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Year : 2002  |  Volume : 12  |  Issue : 1  |  Page : 95-97
Solitary scleroma of larynx - unusual computed tomography findings : A case report


Department of Radiodiagnosis and Imaging, PGIMER; Chandigarh-160012, India

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   Abstract 

Scleroma of the larynx without involvement of the upper respiratory tract is a rare clinical entity. We present a case of isolated laryngeal scleroma with unusual computed tomography findings of a cystic lesion involving the left pyriform sinus.

Keywords: Scleroma, Larynx, Computed tomography

How to cite this article:
Mani N, Bapuraj J R, Nehru V I, Badotra B D, Suri S. Solitary scleroma of larynx - unusual computed tomography findings : A case report. Indian J Radiol Imaging 2002;12:95-7

How to cite this URL:
Mani N, Bapuraj J R, Nehru V I, Badotra B D, Suri S. Solitary scleroma of larynx - unusual computed tomography findings : A case report. Indian J Radiol Imaging [serial online] 2002 [cited 2019 Jun 24];12:95-7. Available from: http://www.ijri.org/text.asp?2002/12/1/95/28428

   Introduction Top


Scleroma is a chronic nonspecific granulomatous disease of the upper respiratory tract caused by Klebsiella Rhinoscleromatis. The disease was first described by Von Hebra in 1870[1]. The disease is endemic in North and Central Africa, Central and South America, Central and Southern Europe, Egypt, Indonesia and India. The patients usually belong to rural areas, with low socioeconomic status and poor hygiene.

The natural history of the disease has four stages (I) the catarrhal/oxidative stage, (ii) the atrophic stage (iii) granulomatous or proliferative stage and (iv) sclerotic or fibrotic stage[2],[3]. Involvement of the larynx, trachea and bronchi are uncommon and usually secondary to extension of the disease process from the upper respiratory tract. Solitary laryngeal involvement is extremely rare with only a few isolated case reports[2],[4]. CT scan findings of laryngeal scleroma have been described in only a few studies [5,6]. This case is being presented because of the rarity of the condition and the unusual CT findings encountered. A review of scleroma of the upper respiratory tract has also been included.


   Case Report Top


A 27 years old farmer came to the otolaryngology clinic with a 2 year complaint of hoarseness of voice. For the past 3 months he had difficulty in breathing which had worsened over the last week. Significant past history included epistaxis and nasal obstruction 2 years prior, when he was detected to have deviated nasal septum

and a septoplasty was done. Examination revealed a moderately built man with respiratory distress and inspiratory stridor. Indirect laryngoscopic examination revealed an upper pharyngeal growth involving both the arytenoids. The aryepiglottic folds and the larynx were not seen. Tracheoscopy was performed under local anesthesia. A contrast enhanced scan was done which revealed a hypodense cystic lesion in the left pyriform sinus. This lesion had a thin smooth wall without any significant enhancement [Figure - 1]. Direct laryngoscopy revealed an upper pharyngeal growth involving the right aryepiglottic fold, both the false vocal cords, arytenoids and extending from the posterior to the anterior commissure; the right pyriform sinus and the postcricoid region were free. Retrograde fibreoptic endoscopy revealed a growth involving the right true vocal cord and the posterior wall converging up to the stoma.

Histologic examination revealed numerous proliferating small blood vessels, plasma cells and a predominantly lymphocytic infiltrate with highly vacuolated foamy macrophages (Mickulicz's cells) [Figure - 2]. In addition degenerated plasma cells (Russel bodies) were seen in the form of large round PAS-Positive masses. The patient was treated with 160 mg oral trimethoprim, 800 mg sulfamethoxazole twice daily for 98 weeks on an outpatient basis. Repeat endoscopic studies revealed no evidence of the disease.


   Discussion Top


Laryngeal scleroma starts below the vocal cords where the squamous epithelium of the cord merges with the columnar ciliated epithelium in the subglottic area[6]. The larynx is reported to be involved in 12-40% [7,8] and the distal trachea and bronchi are rarely involved[9],[10].

Most of the patients (85%) having involvement of the upper respiratory tract belong to the second and third decades of life. Females are more commonly affected than males with a female: male ratio of 4: 1 in case of laryngeal involvement[6]. Laryngeal involvement as the sole manifestation of the disease is considered rare. In a series of 37 patients having scleroma, Abou Seif et al[6] reported 5 patients of laryngotracheal scleroma. All these patients had coexistent nasal involvement. Alfa Monge et al [5] reported a case with isolated laryngeal involvement in which the patient presented with acute exacerbation of respiratory distress and was found to have an upper tracheal mass on a lateral neck X-ray. A computed tomography scan was obtained before the mass was resected and showed subglottic stenosis. CT findings of laryngotracheal scleroma are nonspecific consisting of irregular thickening of the epiglottis and the aryepiglottic folds, irregular concentric narrowing of the subglottic region with web formation and mucosal irregularities in the trachea[6]. Our case demonstrated a hypodense well defined lesion in the pyriform fossa possibly an organized cyst. Based on the CT scan findings, differential diagnosis of such cystic lesions should include infected laryngocele, adenoid cystic carcinoma and cystic chondrosarcoma[11].

CT scanning can be used to assess accurately the extent of the disease. In our case the cystic lesion could be picked up only on the CT scan since the overlying mucosa appeared normal on laryngoscopy. Scleroma involving the larynx can be confused with a neoplastic lesion as was in our case. The other differential diagnosis that can be considered are granulomatous infections such as Wegener's granulomatosis and sarcoidosis[8]. The diagnosis can be established by culture of nasal swabs, serology and by histology.

The management included ensuring an adequate airway and initial antibiotic therapy in the granulomatous stage. In the cicatricial stage the stenosis or obstruction can be treated with periodic endoscopic dilatation[12] or managed with laser, cryosurgery or electrocauterization [13]. Surgical resection and reconstruction of the larynx has also been reported[14].

The diagnosis of scleroma is usually not difficult in endemic areas when the upper respiratory tract including the nasal passage is involved. However, it may pose a challenge in nonendemic areas and when laryngeal involvement occurs without nasal, paranasal or nasopharyngeal involvement.

 
   References Top

1.Von Hebra F. Ueber Ein Eigen Thuemliches Neugebiule An Der Nase. Wein Med Wochnschr 1870; 20: 1-4.  Back to cited text no. 1    
2.Badrawy A, Fouad H, Fathi A. Scleroma affecting the middle ear cavity with report of three cases. Ann Otol Rhinol Laryngol 174; 83; 107-110.  Back to cited text no. 2    
3.Taha A, Fatthi A, Abdel Kadir M. Soliman T. Surgical management of cicatricial postscleromatous subglottic stenosis. J Laryngol Otol 175; 95: 827-833.  Back to cited text no. 3    
4.Reddy Gr, Ahmed SM, Bose KN, Prasad AS, Hayath MS, Reddy ES. Rhinoscleroma of trachea. Ind J Otolaryngol Head Neck Surg 1995; 47: 235-237.  Back to cited text no. 4    
5.Alfaro Monge JM, Fernandez EJ, Soda Merhy A. Scleroma of the lower respiratory tract: Case report and review of literature. J Laryngol Otol 1994; 108: 161-163.  Back to cited text no. 5    
6.Abou Seif SG, Baky FA, El-Ebrashy F, Gaafar HA. Scleroma of upper respiratory passage: a CT study. J Laryngol Otol 1991; 105: 198-202.  Back to cited text no. 6    
7.Goldberg SN, Canails RD. Rhinoscleroma as a cause of airway obstruction. Ear Jose Throat J 1980; 59: 145-149.  Back to cited text no. 7    
8.Soni NK. Scleroma of the larynx. J Laryngol Otol 1997; 111: 438-440.  Back to cited text no. 8  [PUBMED]  
9.Mill RH, Shulman JB, Canalis RF et al. Klebsiella rhinoscleromatis: A clinical and pathological enigma. Otolaryngol Head Neck Surg 1979; 87: 212-221.  Back to cited text no. 9    
10.Hollinger PH, Gelman HK, Wolfe CK. Rhinoscleroma of the lower respiratory tract. Larymgoscope 1977: 87: 1-9.  Back to cited text no. 10    
11.Castelijns JA, Vanden Brekal MWM, Hermans R. Imaging of the larynx. Semin Roentgenol. 2000; 35: 31-41.  Back to cited text no. 11    
12.Acuna RT. Endoscopy of the air passage with special reference to scleroma. Ann Otol Rhinol Laryngol 1973; 82: 765-769.  Back to cited text no. 12  [PUBMED]  
13.Maher Al, El-Kashlan HK, Soliman Y, Galal R. Rhinoscleroma: management by carbondioxide surgical laser. Laryngoscope 1990; 100: 783-788.  Back to cited text no. 13    
14.Rifai M. Laryngotracheal resection for postscleromatous laryngeal stenosis. J Laryngol Otol 1989; 935-938.  Back to cited text no. 14    

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Correspondence Address:
NBS Mani
Department of Radiodiagnosis and Imaging, PGIMER; Chandigarh-160012
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2]

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