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Home , Edema, Stasis dermatitis

Cutaneous Manifestations of Abdominal Arteriovenous Fistulas

Jessica Scruggs, MD; Daniel D. Bennett, MD

Abdominal arteriovenous (A-V) fistulas may be .1-3 We report a case of abdominal aortocaval spontaneous or secondary to trauma. The clini- fistula presenting with lower extremity edema, ery- cal manifestations of abdominal A-V fistulas are thema, and that had been previously diag- variable, but cutaneous findings are common and nosed as venous stasis . may be suggestive of the diagnosis. Cutaneous findings consistent with Case Report abdominal A-V fistula include lower extremity A 51-year-old woman presented to the emergency edema with cyanosis, pulsatile varicose , department with worsening lower extremity swelling, and scrotal edema. redness, and pain. Her medical history included a We present a patient admitted to the hospital diagnosis of congestive failure, chronic obstruc- with lower extremity swelling, discoloration, and tive pulmonary disease, hepatitis C virus, tobacco pain, as well as renal insufficiency. During a prior abuse, and polysubstance dependence. Swelling, red- hospitalization she was diagnosed with venous ness, and pain of her legs developed several years stasis dermatitis; however, CUTISher physical examina- prior, and during a prior hospitalization she had been tion findings were not consistent with that diagno- diagnosed with chronic venous stasis dermatitis as sis. Imaging studies identified and characterized well as neurodermatitis. an abdominal aortocaval fistula. We propose that On admission, the patient had cool lower extremi- dermatologists add abdominal A-V fistula to the ties associated with discoloration and many crusted differential diagnosis of patients presenting with ulcerations. Aside from obesity, her abdominal exam- lower extremity edema with cyanosis, and we ination was unremarkable and no bruits were noted. summarizeDo other physical examinationNot and labo- Her creatinineCopy level was 2.9 mg/dL (reference range, ratory findings that may suggest the diagnosis. 0.6–1.2 mg/dL) and her urea nitrogen level was Cutis. 2011;87:284-286. 63 mg/dL (reference range, 8–23 mg/dL). She had no leukocytosis, and her erythrocyte sedimentation rate and C-reactive level were within reference bdominal arteriovenous (A-V) fistulas often range. Her alkaline phosphatase level and transami- present with cutaneous findings in the lower nases were minimally elevated. A toxicology screen A extremities that may come to the attention of was positive for cocaine. Deep was a dermatologist. Although the clinical presentation ruled out via negative lower extremity ultrasonog- of abdominal A-V fistulas varies, cutaneous findings raphy. Doppler evaluation of her lower extremities are common and can include lower extremity edema revealed normal pulses, but she did not tolerate an with cyanosis, pulsating , and scrotal attempt to obtain blood pressures secondary to pain. Cardiac echocardiography demonstrated an ejection fraction of 65%, bilateral atrial enlarge- Dr. Scruggs is from and Dr. Bennett was from the Department of ment, high right-sided filling pressure, and a normal- , Texas A&M College of Medicine, Temple, and Scott & appearing left ventricle. Abdominal ultrasonography White Memorial Hospital, Temple. Dr. Bennett currently is from the revealed hepatomegaly as well as a prominent inferior Department of Dermatology, Hospital of the University of vena cava (IVC) and hepatic veins. Renal ultraso- Pennsylvania, Philadelphia. nography was normal. The authors report no conflict of interest. Correspondence: Daniel D. Bennett, MD, Department of The dermatology department was consulted to Dermatology, Hospital of the University of Pennsylvania, 3600 Spruce evaluate her findings. The patient reported lower St, 2 Maloney Bldg, Philadelphia, PA 19104. extremity skin pain and denied picking or scratching

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the lesions on the legs. On examination of the lower extremities, she had livedoid change and striking purplish erythema with sharp demarcation on the upper thighs (Figure 1). Her legs were cool to the touch. Slow refill was noted on examination of her . In addition, she had multiple punched out ulcers with yellowish crusts and scattered small pustules. The patient also had excoriated crusted papules on the arms and . Some livedoid change and coolness of the skin was noted on the elbows and hands. No purpura was noted anywhere Figure 1. Erythema, edema, and cyanotic livedoid patches involved the legs. Note the abrupt color change on examination. Based on the physical examination on the upper thighs. Multiple punched out and crusted findings, a vasculopathic process was suspected, but ulcers also were present. was deemed to be unlikely. Secondary infec- tion and colonization of her wounds was confirmed by swab cultures that grew Staphylococcus aureus, coagulase-negative staphylococci, Acinetobacter lwoffii, and Fusarium species. A skin biopsy revealed focal ulceration with acute and epi- dermal hyperplasia with hyperkeratosis suggestive of excoriation and/or trauma. cultures for acid- fast and fungal organisms were negative. Testing for cryoglobulins, classical and perinuclear antineutro- phil cytoplasmic antibodies, histoplasma antigen, and antinuclear antibody was negative. Magnetic resonance angiogram revealed bilater- Aorta ally enlarged common iliac CUTIS veins with megacava, Inferior and there was venous contamination of the lower vena cava extremities suggesting a fistulous communication. A subsequent abdominal aortogram (Figure 2) showed contrast that immediately filled the IVC consistent with a fistula between the distal aorta and the IVC as Figure 2. An abdominal aortogram showed contrast that well as a fistula between the right common iliac immediately filled the inferior vena cava. and Doright common iliac vein. Not Copy Surgical intervention was delayed because of multiple comorbid conditions including Stenotrophomonas bacteremia, endocarditis, and an fistula, similar clinical findings also may be expected episode of septic shock necessitating mechanical with large fistulas involving the abdominal iliac ves- ventilation and aggressive resuscitation. With wors- sels. Abdominal A-V fistulas between the iliac artery ening of her renal function, she was deemed to be a and iliac vein are rare and more frequently result poor surgical candidate. The patient died shortly after from trauma.4 hospice placement. The clinical presentation of abdominal A-V fis- tula is variable; therefore, the diagnosis may be over- Comment looked. Abdominal pain with a pulsatile abdominal Most abdominal aortocaval fistulas are spontaneous, mass, a machinelike abdominal bruit, and acute dysp- resulting from the erosion of an atherosclerotic, aor- nea are believed to be pathognomonic for abdomi- tic, or iliac into an adjacent vein. Sponta- nal A-V fistulas.2 Some cases present without typical neous abdominal aortocaval fistulas also have been findings. Other include back reported to result from syphilitic and mycotic aneu- pain; high-output cardiac failure; renal failure; sys- rysms, as well as from Marfan syndrome, temic ; and signs of venous hyperten- Ehlers-Danlos syndrome, and Takayasu .2 sion including scrotal edema, pulsating varicosities, Traumatic abdominal A-V fistulas may occur follow- priapism, hematuria, and rectal bleeding.1,3 In a ing penetrating and blunt trauma in addition to iatro- discussion of the diagnosis of abdominal A-V fistulas, genic injury during diagnostic or surgical procedures.1 Gilling-Smith and Mansfield3 referred to “the triad of Although our patient was found to have an aortocaval machinery murmur, high output cardiac failure and

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regional venous ” and noted that it was also were clues to the diagnosis. Her renal insuffi- present in only 25 of 148 cases they reviewed. In the ciency also was likely due to her A-V fistula. same review, leg edema was noted in 53 patients and Given the prevalence of cutaneous findings in cyanosis in 34.3 Another review found lower extrem- abdominal A-V fistula, dermatologists are likely to ity edema with mottling and cyanosis to be the pri- encounter similar patients, both as inpatients and out- mary manifestation in 8 of 20 patients. These authors patients. We propose that dermatologists add abdomi- also described frequent findings of cyanotic congested nal A-V fistula to the differential diagnosis of patients lower extremities that contrasted with cool pale upper presenting with edematous lower extremities, espe- extremities and trunk.2 cially when associated with cyanosis and symptoms Prompt diagnosis is desirable and treatment is of . Physical examination and laboratory prompt surgical repair. Unfortunately, the diagno- findings are highly variable; therefore, a high index of sis often is missed or delayed. In one review, the suspicion must be maintained and appropriate studies diagnosis was not made prior to surgery in 38% of and consultations must be obtained urgently. cases.1 In another review, the diagnosis was made intraoperatively in 25% of cases.2 Multiple radio- REFERENCES graphic studies may be helpful in the diagnosis and 1. Cinara IS, Davidovic LB, Kostic DM, et al. Aorto-caval localization of abdominal A-V fistulas. Abdominal fistulas: a review of eighteen years experience. Acta Chir color Doppler ultrasonography, computed tomogra- Belg. 2005;105:616-620. phy, and magnetic resonance imaging are noninvasive 2. Brewster DC, Cambria RP, Moncure AC, et al. Aortocaval and often sufficient.5,6 Angiography may be useful and iliac arteriovenous fistulas: recognition and treatment. if the diagnosis is unclear or if more detail is neces- J Vasc Surg. 1991;13:253-264. sary prior to surgery.2,6 Multidetector-row computed 3. Gilling-Smith GL, Mansfield AO. Spontaneous abdomi- tomography and virtual angioscopy also have been nal arteriovenous fistulae: report of eight cases and review suggested to delineate the anatomy of a fistula prior of the literature. Br J Surg. 1991;78:421-425. to surgery.7 4. Gregoric ID, Jacobs MJ, Reul GJ, et al. Spontaneous com- Our patient’s chronic course, obesity, and his- mon iliac manifested by acute renal tory of substance abuse all likelyCUTIS contributed to the failure: a case report. J Vasc Surg. 1991;14:92-97. delayed diagnosis of abdominal aortocaval fistula. The 5. Lin PH, Bush RL, Lumsden AB. Aortocaval fistula. J Vasc lack of eczematous changes or evidence of hemosid- Surg. 2004;39:266. erin deposition militated against a diagnosis of stasis 6. Takaseya T, Hiromatsu S, Akashi H, et al. A case of dermatitis. We found the purplish color of her skin to unilateral leg edema due to abdominal be suggestive of vascular compromise, and we hypoth- with aortocaval fistula. Ann Thorac Cardiovasc Surg. esized that the sharp demarcation noted on the upper 2007;13:135-138. thighDo was suggestive of involvement Not of the deep ves- 7. SalernoCopy S, Romano I, De Luca T, et al. MDCT and virtual sels of the leg. Our patient’s echocardiographic find- angioscopy in spontaneous aortocaval fistula [published ings of high right-sided filling pressure and abdominal online ahead of print September 29, 2006]. Int J Cardiovasc ultrasonography findings of enlarged abdominal veins Imaging. 2007;23:635-638.

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