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Washington University Experience | NEOPLASMS (MESENCHYMAL, NON-MENINGOTHELIAL) | Hemangioblastoma - Von Hippel Lindau | 29A0 Case 29 History
Case 29 History ---- The patient was a 58 year old male with von Hippel-Lindau syndrome and multiple intracranial hemangioblastomas (diagnosed in 8/76). The patient’s surgical history began with a large posterior fossa hemangioblastoma treated with stereotactic radiosurgery. Surgical resection was not offered at that time because of the patient's poor pulmonary status. An MRI performed two months prior to the patient's demise showed the posterior fossa mass to be enlarging. A VP shunt had been placed for hydrocephalus approximately one year previously. He was also status-post bilateral adrenalectomy for pheochromocytomas. Additionally, he was status-post laser surgery to the right eye (x3) for recurrent hemangioblastoma. The patient was admitted in 10/1/94 for dyspnea and cough productive of phlegm. There was no associated history of headaches, seizures, visual defects or changes in bowel or bladder function. On examination, he was afebrile and in mild respiratory distress. A resting tremor was observed on neurological examination, as well as nystagmus with lateral gaze. A chest x-ray on admission was without abnormality. However, by 10/4/94 he had developed right upper lung infiltrates, fever, and purulent sputum, thought to be consistent with aspiration pneumonia. He was placed on IV antibiotics. A barium swallow showed evidence of chronic reflux and a VJ tube was placed. The patient's hospital course was complicated by deteriorating pulmonary function. He was intubated for respiratory failure. Sputum culture was positive for pseudomonas and Klebsiella pneumonia. He was coded on 10/17 /94 and resuscitated. A tracheotomy was performed. Shortly thereafter the patient became febrile and hypotensive and died.
