Case Of the Week (COW) 02 Nov 2014
Answer:
Jarcho-Levin syndrome (JLS) or spondylothoracic dysotosis(STD)
Findings:
Short neck Short trunk Constricted thorax. Infantogram shows crab-like or fan-like radiation of the ribs.
Discussion:
1) Rare autosomal recessive heterogenous disorder which can occur to variable severity.
2) It is primarily characterized by a short neck, short trunk , normal length limbs and a constricted thorax (narrow fetal thorax) due to multiple rib (crab-like or fan-like radiation of the ribs) and vertebral anomalies at all levels of the vertebral column, including Butterfly vertebrae, Hemivertebrae, Fused vertabrae and Hypoplastic vertebrae.
Contributed By:
Dr. Balaji Ayyamperumal
Tanjore Medical College
Answer:
Jarcho-Levin syndrome (JLS) or spondylothoracic dysotosis(STD)
Findings:
Short neck Short trunk Constricted thorax. Infantogram shows crab-like or fan-like radiation of the ribs.
Discussion:
1) Rare autosomal recessive heterogenous disorder which can occur to variable severity.
2) It is primarily characterized by a short neck, short trunk , normal length limbs and a constricted thorax (narrow fetal thorax) due to multiple rib (crab-like or fan-like radiation of the ribs) and vertebral anomalies at all levels of the vertebral column, including Butterfly vertebrae, Hemivertebrae, Fused vertabrae and Hypoplastic vertebrae.
Contributed By:
Dr. Balaji Ayyamperumal
Tanjore Medical College