HYDROMETROCOLPOS IN TWO NEONATES

K. Smets. B. De Wilde

Dept. neonatology. Gent University Hospital, Belgium

CASE 1: twin pregnancy after ICSI; at 32 weeks: cystic abdominal mass, hydronephrotic unique kidney in one fetus; at 35 weeks: ROM, breech, abdominal mass for which caesarean section: girl; Apgar 1/6; distended abdomen; short palpebral fissures; asymmetric implantation of the nipples; bifid right thumb; anal atresia; single urogenital orifice; inferior vena cava compression syndrome. Imaging: ultrasound: cystic mass: thin walled, midline position, posterior to the bladder, diameter 6.5 cm, fluid-debris level; hydronephrotic unique left kidney; RX: opacified abdominal mass; displaced bowel gass; skoliosis of lower lumbal and sacral spinal column; hemivertebrae L4-L5; 13 pairs of ribs. Endoscopy via urogenital sinus after evacuation of 160 mL fluid: remnants of vaginal membrane, dilated vagina, rectovaginal fistula. Vesicoscopy: single ureteric orifice; short urethra, connected with vagina under 90° angle. Management: surgical colostomy and vesicostomy.

CASE 2: at 34 weeks gestation dilated 'bladder'; at 35 weeks ROM; caesarean section for breech presentation; girl; Apgar 9/9; small ears, distended abdomen, firm mass pelvis to umbilicus, anal atresia, single urogenital orifice. Imaging: ultrasound: cystic midline mass with fluid-debris level; mild hydronephrosis; urinary bladder not visualized; RX: displaced bowel gass. Endoscopy via urogenital sinus after evacuation of 90 mL fluid: vaginal membrane, dilated vagina and uterus, rectovaginal fistula. Management: surgical colostomy and vesicostomy.

DISCUSSION: incidence: 1/16.000 female births, 1/60.000 live and still births.

Definition: vaginal obstruction (atresia, stenosis, imperforate hymen, membrane) + excessive mucus production + vaginal dilatation (HYDROCOLPOS) or uterine + vaginal dilatation (HYDROMETROCOLPOS).

Associated malformations: genital, urological (renal agenesis, ectopic ureter), gastrointestinal (anal atresia, esophageal atresia, duodenal atresia), skeleton (polydactyly, sacral hypoplasia), fistulas (rectovaginal, urogenital), heart, brain.

Diagnosis: prenatally: cystic intra-abdominal mass (DD distended urinary bladder, hydronephrosis, ovarian cysts, reduplication of the sigmoid, sacrococcygeal teratoma, mesenteric cysts, cystic tumors, anterior meningoceles), associated malformations; postnatally: clinical presentation (distended abdomen, abnormal external genitalia-single urogenital orifice, associated malformations), ultrasound, X ray, endoscopy.

Management: postnatal drainage of vagina, uterus and urinary bladder or prenatal drainage for decompression of distended genital organs, reduction of urinary obstruction and enhancement of ultrasonographic image; derivation of intestinal tract (colostomy); permanent (anatomic) reconstruction at the age of six months.