Spontaneous brachial pseudo-aneurysm in a 12-year-old with kyphoscoliosis-type Ehlers-Danlos Syndrome.

The Ehlers-Danlos Syndrome (EDS) is a rare connective tissue disorder characterised by fragility of the soft connective tissues and widespread manifestations in skin, ligaments, joints, blood vessels and internal organs. We report a case of a 12-year-old boy, previously diagnosed with kyphoscoliosis-type EDS (type VI), presenting with a left brachial artery pseudo-aneursym with history of multiple spontaneous and post-traumatic arterial ruptures. Surgical management of this patient was performed successfully by primary repair of brachial artery lesion.

Pseudoaneurysm of the supracoeliac aorta following blunt trauma in a 83-year-old woman.

We report on the case of an 83-year-old hemiplegic female patient presenting with post-traumatic pseudoaneurysm of the supracoeliac aorta, treated with 38 mm x 100 mm Talent Endoluminal Stent-Graft (Medtronic Inc., Santa Rosa, CA) using local anaesthesia. The patient was discharged on day 3 and the 8 month follow-up was without complications.

Coronary artery bypass grafting in a 12-year-old girl with familial hypercholesterolemia.

We present the case of a 12-year-old girl with familial hypercholesterolemia and coronary artery disease. She underwent triple-vessel coronary artery bypass grafting with bilateral pedicled internal mammary artery grafting without adverse events. Pediatric patients with familial hypercholesterolemia may present with premature coronary atherosclerosis requiring coronary artery bypass grafting. In situ internal mammary artery grafts should be the graft of choice.

Modified central aortopulmonary shunt with end-to-end anastomosis of the shunt to the pulmonary trunk: Early results of a novel shunt procedure for first-stage palliation of infants with pulmonary atresia and hypoplastic pulmonary arteries.

OBJECTIVE: The aim was to investigate a novel palliative shunt performed between the ascending aorta and the main pulmonary artery in patients with pulmonary atresia. PATIENTS AND METHODS: Thirteen patients with intracardiac defects [tetralogy of Fallot (seven patients); tetralogy of Fallot and major aortopulmonary collateral arteries (one patient); ventricular septal defect and major aortopulmonary collateral arteries (two patients); tricuspid atresia (three patients)] and pulmonary atresia underwent shunt operation. Patient selection was made based on preoperative echocardiography and perioperative findings. Eight of the patients were female and five were male. Ages and weight ranged between 1-235 days and 2,950-7,900 g, respectively. Preoperative room air oxygen saturation ranged between 68-83 %. RESULTS: Operations were performed through a median sternotomy. The main pulmonary artery was transected from the right ventricular outflow tract and using a 3-3.5 mm diameter graft, a modified central aortopulmonary shunt was created (graft anastomosis was end-to-end to the pulmonary artery and side-to-side to the aorta). Postoperative oxygen saturation increased 5-10% and diastolic blood pressure decreased by 3-6 mmHg. One hospital mortality occurred, due to sepsis, and two late deaths, one due to pneumonia and one sudden death, occurred in the follow-up period. Patients were followed up for 3-16 months after the operations. During the follow-up period four patients received corrective operations, one unifocalization and three total corrections. CONCLUSION: Modified central aortopulmonary shunt with end-to-end anastomosis of the shunt to the pulmonary trunk is a novel palliative shunt operation. Our early results with this technique indicate rapid palliation and early achievement of bilateral homogenous adequate pulmonary artery size with a low incidence of overflow to pulmonary circulation and minimal arterial distortion.

Bronchogenic cyst invading right atrium in a 5-year-old.

Primary bronchogenic cysts of cardiac origin or extension are rare. We report here on a 5-year-old girl with a bronchogenic cyst with a diameter of 5.0 x 4.5 x 4.5 cm extending to the right atrial wall. Tumor enucleation and resection of the cyst together with the invaded right atrial wall was performed through a right posterolateral thoracotomy and an opening in the lateral pericardium. Resection of intrapericardial bronchogenic cysts is possible, although extensive invasion of cardiac structures may necessitate the use of cardiopulmonary bypass through a sternotomy.

Thymoma: surgical results of 73 cases.

PURPOSE: Thymomas are uncommon tumours; they represent a broad spectrum of morphological and clinical behaviour. The purpose of this study is to identify the factors that influence survival. METHOD: Seventy-three patients with thymoma who underwent thymectomy between 1980 and 2002 were reviewed. Prognostic factors assessed were: age, sex, presenting symptoms, histological features, stage at diagnosis and extent of surgical resection. RESULTS: Complete resection was achieved in 67 patients and incomplete in 6. Survival rates of patients in the 1st or 2nd stages were 100% and 92.8% for 10 years, respectively. A high incidence of myasthenia gravis (MG) associated thymomas (80.8%) was found. These patients had better survival rates when compared to non-myasthenic patients. However the mortality rate for malign thymomas was high (65.7%) due to tumoral invasion. The histological types of thymoma did not influence the survival rate. CONCLUSIONS: The association of MG with thymoma enables earlier detection of tumour, thus higher survival rates were achieved in these patients. Survival was also related to the stage of the tumour at diagnosis and the completeness of resection.

Recurrent apical cardiac hydatid cyst presenting with angina.

Hydatid disease is a parasitic infection caused by the larvae of tapeworm Echinococcus Granulosus. Hydatid cyst of the heart is an uncommon presentation of human echinococcosis which may lead to life-threatening conditions. We present a rare case of recurrent pericardial cyst in a 42-year-old man presenting with chest pain and ECG findings. We were able to avoid risks of resternotomy with a limited anterolateral thoracotomy. A direct enucleation of the cyst was possible without the need for cardiopulmonary bypass. He was free of complications at 6-month follow-up. Diagnosis should be suspected in every case of anginal symptoms or cyst-like mass in persons coming from areas where echinococcus granulosus is endemic.

Treatment of chronic type B aortic dissection by endovascular grafting in a previously CABG patient. Case report.

High mortality rates resulting from the surgical treatment of Type B aortic dissections have played an important role in seeking newer alternative therapy modalities for the treatment of the pathology. Especially since the early 1990s, endovascular grafting has become a popular option in the treatment of aortic diseases and with the increasing experience and advances in technology, it has, in recent years, become an alternative approach for the treatment of aortic dissections. In this report, we present the treatment of a 56-year-old male patient with a history of a coronary artery bypass grafting who presented with chronic type B aortic dissection. Endovascular stent graft implantation was performed with a hybrid therapy type of preliminary right subclavian artery to left subclavian artery bypass, followed by endovascular stent graft implantation to the dilated aneurismal segment of the descending aorta, for the treatment of chronic type B aortic dissection. The procedure and the postoperative course were uneventful; moreover, they were very comfortable for the patient undergoing an investigation of the descending aorta, when compared with the conventional surgical treatment modalities.

Use of a bifurcated ePTFE graft for off-pump extracardiac Fontan completion.

A 6-year-old girl underwent off-pump extracardiac Fontan completion. Intrapericardial inferior caval vein was found to be accompanied by the hepatic vein on the left side; a 20 x 10 x 10-mm bifurcated ePTFE graft was used without external shunting. This approach is practical for the surgeons and may result in lower perioperative Fontan pressures/transpulmonary gradients as well as a shorter intubation period and hospital stay.

Aortic arch replacement without circulatory arrest: a simplified technique with two grafts.

Aortic arch reconstruction remains a challenge for the cardiothoracic surgeon. We present our simplified technique in which we used two grafts for arch reconstruction without circulatory arrest. It is a relatively easy and quick technique consisting of a unique modification of the selective antegrade cerebral circulation system without the need for circulatory arrest in selected cases.

Extracardiac Fontan operation without cardiopulmonary bypass.

AIM: The avoidance of cardiopulmonary bypass and aortic cross-clamping in patients possessing single ventricular physiology has potential advantages including preservation of ventricular and pulmonary functions; early extubation, decreased incidence of pleural effusions and decreased requirement of inotropic agents and blood products. In this study, we assessed the postoperative outcome of patients who have undergone extracardiac Fontan operation performed without cardiopulmonary bypass. METHODS: Between March 1999 and August 2002, 10 consecutive patients (6 males and 4 females) underwent extracardiac Fontan operation without cardiopulmonary bypass. The age of patients ranged between 1.5 to 12 (5.2+/-3.1) years. All the patients requiring any intracardiac intervention were excluded from the study. Previous operations of the patients were modified Blalock-Taussig shunt procedure in 3 patients, bidirectional cavopulmonary shunt operation in 2 patients and pulmonary arterial banding in 1 patient. All operations were performed without cardiopulmonary bypass. Bidirectional cavopulmonary anastomosis was performed by using a transient external shunt constructed between the superior vena cava and right atrium. An appropriate sized tube graft was anastomosed to the inferior surface of right pulmonary artery. Finally, inferior vena cava to tube grafts anastomosis was performed with the aid of another external shunt constructed between inferior vena cava and right atrium. During the procedure central venous pressure, blood pressure and arterial oxygen saturation levels were continuously monitored and recorded. RESULTS: The mean intraoperative Fontan pressure was 16.1+/-2.75 mmHg. Intraoperative fenestration was required in 4 patients with a Fontan pressure above 18 mmHg. There were no intra and postoperative deaths. Three patients required mild doses of inotropic support during the postoperative period. All patients were weaned off mechanical ventilation within 24 h. The mean arterial oxygen saturation raised from 74.5+/-4.2% to 93.5+/-2%. Arterial oxygen saturation was 95+/-0.6% in 6 patients without fenestration and 91.2+/-0.5% in 4 patients with fenestration (P=0.001). All patients were in sinus rhythm postoperatively. Only 2 patients required blood transfusion. Two patients suffered from prolonged pleural effusion (more than 7 days). The mean intensive care unit and hospital stay periods were 3.3+/-1.5 and 15.4+/-5.3 days, respectively. CONCLUSIONS: The extracardiac Fontan operation performed without cardiopulmonary bypass provides good results in short and midterm follow-up periods with improved postoperative hemodynamics.

Anomalous dual drainage of the right pulmonary veins in a patient with cor triatriatum: report of a case without scimitar sign.

The majority of patients with partial anomalous drainage of pulmonary veins are asymptomatic during infancy and childhood. Patients with significant left-to-right shunt develop symptoms and benefit from early corrective surgery. Anomalous pulmonary veins draining into inferior vena cava is very rare and frequently encountered in association with scimitar syndrome. The purpose of this case report is to describe a non-scimitar patient with cor triatriatum who had anomalous dual drainage of right pulmonary veins into inferior vena cava/left atrium and anomalous connection of persistent left superior vena cava with a common pulmonary venous chamber. The patient underwent an operation with redirection of anomalous pulmonary venous drainage into left atrium and ligation of persistent left superior vena cava.

[The vascular manifestations of Behçet's disease: a case report]. / Les manifestations vasculaires de la Maladie de Behçet: a propos d'un cas.

Behçet's disease is an inflammatory vasculitis which affects the arteries and veins. The vascular pathologies are the rare complications of this disease. We present here a patient with Behçet's disease who has been hospitalized several times because of plurifocal vascular manifestations.

The surgical treatment of abdominal aortic aneurysms in patients with light to moderate renal dysfunction.

OBJECTIVE: The incremental surgical risk caused by mild or moderate renal dysfunction in patients undergoing surgery for an abdominal aortic aneurysm is not well defined. The aim of this study is to analyze the prognostic significance of mild or moderate renal dysfunction in 79 patients who have undergone repair of an infrarenal abdominal aortic aneurysm at our institution. METHODS: The postoperative results of 35 non-dialysis-dependent patients (Group I) with renal dysfunction were included and compared with a group of 44 patients (Group II) with similar characteristics of age, gender and pre-operative risk factors. All operations were performed through a standard transperitoneal median laparotomy. RESULTS: The mean operation and aortic cross-clamp times did not differ significantly between the two groups (234+/-15 min vs. 189+/-17min ; p = 0.25 and 57+/-12 min. vs. 52+/-9 min. ; p = 0.52). Early operative mortality was 5.71% in patients with renal dysfunction and did not differ significantly when compared with the other group of patients 4.5%; p = 0.083). Blood requirement was higher (p < 0.01) and the hospital stay was longer (p < 0.001) in group I. There were 18 patients in group I (51.4%) and three patients in group II (6.8%) in whom the pre-operative serum creatinine level increased above 2.5 mg/dl during the postoperative period. The postoperative increase of creatinine level differed significantly in patients with pre-operative renal dysfunction (p < 0.001). There were nine patients (25.7%) in group I and four patients (9.1%) in group II who required mechanical ventilation for more than 24 hours (p = 0.025). There was no significant difference between the two groups in terms of cardiac morbidity p = 0.234). CONCLUSION: Patients with even a mild or moderate degree of renal dysfunction require close monitoring and prolonged hospital care during the postoperative period. Although satisfactory early surgical outcomes may be expected in this particular group of patients, pulmonary and renal morbidity rates are higher than in patients with normal renal function.

A comparison between minilaparotomy and standard median laparotomy for reconstruction of aorto-iliac occlusive disease.

OBJECTIVE: Minimally invasive surgical procedures have become increasingly used in all surgical branches. In this respect we compared the minilaparotomy (ML) technique with standard median laparotomy (SML) for the surgical treatment of aorto-iliac occlusive disease. METHODS: 120 patients were included in this prospective study, with 60 patients in the minilaparotomy group and 60 patients in standard median laparotomy group. The two approaches were used within the same time period. Both groups were compared in terms of operating and aortic cross clamp time, the amount of blood transfusion, length of stay in the intensive care unit and hospital, and time for normalization of bowel functions. RESULTS: There were no significant differences in terms of the amount of blood transfusion, or aortic cross clamp time. The minilaparotomy approach has the advantages of less operating time, earlier return to a general diet, decreased length of stay in the intensive care unit and hospital and reduced cost. CONCLUSIONS: As a conclusion, we believe that the minilaparotomy technique will be the standard treatment of choice for aorto-iliac occlusive disease in the future with reduced use of facilities and lower cost than the laparoscopic or the standard technique.

Anterior spinal artery syndrome after infrarenal abdominal aortic surgery.

BACKGROUND: Neurological complications such as paraplegia or paraparesis due to spinal cord ischemia has been an unpredictable, devastating event after infrarenal abdominal aortic surgery. The aim of our study is to focus the importance of this entity and in this connection to review the vascular anatomy of the spinal cord, incidence and etiology of spinal cord ischemia, methods of prevention, and management of the patient. METHODS: Eight patients were identified with spinal cord ischemia manifested by paraplegia or paraparesis after 1331 abdominal aortic operations. All the patients who had spinal cord ischemia were examined for risk factors. RESULTS: Three anterior spinal artery syndrome neurological recovery occurred but 5 of them remained unchanged. Two patients died within 30 days of operation. CONCLUSIONS: Complete paraplegia due to ischemic spinal cord injury was thought to be caused by interruption of critical collateral blood supply to the spinal cord. For this reason avoidance of prolonged aortic cross clamp time, hypotension, and its associated low flow to the spinal cord, paying attention to prevent atheromatous embolization of Adamkiewicz artery and pelvic circulation can prevent this complication. If the greater medullary artery is anomalously low from the anatomic localization the blood supply of the cord may be inadequate, so this complication cannot be avoided.

Uncommon complication of arterial switch operation: tracheobronchial compression.

We describe 2 patients in whom symptoms of airway compression developed after arterial switch operation for correction of the transposition of the great arteries. The clinical features, diagnosis, management, and proposed mechanisms of this complication are described.

Case of spontaneous haemothorax associated with an endodermal sinus tumour.

Spontaneous haemothorax is rare in infants. A case is reported of a nine month old infant who was found to have an endodermal sinus tumour.