The Journal of Bone and Joint Surgery (American) 85:399-408 (2003)
© 2003 The Journal of Bone and Joint Surgery, Inc.
The Characteristics of Thoracic Insufficiency Syndrome Associated with Fused Ribs and Congenital Scoliosis
Robert M. Campbell, Jr., MD,
Melvin D. Smith, MD,
Thomas C. Mayes, MD,
John A. Mangos, MD,
Donna B. Willey-Courand, MD,
Nusret Kose, MD,
Ricardo F. Pinero, MD,
Marden E. Alder, DDS,
Hoa L. Duong, MD and
Jennifer L. Surber, BS
Investigation performed at The Thoracic Institute, Christus Santa Rosa Children's Hospital, San Antonio, Texas
Robert M. Campbell Jr., MD
Thomas C. Mayes, MD
John A. Mangos, MD
Donna B. Willey-Courand, MD
Marden E. Alder, DDS
Jennifer L. Surber, BS
Department of Orthopaedics (R.M.C. Jr.), Department of Pediatrics and Division of Critical Care (T.C.M.) and Pediatric Pulmonology (J.M. and D.B.W.-C.), Department of Dental Diagnostic Sciences (M.A.), and Medical School (J.S.), University of Texas Health Science Center at San Antonio, Mail Code 7774, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail address for R.M. Campbell Jr.: campbellr{at}uthscsa.edu Please address requests for reprints to R.M. Campbell Jr.
Melvin D. Smith, MD
11732 Mill Rock Road, San Antonio, TX 78230
Nusret Kose, MD
Department of Orthopedics and Traumatology, Osmangazi University, Eskisehif 26480, Turkey
Ricardo F. Pinero, MD
333 North Santa Rosa Street, Suite F2658, San Antonio, TX 78207
Hoa L. Duong, MD
2020 Babcock Road, Suite 29, San Antonio, TX 78229
In support of their research or preparation of this manuscript, one or more of the authors received grants or outside funding from the National Organization of Rare Disorders and from the Office of the Orphan Products Development Division of the Food and Drug Administration (FDA PHS grant 2590). In addition, one of the authors (R.M.C. Jr.) received royalties from Synthes Spine Corporation, L.P. 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.
Read in part at the Annual Meetings of the American Academy of Orthopaedic Surgeons, Orlando, Florida, March 16, 2000; and the Scoliosis Research Society, Cleveland, Ohio, September 19, 2001.
• Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth.
• The rare condition of fused ribs and congenital scoliosis may result in a three-dimensional thoracic deformity with adverse effects on thoracic growth and function with development of thoracic insufficiency syndrome.
• The normal thorax is defined by two characteristics: normal, stable volume and the ability to change that volume. Volume depends on the width and depth of the rib cage, and the thoracic spine provides height. The ability to change volume, termed thoracic function, is provided by the diaphragm and the secondary muscles of respiration.
• On radiographs, the loss of the vertical height of the lung of the concave, restricted hemithorax is defined by the percentage of space available for the lung.
• Spine rotation causes a windswept thorax, with both restriction of the volume of the convex hemithorax and restriction of the motion of the involved ribs.
• Constrictive three-dimensional deformity of the thorax may cause extrinsic, restrictive lung disease.
• Progressive thoracic insufficiency syndrome is diagnosed on the basis of clinical signs of respiratory insufficiency, loss of chest wall mobility as demonstrated by the thumb excursion test, worsening indices of three-dimensional thoracic deformity on radiographs and computed tomography scans, or a relative decline in percent predicted vital capacity due to thoracic "failure to thrive," as demonstrated by pulmonary function tests.
• Treatment of progressive thoracic insufficiency syndrome should provide an acute increase in the thoracic volume with stabilization of any flail chest-wall defects and maintain these improvements as the patient grows, without the need for spine fusion.
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