Bilateral Lacrimal Gland Enlargement Research Articles

Bilateral lacrimal gland enlargement as a clinical manifestation of sarcoidosis

Sarcoidosis is an inflammatory disorder of unknown etiology. The main characteristics of sarcoidosis are non-caseating granulomas which can be formed in affected organs. Eye and its adnexal tissues can also be involved. In the early stages of sarcoidosis, the clinical picture may be mild and not always specific. Although the early signs of sarcoidosis are lung associated, we present a clinical case with diffuse enlargement of the lacrimal glands as a first sign of systemic sarcoidosis. A 21-years-old woman presented to the ophthalmological center with a 2-weeks history of having swollen, painful upper eyelids. Primary eye examination revealed palpable tender masses in the locations of the lacrimal glands. Characteristic for sarcoidosis X-ray findings and blood tests demanded lacrimal gland and lung biopsy for diagnosis verification. In spite sarcoidosis of lacrimal glands is a rare condition, it has to be taken into consideration in the differential diagnosis of lacrimal gland masses.

Bilateral lacrimal gland enlargement in a patient with thyroid orbitopathy

: Thyroid Orbitopathy is a condition associated with thyroid dysfunction. In addition to exophthalmos, eyelid retraction, eyelid oedema, restrictive extra-ocular myopathy, lacrimal gland enlargement can also be observed in these patients. However thyroid orbitopathy is not usually been considered in the differential diagnosis of cases of isolated lacrimal gland enlargement. An elderly male patient at our institution presented with bilateral lacrimal gland enlargement as the clinical or radiologic sign and was later diagnosed as thyroid orbitopathy. Computerized tomography and/or magnetic resonance imaging of the orbits were obtained and demonstrated isolated lacrimal gland enlargement with enlargement of lateral rectus and inferior rectus. A case of bilateral lacrimal gland enlargement found in a patient with thyroid orbitopathy was noted. Patient was investigated and kept on follow up.: This case represents, enlargement of lacrimal gland as a presenting sign of thyroid orbitopathy. Further clinical and radiological studies are essential for a better understanding of the timing of lacrimal gland involvement. In such patients presenting with lacrimal gland enlargement, thyroid orbitopathy should be strongly considere d in the differential diagnosis in addition to causes such as of dacryo-adenitis and neoplasms.

Orbital Sarcoidosis in a High TB Endemic Country – A Case Series from South India

ABSTRACT Aim: We report a series of orbital sarcoid in a south Indian patient population and their outcome. Materials & Methods: Retrospective interventional case series. Results: Eleven out of 144cases of biopsy-proven sarcoidosis had orbital involvement. The mean age was 50.64 ± 9.52years and was more commonly seen in women (90.91%). Unilateral presentation with right orbit involvement was more common. Bilateral lacrimal gland enlargement (4cases), inferior orbital mass presenting as proptosis (4cases), preseptal mass above upper eyelid (2cases) and orbital mass encasing the globe (1case) were the different types of orbital presentation. All cases had a dry eye and a negative mantoux test. CT thorax was abnormal in 7cases (63.61%). Biopsy in all cases showed noncaseating granulomatous inflammation. Oral steroids were the standard mode of treatment and none of the cases had recurrences. Conclusions: Orbital sarcoidosis is seen in a high TB endemic country like India and is definitely underreported.

Long-Term Outcomes of Oral Anti-Tubercular Therapy in Patient with Tubercular Dacryoadenitis: A Case Series

ABSTRACTPurpose: To describe the clinico-radiological features and long-term outcomes in patients with tubercular dacryoadenitis (TbD)Methods: Retrospective, observational study of TbD patients who underwent a thorough clinical examination, orbital imaging study, and tailored ancillary investigations. Polymerase chain reaction (PCR) and microscopy were done in specific cases. A 4-drug anti-tubercular therapy (ATT) was started and clinical response was monitored in all. Patients with a minimum follow-up of 6 months “off-ATT” were included.Results: All patients were women and three presented with pain, blepharoptosis, and bilateral involvement. In all, ESR was raised, Mantoux test was positive and orbital imaging revealed enlarged lacrimal gland/s. Positive PCR and granulomatous inflammation on microscopy were seen in two patients. At a mean follow-up of 17.25 months, all women responded with no relapse or clinical recurrence.Conclusion: Bilateral lacrimal gland enlargement, positive Mantoux & PCR with early response to ATT may provide sufficient evidence for diagnosing TbD.

Bilateral subacute lacrimal gland enlargement mimicking dacryoadenitis in a 7-year-old boy: a rare adverse effect of valproic acid (sodium valproate)

A healthy 7-year-old boy presented with bilateral symmetrical lacrimal gland enlargement; a week later salivary gland enlargement was also noted. Clinical investigations suggested no diagnosis, and surgical biopsy was considered. Valproic acid (sodium valproate), which he was taking for absence seizures, has been reported to cause salivary gland swelling in adults. Suspecting that a similar mechanism could be causal, the drug was discontinued. Complete resolution of the lacrimal and salivary gland enlargement rapidly ensued. This is the first report of lacrimal gland enlargement caused by valproic acid.

IgG4-related disease in idiopathic sclerosing orbital inflammation

AimsTo investigate the frequency of IgG4-related disease (IgG4-RD) among patients previously diagnosed with idiopathic sclerosing orbital inflammation (ISOI), and to compare the clinical features and treatment outcomes of patients with...

IgG4-Related Disease

Immunoglobulin G4-related disease (IgG4-RD) is a multifocal inflammatory disorder that causes tumefactive lesions with a dense lymphoplasmacytic infiltrate rich in IgG4 plasma cells and storiform-pattern fibrosis. The clinical symptoms are relatively mild, and the condition is usually recognized by organ swelling and damage. When referring to the ophthalmic manifestations of IgG4-RD, the term IgG4-related ophthalmic disease (IgG4-ROD) is used. IgG4-ROD is characterized by bilateral lacrimal gland enlargement accompanied by 3 distinctive features: infraorbital nerve enlargement, extraocular myositis, and compressive optic neuropathy. IgG4 implies an underlying systemic disease process requiring evaluation to detect other systemic involvement. This includes hypophysitis and hypertrophic pachymeningitis, entities of neuro-ophthalmic interest. IgG4-ROD usually responds favorably to systemic corticosteroids but may be complicated by relapse during steroid taper. Rituximab has been shown to be effective for controlling steroid-refractory IgG4-RD. In contrast to IgG4-RD, an increasing number of cases of extranodal marginal B-cell lymphoma (MALT type) associated with IgG4-ROD have been described. IgG4 may be a risk factor for later emergence of low-grade B-cell lymphoma.

A Case of Epstein-Barr Virus-Related Dacryoadenitis

Purpose: To report a case of Epstein-Barr virus-related dacryoadenitis. Case summary: A 19-year-old female presented with pain, swelling and redness of both upper eyelids. She experienced rhinorrhea, cough and fever for the previous two weeks. Physical exam showed bilateral cervical lymph node enlargement, and orbital computed tomography (CT) revealed diffuse enlargement and inflammation of both lacrimal glands. Serological testing revealed elevated titers for Epstein-Barr virus nuclear antigen immunoglobulin G (IgG) and Epstein-Barr viral capsid antigens IgG and immunoglobulin M (IgM). Based on these results, clinical diagnosis of Epstein-Barr virus-related acute dacryoadenitis was made. The inflammation subsided after conservative management. Relapse was not observed during the three-month follow-up period. Conclusions: Based on acute bilateral lacrimal gland enlargement, systemic symptoms such as fever, rhinorrhea, cough, lymphadenopathy and serologically-positive results for Epstein-Barr virus, Epstein-Barr virus-related acute dacryoadenitis can be clinically diagnosed and treated with conservative therapy. J Korean Ophthalmol Soc 2014;55(9):1372-1375

Unusual Involvement of IgG4-Related Sclerosing Disease in Lacrimal and Submandibular Glands and Extraocular Muscles

Chronic sclerosing sialadenitis, also known as Kuttner tumor, is a chronic inflammatory disease of the salivary glands that is reported in a few cases in medical literature. Recent reports suggest that certain aspects of sclerosing diseases, including chronic sclerosing sialadenitis or dacryoadenitis, should be classified under immunoglobulin G4 (IgG4)-related sclerosing disease based on immunohistochemical studies. This study reports an unusual case of IgG4-related sclerosing disease appearing simultaneously in the lacrimal glands, submandibular glands, and extraocular muscles. A 56-year-old male presented with complaints of bilateral eyelid swelling and proptosis that began two years ago. Computed tomography confirmed that bilateral submandibular enlargements also existed five years ago in the subject. Orbital computed tomography and magnetic resonance imaging revealed bilateral lacrimal gland enlargement and thickening of extraocular muscles. Typical findings of chronic sclerosing dacryoadenitis were revealed upon pathologic exam of the right lacrimal gland. Immunostaining revealed numerous IgG4-positive plasma cells. Through these clinical features, we make a diagnosis of IgG4-relataed sclerosing disease in the subject.

Nasal Cavity Eosinophilic Angiocentric Fibrosis: CT and MR Imaging Findings

EAF consists of exceedingly rare lesions and presently lacks specific imaging reports. The purpose of this study was to determine the CT and MR imaging features of nasal cavity EAF. We retrospectively reviewed 6 patients with histology-proven EAF in the nasal cavity. All 6 patients underwent CT and 3 patients also underwent MR imaging. The following imaging features were reviewed: 1) location, 2) shape, 3) margin, 4) CT attenuation, and 5) MR imaging signal intensity. In addition, the accompanying changes and the TIC of the DCE MR imaging were analyzed. All occurrences of EAF were located at the anterior nasal cavity, arising from the nasal septum in 4 patients, with irregular shape, and the lateral nasal wall in 2, with oval configuration. The mean size was 29.8 mm (range 12-52 mm). On nonenhanced CT, EAF lesions appeared isoattenuated relative to gray matter. On the T1-weighted image, the lesions were isointense in 3 (100%) patients. On the T2-weighted image, the lesions were isointense in 1 (33.3%) patient and hypointense in 2 (66.7%) patients. Occurrences of EAF revealed moderate inhomogeneous contrast enhancement. Three patients underwent DCE MR imaging and the TICs exhibited a rapidly enhancing and slow washout pattern. One patient had bilateral lacrimal gland enlargement, which displayed low signal intensity on the T2-weighted image. A familiarity with imaging findings, including lesion location, isoattenuation and bony changes, and hypointensity on T2-weighted images may help to accurately diagnose this rare entity.

Dacrioadenite bilateral causada pelo vírus Epstein-Barr: relato de caso

The authors present a rare case of acute bilateral dacryoadenitis in association with infectious mononucleosis. A 27 year-old woman had acute bilateral lacrimal gland enlargement with inflammatory signs. The CT scan findings and laboratorial investigations confirmed the diagnosis of infectious mononucleosis. The systemic treatment with intravenous steroids was initiated leading to complete remission of the signs and symptoms. Dacryoadenitis is an uncommon manifestation of infectious mononucleosis and may minimize other signs of the disease. Mononucleosis has to be considered in acute dacryoadenitis cases. Systemic steroids contribute to the fast regression of inflammatory condition.

Wegener Granulomatosis Causing Compressive Optic Neuropathy in a Child

Wegener granulomatosis is an uncommon illness in children that is known to cause myriad ophthalmic complications, but it is rarely a cause of compressive optic neuropathy. A 17-year-old Hispanic boy with Wegener granulomatosis developed unilateral loss of vision, pain, and proptosis of the left eye. CT findings revealed enlargement of bilateral lacrimal glands with compression of the left optic nerve. The patient was admitted for high-dose intravenous corticosteroids and daily oral cyclophosphamide treatment. The patient's vision, pain, and proptosis improved dramatically, and he is now stable on mycophenolate mofetil and prednisone.

Lacrimal gland enlargement in sickle cell disease

Sickle cell disease (SCD) may have ocular complications. We present the a case of a patient with SCD, with an acute painful episode involving the limbs and periorbital region, who also twice developed an unusual complication of recurrent bilateral lacrimal gland enlargement, not previously reported in the medical literature.

Bilateral lacrimal gland enlargement due to post-transplant lymphoproliferative disorder

Bilateral lacrimal gland enlargement due to post-transplant lymphoproliferative disorder

Bilateral Dacryops: Correlation of Clinical, Radiologic, and Histopathologic Features

Apostolos JT, Deshmukh M, Sanelli PC, Brazzo BG e report a case of bilateral lacrimal gland enlargement due to dacryops. Dedicated contrastenhanced CT of the orbits showed bilateral peripherally enhancing cystic structures that were contiguous with the palpebral lobes of the lacrimal glands. Histopathology confirmed the diagnosis of bilateral dacryops, an extremely rare clinical entity and even rarer bilaterally. Tears are produced by the lacrimal and accessory lacrimal glands and drain through the lacrimal ducts. Their function is to keep the ocular surface moist and clear of debris and bacteria. The major lacrimal glands are located at the anterior superolateral aspects of the orbits, adjacent to the tendons of the superior and lateral rectus muscles. The gland is separated into two lobes: the orbital and the palpebral [1]. Loss of lacrimal ductule contractility combined with distention of the duct wall results in a condition called “dacryops” [2]. Dacryops refers to a cyst of the lacrimal gland and can develop anywhere the lacrimal tissue is present. Cysts develop insidiously and present as painless masses on the eyelids. Their clinical presentation is rare, and their bilateral presentation is encountered even less frequently. We present the clinical, histopathologic, and CT appearance of bilateral dacryops as a unique case.

CT and MR findings of bilateral lacrimal gland enlargement in Sjögren syndrome

Two women with primary Sjögren syndrome underwent computed tomography (CT) and magnetic resonance (MR) imaging because of bilateral lacrimal gland enlargement. Histopathologic confirmation was obtained in both patients. Of the four lacrimal glands, one had lymphoepithelial disease, two had pseudolymphoma and one had mucosa-associated lymphoid tissue (MALT) lymphoma, respectively. From the imaging findings, however, it was not possible to differentiate benign lymphoproliferative disorders and malignant lymphoma.

Rosai–Dorfman disease presenting as bilateral lacrimal gland enlargement

PURPOSE: To report a patient with bilateral lacrimal gland enlargement as the initial manifestation of Rosai–Dorfman disease. METHODS: Case report. RESULTS: A 14-year-old female presented with left lacrimal gland enlargement followed by right lacrimal gland enlargement 11 weeks later. Bilateral lacrimal gland biopsies were performed, and histopathologic examination revealed the diagnosis of Rosai–Dorfman disease. CONCLUSION: Patients with Rosai–Dorfman disease may present with bilateral lacrimal gland swelling in the absence of lymphadenopathy. Rosai–Dorfman disease should be considered in the differential diagnosis of bilateral lacrimal gland enlargement.

SJÖGREN'S SYNDROME

Sjogren's syndrome is a chronic systemic inflammatory disorder characterized by the presence of dry eyes and dry mouth. Dryness in the eyes and mouth are secondary to diminished glandular production secondary to lymphocytic and plasma cell infiltration. Because of the prominent autoimmune features of this condition, Sjogren's syndrome has recently been referred to as an autoimmune exocrinopathy. 32 Although there is no universally accepted definition of Sjogren's syndrome, the diagnostic triad of keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth) associated with the presence of a connective tissue disorder is commonly employed. 7 Dry eyes may occur with or without lacrimal gland enlargement. Dry mouth may occur with or without salivary gland enlargement. Both primary and secondary forms of this disorder are recognized. Although primary Sjogren's syndrome may be a distinct disorder in the absence of another known connective tissue disease, the most frequent association of Sjogren's syndrome and a connective tissue disorder is with rheumatoid arthritis. Approximately one half of all patients with rheumatoid arthritis will present with Sjogren's syndrome whereas a less frequent association is seen with less common connective tissue disorders such as systemic lupus erythematosus or scleroderma. 30,49 It is important to distinguish these patients from patients that present with sicca complex, which includes only the presence of ocular and oral dryness. Because patients with primary sicca do not appear to have the systemic disease associations that are discussed in this article, they clearly would have a more favorable prognosis. Historically, features of Sjogren's syndrome have been recognized for over a decade. In 1892, Mikulicz reported the findings of bilateral parotid and lacrimal gland enlargement associated with significant round cell inflammation. 24 Although numerous reports appeared between 1883 and 1933 regarding patients with Mikulicz's syndrome, it was not until 1933 that Heinrich Sjogren described the syndrome that bears his name. 31 It is now generally accepted that Sjogren's syndrome and Mikulitz's disease are in fact variants of the same disorder. 25 It is important for the clinician to consider the diagnosis of Sjogren's syndrome in a patient who presents with suspected allergic disease. Patients with Sjogren's syndrome may present not only with dry eyes and dry mouth but may have prominent respiratory and ocular manifestations that are suspected to be of allergic origin. Because Sjogren's syndrome is suspected to affect 2 to 3 million individuals in the United States alone, 46 it is considered to be in fact the most common autoimmune disease process. Given its reasonably frequent occurrence and its potential for associated lymphoid malignancies, it is crucial that the clinician be aware of the manifestations of this disorder.

Primary Localized Amyloidosis of the Lacrimal Glands

Primary localized amyloidosis causing bilateral lacrimal enlargement is rare. The pathogenesis of amyloid deposition within the orbit and other body tissues has not been fully elucidated. The authors report the case of a 72-year-old woman who presented with bilateral lacrimal gland enlargement secondary to amyloid infiltration. The chemical nature of the deposit was characterized using light microscopy, immunohistochemistry, and immunoelectron microscopy. The primary (immunocytic) nature of the amyloid was confirmed by immunohistochemistry demonstrating the presence of monoclonal lambda light chains in the amyloid deposits and in the plasma cells. Using immunoelectron microscopy, amyloid deposits were seen containing lambda light chains in macrophages. It has been postulated that the macrophage has a role in amyloid deposition. The authors believe this to be the first published report of immunoelectron microscopy use in orbital amyloidosis, and that this technique has helped further their understanding of the nature and pathogenesis of this condition.

Chronic bilateral dacryo-adenitis in identical twins: a possible incomplete form of Sjögren syndrome

We report an unusual case of chronic bilateral dacryo-adenitis in 10-year-old identical twin sisters. Both girls presented with bilateral lacrimal gland enlargement and developed moderate xerophthalmia and keratitis. Both the lacrimal and minor salivary gland biopsies showed a non-granulomatous inflammatory infiltration of mono-nuclear cells. All granulomatous diseases and neoplasms could therefore be ruled out and only Sjögren syndrome and very few other forms of chronic dacryo-adenitis remained as possible diagnoses. Both patients and their parents were evaluated for auto-antibodies. Very low titres of smooth muscle antibodies were found in one, antinuclear antibodies in two and anti-dsDNA antibodies in all four members of the family. Even though the titres of antinuclear and anti-dsDNA antibodies increased in one of the sisters, both patients did not develop any sign or symptom of a systemic connective tissue disease. During the 6 years' follow up, both patients showed persistent tarsal gland enlargement but no other symptoms apart from a moderate xerophthalmia and occasional mild keratitis.