Characteristics of the odontological management of patients with progressive ossifying fibrodyplasia

Aicha Ibourk,Sidi Mohammed Bouzoubaa,Ihsane Ben Yahya

doi:10.1051/mbcb/2019016

Abstract

Introduction: Progressive ossifying fibrodysplasia (POF) is a rare autosomal dominant disease characterized by the congenital malformation of the big toes and progressive postnatal heterotopic ossification of soft tissues with characteristic anatomical profiles. The maxillofacial region may also be affected. Observation: A 24-year-old man was referred by a traumatologist for the restoration of the oral cavity. He showed characteristic signs of POF. Oral clinical examination showed limitation of the oral opening, multiple dental caries, dental necrosis, and an asymptomatic impacted 48. Management included motivation for oral hygiene, scaling and dental extractions, followed by a prescription of steroidal anti-inflammatory drugs at a single dose of 2 mg/kg/day for 4 days. Discussion: The management of patients with POF in oral surgery has particularities. Dental care must be performed in brief sessions. The patient must be in a semi-sitting position, with the neck held upright, to avoid hyper extension of the neck and to improve comfort and safety. A prescription for corticosteroids is necessary after dental care to prevent possible heterotopic ossification. Through this article, we highlight the characteristics of POF, therapeutic attitude, and precautions to take to avoid possible complications.

Highlights

Progressive ossifying fibrodysplasia (POF) is a rare autosomal dominant disorder characterized by congenital malformation of the big toes and progressive postnatal heterotopic ossification of the soft tissues with characteristic anatomic profiles [1,2,3,4,5]
Some deaths have been attributed to complications of general anesthesia due to intubation and ventilation difficulties
POF is a rare genetic disease that affects approximately one in two million people. This disease is characterized by skeletal malformations and progressive heterotopic ossification of the skeletal muscles, tendons, ligaments, and fascia [11,12]

Summary

Introduction

Progressive ossifying fibrodysplasia (POF) is a rare autosomal dominant disorder characterized by congenital malformation of the big toes and progressive postnatal heterotopic ossification of the soft tissues with characteristic anatomic profiles [1,2,3,4,5]. The patient underwent two hip surgeries in 2010 and 2016, following alterations of the hip and hip joint mobility He presented characteristic clinical signs of POF: decreased mobility of the peripheral and spinal joints, laterodeviation of the hallux (Fig. 1), and deformities following heterotopic ossifications in his back (Fig. 2) and hand (Fig. 3). A 6-month follow-up did not reveal any heterotopic ossifications resulting from the procedures

Discussion

Conclusion