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Curr Opin Pediatr. 2009 Jun;21(3):333-43. doi: 10.1097/MOP.0b013e328329a500.

Management of thoracic insufficiency syndrome.

Author information

1
University of Pennsylvania School of Medicine, Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. mayero@e-mail.chop.edu

Abstract

PURPOSE OF THE REVIEW:

Thoracic insufficiency syndrome (TIS) was first characterized by Campbell and Smith in 2003, as the inability of the thorax to support normal respiration and growth. The range of thoracospinal disorders within TIS is broad and some have poor prognosis, but all have been challenging to treat effectively. This review will describe the common approaches and report on the published outcomes of each approach.

RECENT FINDINGS:

Over the last 15 years progress has been made in refining the approach to treating TIS, and more recently functional outcomes have been published for some of the techniques used. A number of recent reports have demonstrated spinal growth that meets normal rates after insertion of expandable devices to support the spine and preserve growth. However, in the short term pulmonary function outcomes indicate stabilization of lung function as opposed to regaining function, though there is a paucity of published data.

SUMMARY:

There are a number of effective surgical interventions for treating the underlying thoracospinal disorders in TIS; however, though the short-term effect on pulmonary function indicates stabilization the longitudinal effect still needs to be elucidated.

PMID:
19454896
DOI:
10.1097/MOP.0b013e328329a500
[Indexed for MEDLINE]

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