Arterio-venous fistula of the ear By D. K.
MUKHERJEE
(Enugu) and P.
OKEOWO
(Lagos)
Summary A CASE of congenital arterio-venous fistula involving the external ear in a 33year-old female patient is described in this paper. She presented to the hospital with severe haemorrhage from the swelling over the ear necessitating immediate admission into hospital and transfusion of whole blood. The treatment of the case is described in detail. She is alive and well 3 years after her definitive treatment. Introduction Arterio-venous fistulae are abnormal direct communications between arteries and veins. There can be (1) direct fistulous communication between artery and vein, (2) A.V. fistulae associated with interposed aneurysmal dilated sacs, (3) cirsoid aneurysms in which there are multiple communications between the artery and vein and marked dilatation of arteries and veins in the vicinity of the lesion or (4) arterio-venous fistulae in vascular tumours like haemangiomas and haemangio-endotheliomas. There are two types of A.V. fistulae—(1) Traumatic type and (2) the congenital type. The traumatic type of A.V. fistula is caused by injury to the walls of the artery and vein by a foreign body in such a way that blood from the artery finds its way into the vein, either at the time of injury or by erosion of the vascular walls at a later date. The vein then becomes enlarged and tortuous due to arterial blood pressure. This entrance of arterial blood into the venous system may cause serious embarrassment of the right side of the heart, and cardiac hypertrophy (Davis, 1960). In the congenital variety of A.V. fistula, the arterial and venous components of the vascular system—which develop together from a common vascular tube in early foetal life—fail to separate from each other—thereby resulting in communications (usually multiple) between otherwise normal arteries and veins. The localized type of this group is usually cirsoid in nature—with multiple communications between the arteries and veins—and it usually occurs in the neck. The generalized type usually occurs in the extremities. Arterio-venous fistulae—whether congenital or acquired (traumatic variety) —are uncommon conditions, and an A.V. fistula involving the ear is a rarity indeed. A literature-search by the W.H.O. Medline service in Geneva (1976) turned up only one reference to this condition in all journals the world over in the last 10 years. This was in a 35-year-old male patient with a traumatic A.V. fistula (Ramadass, 1967). In this paper we are reporting another case of A.V. fistula involving the auricle. Case report A 33-year-old female patient reported to the E.N.T. ciinic of the Lagos University Teaching Hospital on 5.7.73 complaining of a swelling of the left ear
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for the last 3 years and bleeding from it for 7 days. Careful questioning elicited the fact that the swelling first appeared as a small lump at the back of the left ear 3 years earlier. There was no history of any trauma. It had gradually increased in size—more so since her pregnancy in 1972. She never felt any pain over the swelling but had a sensation of heat in the area. She started bleeding from the swelling 7 days before presenting, without any trauma, and had to be admitted to the Casualty Department for observation. Two units of whole blood had to be transfused. Examination of the area on 5 July 1973 revealed thefindingsas indicated in Figures 1 and 2. She continued oozing from the ulcerated areas over the swelling even after the application of a firm pressure-dressing and needed 6 units of whole blood in the 7 days following her admission to hospital. A blood-examination on 20 July 1973 revealed a haemoglobin count of 83 per cent with W.B.C. count of 8,000/ cmm (neutrophils 68 per cent, lymphocytes 16 per cent, eosinophils 12 per cent and monocytes 4 per cent). E.S.R. was 24 mm/lst hour. Blood biochemistry revealed HCO3—21 m.eq/litre, chlorides 104 m.eq/1, Na—134 m.eq/1, K— 4 m.eq/1, Ca—9-6 mg/100 ml, blood urea—19 mg/100 ml. Left carotid angiography was performed on her on 19 July 1973 (Figs. 3 and 4). It was reported as : 'There is a subcutaneous arterio-venous malformation measuring about 4 cm in width and 6 cm in height, situated in the left mastoid area. It contains many
Vigorous pulsation. Stops on pressing the vessel in the pre-auricular area Vigorous pulsation pre-auricular area Areas of Ulceration
Lump post-auricular area. Soft and comp ressible. Vigorous pulsation.
Moderate pulsation, but not as prominent as the pre-auricular one feftW FIG. 1
Schematic diagram showing the clinical findings in the left auricular area.
CLINICAL RECORDS
171
FIG. 2 Pre-operative photograph of the left car.
wide vessels. It is fed by a wide branch from the external carotid artery, probably its occipital branch. This feeding artery has its take-off about 2 cm cranial to the carotid bifurcation in the neck. The internal carotid artery does not appear to take part in the malformation. The facial branches of the external carotid are incompletely filled indicating collateral circulation from the opposite side.' She was operated on under a general anaesthetic on 23.7.73. A vertical skinincision was made in the left pre-auricular area over the area of maximum pulsation. The skin only was reflected. The sub-cutaneous tissues were carefully dissected and reflected. Massive vessels exposed. There was one artery and one vein—each of at least 1 in diameter—with numerous thin-walled short communications between them. The vein was bifurcating into two tributaries opposite the region between the tragus and helix. The main two vessels were separated from neighbouring soft tissues and ligatcd (multiply) with black silk. The two tributaries of the vein were also ligated. There was very severe bleeding (arterial spurting type) when any of the thin-walled communicating channels were
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D. K. MUKHERJEE AND P. OKEOWO
FIG. 2a Pre-operativc photograph of the left ear.
inadvertantly punctured. After the procedure, the skin was sutured with nylon. No drain. She needed 4 units of blood during the operation. The findings on 26.7.73 (4th day post-operative)—(a) no pulsation in preauricular area, (b) no pulsation in supra-auricular area, (c) swelling along the helix much reduced, (d) moderate pulsation in the post-auricular area, and (e) below the lobe of ear. (f) There was some coldness of skin of the helix about 1 in from the superior corner. There was also some oedema around the left eye— possibly due to obstruction to venous drainage. There was obviously another communicating channel (artery and vein) behind the auricle but since the first operation left part of the helical skin anoxic—it was
FIG. 3 Left Carotid angiography—arterial phase.
FIG. 4 Left Carotid angiography—venous phase.
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D. K. MUKHERJEE AND P. OKEOWO
FIG. 5 Post-operative photograph. It may be noted that the left ear is a little larger in size than the right ear.
decided to postpone the operation of ligation of these until the auricular skin had recovered from its anoxic state. The second operation was performed a month after the first. A huge postauricular artery and vein of 1 in. diameter each were found and ligated with black silk (multiple ligatures). She needed 3 units of blood this time. Even after this operation, the auricular swelling did not subside completely and it was then decided to carry out a carotid ligation. On 3.12.73 she was operated on under G.A. A longitudinal incision along the anterior border of the left sterno-mastoid muscle was given and the great vessels of the neck exposed. A very interesting finding was made at this stage. There was only one carotid artery {internal carotid). No external carotid—branch of the common carotid artery—was found. A thorough search of the area failed to reveal any trace of it. There was one vessel arising from the internal carotid artery coursing upwards and laterally towards the auricle. Since there was no external carotid artery, it was decided to ligate the common carotid artery. This was carried out. She made an uneventful recovery. The swelling over the left auricle subsided completely. She has since then been seen periodically and been doing well (Fig. 5). There has been no further recurrence of the auricular swelling and no episodes of bleeding from the ear.
CLINICAL RECORDS
175
FIG 5a Post-operative photograph of the left auricular area.
Discussion The case described is one of a congenital A.V. fistula involving the auricle. The absence of the external carotid artery can probably be explained by the fact that the superficial temporal tmd occipital branches of it had already been ligated before the carotid system of vessels was exposed in the neck. The facial branch was poorly developed or may have been absent—as already indicated in the carotid angiogram (looking back at it from this angle rather than the suggestion that the poor filling was because of collateral circulation from the other side). It would thus seem to be, in retrospect, that the ascending pharyngeal, the superior thyroid, the lingual and the facial branches of the external carotid artery were congenitally absent or may have atrophied as the major portion of the external carotid blood—flow was going to the gigantic superficial temporal and occipital arteries—and leaking out into the venous system. The vessel going towards the auricular area was an anomalous blood vessel arising from the internal carotid artery. Thus it would appear that there were a few interesting vascular anomalies uncovered in this case. The primary cause would seem to be the congenital A.V. connections involving the superficial temporal and the occipital arteries and veins, leading to gigantic enlargement of these two sets and atrophy of the other vessels of the external carotid system.
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D. K. MUKHERJEE AND P. OKEOWO
It has been suggested that the best treatment of an arterio-venous fistula involving the ear is either excision of the whole A.V. fistulous mass or amputation of the auricle itself (Dingman and Grabb, 1965). However, this case has shown conclusively that ligation of all the feeding vessels can lead to a very satisfactory result, both functionally and also cosmetically. It is hoped that in future cases of this type will be dealt with by this multiple ligation method rather than the excision of the whole fistulous mass which is a tremendously bloody, difficult and risky procedure. Amputation of the auricle should not be considered until every other avenue of treatment has been explored and found unavailing. Acknowledgement The permission granted by the Director of Administration of Lagos University Teaching Hospital allowing the publication of the case-finding is gratefully acknowledged. The contributions of the Medical Illustration units of Lagos University Teaching Hospital (for the photographs) and the University of Nigeria Teaching Hospital, Enugu (for the angiogram photographs and the clinical illustration) are remembered with thankfulness. REFERENCES DAVIS, L. (1960) Christopher's Textbook of Surgery, 7th ed. Longmans, Philadelphia and London, p, 1358. DINGMAN, R. O., and GRABB, W. C. (1965) Plastic and Reconstructive Surgery, 35, 620. RAMADASS, T. (1967) Journal of Laryngology and Otology, 81, 1171. W.H.O. MEDLINE RESEARCH CENTRE, Geneva (1976) Search No. 2963. Dept. of Otolaryngology, University of Nigeria, Enugu Campus, Anambra State, Nigeria.
MUKHERJEE
(Enugu) and P.
OKEOWO
(Lagos)
Summary A CASE of congenital arterio-venous fistula involving the external ear in a 33year-old female patient is described in this paper. She presented to the hospital with severe haemorrhage from the swelling over the ear necessitating immediate admission into hospital and transfusion of whole blood. The treatment of the case is described in detail. She is alive and well 3 years after her definitive treatment. Introduction Arterio-venous fistulae are abnormal direct communications between arteries and veins. There can be (1) direct fistulous communication between artery and vein, (2) A.V. fistulae associated with interposed aneurysmal dilated sacs, (3) cirsoid aneurysms in which there are multiple communications between the artery and vein and marked dilatation of arteries and veins in the vicinity of the lesion or (4) arterio-venous fistulae in vascular tumours like haemangiomas and haemangio-endotheliomas. There are two types of A.V. fistulae—(1) Traumatic type and (2) the congenital type. The traumatic type of A.V. fistula is caused by injury to the walls of the artery and vein by a foreign body in such a way that blood from the artery finds its way into the vein, either at the time of injury or by erosion of the vascular walls at a later date. The vein then becomes enlarged and tortuous due to arterial blood pressure. This entrance of arterial blood into the venous system may cause serious embarrassment of the right side of the heart, and cardiac hypertrophy (Davis, 1960). In the congenital variety of A.V. fistula, the arterial and venous components of the vascular system—which develop together from a common vascular tube in early foetal life—fail to separate from each other—thereby resulting in communications (usually multiple) between otherwise normal arteries and veins. The localized type of this group is usually cirsoid in nature—with multiple communications between the arteries and veins—and it usually occurs in the neck. The generalized type usually occurs in the extremities. Arterio-venous fistulae—whether congenital or acquired (traumatic variety) —are uncommon conditions, and an A.V. fistula involving the ear is a rarity indeed. A literature-search by the W.H.O. Medline service in Geneva (1976) turned up only one reference to this condition in all journals the world over in the last 10 years. This was in a 35-year-old male patient with a traumatic A.V. fistula (Ramadass, 1967). In this paper we are reporting another case of A.V. fistula involving the auricle. Case report A 33-year-old female patient reported to the E.N.T. ciinic of the Lagos University Teaching Hospital on 5.7.73 complaining of a swelling of the left ear
170
D. K. MUKHERJEE AND P. OKEOWO
for the last 3 years and bleeding from it for 7 days. Careful questioning elicited the fact that the swelling first appeared as a small lump at the back of the left ear 3 years earlier. There was no history of any trauma. It had gradually increased in size—more so since her pregnancy in 1972. She never felt any pain over the swelling but had a sensation of heat in the area. She started bleeding from the swelling 7 days before presenting, without any trauma, and had to be admitted to the Casualty Department for observation. Two units of whole blood had to be transfused. Examination of the area on 5 July 1973 revealed thefindingsas indicated in Figures 1 and 2. She continued oozing from the ulcerated areas over the swelling even after the application of a firm pressure-dressing and needed 6 units of whole blood in the 7 days following her admission to hospital. A blood-examination on 20 July 1973 revealed a haemoglobin count of 83 per cent with W.B.C. count of 8,000/ cmm (neutrophils 68 per cent, lymphocytes 16 per cent, eosinophils 12 per cent and monocytes 4 per cent). E.S.R. was 24 mm/lst hour. Blood biochemistry revealed HCO3—21 m.eq/litre, chlorides 104 m.eq/1, Na—134 m.eq/1, K— 4 m.eq/1, Ca—9-6 mg/100 ml, blood urea—19 mg/100 ml. Left carotid angiography was performed on her on 19 July 1973 (Figs. 3 and 4). It was reported as : 'There is a subcutaneous arterio-venous malformation measuring about 4 cm in width and 6 cm in height, situated in the left mastoid area. It contains many
Vigorous pulsation. Stops on pressing the vessel in the pre-auricular area Vigorous pulsation pre-auricular area Areas of Ulceration
Lump post-auricular area. Soft and comp ressible. Vigorous pulsation.
Moderate pulsation, but not as prominent as the pre-auricular one feftW FIG. 1
Schematic diagram showing the clinical findings in the left auricular area.
CLINICAL RECORDS
171
FIG. 2 Pre-operative photograph of the left car.
wide vessels. It is fed by a wide branch from the external carotid artery, probably its occipital branch. This feeding artery has its take-off about 2 cm cranial to the carotid bifurcation in the neck. The internal carotid artery does not appear to take part in the malformation. The facial branches of the external carotid are incompletely filled indicating collateral circulation from the opposite side.' She was operated on under a general anaesthetic on 23.7.73. A vertical skinincision was made in the left pre-auricular area over the area of maximum pulsation. The skin only was reflected. The sub-cutaneous tissues were carefully dissected and reflected. Massive vessels exposed. There was one artery and one vein—each of at least 1 in diameter—with numerous thin-walled short communications between them. The vein was bifurcating into two tributaries opposite the region between the tragus and helix. The main two vessels were separated from neighbouring soft tissues and ligatcd (multiply) with black silk. The two tributaries of the vein were also ligated. There was very severe bleeding (arterial spurting type) when any of the thin-walled communicating channels were
172
D. K. MUKHERJEE AND P. OKEOWO
FIG. 2a Pre-operativc photograph of the left ear.
inadvertantly punctured. After the procedure, the skin was sutured with nylon. No drain. She needed 4 units of blood during the operation. The findings on 26.7.73 (4th day post-operative)—(a) no pulsation in preauricular area, (b) no pulsation in supra-auricular area, (c) swelling along the helix much reduced, (d) moderate pulsation in the post-auricular area, and (e) below the lobe of ear. (f) There was some coldness of skin of the helix about 1 in from the superior corner. There was also some oedema around the left eye— possibly due to obstruction to venous drainage. There was obviously another communicating channel (artery and vein) behind the auricle but since the first operation left part of the helical skin anoxic—it was
FIG. 3 Left Carotid angiography—arterial phase.
FIG. 4 Left Carotid angiography—venous phase.
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D. K. MUKHERJEE AND P. OKEOWO
FIG. 5 Post-operative photograph. It may be noted that the left ear is a little larger in size than the right ear.
decided to postpone the operation of ligation of these until the auricular skin had recovered from its anoxic state. The second operation was performed a month after the first. A huge postauricular artery and vein of 1 in. diameter each were found and ligated with black silk (multiple ligatures). She needed 3 units of blood this time. Even after this operation, the auricular swelling did not subside completely and it was then decided to carry out a carotid ligation. On 3.12.73 she was operated on under G.A. A longitudinal incision along the anterior border of the left sterno-mastoid muscle was given and the great vessels of the neck exposed. A very interesting finding was made at this stage. There was only one carotid artery {internal carotid). No external carotid—branch of the common carotid artery—was found. A thorough search of the area failed to reveal any trace of it. There was one vessel arising from the internal carotid artery coursing upwards and laterally towards the auricle. Since there was no external carotid artery, it was decided to ligate the common carotid artery. This was carried out. She made an uneventful recovery. The swelling over the left auricle subsided completely. She has since then been seen periodically and been doing well (Fig. 5). There has been no further recurrence of the auricular swelling and no episodes of bleeding from the ear.
CLINICAL RECORDS
175
FIG 5a Post-operative photograph of the left auricular area.
Discussion The case described is one of a congenital A.V. fistula involving the auricle. The absence of the external carotid artery can probably be explained by the fact that the superficial temporal tmd occipital branches of it had already been ligated before the carotid system of vessels was exposed in the neck. The facial branch was poorly developed or may have been absent—as already indicated in the carotid angiogram (looking back at it from this angle rather than the suggestion that the poor filling was because of collateral circulation from the other side). It would thus seem to be, in retrospect, that the ascending pharyngeal, the superior thyroid, the lingual and the facial branches of the external carotid artery were congenitally absent or may have atrophied as the major portion of the external carotid blood—flow was going to the gigantic superficial temporal and occipital arteries—and leaking out into the venous system. The vessel going towards the auricular area was an anomalous blood vessel arising from the internal carotid artery. Thus it would appear that there were a few interesting vascular anomalies uncovered in this case. The primary cause would seem to be the congenital A.V. connections involving the superficial temporal and the occipital arteries and veins, leading to gigantic enlargement of these two sets and atrophy of the other vessels of the external carotid system.
176
D. K. MUKHERJEE AND P. OKEOWO
It has been suggested that the best treatment of an arterio-venous fistula involving the ear is either excision of the whole A.V. fistulous mass or amputation of the auricle itself (Dingman and Grabb, 1965). However, this case has shown conclusively that ligation of all the feeding vessels can lead to a very satisfactory result, both functionally and also cosmetically. It is hoped that in future cases of this type will be dealt with by this multiple ligation method rather than the excision of the whole fistulous mass which is a tremendously bloody, difficult and risky procedure. Amputation of the auricle should not be considered until every other avenue of treatment has been explored and found unavailing. Acknowledgement The permission granted by the Director of Administration of Lagos University Teaching Hospital allowing the publication of the case-finding is gratefully acknowledged. The contributions of the Medical Illustration units of Lagos University Teaching Hospital (for the photographs) and the University of Nigeria Teaching Hospital, Enugu (for the angiogram photographs and the clinical illustration) are remembered with thankfulness. REFERENCES DAVIS, L. (1960) Christopher's Textbook of Surgery, 7th ed. Longmans, Philadelphia and London, p, 1358. DINGMAN, R. O., and GRABB, W. C. (1965) Plastic and Reconstructive Surgery, 35, 620. RAMADASS, T. (1967) Journal of Laryngology and Otology, 81, 1171. W.H.O. MEDLINE RESEARCH CENTRE, Geneva (1976) Search No. 2963. Dept. of Otolaryngology, University of Nigeria, Enugu Campus, Anambra State, Nigeria.
