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Washington University Experience | VASCULAR | Hydrocephalus, Post-Hemorrhagic | 3A0 Case 3 History
Case 3 History ---- This male neonate was born at 34 weeks estimated gestational age to a 15 year old G1, P0 mother whose pregnancy was complicated by pre-eclampsia, polyhydramnios and treated chlamydia. A fetal ultrasound performed at approximately week 22, was reportedly interpreted as normal. Maternal serologies were unremarkable. The patient's mother presented to Barnes Jewish Hospital Labor and Delivery (L&D) after premature rupture of membranes. The patient was delivered via cesarean section following development of non-reassuring fetal heart tones and decelerations. At delivery, he was noted to have generalized edema and to show no movement or respiratory effort. Initial APGAR scores were 2, 3, 3 and 4 (at one, five, ten, and fifteen minutes, respectively). The patient was intubated, instilled with surfactant, and transferred to the St. Louis Children's Hospital Neonatal Intensive Care Unit (SLCH NICU) with an initial diagnosis of non-immune hydrops. Initial physical exam in the NICU showed generalized edema, weight 2.49 kg, length 48 cm, head circumference 35 cm, temperature 37C, heart rate 120 bpm and blood pressure 66/36. Examination of his head showed widely spaced sutures, and dysmorphic features including low set and posteriorly rotated ears and clinodactyly. The patient took no spontaneous breaths. The abdomen had absent bowel sounds. The skin showed petechiae throughout, clinically interpreted as DIC, and was treated with blood products. The neurologic exam was remarkable for lack of spontaneous movement, hypotonicity, absent suck reflex, absent pupillary reflexes, absent corneal reflexes and absent gag reflex. Head ultrasound, performed on day 1, revealed intraventricular and right parenchymal hemorrhage with blood extending into the third and fourth ventricles which were enlarged, consistent with hydrocephalus. EEG demonstrated continuous electrographic seizure activity which responded to fosphenytoin, phenobarbital and levetiracetam. Efforts to identify the cause of the patient's hydrops were unrevealing. Echocardiographic examination was unremarkable, TORCH serologies were negative, multiple blood cultures showed no growth. DIC resolved after multiple transfusions, well before his death. Genetic screening identified no chromosomal abnormalities. Biochemical evaluation revealed normal serum amino acids and organic acids. A skeletal survey was notable for thin long bones and bilaterally dislocated elbows. Through the course of the hospitalization, the patient was entirely ventilator dependent, only over-breathing the ventilator settings initially. In the days prior to death the patient became oliguric, and showed worsening anasarca. The decision was made not to escalate care and the patient died. The patient’s age at death was 21 days.
