Dacryoadenitis: do not forget ANCA vasculitis.

Objective: This paper aimed to describe another form of aggressive limited Granulomatosis with polyangiitis (GPA) revealed by dacryoadenitis. Methods and results: We report an unusually limited GPA in a 48-year-old man presenting with bilateral proptosis. She had never presented kidney or pulmonary manifestations, but her disease was persistently active including oto-rhino-laryngological manifestations, dacryoadenitis, and neurological manifestations unresponsive to corticosteroids and immunosuppressors. Discussion: Granulomatosis with polyangiitis (GPA) is an auto-immune inflammatory vasculitis. Involvement of lacrimal glands as the first presentation is uncommon. It is characterized by the development of granulomas. Patients with orbital mass without lacrimal gland involvement have a higher rate of systemic disease, a severe clinical course, and a higher rate of recurrences. A patient with dacryoadenitis seems to be with a good prognosis. Eye manifestations were significantly more common in patients with pachymeningitis. MPO-ANCA-positive pachymeningitis was more frequent in older female patients. PR3-ANCA-positive pachymeningitis had more severe neurological damage. Induction treatment consists of intravenous methylprednisolone (IV) associated with cyclophosphamide. Conclusion: Faced with dacryoadenitis, it is important to screen for ANCA-associated vasculitis. Abbreviations: GPA = Granulomatosis with polyangiitis, ANCA = Antineutrophil Cytoplasmic Antibodies.

Immunoglobulin G4-related Dacryoadenitis Successfully Treated with Baricitinib.

A 48-year-old woman visited our hospital because of bilateral lacrimal gland enlargement. Her serum immunoglobulin G4 (IgG4) level was high, and positron emission tomography-computed tomography showed significant positive findings in the bilateral lacrimal gland. A biopsy revealed a considerable increase in IgG4/CD138, leading to a diagnosis of IgG4-related dacryoadenitis. The disease did not respond to steroid therapy, so treatment was started with baricitinib because of exacerbation of the original atopic dermatitis and dacryoadenitis after the second dose of the coronavirus disease 2019 (COVID-19) vaccine. Baricitinib was effective for resolving both dermatitis and dacryoadenitis, and steroids were able to be discontinued. The IgG4 level also improved.

Chronic bilateral dacryoadenitis caused by SARS-CoV-2 infection: a case report.

BACKGROUND: Dacryoadenitis is inflammation of the lacrimal gland, mainly caused by viral infection. It can also be caused by bacterial pathogens and non-infectious processes such as auto-immune diseases and malignancy. Chronic dacryoadenitis is rarely linked to SARS-CoV-2 infection, with only five reports in the literature. REPORT: A 26-year-old Arab woman experienced chronic inflammatory dacryoadenitis after a mild SARS-CoV-2 infection, which was successfully treated with oral prednisone. CONCLUSIONS: Dacryoadenitis can occur due to inflammation caused by either SARS-CoV-2 exposure. The treatment typically involves the administration of steroids, with duration to be decided based on clinical response.

Dacryoadenitis post COVID-19 infection and immunization.

Dacryoadenitis can be a rare presentation following COVID-19 infection or can be an immunological response post COVID-19 vaccination. Herein we report two cases of lacrimal gland involvement, one post COVID-19 infection, and the other post COVID-19 vaccination. A definitive causal relationship, however, remains uncertain.

An Unusual Case of Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type Masquerading as Dacryocystitis and Sinusitis.

Extranodal natural killer (NK)/T-cell lymphoma, nasal type (NNKTL) is a rare and highly aggressive non-Hodgkin lymphoma originating from NK or γδ T cells infected by Epstein-Barr virus (EBV). In the United States, NNKTL is usually noted in people of Asian or Hispanic descent. Natural killer/T-cell lymphoma, nasal type commonly involves the upper aerodigestive tract, including the nasopharynx, nasal cavity, Waldeyer's ring, and oropharynx. Extensive local destruction and invasion has been noted, especially of the paranasal sinuses, hard palate, and central nervous system; involvement of the nasolacrimal duct with dacryocystitis is yet to be reported. We report a rare case of a Hispanic man with extranodal NNKTL masquerading as persistent dacryocystitis and necrotizing sinusitis unresponsive to antibiotics and surgical intervention. An extensive background of necrosis and inflammation was noted on pathology, and additional analysis with immunohistochemistry and in situ hybridization after repeat biopsy were necessary for accurate diagnosis.

Epstein-Barr virus-related dacryocystitis: a case report.

BACKGROUND AND OBJECTIVE: Acute dacryocystitis is an atypical and rare manifestation of pediatric mononucleosis still widely underdiagnosed in clinical practice. We report this rare condition and describe challenges in its diagnosis and treatment on the basis of a presented case. CASE PRESENTATION: A 6-year-old Caucasian girl without any ophthalmic history was admitted for right preseptal cellulitis requiring intravenous antibiotic therapy. During hospitalization, she developed a fluctuating lump in the nasolacrimal region which resembled an abscess, both clinically and radiologically. There was no spontaneous purulent discharge. Serology was positive for acute mononucleosis and Epstein-Barr virus-related dacryocystitis was diagnosed. Following multidisciplinary discussion, she was treated conservatively with digital lacrimal sac massages and intravenous antibiotic therapy with an excellent outcome. DISCUSSION: This rare form of Epstein-Barr virus is poorly documented in the literature, and thus barely known. As initial symptoms are nonspecific (rhinitis, fever, eyelid edema and erythema lack of purulent discharge, and moderate bilateral cervical lymphadenopathy), diagnosis is often difficult. Nevertheless, differentiating between dacryocystitis and abscess is crucial to select the appropriate treatment and avoid unnecessary, potentially harmful surgery. Conservative management of dacryocystitis appears to be the gold standard of treatment. CONCLUSION: Acute dacryocystitis in children free of ophthalmic history should raise suspicion of primary Epstein-Barr virus infection. With conservative treatment, prognosis appears to be excellent; therefore, surgery should be avoided as much as possible.

Clinical and Radiological Features of Diffuse Lacrimal Gland Enlargement: Comparisons among Various Etiologies in 91 Biopsy-Confirmed Patients.

OBJECTIVE: To compare the clinical and radiological features of various etiologies of chronic diffuse lacrimal gland enlargement. MATERIALS AND METHODS: We retrospectively reviewed 91 consecutive patients who underwent surgical biopsy for chronic diffuse lacrimal gland enlargement and were diagnosed with non-specific dacryoadenitis (DA) (n = 42), immunoglobulin G4-related dacryoadenitis (IgG4-RD) (n = 33), and lymphoma (n = 16). Data on patient demographics, clinical presentation, and CT imaging findings (n = 73) and MRI (n = 43) were collected. The following radiologic features of lacrimal gland enlargement were evaluated: size, unilaterality, wedge sign, angle with the orbital wall, heterogeneity, signal intensity, degree of enhancement, patterns of dynamic contrast-enhanced, and apparent diffusion coefficient value. Radiological features outside the lacrimal glands, such as extra-lacrimal orbital involvement and extra-orbital head and neck involvement, were also evaluated. The clinical and radiological findings were compared among the three diseases. RESULTS: Compared to the DA and IgG4-RD groups, the lymphoma group was significantly older (mean 59.9 vs. 46.0 and 49.4 years, respectively; p = 0.001) and had a higher frequency of unilateral involvement (62.5% vs. 31.0% and 15.2%, respectively; p = 0.004). Compared to the IgG4-RD and lymphoma groups, the DA group had significantly smaller lacrimal glands (2.3 vs. 2.8 and 3.3 cm, respectively; p < 0.001) and a lower proportion of cases with a wedge sign (54.8% vs. 84.8% and 87.5%, respectively; p = 0.005). The IgG4-RD group showed more frequent involvement of the extra-orbital head and neck structures, including the infraorbital nerve (36.4%), paranasal sinus (72.7%), and salivary gland (58.6%) compared to the DA and lymphoma groups (4.8%-28.6%) (all p < 0.005). CONCLUSION: Patient age, unilaterality, lacrimal gland size, wedge sign, and extra-orbital head and neck involvement differed significantly different between lymphoma, DA, and IgG4-RD. Our results will be useful for the differential diagnosis and proper management of chronic lacrimal gland enlargement.

Bilateral Dacryoadenitis and Central Nervous System Involvement in a Child With Kimura Disease.

Kimura's disease (KD) is a systemic inflammatory condition characterized by lymphadenopathy and subcutaneous nodules in the head and neck region. The lesions have a distinctive histopathological pattern formed by follicular hyperplasia, eosinophilic infiltrates, fibrosis, and vessel proliferation. The disease may occur at all ages but predominates among young males with autoimmune dysfunctions. Visceral and orbital involvement is uncommon. We report a girl with KD who developed bilateral enlargement of the lacrimal glands and a lesion in the left lateral ventricle of the brain indistinguishable from a central nervous system neoplasia. A biopsy of both the lacrimal gland and the lateral ventricle was consistent with KD.

Acute-onset dacryoadenitis following immunisation with mRNA COVID-19 vaccine.

A 14-year-old boy was referred to the ophthalmology department with a 4-day history of rapid-onset right upper lid pain, swelling and erythema starting 9 hours after his first dose of COVID-19 mRNA vaccination (BNT162b2/Comirnaty, Pfizer-BioNTech). On examination, he had significant right upper lid ptosis, oedema and erythema, with associated limitation of right eye abduction and elevation. He was found to have acute dacryoadenitis with orbital inflammatory disease on clinical and laboratory investigations. He was given tapering oral prednisone and had full resolution of symptoms within 2 weeks. This is the first known case of orbital inflammation after COVID-19 mRNA vaccination. Given the temporal association between the patient's vaccination and symptom onset, we believe it is likely that immunisation prompted the onset of disease.

Epiphora and Dacryocystitis After Transcanalicular Laser-assisted Dacryocystorhinostomy due to an Undiagnosed Dacryolith.

A 45-year-old female patient who had previously undergone endoscopic dacryocystorhinostomy (EN-DCR) at another surgical center was diagnosed with functional failure after DCR and underwent an uneventful transcanalicular laser-assisted DCR (TCL-DCR). After 5 months, the patient underwent endoscopic examination due to persistent epiphora and dacryocystitis attacks. Endonasal visualization revealed a large dacryolith that filled the ostium and sac and was removed en bloc mechanically with forceps. Carbonized material on the dacryolith's superior part indicated that it was perforated through-and-through by the laser beam during TCL-DCR. The patient's complaints were resolved completely following the removal of the dacryolith. The transcanalicular lacrimal procedures do not allow complete visualization of the contents of the lacrimal sac, and the laser beam can ablate even an undetected dacryolith and may result in unsuccessful DCR, although the surgical course may look completely uneventful.

Acute dacryoadenitis in a patient with SARS-CoV-2 infection.

We describe a retrospective case report of dacryoadenitis associated with orbital inflammatory disease in a patient with confirmed SARS-CoV-2 infection.A 22-year-old previously fit and healthy male presented with 4-day history of right ocular redness, eyelid swelling and blurred vision associated with discomfort and pain in the lacrimal gland area. He was found to have right acute dacryoadenitis based on clinical examination and orbital imaging. One day after initiation of oral antibiotic and non-steroidal anti-inflammatory therapy, he developed worsening of the orbital inflammation and partial ophthalmoplegia. Oral steroids were commenced resulting in rapid resolution of symptoms within a few days and clinical stability at 2 months.The patient did not have any systemic features of COVID-19 but he was in close contact with his mother and with his partner who both had respiratory symptoms and tested positive for SARS-CoV-2 antigen (PCR testing) 4 weeks prior. PCR testing from nasopharyngeal swab was negative for SARS-CoV-2 RNA; however, the serological test was positive for IgM/IgG SARS-CoV-2 antibodies. Extensive laboratory workup including infectious and autoimmune screening and chest x-ray were unremarkable.Orbital inflammatory disease due to infectious process or immunological response may potentially occur in COVID-19 patients, although the causal relationship remains uncertain.

Natural killer T-cell lymphoma causing bilateral recurrent recalcitrant dacryocystitis.

A 35-year-old female with a history of chronic extensive rhinosinusitis, previously treated twice with functional endoscopic sinus surgery, presented with recurrent dacryocystitis despite prior dacryocystorhinostomy. Histopathological specimens taken at the most recent sinus surgery demonstrated a lymphocytic inflammatory reaction without evidence of angiodestruction or necrosis. Flow cytometry was normal. Over the following 9 months, the patient developed worsening hypertelorism and bilateral recurrent acute dacryocystitis with a fistula tract to the skin. Neuroimaging revealed a hyperintense enhancing soft tissue expansion into the periorbital regions, invading the nasolacrimal canals, and obstructing the paranasal sinuses. A skin biopsy at the fistula site revealed natural killer T-cell lymphoma. Metastatic work-up disclosed lung, spleen, and bone marrow involvement. The patient underwent chemotherapy with mixed clinical response, and ultimately passed away from metastatic disease. The authors present a rare case of natural killer T-cell lymphoma involving the nasolacrimal sac, presenting as recurrent dacryocystitis and diagnosed by skin biopsy of the fistula site.

Congenital Nasolacrimal Duct Obstruction Update Study (CUP Study): Paper 4-Infantile Acute Dacryocystitis (InAD)-Presentation, Management, and Outcomes.

PURPOSE: To study the presentation, management, and outcomes of infantile acute dacryocystitis. METHODS: Retrospective study of infants diagnosed with acute dacryocystitis over a period from June 2016 to December 2019. Data collected include demographics, clinical history, presenting features, management, complications, and outcomes. Treatment provided was intensive medical care followed by early probing under endoscopic guidance. Further interventions, where needed, were performed based on intraoperative findings during probing. Successful outcomes were defined as resolution of infection, subjective relief from epiphora, and anatomical patency determined by a normal fluorescein dye disappearance test. RESULTS: Twenty-seven eyes of 27 infants were analyzed during the study period. The mean age of infants was 3.26 months, and males were more affected than females (male:female 15:12). The mean duration of symptoms was 4.66 weeks, with 96% (26/27) presenting with symptoms of redness, watering, discharge, and swelling. Preseptal cellulitis was seen in 74% (20/27) infants, lacrimal abscess in 67% (18/27) infants, and lacrimal fistula in 37% (10/27). Most infants had complex congenital nasolacrimal duct obstruction (62%, 13/27), of which 7 also had intranasal cysts. One infant successfully underwent endoscopic dacryocystorhinostomy. At a mean follow-up period of 7.95 months, successful outcomes were observed in 90.4% (19/21) infants. The 2 failed cases were complex congenital nasolacrimal duct obstruction with associated sac diverticula and are scheduled for an endoscopic dacryocystorhinostomy. CONCLUSION: Infantile acute dacryocystitis is a distinct clinical entity. The outcomes of systemic antibiotics and early probing are excellent.

Small Cell Neuroendocrine Carcinoma Presenting as Recurrent Dacryocystitis: Case Report of a Rare Entity.

An 84-year-old man presented with a localized, firm, tender mass over the right lacrimal sac. He had a history of acute dacryocystitis in the same eye 6 months before presentation, which resolved with antibiotics followed by uneventful dacryocystorhinostomy. At repeat presentation, the patient underwent orbital imaging and excisional biopsy of the lesion. Histologic studies revealed a small cell neuroendocrine carcinoma. The patient was subsequently treated with chemotherapy and radiation. Although there are rare reports of small cell neuroendocrine carcinoma originating in the sino-orbital-lacrimal region, this is the first report of tumor presentation with acute dacryocystitis in a patient with prior dacryocystorhinostomy.