Urinary Type Hydrometrocolpos and Polydactyly in Two Newborns: Case Report

Document Type : Case Report


1 Family Health Institute, Maternal-Fetal and Neonatal Research Center, Tehran University of Medical Sciences, Tehran, Iran

2 Breastfeeding Research Center, Tehran University of Medical Sciences, Tehran, Iran

3 Department of Pediatric Surgery, Tehran University of Medical Sciences, Tehran, Iran


Background: Abdominal masses secondary to urinary retention are rare among female neonates and approaching this pathologic condition, inevitably, poses a diagnostic challenge. Hydrometrocolpos is one example of this disease category which is responsible for only 15% of such cases whose association with polydactyly appears to be even less common. The present study aimed to report the accounts of two neonates with cystic abdominal masses diagnosed as urinary type hydrometrocolpos secondary to urogenital sinus anomaly. Autosomal recessive disorders are characterized by vaginal atresia with hydrometrocolpos, polydactyly, congenital heart defects, and non-immune mediated hydrops fetalis. The triad of hydrometrocolpos, polydactyly, and cardiac anomaly in the two patients presented herein is strongly suggestive of a case of McKusick-Kaufman syndrome.
Case report: This study reported two neonates with abdominal mass, polydactyly, and genitourinary tract malformation, with no family history. Relief of urinary obstruction by exploratory laparotomy, aspiration of fluid, and vaginal reconstruction gradually reversed the hydronephrosis and renal failure. 
Conclusion: It can be concluded thatin hydrometrocolpos causing urinary obstruction, decompression of hydrometrocolpos can save the kidneys.


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