Clinical Aspects And Conservative Dental Management of a Patient With Fibrodysplasia Ossificans Progressiva

Abstract

Aim: To present the clinical findings of a patient with Fibro dysplasia Ossificans Progressiva (FOP), highlighting peculiari ties of dental treatment in patients with this condition. Background: FOP is a rare genetic disease characterized by skeletal malformations and ectopic ossifications in skeletal muscles, tendons, ligaments and aponeurosis. Exacerbation of these ossifications can be caused by dental treatment, resulting in disease progression. Case description: A 26yearold male patient with a diagnosis of FOP was referred to our service for dental treatment. The patient presented decreased mobility in peripheral joints (knees and elbows), postural disability (ankylosis of the vertebral column), lateral deviation and shortness of the hallux, as well as heterotopic ossifications on the hands and back. The implementation of conservative dental procedures, such as oral hygiene instructions and recurrent topical fluoride applications, were performed in addition to endodontic and restorative treatments. Brief dental appointments were conducted without using regional anesthesia or dental dam clamps. The dental chair was positioned at 45° to provide more comfort and to avoid exacerbating the disease. The patient has now completed 6 months of followup and is free of heterotopic ossifications resulting from dental treatment. Conclusion: The dental treatment modifications implemented for the present case were sufficient to establish good oral health and to prevent the formation of heterotopic ossifications in the maxillofacial region. Clinical significance: FOP is a rare disease dentists must familiarize themselves with to provide adequate, personalized treatment, which minimizes traumas that may exacerbate the disease.

Temporomandibular joint ankylosis—“Knowing when not to operate”: Case report and qualitative systematic review of literature

“…Known AD pattern of inheritance makes prompt diagnosis and genetic counseling mandatory 18 . Lastly, the awareness of the existence of this disorder is lacking among clinicians leading to unnecessary invasive diagnostic procedures (like biopsy) leading to worsening of the condition 20 . Especially, in developing countries like India, the data on the patients with FOP are lacking but the patients with TMJA are high.…”

“…Fifthly, many patients may report with dental complications of long‐standing TMJA in FOP. In such patients, any intramuscular administration of local anesthesia, for example, IANB is contraindicated for reasons of intramuscular HO 20 . Such cases must be treated under GA administered with a fiberoptic bronchoscope.…”

“…In such patients, any intramuscular administration of local anesthesia, for example, IANB is contraindicated for reasons of intramuscular HO. 20 Such cases must be treated under GA administered with a fiberoptic bronchoscope. Overzealous manipulation of the neck and overstretching of the jaw 11 Dental extractions should be carried as atraumatic as possible through the buccal approach.…”

“…18 Lastly, the awareness of the existence of this disorder is lacking among clinicians leading to unnecessary invasive diagnostic procedures (like biopsy) leading to worsening of the condition. 20 Especially, in developing countries like India, the data on the patients with FOP are lacking but the patients with TMJA are high. In short, awareness of FOP among the maxillofacial fraternity will prevent precipitation in susceptible normal patients, exacerbation in the existing ones, and consideration palliative procedures for dental ailments in patients with FOP.…”