achieved in all cases. Decortication with complete excision of thickened parietal pleura with stripping of the visceral peel and release of the entrapped lung was performed in all 38 patients. In 5 children along with decortication, repair of bronchopleural fistula due to necrotizing pneumonia was performed. Bilateral thoracotomies with pericystectomies were performed with a gap of 3 weeks for lung hydatid. Lung expansion assessment at 3 months was satisfactory. Ultrasound assessment of the latissimus dorsi muscle and serratus anterior muscle at 4 weeks revealed complete integrity in all the cases. Most of the patients achieved satisfactory motor and esthetic outcomes. Conclusions: Muscle-sparing skin crease incision posterolateral thoracotomy is a viable alternative to standard posterolateral muscle cutting thoracotomy, especially in a resource-challenged setting. The technique is easy to perform with satisfactory exposure.

Pediatric Vascular Anomalies: A Clinical and Radiological Perspective.

According to the International Society for the Study of Vascular Anomalies (ISSVA) classification, vascular anomalies include a diverse range of pathologies, classified as either vascular tumors or vascular malformations. This classification, last revised in 2018, aims to explain the biological basis of vascular lesions and help clinicians to manage the anomalies. In vascular tumors, there are proliferative changes of endothelial cells, while vascular malformations primarily consist of structural vascular abnormalities. Infantile hemangioma is the most common soft-tissue vascular tumor. Vascular malformations are an extensive group of malformations of the arterial, venous, and lymphatic systems, either in isolation or in combination. Radiological evaluation plays a key part in the management of pediatric patients with these entities. The understanding of sonography and magnetic resonance imaging findings entails its correlation with clinical findings at the time of scanning.

Achondroplasia: Clinical, Radiological and Molecular Profile from Rare Disease Centre, India.

Achondroplasia is the most common autosomal dominant form of skeletal dysplasia and is caused by heterozygous mutations of the fibroblast growth factor receptor 3 ( FGFR3 ) gene at region 4p16.3. This study highlights the data of achondroplasia cases, clinical spectrum, and their outcome from small cities and the region around Rajasthan. The data for analysis were collected retrospectively from genetic records of rare disease clinic in Rajasthan. Clinical profile, radiographic features, molecular test results, and outcome were collected. There were 15 cases, including eight males and seven females, in this cohort. All had facial hypoplasia, depressed nasal bridge, prominent forehead, and characteristic radiographic features. A total of 14 cases were sporadic and one case was inherited from the mother. Mutation analysis showed 13 out of 15 cases with the p.Gly380Arg mutation in the FGFR3 gene. Hydrocephalus was developed in three cases, required shunting in two cases.

Schmid Type Metaphyseal Chondrodysplasia with a Novel COL10A1 Mutation.

Schmid type metaphyseal chondrodysplasia (SMCD) is a rare skeletal dysplasia, characterized by short stature, short limbs, bowing of the legs, and radiographic features of metaphyseal irregularities with fraying and splaying, more severe at the knee. It is caused by mutations of the COL10A1 gene. The authors present an Indian patient with a novel COL10A1 gene mutation.

Intestinal Perforation in Obstructed Umbilical Hernia due to Wedged Plum Seed.

The foreign body ingestion is a rare cause of gastrointestinal perforation in children and is typically seen with sharp foreign bodies or button batteries. Herein, we report an 11-month old male baby who presented with obstructed umbilical hernia. Abdominal radiograph showed dilated small bowel loops, while ultrasonography and CT scan suggested presence of a foreign body. Laparotomy revealed obstructed umbilical hernia with a plum seed being stuck in the terminal ileum causing intestinal perforation. Resection and anastomosis of intestine was performed.

Gallbladder Duplication Associated with Gastro-Intestinal Atresia.

Gallbladder duplication in association with other GIT anomalies is a rare entity. We report two neonates; one with duodenal atresia and the other newborn with pyloric atresia, ileal atresia and colonic atresia, both were associated with gallbladder duplication which has not been reported earlier.

More Distally Located Duodenal Webs: A Case Series.

Duodenal atresia is a frequent cause of intestinal obstruction in the newborn. Obstruction due to duodenal web is infrequent, but its location other than second part of duodenum is rare with only a few cases reported in the literature. We are reporting three patients where we found duodenal webs at unusual locations. In one neonate the web was located at third part of duodenum and in other two patients the web was present at duodeno-jejunal junction (DJ).