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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 534-536

Systemic sarcoidosis presenting as nasolacrimal duct obstruction – A rare presentation


1 Department of Orbit, Oculoplasty and Ocular Oncology, Giridhar Eye Institute, Kochi, Kerala, India
2 Department of Pathology, Vision Research Foundation, Chennai, Tamil Nadu, India

Date of Submission01-Jun-2021
Date of Acceptance13-Jan-2022
Date of Web Publication13-Apr-2022

Correspondence Address:
Shebin Salim
Department of Orbit, Oculoplasty and Ocular Oncology, Giridhar Eye Institute, Kochi, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1524_21

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  Abstract 


Lacrimal sac sarcoidosis is rare and reported in 7.7% of the cases of systemic sarcoidosis. Nasolacrimal duct obstruction, as the initial presentation of sarcoidosis, is very rare. A 50-year-old woman presented to us with complaints of watering and discharge from the right eye for the past 3 months. On examination, she was found to have a right-sided nasolacrimal duct obstruction and underwent external dacryocystorhinostomy (DCR). Intraoperatively, a biopsy was done because of the abnormally thickened sac wall. The histopathology examination was suggestive of granulomatous inflammation. After ruling out tuberculosis and fungal infection with the appropriate investigation, a diagnosis of pulmonary sarcoidosis with lacrimal sac involvement was made because of the mediastinal lymphadenopathy on computed tomography. She was treated with a course of oral steroids along with immunosuppressants for 3 months. There was no evidence of recurrence at 8 months of follow-up.

Keywords: Lacrimal sac sarcoidosis, nasolacrimal duct obstruction, nasolacrimal system sarcoidosis


How to cite this article:
Salim S, Pauly M, Krishnakumar S. Systemic sarcoidosis presenting as nasolacrimal duct obstruction – A rare presentation. Indian J Ophthalmol Case Rep 2022;2:534-6

How to cite this URL:
Salim S, Pauly M, Krishnakumar S. Systemic sarcoidosis presenting as nasolacrimal duct obstruction – A rare presentation. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 18];2:534-6. Available from: https://www.ijoreports.in/text.asp?2022/2/2/534/342888



Sarcoidosis is a multisystem granulomatous disease of unknown etiology. The incidence is more common in females as compared to males. Though the lungs are the most commonly involved organ, they can affect any system in the body. Uveitis is the most common ocular manifestation. Ocular adnexal involvement is rare. Among the periocular tissues, lacrimal gland involvement is the most common, followed by the orbit, eyelids, and lacrimal sac.[1] Lacrimal sac involvement in sarcoidosis has been reported in 7.7% of the cases.[1] Nasolacrimal duct obstruction as the initial presentation of sarcoidosis is very rare. Only six cases are reported in the past 20 years [Table 1].[2],[3],[4],[5],[6],[7] Here we report a case of systemic sarcoidosis presented as nasolacrimal duct obstruction as the initial manifestation. The study adhered to the tenets of the Declaration of Helsinki.
Table 1: Review of the literature of past 20 years

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  Case Report Top


A 50-year-old woman presented to us with complaints of watering and discharge from the right eye for the past 3 months. On examination, she was found to have a right-sided nasolacrimal duct obstruction. Her best-corrected visual acuity was 6/6 on both eyes. The rest of her anterior and posterior segment examination was within normal limits. She also had systemic hypertension, for which she was taking treatment for the past 2 months. She underwent a right-sided external dacryocystorhinostomy (DCR) to relieve the symptoms of epiphora and discharge. Intraoperatively, the lacrimal sac wall was found to be abnormally thickened and hence a biopsy was done. The histopathology examination of the lacrimal sac tissue showed multiple foci of discrete non-caseating granulomas comprising epithelioid histiocytes, a few Langhans giant cells, and scattered lymphoplasmacytic infiltrates, suggestive of granulomatous inflammation [Figure 1]a. Gomori methenamine silver staining was negative for fungus. She was further evaluated to rule out tuberculosis and sarcoidosis considering the histopathology. Tuberculin skin test and cartridge-based nucleic acid amplification test (CBNAAT) of the sputum to detect tuberculosis were negative. Serum angiotensin-converting enzyme level was 37.5 units/liter (biological reference interval <40). Chest X-ray showed a prominent hilar shadow. Computed tomography (CT) scan of the thorax revealed mediastinal lymphadenopathy [Figure 1]b. Based on investigations, a diagnosis of systemic sarcoidosis with pulmonary and lacrimal sac involvement was made. When we retrospectively asked, the patient gave a history of dry cough present for a few years. She was referred to a pulmonologist and rheumatologist for further management. She had received a course of oral steroids along with immunosuppressants in the form of oral methotrexate for 3 months. She was last seen 8 months post-surgery without any evidence of recurrence and is under regular follow-up from the treating rheumatologist.
Figure 1: (a) Histopathology examination showing non-caseating granulomatous inflammation. (eosin and hematoxylin stain, 40× magnification). (b) Computed tomography scan of the thorax: mediastinal window showing the mediastinal lymph node enlargement

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  Discussion Top


Sarcoidosis is a multisystem disease characterized by a T-helper response in which CD4+ T lymphocytes and activated macrophages form granulomas in the affected organs. Sarcoidosis can be asymptomatic in many cases and sometimes shows spontaneous remission. In severe cases, it can cause lung fibrosis and respiratory failure. Hence, diagnosing the disease entity is important for timely intervention. The diagnosis of systemic sarcoidosis is mainly based on three criteria: (1) typical clinical and radiological features, (2) pathological evidence of non-caseating granulomas, and (3) exclusion of other similar diseases.[8] A transbronchial lung/mediastinal lymph node biopsy is not always needed if any other possible site for a biopsy are available. Also, clinical and/or radiological features alone may be diagnostic for patients with Stage 1 or Stage 2 disease.[8] In the present case, the patient had symptoms in the form of nasolacrimal duct obstruction and dry cough, mediastinal lymphadenopathy on chest imaging, and non-caseating granulomas on sac wall biopsy.

Lacrimal sac sarcoidosis is very rare and is reported to be associated with the upper respiratory system sarcoidosis.[3] It presents as nasolacrimal duct obstruction with symptoms of watering and discharge. There can be associated nasal congestion and or medial canthal lesion.[3],[7] Avisar et al.[3] noted 57% of patients having additional symptoms of nasal congestion. The lacrimal sac and nasal mucosa in sarcoid involvement are found to be abnormally thickened, yellowish, crusty, edematous, and friable, with nodules sometimes.[3],[5],[9] In our case, the sac wall appeared abnormally thickened but the nasal mucosa was normal.

The mainstay of treatment in sarcoidosis is immunosuppressive therapy. The indications include symptomatic, progressive disease, critical organ involvement (ocular, cardiac, and nervous systems), or any organ dysfunction. Various agents used for treating sarcoidosis include corticosteroids, hydroxychloroquine, steroid-sparing agents (methotrexate, azathioprine, leflunomide, mycophenolate mofetil), and anti-tumor necrosis factor (TNF)-α inhibitors.[10]

Literature shows an increased risk of failure following DCR in patients with lacrimal sac sarcoidosis.[1],[7] This is attributed to the ongoing inflammation at the site of the nasolacrimal anastomosis. However, in our case, the patient is doing well without any recurrence of epiphora at the 8-month follow-up. Nevertheless, a careful follow-up is advocated, given the high risk of failure.

There are different opinions regarding postoperative immunosuppressive therapy in lacrimal sac sarcoidosis. Although Ramlee et al.[5] report satisfactory results post-DCR surgery in patients with sarcoidosis without any additional systemic therapy, Chapman et al.[9] suggest modifications in systemic treatment, as the development of nasolacrimal duct obstruction in patients in whom sarcoidosis is thought to be well controlled, may indicate that the overall disease activity is greater than suspected. A large rhinostomy ostium and placement of stent intraoperatively are suggested to increase the success rate.[9] Administration of corticosteroid eye drops and nasal spray in the presence of intranasal granulation tissue has also been suggested to improve the outcome.[3],[10] Systemic corticosteroids and/or immunosuppressives are used to suppress the lacrimal drainage system and nasal mucosal inflammation and prevent obstruction of the ostium by granulation tissue. Intralesional triamcinolone acetonide injections are advised in cases of severe nasal mucosal involvement.[11]


  Conclusion Top


To conclude, a tissue biopsy is warranted even in routine DCR procedures, where the lacrimal sac appears abnormal intraoperatively. Additional investigations shall be done depending on the histopathology findings. Though sarcoidosis is benign in most cases, it can be life-threatening in severe cases; hence, establishing the diagnosis and timely intervention is important.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Prabhakaran VC, Saeed P, Esmaeli B, Sullivan TJ, McNab A, Davis G, et al. Orbital and adnexal sarcoidosis. Arch Ophthalmol 2007;125:1657-62.  Back to cited text no. 1
    
2.
Nassif SJ, Ruiz D, Callahan A, Rebeiz EE. Nasolacrimal duct obstruction: An unusual presentation of sarcoidosis. Ear Nose Throat J 2020;31:145561320950494.  Back to cited text no. 2
    
3.
Avisar I, McNab AA, Dolman PJ, Patel B, Desousa JL, Selva D, et al. Endonasal dacryocystorhinostomy for nasolacrimal duct obstruction in patients with sarcoidosis. Orbit 2013;32:225-30.  Back to cited text no. 3
    
4.
Blouin MJ, Black DO, Fradet G. Recurrent dacryostenosis as initial presentation of sarcoidosis. Case Rep Otolaryngol 2012;2012:870527.  Back to cited text no. 4
    
5.
Ramlee N, Ramli N, Liza-Sharmini AT. Extrapulmonary sarcoidosis: Unusual cause of epiphora. Singapore Med J 2007;48:e168-70.  Back to cited text no. 5
    
6.
Merkonidis C, Brewis C, Yung M, Nussbaumer M. Is routine biopsy of the lacrimal sac wall indicated at dacryocystorhinostomy? A prospective study and literature review. Br J Ophthalmol 2005;89:1589-91.  Back to cited text no. 6
    
7.
Garcia GH, Harris GJ. Sarcoid inflammation and obstruction of the nasolacrimal system. Arch Ophthalmol 2000;118:719-20.  Back to cited text no. 7
    
8.
Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999;160:736-55.  Back to cited text no. 8
    
9.
Chapman KL, Bartley GB, Garrity JA, Gonnering RS. Lacrimal bypass surgery in patients with sarcoidosis. Am J Ophthalmol 1999;127:443-6.  Back to cited text no. 9
    
10.
Wijsenbeek MS, Culver DA. Treatment of sarcoidosis. Clin Chest Med 2015;36:751-67.  Back to cited text no. 10
    
11.
Krespi YP, Kuriloff DB, Aner M. Sarcoidosis of the sinonasal tract: A new staging system. Otolaryngol Head Neck Surg 1995;112:221-7.  Back to cited text no. 11
    


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