Complex gastroschisis with apple peel jejunoileal atresia, primary closure, and Santulli procedure as a surgical alternative. Case report
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José Luis Castillo-Clavijo1, Patricio F Gálvez-Salazar2, Mariana Ángel-Correa3
1Auxiliary Professor, Hospital Universitario del Valle-Evaristo García, Universidad del Valle, Cali, Colombia.
Insights
Gastroschisis with jejunoileal atresia type IIIB is a rare condition with high mortality. Surgical correction using the Santulli technique and advancements in neonatal care can improve survival rates for complex congenital anomalies.
Area of Science:
- Neonatal Surgery
- Pediatric Surgery
- Gastroenterology
Background:
- Gastroschisis is an abdominal wall defect, complex when involving gastrointestinal issues like atresia.
- Jejunoileal atresia, particularly the rare Apple Peel (type IIIb) variant, presents significant mortality risks.
- This case involves a preterm infant with both gastroschisis and type IIIB jejunoileal atresia.
Purpose of the Study:
- To present a case of a preterm neonate with gastroschisis and complex jejunoileal atresia (type IIIB).
- To illustrate the surgical management and outcomes of this rare congenital condition.
- To highlight the utility of the Santulli technique in managing intestinal atresia with significant caliber discrepancy.
Main Methods:
- Initial surgical closure of the abdominal wall defect in a 30-week preterm infant.
- Second-stage surgery at four weeks to address jejunoileal atresia type IIIB with an 8:1 caliber difference.
- Application of the Santulli technique for anastomosis following proximal sac remodeling with a mechanical stapler.
- Postoperative management including early enteral feeding, stoma occlusion, and definitive closure.
Main Results:
- Successful primary closure of gastroschisis.
- Management of jejunoileal atresia type IIIB using the Santulli technique.
- Progressive enteral feeding initiated on day 6, leading to successful stoma management and eventual definitive closure.
Conclusions:
- Gastroschisis and complex intestinal atresia carry high mortality rates.
- Advances in prenatal diagnosis, neonatal intensive care, and surgical techniques are vital for improving survival.
- The Santulli procedure is a valuable option for intestinal atresias with severe caliber discrepancies, offering an alternative when primary anastomosis is not feasible.
Background:
Gastroschisis is a closure defect of the abdominal wall classified as complex when it presents with necrosis, volvulus, or atresia of the gastrointestinal tract. Jejunoileal atresia is caused by abnormal closure, discontinuity, or narrowing of the intestine. Apple Peel or type IIIb is the rarest presentation, with an incidence of 1.3 per 10,000 live births. In addition to presenting a high mortality rate.
Presentation Of Case:
We present a preterm newborn patient of 30 weeks with a diagnosis of gastroschisis and jejunoileal atresia type IIIB. The congenital wall defect was closed in the first surgical stage, and he was then taken at four weeks to correct the atresia. In the second surgery, we found a difference in intestinal calibers of 8:1, and the surgical team decides to perform remodeling of the proximal sac with a mechanical stapler and perform anastomosis using the Santulli technique. On day 6 of life, enteral feeding began through a nutrition tube localized under intestinal anastomosis with progressive nutritional increase. Subsequently, intermittent and progressive occlusion of the stoma was performed, leading the patient to a definitive surgical closure one month later.
Conclusions:
The mortality rate for gastroschisis and complex intestinal atresia is high. Advances in prenatal diagnosis, neonatal intensive care, and proper surgical correction are crucial to improving survival rates. The Santulli procedure is a surgical alternative for intestinal atresias with a caliber discrepancy greater than 4 to 1 or when the characteristics of the distal part do not allow a primary anastomosis to be performed.
