Cerebellar cavernous malformation

Cerebellar giant intracranial cavernous malformation are extremely rare, with only seven cases reported based on English literature. These lesions are most commonly seen in the pediatric age group, which is known to have an increased risk of hemorrhage.

They are in children symptomatic lesions.

Radiologically and clinically they can mimic a tumor with bleed, having to be considered in the differential diagnosis of neoplastic lesions. Cerebellar GCMs might be suspected in the presence of large hemorrhagic intra-axial mass with “bubbles of blood,” multi-cystic appearance, surrounded by hemosiderin rim, fluid-fluid levels, and accompanying edema-mass effect. Careful radiological study provides a preoperative diagnosis, but its confirmation requires histopathological examination. Complete surgical removal should be attempted when possible ¹⁾.

41 patients with identified CCMs were retrospectively reviewed. Of these, 11 patients (26.8%) were found to have associated developmental venous anomaly. Zhang et al. compared the clinical profile of the two groups of patients (CCMs with and without DVAs). The CCMs with DVAs cases underwent radical resection of the CCMs, and the distal radicles of the DVAs that directly drain from the CCMs were coagulated and dissected at the length of the CCMs.

There were no statistically significant differences between the two groups with regard to age, sex, location and size of lesions, multiplicity, and surgical prognosis. The patients with CCMs with DVAs did not experience any brain swelling or hemorrhagic tendency intraoperatively. The postoperative course was uneventful for all of the 36 surgical patients with the exception of two of the patients with CCMs with associated DVAs, who suffered from serious cerebellar edema, and one of these two patients underwent an emergency suboccipital decompression craniotomy. With the exception of three patients who were lost to follow-up (mean, 22.3 months), all of the CCMs patients exhibited good long-term prognosis (modified Rankin scale values of 0-2) and no reoccurrence.

It is not rare that associated DVAs occur in CCMs. The total removal of the CCM combined with the coagulation and dissection of the distal radicles of DVA at the length of the associated CCM may result in good long-term prognosis in patients ²⁾

Tyagi et al. from Bengaluru, India, reported a 15-year-old boy presented with simultaneous right frontal and cerebellar hemorrhage in the superior vermis with hydrocephalus. The patient underwent a ventriculoperitoneal shunt, and his magnetic resonance imaging (MRI) revealed multiple cavernomas with bleed in the above-mentioned locations. The patient underwent a midline suboccipital craniotomy and excision of the cavernoma. The supratentorial lesions were left in situ in lieu of small size, no history of seizures, mass effect, or other neurological deficits. The patient recovered well from surgery with significant improvement in truncal ataxia. He remained asymptomatic for supratentorial lesions at follow-up. Cavernomas should be considered as differential diagnoses in cases of multiple intraparenchymal hemorrhages, especially in pediatric patients. The surgical management should be rationalized based on the lesion location, the eloquent of the surrounding parenchyma, mass effect, and the risks of re-rupture. Due to the rarity of multiple simultaneous hemorrhages, the management of multiple cavernomas remains controversial. The patient's relatives can be screened with MRI to rule out the familial form of the disease. Strict clinical and radiological follow-up is a must in such patients ³⁾.

A case of a healthy young adult, who presented with acute onset of headache, dizziness, and nausea with intermittent episodes of vomiting for four days. Brain tomography imaging at presentation revealed likely multiple foci of intracranial hemorrhage; however, magnetic resonance imaging (MRI) showed findings suggestive of an underlying cavernoma that had bled, in addition to a coexisting DVA. The patient was discharged home with no deficits. Outpatient follow-up five months later revealed no symptoms or neurologic deficits ⁴⁾.

An 18-month-old girl presented with a 6-month history of cervical torticollis and upper extremities clumsiness. An MRI revealed a 57 × 46 × 42 mm multi-cystic, left cerebellar hemisphere mass, showing areas of hemorrhages and cysts with various stages of thrombus. There was no enhancement with contrast. Cerebral angiography ruled out an arteriovenous malformation. She underwent a left paramedian occipital craniotomy, and macroscopic gross total resection was accomplished.

Histopathologic examination was consistent with a cavernous malformation. After surgery, the patient had no new neurological deficit and an uneventful postoperative recovery. Follow-up MRI confirmed total removal of the lesion ⁵⁾.