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Thoracic Elongation in Type 3 Osteogenesis Imperfecta Patients with Thoracic Insufficiency Syndrome.
MedLine Citation:
PMID:  23138403     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
ABSTRACT: Study Design. Prospective patient series.Objective. To evaluate outcome of children suffering from severe osteogenesis imperfecta (OI) with thoracic insufficiency syndrome (TIS), who were treated with a novel expandable spino-thoracic fixation device.Summary of Background Data. Current treatment options for osteogenesis imperfecta (OI) patients with TIS are limited. As in other patients with TIS, thoracic elongation is the treatment of choice; however, use of vertical expandable prosthetic titanium rib (VEPTR) device is contraindicated for patients with poor bone quality.Methods. From 2007-2009, four type III OI patients with TIS, ages 8-12 years, were treated with a novel expandable spino-thoracic fixation device. Pre- and postoperative mobility, Cobb angle, PO2, PCO2, forced vital capacity (FVC), forced expiratory flow (FEV), use of accessory muscles when breathing, body weight, complication rates, and patient satisfaction were assessed.Results. Prior to surgery two patients were wheel-chair bound and two walked with assistance devices. Average follow-up was 24 months (range 14-36 months). Mean Cobb angle was improved up to 32% in the coronal plane. Pulmonary function improved in all patients, with mean increases of 45% in FVC, 93% in FEF, 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 <3 weight percentile per age group to the 3-7 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's angle) by over 30%, with no significant complications. Due to the rarity of this condition, a multi-center study to gain experience in a larger number of patients is recommended.
Authors:
Leon Kaplan; Yair Barzilay; Amir Hashroni; Eyal Itshayek; Josh E Schroeder
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-7
Journal Detail:
Title:  Spine     Volume:  -     ISSN:  1528-1159     ISO Abbreviation:  Spine     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-9     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7610646     Medline TA:  Spine (Phila Pa 1976)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Spine Unit, 1Orthopaedic and 2Neurosurgery Departments. Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
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