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Published on: October 20, 2017
Intramedullary cavernoma presenting with hematomyelia: report of two girls
Erwin M J Cornips1, Pauline A C P Vinken, Mariel Ter Laak-Poort
1Department of Neurosurgery, Maastricht University Medical Center, P. Debyelaan 25, P.O. Box 5800, 6202 AZ, Maastricht, The Netherlands. e.cornips@mumc.nl
Insights
Intramedullary cavernomas (ImC) in children are rare, but prompt surgical intervention can lead to good outcomes. Early treatment of pediatric ImC, even with severe deficits, offers a positive prognosis.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Vascular Malformations
Background:
- Intramedullary cavernomas (ImC) are rare in children, with fewer than 20 reported cases.
- Cases with unfavorable outcomes may be underreported, particularly in female patients.
- This study presents two pediatric cases of ImC presenting with acute, severe neurological deficits.
Observation:
- A 10-year-old girl experienced sudden paraparesis and paraplegia due to a thoracic ImC and hematomyelia.
- A 7-year-old girl developed cervicalgia and hemiparesis from a cervical ImC and hematomyelia.
- Both patients underwent surgical intervention, with one requiring immediate surgery and the other after initial recovery.
Findings:
- The 10-year-old girl achieved independent ambulation and continence post-surgery with no recurrence at 2 years.
- The 7-year-old girl showed gradual recovery with minimal residual deficit and became asymptomatic.
- The syndromal case exhibited intermittent symptoms and residual cavernoma, which eventually resolved.
Implications:
- Symptomatic ImC in children can have a surprisingly good prognosis with adequate surgical treatment.
- Surgical intervention, whether acute or delayed, can lead to significant neurological recovery.
- Subtotally resected lesions and syndromal cases may require further monitoring and treatment due to recurrence risk.
Introduction:
Less than 20 children with intramedullary cavernoma (ImC) have been reported in the English literature; however, cases with an unfavorable outcome may be underreported. Whereas these are predominantly boys, we report two girls who presented with hematomyelia (one cervical, one thoracic) and an acute, severe neurological deficit.
Case Material:
A 10-year-old girl complaining about lower thoracic pain for several days suddenly developed lower body dysesthesias and paraparesis. Magnetic resonance (MR) demonstrated hematomyelia (T8-T11), intramedullary edema (T6-L1), and an ImC at T9-T10. Within an hour, she progressed to paraplegia and was therefore operated immediately. She slowly recovered regaining independent ambulation and continence. MR after 2 years shows no recurrence. A 7-year-old girl suddenly developed cervicalgia and paresis of her left arm and leg. MR demonstrated hematomyelia and an ImC at C4-C6. She gradually recovered with minimal residual deficit at 3 months and was subsequently operated uneventfully. Multiple cerebral cavernomas and a familial autosomal cavernous malformation syndrome were diagnosed. The following 1.5 years, she complained of intermittent cervicalgia and left brachial dysesthesias, with MR suggesting active residual cavernoma. Interestingly, her complaints gradually disappeared, and she is currently asymptomatic. MR after 3.5 years shows minimal cord swelling no longer suggesting active residual cavernoma.
Conclusion:
With adequate surgical treatment either in the acute phase in case of dramatic deterioration or after clinical recuperation, prognosis of symptomatic ImC may be surprisingly good. However, subtotally resected lesions and/or syndromal cases may recur, requiring further treatment. Definitive answers await more cases with longer follow-up.
