|Year : 1982 | Volume
| Issue : 1 | Page : 57-59
Brown's syndrome associated with congenital ptosis
Vasudev Anand Rao, GC Sood, Santosh Kumar
Department of Ophthalmology, Jawaharlal Institute of Post Graduate Medical Education and Research Pondicherry, India
Vasudev Anand Rao
JIPMER, Pondicherry 605 006
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Rao VA, Sood G C, Kumar S. Brown's syndrome associated with congenital ptosis. Indian J Ophthalmol 1982;30:57-9
Brown's syndrome or superior oblique tendon sheath first described by Brown in 1950 is rare, It has the following features-apparent complete paralysis of the inferior oblique, no secondary contracture of the direct antagonist namely the homolateral superior oblique; down-drift of the affected eye on abduction, normal movements of the affected eye on abduction; the palpebral fissure on the affected side widens when the affected eye is abducted and limitation of movement of the affected eye on forced duction test in the field of action of the inferior oblique muscle.
The purpose of the present paper is to report a case of this syndrome which was bilateral and associated with ptosis and telecanthus.
| Case report|| |
A 20 year old male patient came to JIPMER Eye OPD with complaint of drooping of upper lid both eyes and squinting alternatively outwards both eyes since birth.
Local examination revealed bilateral ptosis and telecanthus. There was an alternate divergent squint of 15°. The action of right and left inferior oblique was deficient in laevo and dextro elevation respectively. There was depression of the abducted eye on dextro and laevo version. Movements were full in dextro and laevo depression. The widening of the palpebral fissure could not be shown due to the associated ptosis.
Visual acuity in right eye with
binocular vision with Worth's 4 dot test or on the synoptophore.
Under general anaesthesia there was limitation of movement in both eyes on forced duction test in the field of action of the inferior oblique muscle.
| Discussion|| |
Brown attributed the syndrome to a congenitally short sheath of the superior oblique which is firmly attached to the trochlea. When the eye is straight or abducted the sheath is loose and allows full rotation. However, when the eye is abducted the congenitally short sheath acts as a tight band which prevents elevation. In 1973 Brown identified 2 types true and simulated sheath syndrome. In the latter the posterior part of the superior oblique muscle and tendon are thickened due to inflammation extending from the ethmoid sinus, from orbital fractures or from frontal sinus surgery. Ours is a case of true Brown's syndrome since it was present at birth, there was no inflammatory episode, trauma or surgery. Goal and Gogi reported a case of the syndrome which was due to sub-clinical inflammation in the region of the trochlea of the superior oblique. When the inflammation subsided there was marked improvement in the ocular motility.
In the present case the condition was bilateral and was associated with congenital ptosis and telecanthus. Jain and Chander have also described association of congenital ptosis with this syndrome. Brown reported associated coloboma of choroid in one case while Hansen6 reported congenital cardiac anomalies in 2 cases. However in our case there was no associated congenital abnormality elsewhere in the body. Gupta and Hari Gopal3 have described this syndrome in 8 members of a family.
Brown has shown that the limitation of elevation disappears when the sheath of the superior oblique has been served. Crawford et a1 divided the final results following tenotomy of the superior oblique into 3 groups. (1) Patients with partial residual Brown's syndrome who gradually improved spontaneously over several years, (2) Patients who had minimal or moderate superior oblique muscle paresis, (3) Patients with marked superior oblique muscle paresis.
| Summary|| |
A rare case of true Brown's syndrome associated with congenital ptosis and telecanthus is reported.
| References|| |
Brown H.W. 1950, Symposium, p. 205 C.V, Mosby Co, St. Louis.
Goel, B.S. and Gogi, R 1968, Orient Arch. Ophthalmol. 6 : 302.
Gupta, J.S. and Gopal Hari 1969, Orient Arch. Ophthalmol. 7 : 332.
Brown, H.W. 1973, Doc. Ophthalmol. 34: 123.
Jain, LS. and Chander B 1963, Orient. Arch. Ophthalmol. 1 :311.
Hansen, E 1968, Acta Ophthalmologica, 46 : 469.
Crawford J.S. Ortan, R.B, and Daily S 1980, Amer J. Ophthalmol 89 : 824.
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