Pediatric cavernous malformation in the central nervous system: report of 66 cases

Abstract

Objective: To investigate the clinical features and treatment outcome of pediatric cavernous malformation (CM) in the central nervous system.

Patients and methods: Retrospective review of clinical data and treatment outcome for 66 pediatric patients under the age of 18 treated for CM at Huashan Hospital within the past 10 years from January 1998 to December 2007.

Results: In our consecutive series, the mean age at onset of the CM was 11.6 years (age from 15 months to 17.8 years), and the boy-to-girl ratio was 1.54:1 (40:26). Main initial clinical manifestations in cerebral CM (CCM) included: seizures (47.7%, 31/65), headache (46.2%, 30/65), intracranial hemorrhage (20%, 13/65), and focal neurological deficits (12.3%, 8/65). There were 59 children with single CM and 7 children with multiple CMs: 55 (83.3%) with supratentorial CM, 6 (9.1%) with infratentorial CM, 4 (6.1%) with supra- and infratentorial CM, and 1 (1.5%) with intramedullary CM. Sixty-two (93.9%; 62/64) children underwent microsurgical operations, and with the help of neuronavigation (19 cases), intraoperative ultrasonography (6 cases), and neuronavigation combined with intraoperative ultrasonography (3 cases). And 4 (6.1%; 4/66) children did not undergo any surgical intervention. There was no major morbidity or mortality from surgical procedures. Follow-up data of 50 children was available with a mean follow-up time of 39.1 months (range from 5 to 112 months), including 46 operated children and 4 children managed without operation. In the 46 operated children, the overall long-term post-treatment results were satisfactory: 73.9% no sign or symptom associated with CMs, 19.6% improved obviously, only 1 (2.2%) boy with unrestored paraplegia incurred by spinal cord CM, and 2 boys (4.3%) with controllable seizures occurring after initial 5 symptom-free years (one without need of antiepileptic drugs, AEDs). Of 24 children followed-up with preoperative seizures, only 1 boy needed therapeutic AEDs and the others did not need any after resections of the CMs. Three children managed without operations (2 with headache and 1 with seizures) had spontaneous disappearance of symptoms and stable intracerebral lesions at follow-up MRI images.

Conclusions: For symptomatic solitary CM, the treatment of choice is complete microsurgical excision preceded by careful anatomical and functional evaluation, and the risk of operation can be decreased to lower level with the help of neuronavigation and intraoperative ultrasonography. For multiple CM, the treatment modalities must be considered cautiously. A much longer follow-up remains mandatory for appropriate treatment strategies.