Dacryocystitis in a patient with Samter's triad.

Samter's triad, also known as aspirin-exacerbated respiratory disease, is characterized by nasal polyposis, bronchial asthma, and aspirin intolerance. Here, we present a case of a 36-year-old woman with a history of Samter's triad and recurrent dacryocystitis. After combined dacryocystorhinostomy and endoscopic sinus surgery, pathological specimens of the lacrimal sac showed respiratory fibrosis with chronic inflammation and eosinophilic infiltration. Our case demonstrates that Samter's triad is a potential etiology for inflammatory nasolacrimal duct obstruction. Copyright:

Introduction

Samter's triad, also known as aspirin-exacerbated respiratory disease (AERD), is a condition involving nasal polyposis, bronchial asthma, and aspirin intolerance. It is characterized by chronic sinonasal inflammation and increased tissue eosinophils.[1] Here, we present a patient who presented with recurrent dacryocystitis secondary to eosinophilic inflammation of the nasolacrimal system. The collection and evaluation of protected patient health information were compliant with the Health Insurance Portability and Accountability Act.

Case Report

A 36-year-old woman presented to the emergency department with a one week of worsening right-sided epiphora, periorbital erythema, and lower eyelid swelling. Her medical history was notable for Samter's triad, bilateral maxillary antrostomy and uncinectomy, and recurrent dacryocystitis. She had taken six days of amoxicillin–clavulanic acid for dacryocystitis as an outpatient without improvement. She denied any visual symptoms. Physical examination was notable for right periorbital edema and erythema with tenderness over the lacrimal sac [Figure 1]. Computed tomography scan of her sinuses demonstrated a distended right lacrimal sac measuring 2.2 cm × 1.2 cm with extensive adjacent inflammatory changes consistent with dacryocystitis secondary to nasolacrimal duct obstruction (NLDO) as well as pan-sinus disease [Figure 2a]. Laboratories were notable for marked eosinophilia (1290 cells/uL). She was hospitalized for intravenous vancomycin/piperacillin–tazobactam and surgical drainage of the lacrimal sac abscess. Cultures of the expressed pus demonstrated no growth. After her symptoms resolved, she was discharged on oral ciprofloxacin and outpatient follow-up with ophthalmology and otolaryngology.

Figure 1

External photograph of the patient upon presentation, demonstrating periorbital erythema and edema

Figure 2

(a) Computed tomography scan of the orbits demonstrating a distended lacrimal sac with adjacent inflammatory changes. (b) Coronal computed tomography scan demonstrating the absence of obstructive polyps at the level of Hasner's valve

Three months later, the patient underwent combined nasal endoscopy with bilateral maxillary antrostomy, total sphenoidotomy, frontal sinusotomy, nasal polypectomy, and right dacryocystorhinostomy with the placement of Crawford stents. Intraoperatively, the lacrimal sac, canaliculi, and adjacent soft tissue were noted to be scarred and friable. Surgical pathology of the lacrimal sac demonstrated respiratory fibrosis with chronic inflammation and eosinophilic infiltration of greater than 100 eosinophils per high-powered field [Figure 3]. Given her history of Samter's triad, she was referred to immunology. Extensive workup including antineutrophil cytoplasmic antibody, myeloperoxidase antibody, proteinase-3 antibody, ferritin, thyroid-stimulating hormone, complement testing, immunoglobulin E (IgE) antibody, tryptase, histamine release assay, and Toxocara antibody was negative. After premedication with prednisone, inhaled fluticasone propionate/salmeterol, and montelukast sodium, she underwent aspirin desensitization therapy. After 1 year of follow-up, the patient reports significant improvement in respiratory symptoms with no further episodes of dacryocystitis.

Figure 3

Microscopic images demonstrating (a) Respiratory mucosa with areas of acute and chronic inflammation and fibrosis, ×4 (b) The inflammation is submucosal, and scattered, sparing the epithelium, ×10 (c and d) At higher power, fibrosis and abundant eosinophils can be appreciated along with lymphocytes, ×40

Discussion

NLDO is categorized as primary NLDO or secondary NLDO, which can be caused by infection, inflammation, neoplasm, or mechanical obstruction.[2] The potential inflammatory etiologies of NLDO are granulomatosis with polyangiitis, sarcoidosis, pyogenic granuloma, radiation or chemotherapy induced.[3] Here, we report a case of secondary NLDO associated with Samter's triad. Although other disorders such as eosinophilic angiocentric fibrosis, allergic fungal sinusitis, and immunoglobulin G 4 disease have been reported to cause eosinophilic infiltrates in the nasolacrimal system, the presentation reported here was not consistent with these disease processes.[456]

Samter's triad, also known as AERD or Widal's triad, is a well-known disease entity defined by nasal polyposis, asthma, and aspirin sensitivity.[1] First described by Widal et al. in 1922, it most commonly presents in patients 29–34 years of age, starting with upper respiratory symptoms and progressing to involve the lower respiratory tract 2 to 5 years later.[78] Although the exact pathophysiology of the disease is unclear, it is thought to be a non IgE hypersensitivity reaction related to abnormal arachidonic acid metabolism in the cyclooxygenase (COX) and 5-lipoxygenase (5-LO) pathways [Figure 4].[189]

Figure 4

An overview of the mechanism that results in the symptoms of Samter's triad. A decrease in the activity of the cyclooxygenase pathway results in increased arachidonic acid metabolism by the 5-lipoxygenase system. The resultant increase cysteinyl leukotrienes and interleukin -4 lead to the characteristic manifestations of Samter's triad. COX = cyclooxygenase; 5-lipoxygenase; Cys-LT = cysteinyl leukotrienes; IL-4 = interleukin 4

In patients with Samter's triad, anti-inflammatory prostaglandins, particularly prostaglandin E2, produced by the COX system are decreased, while cysteinyl leukotrienes (Cys-LT) produced by the 5-LO pathway are increased.[8] Shunting of arachidonic acid metabolism toward the production of leukotrienes is exacerbated by COX-1 inhibition, which further increases leukotriene production.[9] Increased Cys-LT and associated increases in interleukin-4 expression cause vascular permeability, bronchoconstriction, and increased mucous secretion, leading to the characteristic manifestations of Samter's triad.[9] Increased Cys-LT also results in eosinophilic chemotaxis, resulting in the increase in tissue eosinophils that is histopathologically characteristic of the disease process.[18] Although not previously reported, we believe the pathophysiologic process effecting respiratory mucosa in Samter's triad may also affect the mucosa of the nasolacrimal system given their relative proximity and shared histologic features.[10] In our patient, chronic eosinophilic inflammation may have resulted in fibrosis, causing an acquired NLDO with resulting recurrent dacryocystitis.

There is a previously reported case of external obstruction of the nasolacrimal system due to nasal polyposis in a patient with Samter's triad.[11] Our patient had significant nasal polyposis; however, CT imaging did not demonstrate evidence of external obstruction [Figure 2b]. Although the possibility of polyps or previous surgical scarring causing external obstruction cannot be discounted, in our case, there was also evidence of eosinophilic inflammation of the lacrimal sac. This raises the possibility that the lacrimal sac itself was primarily involved in the disease process. To the authors’ knowledge, this is the first reported case of direct involvement of the nasolacrimal system in a patient with Samter's triad.

The treatment for Samter's triad is aspirin desensitization. Although aspirin desensitization therapy has not been studied in patients with NLDO, it has been shown to be effective at reducing sinonasal symptoms and improving quality of life through a poorly understood mechanism.[8] With surgical intervention alone, recurrence of nasal congestion is common, and 40% of patients may have recurrence of nasal polyposis without symptoms.[8] Our patient has had no further episodes of dacryocystitis and has improvement in her respiratory symptoms. The continuation of aspirin desensitization therapy may prevent the further progression of her disease.

Our case demonstrates that Samter's triad is a potential etiology for inflammatory secondary acquired NLDO and should be considered in young patients with recurrent dacryocystitis and eosinophilia. Further studies are needed to better characterize how Samter's triad may impact the nasolacrimal system.

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.

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Conflicts of interest

There are no conflicts of interest.