|DIAGNOSTIC AND THERAPEUTIC CHALLENGES
|Year : 2017 | Volume
| Issue : 3 | Page : 218-219
Marian Pauly, Mekhla Naik, TJ Maya
Department of Orbit, Oculoplasty and Ocular Oncology, Giridhar Eye Institute, Kochi, Kerala, India
|Date of Web Publication||30-Jan-2018|
Dr. Marian Pauly
Department of Orbit, Oculoplasty and Ocular Oncology, Giridhar Eye Institute, Kochi - 682 020, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Pauly M, Naik M, Maya T J. Double-trouble. Kerala J Ophthalmol 2017;29:218-9
A 57-year-old male presented to us in March 2014 with complaints of bulging of right eye and drooping of right upper lid for the last 10 months which started in 2013 with redness and lid edema, gradual in onset, progressive in nature. He was treated with systemic antibiotics and steroids elsewhere. Because of the persistent symptoms, he underwent a complete body workup along with imaging and incisional biopsy.
All systemic investigations including ultrasonography – abdomen and neck, Mantoux test, and cerebrospinal fluid analysis (polymerase chain reaction [PCR] for acid-fast bacilli [AFB]) were normal. Magnetic resonance imaging (MRI) - brain and magnetic resonance angiography with contrast showed enhancing heterogeneous mass in the extraconal, preseptal space of the right orbit. Incisional biopsy done elsewhere showed granulomatous inflammation and focal necrosis. AFB was negative and Gomori methenamine silver (GMS) stain was negative for fungus. The patient was advised to undergo antituberculosis treatment (ATT). However, the patient deferred ATT and consulted elsewhere and started ayurvedic treatment for the same.
He was a known case of diabetic mellitus for 9 years on treatment and had recently diagnosed ischemic heart disease awaiting coronary artery bypass grafting. He also gave a family history of glaucoma in mother.
On examination [Figure 1], the patient had a chin up position with severe ptosis; levator palpebrae superioris action 2 mm and a scar of previous surgery was present over the upper lid. Orbital evaluation revealed 4 mm nonaxial proptosis with hypoglobus. Fullness was noted in the subbrow area. Ocular movement examination showed gross restriction of elevation, mild restriction of abduction and adduction, and normal depression.
His uncorrected visual acuity was 6/24 in the right eye and 6/60 in the left eye. Vision improved to 6/12 N6 in the right eye with correction of −2.75 DS (add + 2.75) and 6/9 N6 in the left eye with correction of −3.50 DS (add + 2.75). Anterior-segment examination was within normal limits. No relative afferent pupillary defect was present. Lens was clear. Fundus showed cup-disc ratio of 0.7 in the right eye and 0.9 in the left eye and mild nonproliferative diabetic retinopathy in the right eye. Intraocular pressure was 20 mmHg in the right eye and 15 mmHg the in left eye by applanation tonometry. Pachymetry was 502 μM in right eye and 486 μM in left eye. Corrected intraocular pressure was 23 mmHg in right eye and 19 mmHg in left eye. Color vision was normal in both eyes.
MRI was repeated on June 2013 and April 2014 [Figure 2]a and [Figure 2]b, in which the mass showed an increase in size to 19 mm. The second opinion on the original histopathology slide was taken which showed granulomatous inflammation with necrosis.
|Figure 2: (a) Clinical picture showing ocular motility, (b) MRI Orbit showing the mass in superotemporal quadrant|
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How will you manage this case?
Dr. Mangesh Dhobekar M. S,
Senior Consultant and Head of Orbit, Oculoplasty and Ocular Prosthetic services
Shri Ganapati Netralaya, Jalna, Maharashtra, India
In a diffuse orbital mass lesion with human placenta extract, granulomatous inflammation and focal necrosis will point toward orbital TB in India.
If AFB/GMS stain was negative, the patient will need to have ancillary tests such as Mx/chest X ray/erythrocyte sedimentation rate.
PCR will need fresh biopsy; if a patient is ready, that would be my first choice: rebiopsy and PCR.
If PCR/ancillary tests were positive, ATT.
If patient is not ready for rebiopsy and/or all ancillary tests negative, he/she will need p-ANCA/c-ANCA/RA/serum ACE to rule out other comparatively rare inflammations and trial of systemic steroids and steroid sparing agents SOS.
If there is no response to steroids, then empirical ATT.
Dr. Mohd Shahid Alam M. S
Assistant Professor, Institute of Ophthalmology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
Granulomatous infection with necrosis is an incomplete pathological diagnosis in case of orbital lesion. I would suggest PCR for Mycobacterium tuberculosis and ask the pathologist to comment upon the presence of plasma cell. If plasma cells are present, I will rule out IgG4 orbital pathology. If not, I will do a systemic workup further to rule out Wegener's granulomatosis. Xanthogranuloma is another possibility which the pathologist has to comment. However, further hematological investigations and repeat biopsy are needed to reach the diagnosis.
Dr. Ravija Patel M. S.
Assistant Professor, M and J Institute of Ophthalmology, Ahmedabad, Gujarat, India
It is indeed a challenging case that is, the patient not responding to steroids for granulomatous inflammation. It can be rare inflammation or steroid-resistant case. I prefer to take opinion from rheumatologist and consider immunosuppressive. I would like to rule out IgG4-related systemic disease also.
| Concluding Remarks|| |
When a patient presents with inflammatory mass lesions not responding to steroids or with partial response to steroids, we consider a possibility of rare inflammations or tumors. Hence, vasculitic workup along with repeat biopsy is needed. Preferably, the pathology has to be read by a person who is specialized in ocular pathology which will give a definite diagnosis.
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Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2]