Anaesthetic management in neonate with rare finding of absent ribs as a part of Jacho Levin syndrome posted for thoracolumbar meningomyelocele repair: A case report

The congenital absence of ribs is a very rare finding. It can manifest as a standalone anomaly or as part of the syndrome. We came across a case in which the absence of ribs was associated with a neural tube defect. These findings were in favor of Jarcho Levin syndrome. Jarcho Levin syndrome, also known as spondylocostal dysostosis, is an uncommon genetic disorder with a global incidence of 1/40000 births. Very few case reports of it are in the Indian literature. It is characterized by vertebral malformations and the congenital absence of ribs, resulting in a crab-like appearance in the chest. It is linked to a wide range of non-skeletal abnormalities, including hydrocephalus, neural tube defects, tracheal anomalies, and abnormalities in the cardiac, renal, gastrointestinal, and urinary systems. The preoperative evaluation should be thoroughly done with a detailed systemic examination for the multisystem affliction of this syndrome. The anesthetic challenges in neonates with Jarcho Levin syndrome includes challenging airways due to defects in skeletal growth and hydrocephalus, proper positioning to avoid sac rupture, prone position ventilation, temperature control and fluid management. The prone position ventilation can be complicated by the absence of ribs and decreased respiratory reserve due to associated scoliosis and kyphosis. This case report highlights the anesthetic management of a three-day-old neonate with Jarcho Levin syndrome posted for thoracolumbar meningomyelocele repair.