Claudia Florida Costea1, Andrei Cucu2*, Victor Vlad Costan3, Gabriela Florența Dumitrescu4, Anca Sava5 and Dana Mihaela Turliuc2,6,
1Department of Ophthalmology, Gr. T. Popa University of Medicine and Pharmacy, Iassy, Romania
2Neurosurgery Unit II, Prof. Dr. Nicolae Oblu Emergency Clinical Hospital, Iassy, Romania
3Department of Surgery, Gr.T. Popa, University of Medicine and Pharmacy of Iassy, Romania
4Department of Pathology, Prof. Dr. Nicolae Oblu, Emergency Clinical Hospital, Iassy, Romania
5Department of Anatomy, Grigore T.Popa, University of Medicine and Pharmacy, Iassy, Romania
6Department of Neurosurgery, Gr.T. Popa, University of Medicine and Pharmacy, Iassy, Romania
Received: 19 October, 2015; Accepted: 07 November, 2015; Published: 10 November, 2015
Andrei Cucu, MD, Neurosurgery Unit II, “Prof. Dr. Nicolae Oblu” Emergency Clinical Hospital, Iasi, Romania, Tel:0040-748378468; E-mail:
Costea CF, Cucu A, Costan VV, Dumitrescu GF, Sava A, et al. (2015) Visual Impairment in Orbitofrontal and Sphenoidal Fibrous Dysplasia Associated with Sphenoid Sinus Mucocele. J Clin Res Ophthalmol 2(4): 054-57. 10.17352/2455-1414.000022
© 2015 Costea CF, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Fibrous dysplasia; Visual impairment; Sphenoid sinus mucocele
The orbitofrontal fibrous dysplasia is a rare, non-malignant disease that involves the development and thickening of craniofacial bones, causing facial asymmetry, visual, neurological and dental complications. Decreased visual acuity in orbitofrontal and sphenoidal fibrous dysplasia appears as a result of compressive lesions on the optic nerve. The aim of this article is to present the ophthalmologic complications due to the association of two entities that both involved the optic nerve: orbitofrontal fibrous dysplasia associated with a large sphenoid sinus mucocele. We report the case of a 20-years old male patient, who was admitted to the Neurosurgery Unit II of “Prof. Dr. Nicolae Oblu” Emergency Clinical Hospital, Iassy, Romania, for headache, sudden decrease in visual acuity of both eyes in the last month, though more markedly in his right eye and facial deformity with right frontal bony bulging. The patient hadn't in his previous medical history any dermatological or endocrinological diseases. The ophthalmologic examination revealed that the visual acuity of the right eye was hand movements and best corrected visual acuity of the left eye was 20/60. Conventional X-ray, CT and MRI investigations showed an orbitofrontal and sphenoidal fibrous dysplasia associated with a large sphenoid sinus mucocele, located in the anterior and middle cranial fossa. Two surgical procedures were performed, the first being an attempt to perform a bilateral decompression of the optic nerve and in the second one, was evacuated the large sphenoid sinus mucocele, that compressed the optic chiasm. The first histopathological examination confirmed that the orbitofrontal and sphenoidal lesion had a typical appearance of fibrous dysplasia and the second biopsy revealed a sphenoid sinus mucocele. Six months after the evacuation of the sphenoid sinus mucocele, patient recovered completely his vision only in the left eye (20/20) and the right eye had no light perception, he also developed a peripheral right facial nerve palsy.
Fibrous dysplasia is an abnormal development of a bone mass in terms of structure and growth .
This is a rare disease with a benign evolution caused by the post-zygotic activating mutations of the GNAS gene  on chromosome 20q13  found in 71.9-86% of fibrous dysplasia cases  and that results in the inhibition of the differentiation and proliferation of the bone-forming stromal cells that leads to the replacement of the bone with fibrous tissue and immature thin bone trabeculae. Visual complications occur most often in case of spheno-ethmoid complex .
On the other hand, sphenoid sinus mucocele appears due to obstruction of mucinous gland ostium and can affect adjacent optic nerve, leading to visual impairment and even to blindness . The aim of this article is to present the ophthalmologic complications due to the association of two entities that both involved the optic nerve: orbitofrontal fibrous dysplasia associated with a large sphenoid sinus mucocele.
We report the case of a 20-years old male patient, who was admitted to the Neurosurgery Unit II of “Prof.Dr.Nicolae Oblu” Emergency Clinical Hospital, Iassy, Romania for headache, sudden decrease in visual acuity of both eyes in the last month, though more markedly in his right eye and facial deformity. In his past medical history there was a progressive visual impairment, in the right eye for the last three years. The patient had no other significant diseases, nor any skin lesions, either precocious puberty or other endocrine abnormalities. General clinical examination revealed moderate facial deformity, with right frontal bony bulging.
The ophthalmological examination revealed a visual acuity of the right eye (VA RE) = hand movements and best corrected visual acuity of the left eye (VA LE) = 20/60; there was an afferent pupillary defect in the right eye; the ophthalmoscopic examination revealed optic disc atrophy in the right eye and normally looking optic disc in the left eye.
Skull X-ray showed a “ground-glass” appearance of the frontal bone (Figure 1A).