George H. Thompson, M.D.
Research authored by Dr. Thompson
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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.
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