Recurrent Orbital Inflammation Due to Fibrous Dysplasia
22 February 2017 (online)
Fibrous dysplasia (FD) is a histopathologically benign, sporadic, slowly progressive pathology of the skeleton that may affect single or multiple bones. The most frequent localization is the maxilla; however, the involvement of the orbital walls is not uncommon. The ocular consequences of the craniofacial dysplasia (CFD) are of particular concern.
A 33-year-old man had been referred to the ENT department with a history of recurrent severe inflammations of the left orbit with persistent pain ([Fig. 1]). Over a period of 5 months, he had been hospitalized three times due to exacerbation of the orbital inflammation, treated chronically with antibiotics and high-dose systemic steroids.
Computed tomographic (CT) scans revealed bone asymmetry with ground-glass appearance and lacrimal fossa being obliterated with expanded bony tissue ([Fig. 2]). Magnetic resonance imaging (MRI) scans showed slightly enlarged left lacrimal gland ([Fig. 3]). Significant proptosis (20 mm left, 17 mm right) and downward displacement of the upper left eyelid was observed. Probing and irrigation of the upper and lower canaliculus revealed proper functional status of lacrimal drainage system with no obstruction at any level. Patient underwent surgical treatment. Through the incision in the left eyebrow, the periosteum of the orbital roof was detached. Cranio-orbital shaping was performed to provide enough room for the lacrimal gland and the orbital tissues. During the 7-month follow-up, no signs of inflammation were observed and the pain had been relieved. Restoration of the facial aesthetics was clinically observed and ophthalmologic evaluation was within the normal limits. The diagnosis of FD was confirmed.
Controversy surrounds many problems in the management of CFD. There are no clear guidelines. In general, there are three widely accepted approaches: watchful monitoring, pharmacological treatment, and surgery. The decision is made on the basis of the location of the lesions, age of the patient, the dynamics of the disorder, symptoms, and the patient's expectations. Medications are sometimes used to limit the expansion of the disease. Most commonly used are systemic steroids, bisphosphonate, calcitonin, and etidronate, but there is no proof of long-term benefits. Long-term case–control studies revealed better outcomes in asymptomatic patients managed expectantly. On the other hand, some authors postulate that CFD is fraught with the risk of rapid progression of visual deterioration. These authors advocate for the elective surgical treatment. Conservative bone modeling is the most commonly used surgical technique. This method is fraught with less risk of complications and recommended especially for patients with only cosmetic disfigurement. Some authors postulate aggressive radical resections. Majority of publications on the CFD focuses on the problem of narrowing of the optic canal and the decompression of the optic nerve. To our knowledge, this is the first case presentation in the literature of recurrent severe orbital inflammation caused by compression of lacrimal gland due to expansion of abnormal bony tissue in CFD. There are no established protocols for the treatment of this rare condition. From our experience, conservative bone contouring can be a sufficient method for the prevention of functional impairment and the restoration of facial aesthetics in the course of orbital fibrous dysplasia especially in mature patients.