Sporadic Occurrence of Jarcho-Levin Syndrome in an Ivorian Newborn

Abstract

We report on an isolated chest-wall asymmetry with imaging findings of multiple vertebral and related rib defects in an Ivorian male newborn. He was born of a healthy and young couple without parental lineage, neither family malformative history nor teratogen exposure. This clinical presentation advocates Jarcho-Levin syndrome, a rare sporadic or familial disorder inherited as autosomal dominant or recessive mode and manifested by extensive vertebral segmentation defects with distinctive rib structural and morphological anomalies. According to our belief, this disorder has not been previously traced in the sub-Saharan African area.

Neural Tube Defects in Jarcho-Levin Syndrome: Study of Twenty-Eight Cases

“…Its main clinical signs are asymmetric chest wall, short neck-trunk stature, scoliosis, and multiple radiological vertebral and related rib defects with mild thoracic restriction. Treatment requires earlier respiratory support and later orthopedic or surgical management [2]. This syndrome is usually diagnosed in newborns.…”

A rare costovertebral malformation in a Kenyan infant

“…[4] There are few case reports from sub-Saharan Africa. [5,6] Imaging shows characteristic vertebral and thoracic anomalies involving fusion of the ribs and hemivertebrae, giving the ribcage a 'crab-like' appearance, kyphoscoliosis, and other associated visceral organ anomalies. [1][2][3] Prenatal three-dimensional (3D) ultrasound as early as 12 weeks can visualise increased fetal nuchal transparency, absent or deformed ribs, deformed vertebrae and distorted spinal architecture including shortening, kyphoscoliosis and spina bifida.…”

Anaesthetic management of a patient with Jarcho-Levin syndrome

“…Jarcho–Levin syndrome (JLS) is a rare, congenital, inherited costovertebral dysplasia with an estimated global incidence of 1/40,000 births. [ 1 2 ] Though exact incidence in Indian context is not known, there are few case reports describing it in Indian population. [ 3 ] We report a case of JLS, associated anaesthetic challenges and their management.…”