|
 |
| BRIEF REPORT |
|
| Year : 1999 | Volume
: 47
| Issue : 2 | Page : 124-126 |
|
Isolated myositis: A rare presentation of intraorbital ectopic lacrimal gland
N Suneetha, V Nirmala, Reji Koshy Thomas, RR Battu, Raly V Job, Abdul Rawoof
St. John's Medical College, Bangalore, India
Correspondence Address:
N Suneetha
Department of Ophthalmology, St. John's Medical College, Bangalore - 560 034
India
 Source of Support: None, Conflict of Interest: None  | Check |
How to cite this article:
Suneetha N, Nirmala V, Thomas RK, Battu R R, Job RV, Rawoof A. Isolated myositis: A rare presentation of intraorbital ectopic lacrimal gland. Indian J Ophthalmol 1999;47:124-6 |
This report describes a rare aberrant manifestation of ectopic lacrimal gland and its successful management in a child, who presented with severe systemic symptoms but an almost total absence of local orbital symptoms.
Intraorbital ectopic lacrimal gland is a rare entity and there are few case reports in the medical literature.[1][2][3] Owing to its rarity and location, a clinical diagnosis is hardly ever made. In the case reported here, intraorbital ectopic lacrimal gland tissue was found adjacent to the left lateral rectus muscle, inducing a severe myositis and simulating orbital inflammation.
| Case Report | |  |
A 12-year-old boy presented to the casualty with complaints of headache and vomiting for the past 15 days. He was admitted for investigation and treatment. The child appeared severely prostrated. A thorough paediatric evaluation including complete blood count, urine routine and microscopy, ocular fundi and skull X-rays including lateral, Caldwell and Waters' views, were normal. A detailed ophthalmological evaluation was recommended as he complained of mild ocular discomfort in the left eye. Ocular examination revealed a normal right eye. There was a diffuse swelling of the left upper lid, with no palpable mass, minimal axial proptosis (2 mm) and restricted adduction. Conjunctival congestion was present adjacent to the lateral canthus [Figure - 1]. Anterior segment and fundus were normal. Visual acuity in each eye was 6/6. Computerised tomographic (CT) scan of the brain was normal. CT scan of the orbit revealed a 2x1x1.2cm heterogeneously enhancing, soft tissue cystic mass in the mid-orbit in relation to the thickened left lateral rectus muscle [Figure - 2] and [Figure - 3]. B-scan ultrasonography showed a grossly enlarged left lateral rectus muscle just behind its insertion and extending posteriorly. The enlargement appeared uniform with low to medium internal reflectivity suggestive of myositis [Figure - 4]. There appeared to be cystic mass attached to the enlargement muscle in the mid-orbit. No scolex was noted within the cyst. A tentative diagnosis of myositis of the left lateral rectus probably due to cysticercal cyst was entertained. However, anticysticercal antibodies were negative. In the light of a negative ELISA and the absence of a pathognomic appearance of a cysticercal cyst on CT scan and ultrasonography (absence of scolex), systemic albendazole was withheld. The child was started on systemic steroid which was slowly tapered.
Pain and vomiting subsided with the steroids, but the proptosis, fullness in the lids and restricted adduction persisted. Hence, a decision to surgically excise the cyst was made. Under general anaesthesia, the lateral rectus was approached via a transconjunctival incision. The plane of incision was between the lateral rectus and the periorbita. The entire muscle up to the insertion was thickened. Multiple adhesions were found between the muscle sheath and the periorbita. Release of these adhesions revealed a firm white nodule measuring 0.5x1cm with ill-defined capsule attached by fibrous adhesions to the outer surface of the lateral rectus muscle and the surrounding periorbita. This was located in the mid-orbit, extending posteriorly. The mass was excised in toto and sent for histopathological examination. The post-operative period was uneventful with total subsidence of all signs and symptoms. Histopathological study of the excised nodule revealed glandular tissue consistent with lacrimal gland tissue [Figure - 5]. It also showed areas of necrosis, fibrosis and chronic inflammatory cell infiltrates. One year later the patient was symptom-free with no proptosis and with full range of ocular motility.
| Discussion | | |
Ectopic or aberrant lacrimal gland refers to the occurrence of this tissue in an area where it does not normally occur. Ectopic lacrimal gland has been found in the bulbar conjunctiva,[4],[5] outer canthus, caruncle, intraocular,[6] and intraorbital regions.[1][2][3] Of these, the intraocular ectopic lacrimal gland is least common, followed by intraorbital ectopic lacrimal gland. A review of literature documents as few as 5 reports of a total of 12 cases of intraorbital ectopic lacrimal gland. Green and Zimmerman[2] have reported the largest series of 8 cases of intraorbital ectopic lacrimal gland.
The ectopic gland continues to secrete tears like its normal counterpart. As these choriostomatous lesions have no ductal system to discharge their secretions, cyst formation is common. These cysts may rupture to release tears into the orbital tissues, where an inflammatory reaction occurs. This could explain the acute onset of symptoms in this patient. The recurrence of symptoms that have been reported could possibly be due to the presence of other loci of ectopic lacrimal gland within the same orbit or malignant transformation of the ectopic gland. An interesting feature noted was the severity of the systemic symptoms despite the lack of local orbital symptoms. One therapeutic pointer correlating the two was the dramatic improvement in systemic and local signs and symptoms after institution of steroid therapy and their total disappearance, without recurrence, after surgery. That such clinical presentation is possible in a child with ongoing local inflammatory process is of considerable clinical importance.
| References | | |
| 1. | Boudet MM, Bertezene. Exophthalmic par adenome lacrymal en position ectopique (angiographie del' orbite). Bull Soc Franc Ophthalmol 1964;64:624-27.  |
| 2. | Green WR, Zimmerman LE. Ectopic lacrimal gland tissue: report of eight cases with orbital involvement. Arch Ophthalmol 1967;78:318-27. [ PUBMED] |
| 3. | Boccate P, Blandamura S, Midena E, Carollo C. Orbital ectopic lacrimal gland tissue simulating a neoplasm. Report of a case with fine needle aspiration biopsy diagnosis. Acta Cytol 1992;36:737-43. |
| 4. | Francois J, Rabaey M. Adenoma of the limbal conjunctiva. Br J Ophthalmol 1951;35:237-41. [ PUBMED] |
| 5. | Rao VA, Kwatra V, Puri A. Cyst of ectopic (choriostomatous) lacrimal gland. Indian J Ophthalmol 1989;27:189-90. |
| 6. | Bruch G. Aberrant glandular tissue in the iris. Trans Am Acad Ophthalmol Otolaryngol 1952;56:47-51. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]
|
.png "Santen")
Search Pubmed for