Improvement of mouth opening for a patient with fibrodysplasia ossificans progressiva: a case report

Abstract

The aim of this article is to describe the care of a patient with fibrodysplasia ossificans progressiva (FOP) and to provide dentists with a guide for how to safely care for patients with FOP. Treatment improved the patient's limited mouth opening. FOP is a rare autosomal dominant disorder characterized by congenital malformation of the fingers and toes by heterotopic ossification progressiva of the connective tissue. This ossification causes a limitation in osteoradicular mobility, mainly affecting the spine, shoulders, hips, and peripheral joints. The disease can manifest from pregnancy until adulthood, with no greater prevalence associated with race or gender. Although rare, the disease can be easily identified by its clinical features, and diagnosis can be confirmed by a radiographic examination. There is no known effective treatment for this disease. All therapeutic treatment must be conservative to avoid any condition that may cause heterotopic ossification. Guidelines to prevent new ossifications are important for patients with FOP. Dental professionals should be cautious in planning treatment, avoiding anesthesia, especially in the mandible, to prevent ankylosis of the temporo-mandibular joints. The prevention of dental caries is essential to avoid the need for more invasive treatment.

Progressive ossificans fibrodysplasia endodontic management: Case report

“…Local anesthetics may be used in other ways in POF patients to reduce soft tissue trauma, either through infiltration into the periodontal ligament or in intraligamentar area, or using mucosal infiltrative injections or intrapulpal infiltration . The latter, as performed in the present clinical case, is an effective alternative, but its clinical applicability is difficult especially in posterior teeth due to the difficulty of access, limitation of buccal opening or behavioral reasons of the patient, and general anesthesia may be necessary.…”

Temporomandibular joint ankylosis as part of the clinical spectrum of Carey–Fineman–Ziter syndrome?

“…A well-known association between TMJ ankylosis and clinical syndrome in children is the association between TMJ ankylosis and Fyblodysplasia Ossificans Progressiva (FOP), a rare inherited disorder of the connective tissue, with autosomal dominant inheritance, in which skeletal abnormalities are associated with progressive ossification of striated muscle and soft connective tissue [Braga et al, 2011;Pignolo et al, 2013].…”