A case report of large hydrometrocolpos in neonate

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.

Hydrometrocolpos in an infant with urological complications: a rare case and review of literature

“…Hydrometrocolpos is a rare congenital condition with an incidence of about 0.006% [ 2–4 ]. It can be caused by imperforate hymen, transverse vaginal septum, cloacal malformation, distal vaginal agenesis and persistent urogenital sinus, with the former being the most prevalent congenital anomaly [ 1 , 3 , 5 , 6 ].…”

“…Hydrometrocolpos can be isolated or associated with syndromes such as the Mckusick Kaufman syndrome, Mayer–Rokitansky–Küste–Hauser syndrome and Herlyn–Werner–Wundelich syndrome [ 2–4 , 7 ]. Some cases of hydrometrocolpous may have associated abnormal connection with the urinary tract or gastrointestinal tract (fistulae) [ 5 , 8 ].…”

“…Hydrometrocolpos is characterised by distension of the vagina and uterus as a result of accumulation of fluid due to distal obstruction presenting as a cystic abdominal mass [ 2 , 5 , 7 ]. There are a number of conditions that presents with cystic mass in children including ovarian cyst, cystic sacrococcygeal teratoma, enteric duplication, urachal cyst and cystic renal masses [ 1 , 2 , 8 ]. It can result into various complications due to pressure effect on the surrounding structures.…”

“…It can result into various complications due to pressure effect on the surrounding structures. These include urinary tract obstruction, recurrent urinary tract infection, urine retention (renal insufficiency), rupture of the urinary bladder, sepsis, intestinal obstruction and constipation [ 2 , 4 , 5 , 7 ]. Our case presented both constipation and retention of urine due to a severely dilated uterus and vaginal cavity.…”

“…Hydrometrocolpos is the accumulation of secretions in both the vaginal and uterine cavity. It is a rare condition with an incidence of around 0.006% per year in full-term babies [ 1 , 2 ]. It is caused by the failure of drainage of cervical/vaginal mucous secretions as a result of distal vaginal obstruction.…”