Tracheostomy for the pediatric patient with fibrodysplasia ossificans progressiva: a case report

Abstract

Background

Fibrodysplasia ossificans progressiva (FOP) is an extremely rare connective tissue disease characterized by subsequent ossification of skeletal muscles, tendons, ligaments, and other fibrous tissues. The ossification of these tissues progresses during childhood and leads to limb and trunk deformities. Since any surgery may trigger subsequent ossification, it is relatively contraindicated for patients with FOP. In this report, we describe our experience in performing tracheostomy in a pediatric patient with FOP who developed a restrictive respiratory disorder due to progressive deformity of the trunk.

Case presentation

A 12-year-old boy, diagnosed with FOP at the age of one, was referred for a tracheotomy after requiring 2 months of oral intubation and mechanical ventilation due to severe deformity-induced dyspnea. After changing from oral intubation to nasal intubation, we carefully considered the indications and benefits of tracheostomy in patients with FOP. Eventually, tracheostomy was successfully performed using our surgical design: creating a skin incision at the level of the cricoid cartilage that can always be identified, creating inverted U-shaped incision on the anterior tracheal wall to make a flap, and suturing the entire circumference of the tracheotomy and skin. One month after the surgery, he regained normal breathing and pronunciation and returned to school. The patient showed no unfavorable postoperative outcomes over a 4-year follow-up period.

Conclusions

Tracheostomy in our pediatric case of FOP required careful perioperative management. However, it could effectively improve the patient’s quality of life.