Case 4 History ---- The patient was a four day old, 32 weeks EGA, male. The mother was a 24 year old G1P1001 (blood type 0, Rh +, GBS negative, hepatitis B negative, Rubella immune, VDRL non-reactive, gonococcus negative) woman with an abnormal triple screen (elevated maternal alpha-fetoprotein). Chorionic villi sampling showed 46 XY. At twenty weeks of gestation, a fetal bladder outlet obstruction and ascites was noted and an in utero vesicoamniotic shunt was placed x 4. The pregnancy was also complicated by oligohydramnios (amino fusion x 3) and preterm rupture of membranes. At birth he weighed 2500g. He immediately showed respiratory distress (heart rate > 100, retractions) and was intubated with surfactant given. His Apgars were 5 at 1 minute and 8 at 5 minutes. Chest x-ray showed the lung fields to extend only to the T6 level. The bladder outlet obstruction was confirmed and postnatal suprapubic tube was placed. The patient had increased abdominal fluid, hydroureter, and a possible "prune belly syndrome." Over the next four days of the patient's life, he had decreased urine output, increased abdominal fluid, edema, increased creatinine in addition to persistent hypotension. The patient's unconjugated bilirubin ranged from 7.0 mg/dl to 11.3 mg/dl during his hospitalization. Finally, respiratory support was withdrawn and the patient expired.
