Schwannosis induced medullary compression in VACTERL syndrome.

Hdl Handle:
http://hdl.handle.net/10147/146399
Title:
Schwannosis induced medullary compression in VACTERL syndrome.
Authors:
Treacy, A; Redmond, M; Lynch, B; Ryan, S; Farrell, M; Devaney, D
Affiliation:
Department of Histopathology, Children's University Hospital, Temple Street, Dublin, Ireland. anntreacy@mac.com
Citation:
Schwannosis induced medullary compression in VACTERL syndrome., 28 (5):384-6 Clin. Neuropathol.
Journal:
Clinical neuropathology
Issue Date:
21-Oct-2011
URI:
http://hdl.handle.net/10147/146399
PubMed ID:
19788055
Abstract:
A 7-year-old boy with a history of VACTERL syndrome was found collapsed in bed. MRI had shown basilar invagination of the skull base and narrowing of the foramen magnum. Angulation, swelling and abnormal high signal at the cervicomedullary junction were felt to be secondary to compression of the medulla. Neuropathologic examination showed bilateral replacement of the medullary tegmentum by an irregularly circumscribed cellular lesion which was composed of elongated GFAP/S 100-positive cells with spindled nuclei and minimal atypia. The pathologic findings were interpreted as intramedullary schwannosis with mass effect. Schwannosis, is observed in traumatized spinal cords where its presence may represent attempted, albeit aberrant, repair by inwardly migrating Schwann cells ofperipheral origin. In our view the compressive effect of the basilar invagination on this boy's medulla was of sufficient magnitude to have caused tumoral medullary schwannosis with resultant intermittent respiratory compromise leading to reflex anoxic seizures.
Item Type:
Article
Language:
en
MeSH:
Abnormalities, Multiple; Brain Diseases; Child; Decompression, Surgical; Fatal Outcome; Humans; Magnetic Resonance Imaging; Male; Medulla Oblongata; Occipital Bone; Schwann Cells; Spinal Cord Diseases; Syndrome
ISSN:
0722-5091

Full metadata record

DC FieldValue Language
dc.contributor.authorTreacy, Aen
dc.contributor.authorRedmond, Men
dc.contributor.authorLynch, Ben
dc.contributor.authorRyan, Sen
dc.contributor.authorFarrell, Men
dc.contributor.authorDevaney, Den
dc.date.accessioned2011-10-21T15:24:40Z-
dc.date.available2011-10-21T15:24:40Z-
dc.date.issued2011-10-21T15:24:40Z-
dc.identifier.citationSchwannosis induced medullary compression in VACTERL syndrome., 28 (5):384-6 Clin. Neuropathol.en
dc.identifier.issn0722-5091-
dc.identifier.pmid19788055-
dc.identifier.urihttp://hdl.handle.net/10147/146399-
dc.description.abstractA 7-year-old boy with a history of VACTERL syndrome was found collapsed in bed. MRI had shown basilar invagination of the skull base and narrowing of the foramen magnum. Angulation, swelling and abnormal high signal at the cervicomedullary junction were felt to be secondary to compression of the medulla. Neuropathologic examination showed bilateral replacement of the medullary tegmentum by an irregularly circumscribed cellular lesion which was composed of elongated GFAP/S 100-positive cells with spindled nuclei and minimal atypia. The pathologic findings were interpreted as intramedullary schwannosis with mass effect. Schwannosis, is observed in traumatized spinal cords where its presence may represent attempted, albeit aberrant, repair by inwardly migrating Schwann cells ofperipheral origin. In our view the compressive effect of the basilar invagination on this boy's medulla was of sufficient magnitude to have caused tumoral medullary schwannosis with resultant intermittent respiratory compromise leading to reflex anoxic seizures.-
dc.language.isoenen
dc.subject.meshAbnormalities, Multiple-
dc.subject.meshBrain Diseases-
dc.subject.meshChild-
dc.subject.meshDecompression, Surgical-
dc.subject.meshFatal Outcome-
dc.subject.meshHumans-
dc.subject.meshMagnetic Resonance Imaging-
dc.subject.meshMale-
dc.subject.meshMedulla Oblongata-
dc.subject.meshOccipital Bone-
dc.subject.meshSchwann Cells-
dc.subject.meshSpinal Cord Diseases-
dc.subject.meshSyndrome-
dc.titleSchwannosis induced medullary compression in VACTERL syndrome.en
dc.typeArticleen
dc.contributor.departmentDepartment of Histopathology, Children's University Hospital, Temple Street, Dublin, Ireland. anntreacy@mac.comen
dc.identifier.journalClinical neuropathologyen
dc.description.provinceLeinster-

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