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Scleroma of the upper air-passages
44
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
Scleroma of the Upper Air-passages A CLINICO-RADIOLOGICAL
STUDY
OF 84 CASES
G. E. M A S S O U D , F.F.R., AND H. K. A W W A D , M.D.
From the Radiological Department, University of Alexandria, Egypt S C L E R O M A is a specific g r a n u l o m a affecting, mainly, the nose. T h e u p p e r air-passages, trachea, a n d b r o n c h i m a y also b e involved, e i t h e r w i t h or w i t h o u t a p p a r e n t c o n t i n u i t y w i t h t h e nasal granuloma. Histologically, the lesion has a characteristic appearance. T h e c o r i u m a n d s u b m u c o s a are infiltrated b y g r a n u l o m a t o u s tissue c o m p o s e d of: (a) Vacuolated f o a m y histiocytes (Mikulicz cells); (b) P l a s m a cells; (c) D e g e n e r a t e d hyalinized p l a s m a cells (Russel's bodies) ; (d) N e u t r o p h i l s ; (e) A v a r y i n g n u m b e r of capillaries; a n d ( f ) Fibrocytes, fibroblasts, a n d collagen. Yasenetskiy a n d Y a m o p l o s k a y (1956) s h o w e d t h a t t h e degree of sclerosis is n o t indicative of t h e age of the pathological process. I t can o c c u r i n very early lesions, whereas active cellular infiltrations m a y b e f o u n d in old granulomas. T h e causative a g e n t is suggested to b e a strain of the F r i e d l a n d e r ' s bacillus ( F r i s c h bacillus, Bacillus mucosus capsulatus, or Bacillus rhinoscleromatis). T h e o r g a n i s m s c a n always b e r e c o v e r e d f r o m g e n u i n e cases of scleroma ( C u n n i n g , 1949; Falcao a n d Preto, 1947; and Gugid, 1956). T h e m o d e of infection is u n c e r t a i n . M a n - t o - m a n infection is n o t confirmed. T h e o r g a n i s m s could b e f o u n d in t h e soils of gardens of p a t i e n t s w i t h scleroma a n d oz~ena in e n d e m i c areas ; t r a n s m i s s i o n t h r o u g h t h e cultivated soil was, therefore, suggested (Guild, 1956). S c l e r o m a is a n e n d e m i c disease p r e v a l e n t in E a s t e r n Europe, Russia, S o u t h America, a n d certain Far E a s t e r n islands. I t can b e c o n s i d e r e d e n d e m i c in Egypt. T h e p r e s e n t r e p o r t deals w i t h the clinical a n d radiological s t u d y of 84 cases of s c l e r o m a seen a n d treated b y X rays d u r i n g t h e years 1951-6, in the Radiotherapy Department of t h e Alexandria U n i v e r s i t y Hospitals. All cases were histologically proved.
THE CLINICAL MATERIAL S e x . - - T h e cases were equally d i s t r i b u t e d b e t w e e n t h e two sexes. A g e . - - T h e m e a n age in this series was 29 years; t h e y o u n g e s t was 12 years a n d t h e oldest 58 years. M o r e t h a n t w o - t h i r d s of t h e p a t i e n t s were b e t w e e n 15 a n d 35 years. Oeeupation.--More t h a n 90 p e r cent of o u r p a t i e n t s come f r o m a n d live in r u r a l areas a n d work o n t h e land. Signs, Symptoms, and Radiological Features of Seleroma.--The initial s y m p t o m s were referred to t h e nose in 74 patients, whereas t h e y were laryngeal in only i o cases. Tables I a n d 11 s u m m a r i z e the m a i n complaints at t h e clinical onset of t h e disease. T h e anatomical d i s t r i b u t i o n of t h e g r a n u l o m a as p r e s e n t e d on the first clinical e x a m i n a t i o n is s h o w n in
Table III.
T h e g r a n u l o m a starts as s u b m u c o s a l infiltration a n d t h i c k e n i n g in t h e nose, paranasal sinuses, a n d n a s o p h a r y n x . A t first t h e lesion is pale r e d in colour, soft a n d n o n - u l c e r a t e d , t e n d i n g to f o r m p o l y p o i d
Table L - - T H E INITIAL SYMPTOMS OF RHINOSCLEROMA CASES Symptom Number of Cases I. Nasal obstruction : - a. Unilateral 34 b. Bilateral I4 I2 2. Nasal bleeding 6 3. Nasal swelling 4. Nasal discharge 4 5. Nasal obstruction and discharge 4
Total 74 Table //.--THE
i. 2. 3. 4.
I N I T I A L SYMPTOMS OF SCLEROMA CASES
Symptom
Change of voice Pain m the throat Dyspncea Cough
LARYNGO-
Number of Case~ 5
2 2 I Total
IO
masses. U l c e r a t i o n w i t h c r u s t i n g a n d discharge occur later, especially in t h e nose a n d larynx. W i t h t h e o c c u r r e n c e of fibrosis t h e g r a n u l o m a b e c o m e s pale, firm, a n d pitted. A c t i v e g r a n u l o m a t o u s infiltrations m a y occur w i t h i n t h e scarred areas.
1. Signs and Symptoms of Rhinoscleroma.--The nose was free i n only z cases of p u r e laryngoscleroma.
Table III.--SHOWlNG THE ANATOMICAL INVOLVEMENT AT
THE Site
FIRST
CLINICAL EXAMINATION Number of Cases
Nasal 82 Paranasal slnuses* 32 Palate and fauces 19 Pharynx I4 Larynx 18 * As we did not attempt a hmstologlcalproof of the involvement o:f the paranasal sinuses by the granuloma, we assumed that cases showing definite radiological changes in the air-sinuses together with opacity on transilluminafion were so involved. a. Nasal o b s t r u c t i o n of a varying degree of severity was p r e s e n t in all cases of rhinoscleroma. T h e o b s t r u c t i o n was unilateral in 9 cases. Bilateral o b s t r u c t i o n was almost always u n e q u a l o n b o t h sides. b. G r a n u l o m a t o u s masses of t h e nose. T h e g r a n u l o m a was a p p a r e n t l y unilateral in 9 cases a n d bilateral in 73. Its c o m m o n e s t situations were t h e nasal s e p t u m a n d t h e region of the inferior t u r b i n a t e s , w h e r e t h e y could b e easily detected b y a n t e r i o r rhinoscopy. I t m a y e x t e n d forwards to t h e vestibule. E x t e n s i o n b e y o n d the m u c o c u t a n e o u s j u n c t i o n w i t h s u b c u t a n e o u s infiltration of t h e u p p e r lip was n o t e d i n 4 patients (Fig. i). F i b r o u s m e m b r a n e s a n d d i a p h r a g m s f o r m in t h e l o n g - s t a n d i n g cases, a n d these are also n o t e d after irradiation (Fig. 2).
SCLEROMA
OF
THE
UPPER
AIR-PASSAGES
45
c. Nasal expansion and deformity. A characteristic deformity can be produced by rhinoscleroma, viz., expansion of one or both sides of the nose and nares, with broadening, depression, and flattening of the nasal bridge (Fig. 3). A discharging sinus was seen at the junction of the nasal bones and cartilages on either side in i instance. In another patient the expansion of the ethmoid
air cells, together with left chronic dacryocystitis, produced bilateral inner canthal swellings (Fig. 4). d. Nasal bleeding: actual epistaxis occurred in 9 cases. Blood-stained discharge is more common, and was complained of by 55 patients. e. Nasal discharge: all patients of rhinoscleroma complained of some form of nasal discharge. Fcetor,
Fig. i. -Non-ulcerated rhinoscleroma of nasai s e p t u m m both nares. N o t e extension beyond the mucocutaneous iunction.
Fzg. 2 . - - F i b r o u s adhesions and m e m b r a n e s in the nasal vestlbule. Ulcerated granuloma of the soft palate with extension to the hard palate.
Fag. 3 . - - N a s a l deformity w i t h expansion of left side of nose.
Fzg. 4 . - - N a s a l deformity. L e f t dacryoeystltlS. R i g h t ethmoldal swelling. F l a t t e m n g of the nasal bridge.
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not unlike t h a t of ozcena, is a c o m m o n complaint. T h e discharge m a y b e b l o o d - s t a i n e d and m a y contain crusts. f. Pain a n d b u r n i n g sensation of t h e nose were c o m p l a i n e d of b y 7 patients. g. A n o s m i a was p r e s e n t ' i n 4 cases.
iii. A t r o p h y of t h e t u r b i n a t e s , especially t h e inferior, was seen i n 7 patients. iv. A t r o p h y a n d t h i n n i n g of the s e p t u m w i t h or w i t h o u t deviation was detectable in 26 cases.
Fig. 5 . - - W l d e m n g o f n a s a l c a v i t i e s . T h i n m n g a n d outwards expansion of the lateral nasal boundaries. N o t e polypoidal thickening of the nasal mucosa. Cloudiness of the left antrum.
Fig. 6 . - - E x p a n s i o n and thinning out of the lateral nasal boundarms. T h i n n i n g of the nasal septum. Thickening of nasal mucosa, polypoldal in parts.
F~g. 7.--Concentric nasal mucosaI thickemng. Locabzed outward displacement of the lower segment of the lateral nasal wall on the right side encroaching on the right antrum. Cloudiness of both antra.
h. T h e radiographic signs: t h e following signs m a y b e detectable : - i. T h i c k e n i n g of the nasal m u c o s a ; this m a y take the f o r m of either diffuse t h i c k e n i n g parallel to the b o n y b o u n d a r i e s or polypoidal f o r m a t i o n (Figs. 5, 6). ii. E x p a n s i o n of the nasal cavity a n d t h e vestibule was detected in 36 p a t i e n t s (Figs. 5, 6). T h e lateral nasal b o n y b o u n d a r i e s a n d t h e nasal b o n e s were t h i n n e d a n d displaced outwards. I n one i n s t a n c e these changes were localized to t h e lower p a r t of the r i g h t nasal fossa (Fig. 7). I n a n o t h e r instance sclerosis a n d t h i c k e n i n g of t h e nasal b o n e s were noted. T h i s was due to a n e l e m e n t of s e c o n d a r y infection, as clinically t h e r e was a sinus o n e i t h e r side at t h e j u n c t i o n of t h e nasal bones a n d cartilages (Fig. 8).
Fig. 8.--Seleroms of the nasal bones (due to secondary infection from discharging sinuses). Atrophic displaced septmn. Polypoid formation in the right antrum. Concentric mucosal thickening of the left antrum.
2. The Paranasal Sinuses.--The findings in t h e paranasal sinuses were elicited b y clinical e x a m i n a tion, t r a n s i l l u m i n a t i o n , a n d r a d i o g r a p h i c e x a m i n a tions : - a. T h e maxillary a n t r a : see Table I V . b. T h e frontal air sinuses s h o w e d cloudiness a n d opacity in 4 cases, whereas polypoidal masses were detectable in I case. F r o n t a l h e a d a c h e was a p r o m i n e n t s y m p t o m in 3 cases.
SCLEROMA
OF
THE
UPPER
AIR-PASSAGES
47
c. Expansion of the ethmoid air cells on one side w i t h inner canthal swelling was present in I case. Haziness of the ethmoidal air-cells w i t h absorption of the intercellular septa was observed in 4 instances.
the fauces, the tonsils, and the adjoining parts of the tongue. T h e palatine granuloma was apparently discontinuous w i t h the nasal one in I4 instances. T h e hard palate was infiltrated in continuity with the soft palate in z patients (Fig. 2). Perforation of
Fzg. 9 . - - D e p r e s s i o n of floor of maxdlary antrum. P l u m p soft
Fig. ~ o . - - D ~ r e c t extension of nasal granuloma into the dorsum of soft palate.
palate and uvula with irregular pitted ventral surface.
Fig. i i . - - P l u m p soft palate with smooth surface. Uvula is free.
3. The Palate and Fauces.--Continuity
of the nasal granuloma w i t h that of the soft palate across the posterior nares was noted in 5 patients (Fig. IO)., In 2 of these the granuloma e x t e n d e d to the pillars of Table I V . - - R A D I O G R A P H I C MAXILLARY
FINDINGS ANTRA
IN THE
NUMBER TYPE OF AFFECTION
Unilateral
Bilateral
2
8
I
9
IO
2
ii
13
" 5o
t. Free
z. Simple cloudiness and opacity (Figs. 5 a n d 6) 3. Concentric mucosalthickening (Fig. 8) $. Polypoidal mucosal thickening (Fig. 8) 3. Expansion of the antrum with depression of the floor
(Fig. 9)
Total
IO
Fzg. I2.
surface.
H u g e soft palate and uvula with wavy pitted dorsal N o t e continuity with the nasal granuloma.
the hard palate b y a granuloma of the nasal floor was seen twice. A t first the soft palatal granuloma forms a p l u m p spindle-shaped swelling (Figs. 9, i i , I2, I3). Later, fibrosis will cause its contraction, pitting, and irregularities, especially of the dorsum of the soft palate and uvula (Fig. i4). In one patient adhesions developed b e t w e e n the posterior pharyngeal wall and the soft palate. 4. Pharyngoscleroma.--In all except 2 cases of pharyngoscleroma there was an obvious continuity w i t h the nasal lesion across the posterior nares. T h e lateral wails of the nasopharynx are the c o m m o n e s t sites of affection. In i instance the granuloma extended d o w n w a r d s to the posterior wall of the oropharynx. T i n n i t u s and Eustachian tubal deafness were complained of b y 8 patients. 5. Laryngoscleroma.--In this series there are 2 examples of pure laryngoscleroma.
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In all cases of laryngoscleroma the subglottic region was involved. Granulomatous infiltration of one or both cords was seen in 5 patients, whereas the laryngeal surfaces of the aryepiglottic folds and
OF
RADIOLOGISTS
Other specific granulomas (such as syphilis, tuberculosis, and leishmaniasis), as well as tumours, were excluded by histological examination and the appropriate bacteriological and serological tests.
Fzg. I 4 . - - C o n t r a c t e d
Fig. I 3 . - - L e f t nasal expansion w i t h granulomatous masses m left nares. A big non-ulcerated sweIhng of soft palate and uvula, w i t h ddated veins over surface.
the epiglottis were implicated in 4 cases (Fig. 15). Fibrosis of the laryngeal lesions had two effects : - a. Concentric narrowing of the subglottic airway and the upper part of the trachea (Fig. 16). b. D e f o r m i t y and contraction of the epiglottis, aryepiglottic folds, and vocal cords. T h e symptoms of laryngoscleroma are cough, hoarseness of voice, local pain, discomfort, and Table
DOSE
V.--RESULTS
NUMBER OF CASES
Heavy dose M e d i u m dose Small dose
I4 5° 13
Total
77
soft palate and uvula w i t h pitted, irregular surface.
Ozcena and atrophic rhinitis may be the end stage of scleroma. Active granulomatous infiltrations m a y coexist with areas of atrophic sclerosed nasal mucosa. T h i s combination m u s t be carefully observed before radiation therapy. Bacteriological and histological examination will reveal such a combination. Radiotherapy of S c l e r o m a . - - V a r i o u s chemotherapeutics and antibiotics have been tried in the treatment of sclerorna. T h e general opinion is that the known antibiotics help to clear up the associated secondary infection, with little or no effect on the scleromatous lesion (Cunning, 1949; and Pradilloj, 1956), T w e l v e of our cases have received streptomycin treatment for periods varying from 2o-5o days before radiotherapy without apparent improvement, X - r a y therapy was the main line of treatment in our cases. A n X - r a y beam of a H . V . L . = 1"o5 ram. Cu. was used. T h e arrangement and size of the X - r a y portals were adapted to the distribution of the lesions in the individual cases.
OF X - R A Y T R E A T M E N T I N 7 7
]~ELIEF AFTER RELIEF AFTER FIRST SECOND T R E A T M E N T TREATMENT 9 29 I
expectorations that may be blood-stained. Dyspncea and stridor were complained of by 8 patients and necessitated tracheotomy. Fcetor was a c o m m o n complaint. Diagnosis o f S c l e r o m a . - - T h e diagnosis of our cases was based chiefly on: (i) Clinical examination; (2) Biopsy specimens taken f r o m the different anatomical sites; and (3) Radiographic investigation.
5 I2 2
CASES
I~ECEIVING MORE T H A N 2 SESSIONS m
7 9
INTRACTABLE CASES m 2 i
In an attempt to evaluate the effective X-ray dose in scleroma we irradiated our cases to three different dose levels : - a. Small dose: 6 o o - I o o o r in 2-4 weeks. b. M e d i u m dose: 15oo-2ooo r in 3-4 weeks. c. H i g h dose: of 3o0o-4ooo r in 5 weeks. T h e cases were followed for varying periods of I - 4 years. Table g summarizes the results of these
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
Scleroma of the Upper Air-passages A CLINICO-RADIOLOGICAL
STUDY
OF 84 CASES
G. E. M A S S O U D , F.F.R., AND H. K. A W W A D , M.D.
From the Radiological Department, University of Alexandria, Egypt S C L E R O M A is a specific g r a n u l o m a affecting, mainly, the nose. T h e u p p e r air-passages, trachea, a n d b r o n c h i m a y also b e involved, e i t h e r w i t h or w i t h o u t a p p a r e n t c o n t i n u i t y w i t h t h e nasal granuloma. Histologically, the lesion has a characteristic appearance. T h e c o r i u m a n d s u b m u c o s a are infiltrated b y g r a n u l o m a t o u s tissue c o m p o s e d of: (a) Vacuolated f o a m y histiocytes (Mikulicz cells); (b) P l a s m a cells; (c) D e g e n e r a t e d hyalinized p l a s m a cells (Russel's bodies) ; (d) N e u t r o p h i l s ; (e) A v a r y i n g n u m b e r of capillaries; a n d ( f ) Fibrocytes, fibroblasts, a n d collagen. Yasenetskiy a n d Y a m o p l o s k a y (1956) s h o w e d t h a t t h e degree of sclerosis is n o t indicative of t h e age of the pathological process. I t can o c c u r i n very early lesions, whereas active cellular infiltrations m a y b e f o u n d in old granulomas. T h e causative a g e n t is suggested to b e a strain of the F r i e d l a n d e r ' s bacillus ( F r i s c h bacillus, Bacillus mucosus capsulatus, or Bacillus rhinoscleromatis). T h e o r g a n i s m s c a n always b e r e c o v e r e d f r o m g e n u i n e cases of scleroma ( C u n n i n g , 1949; Falcao a n d Preto, 1947; and Gugid, 1956). T h e m o d e of infection is u n c e r t a i n . M a n - t o - m a n infection is n o t confirmed. T h e o r g a n i s m s could b e f o u n d in t h e soils of gardens of p a t i e n t s w i t h scleroma a n d oz~ena in e n d e m i c areas ; t r a n s m i s s i o n t h r o u g h t h e cultivated soil was, therefore, suggested (Guild, 1956). S c l e r o m a is a n e n d e m i c disease p r e v a l e n t in E a s t e r n Europe, Russia, S o u t h America, a n d certain Far E a s t e r n islands. I t can b e c o n s i d e r e d e n d e m i c in Egypt. T h e p r e s e n t r e p o r t deals w i t h the clinical a n d radiological s t u d y of 84 cases of s c l e r o m a seen a n d treated b y X rays d u r i n g t h e years 1951-6, in the Radiotherapy Department of t h e Alexandria U n i v e r s i t y Hospitals. All cases were histologically proved.
THE CLINICAL MATERIAL S e x . - - T h e cases were equally d i s t r i b u t e d b e t w e e n t h e two sexes. A g e . - - T h e m e a n age in this series was 29 years; t h e y o u n g e s t was 12 years a n d t h e oldest 58 years. M o r e t h a n t w o - t h i r d s of t h e p a t i e n t s were b e t w e e n 15 a n d 35 years. Oeeupation.--More t h a n 90 p e r cent of o u r p a t i e n t s come f r o m a n d live in r u r a l areas a n d work o n t h e land. Signs, Symptoms, and Radiological Features of Seleroma.--The initial s y m p t o m s were referred to t h e nose in 74 patients, whereas t h e y were laryngeal in only i o cases. Tables I a n d 11 s u m m a r i z e the m a i n complaints at t h e clinical onset of t h e disease. T h e anatomical d i s t r i b u t i o n of t h e g r a n u l o m a as p r e s e n t e d on the first clinical e x a m i n a t i o n is s h o w n in
Table III.
T h e g r a n u l o m a starts as s u b m u c o s a l infiltration a n d t h i c k e n i n g in t h e nose, paranasal sinuses, a n d n a s o p h a r y n x . A t first t h e lesion is pale r e d in colour, soft a n d n o n - u l c e r a t e d , t e n d i n g to f o r m p o l y p o i d
Table L - - T H E INITIAL SYMPTOMS OF RHINOSCLEROMA CASES Symptom Number of Cases I. Nasal obstruction : - a. Unilateral 34 b. Bilateral I4 I2 2. Nasal bleeding 6 3. Nasal swelling 4. Nasal discharge 4 5. Nasal obstruction and discharge 4
Total 74 Table //.--THE
i. 2. 3. 4.
I N I T I A L SYMPTOMS OF SCLEROMA CASES
Symptom
Change of voice Pain m the throat Dyspncea Cough
LARYNGO-
Number of Case~ 5
2 2 I Total
IO
masses. U l c e r a t i o n w i t h c r u s t i n g a n d discharge occur later, especially in t h e nose a n d larynx. W i t h t h e o c c u r r e n c e of fibrosis t h e g r a n u l o m a b e c o m e s pale, firm, a n d pitted. A c t i v e g r a n u l o m a t o u s infiltrations m a y occur w i t h i n t h e scarred areas.
1. Signs and Symptoms of Rhinoscleroma.--The nose was free i n only z cases of p u r e laryngoscleroma.
Table III.--SHOWlNG THE ANATOMICAL INVOLVEMENT AT
THE Site
FIRST
CLINICAL EXAMINATION Number of Cases
Nasal 82 Paranasal slnuses* 32 Palate and fauces 19 Pharynx I4 Larynx 18 * As we did not attempt a hmstologlcalproof of the involvement o:f the paranasal sinuses by the granuloma, we assumed that cases showing definite radiological changes in the air-sinuses together with opacity on transilluminafion were so involved. a. Nasal o b s t r u c t i o n of a varying degree of severity was p r e s e n t in all cases of rhinoscleroma. T h e o b s t r u c t i o n was unilateral in 9 cases. Bilateral o b s t r u c t i o n was almost always u n e q u a l o n b o t h sides. b. G r a n u l o m a t o u s masses of t h e nose. T h e g r a n u l o m a was a p p a r e n t l y unilateral in 9 cases a n d bilateral in 73. Its c o m m o n e s t situations were t h e nasal s e p t u m a n d t h e region of the inferior t u r b i n a t e s , w h e r e t h e y could b e easily detected b y a n t e r i o r rhinoscopy. I t m a y e x t e n d forwards to t h e vestibule. E x t e n s i o n b e y o n d the m u c o c u t a n e o u s j u n c t i o n w i t h s u b c u t a n e o u s infiltration of t h e u p p e r lip was n o t e d i n 4 patients (Fig. i). F i b r o u s m e m b r a n e s a n d d i a p h r a g m s f o r m in t h e l o n g - s t a n d i n g cases, a n d these are also n o t e d after irradiation (Fig. 2).
SCLEROMA
OF
THE
UPPER
AIR-PASSAGES
45
c. Nasal expansion and deformity. A characteristic deformity can be produced by rhinoscleroma, viz., expansion of one or both sides of the nose and nares, with broadening, depression, and flattening of the nasal bridge (Fig. 3). A discharging sinus was seen at the junction of the nasal bones and cartilages on either side in i instance. In another patient the expansion of the ethmoid
air cells, together with left chronic dacryocystitis, produced bilateral inner canthal swellings (Fig. 4). d. Nasal bleeding: actual epistaxis occurred in 9 cases. Blood-stained discharge is more common, and was complained of by 55 patients. e. Nasal discharge: all patients of rhinoscleroma complained of some form of nasal discharge. Fcetor,
Fig. i. -Non-ulcerated rhinoscleroma of nasai s e p t u m m both nares. N o t e extension beyond the mucocutaneous iunction.
Fzg. 2 . - - F i b r o u s adhesions and m e m b r a n e s in the nasal vestlbule. Ulcerated granuloma of the soft palate with extension to the hard palate.
Fag. 3 . - - N a s a l deformity w i t h expansion of left side of nose.
Fzg. 4 . - - N a s a l deformity. L e f t dacryoeystltlS. R i g h t ethmoldal swelling. F l a t t e m n g of the nasal bridge.
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not unlike t h a t of ozcena, is a c o m m o n complaint. T h e discharge m a y b e b l o o d - s t a i n e d and m a y contain crusts. f. Pain a n d b u r n i n g sensation of t h e nose were c o m p l a i n e d of b y 7 patients. g. A n o s m i a was p r e s e n t ' i n 4 cases.
iii. A t r o p h y of t h e t u r b i n a t e s , especially t h e inferior, was seen i n 7 patients. iv. A t r o p h y a n d t h i n n i n g of the s e p t u m w i t h or w i t h o u t deviation was detectable in 26 cases.
Fig. 5 . - - W l d e m n g o f n a s a l c a v i t i e s . T h i n m n g a n d outwards expansion of the lateral nasal boundaries. N o t e polypoidal thickening of the nasal mucosa. Cloudiness of the left antrum.
Fig. 6 . - - E x p a n s i o n and thinning out of the lateral nasal boundarms. T h i n n i n g of the nasal septum. Thickening of nasal mucosa, polypoldal in parts.
F~g. 7.--Concentric nasal mucosaI thickemng. Locabzed outward displacement of the lower segment of the lateral nasal wall on the right side encroaching on the right antrum. Cloudiness of both antra.
h. T h e radiographic signs: t h e following signs m a y b e detectable : - i. T h i c k e n i n g of the nasal m u c o s a ; this m a y take the f o r m of either diffuse t h i c k e n i n g parallel to the b o n y b o u n d a r i e s or polypoidal f o r m a t i o n (Figs. 5, 6). ii. E x p a n s i o n of the nasal cavity a n d t h e vestibule was detected in 36 p a t i e n t s (Figs. 5, 6). T h e lateral nasal b o n y b o u n d a r i e s a n d t h e nasal b o n e s were t h i n n e d a n d displaced outwards. I n one i n s t a n c e these changes were localized to t h e lower p a r t of the r i g h t nasal fossa (Fig. 7). I n a n o t h e r instance sclerosis a n d t h i c k e n i n g of t h e nasal b o n e s were noted. T h i s was due to a n e l e m e n t of s e c o n d a r y infection, as clinically t h e r e was a sinus o n e i t h e r side at t h e j u n c t i o n of t h e nasal bones a n d cartilages (Fig. 8).
Fig. 8.--Seleroms of the nasal bones (due to secondary infection from discharging sinuses). Atrophic displaced septmn. Polypoid formation in the right antrum. Concentric mucosal thickening of the left antrum.
2. The Paranasal Sinuses.--The findings in t h e paranasal sinuses were elicited b y clinical e x a m i n a tion, t r a n s i l l u m i n a t i o n , a n d r a d i o g r a p h i c e x a m i n a tions : - a. T h e maxillary a n t r a : see Table I V . b. T h e frontal air sinuses s h o w e d cloudiness a n d opacity in 4 cases, whereas polypoidal masses were detectable in I case. F r o n t a l h e a d a c h e was a p r o m i n e n t s y m p t o m in 3 cases.
SCLEROMA
OF
THE
UPPER
AIR-PASSAGES
47
c. Expansion of the ethmoid air cells on one side w i t h inner canthal swelling was present in I case. Haziness of the ethmoidal air-cells w i t h absorption of the intercellular septa was observed in 4 instances.
the fauces, the tonsils, and the adjoining parts of the tongue. T h e palatine granuloma was apparently discontinuous w i t h the nasal one in I4 instances. T h e hard palate was infiltrated in continuity with the soft palate in z patients (Fig. 2). Perforation of
Fzg. 9 . - - D e p r e s s i o n of floor of maxdlary antrum. P l u m p soft
Fig. ~ o . - - D ~ r e c t extension of nasal granuloma into the dorsum of soft palate.
palate and uvula with irregular pitted ventral surface.
Fig. i i . - - P l u m p soft palate with smooth surface. Uvula is free.
3. The Palate and Fauces.--Continuity
of the nasal granuloma w i t h that of the soft palate across the posterior nares was noted in 5 patients (Fig. IO)., In 2 of these the granuloma e x t e n d e d to the pillars of Table I V . - - R A D I O G R A P H I C MAXILLARY
FINDINGS ANTRA
IN THE
NUMBER TYPE OF AFFECTION
Unilateral
Bilateral
2
8
I
9
IO
2
ii
13
" 5o
t. Free
z. Simple cloudiness and opacity (Figs. 5 a n d 6) 3. Concentric mucosalthickening (Fig. 8) $. Polypoidal mucosal thickening (Fig. 8) 3. Expansion of the antrum with depression of the floor
(Fig. 9)
Total
IO
Fzg. I2.
surface.
H u g e soft palate and uvula with wavy pitted dorsal N o t e continuity with the nasal granuloma.
the hard palate b y a granuloma of the nasal floor was seen twice. A t first the soft palatal granuloma forms a p l u m p spindle-shaped swelling (Figs. 9, i i , I2, I3). Later, fibrosis will cause its contraction, pitting, and irregularities, especially of the dorsum of the soft palate and uvula (Fig. i4). In one patient adhesions developed b e t w e e n the posterior pharyngeal wall and the soft palate. 4. Pharyngoscleroma.--In all except 2 cases of pharyngoscleroma there was an obvious continuity w i t h the nasal lesion across the posterior nares. T h e lateral wails of the nasopharynx are the c o m m o n e s t sites of affection. In i instance the granuloma extended d o w n w a r d s to the posterior wall of the oropharynx. T i n n i t u s and Eustachian tubal deafness were complained of b y 8 patients. 5. Laryngoscleroma.--In this series there are 2 examples of pure laryngoscleroma.
48
JOURNAL
OF
THE
FACULTY
In all cases of laryngoscleroma the subglottic region was involved. Granulomatous infiltration of one or both cords was seen in 5 patients, whereas the laryngeal surfaces of the aryepiglottic folds and
OF
RADIOLOGISTS
Other specific granulomas (such as syphilis, tuberculosis, and leishmaniasis), as well as tumours, were excluded by histological examination and the appropriate bacteriological and serological tests.
Fzg. I 4 . - - C o n t r a c t e d
Fig. I 3 . - - L e f t nasal expansion w i t h granulomatous masses m left nares. A big non-ulcerated sweIhng of soft palate and uvula, w i t h ddated veins over surface.
the epiglottis were implicated in 4 cases (Fig. 15). Fibrosis of the laryngeal lesions had two effects : - a. Concentric narrowing of the subglottic airway and the upper part of the trachea (Fig. 16). b. D e f o r m i t y and contraction of the epiglottis, aryepiglottic folds, and vocal cords. T h e symptoms of laryngoscleroma are cough, hoarseness of voice, local pain, discomfort, and Table
DOSE
V.--RESULTS
NUMBER OF CASES
Heavy dose M e d i u m dose Small dose
I4 5° 13
Total
77
soft palate and uvula w i t h pitted, irregular surface.
Ozcena and atrophic rhinitis may be the end stage of scleroma. Active granulomatous infiltrations m a y coexist with areas of atrophic sclerosed nasal mucosa. T h i s combination m u s t be carefully observed before radiation therapy. Bacteriological and histological examination will reveal such a combination. Radiotherapy of S c l e r o m a . - - V a r i o u s chemotherapeutics and antibiotics have been tried in the treatment of sclerorna. T h e general opinion is that the known antibiotics help to clear up the associated secondary infection, with little or no effect on the scleromatous lesion (Cunning, 1949; and Pradilloj, 1956), T w e l v e of our cases have received streptomycin treatment for periods varying from 2o-5o days before radiotherapy without apparent improvement, X - r a y therapy was the main line of treatment in our cases. A n X - r a y beam of a H . V . L . = 1"o5 ram. Cu. was used. T h e arrangement and size of the X - r a y portals were adapted to the distribution of the lesions in the individual cases.
OF X - R A Y T R E A T M E N T I N 7 7
]~ELIEF AFTER RELIEF AFTER FIRST SECOND T R E A T M E N T TREATMENT 9 29 I
expectorations that may be blood-stained. Dyspncea and stridor were complained of by 8 patients and necessitated tracheotomy. Fcetor was a c o m m o n complaint. Diagnosis o f S c l e r o m a . - - T h e diagnosis of our cases was based chiefly on: (i) Clinical examination; (2) Biopsy specimens taken f r o m the different anatomical sites; and (3) Radiographic investigation.
5 I2 2
CASES
I~ECEIVING MORE T H A N 2 SESSIONS m
7 9
INTRACTABLE CASES m 2 i
In an attempt to evaluate the effective X-ray dose in scleroma we irradiated our cases to three different dose levels : - a. Small dose: 6 o o - I o o o r in 2-4 weeks. b. M e d i u m dose: 15oo-2ooo r in 3-4 weeks. c. H i g h dose: of 3o0o-4ooo r in 5 weeks. T h e cases were followed for varying periods of I - 4 years. Table g summarizes the results of these