V. Deutsch

Hepatic Telangiectases and Portosystemic Encephalopathy in Osler-Weber-Rendu Disease

Summary A patient suffering from hereditary hemorrhagic telangiectases presented with an epigastric bruit and experienced, for 6 months prior to her death, repeated episodes of portosystemic encephalopathy. Angiography demonstrated tremendous dilation of the hepatic arteries and extensive portacaval shunting of blood through telangiectatic channels in the liver parenchyma. The latter were confirmed by biopsy and, later, at autopsy, which also revealed the absence of hepatic scarring. The occurrence of portosystemic encephalopathy due to hepatic telangiectases represents a new aspect of Osler-Weber-Rendu disease.

Juxtaposition of atrial appendages: Angiocardiographic observations

Abstract The angiographic features of four cases of left-sided juxtaposition and one case of right-sided juxtaposition of the atrial appendages are described. The anomalous appendage was cephalad and dorsal to the normal position of the left atrial appendage and was larger than normal during atrial diastole; the appendage located on the anatomically correct side was caudad and its diastolic size was less than normal. The four cases of left-sided juxtaposition were all associated with transposition of the great vessels. Right-sided juxtaposition was detected inadvertently in a patient with a congenital obstructive lesion of the mitral valve. Left-sided juxtaposition can be demonstrated angiocardiographically by opacification of the right atrium, whereas right-sided juxtaposition can be seen by contrast injection into any cardiac chamber but the left ventricle, with certain exceptions.

Criss-cross heart—a case with horizontal septum, complete transposition, pulmonary atresia and ventricular septal defect

SummaryCriss-cross heart is a recently described anomaly in which the systemic and pulmonary blood streams cross at the atrioventricular (AV) level, without mixing. A case of criss-cross heart is described in which the right atrium, in a solitus position, communicated with a left-superior positioned, morphologically right ventricle, and the left atrium communicated with a normally located, morphologically left ventricle. The interventricular septum occupied a horizontal plane. Associated defects were complete d-transposition of the great arteries with l-positioned aorta, pulmonary atresia, venticular septal defect, atrial septal defect, and patent ductus arteriosus. To the best of our knowledge this is the first angiocardiographic demonstration of this rare combination of lesions.The literature on criss-cross heart and horizontal septum is reviewed. It is stressed that regardless of whether the criss-cross phenomenon is an anatomical fact or an angiocardiographic illusion, it is an established angiocardiographic entity and should be recognized as such.

Retroesophageal right aortic arch

SummaryRight aortic arch with left descending aorta and a retroesophageal aortic segment is an uncommon congenital vascular malformation which may cause symptoms of compression from a vascular “ring.” The presence of this malformation may be suspected by the findings on barium swallow and the diagnosis confirmed by angiocardiography. Surgical intervention may be necessary in patients who have severe symptoms.

Cavernous hemangioma of the spleen; angiographic observations.

A 65 year old female Arab patient was admitted because of swelling of the abdomen, which was first noted four months previously. In the past she had complained of weakness and loss of weight. There was no history of vomiting or change in bowel habit. She denied previous illnesses and had never been hospitalised. On examination she appeared ill and emaciated. A hemangioma 2 x 2 cm in size was seen on the left side of the tongue. The rest of the oral mucosa was normal. Small mobile lymph nodes were palpable in both axillae and inguinal regions. There was no rash on the skin nor signs of pruritus. The lungs were clear and the heart sounds were normal. A systolic murmur grade II/VI was heard over the precordium. Blood pressure was 160/100. The spleen was enlarged, its lower border reaching 4 cm below the umbilicus. It was firm and non tender. The liver was palpable 6 cm below the costal margin in the mid-

Right aortic arch with left descending aorta (circumflex aorta) Roentgenographic diagnosis

Right aortic arch with retroesophageal segment is a rare anomaly with distinctive roentgenographic features. We present 2 patients, who on the barium oesophogram had an additional finding which has not previously been described. The typical features include an indentation on the right upper barium filled oesophagus (in the antero-posterior view), an oblique indentation descending from right to left (caused by the retroesophageal segment) and the left descending aorta. The oblique indentation has not previously been described. The embryology and incidence of the lesion are briefly discussed.

Contracted form of endocardial fibroelastosis: angiographic diagnosis and necropsy confirmation.

Endocardial fibroelastosis, a condition in which there is diffuse thickening of the left ventricular endocardium, may be divided into two types, primary and secondary, the latter being associated with and usually secondary to cardiac anomalies [1, 5]. The primary form may be subdivided into two types--a dilated form, which is far more common and in which the left ventricle is large, and a contracted form in which the left ventricle is normal or small in size [1]. There are a few reports illustrating the features of the contracted form [3, 6, 7]. In these cases the left ventricle was usually of normal size and contracted well. In fact, in some cases the left ventricle was even thought to be somewhat enlarged [3]. We here describe a case of primary contracted endocardial fibroelastosis in which the diagnosis was made by angiocardiography and confirmed at necropsy.

Liposarcoma of the renal capsule in a 7 year old girl

An unusual case is reported of a liposarcoma of the renal capsule in a 7 year old girl. Initial radiological studies revealed a fatty tumor related to the lower pole of the kidney. Selective renal angiography located the tumor to renal capsule and demonstrated its malignant nature.

Congenital Renal Arterio-Venous Fistula

From the Department of Diagnostic Radiology, The Chaim Sheba Medical Center, Tel Hashomer, and the Tel Aviv University Medical School, Tel Aviv, Israel. Congenital renal arterio-venous (A-V) fistulas are rare. Of 121 cases of renal A-V fistulas reported in the literature, 32 cases were considered congenital or idiopathic in origin.5, 6The application of sophisticated angiographic procedures has led to a greater awareness of renal A-V fistula. As a result, many features of the entity are now better understood and diagnosis is being considered earlier. Herein we present a case of renal congenital A-V fistula and review the diagnostic features.

Duplication and Paired Inferior Vena Cava With an Absent Right Kidney

* From the Department of Diagnostic Radiology, Chaim Sheba Medical Center, Tel Hashomer, and the Tel Aviv University Medical School, Tel Aviv, Israel † Associate Professor of Radiology, Stanford University School of Medicine, Stanford, California The embryological development of the inferior vena cava, and particularly its postrenal division, is exceptionally complex. Simultaneous embryological differentiation and regression of various segments of three paired longitudinal venous channels draining the lower extremities and abdomen takes place, (4, 6, 6). The evaluation of these variations of the inferior cava is now a necessity, in the light of recent progress in vascular surgery, in particular ligation or plication of the inferior vena cava for prevention of pulmonary embolism, and portocaval shunting for the treatment of bleeding esophageal varices. Recognition of these anomalies is simplified by angiography and venography. The report presented here describes an exceedingly rare type of caval anomaly found incidentally at angiography which had been performed for evaluation of