An Atypical Variant of Apple Peel Atresia: Reporting a Rare Case
- PMID:31819834
- PMCID: PMC6886733
- DOI: 10.7759/cureus.6047
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.
Keywords: apple peel atresia; congenital malformation; gastrografin study; jejunoileal atresia.
Copyright © 2019, Bin Arif et al.
Conflict of interest statement
The authors have declared that no competing interests exist.
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References
- Epidemiology of small intestinal atresia in Europe: a register-based study. Best KE, Tennant PW, Addor MC, et al. Arch Dis Child Fetal Neonatal Ed. 2012;97:353–358. - PubMed
- Clinical experience of complex jejunal atresia. Lee SH, Cho YH, Kim HY, Park JH, Byun SY. Pediatr Surg Int. 2012;28:1079–1083. - PubMed
- Small gut atresia in neonates. Kamal A, Khan K, ur Rahman I, Khan A.http://www.jamc.ayubmed.edu.pk/index.php/jamc/article/download/2946/1388 J Ayub Med Coll Abbottabad. 2010;22:64–66. - PubMed
- Outcome of Bishop Koop procedure in neonatal jejunoileal atresias: a retrospective analysis. Imran M, Rehman HU, Rehman IU, Waheed T, Khan I.https://pdfs.semanticscholar.org/476a/ec97c9320fed0fb9bed6ae76af7b9a9d2d... KUST Med J. 2011;3:52–56.
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