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.

Autopsy and postnatal follow-up of prenatally diagnosed ventricular outpouchings.

OBJECTIVES: Prenatal ventricular outpouchings (VOs), which include congenital ventricular aneurysms (CAs) and congenital ventricular diverticula (CD), are very rare. We describe the features and outcomes of prenatal VOs diagnosed over a 4-year period. METHODS: Retrospective cohort study of cases of prenatal diagnoses of CAs and CD at our center between June 2014 and January 2018. The prenatal and postnatal echocardiogram data were reviewed, and telephone follow-up was conducted of liveborn cases. RESULTS: A total of 25 VOs were identified. Two were lost to follow-up, 15 chose termination of pregnancy, and eight resulted in livebirths. Only two cases underwent autopsy: Histopathology showed that the CA wall was substituted by collagen fibers. At follow-up, none of the eight liveborn babies experienced adverse events, and three VOs near the tricuspid annulus almost disappeared, though one was extremely large. CONCLUSIONS: In our center, all liveborn babies with VO had good prognoses. We hypothesize that VOs located near the right ventricular annulus may be caused by prenatally unbalanced pressure, given their decrease in size after birth when the right heart pressure declines.

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.

Intestinal obstruction with a twist: a rare case of congenital portal vein aneurysm causing intestinal obstruction.

Bilious vomiting is often a presenting feature of upper intestinal obstruction in newborn. We present a case of intestinal obstruction in a newborn baby caused by abnormal vascular band arising from portal vein aneurysm in association with a midgut volvulus. Congenital anomalies of portovenous system are very rare, and it usually presents with portal hypertension in late infancy or childhood. In this particular child, the portal vein aneurysm contributed to intestinal obstruction due to both a failure of intestinal rotation and a mechanical band over the transverse colon.

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.

Prenatal sonographic diagnosis of a vitelline vein aneurysm.

We report a case of vitelline vein aneurysm detected at 23 weeks of gestation. Few postnatal cases of vitelline vein aneurysm have been reported; however, due to their similar appearances most of them were considered initially as umbilical vein dilatations. The accurate prenatal diagnosis of vitelline vein aneurysm and early postnatal surgical treatment are crucial steps to prevent postnatal obliterative extension of thrombosis that might cause severe neonatal morbidity.

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 and portal biliopathy.

Highlight Kurtcehajic and colleagues present a rare case of congenital portal vein aneurysm (PVA) with biliopathy. Symptoms associated with PVA occur in less than 10% of cases. Imaging modalities showed the PVA partially compressing the common and right hepatic ducts. Conservative treatment markedly lowered bilirubin levels and relieved the abdominal pain.

Congenital internal jugular vein aneurysm in an infant: A rare entity.

A 1-month old baby boy presented with a mass at the root of the neck. On investigation, a saccular aneurysm arising from the internal jugular vein was diagnosed. The aneurysm was excised after ligating the patent internal jugular vein above and below the origin of the aneurysm. Histopathology confirmed the diagnosis of a vascular malformation. Vascular malformation of the internal jugular vein, presenting as neck mass, is extremely rare with no case described in neonates. We present one such interesting case.

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.