VACTERL Associated with a Rare Limb Anomaly Combined with Edward (Trisomy 18) Syndrome.

VACTERL association is a heterogeneous condition that includes at least three out of six core structural defects which occur together more commonly than would be expected by chance alone. We report a rare association of VACTERL with unilateral proximal focal femoral deficiency and trisomy 18 syndrome.

Technique of Antireflux Procedure without Creating Submucosal Tunnel for Surgical Correction of Vesicoureteric Reflux during Bladder Closure in Exstrophy.

AIM: To report the clinical application of the new surgical technique of antireflux procedure without creating submucosal tunnel for surgical correction of vesicoureteric reflux during bladder closure in exstrophy. MATERIALS AND METHODS: Based on the report of published experimental technique, the procedure was clinically executed in seven patients of classic exstrophy bladder with small bladder plate with polyps, where the creation of submucosal tunnel was not possible, in last 18 months. Ureters were mobilized. A rectangular patch of bladder mucosa at trigone was removed exposing the detrusor. Mobilized urteres were advanced, crossed and anchored to exposed detrusor parallel to each other. Reconstruction included bladder and epispadias repair with abdominal wall closure. The outcome was measured with the assessment of complications, abolition of reflux on cystogram and upper tract status. RESULTS: At 3-month follow-up cystogram, reflux was absent in all. Follow-up ultrasound revealed mild dilatation of pelvis and ureter in one. CONCLUSIONS: The technique of extra-mucosal ureteric reimplantation without the creation of submucosal tunnel is simple to execute without risk and complications and effectively provides an antireflux mechanism for the preservation of upper tract in bladder exstrophy. With the use of this technique, reflux can be prevented since the very beginning of exstrophy reconstruction.

Antireflux Status Post Roux-en-Y anastomosis: An Experimental Study for Optimal Antireflux Technique.

BACKGROUND: Roux-en-Y hepaticojejunostomy has been a gold standard to establish biliary-enteric anastomosis for various surgical indications, but associated with variable incidences of cholangitis. This experimental study was conducted to report a modification in Roux-en-Y anastomosis for possible better alternative to provide antireflux procedure after Roux-en-Y biliary-enteric anastomosis with the aim to minimize the possibility of reflux and its consequences. MATERIALS AND METHODS: For experimental study, the required fresh segment of Lamb's small intestine was procured. Three sets of Roux-en-Y anastomosis were created for each experiment. In set 1, there was simple Roux-en-Y anastomosis. In set 2, Roux-en-Y anastomosis along with 4-5 cm long spur between the hepatic and duodenal limbs was created. In set 3, in addition to Roux-en-Y with creation of spur, additional antireflux mechanism was created at the junction of upper two-third and lower one-third of the hepatic limb. Saline mixed contrast was infused by infusion pump to raise the intraluminal pressure to more than 10 cm of H2O. X-ray was taken at that time. RESULTS: In set 1, all preparations demonstrated reflux of contrast in the hepatic limb. The set 2 also demonstrated the same findings of 100% reflux in the hepatic limb. In set 3, No reflux was observed in 8 (80%) preparations while remaining 2 (20%) preparations reveal partial reflux. CONCLUSION: This experimental study suggests that the provision of spur and additional valve may be able to decrease the possibility of reflux in Roux-en-Y biliary-enteric anastomosis.

Lipoencephalocele in a Child: An Uncommon Swelling in the Occipital Region.

Neural tube defects are common congenital malformations of the central nervous system. The 3 most common neural tube defects are anencephaly, myelomeningocele, and encephalocele. Lipoencephalocele is an extremely uncommon entity with sporadic reports in the literature. We treated a 4-year-old gild with occipital lipoencephalocele. This report presents the clinical presentation and management of the patient along with a review of the relevant literature.

Type V Congenital Pouch Colon: An Extremely Rare Variant of Anorectal Malformations.

Type V is the rarest form of congenital pouch colon with only four cases reported till date. We report this anomaly in a 6-month-old boy. He was managed successfully with excision of distal pouch and coloplasty of proximal pouch along with abdominoperineal posterior sagittal anorectoplasty. We recommend preservation of proximal pouch in such cases.

Neonatal Paratesticular Scrotal Mass: A Rare Case of Atypical Neurofibromatous Neoplasm with Uncertain Biologic Potential.

ABSTRACT: Atypical neurofibromatous neoplasm with uncertain biologic potential presenting as a paratesticular scrotal mass in a neonate with congenital giant melanocytic nevus is rare. Only one such case of neonatal scrotal neurofibroma has been reported earlier. We report an additional case and its management.

Complicated duodenal perforation in children: Role of T-tube.

Background: Diagnosis of duodenal perforation (DP) in children is often delayed. This worsens the clinical condition and complicates simple closure. Objectives: To explore the advantages of using T-tube in surgeries for DP in children. Patients and Methods: A retrospective study was conducted on all patients of DP managed in the Department of Paediatric surgery at a tertiary centre from January 2016 to December 2020. Clinical, operative and post-operative data were collected. Patients, with closure over a T-tube to ensure tension-free healing, were critically analysed. Results: A total of nine DP patients with ages ranging from 2 years to 9 years were managed. Five (55.6%) patients had blunt abdominal trauma; a 2-year-old male had perforation following accidental ingestion of lollypop-stick while a 3-year-old male had DP during endoscopic evaluation (iatrogenic) of bleeding duodenal ulcers; cause could not be found in other 2 (22.2%) patients. Of the five patients with blunt abdominal trauma, 4 (80%) had large perforation with oedematous bowel, necessitating repair over T-tube. Both patients with unknown causes had uneventful outcomes following primary repair with Graham's patch. Patients with lollypop-stick ingestion and iatrogenic perforation did well with repair over T-tube. The only trauma patient with primary repair leaked but subsequently had successful repair over a T-tube. One patient with complete transection of the third part of the duodenum and pancreatic injury who had repair over T-tube died due to secondary haemorrhage on the 10th post-operative day. Conclusion: Closure over a T-tube in DP, presenting late with oedematous bowel, ensures low pressure at the perforation site, forms a controlled fistula and promotes healing, thereby lessening post-operative complications.

Wound Closure in Large Neural Tube Defects: Role of Rhomboid Flaps.

BACKGROUND: Large wounds following surgery for neural tube defects are difficult to close; physical wound characteristics such as position and dimension would serve as a guide for their surgical closure. AIM: To study how wound dimension determines the choice between primary and rhomboid flap closure of skin defects following surgery for neural tube defects. MATERIALS AND METHODS: A retrospective study was carried out on cases of neural tube defects operated in the department of paediatric surgery at a tertiary center for 3 years from January 2015 to December 2017. Data regarding clinical features, location, wound dimensions following surgery, any bony deformity, method of closure used, distance of wound from anus, and postoperative complications were collected and analyzed. RESULTS: A total of 114 cases were operated during this period; 86/114 had primary closure, whereas 28/114 needed rhomboid flap for tension-free cover. Primarily closed wounds had a biphasic distribution of (long axis)/(short axis) ratio (with values either >1.65 or <0.63), whereas those covered by rhomboid flaps had a mean ratio of 1.25 (range, 0.71-1.45). All six cases with bony deformity needed rhomboid flaps. Although all lipomeningomyelocele defects could be primarily closed, all rachischisis needed flap cover. Infected lesions had a mean wound distance of 5.3cm from posterior anal margin. CONCLUSION: Defect's position, its size and shape, and any bony deformity determine the choice of closure of postoperative wound. The versatile, safe, and universal rhomboid flap is an aesthetic solution to the large skin defects in patients of neural tube defects.

Spontaneous bladder rupture: unusual presentation in a haemophilic child.

A 4-year-old boy child presented with abdominal pain, haematuria, lower abdominal mass and urinary retention. There was no family history of bleeding. Ultrasound abdomen revealed an intravesical blood clot, rupture of the anterior bladder wall and perivesical haematoma. Contrast-enhanced CT (CECT) confirmed the findings. The child had a history of previous gum bleeding which prompted work-up for coagulation disorder. Investigations confirmed haemophilia. Following clotting factor replacement, surgical exploration was done. Intraoperatively, a 3 cm extraperitoneal, longitudinal tear on the anterior bladder wall was found along with haematoma in the bladder and perivesical space. Evacuation of the haematoma and repair of the bladder tear was performed. Postoperative period was uneventful, and the child was asymptomatic at 6-month follow-up. To the best of our knowledge, spontaneous bladder rupture has not been reported in a haemophilic child.

Two Cases of Massively Prolapsed Patent Vitellointestinal Duct.

Patent vitellointestinal duct (PVID) is a benign congrnital anomaly ususally presenting with fecal discharge from the umbilicus. In this report, we describe two cases of PVID presented with massive bowel prolapse through the PVID and signs of intestinal obstruction. Surgery revealed prolapse of the ileal intussusceptum through the PVID. Both of the babies were sucssesfully managed with surgery.