Abstract :
2hrs old female, delivered at 38 weeks of gestation with abdominal distension with antenatal Ultrasound suggestive of 12*6 cm cystic mass in lower abdomen, mild hydronephrosis, Bilateral Renal pyelectesis. On examination she had lower abdominal distension, vulval oedema, lower limb oedema and also all four limb polydactyly. Post-natal Ultrasound examination revealed the presence of hypoechoic and corpusculated 12*6 cm cystic lesion in the midline with thick wall in continuity with vagina suggestive of Large hydrometracolpos with bilateral mild hydronephrosis, both ovaries not visualised. Hysteroscopic examination showed obstruction due to imporforated hymen and narrowing at level of cervix and very thick mucoid secretion was found and drainage of about 100ml milky thick mucoid collection under USG guidance was done. 2DEcho was normal. According to these physical features, baby is likely to have McKusick-Kaufman syndrome.
Keyword :
Congenital hydrometrocolpos, Pelvic mass, Hydronephrosis, Polyductyly.