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Washington University Experience | NEOPLASMS (GLIAL) | Subependymal Giant Cell Astrocytoma (SEGA) | 19A0 Case 19 History
Case 19 History ---- This was a 38-5/7 week gestation infant girl born to a 22-year-old G2P2 mother. Pregnancy was complicated by antenatal diagnosis (at 32 weeks gestation) of a large intracardiac ventricular tumor with multiple smaller cardiac tumors as well as hydrops fetalis. There was concern for pulmonary hypoplasia secondary to compression by the mass and she was given a poor prognosis. She had improvement in ascites, subcutaneous edema, pleural and pericardial effusions without intervention. Fetal MRI showed bilateral subependymal nodules and lesions consistent with cortical tubers. Two days prior to delivery there was worsening of her pericardial effusion. She was delivered by C-section with Apgars 1, 2, and 5 at one, five, and fifteen minutes. She required intubation within the first two minutes of life with inspiratory pressures up to 30, was hypoxemic into 50s and 60s, required two minutes of chest compression, 3 doses of epinephrine, and pericardiocentesis at 45 minutes of life, which aspirated 5-8 ml fluid. CXR showed a mass in the entire left hemithorax with mediastinal shift to the right. Shortly after admission to the NICU, she developed a cardiac arrhythmia. She remained acidotic and hypoxemic and the family withdrew care. She died 5 hours after delivery. Diagnoses included intracardiac tumor, likely rhabdomyosarcoma, and tuberous sclerosis. There is no family history of tuberous sclerosis or of congenital heart disease.
