Thoracic insufficiency syndrome in patients with fibrodysplasia ossificans progressiva

Authors: Kaplan, Frederick¹; Glaser, David²

Source: Clinical Review in Bone and Mineral Metabolism, Volume 3, Numbers 3-4, September 2005 , pp. 213-216(4)

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Abstract:

Patients with fibrodysplasia ossificans progressiva (FOP) develop thoracic insufficiency syndrome (TIS), which can lead to life-threatening complications. Features contributing to TIS in patients with FOP include costovertebral malformations with orthotopic ankylosis of the costovertebral joints; ossification of intercostal muscles, paravertebral muscles, and aponeuroses; and progressive spinal deformity, including kyphoscoliosis or thoracic lordosis. Pneumonia and right-sided congestive heart failure are the major life-threatening hazards that result from TIS in patients with FOP. Prophylactic measures to maximize pulmonary function, minimize respiratory compromise, and prevent influenza and pneumonia may be helpful in decreasing the morbidity and mortality from TIS in patients with FOP.

Keywords: Fibrodysplasia ossificans progressiva (FOP); thoracic insufficiency syndrome; costovertebral malformations; heterotopic ossification; kyphoscoliosis

Document Type: Research article

DOI: http://dx.doi.org/10.1385/BMM:3:3-4:213

Affiliations: 1: Department of Orthopaedic Surgery, The University of Pennsylvania School of Medicine, Silverstein Two, 3400 Spruce St., 19104, Philadelphia, PA, Email: frederick.kaplan@uphs.upenn.edu 2: Department of Orthopaedic Surgery, The University of Pennsylvania School of Medicine, Silverstein Two, 3400 Spruce St., 19104, Philadelphia, PA,

Publication date: 2005-09-01