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Intramedullary cavernoma presenting with hematomyelia: report of two girls.
Childs Nerv Syst. 2010 Mar; 26(3):391-8.CN

Abstract

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.

Authors+Show Affiliations

Department of Neurosurgery, Maastricht University Medical Center, P. Debyelaan 25, P.O. Box 5800, 6202 AZ, Maastricht, The Netherlands. e.cornips@mumc.nlNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

19865818

Citation

Cornips, Erwin M J., et al. "Intramedullary Cavernoma Presenting With Hematomyelia: Report of Two Girls." Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery, vol. 26, no. 3, 2010, pp. 391-8.
Cornips EM, Vinken PA, Ter Laak-Poort M, et al. Intramedullary cavernoma presenting with hematomyelia: report of two girls. Childs Nerv Syst. 2010;26(3):391-8.
Cornips, E. M., Vinken, P. A., Ter Laak-Poort, M., Beuls, E. A., Weber, J., & Vles, J. S. (2010). Intramedullary cavernoma presenting with hematomyelia: report of two girls. Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery, 26(3), 391-8. https://doi.org/10.1007/s00381-009-1012-6
Cornips EM, et al. Intramedullary Cavernoma Presenting With Hematomyelia: Report of Two Girls. Childs Nerv Syst. 2010;26(3):391-8. PubMed PMID: 19865818.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Intramedullary cavernoma presenting with hematomyelia: report of two girls. AU - Cornips,Erwin M J, AU - Vinken,Pauline A C P, AU - Ter Laak-Poort,Mariel, AU - Beuls,Emile A M, AU - Weber,Jacobine, AU - Vles,Johannes S H, Y1 - 2009/10/29/ PY - 2009/09/15/received PY - 2009/10/30/entrez PY - 2009/10/30/pubmed PY - 2010/5/15/medline SP - 391 EP - 8 JF - Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery JO - Childs Nerv Syst VL - 26 IS - 3 N2 - 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. SN - 1433-0350 UR - https://www.unboundmedicine.com/medline/citation/19865818/Intramedullary_cavernoma_presenting_with_hematomyelia:_report_of_two_girls_ L2 - https://doi.org/10.1007/s00381-009-1012-6 DB - PRIME DP - Unbound Medicine ER -