Congenital arteriovenous fistulas of the hand Richard H. Gelberman, M.D., San Diego, Calif., and J. Leonard Goldner, M.D., Durham, N. C.
T here is little agreement regarding the management of the congenital arteriovenous fistula of the hand. Since a congenital arteriovenous fistula of the hand was first surgically ligated in 1867, I authors have consistently recorded frustration in regard to its operative management. Excision in any but the most sharply delineated fistulas often has been unsuccessful and has been associated with serious complications. 2- 6 The trauma of operation reportedly has caused the lesion to progress with locally invasive characteristics similar to malignancy. 6 A process apparently localized to a single digit may extend proximally to involve the forearm or arm. Reports of the treatment of congenital arteriovenous fistulas usually describe multiple operations with repeated skin sloughs, gradual proximal extension, digital ischemia, and with occasional loss of the digit. The purpose of this paper is to review the operative treatment of the congenital arteriovenous fistula, to report four previously unrecorded cases, and to suggest some diagnostic studies which may help to differentiate those digits amenable to operative excision of the fistulous area from those best treated conservatively or by amputation of the digit.
Review of the literature Seventy operations in 37 patients with congenital arteriovenous fistulas of the hand have been recorded. Kittredge, Kanick, and Finby, 7 Curtis,3 Szilagyi et al., 8 Lewis,4 and Pemberton and Saint9 described extension of the fistulous mass after attempted excision. Others have reported proliferation and proximal extension with worsening of symptoms following ligation of the feeding artery. 2-6, 8, 10 Still others have described poor results after amputation in patients with diffuse fistulas involving more than one digit. 3, 7, 9-11 Three basic operations have been described for arFrom the Duke University Medical Center, Durham, N. C. Received for publication May 24, 1977.
teriovenous fistulas in the hand: excision of the fistula in one or two stages, ligation of the feeding arteries or communicating branches, and amputation. Twenty-eight attempts at excision have been reported. Only 12 of the 28 one- or two-stage excisions were successful; 16 had persistent or increased symptoms. 3, 4, 9, 11-14 Three of the procedures were followed by gangrene of the digit. One attempted excision was followed by injection of a sclerosing agent,12 but that procedure also was unsuccessful. Coursley, Ivins, and Barker l3 reported on 18 upper extremity arteriovenous fistulas treated by surgical excision. Although the reports were not sufficiently detailed to include in this review, their discussion is pertinent. Of 18 localized fistulas in the localized upper extremity, 12 were treated by excision, with good results in seven, fair results in one, and recurrence in four. Of 35 diffuse fistulas, excision was attempted in six with recurrence in five. Amputation was necessary in two. The danger following excision of diffuse fistulas is the probability of rapid recurrence and extension of the lesion. Twenty-eight amputations have been performed in 18 patients. 2- 4, 5,7,9-11 Nine of 19 finger or ray amputations were curative. The others resulted in persistent or worse symptoms with proximal proliferation of the mass. Nine of nine hand, forearm, and arm amputations were curative in eliminating the fistulas. Again, the differentiating factor appeared to be the diffuseness of the lesion. Ifthe lesion was amputated en masse, the procedure usually was curative, even if a dilated proximal feeding vessel was crossed. If fistulous material was crossed, however, proximal extension and worsening of symptoms were the rule. Fourteen patients had ligation of the feeding artery, proximal ligation and excision, or ligation of communicating branches as the sole procedure. I, 4, 7, 8,11 In only one was the procedure curative. Proximal vessel ligation now is considered to be an ineffective method of controlling the lesion.
Revised for publication Oct. 12, 1977.
Material and results
Reprint requests: J. Leonard Goldner, M.D., Division of Orthopaedic Surgery, Duke University Medical Center, Durham, NC 27710.
A review of the records of Duke University Medical Center for the past 15 years disclosed four patients with
0363·5023178/0503·0451 $00.40/0 © 1978 American Society for Surgery of the Hand
THE JOURNAL OF HAND SURGERY
451
452
The Journal of
Gelberman and Goldner
HAND SURGERY
Fig. 2. Preoperative brachial arteriogram. Fig. 1. Brachial arteriogram demonstrating the fistula 4 years prior to operation. congenital arteriovenous fistulas of the hand . Table I summarizes the clinical features for each of these patients. The duration of clinical symptoms and signs prior to surgical treatment ranged from I to II years and averaged 6 years. In all four patients, the presence of the arteriovenous fistula resulted in a mildly painful enlargement of the digit. In one patient the natural progress of the tumor was demonstrated by arteriograms over a 4-year period . (Figs. I and 2). Xeroradiograms demonstrated osseous abnormalities prior to operation in one patient . Arteriograms were performed before operation on three patients . One patient had a number of diagnostic studies which we feel helped to determine the precise involvement of the hand. The operative management of the patients varied with the extent of the lesion (Table II). Of four of our patients with arteriovenous fistulas, one was cured by amputation. One well localized fistula was excised successfully with preservation of the central feeding arteries to the digit. In the other two patients a staged excision was done. A total of six procedures with ultimate partial amputation in one patient was necessary before satisfactory control of the fistulas was achieved (Table II). Of the three patients with digital lesions, one
was treated by amputation and two had staged resections. One of these ultimately had an amputation through the middle phalanx. The other had a third procedure for recurrent fistula and achieved a satisfactory result but with an enlarged asymptomatic digit.
Discussion Less than half of the surgical procedures reviewed (29 of 70), including those in our own four cases, have resulted in a long-term decrease in symptoms. Several observations regarding the indications for and technique of excision can be made from this experience . Fistulas with bony involvement have been poorly controlled by excision, and recurrence has been inevitable. 6 • 15 Excision of the entire fistulous mass with maintenance of main channel continuity has been essential. If one main vessel is not preserved and collaterals are inadequate, ischemia of the digit usually occurs. The most accurate predictor of the results of excision has been the diffuseness of the mass. Those lesions which were well localized with single feeding vessels without bony involvement have responded well to excision. The diffuse digital mass with extensive involvement of the palm and adjacent digits have responded poorly. The preoperative evaluation of the patient with a congenital arteriovenous fistula of the hand must provide some specific information if a successful surgical procedure is to be carried out. Attempts at excision
Vol. 3, No . 5 September, 1978
Congenital arteriovenous fistulas of hand
453
Table I. Congenital arteriovenous fistulas
C)
II
F
CR
33
F
DH
59 13
F F
ST
Duration of symptoms (yr)
Clinical features
Patient
9
Progressive pain and swelling, dilated superficial veins, enlarged digit Pain and swelling; prior attempted excision with rapid recurrence Painful palmar mass Pain and swelling, enlarged digit
are not warranted in the presence of intraosseous extension, where involvement is best demonstrated by roentgenography or xeroradiography. The diffuseness of the mass, the involvement of adjacent digits, and the degree of proximal extension are demonstrated best by arteriography and thermography. Alterations in skin blood flow also have been demonstrated in patients with extensive congenital arteriovenous fistulas studied with strain gauge plethysmography.15-17 Flow values have been related to the extent of the fistula. Skin flow measurements also have shown a decrease in areas distal to the fistula and correlate with the severity of shunting. We have been measuring pulse volume flow and skin temperatures to heIp to determine the extent of decrease in distal blood flow . Some information about the diffuseness of the lesion and the likelihood of successful excision without subsequent ischemic changes can be obtained. Our efforts have been to establish a treatment protocol that will reasonably predict the response to operation. We feel that one should use all available diagnostic means to determine the exact anatomic extent of the fistula; that is, involvement of the adjacent digits and bone, proximal extension, and amount of physiological decrease in distal blood supply, before considering surgical excIsIon. Evaluation should include a roentgenogram, xeroradiogram , pulse volume flow recording, skin temperature recording, and brachial arteriogram. If the preoperative evaluation demonstrates a localized fistula without osseous involvement, with discrete feeding vessels, and with adequate distal digital vascularity, excision in one or two stages, using the pulse transducer in the operating room, can be undertaken. ls If single-channel continuity cannot be maintained during the procedure, a vein graft, similar to that described by Leonard and Vassosl 9 for lower extremity fistulas, can be used. If there is intraosseous involvement or significant decreased distal blood flow, amputation of a digit or ray, along with excision of any adjacent mass is the procedure of choice. Patients with
4
II
Table II. Congenital arteriovenous fistulas Patient
Treatment
C)
Staged resection with subsequent partial amputation Staged resection with subsequent surgery for residual tumor One-stage excision Primary ray deletion
CR DH ST
diffuse fistulas with involvement of more than one digit, and with proximal vessel dilatation, should be treated by operation only as a last resort. Proximal vessel ligation is not recommended. REFERENCES I . Gherini: Societe Imperial de Chirurgie (abstract) . Gaz Hop Civ Milit 40:303 , 1867 2. Bogumill GP: Clinico-pathological correlation in a case of congenital arterio-venous fistula . Hand 9:60-64, 1977 3. Curtis RM: Congenital arteriovenous fistulae of the hand. J Bone Joint Surg 35-A:917-928, 1953 4. Lewis DW: Congenital arteriovenous fistulae. Lancet 2:621, 1930 5 . Neviaser RJ, Adams JP: Vascular lesions in the hand. Current management. Clin Orthop 100:111-119, 1974 6. Weinberg M Jr, Steiger Z, Fell EH: Unusual congenital anomalies of the arteriovenous system . Surg Clin North Am 40:67-74, 1960 7. Kittredge RD, Kanick V, Finby N: Arteriovenous fistulas. Am J Roentgenol Radium Ther Nuel Med 100:431-445, 1967 8. Szilagyi DE, Elliott JP, DeRusso FJ, et al: Peripheral congenital arteriovenous fistulas. Surgery 57:61-81, 1965 9. Pemberton J, Saint JH: Congenital arteriovenous communications. Surg Gynecol Obstet 46:470-483, 1928 10. Greenhalgh RM, Rosengarten DS, Calnan JS: A single congenital arteriovenous fistula of the hand . Br J Surg 59:76-78 , 1972 II. Cross FS, Glover DM, Simeone FA, et al: Congenital arteriovenous aneurysms. Ann Surg 148:649-665, 1958 12 . Bertelsen A, Dohn D: Congenital arteriovenous com-
454
13.
14.
15. 16.
Gelberman and Goldner
munications of extremities. Acta Chir Scand 105:448459, 1953 Coursley G, Ivins JC, Barker NW: Congenital arteriovenous fistulas in extremities: Analysis of 69 cases. Angiology 7:201-217, 1956 Horton BT, Ghormley RK: Congenital arteriovenous fistulas of extremities visualized by arteriography. Surg Gynecol Obstet 60:978-983, 1935 Nisbet NW: Congenital arteriovenous fistula in the extremities. Br J Surg 41:658, 1953 Wakim KG, Janes JM: Influence of arteriovenous fistula
The Journal of HAND SURGERY
on the distal circulation in the involved extremity. Arch Physiol Med 39:431, 1958 17. Yao ST, Needham TN, Lewis JB, et al: Limb blood flow in congenital arteriovenous fistula. Surgery 73:80-84, 1973 18. Bingham HG, Lichti E: Use of ultrasound transducer (Doppler) to localize peripheral and arteriovenous fistulae. Plast Reconstr Surg 46:151-154, 1970 19. Leonard FC, Vassos GA Jr: Congenital arteriovenous fistulation of the lower limb. N Engl J Med 245:885, 1951
INFORMATION FOR AUTHORS Most of the provisions of the Copyright Act of 1976 became effective on January 1, 1978. Therefore, all transmittal letters must be accompanied by the following statement, signed by each author: "The undersigned author(s) transfers all copyright ownership of the manuscript entitled (title of article) to the American Society for Surgery of the Hand in the event the work is published. The author(s) warrants that the article is original, is not under consideration by another journal, and has not been previously published." Authors will be consulted, when possible, regarding republication of their material.
T here is little agreement regarding the management of the congenital arteriovenous fistula of the hand. Since a congenital arteriovenous fistula of the hand was first surgically ligated in 1867, I authors have consistently recorded frustration in regard to its operative management. Excision in any but the most sharply delineated fistulas often has been unsuccessful and has been associated with serious complications. 2- 6 The trauma of operation reportedly has caused the lesion to progress with locally invasive characteristics similar to malignancy. 6 A process apparently localized to a single digit may extend proximally to involve the forearm or arm. Reports of the treatment of congenital arteriovenous fistulas usually describe multiple operations with repeated skin sloughs, gradual proximal extension, digital ischemia, and with occasional loss of the digit. The purpose of this paper is to review the operative treatment of the congenital arteriovenous fistula, to report four previously unrecorded cases, and to suggest some diagnostic studies which may help to differentiate those digits amenable to operative excision of the fistulous area from those best treated conservatively or by amputation of the digit.
Review of the literature Seventy operations in 37 patients with congenital arteriovenous fistulas of the hand have been recorded. Kittredge, Kanick, and Finby, 7 Curtis,3 Szilagyi et al., 8 Lewis,4 and Pemberton and Saint9 described extension of the fistulous mass after attempted excision. Others have reported proliferation and proximal extension with worsening of symptoms following ligation of the feeding artery. 2-6, 8, 10 Still others have described poor results after amputation in patients with diffuse fistulas involving more than one digit. 3, 7, 9-11 Three basic operations have been described for arFrom the Duke University Medical Center, Durham, N. C. Received for publication May 24, 1977.
teriovenous fistulas in the hand: excision of the fistula in one or two stages, ligation of the feeding arteries or communicating branches, and amputation. Twenty-eight attempts at excision have been reported. Only 12 of the 28 one- or two-stage excisions were successful; 16 had persistent or increased symptoms. 3, 4, 9, 11-14 Three of the procedures were followed by gangrene of the digit. One attempted excision was followed by injection of a sclerosing agent,12 but that procedure also was unsuccessful. Coursley, Ivins, and Barker l3 reported on 18 upper extremity arteriovenous fistulas treated by surgical excision. Although the reports were not sufficiently detailed to include in this review, their discussion is pertinent. Of 18 localized fistulas in the localized upper extremity, 12 were treated by excision, with good results in seven, fair results in one, and recurrence in four. Of 35 diffuse fistulas, excision was attempted in six with recurrence in five. Amputation was necessary in two. The danger following excision of diffuse fistulas is the probability of rapid recurrence and extension of the lesion. Twenty-eight amputations have been performed in 18 patients. 2- 4, 5,7,9-11 Nine of 19 finger or ray amputations were curative. The others resulted in persistent or worse symptoms with proximal proliferation of the mass. Nine of nine hand, forearm, and arm amputations were curative in eliminating the fistulas. Again, the differentiating factor appeared to be the diffuseness of the lesion. Ifthe lesion was amputated en masse, the procedure usually was curative, even if a dilated proximal feeding vessel was crossed. If fistulous material was crossed, however, proximal extension and worsening of symptoms were the rule. Fourteen patients had ligation of the feeding artery, proximal ligation and excision, or ligation of communicating branches as the sole procedure. I, 4, 7, 8,11 In only one was the procedure curative. Proximal vessel ligation now is considered to be an ineffective method of controlling the lesion.
Revised for publication Oct. 12, 1977.
Material and results
Reprint requests: J. Leonard Goldner, M.D., Division of Orthopaedic Surgery, Duke University Medical Center, Durham, NC 27710.
A review of the records of Duke University Medical Center for the past 15 years disclosed four patients with
0363·5023178/0503·0451 $00.40/0 © 1978 American Society for Surgery of the Hand
THE JOURNAL OF HAND SURGERY
451
452
The Journal of
Gelberman and Goldner
HAND SURGERY
Fig. 2. Preoperative brachial arteriogram. Fig. 1. Brachial arteriogram demonstrating the fistula 4 years prior to operation. congenital arteriovenous fistulas of the hand . Table I summarizes the clinical features for each of these patients. The duration of clinical symptoms and signs prior to surgical treatment ranged from I to II years and averaged 6 years. In all four patients, the presence of the arteriovenous fistula resulted in a mildly painful enlargement of the digit. In one patient the natural progress of the tumor was demonstrated by arteriograms over a 4-year period . (Figs. I and 2). Xeroradiograms demonstrated osseous abnormalities prior to operation in one patient . Arteriograms were performed before operation on three patients . One patient had a number of diagnostic studies which we feel helped to determine the precise involvement of the hand. The operative management of the patients varied with the extent of the lesion (Table II). Of four of our patients with arteriovenous fistulas, one was cured by amputation. One well localized fistula was excised successfully with preservation of the central feeding arteries to the digit. In the other two patients a staged excision was done. A total of six procedures with ultimate partial amputation in one patient was necessary before satisfactory control of the fistulas was achieved (Table II). Of the three patients with digital lesions, one
was treated by amputation and two had staged resections. One of these ultimately had an amputation through the middle phalanx. The other had a third procedure for recurrent fistula and achieved a satisfactory result but with an enlarged asymptomatic digit.
Discussion Less than half of the surgical procedures reviewed (29 of 70), including those in our own four cases, have resulted in a long-term decrease in symptoms. Several observations regarding the indications for and technique of excision can be made from this experience . Fistulas with bony involvement have been poorly controlled by excision, and recurrence has been inevitable. 6 • 15 Excision of the entire fistulous mass with maintenance of main channel continuity has been essential. If one main vessel is not preserved and collaterals are inadequate, ischemia of the digit usually occurs. The most accurate predictor of the results of excision has been the diffuseness of the mass. Those lesions which were well localized with single feeding vessels without bony involvement have responded well to excision. The diffuse digital mass with extensive involvement of the palm and adjacent digits have responded poorly. The preoperative evaluation of the patient with a congenital arteriovenous fistula of the hand must provide some specific information if a successful surgical procedure is to be carried out. Attempts at excision
Vol. 3, No . 5 September, 1978
Congenital arteriovenous fistulas of hand
453
Table I. Congenital arteriovenous fistulas
C)
II
F
CR
33
F
DH
59 13
F F
ST
Duration of symptoms (yr)
Clinical features
Patient
9
Progressive pain and swelling, dilated superficial veins, enlarged digit Pain and swelling; prior attempted excision with rapid recurrence Painful palmar mass Pain and swelling, enlarged digit
are not warranted in the presence of intraosseous extension, where involvement is best demonstrated by roentgenography or xeroradiography. The diffuseness of the mass, the involvement of adjacent digits, and the degree of proximal extension are demonstrated best by arteriography and thermography. Alterations in skin blood flow also have been demonstrated in patients with extensive congenital arteriovenous fistulas studied with strain gauge plethysmography.15-17 Flow values have been related to the extent of the fistula. Skin flow measurements also have shown a decrease in areas distal to the fistula and correlate with the severity of shunting. We have been measuring pulse volume flow and skin temperatures to heIp to determine the extent of decrease in distal blood flow . Some information about the diffuseness of the lesion and the likelihood of successful excision without subsequent ischemic changes can be obtained. Our efforts have been to establish a treatment protocol that will reasonably predict the response to operation. We feel that one should use all available diagnostic means to determine the exact anatomic extent of the fistula; that is, involvement of the adjacent digits and bone, proximal extension, and amount of physiological decrease in distal blood supply, before considering surgical excIsIon. Evaluation should include a roentgenogram, xeroradiogram , pulse volume flow recording, skin temperature recording, and brachial arteriogram. If the preoperative evaluation demonstrates a localized fistula without osseous involvement, with discrete feeding vessels, and with adequate distal digital vascularity, excision in one or two stages, using the pulse transducer in the operating room, can be undertaken. ls If single-channel continuity cannot be maintained during the procedure, a vein graft, similar to that described by Leonard and Vassosl 9 for lower extremity fistulas, can be used. If there is intraosseous involvement or significant decreased distal blood flow, amputation of a digit or ray, along with excision of any adjacent mass is the procedure of choice. Patients with
4
II
Table II. Congenital arteriovenous fistulas Patient
Treatment
C)
Staged resection with subsequent partial amputation Staged resection with subsequent surgery for residual tumor One-stage excision Primary ray deletion
CR DH ST
diffuse fistulas with involvement of more than one digit, and with proximal vessel dilatation, should be treated by operation only as a last resort. Proximal vessel ligation is not recommended. REFERENCES I . Gherini: Societe Imperial de Chirurgie (abstract) . Gaz Hop Civ Milit 40:303 , 1867 2. Bogumill GP: Clinico-pathological correlation in a case of congenital arterio-venous fistula . Hand 9:60-64, 1977 3. Curtis RM: Congenital arteriovenous fistulae of the hand. J Bone Joint Surg 35-A:917-928, 1953 4. Lewis DW: Congenital arteriovenous fistulae. Lancet 2:621, 1930 5 . Neviaser RJ, Adams JP: Vascular lesions in the hand. Current management. Clin Orthop 100:111-119, 1974 6. Weinberg M Jr, Steiger Z, Fell EH: Unusual congenital anomalies of the arteriovenous system . Surg Clin North Am 40:67-74, 1960 7. Kittredge RD, Kanick V, Finby N: Arteriovenous fistulas. Am J Roentgenol Radium Ther Nuel Med 100:431-445, 1967 8. Szilagyi DE, Elliott JP, DeRusso FJ, et al: Peripheral congenital arteriovenous fistulas. Surgery 57:61-81, 1965 9. Pemberton J, Saint JH: Congenital arteriovenous communications. Surg Gynecol Obstet 46:470-483, 1928 10. Greenhalgh RM, Rosengarten DS, Calnan JS: A single congenital arteriovenous fistula of the hand . Br J Surg 59:76-78 , 1972 II. Cross FS, Glover DM, Simeone FA, et al: Congenital arteriovenous aneurysms. Ann Surg 148:649-665, 1958 12 . Bertelsen A, Dohn D: Congenital arteriovenous com-
454
13.
14.
15. 16.
Gelberman and Goldner
munications of extremities. Acta Chir Scand 105:448459, 1953 Coursley G, Ivins JC, Barker NW: Congenital arteriovenous fistulas in extremities: Analysis of 69 cases. Angiology 7:201-217, 1956 Horton BT, Ghormley RK: Congenital arteriovenous fistulas of extremities visualized by arteriography. Surg Gynecol Obstet 60:978-983, 1935 Nisbet NW: Congenital arteriovenous fistula in the extremities. Br J Surg 41:658, 1953 Wakim KG, Janes JM: Influence of arteriovenous fistula
The Journal of HAND SURGERY
on the distal circulation in the involved extremity. Arch Physiol Med 39:431, 1958 17. Yao ST, Needham TN, Lewis JB, et al: Limb blood flow in congenital arteriovenous fistula. Surgery 73:80-84, 1973 18. Bingham HG, Lichti E: Use of ultrasound transducer (Doppler) to localize peripheral and arteriovenous fistulae. Plast Reconstr Surg 46:151-154, 1970 19. Leonard FC, Vassos GA Jr: Congenital arteriovenous fistulation of the lower limb. N Engl J Med 245:885, 1951
INFORMATION FOR AUTHORS Most of the provisions of the Copyright Act of 1976 became effective on January 1, 1978. Therefore, all transmittal letters must be accompanied by the following statement, signed by each author: "The undersigned author(s) transfers all copyright ownership of the manuscript entitled (title of article) to the American Society for Surgery of the Hand in the event the work is published. The author(s) warrants that the article is original, is not under consideration by another journal, and has not been previously published." Authors will be consulted, when possible, regarding republication of their material.
