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Query: UMLS:C0037315 (sleep apnea)
 8,000 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thoracic insufficiency syndrome (TIS) is a collection of chest and spine malformations that results in progressively restrictive pulmonary mechanics and an inability of the thorax to adequately support lung growth. Many children with TIS are too young to perform standard pulmonary function tests, yet need functional assessments of their restrictive thoracic disease. We report on the sleep architecture and frequency of sleep-related breathing abnormalities in 11 children with TIS who underwent overnight polysomnography from retrospective chart review. Ten of 11 (92%) had sleep disordered breathing as defined by currently accepted criteria of apnea-hypopnea index (AHI) >2 events/hr. The median AHI was 4.3 events/hr, with obstructive hypopneas (median 3.7 events/hr) accounting for 75% of abnormalities. Respiratory events occurred most frequently during REM sleep (median REM-AHI 17.3 events/hr), and were associated with oxyhemoglobin desaturation, and rarely carbon dioxide retention. Sleep disordered breathing with hypoxemia appears to be a common but under recognized problem among children with TIS. Polysomnogram may have a role as a non-invasive screening tool used in conjunction with other functional respiratory assessments in children with TIS, and warrants further study in a prospective manner.
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PMID:Sleep-disordered breathing in children with thoracic insufficiency syndrome. 2042 54

We present a direct anterior sternal split expansion as a surgical option for a case of severe Thoracic Insufficiency Syndrome (TIS) in an arthrogryposis-like patient. This patient's clinical features were published as a newly described syndrome: Adam Robert Wright Syndrome. The patient born with this syndrome displays characteristic craniofacial abnormalities, severe thoracic insufficiency syndrome, cleft palate, limb contractures, arthrogryposis, pulmonary hypoplasia, cryptorchidism, ophthalmoplegia and retinopathy, with normal intelligence. His severe thoracic insufficiency necessitated an urgent life-saving surgical intervention for a progressively worsening sleep apnoea and respiratory distress. We present a review of published data of sternal expansion thoracoplasty from 1965 to 2007 found in the literature. We demonstrate that direct anterior sternal split thoracoplasty with autogenous rib grafts is an effective technique for the acute management of thoracic insufficiency syndrome in this specific case. This procedure provided our patient with symptomatic benefit. To our knowledge, this is the only reported surgical management of thoracic insufficiency syndrome demonstrating a statistical improvement in chest wall compliance and tidal volume. We show that direct anterior sternal split expansion is a surgical treatment option in some patients with thoracic insufficiency syndrome. Our surgical strategy for the management of severe thoracic insufficiency syndrome in Adam Robert Wright Syndrome provided symptomatic relief and favourable long-term results.
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PMID:Autogenous bone graft for expansion thoracoplasty in Adam Robert Wright syndrome: a case report and review. 2042 50