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  • Title: Thoracic elongation in type III osteogenesis imperfecta patients with thoracic insufficiency syndrome.
    Author: Kaplan L, Barzilay Y, Hashroni A, Itshayek E, Schroeder JE.
    Journal: Spine (Phila Pa 1976); 2013 Jan 15; 38(2):E94-100. PubMed ID: 23138403.
    Abstract:
    STUDY DESIGN: Prospective patient series. OBJECTIVE: To evaluate outcome of children experiencing severe osteogenesis imperfecta (OI) with thoracic insufficiency syndrome (TIS), who were treated with a novel expandable spinothoracic fixation device. SUMMARY OF BACKGROUND DATA: Current treatment options for TIS in patients with OI are limited. As in other patients with TIS, thoracic elongation is the treatment of choice; however, the use of vertical expandable prosthetic titanium rib device is contraindicated for patients with poor bone quality. METHODS: From 2007 to 2009, 4 patients with type III OI with TIS, aged 8 to 12 years, were treated with a novel expandable spinothoracic fixation device. Pre- and postoperative mobility, Cobb angle, PO2, PCO2, forced vital capacity, forced expiratory flow, use of accessory muscles when breathing, body weight, complication rates, and patient satisfaction were assessed. RESULTS: Prior to surgery, 2 patients were wheelchair bound and 2 walked with assistive devices. Average follow-up was 24 months (range, 14-36 mo). Mean Cobb angle improved by up to 32% in the coronal plane. Pulmonary function improved in all patients, with mean increases of 45% in forced vital capacity, 93% in forced expiratory flow, and 43% in PO2. PCO2 declined 30% on average, returning to normal values. Mean patient weight at 6-month follow-up increased by 10.9%. Patients moved from less than 3rd weight percentile per age group to the 3rd to 7th percentiles at the 6-month follow-up, with continuing weight gain. There were no major complications. All patients and their caregivers were satisfied that the procedure led to improved self-image and functional level. CONCLUSION: Thoracic elongation improved pulmonary function, facilitated weight gain, and made an indirect correction of spinal deformities (Cobb angle) by more than 30%, with no significant complications. Because of the rarity of this condition, a multicenter study to gain experience in a larger number of patients is recommended.
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