A rare case of VACTERL association at the Federal Medical Centre, Yenagoa, Bayelsa State, Nigeria.
Okpara LA1, Oriji PC1*, Obagah L1
1Department of Obstetrics and Gynaecology, Federal Medical Centre, Yenagoa, Bayelsa State, Nigeria.
*Correspondence: Dr. Oriji, Peter Chibuzor; +234 706 423 3209; firstname.lastname@example.org
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Background: VACTERL association is a condition comprising multisystemic congenital malformations, causing severe physical disability in affected individuals and is usually a great burden to the family and challenging for the paediatrician. VACTERL/VACTER is characterized by presence of vertebral defects, anal atresia, cardiac defects, trachea-oesophageal fistula, renal anomalies and limb abnormalities.
Case presentation: A live male baby was delivered at the labour ward of the Federal Medical Centre, Yenagoa, following preterm pre-labour rupture of foetal membranes at 30 weeks’ gestational age with multiple congenital malformations. The neonate was immediately transferred to the special care baby unit (SCBU) of the facility for expert care. Baby died within four hours of life.
Conclusion: VACTERL association is a very rare congenital anomaly. Aetiology is unknown and diagnosis is made on clinical picture. Management is quite challenging and multidisciplinary approach is needed to optimize neonatal survival.
Keywords: VACTERL association, Congenital malformations, Multidisciplinary.
Cite this article: Okpara LA, Oriji PC, Obagah L. A rare case of VACTERL association at the Federal Medical Centre, Yenagoa, Bayelsa State, Nigeria. Yen Med J. 2020;2(2):81–83.
- Sadler TW. Birth defects and prenatal diagnosis. In: Langmans Medical Embryology 13th ed. Phildelphia, PA: Wolters Kuwer; 2015:126-140.
- World Health Organisation. Congenital anomalies. World Health Organisation. http://www.who.int/mediacentre/factsheets/fs370/ Accessed February 9, 2020.
- Kumar P. Dysmorphology. In: Kumar P, Burton B, eds. Congenital malformations. Blacklick, OH: McGraw-Hill professional publishing; 2008:3-11.
- Solomon BD, Pineda–Alvarez DE, Raams MS, Bous SM, Keaton AA, Velez JI, Cummings DA. Analysis of component findings in 79 patients diagnosed with VACTERL association. Am J Med Genet A. 2010;152A(9):2236-2244.
- Evans JA, Greenberg CR, Erdile L. Tracheal agenesis revisited: analysis of associated anomalies. Am J Med Genet. 1999;82:415–422.
- Van Rooij IA, Wijers CH, Rieu PN, Hendriks HS, Brouwers MM, Knoers NV, et al. Maternal and paternal risk factors for anorectal malformations: a Dutch case control study. Birth defects. Res A Clin Mol Teratol. 2010,88:152-158.
- Wheeler PG, Weaver DD. Adults with VATER association: Long term prognosis. Am J Med Genet A. 2005;138A:212-217.
- Harris RD, Nyberg DA, Mack LA, Weinberger E. Anorectal atresia: prenatal sonographic diagnosis. AJR Am J Roentgenol. 1987;149:395-400.
- Solomon BD. VACTERL/VATER Association. Orphanet J Rare Dis. 2011;6:56. doi: 10.1186/1750-1172-6-56. Accessed April 13, 2020.
- Oriji PC, Afolabi SA, Omietimi JE, Allagoa D, Doni Doni P, Ozori SE, et al. Aplasia cutis congenita: Uncommon finding of two cases occurring in one patient in two successive deliveries. Yen Med J. 2019;1(1):61-65.
- Lacher M, Froelich S, von Schweinitz D, Dietz HG. Early and long-term outcome in children with oesophageal atresia treated over the last 22 years. Klin padiatr. 2010;222(5):296 -301.
- Levitt MA, Pena A. Outcomes from the correction of anorectal malformations. Curr Opin Pediatr. 2005;17(3):394–401. doi:10.1097/01.mop.0000163665.36798.ac