Expansion Thoracoplasty for Thoracic Insufficiency Syndrome Associated with Jarcho-Levin Syndrome

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Jarcho Levin Syndrome Associated With Aortic Coarctation

Jarcho-Levin syndrome (JLS) is a rare congenital disorder characterized by the presence of vertebral and rib malformations at birth, first described in 1938 by Jarcho and Levin [1,2]. The majority of reported cases have originated in Puerto Rico, it seems that this syndrome is more common in patients with Spanish ancestors. Its frequency in Spain is 0.2 per 100000 newborns with a female predomi...

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Jarcho-Levin syndrome.

literature(2,3). Recently, this syndrome has been divided into two major subtypes: spondylothoracic dysostosis and spondylocostal dysostosis(3,4). We describe two cases of the JarchoLevin syndrome, one of each subtype (spondylothoracic dysostosis and spondylocostal dysostosis). The cases illustrate the typical findings of the syndrome and highlight the differences between the two subtypes of th...

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A child with Jarcho-levin syndrome

In 1938, Jarcho and Levin1 first described a syndrome of congenital abnormalities affecting the spine and the thorax. The syndrome described a spectrum of radiological and skeletal anomalies including abnormal vertebral segmentation or formation defects, rib deformities and short trunk dwarfism. In 1978, Solomon et al2 subdivided patients with the above features into 2 distinct phenotypes: spon...

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[Anaesthesia for infant with Jarcho Levin syndrome: case report].

Jarcho Levin syndrome is a rare disorder. There are various vertebral and costal anomalies. Severe deformities and abnormal fusion of ribs and vertebrae cause respiratory insufficiency and pneumonia. We present anaesthesia in a patient with Jarcho Levin syndrome for vesicoureteral reflux.

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Pulmonary hypoplasia in Jarcho-Levin syndrome.

Jarcho-Levin syndrome, also known as spondylothoracic dysplasia and characterized by short trunk dwarfism, "crab-like" rib cage, with ribs and vertebral defects; it is not uncommon in Puerto Ricans. Many patients die in early infancy due to respiratory compromise associated to lung restriction and the reported cases emphasize mostly the skeletal malformations associated to the syndrome. We repo...

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ژورنال

عنوان ژورنال: JBJS Essential Surgical Techniques

سال: 2015

ISSN: 2160-2204

DOI: 10.2106/jbjs.st.n.00017