An unusual choledochal cyst

We report a case of Caroli's disease associated with diverticulae and choledochocele of the common bile duct, a wide pancreaticobiliary angle and non-cirrhotic portal hypertension. This patient presented with recurrent episodes of cholangitis. To our knowledge, such a range of findings in the same patient has not been previously reported in the English language literature.     

Transesophageal Echocardiography During Radiofrequency Ablation of Left-Sided Free Wall Atrioventricular Accessory Pathways in Wolff-Parkinson-White Syndrome

This study reports our initial experience in performing radiofrequency ablations (RFA) of Wolff-Parkinson-White accessory pathways in the lateral wall of the left ventricle with monitoring and guidance provided by transesophageal echocardiography (TEE). Fifty RFA procedures were performed in 48 patients. TEE was used to detect underlying heart disease (in nine patients), monitor coronary sinus catheterization (in 15 patients), guide atrial transseptal catheterization (in 29 procedures), help position the RFA catheter's tip, and evaluate for possible complications in all patients. The study suggests that TEE may help to guide electrophysiologists during their initial experience with RFA procedures.     

Bundle Branch Reentrant Ventricular Tachycardia:

Sustained Bundle Branch Reentrant Tachycardia. introduction: The clinical, electrophysiologic features and follow-up of 48 patients with inducible bundle branch reentrant (BBR) tachycardia are presented. Methods and Results: Forty-eight patients were identified in whom a diagnosis of BBR tachycardia was made during electrophysiologic evaluation. The clinical presentation was syncope or sudden death in 38 patients, and sustained palpitations during wide QRS complex tachycardia in 5 patients. Electrophysiologic studies were performed in 5 additional patients for various other reasons. Structural heart disease was present in 45 patients. Idiopathic dilated cardiomyopathy and coronary artery disease were the anatomical substrates in 19 (39%) and 24 (50%) patients, respectively, severe aortic regurgitation was present in 2 patients, and no organic heart disease was identified in 3. All 48 patients had evidence of His-Purkinje system disease. BBR tachycardia with left and right bundle branch block morphologies was induced in 46 and 5 patients, respectively, and interfascicular BBR tachycardia was initiated in 2 patients. Ventricular tachycardia of a myocardial origin was induced in 11 patients. Management of BBR tachycardia included transcatheter bundle branch ablation in 28 patients, and antiarrhythmic drug therapy in 16 patients. Four patients were treated with implantablc defibrillators. After a mean follow-up of 15.8 months in 42 patients, there were 13 deaths due to congestive heart failure, 4 sudden cardiac deaths, 3 nonsudden cardiac deaths, and 3 noncardiac related deaths. Conclusion: Sustained BBR, a form of monomorphic ventricular tachycardia, is a highly malignant arrhythmia usually seen in patients with structural heart disease. Three different types of BBR tachycardia are described. If distinguished from ventricular tachycardia of a myocardial origin, catheter ablation of the right bundle branch can be easily performed and effectively eliminates BBR. During follow-up, congestive heart failure is the most common cause of death in this population.     

IMMUNOHISTOCHEMICAL EXPRESSION OF INHIBIN/ACTIVIN SUBUNITS IN EPITHELIAL AND GRANULOSA CELL TUMOURS OF THE OVARY

Dimeric inhibin-A has been shown recently by a sensitive two-site ELISA assay to be a useful tumour marker in granulosa cell tumours of the ovary. It is also elevated in some patients with malignant epithelial ovarian tumours. To identify the precise subunits of inhibin expressed in ovarian tumours, three monoclonal antibodies, R1, E4, and INPRO (against the αC-, βA-, and pro-α-subunits, respectively), were evaluated by immunohistochemistry on a panel of six granulosa cell and nine epithelial tumours. All granulosa cell tumours stained positively with E4 and R1, suggesting expression of dimeric inhibin-A; in two patients where serum levels were measured pre-operatively, they were elevated. The tumours also reacted weakly with INPRO, suggesting the presence of precursor forms of the α-subunit. Eight malignant epithelial ovarian tumours expressed the βA-subunit only, as recognized by E4, while one tumour expressed both α- and βA-subunits, which correlated with an elevated serum inhibin-A level. Thus, while granulosa cell tumours express inhibin, the majority of epithelial tumours probably express activin, a result which needs to be confirmed by serum measurements. © 1997 by John Wiley & Sons, Ltd.