This infant was the product of a twin gestation estimated at 36 weeks by dates and ultrasound. The mother is a 38-year-old gravida 3, para 0020 Caucasian woman. On prenatal ultrasound both infants were found to have enlarged echogenic kidneys with loss of the corticomedullary margins consistent with polycystic kidney disease. Additionally, ultrasound demonstrated severe oligohydramnios. After counseling, the parents elected to carry the children to term. The mother underwent spontaneous rupture of membranes with a forceps assisted vaginal delivery.

The apgar scores of baby Alpha were 1 and 7 at 1 and 10 minutes respectively. The infant was immediately transferred to the neonatal intensive care unit where a renal ultrasound revealed enlarged echogenic kidneys. The patient was placed on ventilatory support and subsequently developed a right pneumothorax which was treated with the placement of a right chest tube. Despite treatment with fluids, the infant failed to produce urine. After discussion with the family a decision was made to withdraw support measures. The patient was extubated on October 19, 2002 and died at 2000 hours.

	Infant with flattened face and loose skin
	Wrinkled skin of hands
	Chest X-ray with pneumothorax, small lungs and enlarged abdomen with upward pressure of the diaphragm
	Opened abdominal cavity showing marked enlargement of the kidneys displacing GI organs.
	After removal of the intestines, you can see the marked enlargement of the kidneys.
	On the left are normal term infant kidneys with fetal lobulations. On the right is the affected kidney from this infant.
	Here the normal (left) and abnormal kidneys are bisected to show the loss of the normal corticomedullary demarcation of the affected kidney.
	Low power view of the affected kidney showing the dilated rays
	Medium power of the dilated cystic structures