Perianeurysmal retroperitoneal fibrosis. An unusual cause of renal failure

Retroperitoneal fibrosis causing ureteral obstruction in association with an abdominal aortic aneurysm has been reported infrequently. However, the clinical presentation of patients with this entity and the histopathologic findings at surgery are similar to those in patients with idiopathic retroperitoneal fibrosis. We describe a patient with perianeurysmal fibrosis and bilateral ureteral obstruction who presented with severe renal failure. The diagnosis of an abdominal aortic aneurysm with perianeurysmal fibrosis was made only at the time of surgery to repair bilateral ureteral obstruction. Previous case reports of perianeurysmal fibrosis are reviewed, and possible pathogenetic mechanisms are discussed. It is important to consider the presence of an occult abdominal aortic aneurysm in patients suspected of having retroperitoneal fibrosis because of the serious prognostic and therapeutic implications.     

Clinical aspects and treatment of inflammatory abdominal aortic aneurysms

An increasing number of inflammatory abdominal aortic aneurysms are reported today, although it is not clear whether these are nosologically independent lesions or a particular evolution of atherosclerotic aneurysms with enhanced phlogistic processes and fibrous reaction. Several pathogenetic theories have been proposed (microfissuration, autoimmunity, lymphatic stasis); clinical symptoms are characterised by the frequent involvement of contiguous retroperineal structures (in particular the urinary excretory tract and duodenum) which may be dislocated or compressed by the neoformation. CT or NMR appear to be the instrumental tests which give the most reliable diagnosis regarding the suspected inflammatory nature of the aneurysm. A correct pre-operative diagnosis is particularly important given that the morphological peculiarities (fibrosis and tenacious synechiae) add considerable pitfalls and difficulties to the operation. In this respect, the use of special technical devices, such as minimum dissection, permit a marked reduction of perioperative complications.     

Retroperitoneal fibrosis and chronic peri-aortitis--new hypothesis

Retroperitoneal fibrosis is characterised by the development of fibrotic mass surrounding the abdominal aorta and its branches. In the one third of cases, the causes of this disease include ergot-derivative drugs, retoperitoneal haemorrhage or urine extravasation and desmoplastic response to a variety of tumours. Retroperitoneal fibrosis is idiopathic in two thirds of cases and is found most commonly as an isolated fibrotic plague centered over the lumbar spine and entrapping one or both ureters. It has been postulated that fibrosis in idiopathic cases is caused by a chronic inflammatory or autoimmune response to antigens leaking into retroperitoneum from atheromatous plagues in the aorta or common iliac arteries. Many findings indicate the active nature of aortic adventitial chronic inflammation associated with human advanced atherosclerosis ("chronic periaortitis") and show its possible progressive potential to the clinically important disease termed "idiopathic retroperitoneal fibrosis" and "inflammatory aneurysm". A definitive differential diagnosis requires not only CT and NMR but also histologic confirmation. Treatment may be surgical or medical, with the best outcome observed in patient receiving both.     

Fetal cells in maternal blood: recovery by charge flow separation

PURPOSE: To screen patients with abdominal aortic aneurysm for popliteal aneurysm and investigate cardiovascular and genetic risk factors associated with aneurysmal disease at more than one site (generalised aneurysmal disease). SUBJECTS, DESIGN AND SETTING: All patients referred to the Regional Vascular Surgical Service at Charing Cross Hospital with unruptured abdominal aortic aneurysm between 1989 and 1993 were screened for popliteal aneurysms, using ultrasonography. MAIN OUTCOME MEASURES: Palpation of a popliteal aneurysm or ultrasonographic detection of popliteal dilatation, where the ratio maximum popliteal fossa diameter/suprageniculate popliteal diameter was > or = 1.5, in relation to cardiovascular and genetic risk factors. RESULTS: Clinical examination detected popliteal aneurysms in only 11/232 patients (5%), but ultrasonography demonstrated the presence of popliteal aneurysm in a further 13 patients, 24/232 in total (10%). Multivariate regression identified four independent factors associated with popliteal dilatation disease: age (p = 0.046), height (p = 0.006), systolic hypertension (p = 0.037) and triglyceride concentration (p = 0.009). Generalised aneurysmal disease and systolic blood pressure were associated with polymorphic variation in the fibrillin-1 gene, but not with variations in the apolipoprotein B and type III collagen genes. CONCLUSIONS: Few patients with abdominal aortic aneurysm (10%) also have popliteal aneurysms: the risk of popliteal dilatation increases with age, height, systolic blood pressure, triglyceride concentration and fibrillin genotype. The strong interaction between fibrillin genotype and blood pressure may contribute to the familial tendency to aortic aneurysm.     

Isolation of Mycoplasma and unstable L-forms from sporadic bovine mastitis

A case of giant cell aortitis causing ascending aortic aneurysm associated with aortic regurgitation is reported. The aneurysm was excised and the aortic valve replaced using a fresh homograft. The patient has been followed up for three and a half years. There is good evidence of correction of the haemodynamic lesion and no evidence of further arteritis or aneurysmal formation. The pathological and clinical problem of this disease are discussed.     

Activation of coagulation and fibrinolysis in patients with abdominal true aortic aneurysm associated with disseminated intravascular coagulation

Two cases of abdominal true aortic aneurysm (AAA) associated with disseminated intravascular coagulation (DIC) were reported. Case 1 was an 81-year-old male who was admitted because of hematoma on the left leg and in whom was found by MRI an aortic aneurysm of 14 cm in diameter. Coagulation studies indicated DIC by revealing thrombocytopenia, hypofibrinogenemia and increased level of FDP. DIC was well controlled by surgical repair of the aneurysm after the administration of a small dose of heparin. Case 2 was a 60-year-old male who was admitted because of lumbago and hematoemesis and in whom was found by CT and echography an aortic aneurysm of 5.5 cm in diameter. Coagulation studies indicated DIC by revealing thrombocytopenia and an increased level of FDP. On the 2nd hospital day, he suddenly died due to the rupture of the aortic aneurysm. In most of 9 cases with AAA without DIC, plasma levels of thrombin-antithrombin III complex, plasmin-alpha 2 plasmin inhibitor complex and FDP-D dimer were also elevated. These findings indicate that the coagulation and fibrinolysis systems were generally activated in patients with AAA, and that DIC tends to occur in patients with a giant aortic aneurysm or an impending ruptured aneurysm.     

Bilateral subclavian artery aneurysm associated with idiopathic cystic medial necrosis

Although cystic medial necrosis, either idiopathic or associated with Marfan's syndrome, usually becomes manifest as an ascending aortic aneurysm, aortic insufficiency, aortic dissection, or a combination of these disorders, a rare case of bilateral subclavian artery aneurysm secondary to idiopathic cystic medial necrosis has occurred. Subclavian artery aneurysms most commonly represent poststenotic dilatation from anterior scalene or cervical rib compression, occasionally are associated with generalized arteriosclerotic peripheral vascular disease, and rarely are secondary to syphilitic or mycotic infections. Subclavian artery aneurysms have a major risk of rupture, embolus, or thrombosis, and therefore should be repaired. A reverse saphenous vein or prosthetic bypass graft from the carotid to the axillary artery provides adequate flow to the upper extremity. The aneurysm should be completely excised if possible, since reexpansion through small collaterals or through insufficient closure by ligation can occur and compress the brachial plexus after successful bypass. The clinical presentation, angiographic findings, and operative repair of a subclavian artery aneurysm secondary to cystic medial necrosis are described.     

Orbital mass in a child causing somnolence, nausea and bradycardia

Aorto-esophageal fistula due to ruptured thoracic aortic aneurysm is very rare but is associated with extremely high mortality. An 81-year-old woman was admitted due to repeated hematemesis. Endoscopic examination revealed ulceration with blood clot on the mid-esophagus and compression of an extra-esophageal mass. The thoracic CT scan revealed an aorto-esophageal fistula due to a ruptured descending thoracic aortic aneurysm. Surgery was performed on April 3, 1996. We report an aorto-esophageal fistula managed successfully in one stage by resection and replacement of the aortic aneurysm with a prosthetic graft and total esophageal resection. The esophagus was reconstructed using orthotopic gastric interposition with omentopexy around the prosthetic aortic graft. The postoperative course was uneventful and there have been no signs of mediastinal sepsis, graft infection or pyothorax 12 months postoperatively. We suggest that the resection of both the aneurysm and the esophagus as well as the immediate reconstruction of the esophagus by orthotopic gastric interposition to obliterate the retrosternal space are important technique in the management of intrathoracic infections.     

A case of Stanford type A acute aortic dissection associated with a distal aortic arch atherosclerotic aneurysm

We report a rare case of Stanford type A acute aortic dissection associated with a distal aortic arch atherosclerotic aneurysm. A 71-year-old female was referred to us with the diagnosis of thrombosed Stanford type A acute aortic dissection, however on the next day transesophageal echocardiography revealed the false lumen has been recanalized. In the operation, there was a distal aortic arch atherosclerotic aneurysm which was unidentified at the preoperation. It is very rare that the dissection originated from atherosclerotic aneurysm and proceeded to proximal and distal portion of the aorta.     

Autologous blood transfusion: the benefits to the patient undergoing abdominal aortic aneurysm repair

The clinical benefits of using intraoperative autologous blood transfusion during abdominal aortic aneurysm bypass surgery become increasingly apparent when use of autologous and homologous blood transfusions is compared. That homologous blood transfusions carry some risk is widely recognized. When autologous blood is used as a sole source of blood transfusion, the risk of transmission of infectious agents and potential immunologic side effects are avoided. A prospective randomized pilot study comparing autologous and homologous blood transfusion in patients undergoing elective infrarenal abdominal aortic aneurysm bypass surgery was undertaken. The purpose of this study was to determine whether autologous blood salvaged intraoperatively may serve as an alternative to homologous blood by comparing the rate of postoperative infection and duration of hospital stay for patients receiving autologous versus homologous blood transfusions. Fifty patients undergoing abdominal aortic aneurysm bypass surgery were prospectively randomly assigned to receive either a homologous or an autologous blood transfusion, with 27 receiving a homologous blood transfusion and 23 receiving an autologous blood transfusion. The data from this study show that the length of hospital stay of patients receiving an autologous blood transfusion intraoperatively was reduced by a mean of 3 days and the risk of postoperative complications such as a systemic inflammatory response or sepsis, was reduced by more than 50%.     

Retroperitoneal approach for repair of inflammatory aortic aneurysms

Inflammatory aortic aneurysms (IAAs) are characterized by a markedly thickened aortic wall and dense perianeurysmal fibrosis. The presence of such inflammation and subsequent organ adherence makes surgical repair of IAAs more challenging than repair of simple atherosclerotic aneurysms. From March 1987 to June 1994, twelve patients underwent surgical repair of an IAA by a single surgeon. Ten patients were men (83%) and the mean age was 68.3 years (range 58 to 93 years). All patients except one were symptomatic with back or abdominal pain, yet none had evidence of aneurysm rupture at operation. Preoperatively 90% (9/10) of the patients had an elevated erythrocyte sedimentation rate (ESR), 100% (12/12) had a predictive CT scan, and all patients with aortic wall pathology specimens had their diagnosis confirmed. Six patients had a left flank retroperitoneal surgical approach, five had a transabdominal approach, and one had ligation and extra-anatomic reconstruction. The choice of repair technique was based on the degree and anatomic distribution of perianeurysmal fibrosis. The 30-day operative mortality rate was 0%. Mean follow-up was 56.3 months. Aortoduodenal fistula occurred in one patient 5 months after transperitoneal repair. Technical difficulties encountered during subsequent transperitoneal repairs led to the evolution of a policy in which the retroperitoneal approach was preferred in all patients with CT evidence of IAA. It is concluded that IAA represents a spectrum of retroperitoneal fibrosis and inflammation that is best treated surgically via a retroperitoneal approach.     

Aortic aneurysm complicated with coarctation of the aorta and Turner syndrome

A 23-year-old female with Turner syndrome and horse-shoe kidney underwent the operation of the coarctation of the aorta associated with the thoracic aortic aneurysm. The aortic aneurysm was located between the left carotid artery and the coarctation of the aorta, and the subclavian artery which was branched away from the aortic aneurysm was also aneurysmal. Aneurysmectomies and the reconstruction of the descending thoracic aorta and the left subclavian artery were performed with knitted Dacron grafts under assisting of the left atriodescending thoracic aortic bypass with Bio-pump. The disease was rare and such a case was not reported previously.     

Aortoduodenal fistula

A primary aortoduodenal fistula is usually associated with an atherosclerotic aortic aneurysm, and a secondary fistula with a leaking anastomotic aortic suture line. Two examples of each are reported. The typical features of a primary fistula are haematemesis or melaena, pain, and a pulsatile abdominal mass; the features of a secondary fistula are haematemesis and melaena with a past history of aortic resection. The initial haemorrhage is rarely fatal: a lag period allows urgent laparotomy. The fistula is diagnosed by dissection of the fourth part of the duodenum from the aorta. The treatment is closure of the duodenum, and resection of an aneurysm if present. Reconstruction is by a graft anastomosed to the aorta proximal to the fistula, if the area is clean, or by an extra anatomical bypass, if the area is heavily contaminated.     

Hypothermic circulatory arrest does not increase the risk of ascending thoracic aortic aneurysm resection

OBJECTIVE: The purpose of this study was to investigate the safety and efficacy of a period of deep hypothermic circulatory arrest (DHCA) during elective replacement of the ascending thoracic aorta. SUMMARY BACKGROUND DATA: DHCA has been implemented in ascending thoracic aortic aneurysm resection whenever the anatomy or pathology of the aorta or arch vessels prevents safe or adequate cross-clamping. Profound hypothermia and retrograde cerebral perfusion have been shown to be neurologically protective during ascending aortic replacement under circulatory arrest. METHODS: The authors conducted a retrospective analysis of 91 consecutive patients who underwent repair of chronic ascending thoracic aortic aneurysms from 1986 to present. The authors hypothesized that patients undergoing DHCA with or without retrograde cerebral perfusion during aneurysm repair were at no greater operative risk than patients who received aneurysm resection while on standard cardiopulmonary bypass. RESULTS: There were no significant differences in hospital mortality, stroke rate, or operative morbidity between patients repaired on DHCA when compared to those repaired on cardiopulmonary bypass. CONCLUSIONS: DHCA with or without retrograde cerebral perfusion does not result in increased morbidity or mortality during the resection of ascending thoracic aortic aneurysms. In fact, this technique may prevent damage to the arch vessels in select cases and avoid the possible complications associated with cross-clamping a friable or atherosclerotic aorta.     

Fate of the excluded abdominal aortic aneurysm sac: long-term follow-up of 831 patients

PURPOSE: Nonresective treatment of the infrarenal abdominal aortic aneurysm by proximal and distal ligation of the aneurysm sac (exclusion) combined with aortic bypass has been previously reported. A 10-year experience with 831 patients undergoing this procedure was reviewed. METHODS: From 1984 to 1994, 831 (761 elective, 70 urgent) of 1103 patients being treated for abdominal aortic aneurysm underwent repair with the retroperitoneal exclusion technique. Perioperative morbidity and mortality, estimated blood loss, transfusion requirements, natural history of the excluded aneurysm sac, and long-term survival were all assessed. RESULTS: The operative mortality rate for patients undergoing exclusion and bypass was 3.4%. The incidence of nonfatal perioperative complications was 5.2%. Colon ischemia requiring resection occurred in 2 (0.2%) of the 831 patients. Estimated blood loss was 638 +/- 557 cc (50 to 330 cc). On follow-up 17 (2%) patients were found to have patent aneurysm sacs as detected by duplex examination. Fourteen patients required surgical intervention. No cases of graft infection or aortoenteric fistula have been noted. CONCLUSION: Retroperitoneal exclusion and bypass is a viable alternative to traditional open endoaneurysmorraphy in surgery for abdominal aortic aneurysm. Most excluded aneurysm sacs have thrombosis without any long- or short-term complications; however, in a small number of patients delayed rupture of patent aneurysm occurs, thus emphasizing the need for diligent follow-up and appropriate intervention.     

Multiple idiopathic arterial aneurysms in children: a case report and review of the literature

A 6-year-old boy from China presented initially at 2 years of age with a pulsatile mass in his right antecubital fossa. He was not fully evaluated until the age of six years, when pan-angiography and computed axial tomography scan revealed multiple aneurysms of his right brachial artery, right radial artery aneurysms, an infrarenal aortic aneurysm, and a right internal carotid artery aneurysm in the region of the cavernous sinus. The patient underwent uneventful repairs of both the abdominal aortic aneurysm and the multiple aneurysms of the right arm. Pathologic evaluation was significant for medial fibrosis of the arterial wall with decreased and disordered elastin fibers. Review of the previously reported cases in children indicate the upper extremity arteries are involved in 92% of patients, the aortoiliac region in 92% of patients, and the renal/mesenteric vessels in 77% of cases. Lower extremity and cerebrovascular arteries are involved to a lesser extent. Children with peripheral aneurysms should have pan-angiography performed before treatment is begun. Surgical repair in these cases has been excellent.     

Surgical implication of aortic dissection on long-term outcome in Marfan patients

We herein review our 17-year surgical experience for the treatment of ascending aortic aneurysm in patients with Marfan syndrome to clarify the risks of increased mortality and reoperation. The subjects consisted of 15 patients who had all undergone surgery for the aortic root and ascending aorta at Niigata University Hospital between July 1978 and January 1995. Aortic valve replacement and ascending aortic wrapping were performed in 5 patients, Bentall or Cabrol operation in 6, and combined aortic arch reconstruction and Cabrol operation in 2, as the initial surgery. Patients who had an aortic dissection (Stanford type A) at initial surgery were assigned to group I (n = 7), while those with an aortic root aneurysm were assigned to group II (n = 8). In group I, 3 patients required a second operation for the remaining aortic arch aneurysm, and 1 died due to a late rupture of the distal aneurysm. In group II, no patient needed a reoperation; however, 1 died due to an intracranial hemorrhage and another due to composite valve graft failure and distal dissection. The results thus indicate that aortic dissection seems to affect long-term outcome, and therefore the combined repair of the aortic root and transverse arch is recommended in Marfan patients with aortic dissection involving the transverse aortic arch.     

Surgery of abdominal aortic aneurysm and concomitant disease of the digestive tract

The incidence of intra-abdominal diseases associated with abdominal aortic aneurysm is increasing, and it is difficult to decide whether to operate the abdominal disease first, the aneurysm first or both simultaneously. Variables used in decision analysis include type, stage and life expectancy of the cancer, rupture rate of abdominal aortic aneurysm. Symptomatic lesion should be treated first. Absolute indication for operation initially on the aneurysm is the presence of symptoms of rupture. Aortic abdominal aneurysmectomy combined with surgical removal of an intestinal disease may present severe risks as infection of the graft and anastomotic leakage, especially during lower abdominal surgery. In this paper authors present four cases of AAA which had intra-abdominal surgical disease. They were treated by one-stage operation with no complications. Criteria to assess timing of surgical treatment of abdominal surgical diseases concomitant to AAA are discussed.     

Infrarenal aortic dimensions in siblings of patients with infrarenal abdominal aortic aneurysm or aortoiliac occlusive disease

OBJECTIVE: To compare the dimensions of the infrarenal aorta and the prevalence of undiagnosed infrarenal abdominal aortic aneurysms in the siblings of patients operated on for either infrarenal abdominal aortic aneurysm or aortoiliac occlusive disease. DESIGN: Prospective screening study. SETTING: University hospital, Finland. SUBJECTS: 220 siblings of patients operated for either abdominal aortic aneurysm or aortoiliac occlusive disease. INTERVENTIONS: Abdominal aortic ultrasound. MAIN OUTCOME MEASURES: Anteroposterior and transversal diameters of infrarenal and suprarenal aorta. RESULTS: There was a group of 5 siblings (4 men and 1 woman, aged 59 years or more) among the aneurysm patients who had a disposition to infrarenal aneurysm formation defined as a dilatation of 30 mm or more. This contrasted with none among the siblings of the patients with aortoiliac occlusive disease (p = 0.07, 95% confidence interval 0.49 to 165.3). The two factors affecting the diameter of the infrarenal aorta were age and sex. The aortic dimensions tended to be bigger in the siblings of the aneurysm patients but the differences were not significant. The ratio of infrarenal to suprarenal diameter was bigger in the siblings of the aneurysm patients also after excluding the cases with detected dilatations (p = 0.05) and in the multivariate analysis the only factor explaining this difference was the type of the disease of the proband. CONCLUSIONS: The screening of male siblings (over 55 years old) of patients with infrarenal abdominal aortic aneurysms might be justified.