Nasal septal perforation: A rare extraintestinal manifestation of Crohn's disease.

Abstract

Nasal manifestations of Crohn's disease are quite rare. They are typified by chronic mucosal inflammation, obstruction, bleeding, and occasionally septal perforation--signs and symptoms that are common to many disease states of the nose. Nasal findings, much like oral lesions, can precede the more typical gastroenterologic manifestations of Crohn's disease. Otolaryngologists should be aware of such an association and consider the diagnosis of Crohn's disease in atypical cases of nasal disease. We report the case of a 12-year-old boy who had severe Crohn's disease and a nasal septal perforation, and we discuss this complication in the context of its otolaryngologic manifestations.

Introduction

Crohn's disease is a chronic inflammatory bowel disease characterized by discontinuous involvement of the entire alimentary tract, from the mouth to the anus. Crohn's disease is characterized by variations in its clinical presentation, the affected tissues, its severity, and the therapeutic response. It also displays an anatomic and histologic diversity that ranges from classic noncaseating granulomatous inflammation to variable acute and chronic inflammation.

Extraintestinal manifestations are protean, and they typically involve the joints, perianal tissues, and uveal tract. [1] Oral manifestations, including aphthous stomatitis, are relatively common and have been well described. [24] Nasal disease and septal involvement are, on the other hand, quite rare. [5,6] In this article, we describe the case of a 12-year-old boy who had severe Crohn's disease and a nasal septal perforation.

Case report

A 12-year-old white boy had a several-year history of Crohn's disease. He initially had recurrent painful oral aphthae and subsequently developed severe granulomatous colitis with extraintestinal involvement of the perianal tissues and the esophagus. Treatment included systemic steroids, oral and rectal mesalamine, metronidazole, and 6-mercaptopurine. He next complained of nasal crusting, mild congestion, and occasional self-limited epistaxis over a period of 6 months. The patient ultimately noticed a hole in his nasal septum.

The patient denied a history of allergy, septal surgery, nasal trauma, or cauterizations. He had had no exposure to cocaine or other local irritants. There was no history of local infectious or other granulomatous or inflammatory diseases. He had not been treated with topical nasal steroids.

The physical examination revealed the presence of several small oral and pharyngeal ulcers and a 1.5 x 1.0-cm anterior nasal septal perforation with adjacent crusting and mildly irregular mucosal edema (figure 1). In light of his complaints of refractory intestinal disturbances, he underwent upper and lower endoscopy, during which time a nasal biopsy was also performed. Biopsy of the tissue surrounding the septal perforation revealed surface ulceration with underlying chronic inflammatory changes without granulomata (figure 2). Acid-fast and fungal stains as well as cultures were negative. Concurrent biopsy of the colon revealed characteristic transmural granulomatous changes consistent with Crohn's disease (figure 3).

Discussion

Crohn's disease is a form of inflammatory bowel disease that features intestinal symptoms such as nonbloody diarrhea, abdominal pain, fatigue, fever, and weight loss. Extraintestinal manifestations are common in Crohn's disease, and they can affect the head and neck. The most common head and neck manifestations are oral lesions. They are more prevalent in patients with other extra-intestinal manifestations, and they sometimes precede gastrointestinal symptoms. [2,7,8] The most frequently noted oral lesions are aphthae, which are found in 10 to 20% of cases. [4,8]

Oral manifestations of Crohn's disease other than aphthae occur in as many as 9% of patients. [2] With a nearly 2-to-1 male predominance, nonaphthous oral Crohn's lesions typically involve the lips, buccal mucosa, gingiva, and retromolar trigone. [2,3] Less often they involve the tongue, palate, and pharyngeal mucosa. [7] Findings can include diffuse and painful swelling of the lips, fissured lips, cobblestoned buccal mucosa, gingival redness and hypertrophy, linear ulcerations, mucosal tags, and angular cheilitis. [8] Tonsillar hyperplasia with noncaseating granulomas secondary to Crohn's has also been reported. [9]

Unlike oral lesions, respiratory tract manifestations are quite uncommon. Laryngeal involvement, which has been reported only rarely, entails the presence of cricoarytenoid inflammation and supraglottic edema, which can lead to airway obstruction, stridor, and throat pain. [2,10,11] Tracheobronchial inflammation is very rarely associated with Crohn's disease. [12]

Nasal involvement in patients with Crohn's disease is similarly unusual. Nasal manifestations were first reported by Kinnear in 1985 in a patient with atrophic rhinitis, chronic mucosal inflammation, and granuloma formation. [5] Mucosal edema, chronic inflammation, and even polypoid changes involving the septum or turbinates might be found on physical examination of Crohn's patients. [6] Symptoms of nasal Crohn's typically include obstruction, rhinorrhea, and epistaxis.

Similar to those in intestinal Crohn's disease, histologic findings in nasal Crohn's are quite variable. Granulomas are not always present. Previous reports of nasal biopsies in Crohn's patients show mainly chronic inflammatory changes with rare giant cells or granulomas. [6] The nasal histologic findings are also similar to oral Crohn's lesions, where granulomas are detected in only 10 to 68% of specimens. [3,13] Biopsies of nasal and oral lesions should be deep enough to include the submucosa because the yield of granulomas can be higher. [8]

The causes of nasal septal perforations include trauma, surgery, cocaine abuse, cauterization, infection, inflammation, and neoplasm. Among the inflammatory causes, Wegener's granulomatosis, systemic lupus erythematosis, sarcoidosis, rheumatoid arthritis, relapsing polychondritis, and Crohn's disease have been implicated. [14,15]

Recognition of Crohn's disease in this setting requires a thorough review of symptoms and a consideration of this diagnosis in atypical or unresponsive cases of nasal disease. Although these patients would typically have a history of Crohn's disease, oral and nasal symptoms can precede the development of gastrointestinal symptoms. Ghandour and Issa described a pediatric patient with unexplained lip swelling and nasal obstruction secondary to a marked septal and turbinate hypertrophy who was several years later diagnosed with Crohn's disease after developing gastrointestinal disease. [8] Plauth et al also recommend an evaluation for Crohn's disease for patients with orofacial granulomatosis or chronic relapsing oral lesions. [3]

Treatment options are limited. Systemic and topical nasal corticosteroids are recommended for nasal Crohn's. [5,6] Oral cavity lesions in Crohn's patients receive limited benefit from topical therapy alone. [3] Thus, it might be that topical steroids alone would be ineffective in nasal Crohn's given their histologic similarities. Moisturization and antibacterial ointments can decrease crusting and secondary infection, and their liberal use is encouraged. The rate of successful surgical closures of septal perforations secondary to a chronic inflammatory condition such as Crohn's disease is low. Consideration should be given to the use of a septal button. As in other diseases of the septum, progressive inflammation can lead to a progressive loss of cartilage and a subsequent saddle deformity. [8]

From the Division of Pediatric Otolaryngology (Dr. Kriskovich and Dr. Kelly) and the Division of Pediatric Gastroenterology (Dr. Jackson), Primary Children's Medical Center and the University of Utah School of Medicine, Salt Lake City.

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