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Urinary Hydrocolpos, Cloacal Malformation and Pre-Axial Polydactyly: A Rare Variant of Neonatal Hydrocolpos

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Abstract

Hydrocolpos is characterized by a vaginal obstruction with cystic dilatation of the vagina. The latter is usually caused by accumulation of cervical and endometrial mucus but in rare instances urine is accumulated through a vesicovaginal fistula proximal to the obstruction. Hydrocolpos and hydrometrocolpos may be associated with other malformations, such as postaxial polydactyly, anal atresia, esophageal atresia, renal agenesis, genital anomalies, and cardiopathy. Each neonate presenting with hydrocolpos should be evaluated for other clinically silent malformations, such as hamartoblastoma of the hypothalamic region. We report a patient with urinary hydrocolpos and cloacal malformation; it is the first case of hydrocolpos with pre-axial polydactyly. We briefly describe embryogenesis of the different types of vaginal obstruction and discuss prenatal and neonatal diagnosis and differential diagnosis.

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... The secretary type contains mucoid viscid material secreted by cervical uterine glands. The urinary variety contains urine which gets collected in the vagina due the unidirectional flow of urine via the urethrovaginal fistula above the level of obstruction [2,26,27]. The communication is usually small and valvular, allowing only oneway flow of urine into the vagina. ...
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1. 1. Congenital hydrocolpos is caused by a combination of vaginal obstruction and fetal cervical gland secretion caused by stimulus of the maternal hormones. 2. 2. The vaginal obstruction may be caused by atresia, imperforate hymen, or possibly by a congenital fold of tissue which causes a valvelike obstruction in a partially stenotic vagina. 3. 3. The most important factor in treatment of this anomaly is the establishment of a correct preoperative diagnosis. 4. 4. When the correct diagnosis is made, most of these patients will be cured by a relatively simple transperineal drainage procedure. 5. 5. Long-term follow-up is essential since the distended, hypertrophic vagina and the associated hydronephrosis tend to develop intercurrent infection during their slow return to normal.