- Elevated ICP: Raised ICP dominates the clinical picture. Headaches are the most common symptom (up to 95% of cases), followed by vomiting, nausea, and lethargy. Papilledema is evident late in the course of the disease, especially if hydrocephalus is associated.
- Ataxia and gait disturbances: Ataxia and gait disturbances are the most frequent neurological deficits, followed by dysmetria, nystagmus, cranial nerve palsy, dysarthria, dysphagia, hypesthesia, and hyperreflexia. Motor deficits (e.g., hemiparesis or paraparesis) are rarer.
- Neck pain: Hemangioblastomas extending to the cervical spinal cord may be complicated by neck pain. Patients with such lesions may present with torticollis. Facial flushing is typical of patients with erythrocythemia (48).
- Sudden onset of symptoms: Acute tumor hemorrhage may occasionally occur, which is typically associated with the rapid development of symptoms referable to the location of hemorrhage.
Patterns of evolution
- Unpredictable: The clinical evolution is unpredictable (53). The growth pattern is generally subtle and progresses slowly. Possible sudden clinical onset depends on quick tumor cyst enlargement and/or peritumoral edema and/or hydrocephalus.
- Frequently asymptomatic: According to Ammerman et al., the tumor size and/or its growth rates are the main factors predicting symptom development (1). Cerebellar hemangioblastomas (tumor + peritumoral cyst) > 69 mm3 and with combined growth rates > 14 mm3/month are prone to cause symptoms and to require surgery. The same is true for brainstem hemangioblastomas > 245 mm3 (7.9 mm in diameter) and growth rates > 0.07 mm3/month.
Time for evolution
- 13 –24 months: The duration of symptoms before diagnosis is typically between 13 and 24 months (14, 59).
Evaluation on Presentation
- See EVALUATION
- Treatment for elevated ICP and hemorrhage: Stabilization is required in children admitted with severe symptoms/signs resulting from markedly elevated ICP and/or tumor hemorrhage and/or brainstem injury (orthostatic hypotension, motor and/or cranial nerve deficit, respiratory failure, somnolence or coma). Such patients should be admitted to the pediatric intensive care unit.
Preparation for definitive intervention, nonemergent
- Brain and spinal cord MRI: MRI of the brain and spinal cord with gadolinium administration is performed to investigate the tumor and rule out associated lesions.
- Medical therapy: Medical therapy is administered to relieve symptoms and allow the preoperative examinations to be completed without clinical worsening (see below).
- Surgical tumor removal: Surgical excision is planned as soon as the preoperative work-up is completed. Prophylactic excision of small, accessible multiple hemangioblastomas should be considered even though the lesions may be asymptomatic.
Preparation for definitive intervention, emergent
- Physical examination: Physical examination is carried out as quickly as possible after stabilization.
- Neuroimaging examination: MRI or, otherwise, CT scan is obtained urgently.
- Steroid and diuretic therapy: Steroids and diuretics are started as soon as the diagnosis has been obtained (see doses below).
- Surgery: Surgery is performed emergently to decompress the posterior fossa, remove the hemangioblastoma, and treat hydrocephalus if it is present. If a specialized surgical team is not available immediately, consideration can be given to treating hydrocephalus via ventriculostomy placement, with the possible risk of upward cerebellar herniation being taken into account.
Medical therapy for elevated ICP may include one or more of the following:
- Dexamethasone: 0.2–0.3 mg/kg IV every 8 hours
- Mannitol: 0.25–1.0 g/kg IV bolus, then 0.25 g/kg every 6-8 hours
- Furosemide (in association with mannitol): 1 mg/kg every 6 hours
- Hypertonic saline: Titrated to increase sodium level and/or lower ICP if ICP is being monitored.
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