A description of two surgical and anesthetic management techniques used for a patient with fibrodysplasia ossificans progressiva.
Fibrodysplasia ossificans progressiva (FOP) is a rare and debilitating genetic disorder of skeletal malformations and progressive heterotopic ossification. Flare-ups are episodic, with bone formation in skeletal muscle and connective tissue leading to ankylosis of major joints of the axial and appendicular skeleton. This report outlines the management of a patient with FOP who had ankylosis of the temporomandibular joint and progressive ossification of the neck structures. The patient underwent two different surgical and anesthetic procedures within a 10-year period to manage his oral pain. The authors compare the surgical techniques, osteotomy versus the more conservative buccal approach, anesthesia techniques, and conventional intubation versus sedated fiberoptic intubation. This report emphasizes the importance of a less invasive surgical technique and an appropriate anesthetic management that reduces the risks, cost, and morbidity associated with routine surgical management of patients with FOP.
Wadenya
Rose
R
University of Pennsylvania School of Dental Medicine, Philadelphia, Attending Dentist, The Children's Hospital, Philadelphia, Pennsylvania, USA. wadenya@dental.upenn.edu
Fulcher
Megan
M
Grunwald
Tal
T
Nussbaum
Burton
B
Grunwald
Zvi
Z
D000771:Anesthesia, Intravenous / Q000379:methods*; D000844:Ankylosis / Q000601:surgery; D020680:Bronchoscopes; D016292:Procedural Sedation; D003731:Dental Caries / Q000628:therapy; D003793:Dental Restoration, Permanent; D005500:Follow-Up Studies; D006801:Humans; D007442:Intubation, Intratracheal / Q000295:instrumentation / Q000379:methods*; D008297:Male; D009221:Myositis Ossificans / Q000473:pathology / Q000601:surgery*; D009334:Neck Muscles / Q000473:pathology; D055100:Optical Fibers; D010027:Osteotomy / Q000379:methods; D011229:Preanesthetic Medication; D013705:Temporomandibular Joint Disorders / Q000601:surgery; D014081:Tooth Extraction / Q000379:methods; D055815:Young Adult