Endoscopic view of osteogenesis imperfecta of the maxilla.
A 43-year-old woman presented with recurrent right cheek and facial pain and recurrent sinusitis. She reported no discomfort or complaints regarding the left side of her face. Evaluation included computed tomography (CT) of the sinuses. CT showed an opacified right maxillary sinus, obliteration of the right middle meatus by soft tissue, and an unusual ossification of the left maxilla and gingival ridge (figure, A and B).
The patient underwent functional endoscopic sinus surgery (FESS) on the right side to improve drainage and ventilation. At the time of surgery, endoscopic visualization of the interior of the left maxillary sinus was carried out through a small middle meatal antrostomy (figure, C). Intraoperatively, an exophytic smooth mass was seen arising from the floor of the left maxillary sinus (figure, D), which corresponded to the CT findings. No tissue looked suspicious, although palpation revealed that it was very hard, as there was no soft tissue in the mass.
It was felt that no further surgery should be done, particularly in view of the fact that the patient had just undergone a dental evaluation. At that evaluation, her oral surgeon diagnosed her with amelogenesis imperfecta and osteogenesis imperfecta of the maxilla.
Osseous lesions in the maxilla can represent ossifying fibroma, fibrous dysplasia, amelogenesis imperfecta, or osteogenesis imperfecta. Osteogenesis imperfecta is a genetic disorder that is characterized by bone fragility. (1) When it affects the area of the maxillary sinus, patients can present with a loss of dentition and maxillary hypoplasia. Dental treatment for associated malocclusion can be risky because of the risk of atypical fractures. In our patient, the dental pathology had no bearing on the patients sinus symptoms, and she received continued care by her oral surgeon.
(1.) Binger T, Rucker M, Spitzer WJ. Dentofacial rehabilitation by osteodistraction, augmentation and implantation despite osteogenesis imperfecta. Int J Oral Maxillofac Surg 2006;35(6):559-62.
From the Section of Otolaryngology, Halifax Medical Center, Daytona Beach, Fla. (Dr. Christmas and Dr. Mirante); the Florida State University School of Medicine, Daytona Beach (Dr. Mirante); and the Section of Otolaryngology, Yale New Haven Hospital-Saint Raphael Campus, and Yale University School of Medicine, New Haven, Conn. (Dr. Yanagisawa).
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|Title Annotation:||RHINOSCOPIC CLINIC|
|Author:||Christmas, Dewey A.; Mirante, Joseph P.; Yanagisawa, Eiji|
|Publication:||Ear, Nose and Throat Journal|
|Article Type:||Case study|
|Date:||Jun 1, 2014|
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