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.

Congenital Pouch Colon with Segmental Dilatation of Ileum: Congenital Pouch Colon Type 6.

Congenital pouch colon (CPC) is classified on the basis of anatomic morphology into five types. Congenital segmental intestinal dilatation associated with anorectal malformation (ARM) is very rare. We are adding two neonates of CPC type 2 associated with segmental dilatation of the ileum to single similar case described in the literature till date. Temporary stomas with excision of either of the pouches should be considered. "Defective mesenchyme formation during organogenesis" due to abnormal mesenchymal precursor cells is hypothesized as the cause for both ARM and different types of CPC.

Anterior Sagittal Anorectoplasty: Our Experience.

CONTEXT: Anterior sagittal anorectoplasty (ASARP) is accepted as one of the techniques for the repair of vestibular fistula (VF) and low-type anomalies, but some may have reservations. AIMS: The aim of the study is to describe the technique, important features, and functional and cosmetic outcomes of ASARP for the treatment of anorectal malformation (ARM) in females. SETTINGS AND DESIGN: A prospective study was performed from 1992 to 2017. MATERIALS AND METHODS: The study included 157 pediatric patients (aged 1-15 years) with diagnosis of ARMs with VF, perineal fistula (perineal ectopic anus), and rectovaginal fistula managed by ASARP. RESULTS: Most cases (36.94%) were 1-6 months' age group; 92.99% of patients (146) were having VF, 5.09% (8) perineal fistula, and 1.91% (3) rectovaginal fistula. Associated anomalies (37) were present in 19.75% (31) of patients. Primary ASARP was performed in 85.35% (134) of cases. The mean operative time was 105 (±15) min. Intraoperative complications were seen in 3.82% (6) of patients. Early postoperative complications were seen in 5.09% (8) of patients - wound infection (4), wound dehiscence (3), and retraction of the rectum (1). Late complications were seen in 12.73% (20) of cases. Overall, five patients developed anal stenosis, two responded to dilatation therapy, and three required anoplasty. The external appearance of the perineum after the 3rd month (postoperatively) was satisfactory in 95.54% (150); overall, 4.46% (7) of patients required the second procedure. Stooling pattern could be assessed in 80.25% (126) of patients at 3 years' age group. Only one had poor outcome with severe soiling (incontinence) and perineal excoriation that also had myelomeningocele. CONCLUSIONS: ASARP is an excellent procedure for VF as it results in optimal correction with minimal sphincteric damage, without additional complexity or difficulties. Primary ASARP is a quick and effective technique and does not require colostomy if performed after due preoperative gut preparation and by an experienced pediatric surgeon.

Kluth Type IIIb6 Esophageal Atresia: Diagnostic Dilemma and Pitfalls of Using Infant Feeding Tube.

We describe three male neonates where infant feeding tube (IFT) passed 18-20 cm in the upper esophageal pouch. A blunt-tipped red rubber catheter confirmed esophageal atresia (EA) with long upper pouch in all three cases. Definitive management revealed EA with tracheoesophageal fistula and long overlapping upper esophageal pouch consistent with Kluth Type IIIb6 variant in two patients. Importance of using red rubber catheter at the pediatric practice instead of IFT is stressed.

Neonatal intestinal obstruction associated with situs inversus totalis: two case reports and a review of the literature.

BACKGROUND: The association of neonatal intestinal obstruction with situs inversus totalis is extremely rare with only few cases reported in the literature to date. This association poses dilemmas in management. We present two such cases (of Indian origin), and briefly discuss the pertinent literature and measures to prevent unfavorable outcome. CASE PRESENTATION: Case 1: a 1-month-old preterm (1300 g) male neonate belonging to Hindu (Indian) ethnicity presented with recurrent bile-stained vomiting, non-passage of stools and epigastric fullness. A babygram and upper gastrointestinal contrast studies revealed situs inversus and suggested proximal jejunal obstruction with midgut volvulus. Exploration confirmed situs inversus totalis along with reverse rotation and midgut volvulus. There was a small gangrenous area in the proximal jejunal loop. A Ladd's procedure, resection of the gangrenous jejunal loop, and jejunojejunal anastomosis was performed. Note was made of the unusual appearance of the intestines suggestive either of fibrous or fatty infiltration. Postoperatively, our patient developed septicemia and died. Case 2: a 4-day-old female neonate belonging to Hindu (Indian) ethnicity, small (1320 g) for gestation, presented with history of non-passage of meconium since birth, refusal to accept feeds, and episodes of recurrent bilious vomiting with abdominal distension. A plain radiogram revealed situs inversus and proximal jejunal obstruction. Ultrasonography of her abdomen revealed renal dysplastic changes in both her kidneys. Laparotomy confirmed multiple jejunoileal atresias with situs inversus totalis. Resection anastomoses was performed for multiple atresias. Our patient passed a few pellets of meconium stools postoperatively; feeds were started gradually on the sixth day. Our patient gradually developed oliguria and renal failure, followed by respiratory distress and generalized edema requiring ventilatory support. She died later due to multiorgan failure. CONCLUSIONS: Clinicians should have high index of suspicion for malrotation with midgut volvulus or intestinal atresias in neonates of situs inversus presenting with bilious vomiting. The surgical treatment should follow the same surgical principles. In situs inversus, because of transposition of viscera, midgut volvulus may occur in an anticlockwise direction, hence derotation is performed clockwise. Prognosis was poor in our series because of low birth weight, late presentation, presence of gangrenous locus in the small bowel and development of septicemia in our first case and multiorgan fibrosis/dysplasia in our second case. Early diagnosis and timely referral is paramount for favorable outcome.

Fetus in fetu: report of two cases.

Fetus-in-fetu (FIF) is a rare and interesting entity characterized byincorporation of a malformed, monozygotic, diamnionic parasitic twin into the body of other normal twin partner. FIF is differentiated from teratoma by its embryological origin, its unusual location in the retroperitoneal space and the presence of vertebral column (axis) often with appropriate arrangement of other organs or limbs around this axis. We report two cases of FIF. Our first case presented at 18 months, while second at 9 year of age. FIF derived their blood supply directly from aorta in both the cases.Our FIF had distinct fetoid features, well developed axial skeleton with a complete spinal column, trunk, intestinal loops, four limbs, well developed fingers and toes, male external genitalia and abundant scalp hairs. Their weightwas 600 grams and 800 grams, respectively. Postoperative period was smooth and on long-term follow up no evidence of recurrence was seen in both the patients.

Salvage splenopexy for torsion of wandering spleen in a child.

The wandering spleen is a rare condition characterized by the absence or underdevelopment of one or all of the splenic suspensory ligaments that resulting in increased splenic mobility and rarely torsion. Preventing infarction is the aim of a prompt surgery by splenopexy. We report a case of salvage splenopexy in torsion of a wandering spleen in a three year old girl presented with severe abdominal pain for three days.

Biliary atresia associated with polysplenia syndrome, dextrocardia, situs inversus totalis and malrotation of intestines.

Biliary atresia (BA) is a rare disease and the end result of a destructive, inflammatory cholangiopathy, leading to fibrosis and biliary cirrhosis. It is classified into syndromic variety with various congenital anomalies and non-syndromic (isolated anomaly). We present here a 1-month-old female child with the syndromic variety of BA associated with polysplenia syndrome, dextrocardia, situs inversus totalis and malrotation of intestines. She developed jaundice in the first week of life. Kasai operation was performed but she developed cholangitis and septicemia 2.5 months after surgery and succumbed later.

Congenital pyloric atresia and associated anomalies: a case series.

Congenital pyloric atresia (CPA) is a very rare surgical condition. Eleven patients with the diagnosis of CPA treated at our hospital were retrospectively studied for the age at diagnosis, sex, presenting symptoms, associated anomalies, operative findings, treatment and outcome. Male: Female is 8:3. The age at diagnosis ranged from one day to three years. Associated anomalies were seen in four (36.6%). These included epidermolysis bullosa (EB) in two, oesophageal atresia with distal trachea-oesophageal fistula in one, colonic atresia in one, sensorineural deafness and dysplastic kidney in one patient. All three types of CPA were observed; six (54.5%) had type 1, four (36.3%) had type 2 and one (9%) had type 3 [(core)]. Different procedures performed were Heineke-Mickulicz pyloroplasty, Finney's pyloroplasty and gastro-duodenostomy. Post-operatively, nine out of eleven did well while other two died giving an overall survival of 81.8%. Sepsis was the cause of death in both of them.