An Atypical Variant of Apple Peel Atresia: Reporting a Rare Case

Abstract

Apple peel intestinal atresia is a rare congenital malformation. It consists of a proximal jejunum ending in a blind pouch and distal small bowel wrapped around its vascular supply in a spiral fashion. A combination of type IIIb jejunoileal atresia (apple peel atresia) and type IV (multiple intestinal atresias) is a rare entity. The diagnosis and management of such complicated cases is a challenge, especially in resource-limited settings. We report a case of a four-day-old female who presented to the neonatal intensive care unit with complaints of vomiting, yellow discoloration of the skin, and failure to pass meconium since birth. The baby was born preterm (34 weeks) via spontaneous vaginal delivery. The physical examination concluded a jaundiced and dehydrated child with a soft, non-tender abdomen and absent gut sounds. X-ray abdomen showed two air-fluid areas in the left hypochondrium. The upper gastrointestinal gastrografin study revealed that contrast opacified the third part of the duodenum and no contrast was observed beyond it. On exploratory laparotomy, proximal jejunal atresia with four distal atresias in apple peel fashion and a viable 20 cm of small bowel was observed. The apple peel segments were supplied by mesenteric vessels. Unfortunately, our patient expired despite all supportive measures. The case highlights the significance of the prenatal and early postnatal diagnosis of such a complex combination of intestinal atresias for adequate and timely management.

Primary Repair of an Apple Peel Jejunal Atresia and Transanastomotic Feeding in a 1.2 Kilogram Preterm Infant

“…2 Apple peel atresia has also been associated with situs inversus, polyspenia, and malrotation. 1 As demonstrated, the patient presented here was noted to have an unusually long appendix, non-rotated bowel, and a Meckel’s diverticulum, a true diverticulum on the anti-mesenteric border that results from the persistence of the congenital vitello-intestinal duct. 4…”

“…1,2 Apple peel atresia is associated with a high rate of premature delivery which further complicates the course of care, 2 as seen in the case presented. A neonate with APA most commonly presents with a distended abdomen, bilious emesis, failure to pass meconium, and respiratory distress, 1 also seen in this case. Compared to patients with ileal atresia, patients suffering from type IIIb jejunal atresia are more likely to suffer from conditions such as cystic fibrosis, gastroschisis, Meckel’s diverticulum, biliary atresia, and other gastrointestinal anomalies.…”

“…Neonatal intestinal obstructions are commonly caused by intestinal atresia which is believed to be secondary to intrauterine mesenteric vascular accident. 1 Among the five types of intestinal atresia, APA remains the rarest form, accounting for less than 10% of all atresia cases. 3 The exact pathogenesis of this rare atresia remains unclear and further investigation is needed to fully understand the cause of this congenital anomaly.…”

“…Apple peel atresia (APA), also known as type IIIb jejunoileal atresia or Christmas tree atresia, is the rarest jejunoileal anomaly with an incidence of .7-.8% in 10,000 live births. 1 Apple peel atresia is characterized by a dilated jejunum or ileum ending in a blind pouch, an absence of the superior mesenteric artery and mesentery, and a helical configuration of the remaining small bowel around its vascular supply. 2 The segment of small bowel with this type of anatomic aberration typically relies on retrograde flow from the ileocolic or right colic artery to supply the distal small bowel with its necessary blood supply.…”

“…3 As APA is quite difficult to manage operatively, historically this condition resulted in significant morbidity and mortality. [1][2][3] In recent years, survival rates in patients with APA have improved markedly due to enhanced knowledge of the disease, early recognition techniques, and refined interventional methodology. Here, we present the case of a 4-day-old, preterm infant who failed to pass meconium and experienced unproductive feeding attempts with bilious emesis.…”