Asymptomatic thrombosis of extrahepatic portal vein aneurysm necessitating hybrid operative repair.

BACKGROUND: Congenital portal vein aneurysm is a rare vascular anomaly with poorly understood natural history. Whereas asymptomatic aneurysms are often managed conservatively, surgery has been used in symptomatic cases complicated by thrombosis or rupture. Surgical experience in management of portal aneurysms is restricted to case studies with limited comparative data and inconsistent reporting of outcomes. A hybrid open and endovascular approach has rarely been described in the literature. METHODS: We present a case of an extrahepatic portal aneurysm which demonstrated changes on surveillance imaging concerning for early asymptomatic thrombosis. Acute thrombus was identified at the time of open aneurysm repair. We review the limited literature regarding management of portal vein aneurysms in non-cirrhotic patients. RESULTS: Our case was complicated by intrahepatic thrombo-embolism, which necessitated hybrid thrombectomy and anticoagulant therapy. The patient remains asymptomatic at three-year follow-up with no recurrent aneurysm or thrombosis on surveillance Doppler and CT imaging. CONCLUSIONS: Altered hemodynamic appearances on Doppler ultrasound and contrast-enhanced CT may warn of impending thrombosis in portal vein aneurysms. Hybrid open and endovascular surgical repair ensures vessel patency and a durable surgical result.

Congenital superior mesenteric artery aneurysm in a 6-week-old infant presenting with upper gastrointestinal bleeding.

Visceral artery aneurysms are rare in infants and children. The majority of cases are caused by genetic syndromes, trauma, or infection. Although the majority of aneurysms are asymptomatic, visceral artery aneurysms can present with abdominal pain, nausea/vomiting, or rupture. Aneurysm rupture can manifest as hemodynamic instability and/or gastrointestinal bleeding. We present the case of a congenital idiopathic aneurysm of the superior mesenteric artery in a 6-week-old infant who presented with gastrointestinal bleeding. We report a stepwise surgical approach to achieving aneurysm exclusion and thrombosis, and highlight the robust mesenteric collateral circulation that can develop in pediatric patients.

Congenital superior caval vein aneurysm in a newborn with cystic lymphangioma: a rare case report.

Dilatation of the superior caval vein is extremely rare, with few cases described among newborns. The association of aneurysm of the superior caval vein and lymphatic malformation is extremely uncommon. We report a case of a female infant with a prenatal diagnosis of superior caval vein aneurysm presenting at birth with a neck mass that was found to be a cystic lymphangioma.

Transarterial Embolization of a Renal Artery Aneurysm Concomitant With Renal Arteriovenous Fistula.

Congenital renal artery aneurysm is uncommon. Moreover, renal artery aneurysm concomitant with a congenital renal arteriovenous fistula is extremely rare. Transarterial embolization is the first-line treatment for these conditions. We report a case of a patient with congenital renal artery aneurysm concomitant with a congenital renal arteriovenous fistula of the upper polar left renal artery which was successfully treated by transarterial embolization with coil, glue, and Amplatzer vascular plug.

True brachial artery aneurysm in a child aged 2†years.

Congenital brachial artery true aneurysms are exceedingly rare. Most are pseudoaneurysms secondary to trauma or infection. We report a boy aged 2 years who presented with painless, pulsatile swelling on the medial aspect of the right arm, 4 cm above the elbow joint that had been present since birth. Spiral CT angiography showed a fusiform aneurysm of the distal right brachial artery with a peripheral crescent-shaped thrombus. Distal arteries were normally opacified. There was no evidence of abnormal dilation or stenosis in any other artery. The aneurysm was surgically resected, with vascularisation re-established using a reversed great saphenous vein graft. His postoperative course was uneventful. Early surgery should be performed for moderately sized to large aneurysms that recently increased in size, exhibited luminal thrombus formation or caused neurovascular distal limb compromise. Early surgery could prevent complications such as a ruptured aneurysm, thromboembolism or limb ischaemia or loss.

Portal vein aneurysm: What to know.

Portal vein aneurysm is an unusual vascular dilatation of the portal vein, which was first described by Barzilai and Kleckner in 1956 and since then less than 200 cases have been reported. The aim of this article is to provide an overview of the international literature to better clarify various aspects of this rare nosological entity and provide clear evidence-based summary, when available, of the clinical and surgical management. A systematic literature search of the Pubmed database was performed for all articles related to portal vein aneurysm. All articles published from 1956 to 2014 were examined for a total of 96 reports, including 190 patients. Portal vein aneurysm is defined as a portal vein diameter exceeding 1.9 cm in cirrhotic patients and 1.5 cm in normal livers. It can be congenital or acquired and portal hypertension represents the main cause of the acquired version. Surgical indication is considered in case of rupture, thrombosis or symptomatic aneurysms. Aneurysmectomy and aneurysmorrhaphy are considered in patients with normal liver, while shunt procedures or liver transplantation are the treatment of choice in case of portal hypertension. Being such a rare vascular entity its management should be reserved to high-volume tertiary hepato-biliary centres.

Congenital aneurysm of the aortomitral intervalvular fibrosa.

A 5-year-old boy was found to have a congenital left ventricular outflow tract (LVOT) aneurysm of the intervalvular fibrosa, LVOT obstruction after repair of a perimembranous ventricular septal defect, and aortic coarctation. The patient underwent successful plication of the aneurysm, resection of the fibrous subaortic stenosis, and septal myectomy.

Giant pulmonary artery aneurysm in a 6-year-old child.

A 6-year-old female child was admitted with complaints of progressive dyspnea on exertion since last 3 years. She was saturating 100% on room air with stable vitals. On clinical examination, left precordial bulge was seen with prominent epigastric pulsations. Chest X-ray showed rightward shift of mediastinum and huge homogenous opacity occupying almost entire left lung field. On two-dimensional echocardiography, a large aneurysmal mass was seen occupying left hemithorax which was suspected to be arising from pulmonary artery but its exact site of origin could not be determined. A small patent ductus arteriosus (PDA) was also seen. PDA could be seen directly supplying the aneurysm (Fig. 1). CT angiography was done to confirm the diagnosis. It showed a huge aneurysmal sac measuring 12 cm × 8.9 cm × 14 cm, arising from left pulmonary artery (LPA) opposite to the site of insertion of PDA. Sac was occupying most of the left lung and pushing down the left diaphragm (Fig. 2). Cause of formation of such a huge aneurysm could not be found out. Surgery was performed and mouth of the sac was closed along with ligation of duct, repair of LPA and drainage of the sac. After surgery, lung expansion was good. Patient was discharged and she is doing well.

Pulmonary artery aneurysm with patent arterial duct: resection of aneurysm and ductal division.

Congenital or acquired aneurysm of the pulmonary artery (PA) is rare. Although aneurysms are described following surgical treatment of patent ductus arteriosus (PDA), occurrence of this lesion in association with PDA without previous surgery is extremely uncommon. An eight-year-old patient with PDA and aneurysm of the main PA is described in this report. Clinical diagnosis of PDA was made upon presentation. Diagnosis of PA aneurysm was suspected on chest x-ray and was confirmed on transthoracic echocardiography. Successful surgical resection of the aneurysm and division of the duct were performed under cardiopulmonary bypass. The patient did well on follow-up both from clinical and echocardiographic point of view.

Subclavian and axillary arterial aneurysms: two case reports / Aneurismas do segmento subclavio-axilar: relato de dois casos

Aneurysms of the subclavian-axillary segment are rare, but when diagnosed they must be treated. This article describes two cases of aneurysms of the upper extremities, one in a subclavian artery and the other in an axillary artery. The first case was a 71-year-old male with a pulsating supraclavicular bulge on the right and muscle weakness in the ipsilateral extremity. Duplex scanning and arteriography confirmed the diagnosis of aneurysm of the right subclavian artery and the patient underwent aneurysmectomy and end-to-end anastomosis. The second case was a 24-year-old female patient, with no history of comorbidities, who presented with a pulsating mass in the right axillary region and paresthesia of the ipsilateral extremity. Duplex scanning and arteriography confirmed an aneurysm in the right axillary artery, which was successfully treated with aneurysmectomy and end-to-end anastomosis. Pathology findings showed that the first case was an atherosclerotic aneurysm and the second was a congenital aneurysm.

Os aneurismas do segmento subclávio-axilar são de ocorrência rara e, uma vez diagnosticados, devem ser tratados. Neste trabalho, relatamos dois casos de aneurismas de membros superiores, sendo um da artéria subclávia e outro da artéria axilar. No primeiro caso, o paciente de 71 anos, sexo masculino, apresentava abaulamento pulsátil supraclavicular direito associado à fraqueza muscular no membro ipsilateral. Foram realizados duplex scan e arteriografia, que confirmaram o diagnóstico de aneurisma da artéria subclávia direita, sendo o paciente submetido à aneurismectomia com anastomose término-terminal. No segundo caso, uma paciente de 24 anos, do sexo feminino, sem antecedentes mórbidos ou comorbidades, apresentava queixa de massa pulsátil na região axilar direita associada à parestesia no membro ipsilateral. O duplex scan e a arteriografia confirmaram o aneurisma da artéria axilar direita, que foi tratado com aneurismectomia e anastomose término-terminal com sucesso. O diagnóstico anátomo-clínico foi de aneurisma aterosclerótico no primeiro caso e, no segundo caso, de aneurisma congênito.

Anomalous splenic artery aneurysm.

Aneurysms of the splenic artery are the most common visceral aneurysm. A splenomesenteric trunk, which involves the splenic artery arising from the superior mesenteric artery (SMA), is rare and occurs in less than 1% of patients. Thus splenic artery aneurysms (SAAs) with an anomalous origin from the SMA are quite rare. We report our experience with the surgical management of a 2.6-cm aneurysm involving a splenic artery arising from the SMA in a 40-year-old woman. This was treated with surgical resection with preservation of the spleen. A discussion about SAAs and the management of aneurysms arising from a splenomesenteric trunk follows.

A rare case of left lung hypoplasia associated with congenital pulmonary artery aneurysm and ventricular septal defect.

This report describes a 3-year-old boy who presented with hemoptysis and a history of congenital heart disease detected at the age of 3 months. Clinical and laboratory evaluation showed a large true congenital pulmonary artery aneurysm of the left main pulmonary artery occupying the entire left hemithorax and two small aneurysms in the right lung. In addition, a restricted ventricular septal defect was detected. It was evident on clinical and histologic grounds that the multiple aneurysms seen in this child stemmed from congenital weakness of the arterial wall in conjunction with increased pulmonary blood flow and elevated pulmonary artery pressure.

Evolution of congenital malformations of the umbilical-portal-hepatic venous system.

OBJECTIVE: The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life. MATERIALS AND METHODS: All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution. RESULTS: Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic. CONCLUSION: Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants.

Repair of Kommerell's diverticulum with aberrant left subclavian artery in an elderly patient with right aortic arch and dysphagia lusoria.

A 60-year-old femalepresented with progressive dysphagia and was found to have a right-sided aortic arch with external posterior compression of the upper esophagus due to severe compression from a Kommerell's diverticulum with an aberrant left subclavian artery. This patient underwent division of the diverticulum with an aorto-subclavian graft implantation under femoro-femoral bypass and recovered uneventfully. A review of the literature and discussion of the surgical management is presented.

Staged coil embolisation for the management of persistent aneurysmal right subclavian artery.

A 70-year-old patient, who had previously undergone open surgical repair of an aneurysmal aberrant right subclavian artery (ARSA), presented with recurrent symptoms and reperfusion of the aneurysm at the site of proximal ligation. This was successfully treated by a staged endovascular procedure.

Aneurysm of the aberrant right subclavian artery: surgical and hybrid repair of two cases in a single center.

The aberrant right subclavian artery (ARSA) aneurysm is rare; however, the risk of rupture and thromboembolism is high, with a postrupture mortality rate of 50%. In this report, we have described two cases of this anomaly. In the first case, a 62-year-old male patient presented with a symptomatic aneurysm of ARSA (maximum diameter of 4 cm) causing chest pain with dyspnea during moderate physical effort. Surgical treatment was performed with aneurysmal exclusion and direct anastomosis of the two heads of the subclavian artery. In the second case, a 72-year-old male patient presented with a symptomatic aneurysm (maximum diameter of 5.1 cm) of ARSA causing dysphagia and dysphonia. In this case, a one-stage hybrid treatment was performed: a bilateral carotid-subclavian bypass was associated with intentional occlusion of both subclavian arteries (by plug positioning) during thoracic endovascular stent-grafting procedures. In both cases, the peri/postoperative course was uneventful and the technical results in our series were excellent at long-term follow-up. Surgical treatment can be safely performed in patients with low operative risk or whenever endovascular technique is not suitable. The ARSA aneurysm, with appropriate anatomy, can be successfully treated by hybrid treatment (combined surgical and endovascular approach). We reckon that this minimally invasive technique helps avoid thoracotomy and could be the treatment of choice in high-risk patients.

Congenital-idiopathic superficial femoral artery aneurysm in a 7-year-old child.

Superficial femoral artery aneurysm in children is distinctly uncommon, and usually results from infection, vasculitis, connective tissue disorder, or trauma. We report a 7-year-old girl who had multiple fusiform aneurysms of the right superficial femoral artery, with no evidence of related disorders. The patient successfully underwent aneurysm resection and femoral artery reconstruction with autogenous saphenous vein. Histologic examination revealed intimal thickening with fibroplasia without severe inflammatory infiltrates or cystic medial necrosis, suggesting a congenital-idiopathic arterial aneurysm. Three years after the procedure, the saphenous vein graft is fully patent and the patient is in good condition.

Compression of the left main coronary artery by a pulmonary artery aneurysm in a patient with tetralogy of Fallot and an absent pulmonary valve.

A case of a 16-year-old female with tetralogy of Fallot and absent pulmonary valve is presented, who on coronary angiography and computerized tomography (CT) angiography had severe compression of the left main coronary artery by the dilated main pulmonary artery. The patient was successfully managed by surgical correction of the intracardiac defect, with right ventricular outflow tract reconstruction by the Contegra(®) bovine jugular vein conduit.