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Washington University Experience | PERIPHERAL NEUROPATHY | 1 NORMAL NERVE ANATOMY | 6 Infant Peripheral Nerve | 4A0 Case 4 History
Case 4 History (1wk effective age) ---- The patient was an eight week old male born at 33 weeks gestation by cesarean section due to premature rupture of membranes and concern for oligohydramnios. Abdominal ultrasound on his 1st day of life demonstrated bilateral hydronephrosis and hydroureters with bilateral cystic dysplasia of the kidneys thought to represent the sequelae of in utero bladder outlet obstruction due to posterior urethral valves. His NICU course was complicated by pulmonary hypoplasia secondary to Potter's sequence requiring mechanical ventilation, prune belly with bladder outlet obstruction and megacystis, bilateral pneumothorax, persistent pulmonary hypertension, resolved, extended-spectrum - lactamase (ESBL) Klebsiella tracheitis, electrolyte abnormalities (hyponatremia, hypokalemia, hypophosphatemia), peritoneal dialysis dependent end stage renal disease, anemia with transfusion, marginal somatic growth, congenital hypothyroidism, and metabolic acidosis. On October 13 he developed increased work of breathing, intermittent bradycardia and worsening abdominal distention with significant atelectasis and colonic dilatation on x-ray. Capillary blood gas demonstrated worsening mixed acidosis, primarily metabolic, with pH 6.96. He was additionally cultured and started on broad spectrum antibiotics. He had worsening bradycardia and acidosis with a pH of 6.6. On October 14 he was in pulseless electrical activity. Resuscitation efforts were unsuccessful and he died. His total age was 33 weeks + 14 days - 42 days premature, effectively a 4-week-old infant. These genitourinary tract, abdominal testes and anterior abdominal wall skeletal muscle hypoplasia/atrophy are the triad of findings of the prune-belly syndrome.
