Scandinavian Journal of Thoracic and Cardiovascular Surgery
ISSN: 0036-5580 (Print) (Online) Journal homepage: http://www.tandfonline.com/loi/icdv19
Spontaneously Thrombosing Congenital Crural Arteriovenous Fistulas: Report of a Case Seppo Santavirta, Saara Tötterman & Björn L. Lindström To cite this article: Seppo Santavirta, Saara Tötterman & Björn L. Lindström (1979) Spontaneously Thrombosing Congenital Crural Arteriovenous Fistulas: Report of a Case, Scandinavian Journal of Thoracic and Cardiovascular Surgery, 13:1, 67-69, DOI: 10.3109/14017437909101789 To link to this article: http://dx.doi.org/10.3109/14017437909101789
Published online: 12 Jul 2009.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=icdv19 Download by: [New York University]
Date: 25 March 2016, At: 20:57
Scand J Thor Cardiovasc Surg 13: 67-69, 1979
SPONTANEOUSLY THROMBOSING CONGENITAL CRURAL ARTERIOVENOUS FISTULAS Report of a Case Seppo Santavirta, Saara Totterman and Bjorn L. Lindstrom From the Fourth Department of Surgery, Universitv Ceniral Hospital. Helsinki, Finland
Downloaded by [New York University] at 20:57 25 March 2016
(Submitted for publication January 30, 1978)
Abstract. A report of spontaneously thrombosing arteriovenous fistulas in the popliteal and crural region jeopardizing the peripheral circulation is presented. A favourable effect was achieved with a femoral-posterior tibial bypass utilising a great saphenous graft.
In contrast to traumatic arteriovenous fistulas, the surgical treatment of the congenital fistulas poses many problems, giving rise to puzzles of diagnosis and management (Szilagyi, Elliot, DeRusso & Smith, 1965; Taylor & Wilkinson, 1967; Szilagyi, Smith, Elliot & Hageman, 1976; Vollmar & Stalker, 1976). Nevertheless, progress in vascular surgery in the last 20 years has t o some extent overcome the therapeutic nihilism concerning multiple ar-
teriovenous fistulas in the extremities. Spontaneously thrombosing congenital arteriovenous fistulas are a rare phenomenon with specific therapeutic aspects. A highly unusual case is presented in the following.
CASEREPORT The patient is a 20-year-old male student. At 14 years of age he was admitted to a hospital in his home town because of a persisting necrotic ulcer in the dorsum of his left foot. The previously healthy patient also reported symptoms of intermittent claudication with pain in his left calf after a 100 meter long walk. When examined in August 1970, the foot measured 24 cm in length versus 26 cm on the contralateral side. Both extremities were equally long, but the muscles in the left calf were atrophic. The peripheral pulsations were normal in both legs. Arteriography revealed multiple arteriovenous fistulas below the knee, the most distal one just above the medial malleolus. A long-term anticoagulant medication was attempted and the patient remained under yearly angiographic controls. The follow-up revealed multiple popliteal and crural
aneurysms and gradually a total thrombosis of the popliteal artery. The ulceration healed slowly. Because of persisting handicap, the patient was admitted in May 1975 to the Fourth Department of Surgery, University Central Hospital, Helsinki. By then the left lower extremity showed swelling and increase of skin temperature. The left arterial pulses were not palpable below the knee. Arteriography was performed and revealed thrombosis of the left popliteal artery (Fig. 1). A rich net of collaterals supplied the arterial circulation to the posterior tibial artery. Three of the arteriovenous fistulas were seen in the distal part of the leg. For improvement of the peripheral circulation and to minimize the deleterious effect of the fistulas, a femoralposterior tibial artery bypass operation with an autogenous saphenous graft was performed on May 12, 1975. The positive effect of the operation was immediate and in a check-up 3 months later the patient was free of all discomfort. Angiography revealed a perfect filling from the femoral artery through the saphenous graft into the posterior tibial artery (Fig. 2). The swelling of the leg and the ulcer in the dorsum of the foot had disappeared. Twenty months after the operation, the patient reported practically no discomfort even in athletic exercises. Intermittent claudication was totally cured. The angiogram on January 16, 1977 presented an aneurysm cranial to the femoral anastomosis of the graft, which had been undetected in the operation. The patient will remain under yearly follow-ups.
DISCUSSION The clinical manifestations of congenital arteriovenous fistulas are extremely varied, from hardly noticeable, small birthmarks t o massive clusters of engorged vascular channels that may grotesquely deform an extremity (Johnson, Ghormly & Dockerty, 1956; Robertson, 1956; Heidrich, Paeprer, Naesseri, Meier, Harbst & Marx, 1975; Shepard, 1975). This malformation is more common in the upper extremity than in the lower and the arteriovenous connections are often
Downloaded by [New York University] at 20:57 25 March 2016
68
s. Suntuvirtu
et
(11.
Fig. 1 . The angiogram demonstrates t h e thrombosed popliteal artery and a network of collaterals supplying the circulation below the knee and the flow in the artenovenous fistulas.
Fig. 2. The angiogram two years after femoral-posterior
multiple (Robertson, 1956). Gigantism of the compromised extremity is not usually striking, but in most patients there is a slight increase in the length of the bones in the extremity involved (Taylor & Wilkinson, 1967; Szilagyi, Smith, Elliot & Hageman, 1976). The patient described above showed muscle atrophy of the diseased extremity. The increased output through the shunts is reported to cause cardiac decompensation in some cases (Taylor & Wilkinson, 1967). The present patient did not show any systemic signs of the arteriovenous shunts of haemodynamic significance.
The vast majority of patients d o well on a carefully supervised conservative regimen, with surgical intervention reserved for individually defined instances (Szilagyi, Smith, Elliot & Hageman, 1976). In congenital arteriovenous fistulas, the results of ligation and excision are unsatisfactory in most cases and with few exceptions their radical cure by surgical means is not possible (Taylor & Wilkinson, 1967). In the present case, the situation was abnormal, as the crural arteriovenous fistulas gradually thrombosed spontaneously. The philosophy was to
tibia1 bypass reveals a good function of the bypass. However, a small aneurysm can be noticed in the superficial femoral artery close the anastomosis. Thrombosis of the popliteal artery has not progressed.
Downloaded by [New York University] at 20:57 25 March 2016
Coi?gmituI arteriovenms fistulas
69
Fig. 4 . A schematic diagram demonstrating the situation after bypass operation. A=femoral artery. B=the saphenous graft. C=one of the arteriovenous fistulas. D= collateral arteries.
of progressive arterial thrombosis in the lower extremity. Fig. 3. The bypass supplies the circulation of the distal part of the leg well.
REFERENCES
attempt an improvement of the peripheral circulation, after the progressing thrombosis had eliminated some proximal fistulas of the leg. This was achieved with a femoral-posterior tibia1 bypass operation utilising a saphenous graft. According to the original plan, the former ulcer area on the dorsum of the left foot and the angiomatous skin surrounding it would have been excised at a second operation and covered with a skin graft. It was hoped to achieve thrombosis of the rest of the fistulas by changing the arterial supply now coming from the distal direction. However, the postoperative course was extremely favourable. Most of the remaining fistulas thrombosed. The ulcer and the other clinical manifestations disappeared. This method should be considered as the surgical treatment of the arteriovenous fistulas with the abnormal situation
Heidrich, v. H.. Paeprer, H . , Naesseri, M . , Meier. J.. Harbst, H . & Marx, H. 1975. Arteriovenose, aneurismatische Fistel der A. intercostalis-V. subclavia bei multiplen Arterien-Aneurysmen. Fortschr Rontgenstr 121, 45. Johnson, E. W., Ghormly, R. K . & Dockerty, M. B. 1956. Hemangiomas of the extremities. Surg Gynec Obstet 102,531. Robertson, D. J. 1956. Congenital arteriovenous fistulae of the extremities. Ann Roy Coll Surg E n g l 5 2 , 73. Shepard, D. H. 1975. Cirsoid arteriovenous malformations of the scalp. J Neurol Neurosurg Psych 38,827. Szilagyi, D., Elliot, J., DeRusso, F. & Smith, R. 1965. Peripheral congenital arteriovenous fistulas. Surgery 57, 61. Szilagyi, D., Smith, R., Elliot, J. & Hageman, J. 1976. Arch Surg 1 1 1 , 4 2 3 . Taylor, G . W. & Wilkinson, J. F. 1967. Congenital arteriovenous fistula. Clinical Surgery. Vascular Surgery and Reficulo-Endothelid System. p. 152. Butterworths, London. Vollmar, J. F. & Stalker. C. G. 1976. The surgical treatment of congenital arteriovenous fistulas in the extremities. J Crrrdiovasr SurA> 17, 340.
ISSN: 0036-5580 (Print) (Online) Journal homepage: http://www.tandfonline.com/loi/icdv19
Spontaneously Thrombosing Congenital Crural Arteriovenous Fistulas: Report of a Case Seppo Santavirta, Saara Tötterman & Björn L. Lindström To cite this article: Seppo Santavirta, Saara Tötterman & Björn L. Lindström (1979) Spontaneously Thrombosing Congenital Crural Arteriovenous Fistulas: Report of a Case, Scandinavian Journal of Thoracic and Cardiovascular Surgery, 13:1, 67-69, DOI: 10.3109/14017437909101789 To link to this article: http://dx.doi.org/10.3109/14017437909101789
Published online: 12 Jul 2009.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=icdv19 Download by: [New York University]
Date: 25 March 2016, At: 20:57
Scand J Thor Cardiovasc Surg 13: 67-69, 1979
SPONTANEOUSLY THROMBOSING CONGENITAL CRURAL ARTERIOVENOUS FISTULAS Report of a Case Seppo Santavirta, Saara Totterman and Bjorn L. Lindstrom From the Fourth Department of Surgery, Universitv Ceniral Hospital. Helsinki, Finland
Downloaded by [New York University] at 20:57 25 March 2016
(Submitted for publication January 30, 1978)
Abstract. A report of spontaneously thrombosing arteriovenous fistulas in the popliteal and crural region jeopardizing the peripheral circulation is presented. A favourable effect was achieved with a femoral-posterior tibial bypass utilising a great saphenous graft.
In contrast to traumatic arteriovenous fistulas, the surgical treatment of the congenital fistulas poses many problems, giving rise to puzzles of diagnosis and management (Szilagyi, Elliot, DeRusso & Smith, 1965; Taylor & Wilkinson, 1967; Szilagyi, Smith, Elliot & Hageman, 1976; Vollmar & Stalker, 1976). Nevertheless, progress in vascular surgery in the last 20 years has t o some extent overcome the therapeutic nihilism concerning multiple ar-
teriovenous fistulas in the extremities. Spontaneously thrombosing congenital arteriovenous fistulas are a rare phenomenon with specific therapeutic aspects. A highly unusual case is presented in the following.
CASEREPORT The patient is a 20-year-old male student. At 14 years of age he was admitted to a hospital in his home town because of a persisting necrotic ulcer in the dorsum of his left foot. The previously healthy patient also reported symptoms of intermittent claudication with pain in his left calf after a 100 meter long walk. When examined in August 1970, the foot measured 24 cm in length versus 26 cm on the contralateral side. Both extremities were equally long, but the muscles in the left calf were atrophic. The peripheral pulsations were normal in both legs. Arteriography revealed multiple arteriovenous fistulas below the knee, the most distal one just above the medial malleolus. A long-term anticoagulant medication was attempted and the patient remained under yearly angiographic controls. The follow-up revealed multiple popliteal and crural
aneurysms and gradually a total thrombosis of the popliteal artery. The ulceration healed slowly. Because of persisting handicap, the patient was admitted in May 1975 to the Fourth Department of Surgery, University Central Hospital, Helsinki. By then the left lower extremity showed swelling and increase of skin temperature. The left arterial pulses were not palpable below the knee. Arteriography was performed and revealed thrombosis of the left popliteal artery (Fig. 1). A rich net of collaterals supplied the arterial circulation to the posterior tibial artery. Three of the arteriovenous fistulas were seen in the distal part of the leg. For improvement of the peripheral circulation and to minimize the deleterious effect of the fistulas, a femoralposterior tibial artery bypass operation with an autogenous saphenous graft was performed on May 12, 1975. The positive effect of the operation was immediate and in a check-up 3 months later the patient was free of all discomfort. Angiography revealed a perfect filling from the femoral artery through the saphenous graft into the posterior tibial artery (Fig. 2). The swelling of the leg and the ulcer in the dorsum of the foot had disappeared. Twenty months after the operation, the patient reported practically no discomfort even in athletic exercises. Intermittent claudication was totally cured. The angiogram on January 16, 1977 presented an aneurysm cranial to the femoral anastomosis of the graft, which had been undetected in the operation. The patient will remain under yearly follow-ups.
DISCUSSION The clinical manifestations of congenital arteriovenous fistulas are extremely varied, from hardly noticeable, small birthmarks t o massive clusters of engorged vascular channels that may grotesquely deform an extremity (Johnson, Ghormly & Dockerty, 1956; Robertson, 1956; Heidrich, Paeprer, Naesseri, Meier, Harbst & Marx, 1975; Shepard, 1975). This malformation is more common in the upper extremity than in the lower and the arteriovenous connections are often
Downloaded by [New York University] at 20:57 25 March 2016
68
s. Suntuvirtu
et
(11.
Fig. 1 . The angiogram demonstrates t h e thrombosed popliteal artery and a network of collaterals supplying the circulation below the knee and the flow in the artenovenous fistulas.
Fig. 2. The angiogram two years after femoral-posterior
multiple (Robertson, 1956). Gigantism of the compromised extremity is not usually striking, but in most patients there is a slight increase in the length of the bones in the extremity involved (Taylor & Wilkinson, 1967; Szilagyi, Smith, Elliot & Hageman, 1976). The patient described above showed muscle atrophy of the diseased extremity. The increased output through the shunts is reported to cause cardiac decompensation in some cases (Taylor & Wilkinson, 1967). The present patient did not show any systemic signs of the arteriovenous shunts of haemodynamic significance.
The vast majority of patients d o well on a carefully supervised conservative regimen, with surgical intervention reserved for individually defined instances (Szilagyi, Smith, Elliot & Hageman, 1976). In congenital arteriovenous fistulas, the results of ligation and excision are unsatisfactory in most cases and with few exceptions their radical cure by surgical means is not possible (Taylor & Wilkinson, 1967). In the present case, the situation was abnormal, as the crural arteriovenous fistulas gradually thrombosed spontaneously. The philosophy was to
tibia1 bypass reveals a good function of the bypass. However, a small aneurysm can be noticed in the superficial femoral artery close the anastomosis. Thrombosis of the popliteal artery has not progressed.
Downloaded by [New York University] at 20:57 25 March 2016
Coi?gmituI arteriovenms fistulas
69
Fig. 4 . A schematic diagram demonstrating the situation after bypass operation. A=femoral artery. B=the saphenous graft. C=one of the arteriovenous fistulas. D= collateral arteries.
of progressive arterial thrombosis in the lower extremity. Fig. 3. The bypass supplies the circulation of the distal part of the leg well.
REFERENCES
attempt an improvement of the peripheral circulation, after the progressing thrombosis had eliminated some proximal fistulas of the leg. This was achieved with a femoral-posterior tibia1 bypass operation utilising a saphenous graft. According to the original plan, the former ulcer area on the dorsum of the left foot and the angiomatous skin surrounding it would have been excised at a second operation and covered with a skin graft. It was hoped to achieve thrombosis of the rest of the fistulas by changing the arterial supply now coming from the distal direction. However, the postoperative course was extremely favourable. Most of the remaining fistulas thrombosed. The ulcer and the other clinical manifestations disappeared. This method should be considered as the surgical treatment of the arteriovenous fistulas with the abnormal situation
Heidrich, v. H.. Paeprer, H . , Naesseri, M . , Meier. J.. Harbst, H . & Marx, H. 1975. Arteriovenose, aneurismatische Fistel der A. intercostalis-V. subclavia bei multiplen Arterien-Aneurysmen. Fortschr Rontgenstr 121, 45. Johnson, E. W., Ghormly, R. K . & Dockerty, M. B. 1956. Hemangiomas of the extremities. Surg Gynec Obstet 102,531. Robertson, D. J. 1956. Congenital arteriovenous fistulae of the extremities. Ann Roy Coll Surg E n g l 5 2 , 73. Shepard, D. H. 1975. Cirsoid arteriovenous malformations of the scalp. J Neurol Neurosurg Psych 38,827. Szilagyi, D., Elliot, J., DeRusso, F. & Smith, R. 1965. Peripheral congenital arteriovenous fistulas. Surgery 57, 61. Szilagyi, D., Smith, R., Elliot, J. & Hageman, J. 1976. Arch Surg 1 1 1 , 4 2 3 . Taylor, G . W. & Wilkinson, J. F. 1967. Congenital arteriovenous fistula. Clinical Surgery. Vascular Surgery and Reficulo-Endothelid System. p. 152. Butterworths, London. Vollmar, J. F. & Stalker. C. G. 1976. The surgical treatment of congenital arteriovenous fistulas in the extremities. J Crrrdiovasr SurA> 17, 340.
![[Multiple congenital arteriovenous fistulas. Case report concerning a late manifestation].](/assets/img/hold.png)
