Clinical features and long-term outcomes of pediatric spinal cord cavernous malformation-a report of 18 cases and literature review

Purpose: Pediatric intramedullary spinal cord cavernous malformation (ISCM) is a rare vascular disease with unclear natural history and long-term outcomes. We aim to determine the demographics, hemorrhagic risk, and long-term outcomes of this rare entity.

Methods: A retrospective review of clinical data and treatment outcomes of pediatric patients treated with ISCM in our institution from 3/2000 to 3/2017 was conducted. In addition, we performed a systematic review of the literature on pediatric ISCM.

Results: Eighteen consecutive pediatric patients were included, with an average age of 12.9 ± 4.7 years (range: 4-18 years) and 66.7% being male. Locations were equally distributed in cervical and thoracic segments, with mean extension of 1.3 ± 0.7 segments. Clinical manifestation included extremity weakness (n = 15, 83.3%), pain (n = 10, 55.6%), sensory disorders (n = 8, 44.4%), sphincter disturbance (n = 6, 33.3%), muscular atrophy (n = 3, 16.7%), and spinal deformity (n = 1, 5.6%). Most patients presented with acute symptoms (n = 11, 61.1%), and 7 (38.9%) of them had severe neurological deficits. The annual retrospective hemorrhagic risk was 7.7 per patient-year. Two patients received conservative management, with one improved neurologically and the other remained unchanged. Total resection was achieved in 12 (75%) of the 16 surgical cases, with 8 patients (50%) improved their clinical outcomes, 7 patients (43.8%) remained unchanged, and 1 (6.3%) worsened. During follow-up, one patient had relapse of ISCM.

Conclusion: Pediatric ISCM appears to have higher hemorrhage risk than their adult counterparts, and they can benefit from surgery whether in the acute phase of neurological deterioration or after clinical recuperation.

Keywords: Cavernous malformation pediatric spinal cord natural history.