Anesthetic Challenges and Management in a case of Jarcho-Levin Syndrome with a Neural Tube Defect in a Newborn

Kaya C., Cebeci H.

Boletin de la Asociacion Medica de Puerto Rico, vol.108, no.2, pp.17-20, 2016 (Scopus) identifier identifier

  • Publication Type: Article / Article
  • Volume: 108 Issue: 2
  • Publication Date: 2016
  • Journal Name: Boletin de la Asociacion Medica de Puerto Rico
  • Journal Indexes: Scopus
  • Page Numbers: pp.17-20
  • Ondokuz Mayıs University Affiliated: Yes

Abstract

Jarcho-Levin syndrome (JLS) is a rare axial skeletal growth disorder characterized by the presence of multi-level costovertebral malformations, a short neck, a short trunk, and kyphoscoliosis at birth. Neural tube defects appear to be common findings in patients with JLS, and chest hypoplasia frequently leads to respiratory insufficiency and death in early childhood. There have been no previous studies in the literature on the anesthetic management of JLS with neural tube defects and hydrocephalus. Therefore, we have reported a case of JLS in a female full-term newborn, who underwent surgery for myelomeningocele repair and ventriculoperitoneal shunting, with the associated anesthetic challenges and their management.