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Congenital arteriovenous fistula of the maxillofacial region
Congenital Arteriovenous Fistula of the Maxillofacial Region I. Rappaport, MD, Irvine, California J. Rappaport, BS, Santa Ana, California
Congenital arteriovenous (AV) fistulas of the maxillofacial structure present many intriguing problems. Their classification has varied with different authors. The terms applied to them include arteriovenous aneurysms, cirsoid aneurysms, racemose aneurysms, aneurysms by anastomosis, plexiform angiomas, aneurysmal varices, and hemangiomas of the bone. Dandy [I] in 1928 discussed the problems of terminology and concluded that all of these terms were merely descriptive, an expression of the lesion and not an expression of its fundamental pathologic features. This confusion was again emphasized by Horton [2] in 1931. Szilagyi et al [3] in 1965 described the embryologic vascular tree as a series of blood spaces in a primitive mesenchyme, with mixed blood without separate venous or arterial channels. Wollard [4] outlined the following sequence of events: (a) the stage of undifferentiated capillary; (b) the retiform stage of large plexiform structures formed by coalescence of the original equal potential capillaries; and (c) the stage of mature vascular stems. These stages were further modified by DeTakats [5] and outlined as: (1) the endothelial stage; (2) the retiform stage; and (3) the stage of gross differentiation. (Figure 1.) It has been thought that arrest in any stage of this development does occur, and the lack of any progressive differentiation in the endothelial stage results in formation of the commonly called hemangiomas of the cavernous or capillary type, whereas arrest of development in the retiform stage may result in formation of the mixed hemangiomas or a true AV fistula. Sabin [6] demonstrated that in certain vessels, the flow of blood may be reversed so that these vessels which function as arteries during one stage of development may function as veins in another stage. Reid [7] pointed out that the direct growth of veins from an artery, such as the cardinal veins from the walls of the aorta, could afford an excellent chance for the persistence of free commu-
From the Division of Plastic Surgery, University of California, Irvine; the Division of Plastic Surgery, Long Beach VA Hospital, Long Beach: and the Head and Neck Regional Center, Santa Ana, California. Reprint requests should be addressed to Irving Rappaport, MD. Division of Plastic Surgery, University of California, Irvine, California 92664. Presented at the Forty-EighthAnnual Meeting of the Pacific Coast Surgical Association, Palm Springs, California, February 20-23. 1977
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nications between the two systems. Szilagyi et al [3] suggested that not all fistulas could be demonstrated angiographically, and called the demonstrable ones macrofistulas and the nondemonstrable ones microfistulas. Although their work concerned the extremities, we believe that the same principle is applicable in the head and neck area. Table I is a modification of a previous communication of ours [8~ based on the Szilagyi classification [3]. It is our belief that a pure macrofistula in the head and neck cannot exist without many communicating microfistulas, and this is primarily the reason that the word “cure” is not applicable, no matter what the modality of treatment. The hemodynamics of an AV fistula were studied in depth by Holman [9]. He pointed out that the blood flow, like flowing water, seeks the path of least resistance. The presence of a macrofistula, therefore, is an invitation for microfistulas to open vascular channels to feed and nourish it. The formation of collateral circulation secondary to the presence of an AV fistula is usually brought about because of the increased volume and pressure differential in the macro- and microfistulous malformation. To pursue this point, we have to delve into the embryology of the area. The internal carotid artery is derived from the dorsal aorta, whereas the external carot,id artery arises from the ventral aorta. The circulation of the two aortas is connected by the branchial arch arteries of which the second arch artery persists as the middle meningeal artery in the adult. It is this artery that joins the carotid arteries intracranially and extracranially. The external carotid artery, through its occipital branch, joins with the vertebral artery through its muscular branches in the suboccipital triangle. As we have discussed in a previous communication [8], these collateral channels increase in response to the AV fistula and should not be confused with the malformations themselves. It is this interrelationship in communication between both the internal and external carotid arteries and the vertebral system that makes management of the maxillary fistula extremely difficult. Further frustrations occur when one discovers there is usually microfistulous collateral circulation between opposite sides of the head.
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Figure 1. AV malformation. Left, endothelial stage. Middle, retiform
Lesions arising in the maxillary bones are extremely rare, and long-term follow-up of this problem in the literature is difficult to trace. Malan and Azzolini [IO] in 1968 discussed congenital AV malformations of the face and scalp. They believed that the pathologic aspects of the congenital malformations could be divided into two groups, the congenital AV fistula and the congenital AV angioma. They preferred surgical treatment, utilizing the same philosophy as that of treating a cancer. Their approach to the mandible was not segmental resection but removal of a portion of the cortex of the mandible, with direct visualization of the angioma with currettement. Surgery proceeded with ligation of the external carotid artery on the ipsilateral side and, in one patient, ligation of both the right and left external carotids as a preliminary approach. Long-term follow-up was not available. Coleman [11,12] believed that many of the shunts were nonfunctional at the time of arteriography and became functionally active when peripheral resistance at the capillary end of the vascular circuit was increased to permit a flow of arterial blood through the shunt. Arteriography was performed by open surgery with temporary occlusion of the distal internal and external carotid arteries. They controlled hemorrhage for the excisional surgery by a preliminary procedure five to seven days
TABLE I
Congenital Vascular
MalfOrf?IafiOnS of
Maxillae
Embryologic Development
Classification Angiographic Histologic
Endothelial
Capillary Cavernous Mixed
Nonfistulous Nonfistulous Nonfistulous
Retiform
Mixed Mixed Mixed
Nonfistulous Microfistulous? Macrofistulous
Gross differentiation
Mixed Mature arteries
Microfistulous? Macrofistulous
40
stage.
Right, gross differentiation staga.
preoperatively, which consisted of ligating major feeding vessels and applying a Crutchfield clamp to the common carotid artery on the ipsilateral and contralateral sides. Their contention was that three-dimensional resection followed by reconstruction offered the patient the best chance of cure. Another form of therapy has been irradiation. In 1940 Watson and McCarthy [13] reported the use of high voltage radiation therapy for treatment of bone hemangiomas in three patients, two with hemangioma of the lumbar vertebrae and one with hemangioma of the mandible. At that time they believed they had permanent control of the disease with this technic. Long-term follow-up has not been available. An interesting long-term follow-up was undertaken by Brown, Sadek, and Robinson [14]. The patient first presented with what was called a sinusidal aneurysm of the mandible for which he underwent emergency surgery in 1952. Apparently he did well for a period of time, and in 1959 bone grafting was performed by Doctor Robinson [14] to bridge the area of resection. At that time it was noted that there was an AV malformation involving the right maxilla, primarily in the soft palate area. The patient had radon seeds implanted, and the swelling subsequently disappeared. In New York in 1969, the patient again presented with an asymptomatic angiomatous lesion involving the area of the right hard and soft palate. An arteriogram of the internal carotid system demonstrated a right suprasellar AV malformation deriving blood supply from the cavernous portion of the right internal carotid and the right anterior cerebellar arteries. The left cerebral circulation did not appear to contribute to the malformation. Supervoltage cobalt radiation of 4,000 r was administered during a four week period. Unfortunately, the previous radiation dosage he had received with the radon seeds could not be determined because the therapist had died and the records were
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not available. Arteriograms taken eleven months after the radiation therapy in New York showed a 20 per cent shrinkage of the AV malformation in the suprasellar area. We located the patient and discussed his problem with him, and except for visual defects and severe headaches, he has had no other symptoms from the AV malformation, and apparently the problem is now in abeyance. Embolization with foreign materials has been another method of treat:.ng vascular malformations during the past sixteen 3r so years. Luessenhop et al [15] in 1965 presented their technic for the very difficult treatment of AV :.esions of the cerebrum, and their success encouraged many surgeons to attempt embolization. Various agents have been used, such as artificial barium-impregnated silicone spheres, and most recently, Olcutt et al [16] have utilized the Seldinger technic with combinations of gel foam, lead pellets, and autogenous muscle as emboli. Described herein are two interesting cases of malformations of the maxillary bone and the longterm problems involveci.
Case Reports Case I. In 1969 an eleven and a half year old girl presented with bleeding of the gingiva on the right side of the maxilla. She had been entirely well until three weeks earlier when she noted pain in her right maxilla, and x-ray films were interpreted as indicating possible sinusitis. Fourvessel arteriograms in October 1969 showed the right internal maxillary artery with its ascending pharyngeal branch markedly hypertrophied and showed filling of a mass of AV malformatior: involving the right maxillary antrum along its floor, extending all the way posteriorly along the superior aveolar ridge. (Figure 2.) Ligation of the external and internal maxillary arteries feeding the right maxilla was performed. Immediately after ligation, the bruit was no longer heard and the thrill could not be felt in the cheek. Ten days postoperatively, repeat angiography was performed, the right carotid artery study demonstrated collateral circulation from the internal carotid artery, and the external maxillary artery continued to partially fill the AV malformation. The caroticotympanic vessels, normally invisible on angiography, became apparent. The cavernous sinus branches and the meningeal branches demonstrated anastomosis with the small branches of the internal maxillary artery. The left external carotid artery at this time showed definite evidence of cross filling with collateral branches to the right maxilla to supply blood to the AV malformation, which obviously had changed from a microto a macrofistula. The vascularity, however, was believed to have been reduced ‘70per cent compared with the preoperative ligation. In view of this study, it was decided to try embolization of the AV malformation, and in November 1969. a mixture of gel foam and triturated muscle was in-
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Figure 2. Case 1. A V fistuta of the maxilla.
jetted by surgical cutdown into the internal maxillary artery. Within several minutes after injection, clotting formed in the internal maxillary artery and its dilated branches, immediately decreasing the vascularity of the right maxillary sinus malformation by a further 75 per cent. However, in January 1970, the left external carotid artery showed a crossover of the ascending pharyngeal branches to the malformation. Injection into right internal carotid artery showed cavernous branches anastomosing with the external branches at the level of the ascending pharyngeal area, with filling of the AV malformation of the right maxilla to approximately 40 per cent of the previous demonstration. In the interval between her next hospitalization in early February 1970 and the embolization, we noted some tightening of her teeth; however, the patient began to bleed from her gingiva again. A rather unusual hyperplastic condition became apparent in her gum tissue, the patient bled profusely, and the teeth began to exfolliate. Emergency surgery was performed in late February 1970. Ligation of the left external carotid artery was performed and some of the same thrombogenic material was injected directly into the AV fistula and also into the posterior palatine artery. Repeat right vertebral angiography performed two weeks later showed prominent occipital communications with deep cervical branches, with evidence of communication with the AV malformation. Right common carotid angiography showed numerous small squamous and cavernous branches of the internal carotid artery anastomosing with the residual ascending pharyngeal artery, which continued to perfuse the malformation. There was also feed via the ophthalmic and ethmoid vessels through an aberrant branch of the vertebral artery into the occipital artery and then retrograde into the internal maxillary artery. In view of these findings, urgent surgery was deemed necessary for control. At t,he time of surgery it was of interest that even with pressure control of the internal and
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Figure 3. Case I. Hypertrophied right opthalmic artery with new ma/formation.
common carotid arteries, there was no effect on the amount of bleeding. Right maxillectomy was performed in a routine manner despite brisk bleeding, and the postoperative recovery period was uneventful. The microscopic studies were of great interest, showing areas of new bone formation and also a lack of any endothelial lining of the large cavities within the bone. However, numerous fibroblasts and proliferating capillaries were found. The patient did very well over the ensuing years. In 1976 she presented with swelling of the right upper lip which did not demonstrate a bruit of pulsation. Repeat four-vessel angiograms showed recanalization of the right external carotid channel to approximately 1 mm diameter, giving rise to a small external maxillary artery that did not appear to supply the AV malformation. A very hypertrophied right ophthalmic artery supplying a new AV malformation was noted. (Figure 3.) The dorsal nasal branch anastomosed with the angular artery of the right external maxillary and extensively supplied the malformation. The ophthalmic lacrimal branches anastomosed with the zygomatic branches, which also ultimately entered the internal ‘maxillary artery via the deep temporal and transverse facial branches. The posterior ethmoidal branch of the ophthalmic artery anastomosed with the sphenopaiatine branch of the internal maxillary artery and supplied the infraorbital artery, which then entered the AV malformation. Left common carotid injection indicated the internal maxillary artery had recanalized and contributed to the malformation. The left labial branches directly cross-communicated with the right labial branches and then entered the malformation. (Figure 4.) The left vertebral artery demonstrated almost immediate opacification of the left occipital artery via deep muscular branches, and
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Figure 4. Case 1. Crossover of left labial artery.
the occipital artery then demonstrated a very prominent opacification of the internal maxillary, external carotid, and external maxillary vessels that subsequently supplied the malformation. There was drainage through the right superior ophthalmic vein into the cavernous sinuses as well as into the venous structures deep within the face. Resection which encompassed the AV malformation was performed without untoward bleeding. Repeat angiography one year later did not show any signs of the previous AV malformation. There was no evidence at this time of the large communicating channels between the vertebral and carotid systems, particularly on the left side. Two minutes after angiography, the patient developed bilateral cortical blindness, secondary to vertebral vasospasm, which cleared in two days. Case II. A thirty-two year old man was in good health until 1971 when he developed a mass in the hard palate. He had had left external carotid artery ligation performed elsewhere, and in 1972 he had a surgical cutdown of the left external carotid artery and catheterization with installation of 1.5 mm diameter barium-coated Silastic? balls. A total of seventy-eight such balls were placed with successive angiograms. It was believed there had been a minimum of 60 per cent reduction of the malformation. The x-ray films prior to this procedure showed a large internal maxillary artery on the left, greatly hypertrophied, filling a large AV malformation of the hard palate with enormous draining
The American Journal of Surgery
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Figure 5. Case II. Large internal mation (arrows).
maxillary
Fistulas
artery malfor-
veins into the pharynx. He did well for two years and then presented with massive bleeding from the oral cavity around the teeth. Two months earlier repeat angiography had shown the blood supply to the malformation coming from the left internal maxillary artery which was being fed by multiple huge collaterals from the left vertebral artery. (Figure 5.) Additional circulation came from the left internal carotid artery through the ophthalmic artery and also from the right external carotid artery through the right internal maxillary artery. The surgical procedure performed was bilateral right external carotid artery skeletonization and removal of sections of the arteries to extirpate as much of the microfistula and as many collateral vessels as possible from the common carotid and vertebral arteries. Then, during the same procedure, maxillectomy, a very difficult procedure due in part to bone hypertrophy, was performed. Before surgery the patient had an audible noise in the left ear, which was consistent with the bruit that we could hear. The noise had decreased enormously after our procedure but was still present postoperatively. Repeat angiography eight days later showed a 2 X 1 cm vascular abnormality in the region of the pterygoid plates near the posterior wall of what had been the left maxillary antrum. (Figure 6.) It was believed that this residual area was causing the audible sound and probably should be eliminated. The supply appeared to be primarily from the left vertebral artery as well as from collateral branches of the right internal carotid artery through the occipital artery. The patient underwent reoperation, and the remainder of the pterygoid plate and the adjoining area of the base of the skull were removed without involving dura. The patient has had an uneventful three years and is continuing his business as an executive on a daily basis. One year later, repeat four-vessel studies showed two small AV malformations in the left pterygoid palatine fossa (Figure 7) supplied by collateral source from the left ophthalmic artery and left vertebral artery and a possible early AV
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Figure 6. Case II. Vascular abnormality goids.
in reglon of ptery-
malformation on the left posterior ethmoid region supplied via the ophthalmic artery. The patient has been asymptomatic, and there does not appear to be any other problem. Comments From our experience with these two cases and similar ones of the head and neck that we have treated, it is apparent that the unsolved problem is determining the extent of the microfistulas, not the collateral circulation. It has not been feasible to study this embryologic deficiency with the modalities we have available, and therefore, it is difficult to plan an adequate surgical resection. To obliterate the microfistulas by means of embolization would require use of a medium that could permeate the microcirculation. In our angiographic studies of the venous runoff of the maxillary arteries, the utilization of such a thrombogenic material may allow the runoff to be carried into the cavernous sinus and cause thrombosis or even retinal vein thrombosis. It is in this area of the microfistula that radiation therapy may have a very significant role, as it does have the ability to thrombose small vessels. At times we have seen laminated calcifications in vascular masses in the head and neck area. We have had one case of this entity present in the mandible and have given the presumptive diagnosis of nonfistulous or microfistulous vascular malformation to the patients who have presented with a phlebolith. (Figures 8 and 9.)
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Figure 7. Case II. New A V fistulas of left pterygoid palatine fossae.
Figure 9. Phleboltte (arrows) In maxilla.
In the three long-term follow-up cases we have not seen any symptomatic macrofistulas develop. Probably it is best not to subject this sort of laminated calcified area to a surgical procedure even though we did explore and remove the malformation from the mandible. If any macrofistula must be intimately enmeshed with microfistulas, similar to
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Figure 8. Phlebollte in mandibular canal.
the phenomenon of field cancerization, then it is easier to understand the hemodynamics of the congenital AV malformation in the bony framework of the head and neck and why ligation of feeding vessels has the potential of lowering resistance in the malformation, which in turn can convert the surrounding microfistulas into macrofistulas. We agree with all that has been said before-that pure ligation of the vessels is absolutely contraindicated. We have seen anastomotic channels develop between vertebral, ascending cervical, deep cervical, occipital, and ascending pharyngeal arteries. The ophthalmic arteries have become involved bilaterally, forming a single large AV macrofistula involving all four major arterial flows to the skull and its contents. Luessenhop et al [15] in 1965 described the utilization of Silastic spheres for embolization; Djindjian et al [17] in 1973 mentioned that embolization did not preclude intervention in vascular tumors and suggested that it be used as a preparatory step to reduce the risk of hemorrhage. They also described the utilization of gel foam and muscle in embolization procedures, and in 1975, Olcutt et al [16] reinforced their conclusion. Our experience with embolization of maxillary macrofistulas makes it questionable whether excisional surgery is made safer or technically easier. We believe that if embolization is to be performed, it should be done bilaterally and if possible, by the Seldinger technic so as to obviate unnecessary ligation of vessels in the area of the microfistulas. If obliterative surgery is attempted, we believe that the
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vessels supplying the area, such as the external carotid artery and its branches, be removed rather than ligated. The question that has not been answered is the role of radiation therapy, and we are contemplating its utilization, depending on the age of the patient and the early visualization of a new macrofistula after extirpative surgery or embolization.
Summary Long-term follow-up is essential to evaluate our present modalities of treatment of arteriovenous malformations of the maxillae. Embolization followed by surgical resection has produced long-term survival in our patient population.
References 1. Dandy W: Atieriovenous aneurysm of the brain. Arch Surg 17: 190, 1928. 2. Horton B: Hemihypertrophy of extremities associated with congenital arteriovenous fistula. Co// Papers Mayo C/in 23: 650, 1931. 3. Szilagyi E, Elliott J, De Russo F, Smith R: Peripheral congenital arteriovenous fistulas. Surgery 57: 61, 1965. 4. Wollard R: The development of the principal arterial stems in the forelimb of the pig. Contributions to Embryology, Carnegie Institute, Washington 14: 139, 1922. 5. DeTakats G: Vascular anomalies of the extremities. Surg Gynecol Obstet 55: 227, 1932. 6. Sabin F: Origin and development of the primitive vessels of the chick and the pig. Contributions to Embryology, Carnegie Institute, Washington 6: 61, 1917. 7. Reid M: Abnormal arteriovenous communications acquired and congenital. Arch Surg 10: 996, 1925. 8. Rappaport I, Yim D: Congenital arteriovenous fistulas of the head and neck. Arch Otolaryngol97: 350, 1973. 9. Holman E. Contributions to cardiovascular phvsiology gleaned from clinical and experimental observations of abnormal atteriovenous communications. J Cardiovasc Surg 3: 48, 1962. 10. Malan E, Azzolini A: Congenital arteriovenous malformations of the face and scalp. J Cardiovasc Surg g(2): 109, 1988. 11. Coleman C, Hoopes J: Congenital arteriovenous anomalies of the head and neck. Plast Reconstr Surg 47: 354, 1971. 12. Coleman C: Diagnosis and treatment of congenital arteriovenous fistulas of the head and neck. Am J Surg 126: 557, 1973. 13. Watson W, McCarthy W: Blood and lymph vessel tumors. Surg Gynecol Obstet 71: 569, 1940. 14. Brown D, Sadek K, Robinson M: Arteriovenous malformations of the mandible, maxilla, orbit and cerebrum. J Oral Surg 31: 553, 1973. 15. Luessenhop A, Kachmann R, Shevlin W, et al: Clinical evaluation of artificial embolization in the management of large cerebal arteriovenous malformation. J Neurosurg 23: 400, 1965. 16. Olcutt C, Newton T, Stoney R, Eprenfeld W: Intra-arterial embolization in the management of atteriovenous malformations. Surgery 79: 3, 1976. 17. Djindjian J, Cophignon J. Theron J. Merland J. Houdart R: Embolization by super selective arteriography from the fermoal route in neuroradiology. Review of 60 cases. fJeworsdiology 8: 20, 1973.
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Discussion Herbert J. Movius, II (Long Beach, CA): Doctor Rappaport has presented a very serious and perplexing problem created by angiodysplasias when they involve the head, particularly the maxilla. Because of their proclivity to hemorrhage, they are more lethal than most angiodysplasias which occur in other places in the body. Nevertheless, wherever they occur, they are tremendous problems. They are always a challenge to manage. My experience is relatively limited to attempts to reduce morbidity due to malformations of the extremities and of the pelvis. Surgical excision of the malformation, attempts to reduce blood flow through the malformation, and amputations have been used and have largely proved unsatisfactory. Radiation also has been used with mixed results, although with newer technics it may actually prove to be an acceptable modality. For a brief period flow may be reduced, and results of a finishing angiogram may be optimistic when as many as possible of the feeding vessels to the fistula are found, ligated, and excised. However, the tremendous potential for collateral development and the opening of new fistulas existing not at the time of the finishing angiogram but three weeks or months later, whatever their origin, results in early return of symptoms. I have had experience with one very large, primary venous angioma that involved the chest wall with seven or eight ribs, held approximately 2 quarts of blood, and could not be excised primarily because of its size and position but was totally and permanently eradicated by numerous injections using sclerotherapy. However, I have used this on other AV malformations without good results. As Szilagyi et al [3] have pointed out, many AV fistulas, whether micro- or macrofistulas, resulting from angiodysplasia will not be demonstrated on arteriography, as in the so-called Klippel-Trenaunay-Weber syndrome. If they occur during the growing period of the individual, they result in tremendous overgrowth of the extremity, which is sometimes extremely disabling. Procedures aimed at arresting growth of the enlarged extremity at the time the other extremity, continuing to grow, approximates the size of this overgrown extremity when it stops growing are helpful. If such a procedure cannot be performed-frequently the patient is not seen in time-then bone-shortening operations are recommended, particularly in the lower extremity, with femural and transmetatarsal amputation of the foot resulting in two extremities that are not too dissimilar and function well. I have performed this operation on two patients, both of whom ski and dance and have very little morbidity from the entire process. Cardiac overload eventually resulting in heart failure may be a serious problem, and it demands prompt management at times. This can occur in the neonatal period. In the past it has been solved by extremity amputation, usually as an emergency, after some less radical flow-limiting procedure has been tried. It is usually successful but, is a horrible thing to do to a newborn infant.
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However, the promising modality of embolization, the study of which has only begun, hopefully will lead to much better management in the future than we have so far been able to accomplish, especially with highly selective angiography and ingenious new embolization technics. Morton M. Woolley (Los Angeles, CA): I will limit my comments to a few of the problems seen in infants and children with macrofistulas. I should like to illustrate with two brief cases. The first is an angiographic study performed on a two. and a half year old boy because of a pulsatile mass in the left neck. This angiogram reveals a large macrofistula between the carotid system and an anomalous vein. At surgery, the external carotid artery was extremely large in comparison with the normally sized internal carotid artery. There was no internal jugular vein, a fact which has been previously reported in association with this type of anomaly. The superior thyroid, lingual, and ascending pharyngeal arteries were divided, and as much of the malformation that could be removed safely was excised. (Slide) Five years later, this angiogram revealed multiple small AV fistulas in the neck. (Slide) Although the patient has a visible and palpable mass in association with an audible bruit, he is an active boy, competes in athletics, and attends school regularly. His parents are quite resistant to any further invasive diagnostic procedures or therapy because of his good general health. I believe they are quite right since he is not symptomatic. Doctor Movius referred to the problem of bleeding from macrofistulas, and I should like to illustrate how severe bleeding can be from an extremity AV macrofistula. (Slide) This is a photograph taken of the arm of a newly born infant admitted with congestive heart failure, because of which a large pulsatile artery was ligated without antecedent angiography. (Slide) An angiogram after ligation revealed additional feeding arteries, which were ligated, and the congestive heart failure was controlled. (Slide) The patient survived an episode of sepsis and was sent home, but bled to death one night when a crust spontaneously separated from the surface of the hemangioma. One must realize the bleeding potential of some arteriovenous fistulas and the benign symptoms of others. Judgment dictates when considering the appropriate therapy for such patients. William K. Ehrenfeld (San Francisco, CA): At the University of California, San Francisco, we have increasingly advocated conservative management of AV malformations. The management is now primarily embolization rather than surgery. At this time we have had experience
46
with six patients who have had embolization of AV malformations of the face and neck. (Slide) Shown are some of the patients in our series before and after embolization. In several patients, the external carotid artery has been ligated. This surgery, unfortunately, is associated with only short-lived relief for the patient. The malformations usually recur very soon after ligation, and the feeding vessel is no longer available for embolization. Our technic is to use the percutaneous approach, embolizing through the catheter with the Seldinger technic. Embolization is often carried out from both sides using each external carotid artery. In addition, it is not uncommon for the patient to require more than one session of embolization. This technic lends itself to repeat sessions which can be separated by months or years. We do not believe that embolization necessarily is curative, but it does supply palliation. One patient who had an AV malformation involving the left lower jaw has gone two years since embolization, and the bruit over the mass has not recurred. Shortly after embolization, numerous “floating” teeth were removed with only minor bleeding. The remaining patients have been controlled, some of whom required more than one embolization session. Irving Rappaport (closing): I would like to emphasize that we obtained follow-ups on cases throughout the country which were reported in the literature. We were amazed at how little follow-up was done in these cases. It is not a matter of collaterals in the area we are particularly interested in-bony structure of the head and neck-but a matter of congenital microfistulas. The recurrences we are seeing confined to bone are primarily from the latter. I do not believe that embolization of large spheres is as effective with microfistulas as the technic of injected thrombogenic material, which was first discussed in 1967, soon after Luessenhop [IS] performed his basic technics intracranially. However, the problem in the maxilla is that the ophthalmic vein and other vein systems that go directly into the saggital sinuses are involved. Therefore, the question is with how small an embolus can we manage the microfistulas and not have a problem intracranially. This problem has not been solved. Therefore, we are looking toward radiation therapy, because some of the carotid body tumors that have been unresectable-and occasionally some are thoroughly unresectable in the elderly-have been palliated with additional supervoltage therapy. To my knowledge, including the literature throughout the world, supervoltage therapy has not been discussed in the management of microfistulas. The only thing we could find was orthovoltage utilized many, many years ago, the judicious use of which may be of some value in small microfistulas. The question is when to do it, and what are the contraindications.
The American Journal of Surgery
Congenital arteriovenous (AV) fistulas of the maxillofacial structure present many intriguing problems. Their classification has varied with different authors. The terms applied to them include arteriovenous aneurysms, cirsoid aneurysms, racemose aneurysms, aneurysms by anastomosis, plexiform angiomas, aneurysmal varices, and hemangiomas of the bone. Dandy [I] in 1928 discussed the problems of terminology and concluded that all of these terms were merely descriptive, an expression of the lesion and not an expression of its fundamental pathologic features. This confusion was again emphasized by Horton [2] in 1931. Szilagyi et al [3] in 1965 described the embryologic vascular tree as a series of blood spaces in a primitive mesenchyme, with mixed blood without separate venous or arterial channels. Wollard [4] outlined the following sequence of events: (a) the stage of undifferentiated capillary; (b) the retiform stage of large plexiform structures formed by coalescence of the original equal potential capillaries; and (c) the stage of mature vascular stems. These stages were further modified by DeTakats [5] and outlined as: (1) the endothelial stage; (2) the retiform stage; and (3) the stage of gross differentiation. (Figure 1.) It has been thought that arrest in any stage of this development does occur, and the lack of any progressive differentiation in the endothelial stage results in formation of the commonly called hemangiomas of the cavernous or capillary type, whereas arrest of development in the retiform stage may result in formation of the mixed hemangiomas or a true AV fistula. Sabin [6] demonstrated that in certain vessels, the flow of blood may be reversed so that these vessels which function as arteries during one stage of development may function as veins in another stage. Reid [7] pointed out that the direct growth of veins from an artery, such as the cardinal veins from the walls of the aorta, could afford an excellent chance for the persistence of free commu-
From the Division of Plastic Surgery, University of California, Irvine; the Division of Plastic Surgery, Long Beach VA Hospital, Long Beach: and the Head and Neck Regional Center, Santa Ana, California. Reprint requests should be addressed to Irving Rappaport, MD. Division of Plastic Surgery, University of California, Irvine, California 92664. Presented at the Forty-EighthAnnual Meeting of the Pacific Coast Surgical Association, Palm Springs, California, February 20-23. 1977
Volume 134, July 1977
nications between the two systems. Szilagyi et al [3] suggested that not all fistulas could be demonstrated angiographically, and called the demonstrable ones macrofistulas and the nondemonstrable ones microfistulas. Although their work concerned the extremities, we believe that the same principle is applicable in the head and neck area. Table I is a modification of a previous communication of ours [8~ based on the Szilagyi classification [3]. It is our belief that a pure macrofistula in the head and neck cannot exist without many communicating microfistulas, and this is primarily the reason that the word “cure” is not applicable, no matter what the modality of treatment. The hemodynamics of an AV fistula were studied in depth by Holman [9]. He pointed out that the blood flow, like flowing water, seeks the path of least resistance. The presence of a macrofistula, therefore, is an invitation for microfistulas to open vascular channels to feed and nourish it. The formation of collateral circulation secondary to the presence of an AV fistula is usually brought about because of the increased volume and pressure differential in the macro- and microfistulous malformation. To pursue this point, we have to delve into the embryology of the area. The internal carotid artery is derived from the dorsal aorta, whereas the external carot,id artery arises from the ventral aorta. The circulation of the two aortas is connected by the branchial arch arteries of which the second arch artery persists as the middle meningeal artery in the adult. It is this artery that joins the carotid arteries intracranially and extracranially. The external carotid artery, through its occipital branch, joins with the vertebral artery through its muscular branches in the suboccipital triangle. As we have discussed in a previous communication [8], these collateral channels increase in response to the AV fistula and should not be confused with the malformations themselves. It is this interrelationship in communication between both the internal and external carotid arteries and the vertebral system that makes management of the maxillary fistula extremely difficult. Further frustrations occur when one discovers there is usually microfistulous collateral circulation between opposite sides of the head.
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Figure 1. AV malformation. Left, endothelial stage. Middle, retiform
Lesions arising in the maxillary bones are extremely rare, and long-term follow-up of this problem in the literature is difficult to trace. Malan and Azzolini [IO] in 1968 discussed congenital AV malformations of the face and scalp. They believed that the pathologic aspects of the congenital malformations could be divided into two groups, the congenital AV fistula and the congenital AV angioma. They preferred surgical treatment, utilizing the same philosophy as that of treating a cancer. Their approach to the mandible was not segmental resection but removal of a portion of the cortex of the mandible, with direct visualization of the angioma with currettement. Surgery proceeded with ligation of the external carotid artery on the ipsilateral side and, in one patient, ligation of both the right and left external carotids as a preliminary approach. Long-term follow-up was not available. Coleman [11,12] believed that many of the shunts were nonfunctional at the time of arteriography and became functionally active when peripheral resistance at the capillary end of the vascular circuit was increased to permit a flow of arterial blood through the shunt. Arteriography was performed by open surgery with temporary occlusion of the distal internal and external carotid arteries. They controlled hemorrhage for the excisional surgery by a preliminary procedure five to seven days
TABLE I
Congenital Vascular
MalfOrf?IafiOnS of
Maxillae
Embryologic Development
Classification Angiographic Histologic
Endothelial
Capillary Cavernous Mixed
Nonfistulous Nonfistulous Nonfistulous
Retiform
Mixed Mixed Mixed
Nonfistulous Microfistulous? Macrofistulous
Gross differentiation
Mixed Mature arteries
Microfistulous? Macrofistulous
40
stage.
Right, gross differentiation staga.
preoperatively, which consisted of ligating major feeding vessels and applying a Crutchfield clamp to the common carotid artery on the ipsilateral and contralateral sides. Their contention was that three-dimensional resection followed by reconstruction offered the patient the best chance of cure. Another form of therapy has been irradiation. In 1940 Watson and McCarthy [13] reported the use of high voltage radiation therapy for treatment of bone hemangiomas in three patients, two with hemangioma of the lumbar vertebrae and one with hemangioma of the mandible. At that time they believed they had permanent control of the disease with this technic. Long-term follow-up has not been available. An interesting long-term follow-up was undertaken by Brown, Sadek, and Robinson [14]. The patient first presented with what was called a sinusidal aneurysm of the mandible for which he underwent emergency surgery in 1952. Apparently he did well for a period of time, and in 1959 bone grafting was performed by Doctor Robinson [14] to bridge the area of resection. At that time it was noted that there was an AV malformation involving the right maxilla, primarily in the soft palate area. The patient had radon seeds implanted, and the swelling subsequently disappeared. In New York in 1969, the patient again presented with an asymptomatic angiomatous lesion involving the area of the right hard and soft palate. An arteriogram of the internal carotid system demonstrated a right suprasellar AV malformation deriving blood supply from the cavernous portion of the right internal carotid and the right anterior cerebellar arteries. The left cerebral circulation did not appear to contribute to the malformation. Supervoltage cobalt radiation of 4,000 r was administered during a four week period. Unfortunately, the previous radiation dosage he had received with the radon seeds could not be determined because the therapist had died and the records were
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not available. Arteriograms taken eleven months after the radiation therapy in New York showed a 20 per cent shrinkage of the AV malformation in the suprasellar area. We located the patient and discussed his problem with him, and except for visual defects and severe headaches, he has had no other symptoms from the AV malformation, and apparently the problem is now in abeyance. Embolization with foreign materials has been another method of treat:.ng vascular malformations during the past sixteen 3r so years. Luessenhop et al [15] in 1965 presented their technic for the very difficult treatment of AV :.esions of the cerebrum, and their success encouraged many surgeons to attempt embolization. Various agents have been used, such as artificial barium-impregnated silicone spheres, and most recently, Olcutt et al [16] have utilized the Seldinger technic with combinations of gel foam, lead pellets, and autogenous muscle as emboli. Described herein are two interesting cases of malformations of the maxillary bone and the longterm problems involveci.
Case Reports Case I. In 1969 an eleven and a half year old girl presented with bleeding of the gingiva on the right side of the maxilla. She had been entirely well until three weeks earlier when she noted pain in her right maxilla, and x-ray films were interpreted as indicating possible sinusitis. Fourvessel arteriograms in October 1969 showed the right internal maxillary artery with its ascending pharyngeal branch markedly hypertrophied and showed filling of a mass of AV malformatior: involving the right maxillary antrum along its floor, extending all the way posteriorly along the superior aveolar ridge. (Figure 2.) Ligation of the external and internal maxillary arteries feeding the right maxilla was performed. Immediately after ligation, the bruit was no longer heard and the thrill could not be felt in the cheek. Ten days postoperatively, repeat angiography was performed, the right carotid artery study demonstrated collateral circulation from the internal carotid artery, and the external maxillary artery continued to partially fill the AV malformation. The caroticotympanic vessels, normally invisible on angiography, became apparent. The cavernous sinus branches and the meningeal branches demonstrated anastomosis with the small branches of the internal maxillary artery. The left external carotid artery at this time showed definite evidence of cross filling with collateral branches to the right maxilla to supply blood to the AV malformation, which obviously had changed from a microto a macrofistula. The vascularity, however, was believed to have been reduced ‘70per cent compared with the preoperative ligation. In view of this study, it was decided to try embolization of the AV malformation, and in November 1969. a mixture of gel foam and triturated muscle was in-
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Figure 2. Case 1. A V fistuta of the maxilla.
jetted by surgical cutdown into the internal maxillary artery. Within several minutes after injection, clotting formed in the internal maxillary artery and its dilated branches, immediately decreasing the vascularity of the right maxillary sinus malformation by a further 75 per cent. However, in January 1970, the left external carotid artery showed a crossover of the ascending pharyngeal branches to the malformation. Injection into right internal carotid artery showed cavernous branches anastomosing with the external branches at the level of the ascending pharyngeal area, with filling of the AV malformation of the right maxilla to approximately 40 per cent of the previous demonstration. In the interval between her next hospitalization in early February 1970 and the embolization, we noted some tightening of her teeth; however, the patient began to bleed from her gingiva again. A rather unusual hyperplastic condition became apparent in her gum tissue, the patient bled profusely, and the teeth began to exfolliate. Emergency surgery was performed in late February 1970. Ligation of the left external carotid artery was performed and some of the same thrombogenic material was injected directly into the AV fistula and also into the posterior palatine artery. Repeat right vertebral angiography performed two weeks later showed prominent occipital communications with deep cervical branches, with evidence of communication with the AV malformation. Right common carotid angiography showed numerous small squamous and cavernous branches of the internal carotid artery anastomosing with the residual ascending pharyngeal artery, which continued to perfuse the malformation. There was also feed via the ophthalmic and ethmoid vessels through an aberrant branch of the vertebral artery into the occipital artery and then retrograde into the internal maxillary artery. In view of these findings, urgent surgery was deemed necessary for control. At t,he time of surgery it was of interest that even with pressure control of the internal and
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Figure 3. Case I. Hypertrophied right opthalmic artery with new ma/formation.
common carotid arteries, there was no effect on the amount of bleeding. Right maxillectomy was performed in a routine manner despite brisk bleeding, and the postoperative recovery period was uneventful. The microscopic studies were of great interest, showing areas of new bone formation and also a lack of any endothelial lining of the large cavities within the bone. However, numerous fibroblasts and proliferating capillaries were found. The patient did very well over the ensuing years. In 1976 she presented with swelling of the right upper lip which did not demonstrate a bruit of pulsation. Repeat four-vessel angiograms showed recanalization of the right external carotid channel to approximately 1 mm diameter, giving rise to a small external maxillary artery that did not appear to supply the AV malformation. A very hypertrophied right ophthalmic artery supplying a new AV malformation was noted. (Figure 3.) The dorsal nasal branch anastomosed with the angular artery of the right external maxillary and extensively supplied the malformation. The ophthalmic lacrimal branches anastomosed with the zygomatic branches, which also ultimately entered the internal ‘maxillary artery via the deep temporal and transverse facial branches. The posterior ethmoidal branch of the ophthalmic artery anastomosed with the sphenopaiatine branch of the internal maxillary artery and supplied the infraorbital artery, which then entered the AV malformation. Left common carotid injection indicated the internal maxillary artery had recanalized and contributed to the malformation. The left labial branches directly cross-communicated with the right labial branches and then entered the malformation. (Figure 4.) The left vertebral artery demonstrated almost immediate opacification of the left occipital artery via deep muscular branches, and
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Figure 4. Case 1. Crossover of left labial artery.
the occipital artery then demonstrated a very prominent opacification of the internal maxillary, external carotid, and external maxillary vessels that subsequently supplied the malformation. There was drainage through the right superior ophthalmic vein into the cavernous sinuses as well as into the venous structures deep within the face. Resection which encompassed the AV malformation was performed without untoward bleeding. Repeat angiography one year later did not show any signs of the previous AV malformation. There was no evidence at this time of the large communicating channels between the vertebral and carotid systems, particularly on the left side. Two minutes after angiography, the patient developed bilateral cortical blindness, secondary to vertebral vasospasm, which cleared in two days. Case II. A thirty-two year old man was in good health until 1971 when he developed a mass in the hard palate. He had had left external carotid artery ligation performed elsewhere, and in 1972 he had a surgical cutdown of the left external carotid artery and catheterization with installation of 1.5 mm diameter barium-coated Silastic? balls. A total of seventy-eight such balls were placed with successive angiograms. It was believed there had been a minimum of 60 per cent reduction of the malformation. The x-ray films prior to this procedure showed a large internal maxillary artery on the left, greatly hypertrophied, filling a large AV malformation of the hard palate with enormous draining
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Figure 5. Case II. Large internal mation (arrows).
maxillary
Fistulas
artery malfor-
veins into the pharynx. He did well for two years and then presented with massive bleeding from the oral cavity around the teeth. Two months earlier repeat angiography had shown the blood supply to the malformation coming from the left internal maxillary artery which was being fed by multiple huge collaterals from the left vertebral artery. (Figure 5.) Additional circulation came from the left internal carotid artery through the ophthalmic artery and also from the right external carotid artery through the right internal maxillary artery. The surgical procedure performed was bilateral right external carotid artery skeletonization and removal of sections of the arteries to extirpate as much of the microfistula and as many collateral vessels as possible from the common carotid and vertebral arteries. Then, during the same procedure, maxillectomy, a very difficult procedure due in part to bone hypertrophy, was performed. Before surgery the patient had an audible noise in the left ear, which was consistent with the bruit that we could hear. The noise had decreased enormously after our procedure but was still present postoperatively. Repeat angiography eight days later showed a 2 X 1 cm vascular abnormality in the region of the pterygoid plates near the posterior wall of what had been the left maxillary antrum. (Figure 6.) It was believed that this residual area was causing the audible sound and probably should be eliminated. The supply appeared to be primarily from the left vertebral artery as well as from collateral branches of the right internal carotid artery through the occipital artery. The patient underwent reoperation, and the remainder of the pterygoid plate and the adjoining area of the base of the skull were removed without involving dura. The patient has had an uneventful three years and is continuing his business as an executive on a daily basis. One year later, repeat four-vessel studies showed two small AV malformations in the left pterygoid palatine fossa (Figure 7) supplied by collateral source from the left ophthalmic artery and left vertebral artery and a possible early AV
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Arteriovenous
Figure 6. Case II. Vascular abnormality goids.
in reglon of ptery-
malformation on the left posterior ethmoid region supplied via the ophthalmic artery. The patient has been asymptomatic, and there does not appear to be any other problem. Comments From our experience with these two cases and similar ones of the head and neck that we have treated, it is apparent that the unsolved problem is determining the extent of the microfistulas, not the collateral circulation. It has not been feasible to study this embryologic deficiency with the modalities we have available, and therefore, it is difficult to plan an adequate surgical resection. To obliterate the microfistulas by means of embolization would require use of a medium that could permeate the microcirculation. In our angiographic studies of the venous runoff of the maxillary arteries, the utilization of such a thrombogenic material may allow the runoff to be carried into the cavernous sinus and cause thrombosis or even retinal vein thrombosis. It is in this area of the microfistula that radiation therapy may have a very significant role, as it does have the ability to thrombose small vessels. At times we have seen laminated calcifications in vascular masses in the head and neck area. We have had one case of this entity present in the mandible and have given the presumptive diagnosis of nonfistulous or microfistulous vascular malformation to the patients who have presented with a phlebolith. (Figures 8 and 9.)
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Figure 7. Case II. New A V fistulas of left pterygoid palatine fossae.
Figure 9. Phleboltte (arrows) In maxilla.
In the three long-term follow-up cases we have not seen any symptomatic macrofistulas develop. Probably it is best not to subject this sort of laminated calcified area to a surgical procedure even though we did explore and remove the malformation from the mandible. If any macrofistula must be intimately enmeshed with microfistulas, similar to
44
Figure 8. Phlebollte in mandibular canal.
the phenomenon of field cancerization, then it is easier to understand the hemodynamics of the congenital AV malformation in the bony framework of the head and neck and why ligation of feeding vessels has the potential of lowering resistance in the malformation, which in turn can convert the surrounding microfistulas into macrofistulas. We agree with all that has been said before-that pure ligation of the vessels is absolutely contraindicated. We have seen anastomotic channels develop between vertebral, ascending cervical, deep cervical, occipital, and ascending pharyngeal arteries. The ophthalmic arteries have become involved bilaterally, forming a single large AV macrofistula involving all four major arterial flows to the skull and its contents. Luessenhop et al [15] in 1965 described the utilization of Silastic spheres for embolization; Djindjian et al [17] in 1973 mentioned that embolization did not preclude intervention in vascular tumors and suggested that it be used as a preparatory step to reduce the risk of hemorrhage. They also described the utilization of gel foam and muscle in embolization procedures, and in 1975, Olcutt et al [16] reinforced their conclusion. Our experience with embolization of maxillary macrofistulas makes it questionable whether excisional surgery is made safer or technically easier. We believe that if embolization is to be performed, it should be done bilaterally and if possible, by the Seldinger technic so as to obviate unnecessary ligation of vessels in the area of the microfistulas. If obliterative surgery is attempted, we believe that the
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vessels supplying the area, such as the external carotid artery and its branches, be removed rather than ligated. The question that has not been answered is the role of radiation therapy, and we are contemplating its utilization, depending on the age of the patient and the early visualization of a new macrofistula after extirpative surgery or embolization.
Summary Long-term follow-up is essential to evaluate our present modalities of treatment of arteriovenous malformations of the maxillae. Embolization followed by surgical resection has produced long-term survival in our patient population.
References 1. Dandy W: Atieriovenous aneurysm of the brain. Arch Surg 17: 190, 1928. 2. Horton B: Hemihypertrophy of extremities associated with congenital arteriovenous fistula. Co// Papers Mayo C/in 23: 650, 1931. 3. Szilagyi E, Elliott J, De Russo F, Smith R: Peripheral congenital arteriovenous fistulas. Surgery 57: 61, 1965. 4. Wollard R: The development of the principal arterial stems in the forelimb of the pig. Contributions to Embryology, Carnegie Institute, Washington 14: 139, 1922. 5. DeTakats G: Vascular anomalies of the extremities. Surg Gynecol Obstet 55: 227, 1932. 6. Sabin F: Origin and development of the primitive vessels of the chick and the pig. Contributions to Embryology, Carnegie Institute, Washington 6: 61, 1917. 7. Reid M: Abnormal arteriovenous communications acquired and congenital. Arch Surg 10: 996, 1925. 8. Rappaport I, Yim D: Congenital arteriovenous fistulas of the head and neck. Arch Otolaryngol97: 350, 1973. 9. Holman E. Contributions to cardiovascular phvsiology gleaned from clinical and experimental observations of abnormal atteriovenous communications. J Cardiovasc Surg 3: 48, 1962. 10. Malan E, Azzolini A: Congenital arteriovenous malformations of the face and scalp. J Cardiovasc Surg g(2): 109, 1988. 11. Coleman C, Hoopes J: Congenital arteriovenous anomalies of the head and neck. Plast Reconstr Surg 47: 354, 1971. 12. Coleman C: Diagnosis and treatment of congenital arteriovenous fistulas of the head and neck. Am J Surg 126: 557, 1973. 13. Watson W, McCarthy W: Blood and lymph vessel tumors. Surg Gynecol Obstet 71: 569, 1940. 14. Brown D, Sadek K, Robinson M: Arteriovenous malformations of the mandible, maxilla, orbit and cerebrum. J Oral Surg 31: 553, 1973. 15. Luessenhop A, Kachmann R, Shevlin W, et al: Clinical evaluation of artificial embolization in the management of large cerebal arteriovenous malformation. J Neurosurg 23: 400, 1965. 16. Olcutt C, Newton T, Stoney R, Eprenfeld W: Intra-arterial embolization in the management of atteriovenous malformations. Surgery 79: 3, 1976. 17. Djindjian J, Cophignon J. Theron J. Merland J. Houdart R: Embolization by super selective arteriography from the fermoal route in neuroradiology. Review of 60 cases. fJeworsdiology 8: 20, 1973.
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Discussion Herbert J. Movius, II (Long Beach, CA): Doctor Rappaport has presented a very serious and perplexing problem created by angiodysplasias when they involve the head, particularly the maxilla. Because of their proclivity to hemorrhage, they are more lethal than most angiodysplasias which occur in other places in the body. Nevertheless, wherever they occur, they are tremendous problems. They are always a challenge to manage. My experience is relatively limited to attempts to reduce morbidity due to malformations of the extremities and of the pelvis. Surgical excision of the malformation, attempts to reduce blood flow through the malformation, and amputations have been used and have largely proved unsatisfactory. Radiation also has been used with mixed results, although with newer technics it may actually prove to be an acceptable modality. For a brief period flow may be reduced, and results of a finishing angiogram may be optimistic when as many as possible of the feeding vessels to the fistula are found, ligated, and excised. However, the tremendous potential for collateral development and the opening of new fistulas existing not at the time of the finishing angiogram but three weeks or months later, whatever their origin, results in early return of symptoms. I have had experience with one very large, primary venous angioma that involved the chest wall with seven or eight ribs, held approximately 2 quarts of blood, and could not be excised primarily because of its size and position but was totally and permanently eradicated by numerous injections using sclerotherapy. However, I have used this on other AV malformations without good results. As Szilagyi et al [3] have pointed out, many AV fistulas, whether micro- or macrofistulas, resulting from angiodysplasia will not be demonstrated on arteriography, as in the so-called Klippel-Trenaunay-Weber syndrome. If they occur during the growing period of the individual, they result in tremendous overgrowth of the extremity, which is sometimes extremely disabling. Procedures aimed at arresting growth of the enlarged extremity at the time the other extremity, continuing to grow, approximates the size of this overgrown extremity when it stops growing are helpful. If such a procedure cannot be performed-frequently the patient is not seen in time-then bone-shortening operations are recommended, particularly in the lower extremity, with femural and transmetatarsal amputation of the foot resulting in two extremities that are not too dissimilar and function well. I have performed this operation on two patients, both of whom ski and dance and have very little morbidity from the entire process. Cardiac overload eventually resulting in heart failure may be a serious problem, and it demands prompt management at times. This can occur in the neonatal period. In the past it has been solved by extremity amputation, usually as an emergency, after some less radical flow-limiting procedure has been tried. It is usually successful but, is a horrible thing to do to a newborn infant.
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However, the promising modality of embolization, the study of which has only begun, hopefully will lead to much better management in the future than we have so far been able to accomplish, especially with highly selective angiography and ingenious new embolization technics. Morton M. Woolley (Los Angeles, CA): I will limit my comments to a few of the problems seen in infants and children with macrofistulas. I should like to illustrate with two brief cases. The first is an angiographic study performed on a two. and a half year old boy because of a pulsatile mass in the left neck. This angiogram reveals a large macrofistula between the carotid system and an anomalous vein. At surgery, the external carotid artery was extremely large in comparison with the normally sized internal carotid artery. There was no internal jugular vein, a fact which has been previously reported in association with this type of anomaly. The superior thyroid, lingual, and ascending pharyngeal arteries were divided, and as much of the malformation that could be removed safely was excised. (Slide) Five years later, this angiogram revealed multiple small AV fistulas in the neck. (Slide) Although the patient has a visible and palpable mass in association with an audible bruit, he is an active boy, competes in athletics, and attends school regularly. His parents are quite resistant to any further invasive diagnostic procedures or therapy because of his good general health. I believe they are quite right since he is not symptomatic. Doctor Movius referred to the problem of bleeding from macrofistulas, and I should like to illustrate how severe bleeding can be from an extremity AV macrofistula. (Slide) This is a photograph taken of the arm of a newly born infant admitted with congestive heart failure, because of which a large pulsatile artery was ligated without antecedent angiography. (Slide) An angiogram after ligation revealed additional feeding arteries, which were ligated, and the congestive heart failure was controlled. (Slide) The patient survived an episode of sepsis and was sent home, but bled to death one night when a crust spontaneously separated from the surface of the hemangioma. One must realize the bleeding potential of some arteriovenous fistulas and the benign symptoms of others. Judgment dictates when considering the appropriate therapy for such patients. William K. Ehrenfeld (San Francisco, CA): At the University of California, San Francisco, we have increasingly advocated conservative management of AV malformations. The management is now primarily embolization rather than surgery. At this time we have had experience
46
with six patients who have had embolization of AV malformations of the face and neck. (Slide) Shown are some of the patients in our series before and after embolization. In several patients, the external carotid artery has been ligated. This surgery, unfortunately, is associated with only short-lived relief for the patient. The malformations usually recur very soon after ligation, and the feeding vessel is no longer available for embolization. Our technic is to use the percutaneous approach, embolizing through the catheter with the Seldinger technic. Embolization is often carried out from both sides using each external carotid artery. In addition, it is not uncommon for the patient to require more than one session of embolization. This technic lends itself to repeat sessions which can be separated by months or years. We do not believe that embolization necessarily is curative, but it does supply palliation. One patient who had an AV malformation involving the left lower jaw has gone two years since embolization, and the bruit over the mass has not recurred. Shortly after embolization, numerous “floating” teeth were removed with only minor bleeding. The remaining patients have been controlled, some of whom required more than one embolization session. Irving Rappaport (closing): I would like to emphasize that we obtained follow-ups on cases throughout the country which were reported in the literature. We were amazed at how little follow-up was done in these cases. It is not a matter of collaterals in the area we are particularly interested in-bony structure of the head and neck-but a matter of congenital microfistulas. The recurrences we are seeing confined to bone are primarily from the latter. I do not believe that embolization of large spheres is as effective with microfistulas as the technic of injected thrombogenic material, which was first discussed in 1967, soon after Luessenhop [IS] performed his basic technics intracranially. However, the problem in the maxilla is that the ophthalmic vein and other vein systems that go directly into the saggital sinuses are involved. Therefore, the question is with how small an embolus can we manage the microfistulas and not have a problem intracranially. This problem has not been solved. Therefore, we are looking toward radiation therapy, because some of the carotid body tumors that have been unresectable-and occasionally some are thoroughly unresectable in the elderly-have been palliated with additional supervoltage therapy. To my knowledge, including the literature throughout the world, supervoltage therapy has not been discussed in the management of microfistulas. The only thing we could find was orthovoltage utilized many, many years ago, the judicious use of which may be of some value in small microfistulas. The question is when to do it, and what are the contraindications.
The American Journal of Surgery