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Year : 2006  |  Volume : 54  |  Issue : 2  |  Page : 123-125

Congenital unilateral ocular fibrosis syndrome secondary to benign congenital tumor

Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, 1, Anna Nagar, Madurai - 625 020, Tamil Nadu, India

Correspondence Address:
P Vijayalakshmi
Pediatric Ophthalmology and Strabismus Clinic, Aravind Eye Hospitals, 1, Annanagar, Madurai - 625 020, Tamilnadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0301-4738.25836

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Congenital unilateral fibrosis, blepharoptosis and enophthalmos syndrome is one of the rare forms of congenital fibrosis of extraocular muscles. The nature and cause of such unilateral inflammation leading to fibrosis is unknown. Prenatal orbital penetration has been proposed as a cause of this syndrome. We report a case of this rare syndrome associated with an adjacent sinus tumor.

Keywords: Extraocular muscles, fibrosis, blepharoptosis, enophthalmos, tumor.

How to cite this article:
Vijayalakshmi P, Jethani J, Kim U. Congenital unilateral ocular fibrosis syndrome secondary to benign congenital tumor. Indian J Ophthalmol 2006;54:123-5

How to cite this URL:
Vijayalakshmi P, Jethani J, Kim U. Congenital unilateral ocular fibrosis syndrome secondary to benign congenital tumor. Indian J Ophthalmol [serial online] 2006 [cited 2022 Jul 1];54:123-5. Available from: https://www.ijo.in/text.asp?2006/54/2/123/25836

Congenital restriction of ocular movements could be due to various abnormalities ranging from isolated fibrosis of single muscle to bilateral involvement of all the extraocular muscles. The full-blown ocular fibrosis syndrome manifests as bilateral ptosis, static external ophthalmoplegia and downward deviation of the eyes. Clinically, it could be congenital or acquired, unilateral or bilateral, affecting one, two or all the muscles and the degree of fibrosis of a muscle could be total or partial.[1],[2] This case is reported for its rare presentation of unilateral congenital fibrosis (left eye) associated with a tumor encroaching on to the medial quadrant of left orbit.

  Case Report Top

A 5-month-old healthy female infant was brought to us with small left eyeball and restricted ocular movements since birth. She was a product of full term normal delivery with birth weight of 2.934 Kgs. Antenatal and postnatal period was uneventful. There was no positive family history of any motility disorder. On examination, right eye was within normal limits in all parameters, left eye was found to be enophthalmic with pseudoptosis [Figure - 1]. Anterior segment, pupillary reaction and fundus were absolutely normal. Ocular movements of the left eye were found to be totally restricted in all directions of gaze [Figure - 1] a, b, c. A provisional diagnosis of unilateral ocular fibrosis was made.

Magnetic resonance imaging (MRI) showed enophthalmos on left side, an abnormal shape and thinning of extraocular muscles suggestive of fibrosis with contracture [Figure - 2]a with an abnormal shape of greater wing of sphenoid on the left side. An additional finding of an expansile lesion from left posterior ethmoidal and sphenoidal sinuses having grey matter isointense signals in precontrast sequences and subtle hypointense signals in T2 weighted images with brilliant nonhomogeneous enhancement after intravenous gadolinium [Figure - 2] a, b, d. Minimal thinning and breach of cribriform plate/ fovea ethmoidalis was seen with subtle extension into extradural compartment of anterior cranial fossa. Minimal encroachment into medial quadrant of left orbit and erosion of lamina was also noted [Figure - 2] a and c. These findings were suggestive of a possibility of congenital pathology of left orbit with coexisting congenital tumor like nasal glioma (heterotopic brain tissue)/ rhabdoid/ teratoid tumor of left posterior ethmoid and sphenoidal sinuses.

  Discussion Top

Congenital fibrosis of extraocular muscles include various abnormalities ranging from isolated fibrosis of a single muscle to bilateral involvement of all the extraocular muscles and has been subdivided into five groups: 1. General fibrosis syndrome, 2. Congenital fibrosis of inferior rectus muscle with blepharophimosis syndrome, 3. Strabismus fixus syndrome, 4. Vertical retraction syndrome, 5. Congenital unilateral fibrosis, blepharoptosis and enophthalmos syndrome.[3] Our patient's motility patterns resembled the last group, which is thought to be the rarest form.[4],[5]

There has been considerable debate over the cause of congenital fibrosis. In such cases the tissue of the extraocular muscle has been found to be abnormal because of the replacement of the normal contractile substance with fibrous tissue, interfering with movement of the eye.[2] The movement interference may result from either lack of pull power within the fibrotic muscle or resistance offered by the fibrotic antagonist muscle.[2],[5] It has been thought that all these disorders of congenital fibrosis are secondary to the changes in the muscles due to the primary developmental dysfunction of one or more of the cranial nerves that innervate the extraocular muscles.[2] Leone et al[4] first described unilateral ocular fibrosis as congenital, nonfamilial disorder with marked unilateral restriction of ocular motility in all directions, ptosis and enophthalmos in otherwise normal patients with normal plain X-rays. At surgery, he found fibrosis of soft tissues. Results of biopsy showed replacement of normal tissue by fibrous tissue. The proposed pathogenesis was prenatal orbital inflammation with no history of maternal infection. Another case[6] reported unilateral ocular fibrosis with bilateral hypoplasia of the optic discs. A case series of three was reported by Effron et al ,[1] one among them showing evidence of an adjacent ethmoidal sinus tumor (benign mesenchymoma). Orbital exploration in 2 cases revealed dense fibrosis of orbital tissues surrounding the extraocular muscles.

Hertle et al[5] reported 4 more cases and reviewed the literature and concluded that there may be an intraconal or extraconal diffuse infiltrating orbital mass associated with such a syndrome. There was also a scar present near the lower lid in the first child similar to that reported by Effron et al .[1] However, none of the cases showed any tumor in the adjacent sinuses. Effron et al ,[1] have postulated that a microinfiltration of the tumor could be the inciting cause for ocular muscle inflammation resulting in fibrosis of the muscles.

Our case resembles the 3rd case of Effron et al with unilateral ocular fibrosis in association with intracranial tumor extending into the orbit, presenting as fibrosis of extraocular muscles. There was a minimal encroachment of tumor into the medial quadrant of left orbit in our case, which is suggestive of some nonspecific inflammation leading to fibrosis. The type of tumor could not be confirmed by biopsy, which we feel is mandatory for further discussion. Fibrosis secondary to presence of benign congenital tumor should be considered as a separate entity from the other cases.

We recommend that all cases of congenital fibrosis syndrome should be advised to undergo MRI of orbit and brain to diagnose or exclude the pathology in the orbit and the surrounding structures.

  References Top

Effron L, Price RL, Berlin AJ. Congenital unilateral orbital fibrosis with suspected prenatal orbital penetration. J Pediatr Ophthalmol Strabismus 1985;22:133-6.  Back to cited text no. 1
Shivram SM, Engle EC, Petersen RA, Robb RM. Congenital fibrosis syndromes. Int Ophthalmol Clin 2001;41:105-13.   Back to cited text no. 2
Harley RD, Rodrigues MM, Crawford JS. Congenital fibrosis of the extraocular muscles. J Pediatric Ophthalmol Strabismus 1978;15:346-58.  Back to cited text no. 3
Leone RC Jr, Weinstein GW. Orbital fibrosis with enophthalmos. Ophthalmic Surg 1972;3:71-5.  Back to cited text no. 4
Hertle RW, Katowitz JA, Young TL, Quinn GE, Farber MG. Congenital unilateral fibrosis, blepharoptosis and enophthalmos syndrome. Ophthalmology 1992;99:347-55.  Back to cited text no. 5
Harley RD, Rodrigues MM, Crawford JS. Congenital fibrosis of the extraocular muscles. Trans Am Ophthalmol Soc 1978;76:197-226.  Back to cited text no. 6


  [Figure - 1], [Figure - 2]

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