Cesarean Section of a 25 3/7 Week Parturient with Fibrodysplasia Ossificans Progressiva: A Case Report

Abstract

Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disease characterized by progressive ossification of tendons, ligaments, fascia, and skeletal muscle. Ossification can occur either spontaneously or as a result of tissue trauma beginning in childhood. Joints typically ankylose and patients frequently become immobile in their 20s. Patient’s frequently die before their 40s because of restrictive pulmonary failure as a result of intercostal muscle involvement.(1) The temporomandibular joint (TMJ) and cervical spine also often ossify resulting in mmobilization of the jaw and neck, making traditional airway management impossible.(2) Although there are case reports relating to anesthesia for dental procedures, there are no case reports related to the anesthesia care of a parturient with FOP. Our patient is a 24 y.o. G1P0 who presented at 22 weeks for a prenatal consultation. Initial evaluation revealed a 5’0” tall, 58 kg female with a fetus developing appropriately. The patient was successfully delivered at 25 3/7 weeks under GETA, with awake nasal intubation, due to preterm labor and vaginal bleeding. Particular attention was made to prevent further muscular trauma to avoid exacerbation of the disease.