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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2013; 23(02): 183-185
DOI: 10.4103/0971-3026.116573
ORIGINAL ARTICLE

The McKusick-Kaufman hydrometrocolpos-polydactyly syndrome: A rare case report

Swapnil P Yewalkar
Department of Radiodiagnosis, B.J.M.C. and S.G.H., Pune, Maharashtra
,
Vikas K Yadav
Department of Radiodiagnosis, B.J.M.C. and S.G.H., Pune, Maharashtra
,
G J Khadse
Department of Radiodiagnosis, B.J.M.C. and S.G.H., Pune, Maharashtra
› Author Affiliations Source of Support: Nill.
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Abstract

A combination of hydrometrocolpos, polydactyly, and cardiac anomlies is the cardinal feature of McKusick-Kaufman syndrome. Neonatal hydrometrocolpos is rare Mullerian duct anomaly caused by obstruction of the vagina. There is an accumulation of mucus secretions proximal to the obstruction. The secretions are secondary to intrauterine and postnatal stimulation of uterine and cervical glands by maternal estrogens. Here, we report a case of 1-day-old neonate who presented with abdominal distension. Ultrasound and computed tomography findings revealed a large abdominopelvic cystic mass posterior to the urinary bladder. Other associated findings were polydactyly and bilateral hydronephosis. Laparotomy confirmed the findings of hydrometrocolpos caused by stenosis/atresia of lower vagina.

Keywords

Hydrometrocolpos - McKusick–Kaufman syndrome - polydactyly - ultrasonography - Computed tomography


Publication History

Article published online:
04 October 2021

© 2013. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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