Cystic adventitial disease

CYSTIC A D V E N T I T I A L

DISEASE

John D. Terry, M.D.,* Jerald R. Schenken, M.D.? Martin R. Lohff, M.D. $ and Delbert D. Neis, M.D. w

Abstract Cystic adventitial disease represents an unusual cause of peripheral vascular insufficiency. A mucinous cyst in the outer media or adventitia progressiveIy compromises the arterial lumen. T h e therapy can be either aspiration o f the cyst or resection with graft interposition. We present two cases involving the popliteal artery that were treated by resection. This lesion may be recognized by its angiographic appearance associated with a lack of generalized atherosclerotic disease.

Localized cystic degeneration of peripheral arteries represents an unusual cause of arterial insufficiency. It frequently occurs in patients without generalized arteriosclerosis. It may occur at almost an)' age and has been reported in patients from age 11 to 62 years, z T h e lesion consists of a cystic space within the outer portion of.the arterial media or, more c o m m o n ly, the adventitia. T h e material within the cyst progressively increases in volume, causing the cyst to develop sufficient size to compromise the arterial lumen. Cystic adventitial disease is most c o m m o n in the popliteal artery. At least 115 cases have been reported worldwide, 13 in the United States. 8 It has been described in locations other than the popliteal artery, including the c o m m o n femoral and external iliac arteries. 2' We report two cases. CASE REPORTS CASE 1

A 42 )'ear old white man was seen in 1972 with a two m o n t h history of exercise induced pain a n d

cramping in the left calf associated with numbness and tingling. T h e onset o f symptoms occurred after walking two to three blocks; symptoms were consistently relieved by rest. He was free o f other complaints. T h e medical history included moderate cigarette smoking and mild labile hypertension, u n d e r treatment with chlorothiazide. Physical examination revealed an essentially normal but slightly obese male except for findings in the left leg. T h e left femoral pulse was strong, but the left popliteal, left dorsalis pedis, and left posterior tibial pulses were all significantly diminished. Arteriography revealed smooth tapered stenosis of the left popliteal artery, which reduced the caliber to approximately one-third to one-fourth that of the uninvolved vessel. T h e patient u n d e r w e n t exploration o f the left popliteal artery d u r i n g which a fusiform enlargement of the vessel with a gelatinous appearance was noted. Needle aspiration p r o d u c e d gelantinous material. T h e affected portion of the vessel was then isolated and resected and replacement was effected with a saphenous vein graft. Pathologic examination of the specimen d e m o n s t r a t e d a cystic dilation of the outer portion o f the media compressing the otherwise nor-

Accepted for publication July 1, 1980. *Resident, Department of Diagnostic Radiology, University of Nebraska College of Medicine, Omaha, Nebraska. tProfessor, Department of Pattlolo~-, University of Nebraska College of Medicine. Director, Department of Pathology, Methodist Hospital and Children's Memorial Hospital, Omaha, Nebraska. :[:Associate Professor, Departnlent of Pathology, University of Nebraska College of Medicine. Associate Pathologist, Metlmdist ttospital-and Children's Memorial Hospital, Omaha, Nebraska w Professor, Department of Surgery, Uliiversityof Nebraska College of Medicine. Active Staff, Methodist ttospltal and Clarkson Hospital. Courtesy Staff, Children's Memorial ltospital, lmnmnuel Hospital, Bergan Mercy Hospital,Jennie Edmundson Hospital, Mercy Hospital, Midlands Community Hospital, and Lntheran t[ospital, Omaha, Nebraska HUMAN PATItOLOGY--VOLUME 12, NUMBER 7 July 1981

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mal media and intima. Postoperative pulses were weak but improved over four to five days. Five weeks later the patient was readmitted with a four week history of recurrent claudication. An arteriogram revealed obstruction of the graft. He was reoperated upon and a new graft was placed. He returned six months later because of more claudication. An arteriogram revealed narrowing of tile anterior and posterior tibial branches with an enlarged peroneal branch. Exploration demonstrated a stricture of the distal portion of the vein graft. Resection of tile localized stricture with reanastomosis was performed, including an endarterectomy of tile anterior tibial artery. Postoperatively the pulses were improved and an arteriogram revealed a patent graft site. Tile patient remained asymptomatic at tile time of this writing.

Figure 2, This cross section of the artery shows that the lumen is compressed (arrow) by a cystic advcntitial mass containing clear gelatinous material.

CASE

A 51 )'ear old white man was admitted with a two week history of bilateral calf pain associated with exercise. Pain occurred after less than a one block walk and was accompanied by numbness and tingling of the lateral three toes of each foot. He was a one package per day smoker without a histor), of claudication. The medical history included removal of a renal stone two months earlier. On physical examination the patient was obese and slightly hypertensive (154/84). Both femoral pulses were minimally dimin-

Figure 1. Femoral-popliteal arteriogram. Note the smooth compression type of defect at the most proximal portion of the artery (arrow, top).

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ished. Pedal pulses were normal on tile right and minimally diminished on the left. The skin over the lower extremities was normal. The calves were mildly tender to deep palpation. An aortogram revealed minimal atheromatous change in the abdominal aorta. T h e right leg showed narrowing of the popliteal artery at the level of tile femoral condyles (Fig. 1). The lumen was smoothly compressed to 50 per cent of the diameter of the uninvolved vessel. No irregularity of the intimal surface was seen. During exploration of tile right popliteal artery a fusiform enlargement was noted and found to be cystic. Resection of the involved vessel was performed and a segment of saphenous vein was inserted. Pathologic examination showed a cystic space between tile outer media and the adventitia filled with mucinous material (Fig. 2). The intima and media were essentially intact. Postoperatively pulses were adequate. Microscopic examination revealed a cystic space in tile arterial adventitia, which was unlined and filled with amorphous amphophilic material. T h e material was PAS negative, mucicarmine negative, a n d alcian blue positive (pH 2.5), reverting to negative following exposure to hyaluronidase (Figs. 3, 4). Since the time of discharge, his right great toe had felt cold to him. Four days prior to readmissiou the toe became painfld and developed a reddish hue. The patient was readmitted to the hospital six weeks following the previous discharge and was found to have. normal popliteal pulses bilaterally but markedly decreased pulses in the i'ight dorsalis pedis and posterior tibial arteries. Arteriography demonstrated complete occlusion of tile distal superficial femoral artery at tile site b f prior surgery. This section was removed and replaced by another vein graft. The puIses promptly returned. Histologic examination revealed thrombosis with earl), organization. Some minimal atheromatous changes were present but no cystic change. T h e patient has remained asymptomatic.

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DISEASE-

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Figure 3.

T h e cystic space in tile center is present ill tile adventitia. T h e arterial lumen is compressed on the far right (arrow). ( x

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Figure4, Tile artery shows slight i,uiuml artherosclerosis (top left). The media ks intact. The cyst is present at the junction between the media and adventitia (right). (•

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HUMAN PATHOLOGY--VOLUME 12, NUMBER 7 July 1981

DISCUSSION Case 1 represents a typical presentation o f cystic adventitial disease o f the popliteal artery. T h e patient had no evidence o f generalized arterial disease. H e had a r a t h e r a b r u p t onset o f typical claudication with a resting pulse deficit. T r e a t m e n t with a vein graft was followed by complications that r e p r e s e n t the risks i n h e r e n t in vein grafting at this site. Case 2 is atypical in that the complaints were .bilateral, the onset in each leg o c c u r r i n g at a b o u t the same time. We are not aware that bilateral s y m p t o m s in cystic adventitial disease have been noted previously. T h e r e was little arteriographic evidence o f disease in the patient's left leg. H o w e v e r , symptomatic claudication has been seen in patients with cystic adventitial disease and essentially n o r m a l arteriograms. 13 T h e contents o f the cyst grossly resembled thick mucinous gel. Biochenhical analyses o f the contents o f such cysts have b e e n inconclusive, s T h e i n f o r m a t i o n available suggests that mucopolysaccharide p r e d o m i nates associated with hyaluronic acid, similar to g r o u n d substance. 5,8 In the absence o f s e c o n d a r y thrombosis or pressure necrosis d u e to cyst enlargement, the media and intima are u n a f f e c t e d by the process. T h e s e changes do not resemble those seen in cystic medial necrosis. Cystic medial necrosis is often generalized, p r e d o m i n a t e s in the aorta, is often seen with Marfan's s y n d r o m e , p r o d u c e s microscopic cysts in most cases, and p r o d u c e s multifocal changes. N o n e o f these were n o t e d in o u r cases. Clinically the disease presents with exertional c r a m p i n g in the calf, which later develops into typicall), intermittent claudication. T h e onset may be gradual but m o r e often tends to be s u d d e n a n d o f r a t h e r short duration. Ischemic skin changes are generally absent and g a n g r e n e does not appear. Physical examination usually reveals diminished pulses in the affected leg, o f t e n accentuated by exercise. Flexion o f the knee may also p r o d u c e a decrease in o r loss o f pulse. A r t e r i o g r a p h i c changes are characteristic. A smooth t a p e r e d n a r r o w i n g o f the popliteal a r t e r y is d e m o n s t r a t e d usually just above the knee. In cases o f complete obstruction o r thrombosis the gradual smooth tapering may still be p r e s e n t (the socalled "scimitar" sign) o r t h e r e may be no arteriographic clue. "-'4 In the case o f s u d d e n onset the differential diagnosis includes arterial embolizatiou. Arteriosclerotic a n e u r y s m with thrombosis m a y b e similar clinically. E n t r a p m e n t o f the popliteal a r t e r y may be considered, althougla the a r t e r y is medially deviated in these cases. Rarely a Baker cyst may compress the popliteal artery. T r e a t m e n t in the previously r e p o r t e d cases has been divided into techniques involving resection and r e p l a c e m e n t and techniques that release the contents o f the cyst with o r without a r t e r i o t o m y to verify the absence o f f u r t h e r disease. Resection d o n e in 42 previous cases has d e m o n s t r a t e d a 93 p e r cent initial

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success rate, with an occasional long term complication o f the p r o c e d u r e , s T h e success rate with n o n r e sectional m e t h o d s has been a p p r o x i m a t e l y 89 p e r cent initially, with some failures secondary to t h r o m bosis at a r t e r i o t o m y sites. Cyst r e c u r r e n c e has occ u r r e d in a p p r o x i m a t e l y 10 p e r cent o f the cases, s T h e c u r r e n t r e c o m m e n d a t i o n for evacuation o f the cyst contents without resection seems reasonable except w h e n t h e r e is evidence o f thrombosis, or pressure necrosis o f the i n n e r portion o f the vessel. -"a, 68, ~0, ~4-~G,~8, ~9,2~, z2, 24 Some advocate evacuation o f the cyst contents in all cases, reserving resection for failures? Occasional r e c o m m e n d a t i o n s for routine excision and grafting are f o u n d ) 3 T h e r e are n u m e r o u s theories about the cause o f this condition. Most are based on the similarity o f these cysts to c o m m o n ganglia, but no real evidence is available to s u p p o r t this relationship. T h e c o n c e p t o f an embryologic origin is p o p u l a r t h o u g h u n s u p p o r t ed. At this point t h e r e does not appear-to be a single s u p p o r t e d explanation f o r the unusual condition.

REFERENCES I. Atkins, H. J. B., and Key, L. A.: A case of myxomatous tumor arising in the adventitia of the left external iliac artery. Brit. J. Surg.,34:426, 19-t7. 2. Bliss, B. P.: Cystic myxomatous degeneration of the popliteal artery. Am. Heart J., 68:838, 1964. 3. Bliss, B. P., Rhoades, J., and Harding-Rains, A. J.: Cystic myxomatous degeneration of the popliteal artery. Brit. Med. J., 2:847, 1963. 4. Chandler, J. j.: Popliteal artery occlusion by subadventitial pseudocyst. Surgery, 74:456, 1973. 5. Eastcott, H. H. G.: Cystic myxomatous degeneration of the popliteal artery. Brit. Med. J., 2:1270, 1963. 6. DeLaurentis, D. A., et al.: Mucinous adventitial cysts of the popliteal artery in an ll-year-old girl. Surgery, 74:456, 1973. 7. FIanc, C.: Cystic degeneration of the popliteal artery. Aust. N.Z.J. Surg., 36:243, 1967. 8. Flanigan, D. P., Burnham, S.J., Goodreau,J.J., and Bergan,J. J.: Summary of cases of adventltial cystic disease of the popliteal artery. Ann. Surg., 189:165-175, 1979. 9. Haid, S. P., Conn, J., Jr., and Bergan, J. J.: C)sti~ adventifial disease of the popliteal artery. Arch. Surg., 101:765, 1970. 10. Harris, j. D., and Jepson, R. P.: Cystic degeneration of the popliteal artery. Aust. N.Z.J. Surg., 34:265, 1965. 11. Hierton, T., Lindburg, K., and Rob, C.: Cysticdegeneration of the popliteal artery. Brit. J. Surg., 44:348, 1957. 12. Holmes, J. G.: Cystic adventitial degeneration of the popliteal artery. J.A.M.A., 173:654, 1960. 13. Hudson, R. E. B.: Cardiovascular Pathology. Baltimore, The Williams & Wilkins Co., 1970, Vol. 3, p. S-326-327. 14. Lambley, D. G.: Intermittent claudication due to cystic degeneration of the popliteal artery~ Brit. Med. J., 2:849, 1963. 15. Lau, J., Kim, H. S., and Carcia-Rinaldi, R.: Cystic adventitial disease of the popliteal artery. Vasc. Surg., 11:299-303, 1977. 16. Lead article. Cystic degeneration of the popliteal artery. Brit. Med. J., 4:699, 1970. 17. Leaf, G.: Amiqo acid analysis of protein present in a popliteal artery cyst. Brit. Med. J., 3:415, 1967. 18. Lewis, G. J. T., Douglas, D. M., Reid, W., and Watt, J. K.: Cystic adventitial disease of the popliteal artery. Brit. Med. J.,3:411, 1967.

AUTOFLUORESCENCE IN IDENTIFICATION OF MYOCARDIAL INFARCTS I CARLE 19. Little,J. M., and Goodman,A. H.: Cysticadventitialdisease of the popliteal artery. Brit.J. Surg., 57:708, 1970. 20. Milliken,J. C.: Cysticdegenerationof the popliteal artery in a female. Brit. Med.J., 2:769, 1971. 21. Powis, S. J. A., Morrissey, D. M., and Jones, E. L.: Cystic degeneration of the popliteal artery. Surgery, 67:891, 1970.

22. Roth,J. A., Hearney, P., and Wittmann,C.J.: Cysticadventitial degeneration of the common femoral artery. Arch. Surg., 112:210-212, 1977. 23. Savage,P. E. A.: Cysticdisease of the poplitealartery. Brit.J. Surg., 56:77, 1969. 24. Schlesinger,A., and Gottesman,L.: Cysticdegenerationof the popliteal artery. Am. J. Roentgenol.,127:1043, 1976. Box 14424 Omaha, Nebraska 68114 (Dr. Schenken)

A U T O F L U O R E S C E N C E IN T H E I D E N T I F I C A T I O N OF MYOCARDIAL INFARCTS Birdsall N. Carle, M . D . * Abstract A method is described for demonstrating myocardial necrosis by changes in the autofluorescence of routine hematoxylin and eosin or hematoxylin and eosin-phloxine stained sections using a standard microscope with an epifluorescence attachment. Regions of necrosis fluoresce with a brilliant yellow color clearly distinguishable from the dull olive-green to red-brown fluorescence of the neighboring viable cardiac muscle.

Well established myocardial infarcts are not difficult to identify in routine tissue sections stained with hematoxylin and eosin. However, some suspected infarcts, particularly those of recent 6rigin, present a diagnostic problem. The morphologic changes in the myocardium may be so subtle that it is often difficult or impossible to determine whether tile microscopic appearance represents true necrosis, some other lesion, or some vagary of staining. This is a common question in atttopsy examinations. A simple teclmique utilizing autofluorescence of necrotic heart muscle in standard hematoxylin and eosin sections tins proven very usefltl. Many teclmiques lmve been devised to identify cardiac necrosis and particularly earl}, necrosis. These have included teclmiques to demonstrate fat change in the myocardium, fuchsinoplfilia in earl}, infarction, acid hematin staining, a comparison of fuchsinophilia and fuchsinorrhagia, basic fitchsin staining, and hematoxylin-basic fuchsin-picric acid staining) -7 Histochemical methods relating to glycogen have beel~ examined as well as alterations in succinic dehydrogenase, cytochrome oxidase, phosphorylase, ttridine diphosptmte gh|cose-glycogen transferase, /3hydroxybntyrate dehydrogenases, and isocitrate dehydrogenases, sv' Enzyme decay curves (glutamic

oxaloacetic transaminase, glutamic pyruvic transaminase, lactic acid dehydrogenase) have been compared in normal and infarcted hearts. ~'~ Changes in the sodium to potassium ratio within the myocardium have been reported as being perhaps the most reliable measurement for detecting earl}, infarction, la' la Fluorescence of gross specimens of experimentally infarcted rabbit hearts following antemortem injection of tetracycline has been studied as well as macroscopic identification of earl}, myocardial infarcts by alterations in dehydrogenase activity) ~ ~7 Electron microscopic study of ischemic rat myocardium has been reported) s Most of these methods are summarized in tim World Heahh Organization Scientific Group Report on the Pathologic Diagnosis of Acute Ischaemic Heart Disease? 9 Unfortunately these various attempts have as yet failed to produce a generally accepted method for demonstrating tile subtle changes of earl}, myocardial necrosis. This article presents a method that promises to be of.value in making tiffs determination. Many substances, including stained tissue sections, may exhibit characteristic atttofluorescence under ultraviolet light. While examining a variety of tissues with a fluor~xscence microscope it became apparent that bematoxylin and eosin stained sections

Accepted far publicationJuly I, 1980. *Chief, Laboratoryand PathologyScience, Veterans Administration MedicalCenter, Roseburg, Oregon, HUMAN PATIIOLOGY--VOI.UME 12, NUMBER 7 Jul)' 1981

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