Table of Contents
Washington University Experience | VASCULAR | Hypoxia-Ischemia, fetal-neonatal | White Matter | 16A0 Case 16 History
Case 16 History ---- The patient was a 1010 gm twin (twin A) born at 29 weeks gestation to a 37 year old G4P2 woman. The pregnancy was complicated by placenta previa at 24 weeks treated with bed rest and premature labor treated with magnesium sulfate. Steroids were administered prior to delivery. Delivery was by C-section under general anesthesia because of placenta previa and difficult labor. Apgars were 8 and 9 at l and 5 minutes, respectively. The patient was intubated at 7 minutes of life for retractions and cyanosis and then transferred to the NICU. During the first week of life, the patient required high ventilator settings and pavulon for respiratory distress and a dopamine drip (x 3 days) because of low blood pressure. Pneumothoraces necessitated placement of multiple chest tubes. A patent ductus arteriosus was treated unsuccessfully with chemical therapy and subsequently ligated on day of life #6. Hyperbilirubinemia was treated with phototherapy. Her course during the first six months of life was notable for thrombocytopenia of unknown etiology treated with transfusion, and renal tubular dysfunction associated with acidosis requiring bicarbonate infusion. At age 10 months, she had markedly elevated transaminase ( 3000-4000) attributed to possible hepatic injury. Head ultrasound on day of life 1 was normal. On day of life 3 ultrasound showed left subependymal hemorrhage and on the day of life 13 showed bilateral intraventricular hemorrhage and ventriculomegaly. The hydrocephalus was treated initially with diamox and serial lumbar punctures without success. She had a ventriculostomy placed on day of life 26. Her subsequent course was marked by numerous shunt infections, isolation of the lateral ventricles requiring placement of shunts in both lateral ventricles, and a trapped fourth ventricle. Two attempts at placing a shunt into the fourth ventricle were unsuccessful because of bleeding when dura was incised. She also has numerous episodes of sepsis, the most notable of which was Klebsiella meningitis and sepsis on day of life 97. This was associated with left focal seizures. Whereas her neurologic examination had been described as normal prior to this episode of sepsis, she had a gradually and intermittently deteriorating neurologic exam after. On day of life 118, she was noted to have fisted hands, intermittent left gaze preference and diffusely increased tone. She subsequently developed right exotropia and roving movements suggesting blindness. On day of life 395 the decision was made to not aggressively resuscitate the patient. On day of life 400 she developed episodes of bradycardia and choking/aspiration attributed to possible brainstem compression. A gastrostomy tube was placed for feeding. She also developed severe bronchospasm and clinical evidence of congestive heart failure. She expired in the NICU at age 14 months. ---- At autopsy she experienced many of the neurological complications of premature delivery. Within three weeks of delivery at 29 weeks estimated gestational age the infant had experienced subependymal hemorrhage, intraventricular hemorrhage and ventriculomegaly. At autopsy she had severe leukomalacia which also may result from premature birth. The immediate cause of death was cerebellar herniation and brainstem compression secondary to fourth ventricular hydrocephalus. The hydrocephalus was due to several factors including acquired aqueductal stenosis and poor CSF absorption secondary to the aforementioned intraventricular hemorrhage and recurrent meningitis.
