Recurrent Cervical Carcinoma Presenting as a Primary Aortoduodenal Fistula
Article Outline
Aortoduodenal fistula is a rare cause of gastrointestinal (GI) bleeding, and carries high morbidity and mortality even in modern practice. Cervical carcinoma is a major health threat among adult women, and its recurrence is not uncommon. We herein present a case of primary aortoduodenal fistula because of recurrent cervical carcinoma. Our case demonstrated that diagnosis of primary aortoenteric fistula requires a high index of suspicion and a combination of diagnostic modalities to establish the diagnosis. Prompt diagnosis and rapid treatment are critical in reducing mortality and morbidity. Although rare, metastatic carcinoma can lead to aortoenteric fistula.
Introduction
Primary aortoduodenal fistula (ADF) is a rare complication of abdominal aortic pathology and an even rarer cause of gastrointestinal (GI) bleeding, with an estimated incidence of 0.04-0.07% in the general population.1 Because of the rarity of primary ADF, vascular surgeons may not encounter it in their career. Difficulty in establishing an early diagnosis accounts for the high mortality of this condition. The diagnosis relies on high index of clinical suspicion, and no single diagnostic modality is available that can confirm or exclude the presence of ADF. The diagnosis is typically established by the complementary use of endoscopy, angiography, computed tomography (CT) scan, and exploratory laparotomy. In a review of the published data, more than half of primary aortoenteric fistula (AEF) cases were diagnosed at autopsy, and only 33 of a total of 118 cases received surgery, with 21 patients surviving.2 We herein report the case of a woman with recurrent cervical carcinoma and ADF, and illustrate the potential difficulties in establishing a diagnosis.
Case Reports
A 76-year-old woman was seen in the Emergency Department (ED) with the complaint of hematemesis beginning 2 hours before admission, and intermittent abdominal pain for 1 year. Her history was significant for stage IIA cervical carcinoma for which she received a radical hysterectomy and bilateral pelvic lymph node dissection in 1998. She experienced a pelvic recurrence in 2006 and received concurrent chemoradiation therapy at that time. No signs of recurrence were subsequently noted. She also had a history of hypertension and type 2 diabetes, treated with medication for 10 years.
In the ED, she was pale and appeared ill. Her blood pressure was 83/42 mm Hg, heart rate 122 beats/min, respiratory rate 22/min, body temperature 36.4°C, and her oxygen saturation was 95% on room air. Cardiac examination revealed a regular heart beat without murmur. There was no jugular venous engorgement. Breath sounds were clear on chest auscultation. Abdominal examination revealed one lower midline laparotomy scar as result of the previous hysterectomy, and hypoactive bowel sounds. No hepatomegaly, local tenderness, pulsatile mass, leg edema, limb cyanosis, or peripheral pulse deficits were noted.
Laboratory studies revealed anemia with a hemoglobin of 4.9 g/dL (normal, 13-16 g/dL), and renal insufficiency with a creatinine of 2.23 mg/dL (normal, 0.7-1.2 mg/dL). Chest radiography revealed mild cardiomegaly without pulmonary congestion. The electrocardiography showed sinus tachycardia.
Volume replacement and blood component therapy were administered to treat hypovolemic shock and severe anemia. Her blood pressure stabilized at 132/80 mm Hg after initial management, and no further hematemesis was noted. Panendoscopy (PES) was performed urgently for evaluation of hematemesis, and revealed blood and clots in the stomach, and an ulcerative mass at the second portion of duodenum (Fig. 1). A PES-guided biopsy was obtained. CT scan of abdomen revealed a soft-tissue density surrounding the infrarenal abdominal aorta with extravasation of contrast medium adjacent to duodenum (Fig. 2, arrow). No gallstones or pancreatic mass were noted. A mycotic aortic pseudoaneurysm with ADF formation was considered. The patient experienced an episode of massive hematemesis accompanied by hypotension and severe abdominal and back pain approximately 5 hours after entering the ED, and she was taken to the operating room for an emergent exploratory laparotomy. The proximal abdominal aorta just below left renal vein was clamped for temporary bleeding control. The retroperitoneal para-aortic space was densely fibrotic, and the third and fourth portions of duodenum were firmly adhered to the abdominal aorta. After control of the proximal aorta was achieved, the entire thickened abdominal aorta was excised. A connection between aorta and duodenal lumen was identified, with obstruction by some fresh clots. The proximal and distal stumps of duodenum were divided and closed. After extensive debridement, in situ aortic grafting with an Hemashield Y tube graft was performed to restore lower limb perfusion. Diversion duodenostomy, cholecystostomy, and feeding jejunostomy were created for total enteric exclusion. Her postoperative recovery in the intensive care unit was uneventful. She resumed enteral feeding through jejunostomy on postoperative day (POD) 5, and was extubated on POD 8.
Fig. 1
Panendoscopy revealed an ulcerative mass at the second portion of duodenum.
Fig. 2
Computed tomographic (CT) scan of the abdomen revealed a soft-tissue density surrounding the infrarenal abdominal aorta with extravasation of contrast medium adjacent to duodenum (arrow).
Histopathologic examination of the excised aorta showed metastatic carcinoma arranged in nests, invading from surrounding tissue to the tunica media (Fig 3A, B). The neighboring tissue and aortic wall revealed marked hyaline necrosis with nuclear dusts, atypical stromal cells, and many plasma cells. Findings taken as a whole were consistent with metastatic carcinoma and suspicious for irradiation effects. Subsequent culture of the excised aorta was negative.
Fig. 3
Histopathologic examination of the excised aorta shows metastatic carcinoma arranged in nests invading from the surrounding soft tissue to the tunica media layer of aortic wall (hematoxylin and eosin; A, 40×; B, 200×).
The patient's postoperative course was unremarkable until POD 15 when she developed septic shock. Bile-like content was noted to be draining from the retroperitoneal drain tube, and duodenal stump leakage was suspected. She was discharged on POD 16 in critical condition. The patient and her family believed death was imminent, and according to Taiwanese tradition, prefer to pass at home.
Discussion
Primary AEF is a rare complication of abdominal aortic pathology and a even rarer cause of GI bleeding with an estimated incidence of 0.04-0.07% in the general population.1 Because of the rarity of primary ADF, vascular surgeons may not encounter one in their career. Difficulty in establishing an early diagnosis accounts for the high mortality of this condition. The diagnosis relies on high index of clinical suspicion, and no single diagnostic modality is available that can confirm or exclude the presence of an ADF. The diagnosis is typically established by the complementary use of endoscopy, angiography, CT scan, and exploratory laparotomy. Gastrointestinal bleeding in the presence of negative endoscopy findings and evidence of an aneurysmal aorta suggest primary AEF and requires urgent evaluation by CT and exploratory surgery or endovascular treatment.3 In a review of the published data, more than half of primary AEF cases were diagnosed at autopsy, and only 33 of a total of 118 cases received surgery, with 21 patients surviving.2 Secondary AEF fistula have been noted to occur after endovascular aortic interventions; however, their incidence is likely low.4
In our case, the primary ADF was identified by CT scan after a PES study excluded other common causes of GI bleeding (e.g., peptic ulcer), and rose the suspicion of a malignancy. Hemodynamic collapse developed 4 hours after the first “sentinel” hemorrhage. In a report of 18 patients with AEF by Song et al.,5 the time interval between the initial sentinel hemorrhage and massive life-threatening hemorrhage ranged from 5 hours to 5 months (median, 4 days). The case additionally points out that a high index of suspicion is necessary for establishing the correct diagnosis and that endovascular stent-grafting may play a role in management. Our patient experienced an accelerated course, although diagnosis of ADF was established before hemodynamic collapse and we were able to be more prepared for the surgical challenge. On the basis of our experience, we believe that the CT scan facilitated an earlier diagnosis of the ADF, and thus assisted in improving the immediate surgical outcome.
Underlying etiologies of primary ADF include infection, atherosclerosis, pancreatic carcinoma, ulcers, gallstones, diverticulitis, appendicitis, and foreign bodies.2 Cervical cancer is the second most common cancer among women,6 and although cervical carcinoma recurrence is not uncommon, there has been only one previous report of a primary ADF associated with cervical cancer. Our PubMed search returned only a report by Gupta and Rogers7 of recurrent cervical cancer as a cause of a primary ADF. Our patient underwent radical surgery for cervical carcinoma 11 years prior, and experienced a pelvic recurrence 8 years later. Lymphatic spread of cervical carcinoma was well known, and the route of spreading is typically along the para-aortic lymphatics, with subsequent para-aortic lymphadenopathy. However, local invasion of metastatic cervical carcinoma to the aorta and bowel and the development of an AEF are rare, as evidenced by our review of published data.
In our case, recurrent cervical cancer as the cause of the ADF is based on operative findings (no atherosclerosis, aneurismal changes of aortic wall, or foreign body noted), clinical evidence (i.e., bacterial culture of the ADF was negative, no peptic ulcer was noted on PES, and CT scan revealed no gallstones or pancreatic mass), and histopathologic examination of the ADF. Additionally, post-irradiation effects may have played a role in the ADF formation, as mentioned in the pathological report. Our patient survived the emergent operation, but was not likely to survive the terminal illness. The direct cause of death appears to have been an intra-abdominal abscess resulting from leakage of the proximal duodenal stump.
In summary, our case demonstrated that diagnosis of primary AEF requires a high index of suspicion and a combination of diagnostic modalities to establish the diagnosis. Prompt diagnosis and rapid treatment are critical in reducing mortality and morbidity. Although rare, metastatic carcinoma can lead to AEF.
References
- Primary aortoduodenal fistula: a case report and review of the literature. J Vasc Surg. 2003;37:686–689
- . Primary aortoduodenal fistula: manifestation, diagnosis, and treatment. Surgery. 1984;96:492–497
- . Primary aortoenteric fistula. Br J Surg. 2005;92:143–152
- . Secondary aortoenteric fistula after endovascular aortic interventions: a systematic literature review. J Vasc Interv Radiol. 2008;19(2 Pt. 1):163–165
- Diagnosis and management of primary aortoenteric fistulas–experiences learned from eighteen patients. Surgery. 2008;143:43–50
- Global Cancer Statistics, 2002. CA Cancer J Clin. 2005;55:74–108
- . A unique case of aortoduodenal fistula following carcinoma of cervix. Am J Obstet Gynecol. 1978;131:110–113
PII: S0890-5096(10)00080-4
doi:10.1016/j.avsg.2009.12.017
© 2010 Annals of Vascular Surgery Inc. Published by Elsevier Inc All rights reserved.
