![]() ![]() This syndrome in adults is a rare condition, and its ocular complications are even more unusual. 2 Most patients with this disorder are young children. Woakes' syndrome is characterized by severe recurrent nasal polyps with consecutive destruction of the nasal pyramid, leading to the broadening of the nose, hypertelorism, and periosteal resorption with bony fibrosis. The formation of mucocele and mucopyocele is a consequence of sinus drainage obstruction by pathologies such as nasal polyps, especially when bilateral. 1 Mucocele formation and inflammation, especially in posterior ethmoidal and sphenoid sinuses could cause optic nerve damage either by direct compression or inflammation. Optic nerve insult and visual compromise are well-known complications of sinonasal pathologies such as tumors and infections. Informed consent including publication of photographs in medical journals was obtained from the patient. Unfortunately, visual acuity in the left eye showed no improvement even after the sinus surgery. The histopathologic study of the sinus specimen revealed the inflammatory polyps with mucocele formation without any neoplasia. Thus, based on the clinical and imaging findings, the diagnosis of Woakes' syndrome was made, and endoscopic polypectomy of the nasal cavity and paranasal sinuses was performed 1 week afterward. The ENT consultation revealed anosmia (in paper-strip test), bilateral intranasal polyps, and disfigurement of the nasal bridge. The computed tomography scan showed the dehiscence of the bony wall of the optic canal as passing through the sphenoid sinus on the left side. Morphology of the intraorbital optic nerves appeared normal on MRI without pathological enhancement after contrast injection however, the posterior orbit was remodeled, and the optic nerve passage through the misshaped sphenoid sinus was visible. However, magnetic resonance imaging (MRI) at the time of admission demonstrated the fullness of the whole nasal cavity with the expansion of ethmoidal sinuses and multiple polypoid lesions in the maxillary and sphenoid sinuses. With the initial impression of the right optic neuritis, the patient received 3 g of intravenous methylprednisolone over 3 days, and the vision improved dramatically to 20/25 in the right eye. ![]() The Humphrey visual field test depicted a cecocentral scotoma in the right eye and severe visual field loss in the left eye. No abnormality was found in the right eye on fundus examination however, the optic disc pallor was found in the left eye. ![]() The ocular movements, anterior segment examination, and intraocular pressure in both eyes were within the normal limits. External examination revealed telecanthus and 2 mm proptosis of the left eye. Visual acuity was 20/400 in the right eye and hand motion in the left eye, and a left relative afferent pupillary defect was present. He also had a similar history of vision loss in his left eye 1 year before. A 28-year-old male was referred to the neuro-ophthalmology clinic with a 4-day history of painless decreased vision in the right eye. ![]()
0 Comments
Leave a Reply. |