International Journal of Pediatric Otorhinolaryngology Extra
Volume 2, Issue 4 , Pages 228-230, December 2007

Choanal stenosis in Crohn's disease: A case report and review of literature

  • Domenico Leonardo Grasso

      Affiliations

    • Ear, Nose and Throat Department, IRCCS “Burlo–Garofolo” Children Hospital, Trieste, Italy
    • Corresponding Author InformationCorresponding author. Tel.: +39 0403785256; fax: +39 0403785537.
    • ,
  • Margherita Bussani

      Affiliations

    • Clinical Pathology Department, Ospedale Maggiore, Trieste, Italy
    • ,
  • Stefano Martelossi

      Affiliations

    • Pediatric Department, IRCCS “Burlo–Garofolo” Children Hospital, Trieste, Italy

Received 5 June 2007; accepted 30 June 2007.

Article Outline

Summary 

Crohn's disease is a granulomatous inflammatory bowel disease. Its pathologic findings include non-contiguous chronic inflammation and non-caseating granulomas sometimes with extra-intestinal localizations. Nasal manifestations of Crohn's disease are quite rare. They are characterized by chronic mucosal inflammation, obstruction, bleeding and occasionally septal perforation. The authors describe the case of a 4-year-old girl with Crohn's disease with recurrent epistaxis and narrowing of the right choana, secondary to fibrous tissue.

Keywords: Crohn's disease, Choanal stenosis, Epistaxis, Nasal involvement

 

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1. Introduction 

Crohn's disease is a chronic inflammatory disease of the gastrointestinal tract, associated with disorders of the cell mediated and humoral immunity and characterized anatomically by a giant-cell non-specific granulomatosis .The incidence of the disease is increasing and it is common in young women. Any part of the gastrointestinal tract may be affected, and 9% of cases have painful oral lesions in coincidence with periods of active intestinal disease as well as pharyngolaryngeal localizations have been described [1]. Nasal localizations are rare and they involve the adulthood. They are characterized by chronic mucosal inflammation, obstruction, bleeding and occasionally septal perforations [2]. In this article, we describe a case of nasal mucosa involvement in a 4-year-old patient affected by Crohn's disease and presenting with epistaxis.

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2. Case report 

Patient was affected by Crohn's disease from the age of 8 months. At the onset, weight loss, mucosanguineus diarrhoea, oral aphta, perianal abscesses with the involvement of the whole bowel were present. Patient followed steroid therapy for 4 years. Severe side effects were present as well as late development and bone demineralization. Ciclosporin, azatioprina, talidomid and anti-tumor necrosis factor (TNF) were used without success. In November 1999, a percutaneous gastrostomy was performed. In November 2000, the patient underwent to a total colectomy with terminal ileostomy. Since then, metotrexate in association with anti-TNF were used. In July 2001, ileorectetomy has been performed. In 2002, there was an onset of recurrent epistaxis. Laboratory investigations revealed a haemoglobin level of 12.6gm/dl, an erythrocyte sedimentation rate of 7mm/h, and a white blood cell count of 14,200mm−3. The anterior rhinoscopy exhibited a narrowing of the vestibule, easy mucosal bleeding and narrowing of the right choana, secondary to the presence of fibrous tissue (Fig. 1). The left choana was normal. Biopsy specimens of the nasale septal mucosa showed erosion of the epithelium with lymphoplasmocytic infiltrate and microgranulomas. (Fig. 2, Fig. 3). Steroid therapy with prednison 25mg once a day for 3 weeks obtained resolution from epistaxis for the following year.

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3. Discussion 

Crohn's disease is a chronic inflammatory bowel disease that may affect any part of the gastrointestinal tract. Thirty-six percent of patients with Crohn's disease had extra-intestinal manifestations in a series of 700 patients reviewed by Greenstein et al. [3]. Oral manifestations were found to be present in 4% of this cohort. Similar data were found in 20 (6%) out of 332 patients with Crohn's disease by Croft and Wilkinson [4]. Of 100 patients with Crohn's disease, Basu et al. found 9% with oral manifestations [5]. Laryngeal localizations have also been described [6], [7]. Nasal manifestations of Crohn's disease are quite rare. Macroscopically, a giant-cell granulomatosis without necrosis is present. True granulomas are rarely seen [8]. Kinnear reported in 1985 a 36-year-old female with chronic atrophic rhinitis with ulcerations and granulomatosis [1]. Ernst et al., in 1993, presented a patient with chronic pansinusitis and nasal polyposis obstructing the nasal cavities. The histological exam showed deep ulcerations with inflammation without granulomas [9]. Nasal ulcerations associated with oropharyngeal, esophageal and laryngeal involvements are described by Kelly et al. [10]. Pochon reported a case with multifocal lesions characterized by oedema swelling and nasal polyposis at middle meatus level [8]. Kriskovich et al. reported the case of a 12-year-old boy who had a severe Crohn's disease and a nasal septal perforation [2].

In the case presented here, diffuse atrophy of the nasal mucosa was present. Easy multiple bleeding sites were observed in the whole nasal cavities. The biopsy specimen showed erosion of the mucosal epithelium with lymphoplasmocytic infiltrate and microgranulomas. This is the youngest patient affected by nasal localization of Crohn's disease, in despite of aggressive immunosuppressive treatment, no improvement of nasal mucosa were observed, except for the resolution of epistaxis. Narrowing of the right choana seems to be the results of recurrent scarring tissue that reduced the lumen in the ongoing of disease. Presence of nasal localization of Crohn's disease in a so young patient could be interpreted as a negative prognostic factor of severity of disease, even though we need a wider number of subjects affected for a better understanding.

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References 

  1. Kinnear WJ. Crohn's disease affecting the nasal mucosa. J. Otolaryngol. 1985;14:399–400
  2. Kriskovich MD, Kelly SM, Jackson WD. Nasal septal perforation: a rare extraintestinal manifestation of Crohn's disease.. Ear Nose Throat J. 2000;79:530–533
  3. Greenstein AJ, Janowitz HD, Sachar DB. The extra-intestinal complications of Crohn's disease and ulcerative colitis: a study of 700 patients. Medicine. 1976;55:401–412
  4. Croft CB, Wilkinson AR. Ulceration of the mouth, pharynx and larynx in Crohn's disease of the intestine. Br. J. Surg. 1972;59:249–253
  5. Basu MK, Asquith P, Thompson RA, Cooke WT. Oral manifestation of Crohn's disease. Gut. 1975;16:249–254
  6. Wilder WM, Slagle GW, Hand AM, Watkins WJ. Crohn's disease of the epiglottis, aryepiglottis folds, anus and rectum. J. Clin. Gastroenterol. 1980;2:87–91
  7. Ulnick KM, Perkins J. Extraintestinal Crohn's disease: case report and review of the literature. Ear Nose Throat J. 2001;80:97–100
  8. Pochon N, Dulguerov P, Widgren S. Nasal manifestation of Crohn's disease. Otolaryngol. Head Neck Surg. 1995;113:813–815
  9. Ernst A, Preyer S, Plauth M, Jenss H. Polypöse Pansinusitis al seine ungewönliche, extraintestinale manifestation des morbus Crohn. HNO. 1993;41:33–36
  10. Kelly JH, Montgomery WW, Goodman ML, Mulvaney TJ. Upper airway obstruction associated with regional enteritis. Ann. Otol. 1979;88:95–99

PII: S1871-4048(07)00056-1

doi:10.1016/j.pedex.2007.06.008

International Journal of Pediatric Otorhinolaryngology Extra
Volume 2, Issue 4 , Pages 228-230, December 2007

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