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Cranial maduramycosis
393 TRANSACTIONS OF THE ROYAL SOCIETY OF TROPICAL MEDICINE AND HYGIENE.
Vol. 50.
No. 4.
July, 1956.
CRANIAL
MADURAMYCOSIS BY
B. B R E N D A N H I C K E Y * Khartoum
M a d u r a m y c o s i s affecting t h e b o n e s of t h e skull is a relative rarity as s h o w n b y GRANTHAM HILL (1931), w h e r e i n his series of 184 cases of m a d u r a m y c o s i s seen i n K h a r t o u m , i n only one is the disease r e c o r d e d as affecting the head, b u t the details are n o t given. T h r e e cases, however, have b e e n seen b y m e d u r i n g the past year, all of w h i c h have b e e n t r e a t e d i n the surgical u n i t i n K h a r t o u m Civil Hospital, a n d t h e i r clinical features are presented. CASE 1 Sudanese man aged about 20, a shepherd. This patient was admitted complaining of enlargement of the head and sinuses of the scalp of 8 years' standing. He stated that 8 years ago he was hit on the head with a stick. He then noticed a small nodule appear in the scalp, which gradually spread as a generalized thickening which very slowly involved the whole scalp. Sinuses appeared from time to time which discharged yellow granules and then healed. He had no other symptoms and followed his occupation as an agriculturist. On examination, he was a young man in good general condition whose calvarium and scalp were enlarged and thickened as shown in Fig. 1. The thickening stopped short at the attachments of the scalp which could not be moved over the bone. The head had been kept shaved and numerous healed sinuses were present. X-ray showed a mixed picture of bone destruction and new bone formation involving the whole calvarium, but not the basis cranii. Treatment. A biopsy was performed to confirm the diagnosis. T h e macroscopic appearance was of a typical yellow maduramycosis, confirmed histologically. The disease was so extensive that surgical excision, which would have involved removal of the calvarium and scalp, was impossible. The patient discharged himself as soon as the biopsy wound was healed. CASE 2 Sudanese male aged about 30, a farmer, who was admitted complaining of loss of vision in the right eye, proptosis and headache. He gave a history of having been involved in a fight 8 years previously when he was struck in the eye by somebody's head. For 3 weeks there was a painful swollen area over the right eyebrow, which subsided but reappeared and started to grow slowly and gradually push the eye forwards and downwards. Sight was diminished in the eye about 2 years ago. From time to time sinuses appeared in the region of the eyelids, discharged, and healed. On examination, the patient was in good general condition but had a hideous deformity of the face. The right eye was proptosed and displaced downwards and forwards, about 1½ inches in each direction. e I am indebted to Dr. Moharned Ali Zaki, Director, Sudan Medical Services, for permission to publish these cases, and to Professor H. V. Morgan for taking the clinical photographs.
394
CRANIAL MADURAMYCOSIS
There was gross chemosis and the lids could not be dosed over it. Behind the eye was a firm rather ill-defined swelling occupying the orbit and obviously spreading beyond its confines above and laterally. There were small scars of healed sinuses over the eyelids. X-ray (Fig. 2) showed a diffuse mass of new bone formation in the floor of the anterior cranial fossa on the right side going back to the pituitary fossa, growing well up into cranial cavity and down into the orbit. In spite of the lesion's occupying so much intracranial space, no abnormal neurological signs were found apart from diminution of vision in the right eye and ophthalmoplegia ; the C.S.F. was normal with a pressure of 120 ram. Treatment. A biopsy confirmed the diagnosis of yellow maduramycosis, and later the eye was enucIeated and through the orbit a large mass of infected very dense bony tissue was removed back as far as the optic foramen. T h e dura in the floor of the anterior fossa was exposed and was noted not t o be thickened or adherent. T h e removal was pushed to the limit of safety, but it was doubtful if the whole of the diseased tissue was removed. The patient made a good recovery ; his appearance was much improved and his headaches were relieved.
CASE 3 Sudanese male aged 24, a shepherd, complaining of protrusion of the right eye and diminution of vision. He stated that 4 years previously be noticed a small mass to the outer side of the right orbit. This gradually increased in size and 2 years previously it was removed, but it rapidly reappeared and vision became poor in the eye which commenced to protrude. He also noticed a swelling in the right frontal region which has grown steadily. Small sinuses have appeared from time to time around the right eye which have discharged and then healed. He had no pain nor headache. On examination he was in good general condition. There was a firm diffuse swelling in the right zygomatic region extending into the orbit where it was felt behind the eyelids. There were small scars of healed sinuses over this region. The eye was protruded about ½ inch forward. T h e eye itself had full movements and normal pupil but vision ~as reduced to 6/36 with 4D of papilloedema. Over the right frontal bone there was a firm non-tender thickened area about 2 inches in diameter. A n X-ray of the skull (Fig. 4) showed appearances very similar to those in Case 2, there being a large bony mass in the 'anterior cranial fossa extending well back to the region of the anterior clinoids. I n addition there was an area of periosteal bone formation with underlying patchy local absorption in the right frontal. T h e whole calvarium in Case 1 had the same appearance. Treatment. Operation was undertaken with the intention of clearing Out as much of the diseased tissue from the orbit as possible and commenced by making an incision over the prominent part of the swelling, but only a limited removal was practicable as absolutely torrential arterial haemorrhage from numerous arteries was encountered through the whole of the diseased tissue explored. T h e tissue being tough and fibrous, this was most troublesome to control. As total eradication was patently not possible it was not regarded as proper to risk life and the operation was abandoned. Exploration of the frontal thickening revealed exactly the same state of affairs. T h e tissue removed from both areas showed yellow maduramycosis. T h e patient made a normal recovery and there was some subjective improvement in vision.
DISCUSSION
Etiology. T h e generally accepted m o d e of a c q u i r i n g m a d u r a m y c o s i s is b y t h o r n prick ; t h e foot, the m o s t exposed m e m b e r , b e i n g t h e c o m m o n e s t site, a n d i n q u e s t i o n i n g p a t i e n t s one is always i m p r e s s e d b y t h e f r e q u e n c y of this story. W h i l e t h e r e are few i n h a b i t a n t s of N o r t h e r n S u d a n w h o have n o t b e e n p r i c k e d b y a t h o r n at s o m e time, t h e poorer class of c o u n t r y m a n who is m o s t exposed to s u c h t r a u m a is b y far the m o s t f r e q u e n t sufferer (GRANTHAM HILL, loc. cit.). T h e s e p a t i e n t s were all of this class, b u t n o n e gave a history of t h o r n p r i c k though two gave a h i s t o r y of t r a u m a ; a n d o n e is forced to t h e c o n c l u s i o n t h a t t h e i n f e c t i o n wa, i n t r o d u c e d at t h a t time. T h e t h i r d p a t i e n t d e n i e d t r a u m a , b u t there b e i n g n o other" site ot f u n g u s i n f e c t i o n it c a n only b e c o n c l u d e d t h a t s o m e h o w i n f e c t i o n was i n t r o d u c e d at tha' site directly. I n all cases the yellow f u n g u s , n o t t h e b l a c k as i n GRANTHAM HILL'S on,
Fig. l.-Case 1. Diffuse swelling of the scalp and calvarium with scar of closed sinus.
Fig. 2.-Case 2. (To face page
394)
Dense
retro-orbital
new
bone
formation.
Fig. 3.-Case 3. Dense swelling of frontal and zygomatic region and small numerous scars of white closed sinuses around the eye.
Fig. 4.-Case 3.
Diffuse
retro-orbital new bone formation of frontal bones.
and
irregular
destruction
B. BRENDAN HICKEY
395
recorded case, was found. The prominence of bone involvement is a feature of this variety which has a predilection for invading bone.
Course of the disease. All the cases recorded are remarkable for the degree of chronicity notable even in a chronic disease, and for the late intracranial spread. Two of the patients gave a history of 8 and one of 4 years; and in none of the cases was there any appearance that life was threatened within the near future. It is suggested that this is due to the excellence of the blood supply in the part affected. The body was putting up a high resistance, as is seen in all the biopsy specimens where the fungus was represented only by hyalinized remains. Sinus formation is not a prominent feature, and the sinuses healed with small scars. T h e late interference with function illustrated by the intact occular movements in Case 3, in spite of actual proptosis, is a feature of the disease where one often finds an almost unrecognizable foot or hand riddled with sinuses but with perfectly moving digits. The absence of intracranial spread assumed on clinical grounds in Cases 1 and 3, and proved at operation in Case 2, may well be a further illustration of the insulative properties of the dura, pointed out by TROTTER (1941). Certainly in the other parts of the body the infection ramifies absolutely ruthlessly with even less respect for tissue planes than malignant disease, though tendons and nerves are the last structures to be affected. It is suggested that the maintained nutrition and lack of systemic toxic manifestations is due to the total absence of secondary infection in these cases.
X-ray appearances. These are noteworthy in several respects. The typical X-ray appearance of maduramycosis in a long bone is one of expansion, periosteal new bone formation, and punctate areas of osteoporosis without sequestrum formation. The picture is t y p i c a l - though the uninitiated may confuse it with a n e o p l a s m - and is generally associated with the yellow fungus. A clinical variety seen in the lower end of the radius almost appears to be a primary osseous infection. The black fungus tends to produce greater osseous destruction when it invades bone. The radiological picture here is of two varieties, both of which are seen in Case 3 (Fig. 4). In the base of the skull there is a mass of uniform dense new bone formed, seen also in Case 2. In the affected clavarium, however, the new bone formation is far less dense, and there is evidence of destruction proceeding at the same time. This appearance is similar to the appearance seen in affected long bones. The dense bony mass produced in the base of the skull, in my experience is seen only as a reaction to maduramycosis in that region. The explanation is obscure, but it might be attributable to the fact that the pericranium on the one surface, and the dura on the other, are much more adherent in the base of the skull than the calvarium, and this predominating bone production is a variety of stress reaction to the tension in the space in which the fungus is attempting to grow.
Treatment. This is disappointing. In the absence of any known effective antibiotic, surgical excision is the only treatment that can be undertaken, and in all the cases described the disease was too extensive for a total removal to be achieved. In Case 3, the patient had had a previous operation at an earlier stage, but the disease had recurred, as is our experience with the condition elsewhere in the body. However, even a partial removal of diseased tissue gives relief and appears to check the course of the disease for a time. G
396
CRANIAL MADURAMYCOSIS SUMMARY OF CONTENTS
(I)
Three cases of maduramycosisinvolving the skull are described.
(2) Their clinical features are discussed in detail. (3) The radiological appearances of maduramycosisinvolving bone in general and the skull in particular are presented. REFERENCES GRANTHAMHILL C. (1931). Trans. R. Soc. trop. Med. Hyg., 25, 39. TROTTER, WILFRED(1941). Collected Papers, Oxford Medical Publications, p. 47.
Vol. 50.
No. 4.
July, 1956.
CRANIAL
MADURAMYCOSIS BY
B. B R E N D A N H I C K E Y * Khartoum
M a d u r a m y c o s i s affecting t h e b o n e s of t h e skull is a relative rarity as s h o w n b y GRANTHAM HILL (1931), w h e r e i n his series of 184 cases of m a d u r a m y c o s i s seen i n K h a r t o u m , i n only one is the disease r e c o r d e d as affecting the head, b u t the details are n o t given. T h r e e cases, however, have b e e n seen b y m e d u r i n g the past year, all of w h i c h have b e e n t r e a t e d i n the surgical u n i t i n K h a r t o u m Civil Hospital, a n d t h e i r clinical features are presented. CASE 1 Sudanese man aged about 20, a shepherd. This patient was admitted complaining of enlargement of the head and sinuses of the scalp of 8 years' standing. He stated that 8 years ago he was hit on the head with a stick. He then noticed a small nodule appear in the scalp, which gradually spread as a generalized thickening which very slowly involved the whole scalp. Sinuses appeared from time to time which discharged yellow granules and then healed. He had no other symptoms and followed his occupation as an agriculturist. On examination, he was a young man in good general condition whose calvarium and scalp were enlarged and thickened as shown in Fig. 1. The thickening stopped short at the attachments of the scalp which could not be moved over the bone. The head had been kept shaved and numerous healed sinuses were present. X-ray showed a mixed picture of bone destruction and new bone formation involving the whole calvarium, but not the basis cranii. Treatment. A biopsy was performed to confirm the diagnosis. T h e macroscopic appearance was of a typical yellow maduramycosis, confirmed histologically. The disease was so extensive that surgical excision, which would have involved removal of the calvarium and scalp, was impossible. The patient discharged himself as soon as the biopsy wound was healed. CASE 2 Sudanese male aged about 30, a farmer, who was admitted complaining of loss of vision in the right eye, proptosis and headache. He gave a history of having been involved in a fight 8 years previously when he was struck in the eye by somebody's head. For 3 weeks there was a painful swollen area over the right eyebrow, which subsided but reappeared and started to grow slowly and gradually push the eye forwards and downwards. Sight was diminished in the eye about 2 years ago. From time to time sinuses appeared in the region of the eyelids, discharged, and healed. On examination, the patient was in good general condition but had a hideous deformity of the face. The right eye was proptosed and displaced downwards and forwards, about 1½ inches in each direction. e I am indebted to Dr. Moharned Ali Zaki, Director, Sudan Medical Services, for permission to publish these cases, and to Professor H. V. Morgan for taking the clinical photographs.
394
CRANIAL MADURAMYCOSIS
There was gross chemosis and the lids could not be dosed over it. Behind the eye was a firm rather ill-defined swelling occupying the orbit and obviously spreading beyond its confines above and laterally. There were small scars of healed sinuses over the eyelids. X-ray (Fig. 2) showed a diffuse mass of new bone formation in the floor of the anterior cranial fossa on the right side going back to the pituitary fossa, growing well up into cranial cavity and down into the orbit. In spite of the lesion's occupying so much intracranial space, no abnormal neurological signs were found apart from diminution of vision in the right eye and ophthalmoplegia ; the C.S.F. was normal with a pressure of 120 ram. Treatment. A biopsy confirmed the diagnosis of yellow maduramycosis, and later the eye was enucIeated and through the orbit a large mass of infected very dense bony tissue was removed back as far as the optic foramen. T h e dura in the floor of the anterior fossa was exposed and was noted not t o be thickened or adherent. T h e removal was pushed to the limit of safety, but it was doubtful if the whole of the diseased tissue was removed. The patient made a good recovery ; his appearance was much improved and his headaches were relieved.
CASE 3 Sudanese male aged 24, a shepherd, complaining of protrusion of the right eye and diminution of vision. He stated that 4 years previously be noticed a small mass to the outer side of the right orbit. This gradually increased in size and 2 years previously it was removed, but it rapidly reappeared and vision became poor in the eye which commenced to protrude. He also noticed a swelling in the right frontal region which has grown steadily. Small sinuses have appeared from time to time around the right eye which have discharged and then healed. He had no pain nor headache. On examination he was in good general condition. There was a firm diffuse swelling in the right zygomatic region extending into the orbit where it was felt behind the eyelids. There were small scars of healed sinuses over this region. The eye was protruded about ½ inch forward. T h e eye itself had full movements and normal pupil but vision ~as reduced to 6/36 with 4D of papilloedema. Over the right frontal bone there was a firm non-tender thickened area about 2 inches in diameter. A n X-ray of the skull (Fig. 4) showed appearances very similar to those in Case 2, there being a large bony mass in the 'anterior cranial fossa extending well back to the region of the anterior clinoids. I n addition there was an area of periosteal bone formation with underlying patchy local absorption in the right frontal. T h e whole calvarium in Case 1 had the same appearance. Treatment. Operation was undertaken with the intention of clearing Out as much of the diseased tissue from the orbit as possible and commenced by making an incision over the prominent part of the swelling, but only a limited removal was practicable as absolutely torrential arterial haemorrhage from numerous arteries was encountered through the whole of the diseased tissue explored. T h e tissue being tough and fibrous, this was most troublesome to control. As total eradication was patently not possible it was not regarded as proper to risk life and the operation was abandoned. Exploration of the frontal thickening revealed exactly the same state of affairs. T h e tissue removed from both areas showed yellow maduramycosis. T h e patient made a normal recovery and there was some subjective improvement in vision.
DISCUSSION
Etiology. T h e generally accepted m o d e of a c q u i r i n g m a d u r a m y c o s i s is b y t h o r n prick ; t h e foot, the m o s t exposed m e m b e r , b e i n g t h e c o m m o n e s t site, a n d i n q u e s t i o n i n g p a t i e n t s one is always i m p r e s s e d b y t h e f r e q u e n c y of this story. W h i l e t h e r e are few i n h a b i t a n t s of N o r t h e r n S u d a n w h o have n o t b e e n p r i c k e d b y a t h o r n at s o m e time, t h e poorer class of c o u n t r y m a n who is m o s t exposed to s u c h t r a u m a is b y far the m o s t f r e q u e n t sufferer (GRANTHAM HILL, loc. cit.). T h e s e p a t i e n t s were all of this class, b u t n o n e gave a history of t h o r n p r i c k though two gave a h i s t o r y of t r a u m a ; a n d o n e is forced to t h e c o n c l u s i o n t h a t t h e i n f e c t i o n wa, i n t r o d u c e d at t h a t time. T h e t h i r d p a t i e n t d e n i e d t r a u m a , b u t there b e i n g n o other" site ot f u n g u s i n f e c t i o n it c a n only b e c o n c l u d e d t h a t s o m e h o w i n f e c t i o n was i n t r o d u c e d at tha' site directly. I n all cases the yellow f u n g u s , n o t t h e b l a c k as i n GRANTHAM HILL'S on,
Fig. l.-Case 1. Diffuse swelling of the scalp and calvarium with scar of closed sinus.
Fig. 2.-Case 2. (To face page
394)
Dense
retro-orbital
new
bone
formation.
Fig. 3.-Case 3. Dense swelling of frontal and zygomatic region and small numerous scars of white closed sinuses around the eye.
Fig. 4.-Case 3.
Diffuse
retro-orbital new bone formation of frontal bones.
and
irregular
destruction
B. BRENDAN HICKEY
395
recorded case, was found. The prominence of bone involvement is a feature of this variety which has a predilection for invading bone.
Course of the disease. All the cases recorded are remarkable for the degree of chronicity notable even in a chronic disease, and for the late intracranial spread. Two of the patients gave a history of 8 and one of 4 years; and in none of the cases was there any appearance that life was threatened within the near future. It is suggested that this is due to the excellence of the blood supply in the part affected. The body was putting up a high resistance, as is seen in all the biopsy specimens where the fungus was represented only by hyalinized remains. Sinus formation is not a prominent feature, and the sinuses healed with small scars. T h e late interference with function illustrated by the intact occular movements in Case 3, in spite of actual proptosis, is a feature of the disease where one often finds an almost unrecognizable foot or hand riddled with sinuses but with perfectly moving digits. The absence of intracranial spread assumed on clinical grounds in Cases 1 and 3, and proved at operation in Case 2, may well be a further illustration of the insulative properties of the dura, pointed out by TROTTER (1941). Certainly in the other parts of the body the infection ramifies absolutely ruthlessly with even less respect for tissue planes than malignant disease, though tendons and nerves are the last structures to be affected. It is suggested that the maintained nutrition and lack of systemic toxic manifestations is due to the total absence of secondary infection in these cases.
X-ray appearances. These are noteworthy in several respects. The typical X-ray appearance of maduramycosis in a long bone is one of expansion, periosteal new bone formation, and punctate areas of osteoporosis without sequestrum formation. The picture is t y p i c a l - though the uninitiated may confuse it with a n e o p l a s m - and is generally associated with the yellow fungus. A clinical variety seen in the lower end of the radius almost appears to be a primary osseous infection. The black fungus tends to produce greater osseous destruction when it invades bone. The radiological picture here is of two varieties, both of which are seen in Case 3 (Fig. 4). In the base of the skull there is a mass of uniform dense new bone formed, seen also in Case 2. In the affected clavarium, however, the new bone formation is far less dense, and there is evidence of destruction proceeding at the same time. This appearance is similar to the appearance seen in affected long bones. The dense bony mass produced in the base of the skull, in my experience is seen only as a reaction to maduramycosis in that region. The explanation is obscure, but it might be attributable to the fact that the pericranium on the one surface, and the dura on the other, are much more adherent in the base of the skull than the calvarium, and this predominating bone production is a variety of stress reaction to the tension in the space in which the fungus is attempting to grow.
Treatment. This is disappointing. In the absence of any known effective antibiotic, surgical excision is the only treatment that can be undertaken, and in all the cases described the disease was too extensive for a total removal to be achieved. In Case 3, the patient had had a previous operation at an earlier stage, but the disease had recurred, as is our experience with the condition elsewhere in the body. However, even a partial removal of diseased tissue gives relief and appears to check the course of the disease for a time. G
396
CRANIAL MADURAMYCOSIS SUMMARY OF CONTENTS
(I)
Three cases of maduramycosisinvolving the skull are described.
(2) Their clinical features are discussed in detail. (3) The radiological appearances of maduramycosisinvolving bone in general and the skull in particular are presented. REFERENCES GRANTHAMHILL C. (1931). Trans. R. Soc. trop. Med. Hyg., 25, 39. TROTTER, WILFRED(1941). Collected Papers, Oxford Medical Publications, p. 47.