gms | German Medical Science

Objective: Cavernous hemangiomas (CH) are vascular malformations that usually can be operatively removed with a justifiable risk. Here, we discuss two special situations: Cavernomas presenting as pediatric skull tumors and in eloquent regions.

Methods: We reviewed the medical records and imaging studies of 40 patients (18 women and 22 men) with CHs in eloquent regions and of two 6- and 8-month old babies with solitary and multifocal congenital CHs of the skull, who underwent surgical resection. The cerebral CHs were located in the following eloquent regions: sensorimotor system (n=13), brainstem (n=9), language areas (n=6), cerebellum (deep nuclei) and peduncles (n= 5), basal ganglia (n=5) and visual pathways (n=4). Long-term follow-up of the CHs was available in 33 patients (82,5%) with a follow-up to 80 months. Outcome was evaluated using the modified Rankin scale. Additionally, we analyzed the clinical and radiological signs, surgical approach and clinical course of the two pediatric patients.

Results: Total resection was achieved in all patients with CH in eloquent regions without surgery-related mortality. Neurological deterioration occurred immediately after surgery in 11 patients (27,5%). Thirty patients (91%) had no new deficits or experienced improvement at the last follow- up. According to the modified Rankin scale 13 patients (42%) had a slight (≥ 1 point) and 9 patients a moderate (≥ 2 points) impairment. 27 patients (81%) could live independently or recovered completely. 2 patients (6%) suffered severe permanent neurological deficit after surgery for a CM located in middle cerebellar peduncle and brainstem, respectively. Postoperative improvement was correlated with good preoperative neurological status and younger age. Adverse factors for the occurrence of surgical morbidity were pontine or brainstem location, smaller size of the CM, preoperative hemorrhage, poor neurological status and age. Both pediatric patients suffered from painless slow- growing bone lesions in the left frontal skull involving superior sagittal sinus. An 8-month-old male infant had an additional CH arising from the right parietal skull. The skin over the skull lesions had a yellow appearance. Each tumor had a diameter of 3,0 cm and was of a tender consistency. On the T1- and T2-weighted MR images the bone lesions were hypointense involving inner and outer table of the skull with and without enhancement after contrast administration. Because of tumor progression it was decided to resect the affected bone en bloc. The diagnosis of CH was histologically confirmed. The follow-up was in both cases uneventful.

Conclusion: In our experience in most patients CHs located in eloquent regions can be removed safely. Transient impairment may occur in up to one third of patients, but permanent morbidity is rare. Removal for CHs in eloquent regions may be considered on a case-to-case basis. Patients with brainstem CHs or poor neurological status face a higher risk for postoperative morbidity. Skull CH should be considered in the differential diagnosis of osseous tumors. We recommend complete removal of these lesions to achieve a diagnosis and a permanent cure.