The association of lacrimal gland inflammation with alopecia areata.

PURPOSE: To describe the association of lacrimal gland inflammation with alopecia areata. METHODS: We reviewed the medical records of 4 patients diagnosed with lacrimal gland inflammation who had an antecedent or subsequent episode of alopecia. Data was collected on the presentation age, gender, medical history, disease onset, symptoms and signs, imaging, histopathology, systemic evaluation, management and outcome. Pathology and imaging results were correlated with clinical findings. RESULTS: Three patients were Asian and one Caucasian. Two developed alopecia after presentation for lacrimal inflammation. The remaining two had a history of alopecia totalis (2 years and 10 years). Three of the 4 patients presented or developed other systemic disorders, including seizures, thrombocytopenia, optic neuritis, ulcerative colitis, allergic rhinitis, lymphadenopathy, vasculitic rash and positive p-ANCA values. All received oral corticosteroids, with the addition of methotrexate therapy in one for relapsing inflammation. CONCLUSIONS: Lacrimal gland inflammation and alopecia areata are autoimmune processes that can be seen in association with each other.

A 5-year retrospective review of Asian ectropion: how does it compare to ectropion amongst non-Asians?

INTRODUCTION: This study reviews the differences in demographics and surgical outcomes between ectropion in Asian and non-Asian eyes. MATERIALS AND METHODS: Medical records of surgically corrected ectropion cases from January 2002 to December 2006 were reviewed. Preand postoperative lid-globe apposition was graded: grade 0 with normal lid-globe apposition, grade 1 with punctal ectropion, grade 2 with partial lid eversion and scleral show, grade 3 with conjunctival hyperemia and thickening and grade 4 as for grade 3 with exposure keratitis. RESULTS: Sixty-nine eyes in 50 patients underwent surgical correction of lower lid ectropion, making up 3.3% of all lid procedures performed. Eighty-four percent of patients were above 50 years of age, 72% were males and 88% were Chinese. Involutional change was the commonest aetiology, accounting for the majority of bilateral cases. The mean duration to surgery was 10.0 ± 16.0 months. The most frequent preoperative severity grade was 2. Lateral tarsal strip (LTS) was the commonest procedure performed, comprising 91.3% of eyes. The mean duration of postoperative review was 19.4 ± 19.2 months (range, 1 to 74 months). Postoperative improvement of at least one grade was observed in 98% while normal lid-globe apposition was achieved in 76% of eyes. CONCLUSIONS: Involutional change is the most common cause of ectropion amongst both Asians and non-Asians. Ectropion is less prevalent amongst Asians as a result of anatomical differences and possibly reduced sun exposure. The LTS procedure is the most commonly performed surgical procedure for the successful correction of ectropion in both Asians and non-Asians.

Normative measurements of the Chinese extraocular musculature by high-field magnetic resonance imaging.

PURPOSE: To establish the normal measurements of diameter and cross-sectional area of the Chinese extraocular muscles (EOMs) and optic nerve-sheath complex (ON) as seen on high-field (3-Tesla; 3T) magnetic resonance imaging (MRI). METHODS: Coronal 3T MRI of the orbit (TR, 700 ms; TE, 15 ms; FOV, 160 mm; matrix, 256 x 256; slice thickness, 1 mm; and slice gap, 0) was performed on 80 normal Chinese volunteers. T1-weighted images were analyzed with computer-assisted segmentation. The diameter and the cross-sectional area of EOMs and the ON were measured from the image plane at the posterior aspect of the globe and at 7 mm behind the globe. The maximum diameter of EOM was measured from their respective reconstructed images. RESULTS: The normal measurement (mean +/- SD) of diameter at the posterior aspect of the globe: medial rectus (MR), 3.6 +/- 0.4 mm; inferior rectus (IR), 3.7 +/- 0.8 mm; lateral rectus (LR), 2.3 +/- 0.5 mm; superior oblique (SO), 2.4 +/- 0.6 mm; and the ON, 5.4 +/- 0.7 mm. The normal measurement of diameter 7 mm behind the globe: MR, 3.2 +/- 0.6 mm; IR, 3.9 +/- 0.8 mm; LR, 4.0 +/- 0.8 mm; SO, 2.2 +/- 0.7 mm; and ON, 4.2 +/- 0.5 mm. The normal measurement of cross-sectional area at the posterior aspect of the globe: superior muscle group (SMG), 28.2 +/- 6.5 mm(2); MR, 25.5 +/- 3.2 mm(2); IR, 26.9 +/- 7.2 mm(2); LR, 17.3 +/- 3.9 mm(2); SO, 10.5 +/- 3.5 mm(2); and ON, 27.6 +/- 6.1 mm(2). The normal measurement of cross-sectional area 7 mm behind the globe: SMG, 24.1 +/- 6.6 mm(2); MR, 19.1 +/- 4.6 mm(2); IR, 29.5 +/- 6.6 mm(2); LR, 32.3 +/- 6.5 mm(2); SO, 9.7 +/- 3.9 mm(2); and ON, 17.1 +/- 2.9 mm(2). The normal measurement of maximum diameter: SMG, 4.8 +/- 1.1 mm; MR, 5.1 +/- 0.9 mm; IR, 5.4 +/- 1.0 mm; and LR, 4.5 +/- 0.9 mm. CONCLUSIONS: The Chinese 3T MRI EOMs and ON normative data may be useful in determining pathologic enlargement of the EOMs and ON in various orbital conditions. (ClinicalTrials.gov number, NCT00348413.).

Bilateral orbital varices: an approach to management.

The authors report a case of bilateral orbital varices in a 19-year-old man with a 7-year history of intermittent left proptosis and dystopia. CT demonstrated enhancing lesions with phleboliths and gadolinium-enhanced MRI showed characteristic hyperintense lesions. The asymptomatic right lesion was treated conservatively; the left lesion was excised following intralesional injection of cyanoacrylate. Bilaterality in orbital varices may not be obvious clinically and only diagnosed radiologically. A multidisciplinary approach involving the ophthalmologist and interventional radiologist using intraoperative fluoroscopy enabled accurate characterization, delineation, embolizationm, and excision of the lesion with good hemostatic control.

Nasopharyngeal carcinoma with orbital involvement.

PURPOSE: To describe the clinical presentation of patients with nasopharyngeal carcinoma and orbital involvement. METHODS: A retrospective case series of 9 patients with nasopharyngeal carcinoma presenting with orbital involvement from March 2003 to April 2006 at the Singapore National Eye Centre were reviewed. The clinical profile, neuroradiologic evaluation, and clinical course of these patients were reviewed. RESULTS: There were 3 women and 6 men with stage T4 disease. The mean age was 49.2 years +/- 7.69 years (range, 37-57 years). The mean time from initial diagnosis of nasopharyngeal carcinoma to orbital involvement was 8.2 years (range, 0.8-29 years). One patient had no prior history of nasopharyngeal carcinoma. Four patients presented with an eyelid mass and 2 patients reported tearing. Other symptoms included diplopia, blurred vision, and protrusion of the eye. Imaging revealed tumor involvement of the orbital apex, inferior orbital fissures with intraconal spread, and infiltration of the lacrimal sac. The recurrences were confirmed histologically. Patients were treated with radiotherapy and/or chemotherapy. There were 3 deaths from advanced nasopharyngeal carcinoma during the follow-up period. The time interval from diagnosis of orbital involvement to death ranged from 8 months to 24 months. CONCLUSION: Ocular symptoms and signs may be the initial presentation of recurrent nasopharyngeal carcinoma. One must therefore be aware of possible tumor recurrence in patients with a prior history of nasopharyngeal carcinoma who present with symptoms of tearing or an eyelid mass, as this would enable prompt referral to the oncologist and otorhinolaryngologist.

Epibulbar osseous choristoma: a report of two cases.

Epibulbar osseous choristoma occurs rarely and is commonly mistaken for a dermoid or dermolipoma. We report two cases: an 8-year-old boy and a 79-year-old lady with epibulbar osseous choristomas where computed tomography showed an extraocular lesion in the temporal quadrant and diagnosis was confirmed by excision biopsy. The osseous component was adherent to the sclera in the young patient, while it was loosely attached to the surrounding tissue in the older patient. We believe that the 79-year-old lady is the oldest reported patient with an epibulbar osseous choristoma.

Medial pretarsal adipose tissue in the Asian upper eyelid.

PURPOSE: To report the presence of a localized pad of adipose tissue in the medial anterior tarsal surface of the Asian upper eyelid. METHODS: Clinicohistologic case series. The presence and extent of a localized pad of adipose tissue in the medial pretarsal area were documented: 1) intraoperatively for 51 consecutive upper eyelid surgeries; and 2) in the histologic study of 10 cadaveric upper eyelid specimens. RESULTS: Medial pretarsal adipose tissue, ranging from 5 mm to 22 mm in size, was consistently found in all clinical patients regardless of age, gender, ethnicity, or underlying pathology. Histologic examination of the cadaveric specimens confirmed the presence of a localized pad of adipose tissue on the anterior surface of the superior tarsus medially. CONCLUSIONS: The presence of a localized pad of adipose tissue on the anterior surface of the medial superior tarsus seems to be a consistent finding in Asians.

Visual recovery following emergent orbital decompression in traumatic retrobulbar haemorrhage.

INTRODUCTION: Acute retrobulbar haemorrhage is a potentially sight-threatening condition, and can follow retrobulbar anaesthesia or trauma to the orbit. Acute loss of vision can occur with retrobulbar haemorrhage and is reversible if the condition is recognised and treated early. CLINICAL PICTURE: We report a case of acute retrobulbar haemorrhage following orbital trauma in a 78-year-old Chinese lady. TREATMENT: The patient was on follow-up for a mature cataract in the right eye and had been scheduled for cataract surgery. The patient presented to the emergency department with acute loss of vision in the right eye, severe proptosis and tense periorbital haematoma after she hit her right face following a fall. Computed tomography scans revealed fractures of the floor, lateral and medial walls of the right orbit as well as retrobulbar and periorbital haematoma. There was marked proptosis and tenting of the globe with stretching of the optic nerve. Emergent lateral canthotomy and cantholysis was performed at the emergency department. The patient subsequently underwent surgical evacuation of the orbital haematoma. OUTCOME: The patient's vision in the right eye recovered from no perception of light to light perception over the next few days. After a month of follow-up, the patient underwent right cataract surgery, and her best corrected visual acuity was 6/12 part. CONCLUSION: In severe acute retrobulbar haemorrhage, prompt surgical evacuation of the haematoma can reverse visual loss.

Lacrimal gland amyloidosis.

Primary localized lacrimal gland amyloidosis is an extremely rare occurrence. The typical clinical and radiologic appearances have been suggested but not clearly established. This report describes two patients with amyloid tumor of the lacrimal gland. Both patients were middle-aged women of Asian origin, one a Chinese with unilateral lacrimal gland swelling and the other, a Malay with bilateral lacrimal gland swelling. Neither patient had associated systemic disease. They were otherwise asymptomatic except for prominence over the lacrimal gland region. Neither visual impairment nor evidence of optic nerve dysfunction was noted in either patient. CT findings mimic that of an inflammatory or lymphoproliferative disorder of the lacrimal gland. The diagnosis of amyloidosis was only proved on histology. Further investigations revealed that the disease process was truly localized and not part of a systemic process. Subsequent observation proved no recurrence.

Detection of thyroid peroxidase mRNA and protein in orbital tissue.

OBJECTIVE: We have previously reported that the absence of thyroid peroxidase antibodies (TPOAb) in Graves' disease (GD) was associated with an increased risk of Graves' ophthalmopathy (GO). This observation raised the possibility that TPOAb could act as a protective factor. However, the presence of thyroid peroxidase (TPO) in the orbit has not been previously reported. The aim of this study was to confirm or exclude the presence of orbital TPO. METHODS AND DESIGN: Relative TPO mRNA expression from GO (n=6) and normal (n=5) orbital fat tissue was determined using real-time PCR technique. Orbital fat in the normal group from blepharoplasty represents extraconal (anterior) fat. mRNA expression in fibroblasts grown from these tissues before and after adipocyte differentiation was also documented. Finally, Western blotting was carried out to verify translation of TPO mRNA transcripts. RESULTS AND DISCUSSION: TPO transcripts were detected in the orbital fat tissue obtained from normal and GO subjects using the real-time PCR technique. TPO expression was increased in GO compared to normal (N) tissues. However, TPO expression in cultured fibroblasts was similar in both groups and adipogenesis did not appear to alter TPO expression. Protein was detected by Western blot analysis using the TPO MAB 47 (mAb 47). The predicted 110-kDa band was detected in orbital fat as well as in orbital fibroblasts. Our results suggest the presence of TPO in GO and N orbital tissues. We hypothesise that immune responses directed against orbital TPO might play a role in modulating the clinical expression of GO.

Optic nerve toxoplasmosis and orbital inflammation as initial presentation of AIDS.

PURPOSE: To report a case of toxoplasmosis with optic nerve and orbital involvement as the initial presentation of HIV infection. METHOD: Case report. RESULTS: A 46-year-old zookeeper, who had had right central retinal vein occlusion (CRVO) 2 weeks previously, presented with painless lid and conjunctival swelling and profound visual loss in his right eye (RE). Examination revealed no light perception (NLP) RE with axial proptosis and ocular motility restriction; fundal examination revealed a clinical picture of an ischaemic CRVO. MRI of the brain and orbit showed ring-enhancing targetoid lesions in the brain and inflammatory changes in the right optic nerve, extraocular muscles and orbital fat. He was subsequently found to be HIV positive and had positive toxoplasma IgG serology. CONCLUSIONS: Immunocompromised individuals have an increased likelihood for more severe and atypical presentations; this highlights the need for increased index of suspicion for HIV infection as ocular or orbital disease may be the first manifestation of life-threatening systemic toxoplasmosis.

Lower lid epiblepharon as the presenting feature of spontaneous dural carotid cavernous fistula in an Asian patient.

Lower lid epiblepharon is a relatively common problem among young Asian patients. Occasionally, it may be secondary to underlying orbital pathology. We report a 33-year-old Chinese patient with a dural carotid cavernous fistula who presented with an ipsilateral lower lid epiblepharon in the absence of any other significant signs. After a recurrence following primary corrective lid surgery, she was investigated further because of mild ipsilateral proptosis. CT-scans of the orbit revealed an enlarged superior ophthalmic vein and a dural carotid cavernous fistula was confirmed on angiography. Subsequent embolisation of the fistula led to resolution of the proptosis as well as the epiblepharon. We believe that extension of orbital fat to the inferior tarsal border in Asian eyelids together with a poorly fixed anterior lamella contributes to the occurrence of epiblepharon when the Asian orbit becomes congested. The new onset of such features in the Asian patient should lead one to suspect underlying orbital pathology, even in the absence of other, more classic signs.

Severe Graves' ophthalmopathy after retrobulbar anesthesia for cataract extraction in a patient with mild stable thyroid eye disease.

It has been hypothesized that the distinct anatomic localization of the Graves' triad may be partially explained by pressure and trauma. While there are reports of local trauma clearly contributing to the pathogenesis of pretibial myxedema, direct evidence for a similar mechanism in Graves' ophthalmopathy (GO) has been lacking. We describe a 65-year-old male patient with stable mild Graves' ophthalmopathy of 24 years' duration in whom a retrobulbar block was administered prior to cataract removal. Three weeks after the procedure, he complained of rapidly progressive bilateral diplopia. In 6 months, there was moderate exophthalmos, exposure keratitis, almost complete ophthalmoplegia, and decreasing visual acuity requiring surgical decompression. Postdecompression, inflammatory signs and vision improved but there was complete ophthalmoplegia. The eye signs remained unchanged for the next 4 months but there was exacerbation of the disease within a week of receiving radioiodine despite concomitant steroid administration. Orbital irradiation was finally administered with rapid improvement in extraocular eye muscle function. We hypothesize that local inflammatory and immune responses stimulated by trauma and/or pressure in the retrobulbar compartment, triggered the development of severe ophthalmopathy in this patient. Thyroid-stimulating immunoglobulin (TSI) levels remained markedly elevated despite the clinical improvement suggesting that the beneficial effects of radiotherapy in this case were not mediated by suppressing TSI production.