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Hoarseness due to left recurrent laryngeal nerve paralysis (LRLN) caused by identifiable cardiovascular disease is described as Ortner’s syndrome or Cardiovocal syndrome. This was first described by Ortner in 1897 to describe left recurrent laryngeal nerve palsy secondary to Mitral Stenosis. The common conditions which gave rise to Ortners’ syndrome include mitral stenosis, mitral valve prolapse, aortic aneurysm, septal defect, following cardiothoracic surgery, high altitude pulmonary hypertension, ductal aneurysm, aortic dissection etc. Herewith we are presenting an unusual presentation of Aortic arch aneurysm.

Cardiovocal syndrome or Ortner's syndrome is hoarseness due to left recurrent laryngeal nerve palsy caused by mechanical affection of the nerve from enlarged cardiovascular structures. Chronic thromboembolic pulmonary hypertension is extremely rarely found to cause this syndrome. We describe a case of a 56-year-old patient with sudden onset of hoarseness. The patient had known long standing severe pulmonary hypertension. Fiberoptic laryngoscopy showed left vocal cord palsy. Computed tomography of the neck and chest revealed extensive enlargement of the pulmonary arteries and excluded a malignant tumor. The diagnosis of cardiovocal syndrome was retained. It is important for the radiologist to be aware of this possible etiology causing left recurrent laryngeal nerve palsy and to understand its mechanism.

Ortner's syndrome (left recurrent laryngeal nerve palsy caused by cardiovascular pathology) is described in literature as occurring secondary to a variety of conditions. Spontaneous aneurysm of ductus arteriosus is a rare cause of this condition. We present a case where an adult patient with an aneurysm of the ductus arteriosus presented for the first time at the age of 62 years with hoarseness of voice secondary to left recurrent laryngeal nerve palsy.

In the surgical treatment of abdominal aortic aneurysm, the single proximal cross-clamp can be placed at 3 alternative aortic levels: infrarenal, hiatal, and thoracic. We performed this retrospective study to evaluate the advantages and disadvantages of the 3 main aortic clamping locations. Eighty patients presented at our institution with abdominal aortic aneurysms from March 1993 through May 1998. Fifty of these patients had intact aneurysms and underwent elective surgery, and 30 had ruptured aneurysms that necessitated emergency surgery. Proximal aortic clamping was applied at the infrarenal level in 24 patients (22 from the intact aneurysm group, 2 from the ruptured group), at the hiatal level in 34 patients (22 intact, 12 ruptured), and at the thoracic level (descending aorta) via a limited left lateral thoracotomy in 22 patients (6 intact, 16 ruptured). Early mortality rates (within 30 days) were 4% (2 of 50 patients) among patients with intact aneurysms and 40% (12 of 30 patients) among those with ruptured aneurysms. In the 2 patients from the intact aneurysm group, proximal aortic clamps were applied at the hiatal level. In the ruptured aneurysm group, proximal aortic clamps were placed at the thoracic level in 10 patients, the infrarenal level in 1, and the hiatal level in 1. According to our study, the clinical status of the patient and the degree of operative urgency--as determined by the extent of the aneurysm--generally dictate the proximal clamp location. Patients who present with aneurysmal rupture or hypovolemic shock benefit from thoracic clamping, because it restores the blood pressure and allows time to replace the volume deficit. Infrarenal placement is advantageous in patients with intact aneurysms if there is sufficient space for the clamp between the renal arteries and the aortic aneurysm. In patients with juxtarenal aneurysms, hiatal clamping enables safe and easy anastomosis to the healthy aorta. Clamping at this level also helps prevent late anastomotic aneurysm formation, which is frequently encountered after inadvertent anastomosis of the graft to a diseased portion of the aorta. Further studies are needed in order to confirm these results.

INTRODUCTION

Saccular aortic arch aneurysms in unusual sites may be misdiagnosed as a neoplasm. We present the case of a rare saccular aortic arch aneurysm between trachea and esophagus that resembled a mediastinal neoplasm in the preoperative findings.

PRESENTATION OF CASE

A 63-year-old male with an abnormal mediastinal shadow on chest X-ray was referred to the hospital. An axial plain computed tomogram of the chest revealed mediastinal soft tissue next to the right side of the aortic arch resembling a neoplasm originating from the gap between the trachea and the esophagus. The coronal view constructed by enhanced 64-row multi detector computed tomography revealed the soft tissue was an aneurysm arising from the inner side of the aortic arch. An aortic arch replacement was performed via a median sternotomy.

DISCUSSION

A thoracic aortic aneurysm sometimes behaves like a mediastinal neoplasm. The multiple cross-sectional image from multidetector computed tomography was useful for the correct diagnosis of such an aneurysm.

CONCLUSION

The possibility of an aneurysm should be considered whenever a mass in contact with the aortic wall is identified.

Summary

Tracheal and esophageal compression is a well-recognized complication of aneurysms of the aortic arch. Most of the patients present with dysphagia and/or respiratory insufficiency. In the adult population a right-sided aortic arch is often asymptomatic unless aneurysmal disease develops. This usually occurs at the level of the take-off of an aberrant left subclavian artery and is known as a Kommerell's aneurysm. In spite of its rarity, this condition is clinically relevant because of the mortality associated with rupture, the morbidity caused by compression of mediastinal structures, and the complexity of surgery. In many cases, surgical resection of the aneurysm relieves the symptoms. We present a case in which tracheal compression and bilateral vocal cord palsy caused by an aneurysm arising from Kommerrell's diverticulum. The patient developed respiratory embrassement after extubation and was subsequently treated with continue positive airway pressure (CPAP) with a favorable result.

A 62-year-old man was admitted to the emergency department with chronic dysphagia and lower back pain. Chest radiography revealed a wide mediastinal shadow and an elevated left diaphragm, which proved to be secondary to left phrenic paralysis. The patient was diagnosed with an aneurysm of the descending thoracic aorta and was admitted to the hospital. After the patient was admitted, the aneurysm ruptured into the right chest. The patient underwent an emergency operation to replace the ruptured segment with a synthetic graft. Postoperative recovery and follow-up were uneventful. This report describes an unusual presentation of a thoracic aortic aneurysm. Hemidiaphragmatic paralysis caused by compression of the phrenic nerve is an unusual complication that, to our knowledge, has not been previously reported.

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Summary

A 56 year-old woman presented with a ruptured bilobed wide neck aneurysm of the P2 segment of the PCA, atherosclerotic fusiform basilar artery aneurysm, subarachnoid bleeding with negative CT scan and chronic renal failure. She was managed by a cooperative approach involving neurosurgeons, neuroradiologist, neurointensivist, emergency room physicians, nurses and technicians.

She underwent operation by proximal clipping for the aneurysm of the PCA. Postoperative neurological deficits include homonymous hemianopsia and ipsilateral third nerve palsy. The operation was performed through asubtemporal approach. At surgery, the aneurysm was located in the distal of the P2 segment of PCA, bilobed up and down, no definitive neck with small distal branches, and was treated by proximal clipping of the PCA aneurysm. The fusiform basilar artery aneurysm was severely atherosclerotic and left untouched.

This is a rare case which required a high index of suspicion to detect subarachnoid bleeding from ruptured posterior fossa aneurysm, accurate prediction of the site of bleeding and the location of aneurysm location by conventional angiogram, MRI and MRA, and careful planned surgical strategy with the right approach for the P2 segment of the PCA aneurysm, complicated post operative care with airway management, triple H therapy, nutrition, additional measures and multiple hemodialysis.

Endovascular repair of inflammatory aortic aneurysms has been reported as an alternative to open surgical treatment. In selective cases, adjunctive bypass surgery may be required to provide an adequate landing zone. We report a case of endovascular repair of an inflammatory aortic aneurysm in a patient with Behçet's disease using a carotid-carotid bypass graft to provide an adequate landing zone. A 45-yr-old man with a voice change was referred to our hospital with the diagnosis of saccular aneurysm of the distal aortic arch resulting from vasculitis. Computed tomography showed a thoracic aortic aneurysm with thrombosis. Right to left carotid-carotid bypass grafting was performed. After 8 days, the patient underwent an endovascular stent graft placement distal to the origin of the innominate artery. The patient was discharged with medication and without postoperative complications after 5 days. Hybrid endovascular treatment may be suitable a complementary modality for repairing inflammatory aortic aneurysms.

A 61 year old patient was found to have an aneurysm extending from the aortic root to the suprarenal region. He underwent first stage surgery with aortic root and arch replacement, prosthetic aortic valve replacement, and coronary artery bypass grafting. Four weeks later, he presented with breathlessness and signs of heart failure and pleural effusion. Computed tomography showed that the left atrium was compressed between the aortic aneurysm posteriorly and the left ventricle and sternum anteriorly. Obstruction of the superior vena cava, bronchus, oesophagus, and rarely right atrium by an aortic aneurysm has been described before but presentation with left atrial compression has not been reported.

A right aortic arch is a rare congenital anomaly, with a reported incidence of around 0.1%. A patient with a right aortic arch underwent video-assisted thoracic surgery left lower lobectomy and mediastinal lymph node dissection for squamous cell carcinoma. There was no aortic arch or descending aorta in the left thoracic cavity, but the esophagus. There was no anomaly in the location or branching of the pulmonary vessels, the bronchi, and the lobulation of the lungs. The vagus nerve was found at the level of the left pulmonary artery. The arterial ligament was found between the left subclavian artery and the left pulmonary artery. The recurrent laryngeal nerve was recurrent around the left subclavian artery. A Kommerell diverticulum was found at the origin of the left subclavian artery. The patient experienced no complications. We conclude that video-assisted thoracoscopic lobectomy with mediastinal dissection is feasible for treating lung cancer with a right aortic arch.

Background

Aortic pathology progression and/or procedure related complications following endovascular repair should always be considered mostly in older patients. We herein describe a hybrid procedure for treatment of rapidly expanding thoracoabdominal aneurysm following endovascular treatment of a descending thoracic aortic aneurysm in an older patient.

Case presentation

A 82-year-old man at 18 months after endovascular surgery for a contained rupture of descending thoracic aortic aneurysm revealed a type IV thoracoabdominal aneurysm with significant increase of the aortic diameters at superior mesenteric and renal artery levels. A hybrid approach consisting of preventive visceral vessel revascularization and endovascular repair of entire abdominal aorta was performed. Under general anaesthesia and by xyphopubic laparotomy, the infrarenal aneurysmatic aorta and common iliac arteries were replaced by a bifurcated woven prosthetic graf. From each of the prosthetic branches two reverse 14x7 mm bifurcated PTFE prosthetic grafts were anastomized to both renal arteries and to the celiac axis and superior mesenteric artery, respectively. Vessel ischemia was restricted to the time required for anastomosis. Three 10 cm Gore endovascular stent-grafts for a total length of 15 cm, were used. The overlapping of the stent-grafts was carried out from the bottom upwards, starting from the aorto-iliac prosthetic body up to the healthy segment of thoracic aorta, 40 mm from the previous stent-grafts.

The patient was discharged on the 9th postoperative day.

Conclusion

This technique offers the advantage of a less invasive treatment, reducing the risk of paraplegia, visceral ischaemia and pulmonary complications, mostly in older patients.

Executive Summary

Objective

To conduct an assessment on endovascular repair of descending thoracic aortic aneurysm (TAA).

Clinical Need

Aneurysm is the most common condition of the thoracic aorta requiring surgery. Aortic aneurysm is defined as a localized dilatation of the aorta. Most aneurysms of the thoracic aorta are asymptomatic and incidentally discovered. However, TAA tends to enlarge progressively and compress surrounding structures causing symptoms such as chest or back pain, dysphagia (difficulty swallowing), dyspnea (shortness of breath), cough, stridor (a harsh, high-pitched breath sound), and hoarseness. Significant aortic regurgitation causes symptoms of congestive heart failure. Embolization of the thrombus to the distal arterial circulation may occur and cause related symptoms. The aneurysm may eventually rupture and create a life-threatening condition.

The overall incidence rate of TAA is about 10 per 100,000 person-years. The descending aorta is involved in about 30% to 40% of these cases.

The prognosis of large untreated TAAs is poor, with a 3-year survival rate as low as 25%. Intervention is strongly recommended for any symptomatic TAA or any TAA that exceeds twice the diameter of a normal aorta or is 6 cm or larger. Open surgical treatment of TAA involves left thoracotomy and aortic graft replacement. Surgical treatment has been found to improve survival when compared with medical therapy. However, despite dramatic advances in surgical techniques for performing such complex operations, operative mortality from centres of excellence are between 8% and 20% for elective cases, and up to 50% in patients requiring emergency operations. In addition, survivors of open surgical repair of TAAs may suffer from severe complications. Postoperative or postprocedural complications of descending TAA repair include paraplegia, myocardial infarction, stroke, respiratory failure, renal failure, and intestinal ischemia.

The Technology

Endovascular aortic aneurysm repair (EVAR) using a stent graft, a procedure called endovascular stent-graft (ESG) placement, is a new alternative to the traditional surgical approach. It is less invasive, and initial results from several studies suggest that it may reduce mortality and morbidity associated with the repair of descending TAAs.

The goal in endovascular repair is to exclude the aneurysm from the systemic circulation and prevent it from rupturing, which is life-threatening. The endovascular placement of a stent graft eliminates the systemic pressure acting on the weakened wall of the aneurysm that may lead to the rupture. However, ESG placement has some specific complications, including endovascular leak (endoleak), graft migration, stent fracture, and mechanical damage to the access artery and aortic wall.

The Talent stent graft (manufactured by Medtronic Inc., Minneapolis, MN) is licensed in Canada for the treatment of patients with TAA (Class 4; licence 36552). The design of this device has evolved since its clinical introduction. The current version has a more flexible delivery catheter than did the original system. The prosthesis is composed of nitinol stents between thin layers of polyester graft material. Each stent is secured with oversewn sutures to prevent migration.

Review Strategy

Objectives

To compare the effectiveness and cost-effectiveness of ESG placement in the treatment of TAAs with a conventional surgical approach

To summarize the safety profile and effectiveness of ESG placement in the treatment of descending TAAs

Measures of Effectiveness

Primary Outcome

Mortality rates (30-day and longer term)

Secondary Outcomes

Technical success rate of introducing a stent graft and exclusion of the aneurysm sac from systemic circulation

Rate of reintervention (through surgical or endovascular approach)

Measures of Safety

Complications were categorized into 2 classes:

Those specific to the ESG procedure, including rates of aneurysm rupture, endoleak, graft migration, stent fracture, and kinking; and

Those due to the intervention, either surgical or endovascular. These include paraplegia, stroke, cardiovascular events, respiratory failure, real insufficiency, and intestinal ischemia.

Inclusion Criteria

Studies comparing the clinical outcomes of ESG treatment with surgical approaches

Studies reporting on the safety and effectiveness of the ESG procedure for the treatment of descending TAAs

Exclusion Criteria

Studies investigating the clinical effectiveness of ESG placement for other conditions such as aortic dissection, aortic ulcer, and traumatic injuries of the thoracic aorta

Studies investigating the aneurysms of the ascending and the arch of the aorta

Studies using custom-made grafts

Literature Search

The Medical Advisory Secretariat searched The International Network of Agencies for Health Technology Assessment and the Cochrane Database of Systematic Reviews for health technology assessments. It also searched MEDLINE, EMBASE, Medline In-Process & Other Non-Indexed Citations, and Cochrane CENTRAL from January 1, 2000 to July 11, 2005 for studies on ESG procedures. The search was limited to English-language articles and human studies.

One health technology assessment from the United Kingdom was identified. This systematic review included all pathologies of the thoracic aorta; therefore, it did not match the inclusion criteria. The search yielded 435 citations; of these, 9 studies met inclusion criteria.

Summary of Findings

Mortality

The results of a comparative study found that in-hospital mortality was not significantly different between ESG placement and surgery patients (2 [4.8%] for ESG vs. 6 [11.3%] for surgery).

Pooled data from case series with a mean follow-up ranging from 12 to 38 months showed a 30-day mortality and late mortality rate of 3.9% and 5.5%, respectively. These rates are lower than are those reported in the literature for surgical repair of TAA.

Case series showed that the most common cause of early death in patients undergoing endovascular repair is aortic rupture, and the most common causes of late death are cardiac events and aortoesophageal or aortobronchial fistula.

Technical Success Rate

Technical success rates reported by case series are 55% to 100% (100% and 94.4% in 2 studies with all elective cases, 89% in a study with 5% emergent cases, and 55% in a study with 42% emergent cases).

Surgical Reintervention

In the comparative study, 3 (7.1%) patients in the ESG group and 14 (26.5%) patients in the surgery group required surgical reintervention. In the ESG group, the reasons for surgical intervention were postoperative bleeding at the access site, paraplegia, and type 1 endoleak. In the surgical group, the reasons for surgery were duodenal perforation, persistent thoracic duct leakage, false aneurysm, and 11 cases of postoperative bleeding.

Pooled data from case series show that 9 (2.6%) patients required surgical intervention. The reasons for surgical intervention were endoleak (3 cases), aneurysm enlargement and suspected infection (1 case), aortic dissection (1 case), pseudoaneurysm of common femoral artery (1 case), evacuation of hematoma (1 case), graft migration (1 case), and injury to the access site (1 case).

Endovascular Revision

In the comparative study, 3 (7.1%) patients required endovascular revision due to persistent endoleak.

Pooled data from case series show that 19 (5.3%) patients required endovascular revision due to persistent endoleak.

Graft Migration

Two case series reported graft migration. In one study, 3 proximal and 4 component migrations were noted at 2-year follow-up (total of 5%). Another study reported 1 (3.7%) case of graft migration. Overall, the incidence of graft migration was 2.6%.

Aortic Rupture

In the comparative study, aortic rupture due to bare stent occurred in 1 case (2%). The pooled incidence of aortic rupture or dissection reported by case series was 1.4%.

Postprocedural Complications

In the comparative study, there were no statistically significant differences between the ESG and surgery groups in postprocedural complications, except for pneumonia. The rate of pneumonia was 9% for those who received an ESG and 28% for those who had surgery (P = .02). There were no cases of paraplegia in either group. The rate of other complications for ESG and surgery including stroke, cardiac, respiratory, and intestinal ischemia were all 5.1% for ESG placement and 10% for surgery. The rate for mild renal failure was 16% in the ESG group and 30% in the surgery group. The rate for severe renal failure was 11% for ESG placement and 10% for surgery.

Pooled data from case series show the following postprocedural complication rates in the ESG placement group: paraplegia (2.2%), stroke (3.9%), cardiac (2.9%), respiratory (8.7%), renal failure (2.8%), and intestinal ischemia (1%).

Time-Related Outcomes

The results of the comparative study show statistically significant differences between the ESG and surgery group for mean operative time (ESG, 2.7 hours; surgery, 5 hours), mean duration of intensive care unit stay (ESG, 11 days; surgery, 14 days), and mean length of hospital stay (ESG, 10 days; surgery, 30 days).

The mean duration of intensive care unit stay and hospital stay derived from case series is 1.6 and 7.8 days, respectively.

Ontario-Based Economic Analysis

In Ontario, the annual treatment figures for fiscal year 2004 include 17 cases of descending TAA repair procedures (source: Provincial Health Planning Database). Fourteen of these have been identified as “not ruptured” with a mean hospital length of stay of 9.23 days, and 3 cases have been identified as “ruptured,” with a mean hospital length of stay of 28 days. However, because one Canadian Classification of Health Interventions code was used for both procedures, it is not possible to determine how many were repaired with an EVAR procedure or with an open surgical procedure.

Hospitalization Costs

The current fiscal year forecast of in-hospital direct treatment costs for all in-province procedures of repair of descending TAAs is about $560,000 (Cdn). The forecast in-hospital total cost per year for in-province procedures is about $720,000 (Cdn). These costs include the device cost when the procedure is EVAR (source: Ontario Case Costing Initiative).

Professional (Ontario Health Insurance Plan) Costs

Professional costs per treated patient were calculated and include 2 preoperative thoracic surgery or EVAR consultations.

The professional costs of an EVAR include the fees paid to the surgeons, anesthetist, and surgical assistant (source: fee service codes). The procedure was calculated to take about 150 minutes.

The professional costs of an open surgical repair include the fees of the surgeon, anesthetist, and surgical assistant. Open surgical repair was estimated to take about 300 minutes.

Services provided by professionals in intensive care units were also taken into consideration, as were the costs of 2 postoperative consultations that the patients receive on average once they are discharged from the hospital. Therefore, total Ontario Health Insurance Plan costs per treated patient treated with EVAR are on average $2,956 (ruptured or not ruptured), as opposed to $5,824 for open surgical repair and $6,157 for open surgical repair when the aneurysm is ruptured.

Conclusions

Endovascular stent graft placement is a less invasive procedure for repair of TAA than is open surgical repair.

There is no high-quality evidence with long-term follow-up data to support the use of EVAR as the first choice of treatment for patients with TAA that are suitable candidates for surgical intervention.

However, short- and medium-term outcomes of ESG placement reported by several studies are satisfactory and comparable to surgical intervention; therefore, for patients at high risk of surgery, it is a practical option to consider. Short- and medium-term results show that the benefit of ESG placement over the surgical approach is a lower 30-day mortality and paraplegia rate; and shorter operative time, ICU stay, and hospital stay.

Aortic arch aneurysm, pseudocoarctation, and coronary artery stenosis are extremely rare in Behçet's syndrome. We present the case of a 25-year-old man with Behçet's syndrome who underwent coronary artery bypass grafting for severe stenosis in the proximal left anterior descending coronary artery and concomitant surgical correction of a saccular aneurysm that was causing pseudocoarctation of the aortic arch. The surgery was successful.

A rare case of bilateral third cranial nerve palsy due to a ruptured anterior communicating artery aneurysm is presented. A 68-yr-old woman was semicomatose with bilaterally fixed dilated pupil, abducted eyes, and ptosis. A computed tomography demonstrated extensive hemorrhage spreading around the both Sylvian and interhemisheric fissure without focal mass effect. Intracranial pressure via extraventricular drainage before surgery was 15-50 mmHg. Three months later, brain MRI showed infarction of left posterior cerebral artery territory and lacuna infarction of the pons. Eleven months after aneurysm repair, nerve palsy improved slowly and recovered partially. The patient communicated well with simple words. The author reviewed and discussed the possible mechanism of this rare neuro-ophthalmological manifestation in view of a false localizing sign.

Endovascular treatment of complex thoracic pathologic conditions involving the aortic arch can often be appropriate and safe; however, minimally invasive procedures are not always feasible, especially in emergent cases. We report the case of a 78-year-old woman who emergently presented in hemorrhagic shock with a ruptured chronic dissecting aneurysm that involved the aortic arch. Eight years earlier, she had undergone aortic valve replacement and plication of the ascending aorta, which was complicated a day later by Stanford type B dissection, malperfusion, and ischemia that required an axillobifemoral bypass. At the current admission, we successfully treated her surgically through a left thoracotomy, using moderate hypothermic extracorporeal circulation and advanced organ-protection methods. We discuss the surgical indications and our operative strategy in relation to open surgical repair versus endovascular treatment in patients with complex conditions.

A 27 year old woman presented during the third trimester of her second pregnancy complaining of chest pain. A chest radiograph showed a large aneurysm arising from the distal part of the aortic arch. She was transferred to the regional cardiothoracic centre but died suddenly on arrival. Necropsy confirmed a ruptured saccular aortic aneurysm arising just distal to the left subclavian artery. A history of a road traffic accident at the age of 12 was obtained from relatives after the patient's death. Vascular and haemodynamic changes in pregnancy may have caused the rupture of this previously silent post-traumatic aortic aneurysm.

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Background

Takayasu arteritis is a large vessel vasculitis occurring in young females. We report a rare presentation of Takayasu arteritis in a Sri Lankan woman. She presented with bronchiectasis and left recurrent laryngeal nerve palsy prior to the onset of vascular symptoms. This case illustrates an atypical presentation of this disease and the diagnostic dilemma that the physician may be faced with.

Case presentation

A 39-year-old woman presented with chronic cough, haemoptysis and hoarseness of voice. She had left recurrent laryngeal nerve palsy and high inflammatory markers on investigation. CT thorax revealed aortic wall thickening and traction bronchiectasis. 2 D echocardiogram revealed grade 1 aortic regurgitation compatible with aortitis. She did not have weak peripheral pulses or a blood pressure discrepancy and did not meet American College of Rheumatology (ACR) criteria for diagnosis of Takayasu arteritis at this stage. Tuberculosis, syphilis and sarcoidosis was excluded. While awaiting angiography, she developed left arm claudication and a pericardial effusion. Angiography revealed evidence of Takayasu arteritis and absence of flow in the left subclavian artery. Takayasu arteritis was diagnosed at this stage after a period of eight months from the onset of initial symptoms. She is currently on prednisolone, azathioprine and aspirin.

Conclusion

Bronchiectasis and recurrent laryngeal nerve palsy is a rare presentation of Takayasu arteritis. Atypical presentations can occur in Takayasu arteritis prior to the onset of vascular symptoms. Elevation of inflammatory markers are an early finding. A high degree of suspicion is needed to identify these patients in the early course of the disease.

Trochlear nerve palsy associated with spontaneous subarachnoid hemorrhage (SAH) is known to be a rare malady. We report here on a patient who suffered with left trochlear nerve palsy following rupture of a right posterior communicating artery aneurysm. A 56-year-woman visited our emergency department with stuporous mental change. Her Hunt-and-Hess grade was 3 and the Fisher grade was 4. Cerebral angiography revealed a ruptured aneurysm of the right posterior communicating artery. The aneurysm was clipped via a right pterional approach on the day of admission. The patient complained of diplopia when she gazed to the left side, and the ophthalmologist found limited left inferolateral side gazing due to left superior oblique muscle palsy on day 3. Elevated intracranial pressure, intraventricular hemorrhage or a dense clot in the basal cisterns might have caused this trochlear nerve palsy.

EXECUTIVE SUMMARY

The Medical Advisory Secretariat conducted a systematic review of the evidence on the effectiveness and cost-effectiveness of endovascular repair of abdominal aortic aneurysm in comparison to open surgical repair. An abdominal aortic aneurysm [AAA] is the enlargement and weakening of the aorta (major blood artery) that may rupture and result in stroke and death. Endovascular abdominal aortic aneurysm repair [EVAR] is a procedure for repairing abdominal aortic aneurysms from within the blood vessel without open surgery. In this procedure, an aneurysm is excluded from blood circulation by an endograft (a device) delivered to the site of the aneurysm via a catheter inserted into an artery in the groin. The Medical Advisory Secretariat conducted a review of the evidence on the effectiveness and cost-effectiveness of this technology. The review included 44 eligible articles out of 489 citations identified through a systematic literature search. Most of the research evidence is based on non-randomized comparative studies and case series. In the short-term, EVAR appears to be safe and comparable to open surgical repair in terms of survival. It is associated with less severe hemodynamic changes, less blood transfusion and shorter stay in the intensive care and hospital. However, there is concern about a high incidence of endoleak, requiring secondary interventions, and in some cases, conversion to open surgical repair. Current evidence does not support the use of EVAR in all patients. EVAR might benefit individuals who are not fit for surgical repair of abdominal aortic aneurysm and whose risk of rupture of the aneurysm outweighs the risk of death from EVAR. The long-term effectiveness and cost-effectiveness of EVAR cannot be determined at this time. Further evaluation of this technology is required.

OBJECTIVE

The objective of this health technology policy assessment was to determine the effectiveness and cost-effectiveness of endovascular repair of abdominal aortic aneurysms (EVAR) in comparison to open surgical repair (OSR).

BACKGROUND

Clinical Need

An abdominal aortic aneurysm (AAA) is a localized, abnormal dilatation of the aorta greater than 3 cm or 50% of the aortic diameter at the diaphragm. (1) A true AAA involves all 3 layers of the vessel wall. If left untreated, the continuing extension and thinning of the vessel wall may eventually result in rupture of the AAA. The risk of death from ruptured AAA is 80% to 90%. (61) Heller et al. (44) analyzed information from a national hospital database in the United States. They found no significant change in the incidence rate of elective AAA repair or ruptured AAA presented to the nation’s hospitals. The investigators concluded that technologic and treatment advances over the past 19 years have not affected the outcomes of patients with AAAs, and the ability to identify and to treat patients with AAAs has not improved.

Classification of Abdominal Aortic Aneurysms

At least 90% of the AAAs are affected by atherosclerosis, and most of these aneurysms are below the level of the renal arteries.(1)

An abdominal aortic aneurysm may be symptomatic or asymptomatic. An AAA may be classified according to their sizes:(7)

Small aneurysms: less than 5 cm in diameter.

Medium aneurysms: 5-7cm.

Large aneurysms: more than 7 cm in diameter.

Small aneurysms account for approximately 50% of all clinically recognized aneurysms.(7)

Aortic aneurysms may be classified according to their gross appearance as follows (1):

Fusiform aneurysms affect the entire circumference of a vessel, resulting in a diffusely dilated lesion

Saccular aneurysms involve only a portion of the circumference, resulting in an outpouching (protrusion) in the vessel wall.

Prevalence of Abdominal Aortic Aneurysms

In community surveys, the prevalence of AAA is reported to be between 1% and 5.4%. (61) The prevalence is related to age and vascular risk factors. It is more common in men and in those with a positive family history.

In Canada, Abdominal aortic aneurysms are the 10th leading cause of death in men 65 years of age or older. (60) Naylor (60) reported that the rate of AAA repair in Ontario has increased from 38 per 100,000 population in 1981/1982 to 54 per 100,000 population in 1991/1992. For the period of 1989/90 to 1991/92, the rate of AAA repair in Ontarians age 45 years and over was 53 per 100,000. (60) In the United States, about 200,000 new cases are diagnosed each year, and 50,000 to 60,000 surgical AAA repairs are performed. (2) Ruptured AAAs are responsible for about 15,000 deaths in the United States annually. One in 10 men older than 80 years has some aneurysmal change in his aorta. (2)

Symptoms of Abdominal Aortic Aneurysms

AAAs usually do not produce symptoms. However, as they expand, they may become painful. Compression or erosion of adjacent tissue by aneurysms also may cause symptoms. The formation of mural thrombi, a type of blood clots, within the aneurysm may predispose people to peripheral embolization, where blood vessels become blocked. Occasionally, an aneurysm may leak into the vessel wall and the periadventitial area, causing pain and local tenderness. More often, acute rupture occurs without any prior warning, causing acute pain and hypotension. This complication is always life-threatening and requires an emergency operation.

Diagnosis of Abdominal Aortic Aneurysms

An AAA is usually detected on routine examination as a palpable, pulsatile, and non-tender mass. (1)

Abdominal radiography may show the calcified outline of the aneurysms; however, about 25% of aneurysms are not calcified and cannot be visualized by plain x-ray. (1) An abdominal ultrasound provides more accurate detection, can delineate the traverse and longitudinal dimensions of the aneurysm, and is useful for serial documentation of aneurysm size. Computed tomography and magnetic resonance have also been used for follow-up of aortic aneurysms. These technologies, particularly contrast-enhanced computer tomography, provide higher resolution than ultrasound.

Abdominal aortography remains the gold standard to evaluate patients with aneurysms for surgery. This technique helps document the extent of the aneurysms, especially their upper and lower limits. It also helps show the extent of associated athereosclerotic vascular disease. However, the procedure carries a small risk of complications, such as bleeding, allergic reactions, and atheroembolism. (1)

Prognosis of Abdominal Aortic Aneurysms

The risk of rupture of an untreated AAA is a continuous function of aneurysm size as represented by the maximal diameter of the AAA. The annual rupture rate is near zero for aneurysms less than 4 cm in diameter. The risk is about 1% per year for aneurysms 4 to 4.9 cm, 11% per year for aneurysms 5 to 5.9 cm, and 25% per year or more for aneurysms greater than 6 cm. (7)

The 1-year mortality rate of patients with AAAs who do not undergo surgical treatment is about 25% if the aneurysms are 4 to 6 cm in diameter. This increases to 50% for aneurysms exceeding 6 cm. Other major causes of mortality for people with AAAs include coronary heart disease and stroke.

Treatment of Abdominal Aortic Aneurysms

Treatment of an aneurysm is indicated under any one of the following conditions:

The AAA is greater than 6 cm in diameter.

The patient is symptomatic.

The AAA is rapidly expanding irrespective of the absolute diameter.

Open surgical repair of AAA is still the gold standard. It is a major operation involving the excision of dilated area and placement of a sutured woven graft. The surgery may be performed under emergent situation following the rupture of an AAA, or it may be performed electively.

Elective OSR is generally considered appropriate for healthy patients with aneurysms 5 to 6 cm in diameter. (7) Coronary artery disease is the major underlying illness contributing to morbidity and mortality in OSR. Other medical comorbidities, such as chronic renal failure, chronic lung disease, and liver cirrhosis with portal hypertension, may double or triple the usual risk of OSR.

Serial noninvasive follow-up of small aneurysms (less than 5 cm) is an alternative to immediate surgery.

Endovascular repair of AAA is the third treatment option and is the topic of this review.

Two cases of severe mitral stenosis with both hoarseness, caused by left vocal cord palsy, and dysphagia are described. This combination in the same patient has not, to our knowledge, been previously described. Previous studies have firmly established that vocal cord paralysis in mitral stenosis is a result of compression of the left recurrent laryngeal nerve as it passes around the aortic arch. It is suggested that dysphagia may also develop as a result of neurological damage, in this case to autonomic nerve plexuses supplying the oesophagus, leading to abnormal peristalsis and enabling external compression by a tense left atrium sufficient to cause symptoms

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Oculomotor nerve palsy (ONP) with subarachnoid hemorrhage (SAH) occurs usually when oculomotor nerve is compressed by growing or budding of posterior communicating artery (PcoA) aneurysm. Midbrain injury, increased intracranial pressure (ICP), or uncal herniation may also cause it. We report herein a rare case of ONP associated with SAH which was caused by middle cerebral artery (MCA) bifurcation aneurysm rupture. A 58-year-old woman with clear consciousness suffered from headache and sudden onset of unilateral ONP. Computed tomography showed SAH caused by the rupture of MCA aneurysm. The unilateral ONP was not associated with midbrain injury, increased ICP, or uncal herniation. The patient was treated with coil embolization, and the signs of oculomotor nerve palsy completely resolved after a few days. We suggest that bloody jet flow from the rupture of distant aneurysm other than PcoA aneurysm may also be considered as a cause of sudden unilateral ONP in patients with SAH.

Rupture of the free wall of the left ventricle (LV) is a catastrophic complication occurring in 4% of patients after myocardial infarction (MI) and in 23% of those who die of MI. Rarely the rupture is contained by an adherent pericardium creating a pseudo-aneurysm. This clinical finding calls for emergency surgery. If no ruptures are detectable and myocardium wall integrity is confirmed, we are in the presence of a true aneurysm, which can be treated by means of elective surgery. Differentiation between these two pathologies remains difficult. We report the case of a patient with a true aneurysm, initially diagnosed as pseudo-aneurysm at our institution; we have reviewed the literature on this difficult diagnosis and outlined characteristic findings of each clinical entity.

Endovascular aneurysm repair has considerable potential advantages over the surgical approach as a treatment for thoracic aortic rupture, in part because open surgical repair of ruptured thoracic aortic aneurysms is associated with high mortality and morbidity rates. We describe the successful endovascular deployment of stent-grafts to repair a contained rupture of a descending thoracic aortic aneurysm in an 86-year-old man whose comorbidities prohibited surgery. Two months after the procedure, magnetic resonance angiography showed a patent stent-graft, a patent left subclavian artery, and complete exclusion of the aneurysm.

An aortic arch extending into the neck is a rare congenital anaomaly. Left-sided cervical aortic arch was thought to be much less common than right-sided, but they are now almost equal in incidence in the published reports. This paper describes a case of left-sided cervical aortic arch in an adult, with a previously undescribed association, aneurysm of the descending thoracic aorta. Cervical aortic arch must be considered in the differential diagnosis of pulsatile cervical swellings. The embryological development of the aortic arch is discussed. This is still controversial and it is not possible to be sure of the embryological explanation of this congenital anomaly of the aortic arch from a study of the adult anatomy.

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