Congenital Arteriovenous Fistula in the Gluteal Region --A Report of Five Cases-Shunya SHINDO,Yusuke TADA,Motoaki SHIRAKAWA,Yutaka TAKAYAMA, Tetsurou MIYATA,Osamu SATO,Atsuhiko TAKAGIand Yasuo IDEZUKI ABSTRACT: Five cases o f congenital arteriovenous fistula in the gluteal region have been encountered in our department in the past 20 years. In all cases, the fistulOusmasses were in the connective tissue between the gluteal muscles and well'localized. Preoperative angiography showed the feeding arteries to be the Superior gluteal, the inferior gluteal, a n d / o r the lateral femoral circumflex arteries, and all the arteriovenous fistulae were excised almost completely with success. In this report, we emphasize the importance of precise estimation o f the feeding arteries on preoperative angiography and ligating them before excising the fistulous masses, to ensure safe surgical treatment. KEY WORDS: gluteal region

vascular surgery, arteriovenous fistula, feeding artery,

INTRODUCTION

operative complications. These five cases are outlined in this report.

The congenital

arteriovenous fistula (A-V fistula) remains one o f the most difficult conditions to treat surgically, and a diftuse one is especially difficult to treat. Welllocalized malformations, however, may be managed more safely. Gluteal A-V fistulae are rarely reported, but as they are localized in the buttocks in place o f a diffuse condition, can be operated on. We have experienced five cases of primary A-V fistula in the buttocks in the past 20 years. All the fistulous masses were well localized and occurred in the connective tissue between the muscles so were able to be excised without any post-

The Second Department of Surgery, The Faculty of Medicine, Universityof Tokyo, Tokyo,Japan Reprint requests to: Shunya Shindo, MD, The SecondDepartment of Surgery, The Faculty of Medicine, Universityof Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo113,Japan

CASES Case 1: A 28 year old woman was seen in 1964 complaining o f swelling and pain in her right buttock. She had noticed an asymmetrical appearance o f her buttocks for several years (Fig. 1). O n physical examination, a pulsating mass was seen in her gluteal region, and subsequent angiography confirmed the diagnosis o f a gluteal A-V fistula and established the superior gluteal artery as a feeding artery. At surgery, the feeding artery was first ligated, then the A-V fistula excised together with part o f the right gluteus maximus muscle. H e r postoperative course was uneventful and no functional distress occurred, even though her right gluteus maximus muscle had b e e n partially resected. Case 2: A 33 year old woman was seen in our department in 1966 complaining of

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Shindo et al.

Fig. 1. Preoperative photograph of case 1. The right buttock was larger than the left and physical examination revealed a pulsating mass beneath it. pain in h e r left leg. She h a d noticed a swelling in h e r left buttock since childhood. O n admission, she h a d a spongelike mass with systolic bruit in her left buttock, and subsequent angiogram revealed an arteriovenous angioma, whose feeders were the superior gluteal artery a n d the lateral femoral circumflex artery (Fig. 2). T h e drainage vein was the superior gluteal vein. At surgery, both feeding arteries were ligated extraperitoneally, a n d the gluteal A-V fistula removed. O n the first postoperative day, the pulsation of the left dorsal pedal artery was found to be weak. Arteriography showed an occlusion of the left superficial femoral artery, however no operation was undertaken as she was free from symptoms. Case 3: A 26 year old w o m a n came to our hospital in 1972 complaining of pain, swelling, and localized heat in the left buttock. O n physical examination, an elastic soft pulsating mass measuring 5 X 10 cm was f o u n d in the right buttock a n d a systolic m u r m u r was heard. Arteriogram revealed the superior a n d inferior gluteal arteries to be the feeders of the gluteal A-V fistula (Fig. 3).

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Fig. 2. The angiograph of case 2 showing an angiomatous arteriovenous fistula in the left buttock. The feeding arteries were the superior gluteal and lateral femoral circumflex arteries, and the superior gluteal vein was confirmed as a drainage vein in the late phase.

Fig. 3. The feeding arteries of case 3 were the superior and inferior gluteal arteries. The drainage vein was the inferior gluteal vein.

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Congenital A V fistula in the gluteal r e , on

During the operation, both arteries were ligated at their origin through an extraperitoneal approach, a n d the A-V fistula removed. H e r postoperative course was uneventful, although there r e m a i n e d a small part of the A-V fstula, which was confirmed by angiography on the eighth postoperative day. Case 4: A 17 year old m a n h a d noticed swelling o f his left buttock since he was 14 years old. W h e n he was 15 years old, ligation of the internal iliac artery was carried out in another hospital, leaving the fistula untouched, and there was no change in the size of the mass postoperatively. As a 17 year old, he was kicked in the left buttock, which was followed by fever and pain. O n admission in 1975, he had a 7 ;< 10 cm pulsating mass in his left buttock, a n d the color of the skin h a d changed because o f the hepatoma. Arteriogram showed the feeding arteries to be the inferior gluteal and lateral femoral circumflex arteries. At surgery, the left inferior gluteal artery, internal pudendal artery, and femoral circumflex artery were ligated. T h e superior gluteal artery could not be identified because o f a d h e s i o n resulting from the previous operation. T h e A-V fistula was removed with a loss of 3,000 ml o f blood from the partial resection of the gluteal m a x i m u m muscle (Fig. 4). His postoperative course was uneventful. Case 5: A 46 year old w o m a n was operated on in o u r d e p a r t m e n t for cancer of the left breast. Two days following h e r discharge, she had severe pain in the left buttock a n d discovered a pulsating mass. She refused an operation, although an A-V fistula was confirmed on physical examination. Six years later, she again had severe pain in the same area and disturbance o f h e r gait; she then accepted admission for an operation (Fig. 5). Preoperative angiogram showed the superior gluteal, inferior gluteal, and lateral femoral circumflex arteries to be the feeders. During the operation, p e r f o r m e d in 1986, an interhal iliac artery was ligated extraperitoneally and the lateral femoral circumflex artery

Fig. 4. T h e a r t e r i o v e n o u s fistula o f case 4 p r e s e n t e d a mass f o r m a t i o n a m o n g t h e gluteal nmscles. It was t h e r e f o r e possible to r e m o v e it without resecfing a n y muscle or n e r v e tissue.

Fig. 5. The enhanced computed tomography of case 5 showing a grapelike arteriovenous fistula between the muscles. Although inflammation and hematoma was found around it, isolating it was not difficult. ligated in the groin. T h e A-V fistula was completely removed, and the gluteal muscle preserved. She was discharged after an uneventful recovery on the 14th postoperative day. RESULTS

T h e above cases involved 1 m a n and 4 w o m e n whose ages r a n g e d between 17 and 46 years, with a m e a n age of 30 years. The clinical courses are summarized in Table 1.

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Table 1. Five Cases are Summarized in This Table. The Feeding Arteries were Confirmed by Preoperative Angiographies Cases Age, Sex Side

Symptoms

Feeding Artery

Operative Time Blood Loss

28, F

right swelling pain

superior gluteal

3 hours

1000 ml

33, F

left

superior gluteal lateral femoral circumflex

5 hr. 20 rain.

2000 ml

swelling

Post Op good sup. fem. art.

occluded

3

26, F

left

swelling pain fever

superior gluteal inferior gluteal

3 hr. 45 rain.

400 ml

good

4

17, M

left

swelling pain fever

inferior gluteal lateral femoral circumflex

7 hr. 15min.

3000ml

good

5

46, F

left

pain superior gluteal gait inferior gluteal disturbance lateral femoral circumflex

4 hr. 30min.

1000ml

good

T h e i r chief complaints were pain, swelling and a pulsating mass, and none of the patients had any circulatory problems, such as cardiac failure or coagulopathy, although case 4 had a bleeding episode in his history. In case 1, we excised the A-V fistula with some gluteus maximus muscle. T h e resected mass was examined carefully and most o f the fistula was confirmed to be in the connective tissue between the gluteal muscles, although a small part had extended b e y o n d the fascia. However, separation was easy between the fistulous mass and the gluteus maximus muscles concomitantly resected. Because of this, it seemed possible to excise almost the entire fistulous mass while preserving the gluteal muscles. T h e n e x t four cases t h e r e f o r e underwent operations with successful preservation of the gluteal muscles. In case 2, the superficial femoral artery was occluded postoperatively, which was considered to have b e e n caused by intimal injury due to vascular clamping during t h e operation, and no postoperative dysfunction of the affected extremity resulted.

DISCUSSION

T h e embryogenic process causing the development o f a congenital arteriovenous fistula (A-V fistula) is not fully understood. Woollard 1 described the development of the embryonal vascular system in the forelimb of a pig, and outlined i) the stage o f capillary network, ii) the retiform stage, and iii) the mature vascularization stage. According to his classification, a congenital A-V fistula is a condition caused by the arrest or misdirection of the development of a capillary network or retiform stage. T h e broad spectrum of A-V fistulae is therefore observed in various points in the body. Malan e classified the congenital A-V fistula into the following categories: venous dysplasia, arterial dysplasia, arterial and associated venous dysplasia, and mixed angiodysplasia. On the other hand, Szilagy~ presented the classification as follows: i) hemangioma, ii) microfistulous arteriovenous aneurysm, iii) macrofistulous a r t e r i o v e n o u s a n e u r y s m , and iv) anomalous mature vascular channels. With regard to operability, however, we consider the classification of Kamiya 4 useful. He clas-

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Congenital A V fistula in the gluteal region

sifted the A-V fistula into two categories: diffuse and well localized. Each category was then further divided into a hemodynamically active or inactive condition by clinical manifestation. H e pointed out that a well-localized A-V fistula in a subfascial location was essentially resectable, regardless o f activity. According to Kamiya's classification, a gluteal A-V fistula belongs to a group o f welllocalized and active types. Nevertheless, A-V fistula in the gluteal region is rare in the literature. Natile ~reported seven cases of A-V malformations in the region o f the internal iliac artery, four o f which were in the gluteal region. He speculated that pregnancy would activate arteriovenous malformation o f the internal iliac artery. Decker 6 reported six cases of A-V fistula in the female pelvis including one in the hip. T h e diagnosis of this regional fistula is easily made by physical examination. T h e characteristic manifestations are a pulsating mass with a thrill and vascular bruit, and an asymmetrical appearance of the buttock is also always noticed. To determine the feeding artery, arteriograms of the iliac artery and femoral artery are necessary. All our cases showed the superior or inferior gluteal arteries as feeders, and in two cases, the lateral circumflex femoral artery was also a feeder. T h e most c o m m o n symptom in our cases was pain, and n o n e had hypertrophy or other complications of the leg, such as Parkes-Weber s y n d r o m e or Klippel-Trenaunay-Weber syndrome. 7 All of our cases were well localized and the feeding artery was clearly demonstrated by a preoperative angiography. Most parts of the A-V fistula existed intermuscularly instead o f intramuscularly, and the operation which we recommend for this gluteal A-V fistula is therefore 1) ligation of the feeding arteries extraperitoneally and 2) excision of the AoV fistula thereafter. Simple ligation o f the feeders should not be performed because Other collateral arteries could easily develop. On the other hand, simple removal o f the A-Vfistula makes a dangerous operation due to the risk o f bleeding. In case 4, whose

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superior gluteal artery could not be ligated before resection, 3,000 ml o f blood was lost d u r i n g the o p e r a t i o n . T h e p u r p o s e o f ligating the feeding artery therefore exists in the shrinkage of the A-V fistula and the prevention of blood loss during the operation. Some reports advocated transcatheter embolization with gelfoam s,9 or ethanol, 1~ which seems safer in comparison with an operation. A deficit o f ischiadic nerve function, however, was shown in some cases as a complication, at the etiology o f which is speculated as ischemic damage of the nerve fibers due to the occlusion o f their vasa vasorum. However, in our cases, no neurological complication was noted postoperatively. T h e extraperitoneal approach to the branches of the internal iliac artery is an easy procedure, and therefore, with regard to complications, the surgical ligation of arteries before removal of the A-V fistula is a safer procedure than the embolizing method. Recurrence o f the A-V fistula resulting from incomplete excision is another problem, although no recurrence has been experienced in any o f the above five cases, suggesting the suitability of our procedure for A-V fistula in the hip. T h e characteristics commonly seen in cases o f gluteal A-V fistulae are briefly summarized as follows. Most exist in the connective tissue between the gluteal muscles; the feeding arteries are the superior or inferior gluteal arteries or both; and they are well-localized fistulae w i t h o u t any h e m o d y n a m i c disorders. All these observations suggest that A-V fistulae in this region have similar etiology. It could be concluded that the A-V fistula in the gluteal region is principally resectable while preserving the surounding gluteal muscles and isciadic nerve, and that surgery should be the first choice o f available treatments to cure this disease. (Received for publication on May 15, 1989)

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1968; 31: 799-805. 7. Cotton LT, Sykes BJ. The treatment of diffuse congenital arteriovenous fistulas of the leg. Proc R Soc Med 1969; 62: 245-247. 8. Palmaz JC, New ton TH, Reuter SR, Bookstein JJ. Particulate intraarterial embolization in pelvic arteriovenous malformations. Amer J Radiol 1981; 137: 117-122. 9. Olcott C, Newton TH, Stoney RJ, Ehrenfeld WI~ Intra-arterial embolizatlon in the management of arteriovenofis malformations. Surgery 1976; 79: 3-12. 10. Yakes WF, Pevsner P, Reed M, Donohue HJ, Ghaed N. Serial embolizations of an extremity arteriovenous malformation with alcohol via direct percutaneous puncture. Amer J Radiol 1986; 146: 1038-1040. 11. Uchiyama N, Arai Y, Shinzato J, Tsuji J, Nagata Y, Tonyama K, Shinohara S. A case of sciatic nerve pulsy as a complication of gluteal artery embolization with ethanol. Jpn J Clin Radiol 1984; 29: 997-999. (English Abst.)