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The Journal of Bone and Joint Surgery (American) 83:1495-1502 (2001)
© 2001 The Journal of Bone and Joint Surgery, Inc.

Scientific Article

Spinal Deformities in Noonan Syndrome

A Clinical Review of Sixty Cases

Choon-Ki Lee, MD, Bong-Soon Chang, MD, Young-Mi Hong, MD, Sei Won Yang, MD, Choon-Sung Lee, MD and Joong-Bae Seo, MD

Investigation performed at the Department of Orthopaedic Surgery, Seoul National University Hospital, Seoul, Korea
Choon-Ki Lee, MD
Bong-Soon Chang, MD
Sei Won Yang, MD
Departments of Orthopaedic Surgery (C.-K.L. and B.-S.C.) and Pediatrics (S.W.Y.), Seoul National University Hospital, 28 Yungun-Dong, Chongro-Ku, Seoul 110-744, Korea. E-mail address for C.-K. Lee: choonki{at}plaza.snu.ac.kr

Young-Mi Hong, MD
Department of Pediatrics, Ewha Womans University Hospital, 70 Chongro-6 Ka, Chongro-Ku, Seoul 110-126, Korea

Choon-Sung Lee, MD
Department of Orthopaedic Surgery, Asan Medical Center, University of Ulsan, 388-1 Poongnap-Dong, Songpa-ku, Seoul 138-736, Korea

Joong-Bae Seo, MD
Department of Orthopaedic Surgery, Chungbuk National University Hospital, 48 Gaeshin-Dong, Heungduk-Ku, Cheongju 361-763, Korea

The authors did not receive grants or outside funding in support of their research or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.

Background: Skeletal abnormalities, including spinal deformities, in Noonan syndrome have been described, but no detailed and systematic study of such spinal deformities has been presented in the literature.

Methods: The cases of sixty patients with Noonan syndrome were reviewed retrospectively, and the general appearance, growth disturbance, and mental status of the patients were documented. Spinal deformities were evaluated radiographically, and the frequency, pattern, and severity of the curves were documented.

Results: Spinal deformity was present in eighteen (30%) of the sixty patients. Two patients had congenital spinal deformity. Of the remaining sixteen patients with scoliosis, nine had a single thoracic curve, four had a single thoracolumbar curve, and three had a double major curve. Thoracic lordosis was also present in three of these sixteen patients. No patient had only increased kyphosis or lordosis. The mean age when the spinal deformities were detected was nine years; seven deformities were detected before the age of seven years. Overall, surgery was recommended to eleven of the eighteen patients; it was recommended for the treatment of scoliosis (mean, 68.5°; range, 45° to 125°) in eight patients and for the treatment of an associated thoracic lordosis (8°, 15°, and 18°) in three. Seven of the eleven patients underwent spinal arthrodesis. The operation was deferred in one patient because malignant hyperthermia developed during the induction of anesthesia.

Conclusions: Scoliosis with an associated thoracic lordosis occurs more frequently in Noonan syndrome than has been reported previously. Since the deformities tend to develop early and are relatively severe, a clinical and, if necessary, radiographic assessment of the spine with careful follow-up should be performed for early detection and treatment of spinal deformity. Although malignant hyperthermia is rare, all patients with Noonan syndrome should be considered to be at risk for the development of this complication before operative treatment.


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