Article
Spontaneous intramedullary hemorrhage of a small brainstem hemangioblastoma in a patient with von Hippel-Lindau disease
Spontane intramedulläre Blutung eines kleinen Hirnstammhämangioblastoms bei einem Patienten mit von-Hippel-Lindau-Syndrom
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Published: | May 8, 2006 |
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Objective: Spontaneous hemorrhages in hemangioblastomas are rare. Most bleedings are located in the subarachnoid space. The rare intraparenchymal bleedings are found supratentorially, infratentorially or in the spinal cord. We report an extremely rare case of a brainstem hemorrhage due to a small hemangioblastoma of the medulla oblongata.
Methods: A 47-year-old patient suffered from acute onset of headache, anarthria, inability to swallow, left-sided hemiparesis and hemidysesthesia with changing states of vigilance finally developing acute respiratory failure. Computed tomography (CT) scanning and magnetic resonance imaging (MRI) revealed a small 4 mm hemangioblastoma in the posterior medulla oblongata causing an intraparenchymal hemorrhage and acute triventricular hydrocephalus due to intraventricular hematoma extension.
The acute hydrocephalus was treated by an external ventricular drainage. The intramedullary hemorrhage as well as the hemangioblastoma were removed microsurgically through a posterior median suboccipital approach with minimal foramen magnum enlargement.
Results: Six months after surgery the patient showed good recovery with only slight neurological deficits. He could breathe normally without assistance and spoke with a slightly guttural voice. His difficulty in swallowing was confined to very solid food. He had a minor residual hemiparesis and hemidysesthesia, and was able to ambulate freely and to carry out daily household chores without or with minimal support.
Conclusions: Besides spontaneous intramedullary bleedings usually caused by arterial hypertension or cavernous angiomas, hemangioblastomas should well be taken into consideration as another possible cause of a spontaneous intraparenchymal brainstem hemorrhage. Especially in hemangioblastomas the surgical management strategy is crucial for a successfull result. Therefore the search for a hemangioblastoma should be included into the diagnostic work up in patients with brainstem bleedings.