Vanishing gastroschisis with jejunal atresia and extreme short bowel syndrome: A case series
DOI:
https://doi.org/10.47338/jns.v10.932Keywords:
Vanishing gastroschisis, Extreme short bowel syndrome, Jejunal atresiaAbstract
Background: Vanishing gastroschisis may occur due to spontaneous partial or complete closure of anterior abdominal wall defect around the viscera, leading to small bowel ischemia and resultant entry/exit level atresia and extremely short length of the remaining bowel. The prognosis is very poor, even after aggressive surgery, and requires prolonged total parenteral nutrition.
Case Series: We report two female neonates, one with closed and another with closing vanishing gastroschisis, associated with jejunal atresia and extreme short bowel syndrome. In both patients, the antenatal scans showed gastroschisis without the evidence of vanishing gastroschisis. In both neonates, palliative surgeries were done. Both patients died after a few days due to short bowel syndrome and sepsis.
Conclusion: When antenatally detected gastroschisis presents with closed or closing anterior abdominal wall defect, (vanishing gastroschisis), the parents/caregivers must be counseled about the poor prognosis of this condition. A tailored approach to either palliation or aggressive therapy is essential in this rare condition.
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Frolov P, Alali J, Klein MD. Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature. Pediatr Surg Int. 2010; 26:1135-48.
Schwartz MZ, Timmapuri SJ. Gastroschisis. In: Spitz L, Coran AG, eds. Operative pediatric surgery. 7th ed. CRC press: Taylor & Francis group; 2013; 309-19.
Kilby MD. The incidence of gastroschisis. BMJ. 2006; 332:250-1.
Sergi C, Hager T, Alge A, Hager J. Vanishing gastroschisis: Good outcome after a 10-year follow-up. J Pediatr Surg Case Rep. 2018; 30:77–81.
Basaran UN, Inan M, Gücer F, Yardim T, Pul M. Prenatally closed gastroschisis with midgut atresia. Pediatr Surg Int. 2002; 18:550-2.
Vogler SA, Fenton SJ, Scaife ER, Book LS, Jackson D, Nichol PF, et al. Closed gastroschisis: total parenteral nutrition–free survival with aggressive attempts at bowel preservation and intestinal adaptation. J Pediatr Surg. 2008; 43:1006–10.
Houben C, Davenport M, Ade Ajayi N, Flack N, Patel S. Closing gastroschisis: diagnosis, management, and outcomes. J Pediatr Surg. 2009; 44:343-7.
Kumar T, Vaughan R, Polak M. A proposed classification for the spectrum of vanishing gastroschisis. Eur J Pediatr Surg. 2013; 23:72-5.
Barsoom MJ, Prabulos A, Rodis JF, Turner GW. Vanishing gastroschisis and short-bowel syndrome. Obstet Gynecol. 2000; 96:818-9.
Sisodiya RS, Panda SS, Gupta CK, Sinha SK. Closed gastroschisis with vanished small bowel and jejunal atresia. J Neonatal Surg. 2016; 5:65.
Dennison FA. Closed gastroschisis, vanishing midgut, and extreme short bowel syndrome: Case report and review of the literature. Ultrasound. 2016; 24:170-4.
Kimble RM, Blakelock R, Cass D. Vanishing gut in infants with gastroschisis. Pediatr Surg Int. 1999; 15:483–5.
Ogunyemi D. Gastroschisis complicated by midgut atresia, absorption of bowel, and closure of the abdominal wall defect. Fetal Diagn Ther. 2001; 16:227–30.
Winter LW, Giuseppetti M, Breuer CK. A case report of midgut atresia and spontaneous closure of gastroschisis. Pediatr Surg Int. 2005; 21:415–6.
Sandy JE, Lazar LF, Helm s RA. Vanishing bowel: A therapeutic challenge. Nutr Clin Pract. 2006; 21:401–7.
Buluggiu A, Haddad M, Coste M, Louis-Borrione C, Ughetto F, Guys JM, et al. Intestinal loop lengthening: early treatment of vanishing bowel. Pediatr Surg Int. 2009; 25:449.
Khalil BA, Gillham JC, Foresythe L, Harding R, Johnston T, Wright C, et al. Successful management of short gut due to vanishing gastroschisis–case report and review of the literature. Ann R Coll Surg Engl. 2010; 92:e10–3.
Lawther S, Philip I. The outcome of closing gastroschisis: two case reports and literature review. Eur J Pediatr Surg. 2010; 20:65-6.
Dahl E, Haugen G, Refsum S. Midgut atresia and spontaneously closed gastroschisis: support for a mechanical explanation. Eur J Pediatr Surg. 2011; 21:128–30.
Wood SJ, Samangaya RA, Gillham JC, Morabito A. Gastroschisis and the risk of short bowel syndrome: outcomes and counselling. Neonatol. 2014; 105:5-8.
Abdel-Latif M, Soliman MH, El-Asmar KM, Abdel-Satta r M, Abdelraheem IM, El-shafei E. Closed gastroschisis. J Neonatal Surg. 2017; 6:61.
Singh JK, Yadav DK, Khanna K, Khanna V. Closed gastroschisis with left defect: a rare variant. BMJ Case Rep. 2018: bcr-2017.
Ponce MM, Hermans D, Magnee C, Hubinont C, Biard JM. Vanishing gastroschisis visualized by antenatal ultrasound: a case report and review of literature. Eur J Obstet Gynecol Reprod Biol. 2018; 228:186–90.
Rached EA, Sananes N, Kauffmann-Chevalier I, Becmeur F. Vanishing gastroschisis with a favorable outcome after a 3-year follow-up: A case report and Literature review. Case Rep Obstet Gynecol. 2020; 2020:1-6.
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Copyright (c) 2021 Naresh Pawar, Pramila Sharma, Punit Singh Parihar, Manika Boipai
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