OPHTHALMOLOGY Vol. 16, No. 4 OCTOBER- DECEMBER 2010 ORIGINAL PAPER - PDF document

M E D I C A L M E D I C A L M E D I C A L M E D I C A L M E D I C A L C H A N N E L C H A N N E L C H A N N E L C H A N N E L C H A N N E L OPHTHALMOLOGY Vol. 16, No. 4 OCTOBER- DECEMBER 2010 ORIGINAL PAPER PRESENTATION OF IDIOPATHIC, NON-SPECFIC, ORBITAL INFLMMATION (PSEUDOTUMOR) - STUDY OF 46 CASES. 1- PARTAB RAI ABSTRACT FCPS 2- SYED IMTIAZ ALI SHAH PURPOSE OF STUDY: To discuss clinical features, treatment and outcome of the FCPS disease. 3- SYED AFTAB HUSSAIN SHAH DESIGN: Hospital based prospective study. FCPS DURATION: From September 2001 to date. 4. AZIZULLAH JALBANI PLACE OF STUDY: Department of Ophthalmology, Chandka Medical College FCPS Hospital Larkana. 5. IMDAD ALI ANSAREE MATERIAL AND METHODS: Our study included 46 patients with pseudotumor. From FCPS eye out patient department all patients were selected, where a specific proforma containing informed consent, patient’s bio data, history, clinical examination, investigations, treatment, and follow up was filled .The disease is diagnosed by exclusion criteria, based on history, clinical features and investigations .For exclusion of systemic cause of the disease, all patients were referred to general physician. The follow-up period was carried out for at least 5 years. Only cases of nonspecific inflammatory orbital pseudotumor were included in this study. 1- Associate Professor RESULTS: Of these 46 cases with age range of 12 years to 58 years, 28 (60.86%) were Department of Ophthalmology males and 18 (39.13%) females .The disease was bilateral in11 (23.91%) cases and CHANDKA MEDICAL unilateral in 35(76.08%) cases, of which left orbit was involved in 19(41.30%) cases and COLLEGE LARKANA. right in 16(34.78%) cases. We have seen 18(39.13%) cases of myositis, 13(28.26%) 2- Head of Department of cases of dacryoadenitis, 8(17.39%) cases of anterior pseudotumor, 5(10.86%) cases of Ophthalmology diffuse pseudotumor, and 2(4.34%) cases of apical pseudotumor. The out come of our CHANDKA MEDICAL COLLEGE patients is that, 30 (65.21%) patients who presented earlier were successfully treated by LARKANA. corticosteroids and 3(6.52%) recurrent, steroid resistant, biopsy proven cases were treated 3- Associate Professor by low-dose orbital radiotherapy. Due to late presentation of 13(28.26%) patients Head of Department of Medicine complications were seen, exposure keratopathy 4(8.69%) cases, rectus muscle paresis CHANDKA MEDICAL COLLEGE 3(6.52%) cases, secondary glaucoma 2(4.34%) cases, optic atrophy 2(4.34%) cases and LARKANA. frozen orbit 2(4.34%) cases. The last 6 (13.04%) patients developed complete visual loss 4. Assistant Professor in the end. Department Medicine CONCLUSION: Early presentation of patient, during initial stage of the disease CHANDKA MEDICAL COLLEGE will result in decreased risk of disease extension and ocular complications. LARKANA. 5. Assistant Professor, KEY WORDS: Idiopathic orbital pseudotumor —— 46 cases study Department Medicine CHANDKA MEDICAL COLLEGE INTRODUCTION LARKANA. Orbital pseudotumor is defined as non-specific, idiopathic inflammatory process in the orbit or eye. Unlike cancerous tumors, it cannot invade other tissues or spread elsewhere hence it is called pseudotumor. Among orbital disorders, pseudotumor, after Grave’s disease and lymphoproliferative disease, is a common ophthalmologic disease, about 4.7 Address for Correspondence: to 6.3% 1 . The cause is unknown and usually unilateral (90%). The disease usually DR: PARTAB RAI occurs in adults but may also affect children. Pediatric orbital pseudotumor encompasses FCPS about 6% to 16%. In children, there is a higher incidence of bilateral orbital involvement Associate Professor without evidence of underlying systemic disease. The disease may present acutely, sub- Department of Ophthalmology acutely, or chronically in one orbit or may occur bilaterally and may be recurrennt 2 . CHANDKA MEDICAL COLLEGE Nonetheless, immune response cells, in particular lymphocytes and different stages of LARKANA, LARKANA local fibrosis and neovasculerisation are present in histopathological specimens of Tel: 0333-7567347 pseudotumor in newly formed connective tissue. Indeed, the relative proportion of E mail- dr_partab_rai @ yahoo.com inflammatory cells with respect to collagen fibers is used to differentiate the pseudotumor 594

TABLE 1 SHOWING ANATOMIC LOCALISATION, NUMBER OF PATIENTS, SEX, AGE RANGE AND LATERALITY OF PATIENTS WITH PSEUDOTUMOR. (N =46). S No Cause No of Male Female Age range Laterality Patients 01 Myositis 18 11 7 12y-46y Bil:5 Rt:6 Lt: 7 02 Dacryoadenitis 13 6 7 14y-58y Bil:3 Rt:4 Lt:6 03 Anterior 8 5 3 22y-53y Bil:2 pseudotumor Rt:4 Lt:2 04 Diffuse 5 3 2 18y-57y Bil:1 Pseudotumor Rt:1 Lt: 3 05 Apical 2 2 0 27y-44y Rt:1 Pseudotumor Lt:1 Total 46 cases 27(58.69%) 19(41.30%) 12-5 cases cases 8years . Bil: 11(23.91%) Rt: 16(34.78%) Lt: 19(41.30%) Cases KEY: y= years, RT= right, Lt=left, Bil= bilateral into the more frequently occurring Treatment – 60 to 80mg of oral prednisone and differential leukocyte count (TLC,DLC), inflammatory type versus the rare per day. In improving patients, their steroids fasting blood sugar (FBS), serum- thyroid fibromatous type. Perivascular Inflammatory are tapered off after 2-3 weeks at a rate of stimulating hormone (TSH), thyroxin(T3), changes and secondary inclusion of vessels 5 – 10 mg per week over 2 to 3 months thyronine(T4), rheumatoid arthritis (RA) occurs rather more frequently, which testifies period. Failure to respond requires orbital factor, serum angiotensin converting enzymes to the somewhat “atypical” histological biopsy. If biopsy shows benign process, then (ACE), serum calcium level (S-Ca), anti- pattern of the pseudotumor 3, 4. .The disease low-dose orbital radiotherapy (between 1000- nuclear antibodies (ANA), anti-neutrophilic is classified according to the site of 3000 rads) is advised and if biopsy shows -cytoplasmic antibodies (ANCA),anti- inflammatory process which may be localised lymphoproliferative disorder, then smooth muscle antibodies (ASMA),C- or diffused. Localised forms of inflammation appropriate treatment is instituted. Cyctotxic reactive proteins (CRP), , enzyme linked involve the anterior or posterior (apex) orbit, agents (Cyclophosphamide, low dose immunosorbent assay (ELISA) ,Mantoux test extraocular muscles and lacrimal gland. The cyclosporine) have been used in case (MT), polymerase chain reaction (PCR), severe proptosis can lead to exposure refractory to corticosteroids and radiotherapy . Gram’s staining and culture of purulent keratopathy, diplopia, secondary glaucoma 7 . discharge of lids ,conjunctiva, blood culture and blindness. The radiologic evaluation and sensitivity, radio / imaging techniques consists of CT scan and MRI of orbit. The MATERIAL AND METHODS like ultrasound abdomen, x-ray orbits / chest imaging findings, correlated with the clinical Prospectively we studied 46 patients of / sinus, computerised tomography scanning findings, allow a diagnosis in most cases pseudotumor aged between 18 years to (CT Scan) of orbits, magnetic resonance and obviate the need for a biopsy. For 58 years from September 2001 to date at imaging (MRI) of orbits, ocular B-scan, elucidation and confirmation of the suspected department of ophthalmology, Chandka and orbital soft tissue biopsy in recurrent clinical diagnosis, the CT scan is the medical college hospital larkana .In each cases resistant to steroid therapy .For preferred method because of the inherent case , patients name ,age ,sex ,occupation exclusion of systemic cause of the disease, contrast by different attenuation values of ,address , detailed history, examination, and all patients were referred to general physician the orbital fat, muscle, bony structures, and was noted on a specific proforma . The . All the patients were treated by systemic air in the adjacent paranasal sinuses. diagnosis of this idiopathic disease was based corticosteroids, analgesics, topical eye Extraorbital extension, however, especially on the exclusion of other causes of orbital antibiotic drops and ointment to prevent to the cavernous sinuses is better delineated lesions by history, clinical features of eye secondary ocular infection and exposure on MRI imaging. At times orbital fatty and underlying systemic disease, and keratopathy. The steroids resistant and orbital infiltration and perineuritis are better relevant investigations where necessary like soft tissue biopsy proven pseudotumor delineated on fat suppression T1- weighted complete blood cell count with morphology, patients were treated by local orbital MR images than on CT scans. 5,6 erythrocyte sedimentation rate (ESR), total radiotherapy. The follow-up period was 595