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Closing Gastroschisis, Vanishing Midgut Syndrome and Intra-­Abdominal Volvulus Presenting with Haematemesis at Birth Ahmed Abdelhamid Darwish ‍ ‍

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Closing Gastroschisis, Vanishing Midgut Syndrome and Intra-­Abdominal Volvulus Presenting with Haematemesis at Birth Ahmed Abdelhamid Darwish ‍ ‍ Images in… BMJ Case Rep: first published as 10.1136/bcr-2019-232757 on 26 February 2020. Downloaded from Closing gastroschisis, vanishing midgut syndrome and intra- abdominal volvulus presenting with haematemesis at birth Ahmed AbdElhamid Darwish Department of Pediatric Surgery, DESCRIPTION Bristol Royal Hospital for A 32- weeks- gestation, 1.9 kg girl born with emer- Children, Bristol, UK gency caesarean section due to foetal distress. Ante- natal scans showed gastroschisis with mild bowel Correspondence to dilatation. At birth, she presented with prolapsed Ahmed AbdElhamid Darwish; aadarwish37@ yahoo. co. uk necrotic bowel to the right of the umbilical cord, closed abdominal defect around the small bowel Accepted 5 January 2020 mesentery (figure 1) and with significant haemate- mesis (initially thought to be ingested maternal blood). After resuscitation, urgent laparotomy revealed complex gastroschisis and vanishing small bowel syndrome. The stomach, duodenum and 20 cm of ischaemic jejunum were twisted 180° intra-abdominally with distal atresia, microcolon and absent ileocaecal valve. The rest of the small intestine has vanished with complete necrosis of the prolapsed intestine through the closing gastro- Figure 2 Untwisting the proximal jejunum (colour schisis. After untwisting the proximal jejunum improved gradually) and excision of the necrotic bowel. and excision of the necrotic bowel (figure 2), a tube jejunostomy was inserted at the bulbous end of the atresia on the left upper quadrant to estab- was due to intra-abdominal volvulus with subse- lish proper drainage of this dilated segment and quent foetal distress rather than ingested maternal to avoid re-twisting. Actually, the haematemesis blood. The patient had an uneventful recovery and http://casereports.bmj.com/ is currently on long- term home total parenteral nutrition. A bowel lengthening procedure will be considered in the future due to extreme short bowel length <25 cm. Closing gastroschisis is a challenging subset of complex gastroschisis in which the bowel that is eviscerated through abdominal wall defect has been constricted by the fascia resulting in intestinal stricture, atresia, necrosis or resorption.1 2 Based on a recent classification of closing gastroschisis by on September 27, 2021 by guest. Protected copyright. Perrone et al, this case is defined as Type C (presence of a closing ring with non-viable external bowel with or without an associated atresia). Having <25 cm bowel length, our case is considered having poor prognosis in terms of enteral autonomy.3 Learning points ► Gastroschisis is either simple, complex (associated with bowel atresia) or closing © BMJ Publishing Group gastroschsis with vanishing midgut syndrome. Limited 2020. No commercial ► Haematemesis associated with closing re- use. See rights and permissions. Published by BMJ. gastroschisis is concerning for bowel ischaemia and surgery should not be delayed. To cite: Darwish AA. BMJ ► Ingested maternal blood should be a diagnosis Figure 1 Prolapsed necrotic bowel to the right of the Case Rep 2020;13:e232757. of exclusion opposed to the presumed diagnosis doi:10.1136/bcr-2019- umbilical cord, closed abdominal defect around the small of volvulus. 232757 bowel mesentery. Darwish AA. BMJ Case Rep 2020;13:e232757. doi:10.1136/bcr-2019-232757 1 Images in… BMJ Case Rep: first published as 10.1136/bcr-2019-232757 on 26 February 2020. Downloaded from Contributors Single author work: AAD. The author has operated on this case, ORCID iD wrote the summary and submitted the images. The article was reported and Ahmed AbdElhamid Darwish http:// orcid. org/ 0000- 0001- 5151- 6267 designed by the author. Funding The authors have not declared a specific grant for this research from any REFERENCES funding agency in the public, commercial or not- for- profit sectors. 1 Shalaby A, Davenport M. Closed gastroschisis. Pediatr Surg Int 2011;27:335. Competing interests None declared. 2 Kumar T, Vaughan R, Polak M. A proposed classification for the spectrum of vanishing gastroschisis. Eur J Pediatr Surg 2013;23:72–5. Patient consent for publication Parental/guardian consent obtained. 3 Perrone EE, Olson J, Golden JM, et al. Closing gastroschisis: the good, the bad, and the Provenance and peer review Not commissioned; externally peer reviewed. not- so ugly. J Pediatr Surg 2019;54:60–4. Copyright 2020 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit https://www.bmj.com/company/products-services/rights-and-licensing/permissions/ BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ► Submit as many cases as you like ► Enjoy fast sympathetic peer review and rapid publication of accepted articles ► Access all the published articles ► Re-use any of the published material for personal use and teaching without further permission Customer Service If you have any further queries about your subscription, please contact our customer services team on +44 (0) 207111 1105 or via email at [email protected]. Visit casereports.bmj.com for more articles like this and to become a Fellow http://casereports.bmj.com/ on September 27, 2021 by guest. Protected copyright. 2 Darwish AA. BMJ Case Rep 2020;13:e232757. doi:10.1136/bcr-2019-232757.
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