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Symptomatic syringomyelia occurring as a late complication of posterior fossa medulloblastoma removal in infancy in a boy also suffering from scaphocephaly

El Hassani, Yassine ; Burkhardt, Karim ; Delavellle, Jacqueline ; Vargas, Maria-Isabel ; Boex, Colette ; Rilliet, Benedict

In: Child's Nervous System, 2009, vol. 25, no. 12, p. 1633-1637

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    Titre
    • Symptomatic syringomyelia occurring as a late complication of posterior fossa medulloblastoma removal in infancy in a boy also suffering from scaphocephaly
    Auteur
    • El Hassani, Yassine. Service de Neurochirurgie, Hôpital Cantonal Universitaire, 24 rue Micheli-du-Crest, 1211, Geneva 14, Switzerland
    • Burkhardt, Karim. Unité de Neuropathologie, Centre Médical Universitaire, 1211, Geneva 14, Switzerland
    • Delavellle, Jacqueline. Unité de Neuroradiologie Diagnostique, Service de Radiologie, Hôpital Cantonal Universitaire, 24 rue Micheli-du-Crest, 1211, Geneva 14, Switzerland
    • Vargas, Maria-Isabel. Unité de Neuroradiologie Diagnostique, Service de Radiologie, Hôpital Cantonal Universitaire, 24 rue Micheli-du-Crest, 1211, Geneva 14, Switzerland
    • Boex, Colette. Neuromonitoring, Unité d'épileptologie, Service de Neurologie, Hôpital Cantonal Universitaire, 24 rue Micheli-du-Crest, 1211, Geneva 14, Switzerland
    • Rilliet, Benedict. Service de Neurochirurgie, Hôpital Cantonal Universitaire, 24 rue Micheli-du-Crest, 1211, Geneva 14, Switzerland
    Type de document
    • Postprint
    Identifiant OAI-PMH
    • oai:doc.rero.ch:321993
    Summary
    Introduction: The association of a medulloblastoma and a syringomyelia has been already described in rare instances albeit without symptoms related to the syrinx. Case report: The case of a 23-year-old man operated in infancy for a medulloblastoma and then treated solely with adjuvant chemotherapy is reported. He was also operated in infancy for a scaphocephaly. With a very long time delay, he has developed a Chiari I and a symptomatic cervico-dorsal syringomyelia. The symptoms attributed to the syrinx consisted of a unilateral prurigo over the left arm which was so severe to lead to self-mutilation. Discussion: Clinical and magnetic resonance imaging follow-up after cervico-dorsal decompression shows a significant improvement of the symptoms together with a reduction of the size of the syrinx. This case is discussed in the light of the presumed pathophysiology of the syrinx and its exceptional clinical presentation