Involvement of the Spine in Patients with Multiple Hereditary Exostoses

Roach, James W.; Klatt, Joshua B.; Faulkner, Nathan D.

doi:10.2106/jbjs.h.00762

Cited by 88 publications

(91 citation statements)

References 26 publications

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“…Children with MHE who had had axial imaging had undergone nearly five surgeries on average, typically for the removal of symptomatic osteochondromas or placement of guided growth plates for angular deformity. In contrast to the rheumatoid population, routine plain radiographs will not necessarily show the pathology [6]. Roach et al [6] demonstrated that only 17 % of lesions were identifiable on radiographs.…”

Section: Discussionmentioning

confidence: 99%

“…This causes a disturbance in the negative feedback regulatory system, thus, leading to undifferentiated proliferation of chondrocytes in the metaphyseal region [23][24][25][26][27]. Their composition consists of healthy, lamellar bone and a cartilaginous cap, growing by endochondral bone formation at the metaphysis of long bones [4,6,28,29]. Malignant transformation to a chondrosarcoma has been reported in both the appendicular and axial skeleton, primarily in adults [30][31][32].…”

Section: Discussionmentioning

confidence: 99%

“…Intracanal lesions may cause pain, paresthesias, myelopathy, weakness, or gait disturbance [4,6,[8][9][10]23]. However, spinal cord compression can be difficult to detect in children.…”

Section: Discussionmentioning

confidence: 99%

“…There are numerous published reports of spinal cord impingement and neurologic compromise from intracanal osteochondromas [6,9]. In most series, patients present with pain, weakness, or other significant neurologic findings [4,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23].…”

Section: Introductionmentioning

confidence: 99%

“…In most series, patients present with pain, weakness, or other significant neurologic findings [4,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]. Roach et al found that 12 out of 44 patients screened had lesions encroaching into the spinal canal and, thus, recommended routine magnetic resonance imaging (MRI) or computed tomography (CT) screening of the neuroaxis in patients with MHE to detect compressive lesions prior to the onset of neurologic deficits [6,9]. However, the prevalence of asymptomatic compressive intracanal lesions in patients with MHE has not been confirmed at other centers [6].…”

Section: Introductionmentioning

confidence: 99%

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Spinal stenosis frequent in children with multiple hereditary exostoses

Ashraf

Larson

Ferski

et al. 2013

Journal of Children's Orthopaedics

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Purpose Children with multiple hereditary exostoses (MHE) have numerous osteochondromas, with the most prominent lesions typically over the appendicular skeleton. A recent report noted a high rate of intracanal lesions in this patient population and recommended preventative spinal screening with magnetic resonance imaging (MRI) or computed tomography (CT). We sought to evaluate the prevalence of spinal stenosis from intracanal osteochondromas at our pediatric orthopedic center in order to evaluate if routine screening is warranted. Methods All pediatric patients treated for MHE were retrospectively identified. Records were reviewed to determine demographics, previous orthopedic surgery, and indication and results of axial spine imaging (CT or MRI).Imaging studies were reviewed to evaluate the presence of intracanal and compressive spinal lesions. Results Between 1990 and 2011, axial imaging was performed in nine patients with MHE due to concerns of pain, weakness, and/or dizziness. These patients had moderate disease involvement, with a mean of 4.9 previous orthopedic surgeries to address skeletal osteochondromas. Two patients with MHE had cervical spinal stenosis secondary to intracanal osteochondromas. Both children successfully underwent spinal decompression. Thus, of our MHE population undergoing axial imaging, 22 % were noted to have intracanal lesions. Conclusions Our experience reveals a [20 % rate of compressive intracanal osteochondromas in MHE patients undergoing spinal imaging. These two patients represent 5 % of the MHE patients treated at our center. These lesions may be slow growing, and significant consequences can occur if not identified promptly. Thus, we confer that routine axial screening of the spinal canal may be warranted in these children.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Intracanal lesions may cause pain, paresthesias, myelopathy, weakness, or gait disturbance [4,6,[8][9][10]23]. However, spinal cord compression can be difficult to detect in children.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Spinal stenosis frequent in children with multiple hereditary exostoses

Ashraf

Larson

Ferski

et al. 2013

Journal of Children's Orthopaedics

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Hereditary multiple exostoses with spine involvement in a 4‐year‐old boy

Ezra¹,

Tetteh²,

Diament³

et al. 2010

American J of Med Genetics Pt A

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Hereditary multiple exostosis (HME) is an autosomal dominant disorder characterized by multiple osteochondromas. We describe a case of acute cervical spinal cord compression arising from an exostosis at the lamina of C7 and T1 in a 4-year-old Mexican-American boy with HME. His affected sibling also displayed spinal cord compression because of a bony exostosis. Acute cervical spinal cord compression resulting from osteochondroma is a serious complication of HME. (c) 2010 Wiley-Liss, Inc.

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Syringomyelia in hereditary multiple exostosis

Legare

Modaff

Iskandar

et al. 2016

American J of Med Genetics Pt A

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We describe five children with Hereditary Multiple Exostosis (HME) who also had syringomyelia. Of these, four had a tethered cord/fibrolipoma. No spinal osteochondromas were found in these patients. All had antecedent neurological signs or symptoms that prompted spinal imaging with MRI. Of all patients with HME seen in the Midwest Regional Bone Dysplasia Clinic from 1982 to present, 44% (17/39) of patients had signs or symptoms concerning for possible cord-related neurological findings. However, only 10 of 39 had spinal imaging. Assuming that all individuals with syringomyelia were identified, then 5/39 (13%) were in that way affected. This, of course, is a minimal estimate given that many were not imaged. The incidence of syringomyelia appears to be increased in this population, and seems to be unrelated to spinal osteochondromas. A low threshold for obtaining spinal MRI in patients with Hereditary Multiple Exostosis seems rational. © 2016 Wiley Periodicals, Inc.

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Involvement of the Spine in Patients with Multiple Hereditary Exostoses

Cited by 88 publications

References 26 publications

Spinal stenosis frequent in children with multiple hereditary exostoses

Spinal stenosis frequent in children with multiple hereditary exostoses

Hereditary multiple exostoses with spine involvement in a 4‐year‐old boy

Syringomyelia in hereditary multiple exostosis

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