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Table of Contents
ORIGINAL ARTICLE
Year : 1999  |  Volume : 1  |  Issue : 2  |  Page : 84-87

Primary atrophic rhinitis - Is it hereditary or infectious?


1 Consultant ENT Surgeon, Qateef Central Hospital, Qateef, Saudi Arabia
2 Associate Professor in Otolaryngology, King Faisal university, Al-Khobar, Saudi Arabia

Date of Web Publication16-Jun-2020

Correspondence Address:
FRCS Ezzat E Davlatly
Associate Professor in Otolaryngology King Fahad Hospital of the University P.O. Box 40008 Al-Khobar 31952
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-8491.286867

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  Abstract 


Objective: To study the clinical profile of primary atrophic rhinitis occurring in families as seen today in Saudi Arabia.
Setting. Department of Otolaryngology, Dammam Central Hospital and King Fahd Hospital of the University, AI- Khobar, Saudi Arabia.
Patients and Methods: Study of two families diagnosed with primary atrophic rhinitis, including clinical examination of the siblings of both families.
Results and Conclusion: The probable mode of transmission is different in the two families. Primary atrophic rhinitis is a multifactorial condition that is seen increasingly infrequently in Saudi Arabia with improved living standards and healthier nutrition.

Keywords: Primary, familial, atrophic rhinitis, ozaena


How to cite this article:
Bohllga LA, Davlatly EE. Primary atrophic rhinitis - Is it hereditary or infectious?. Saudi J Otorhinolaryngol Head Neck Surg 1999;1:84-7

How to cite this URL:
Bohllga LA, Davlatly EE. Primary atrophic rhinitis - Is it hereditary or infectious?. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 1999 [cited 2023 Jan 30];1:84-7. Available from: https://www.sjohns.org/text.asp?1999/1/2/84/286867




  Introduction: Top


Atrophic rhinitis or “ozaena” was known to the ancient Greek, Hindu, and Egyptian civilisations. It is a chronic disease characterised by abnormal patency of the nasal passages as a result of progressive atrophy of the nasal mucosa and resorption of the underlying turbinate bone. The resultant viscid secretions dry and form crusts that emit a characteristic foul odour - hence the name ozaena, Greek for stench.[1] It is either primary or secondary. Primary atrophic rhinitis is of unknown aetiology, though a strong family history and poor socio-economic status is common. Secondary atrophic rhinitis may be secondary to chronic granulomatous infections of the nose. More often it is secondary to trauma including surgical insults, or drug induced, including injudicious use of nose drops. Both types are becoming increasingly uncommon in affluent societies.[2] In 1897 Casselberry {3} noted that no one theory of causation would account for all the cases of atrophic rhinitis observed. A 100 years later the aetiology of primary atrophic rhinitis remains a matter of conjecture. Various factors postulated include infective, hormonal, dietary, vascular, premature senility, pituitary dystrophies, hygienic, autonomic imbalance, collagen disorder, developmental , avitaminosis, iron deficiency, and economic. Sibert and Barton, 1980 [4] reported a family in whom the father and 8 of 15 children were affected. Young’s,1987 [5] report of 5 children and 8 adults who underwent surgeiy for atrophic rhinitis included a mother and her child. Lu et al,1995[6] in a prospective study concluded that atrophic rhinitis is transmitted in a mucigenic and multifactorial mode. They found hereditary as well as environmental and socio-economic factors contributed to the causation of the condition. All 8 children reported by Gupta,1977[7] were from lower income groups, and the mother of one child was affected. Singh, 1992[8] states that primary atrophic rhinitis rarely has a definite identifiable cause. He reports on 75 patients, all of poor socioeconomic status, with 20% familial incidence of the condition. Goodman and De Souza,1973[9] suggest that the organisms reported in atrophic rhinitis are probably secondary invaders that have found a favourable medium. Han-Sen,1982(10} in an analysis of 100 cases of atrophic rhinitis supports the infective theory and avitaminosis. Baere! al, 1988 {11} report on the association of primary atrophic rhinitis with anhidrotic ectodermal dysplasia in a 4 months old infant - a sex-linked recessive condition that manifests fully in affected males and partially in females. Burns, 1993 {12] concludes that ozaena is seen today as a manifestation of atrophic rhinitis, most commonly occurring as a hereditary malady, but associated with the injudicious use of nasal sprays and drops. We report primary atrophic rhinitis in 2 families - a father and son, and a mother and daughter. No predisposing factors in all 4 patients were found. Clinical examination of siblings in both families was normal.

Patients reported:

The First Family:

A 41-year old father of 7 was referred with a diagnosis of atrophic rhinitis for consideration for surgery in 1989. Chest x-ray and serology were negative. Nasal swabs repeatedly grew commensals only. Sinus X-rays showed roomy nasal fossa with mucosal thickening of the maxillary sinuses. There was no evidence of any nutritional deficiency. Full blood count, serum proteins and blood chemistry were normal. A long discussion with the patient about his condition and the options available resulted in his choice of medical treatment. His condition responded well to alkaline nasal douching and dextrose/glycerine nose drops. The follow up intervals were gradually lengthened as his nasal crusting diminished. Antibiotics were prescribed when he had upper respiratory tract infections. In 1992 he brought his 8-year old son with him because he suspected the child was suffering from the same condition. Examination showed very wide nasal fossae and greenish crusts bilaterally. Swabs grew commensals only, and serology was negative. The child co-operated well with conservative treatment in spite of his strong dislike of the dextrose/glycerine nose drops. The father was invited to bring in all his other children. They were all examined and no evidence of atrophic rhinitis was found in any of them. The father and son now come every 6 months for follow up.

The Ssecond Family:

Baby R. was first seen aged 8 months with foul nasal discharge for one month. An emergency examination under general anaesthesia by a resident to exclude foreign body was negative. She was seen a month later with the same complaint. Repeat examination under anaesthesia was carried out by the first author. Both nasal fossae were found full of crusts. Removal of the crusts revealed very spacious nasal fossae and marked atrophy of the turbinates. Culture of the crusts grew Klebsiella ozaenae sensitive to amoxycillin. Biopsy revealed marked non-specific inflammatory changes. Chest x-ray and serology were negative. Full blood count and indices were normal. The baby was put on a 3-week course of amoxycillin. Normal saline followed by dextrose/glycerine nose drops were started. Initially the crusts were sucked out every 3 weeks. Crusting gradually diminished and she continued to be seen every 6 weeks. As the baby grew it was apparent that she had complete collapse of her nasal bridge [Figure 1]. Repeat serology was negative. C. T. scan of the nose and sinuses showed roomy nasal fossae, atrophic turbinates and opaque maxillary sinuses [Figure 2]. Two years after the baby’s first presentation the mother reported that she has been using her daughter’s nose drops with a lot of benefit. This came as a surprise, because the mother had been examined and found normal when her daughter’s diagnosis was established. Examination showed very roomy nasal fossae with extensive crusting. Culture from the mother was negative. The mother was invited to bring her 2 other children for examination. A 2-year-old boy and a 4 months old girl had no evidence of atrophic rhinitis. Both mother and daughter are responding well to conservative treatment. Monitoring the 2 younger children for early evidence of disease has been arranged.
Figure 1: Clouse-Up Photograph baby R. showing complete collapse of her nasal bridge

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Figure 2: CT scan of baby R. Showing nasal fossae with very thin atrophic infenrior turbinates and opaque maxillary sinuses

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  Discussion: Top


Primary atrophic rhinitis is seen increasingly infrequently in our practice. This is probably a result of the improved living standards and nutrition of the population of Saudi Arabia. An additional factor in observant Muslims who practice ablution several times a day before praying is the ready access to clean water supply all over the country. Atrophic rhinitis secondary to injudicious surgery forms the bulk of cases seen by us. In contrast to Kamasweran’s,1991 {13} experience in the desert region of Asir in Saudi Arabia with 42 patients treated surgically in one year, our surgical experience over 10 years is limited to 6 patients who opted for bilateral medialisation of the lateral nasal wall via the Caldwell-Luc approach, and 2 patients who chose Young’s closure of the nose. Three of our patients are non-Saudis. Like Kameswaran’s patients the 2 families reported come from desert regions. The first family comes from the desert oasis ofAl-Hassa in the Eastern Province of Saudi Arabia. The second family is from Najran in Asir Region bordering the great desert of Al-Ruba-Al- Khali (The Empty Quarter). The two families reported have a lot in common. Both are working class families, of low socio-economic standard. All four patients responded well to medical treatment. None of the siblings in the 2 families sulTered from the condition. Both families come from desert regions. The differences are however more striking. The mode of transmission in the first family appears to be hereditary. The father had the disease for many years before he suspccted the condition in his son, the fifth of 7 brothers and sisters. Neither the mother nor any of the remaining 6 siblings were affected. This finding practically rules out direct transmission through close contact. In the second family it appears that the baby has transmitted the disease to the mother, probably as a result of the close contact between mother and child. The mother was normal at the time of diagnosis of the baby. No organisms were cultured from the first family on repeated cultures. Klebsiella ozaena was cultured from the daughter in the second family, although the mother’s swab was negative. The first family showed no external nasal deformity. The daughter in the second family had complete flattening of the nasal bridge. Repeat serology for syphilis was negative. Only one child out of 8 reported by Gupta,1977[7] had a saddle shaped nose which was attributed to previous trauma. However, saddle deformity of the nose is not unknown. Baser et al, 1990 [14] reported on the management of saddle nose deformity in 15 adults aged 26 to 50 years. In their patients atrophic rhinitis started between the ages of 22 and 35. Our patient appears to be the first reported case of complete flattening of the nasal bridge in infancy associated with atrophic rhinitis. It is probably due to compromise of the blood supply to the growing nasal and septal bones as a result of the severe inflammatory reaction. The mucosal thickening in the maxillary sinuses [Figure 2] is similar to Pace- Balzan et al’s,l99l [15] report. In a study of 3 patients with atrophic rhinitis by CT they found “marked mucosal thickening in the paranasal sinuses, particularly in the maxillary sinus”. Casselberry{3} in 1897 reported that “the process seemed to involve the nasal bones and cartilages as well as the turbinates, so that there was a suggestion of the saddleback nose”. Goodman and De Souza,1973[9] describe the inflammatory changes observed in atrophic rhinitis as similar to simple chronic rhinitis, only much more severe in degree and extent. Taylor and Young, i 961 [16] have noted that symptoms of atrophic rhinitis may appear or worsen during pregnancy and menstruation. In the mother reported, the hormonal changes of 2 pregnancies in quick succession probably predisposed to her atrophic rhinitis. The father’s dramatic response to conservative treatment supports Shehata’s,l996{]} remark that a good percentage of patients show spontaneous regression after the age 40. Atrophic rhinitis has no cure; and all medical and surgical measures have short or prolonged palliative effect {I }.The variety of medical and surgical treatments claimcd to cure or ameliorate the symptoms and the social stigma of atrophic rhinitis is testament to our ignorance so far of the true aetiology of primary atrophic rhinitis. It appears that atrophic rhinitis is the clinical manifestation of a variety of factors, acting separately or in concert, that damage the ciliated respiratory epithelium of the nose. In the families reported perseverance of the patients with conservative treatment has resulted in marked improvement in their symptoms and in the disappearance of the foul smell.


  Conclusion: Top


Atrophic rhinitis is seen less frequently with the improved living standards and quality healthcare in Saudi Arabia. Two families with primary atrophic rhinitis in a father and son, and a mother and daughter are reported. The probable mode of transmission is different in each family. Primary atrophic rhinitis seems to be the clinical expression of a variety of factors acting singularly or in concert. In well motivated patients conservative treatment pays handsome dividends.

Acknowledgement:

We wish to express our gratitude to Mr Gordon Ntow of the medical photography department of King Fahd Hospital of the University in Al-Khobar for preparing the prints.



 
  References Top

1.
Shehata MA. Current Reviews. Atrophic Rhinitis. A J Otolaryngol 1996 ;I7(2):81-86.  Back to cited text no. 1
    
2.
Barton RPE, Sibert JR. Primary atrophic rhinitis: an inherited condition? J Laryngol Otol 1980;94:979-983.  Back to cited text no. 2
    
3.
Casselberry WE. Discussion on Atrophic Rhinitis. Journal of Laryngology, Rhinology and Otology 1897; Vol. XII - No. VIII. In 100 YEARS AGO. J Laryngol Otol ]997; 111:782.  Back to cited text no. 3
    
4.
Sibert JR, Barton RPE. Dominant inheritance’in a family with primary atrophic rhinitis. J Med Genet 1980;17:39-40.  Back to cited text no. 4
    
5.
Young A. Closure of the Nostrils in Atrophic Rhinitis. J Laryngol Otol 1967;8I:515-524.  Back to cited text no. 5
    
6.
Lu Z. Xie X, Liao L. A preliminary study on etiology of atrophic rhinitis in Zunyi (abstract) Chinese J Otorhinolaryngol 1995;30(1):41-43.  Back to cited text no. 6
    
7.
Gupta SC. Atrophic rhinitis in children. Indian J Pediat 1977;44:352-354.  Back to cited text no. 7
    
8.
Singh I, Atrophic rhinitis: a familial disease? Tropical Doctor, 1992; 22(2):84.  Back to cited text no. 8
    
9.
Goodman WS, De Souza FM. Atrophic Rhinitis. Otolaryngol Clin North Amer 1973;6(3):773-782.  Back to cited text no. 9
    
10.
Han-SenC. The Ozaena Problem. Acta Otolaryngol 1982;93:461-464  Back to cited text no. 10
    
11.
Baer ST, Coulson IH, Elliman D. Anhidrotic ectodermal dysplasia: and ENT presentation in infancy. J Laryngol Otology 1988;102:458-459.  Back to cited text no. 11
    
12.
Burns JL. Ozaena (Archive). J Otolaryngol 1993;22(2): 135.  Back to cited text no. 12
    
13.
Kameswaran aM. Fibre-optic endoscopy in atrophic rhinitis. J Laryngol Otology 1991:105:1014-1017.  Back to cited text no. 13
    
14.
Baser B, Grewal DS, Hiranandani NL. Management of saddle nose deformity in atrophic rhinitis. J Laryngology Otology 1990;104:404-407.  Back to cited text no. 14
    
15.
Pace-Balzan A, Shankar L, Hawke M. Computed tomographic findings in atrophic rhinitis.J Otolaryngol 1991 ;20(6):428432.  Back to cited text no. 15
    
16.
Taylor,M. and Young,A. Studies on atrophic rhinitis. J Laryngol Otol 1961 ;75: 574-590  Back to cited text no. 16
    


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