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Health-Related Quality of Life in Children With Thoracic Insufficiency Syndrome

Vitale, Michael G. MD, MPH*†‡; Matsumoto, Hiroko MA*†‡; Roye, David P. Jr MD*†; Gomez, Jaime A. MD*†; Betz, Randal R. MD§; Emans, John B. MD; Skaggs, David L. MD; Smith, John T. MD#; Song, Kit M. MD**; Campbell, Robert M. Jr MD††

Journal of Pediatric Orthopaedics: March 2008 - Volume 28 - Issue 2 - p 239-243
doi: 10.1097/BPO.0b013e31816521bb
Spine: Original Article

Background: The traditional techniques to treat thoracic insufficiency syndrome (TIS) are not able to stabilize or improve chest wall size or pulmonary function while allowing spine growth. To this end, vertical expandable prosthetic titanium rib (VEPTR) was specifically designed to treat TIS by allowing growth of the thoracic cavity and control/correction of spine deformity. The purpose of this study was to determine quality of life (QOL) of children with TIS and its impact on their parents before and after implantation of the VEPTR and also compare these results to those of healthy children.

Methods: As part of the original multicenter evaluation of the VEPTR, a Child Health Questionnaire (CHQ) was collected preoperatively on 45 patients who were subsequently treated with expansion thoracoplasty using the VEPTR. The average age was 8.2 ± 2.6 years, and the parent form of the CHQ was filled out by the primary caretaker. Patients were divided into 3 diagnostic categories: rib fusion (n = 15), hypoplastic thorax syndromes (n = 17), and progressive spinal deformity (n = 13).

Results: There were significant differences between the study patients and healthy children in physical domains. Compared with parents of healthy children, parents of children with TIS experienced more limitations on their time and emotional lives due to their children's health problems. There were no significant differences in CHQ before and after the surgery except for a significant decrease in the self-esteem among a subgroup of patients with hypoplastic thorax syndromes. There were no significant differences in postoperative QOL between patients who had VEPTR-related complications and patients who did not have the complications.

Conclusions: The children with TIS had lower physical scores and higher caregiver burden scores than healthy children. However, the scores in psychosocial domains were similar to those in healthy children. Our study demonstrated that QOL of children and burden of care in their parents remained the same after VEPTR instrumentation. Children's QOL seemed to be not affected by whether they had VEPTR-related complications or not.

From the *Division of Pediatric Orthopaedics, Department of Orthopaedic Surgery, College of Physicians and Surgeons, Columbia University; †Pediatric Orthopaedic Surgery, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center; ‡International Center for Health Outcomes and Innovation Research and the Joseph L. Mailman School of Public Health, Columbia University, New York, NY; §Shriners Hospital for Children-Philadelphia, Philadelphia, PA; ∥Department of Orthopaedic Surgery, Children's Hospital, Harvard Medical School, Boston, MA; ¶Children's Orthopaedic Center, Children's Hospital Los Angeles, Los Angeles, CA; #Pediatric Orthopaedic Surgery, University of UT, Salt Lake City, UT; **Children's Hospital and Regional Medical Center, Seattle, WA; ††Department of Orthopaedics, University of Texas Health Science Center, San Antonio, TX.

This study was approved by the Institutional Review Board.

Data collection for the Humanitarian Device Exempt Study for FDA approval was supported by Synthes.

Reprints: Michael Vitale, MD, PhD, 600 West 168th St, 7th Flr, New York, NY 10032. E-mail:

© 2008 Lippincott Williams & Wilkins, Inc.