Volume 4, Issue 2 , Pages 72-74, March 2009
Rhinoscleroma in a child
Article Outline
Summary
Rhinoscleroma is an endemic disease in Egypt, it affects mainly the nose but it can extend to the pharynx, larynx, trachea and bronchi. It is more common in females and it usually affects the middle age individuals. Unilateral nasal affection is rare while bilateral presentation is common. In this study, we present a 5-year-old girl with unilateral rhinoscleroma.
Keywords: Rhinoscleroma, Nasal granuloma, Chronic rhinitis, Pediatric rhinitis
1. Introduction
Rhinoscleroma is a chronic granulomatous condition of the nose and other structures of the upper respiratory tract. The infection is due to a capsulate gram-negative bacterium, Klebsiella rhinoscleromatis, which was first described by Von Frisch in 1882 [1].
This disease is endemic in Africa, Central and South America, South central and Eastern Europe, Middle East and China [2].
Rhinoscleroma is found predominantly in rural areas and is commoner where socio-economic conditions are poor. Acquisition of the disease is facilitated by crowding, poor hygiene and poor nutrition. Females are more frequently affected than males (ratio 13:1) and the disease tends to present in the second and third decades of life. There is also a suggestion that iron deficiency may predispose to disease acquisition [3].
We report a case of rhinoscleroma in a 5-year-old child.
2. Case report
A 5-year-old girl presented to the Otolaryngology Clinic of the Pediatric Hospital of Cairo University with large nasal swelling of 1 year duration. The swelling involved almost all the right side of the nose and appeared filling the right nasal cavity (Fig. 1), with circumferential attachment to the skin of the vestibule. It was reddish in color, firm to hard in consistency with irregular surface. Examination of the throat showed no lesions. CT (Fig. 2) showed soft tissue mass involving only the soft tissues of the right side of the nose (cartilaginous part). Biopsy was taken under local anesthesia and proved the diagnosis of rhinoscleroma (Fig. 3). Complete excision of the mass was carried out under general anesthesia through lateral alatomy incision (Fig. 4), it was attached hardly to the alar cartilage but dissection was done successfully. The child received medical treatment (Rifampicin) for 1 month postoperatively. During the follow-up period, the child developed – 1 year after the operation – severe fibrosis that collapsed the right ala with complete obstruction of the right side of the nose and swelling of the right lacrimal sac indicated extension (Fig. 5). Now, the child under medical treatment (Rifampicin), and plastic surgery will be considered after complete eradication of the inflammatory process.
Fig. 1.
A 5-year-old girl with right nasal mass; (A) elevating the right side of the nose; (B) completely filling the nasal cavity.
Fig. 2.
Soft tissue mass involving the right side of the nose with free paranasal sinuses and nasal cavity.
Fig. 3.
Characteristic histopathology of rhinoscleroma shows Mikulicz cells which are large vaculated foamy cells with small nuclei and Russel bodies which are bright red degenerated plasma cells with background of multiple plasma cells (hematoxylin-eosin stain, 400×).
Fig. 4.
(A) The marked line is the site of lateral alatomy incision; (B) elevation of the ala with appearance of the mass.
Fig. 5.
The child after 1 year follow-up with fibrosis of the right side of the nose and swelling of the right lacrimal sac.
3. Discussion
Rhinoscleroma is an endemic disease in Egypt; it is more common in females in the second and third decades of life. The disease usually presents itself bilaterally in the nose. It may affect the nasopharynx and larynx. Laryngeal affection may be life threatening as it may cause severe stridor. Children affection is rare, however, few cases has been reported in the English literature. Karchev and Kabakchiev [4] reported the disease in two children; they detected amyloid-like protein in the histopathologic specimen that may be due to an autoimmune reaction. Fernández-Vozmediano et al. [5] reported rhinoscleroma in three siblings living in a non-endemic area and they suggested neutropenia to be the predisposing factor.
In this study, we report the disease in a 5-year-old Egyptian child presented with unilateral nasal mass that elevated the soft tissues of the right side of the nose. Surgical excision in addition to medical treatment was used in the treatment of the disease. Unfortunately, the child developed severe fibrosis 1 year after the operation.
This disease has three stages; the first is the atrophic stage in which there is crusty foetid discharge and squamous metaplasia and the second is the graulomatous stage in which the patient present by granular masses obstructing the nasal cavity, histological examination of these masses usually show the characteristic Mikulicz cells and Russell bodies, while the last stage is the fibrotic one in which there is marked sclerosis of the soft tissues of the nose [6].
The first presentation of the patient to us was in the graulomatous stage in which the child presented with large unilateral nasal mass that made the diagnosis of sarcoma was expected especially in the absence of affection of other parts of respiratory system. Recurrence of the disease in the form of collapse of the ala of the nose indicated that fibrotic stage has been developed.
Finally, we conclude that rhinoscleroma should be considered in the differential diagnosis of nasal masses of pediatric population and surgical excision cannot eradicate the disease which may recur in a fibrotic stage.
References
- . Zur aetiologie des rhinoscleroms. Wien. Med. Wochenschr. 1882;32:969–972
- . Nasal rhinoscleroma in a nonendemic area: a case report. J. Med. Korean Sci. 2003;18:455–458
- . Iron deficiency in atrophic rhinitis and scleroma. Indian J. Med. Res. 1987;85:576–579
- . Amyloid-like protein in children with rhinoscleroma. Rhinology. 1989;27(1):27–36
- . Rhinoscleroma in three siblings. Pediatr. Dermatol. 2004;21(2):134–138
- . An interpretation of the structural changes responsible for the chronicity of rhinoscleroma. Laryngoscpe. 2001;111:1020–1026
PII: S1871-4048(08)00051-8
doi:10.1016/j.pedex.2008.06.005
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 4, Issue 2 , Pages 72-74, March 2009
