Antenatal diagnosis of microvillus inclusion disease

Microvillus inclusion disease is a rare autosomal recessive disorder due to defective apical surface of the enterocytes presenting with severe watery diarrhea starting at birth. We describe a female infant who had antenatal diagnosis of microvillus inclusion disease. At 36th gestational week of a 32-year-old woman ultrasound examination revealed dilatation of fetal sigmoid colon. The amniotic fluid level was normal. An amniocentesis was performed to rule out congenital sodium and chloride diarrhea in the prenatal period. The patient didn't prefer to undergo genetic tests. In conclusion, prenatal ultrasonographic identification of dilated bowel loops without polyhydramnios suggests differential diagnosis of microvillus inclusion disease in addition to congenital chloride diarrhea, jejunoileal atresia, volvulus, meconium ileus, Hirschsprung disease, enteric duplications, anorectal atresia.

Jejunoileal Atresia: A National Cohort Study

Purpose: Jejunoileal atresia (JIA) is a rare disease. We aimed to determine the overall incidence of this malformation and associated malformations in a national cohort. Furthermore, we compared the treatment results of this cohort with the current literature.Methods: Data from the major health insurance company, which covers ~30% of the German population, were analyzed. All patients with ICD-10-Code Q41.1-9 (atresia of jejunum, ileum, other parts and not designated parts of the small bowel) who underwent any surgical procedure for small bowel were analyzed in a 10-year period between 2007 and 2016.Results: A total of 435 patients were included in the study. The incidence was 2.1 per 10,000 live births. The male:female ratio was 1:2. Sixty-four percent were premature, 21% had associated cardiac anomalies, 16% had abdominal wall defects, 7% had urogenital malformations, and 7% had cystic fibrosis. Sixty percent of all patients with jejunoileal atresia, 57% of patients with accompanying abdominal wall defects and 72% of patients with associated cystic fibrosis required ostomy as the initial procedure. In 25% of all patients, only one intestinal operation was coded. In 39% of patients, two operations were coded. Twelve percent of all patients required feeding gastrostomy or jejunostomy. Sixteen percent of all patients presented with liver-related complications, i.e., cholestasis or liver insufficiency. Six patients underwent an intestinal lengthening procedure (2 Bianchi, 4 STEP). In five patients, initial lengthening was performed within 1 year after the first intestinal operation. Mortality until 1 year after initial surgery was 5%. Of those who died, 88% were premature, 34% had cardiac anomalies and 16% had abdominal wall defects. None had cystic fibrosis. Patients with ostomy significantly more often needed operative central venous line or operative feeding tube. Short bowel was coded significantly more often in these patients.Conclusion: Patients with JIA present with low mortality. The rate of ostomies is higher than in literature. To give clinical recommendations for the initial surgical approach, further clinical research is needed.

A comparative study of surgical procedures in neonatal intestinal atresia - resection and anastomosis, Bishop Koop, Santulli and Mikulicz procedure

Background: The aim of this work was to assess the outcome for patients with jejunoileal atresia who underwent primary anastomosis, Bishop–Koop, Santulli procedure and Mikulicz procedure. Intestinal atresia is one of the most common causes of intestinal obstruction in neonates. But ideal surgical management of this condition remains controversial.Methods: During the period from January 2017 to January 2019, 112 neonates (62 males and 50 females) suspected to have intestinal atresia were operated in NRSMCH, Kolkata. 30 patients were treated with primary resection/ anastomosis, 30 patients underwent Bishop-koop (BK) technique, 27 patients who were treated with Santulli technique and 25 patients who were treated with Mikulicz procedure. Demographics, treatment and outcomes including mortality, morbidity and nutrition status were reviewed and were compared among the four groups.Results: 20 patients (17.86%) died during the perioperative period. Among them, 10 died due to uncontrolled sepsis and 10 due to malnutrition. Mortality was highest in Primary anastomosis group (33.33%) followed by BK group (13.3%), Santulli group (11.1%) and lowest in Mikulicz group (4%). The Mikulicz group experienced the lowest re-operation rate (4%) and Primary anastomosis group experienced highest re-operation (26.7%). This difference was statistically significant (P<0.003).Conclusions: Using Mikulicz procedure in the current study has improved the survival of these patients and minimized the post-operative complications.

A Preliminary Report in Efficacy of The Lateral T-Stoma: Solution for Functional Obstruction and Short Bowel Sydrome in High Jejunoileal Atresia

Objective: This study is a preliminary report in the efficacy of lateral T-stoma (LTS), which we developed as a solution for anastomosis dysfunction and/or short bowel syndrome in the jejunoileal atresia (JIA). Method: The primary pathologies and results of all cases who underwent lateral t-stoma between July 2017-2020 were evaluated retrospectively. Results: Case 1 with meconium pseudocysts secondary to intrauterine volvulus had Type-3A JIA at 50 cm from the Treitz, and end-oblique anastomosis was performed. The LTS was created proximal to the first anastomosis. on Day 15 due to abdominal distension with 5/1 diameter ratio. The patient was discharged 13 days later. The LTS was closed in the 10th month. In Case 2 with Type-2 JIA at 60 cm from the Treitz, primary LTS was created proximal to the anastomosis with 5/1 diameter ratio. Patient was discharged 15 days later. The LTS was closed in the 10th month. End-to-side anastomosis was performed in Case 3 with Type-2 JIA located 70 cm from Treitz. Anastomotic leakage occurred on Day 36, and the LTS was created with 6/1 diameter ratio. The LTS was closed in the 5th month. Prenatally diagnosed Case 4 was postnatally intubated due to pulmonary hypertension, and surfactant was applied. Intrauterine volvulus, meconium pseudocyst and intestinal perforation were detected at 50 cm from the Treitz, and jejunoileal anastomosis together with primary LTS was performed with 5/1 diameter ratio. Nasogastric feeding started on the 10th day, but patient was lost on Day 44 without extubation. Primary repair and end-stoma were performed in Case 5 with gastroschisis/intestinal atresia with perforation. Due to high-flow stomal discharge/weight loss, the ileo-colic anastomosis and LTS were performed proximal to anastomosis with 3/1 diameter ratio at 60 cm from Treitz on Day 68. Patient was discharged on Day 90. In Case 6 with prenatal diagnosis as meconium cyst with Type-2 JIA (at 65 cm from Treitz), primary LTS was created proximal to the anastomosis with 4/1 diameter ratio. The LTS was closed in the 14th month due to high-flow stomal discharge. Conclusion: Creation of lateral T-stoma proximal to the anastomosis in JIAs with large differences in diameters may shorten hospitalization time and relieve functional obstruction/short bowel syndrome.

Five years experiences in diagnosis and management of jejuno-ileal atresia in Kanti children’s hospital

Introduction: Jejuno-ileal atresia is a major cause of neonatal intestinal obstruction. The aim of this study is to evaluate the incidence, clinical presentation, management, and outcome of jejunoileal atresia at our institute over a period of five years. Methods: The medical records of the patients with the diagnosis of jejunoileal atresia during a period of five years (April 2014 to April 2019) were obtained from the hospital record section and surgical intensive care unit, and were reviewed and analyzed. Results: There were 61 cases of jejunoileal atresia among 144 cases of intestinal atresia. Twenty-nine (47.5%) of them were male. Laparotomy with resection of atretic part with anti-mesenteric tapering enteroplasty and end to end anastomosis was done in 15, and resection without tapering enteroplasty with end to end anastomosis was done in 39 patients. Thirty-eight patients (62.3%) were discharged while Twenty-three (37.7%) cases were lost to mortality. Conclusions: Although the mortality and morbidity rate are high in jejunoileal atresia, early diagnosis, improvement in surgical technique, modern ventilatory support and advanced in intensive care unit has led to the significant increase in the survival rate.

Radiological diagnosis of microcolon in a 5-day old neonate: a case report

Microcolon is a radiographic feature of a low intestinal obstruction that result from intrauterine underutilization or what is term unused colon. The finding of microcolon on contrast enema study in newborn with distended abdomen usually suggests jejunoileal obstruction, jejunoileal atresia, meconium ileus, or occasionally totals colonic agangliosis. We are therefore presenting this case to highlight the wonders that imaging will perform in prompt diagnosis and management of this condition.

Duodenal atresia with absence of superior mesenteric artery and apple peel appearance of small gut: A case report

Background: Though duodenal atresia is relatively common among intestinal atresias, its association with apple peel type of jejunoileal atresia and absent superior mesenteric artery (SMA) is very rare. This association contradicts the well-known embryo pathology of duodenal atresia and could be a management challenge. Case presentation: A 4-day-old preterm male baby presented with bilious vomiting. The X-ray abdomen showed a double bubble sign. Laparotomy revealed atresia of the 3rd part of the duodenum. The SMA was absent and the remaining small gut was short, narrow, and twisted around a narrow vascular pedicle arising from the marginal artery. We resected the most distal part of the dilated duodenum and made a wide end to side anastomosis with distal chimney stoma (Bishop Koop enterostomy). The baby did well in the postoperative period. Conclusion: The surgical management of this rare entity was technically challenging. Bishop Koop enterostomy is a safe option when the primary anastomosis is not possible.