George H. Thompson, M.D.
Research authored by Dr. Thompson
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List of Publications via PubMed
(NIH National Library of Medicine)
List of Publications via PubMed
(NIH National Library of Medicine)
Management of Multiple Hereditary Exostosis of The Axial (Hip And Spine) Skeleton
Abstract 2005 MHE Conference
George H. Thompson, M.D.
Professor, Orthopaedic Surgery and Pediatrics, Director,
Pediatric Orthopaedics, Rainbow Babies and Children’s Hospital,
Case Western Reserve University, Cleveland, OH 44106
Exostoses of the axial skeleton are uncommon lesions.
Those involving the spine represent 4 to 7 percent of all primary benign spinal tumors.
They can occur as solitary exostoses or in association with multiple hereditary exostoses (MHE).
Pelvic exostoses, including those involving the proximal femur are also uncommon.
Spinal Lesions: We have recently evaluated our experience with spinal exostoses seen between 1972 and 2002. There were
12 patients, including 7 females and 5 males with a mean age at presentation of 24.2 years (range, 7-52 years).
Five patients had MHE, while 7 had solitary exostoses. The mean age at presentation of the patients with MHE was younger at
16.8 years (range, 7-34 years) compared to 29.5 years (range, 22-52 years) for those with solitary lesions. Eight of the 12
patients had intraspinal lesions. These occurred most commonly in the cervical spine. All five patients with MHE had intraspinal
exostoses. Three at C2 and one each in the thoracic spine and sacrum. The solitary intraspinal lesions occurred in the cervical
(C2 and C6) or thoracic (T11). The most common chief complaint was pain (8 patients). Seven of these lesions resulted in
symptoms consistent with spinal cord nerve root compression. Three patients with MHE and 4 with solitary exostoses had
symptoms or findings consistent with neurological compression.
Eight exostoses were treated surgically with eventual resolution of symptoms. The mean follow-up for patients treated
surgically was 5.6 years (range, 0.5-13 years). Two patients had recurrence following resection of intraspinal lesions. Both had
solitary lesions and underwent successful revision surgery. There was only one complication in the 8 patients treated
operatively. This consisted of an anterior compartment syndrome following prolonged surgery for wide excision and
stabilization of a thoracolumbar exostosis.
Pelvic Lesions: Pelvic lesions in MHE are relatively uncommon. When present, they usually involve the ilium and proximal
femur but occasionally occur in the acetabulum, resulting in progressive subluxation of the femoral head. Lesions involving the
proximal femur are usually a dysplasia rather than a true exostosis, but these too can become enlarged resulting in progressive
hip subluxation. Excision of lesions about the pelvis include excision and possibly a proximally femoral osteotomy, if there is
subluxation of the hip. An enlarging pelvic lesion in a skeletally mature individual is suggestive of malignant degeneration.
Conclusions: Exostoses involving the axial skeleton are relatively uncommon. Those involving the spine have a higher
incidence of neurological symptoms due to spinal cord or nerve root compression. Any child with MHE presenting with
neurological signs or symptoms should be evaluated by both computed tomography and magnetic resonance imaging. Without
appropriate treatment, progressive neurological symptoms can occur. Lesions of the pelvis are also rare and a common site of
malignant degeneration asan adult. The indications for treatment are pain, disfiguring mass, and progressive hip subluxation.
Abstract 2005 MHE Conference
George H. Thompson, M.D.
Professor, Orthopaedic Surgery and Pediatrics, Director,
Pediatric Orthopaedics, Rainbow Babies and Children’s Hospital,
Case Western Reserve University, Cleveland, OH 44106
Exostoses of the axial skeleton are uncommon lesions.
Those involving the spine represent 4 to 7 percent of all primary benign spinal tumors.
They can occur as solitary exostoses or in association with multiple hereditary exostoses (MHE).
Pelvic exostoses, including those involving the proximal femur are also uncommon.
Spinal Lesions: We have recently evaluated our experience with spinal exostoses seen between 1972 and 2002. There were
12 patients, including 7 females and 5 males with a mean age at presentation of 24.2 years (range, 7-52 years).
Five patients had MHE, while 7 had solitary exostoses. The mean age at presentation of the patients with MHE was younger at
16.8 years (range, 7-34 years) compared to 29.5 years (range, 22-52 years) for those with solitary lesions. Eight of the 12
patients had intraspinal lesions. These occurred most commonly in the cervical spine. All five patients with MHE had intraspinal
exostoses. Three at C2 and one each in the thoracic spine and sacrum. The solitary intraspinal lesions occurred in the cervical
(C2 and C6) or thoracic (T11). The most common chief complaint was pain (8 patients). Seven of these lesions resulted in
symptoms consistent with spinal cord nerve root compression. Three patients with MHE and 4 with solitary exostoses had
symptoms or findings consistent with neurological compression.
Eight exostoses were treated surgically with eventual resolution of symptoms. The mean follow-up for patients treated
surgically was 5.6 years (range, 0.5-13 years). Two patients had recurrence following resection of intraspinal lesions. Both had
solitary lesions and underwent successful revision surgery. There was only one complication in the 8 patients treated
operatively. This consisted of an anterior compartment syndrome following prolonged surgery for wide excision and
stabilization of a thoracolumbar exostosis.
Pelvic Lesions: Pelvic lesions in MHE are relatively uncommon. When present, they usually involve the ilium and proximal
femur but occasionally occur in the acetabulum, resulting in progressive subluxation of the femoral head. Lesions involving the
proximal femur are usually a dysplasia rather than a true exostosis, but these too can become enlarged resulting in progressive
hip subluxation. Excision of lesions about the pelvis include excision and possibly a proximally femoral osteotomy, if there is
subluxation of the hip. An enlarging pelvic lesion in a skeletally mature individual is suggestive of malignant degeneration.
Conclusions: Exostoses involving the axial skeleton are relatively uncommon. Those involving the spine have a higher
incidence of neurological symptoms due to spinal cord or nerve root compression. Any child with MHE presenting with
neurological signs or symptoms should be evaluated by both computed tomography and magnetic resonance imaging. Without
appropriate treatment, progressive neurological symptoms can occur. Lesions of the pelvis are also rare and a common site of
malignant degeneration asan adult. The indications for treatment are pain, disfiguring mass, and progressive hip subluxation.
| Dr. Thompson's clinical research |
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Click here to verify.# HON Conduct 282463 and is the patient support link on the US Government Genetics Home Reference (http://ghr.nlm.nih.gov)
website, also linked for Patient Information on The Diseases Database a cross-referenced index of human disease, as well as the
Intute: health & life sciences a free online service providing access to the very best Web resources for education and research located in the UK
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