Síndrome de Cimitarra: revisión a partir de un caso clínico / Scimitar syndrome: a review based on a clinical case

Resumen Así como otros síndromes congénitos considerados raros por su baja incidencia, el diagnóstico del síndrome de la Cimitarra se basa en la presencia de un drenaje venoso anómalo de las venas pulmonares derechas que en muchas ocasiones se asocia con la presencia de otras malformaciones pulmonares y cardíacas. La gran mayoría pasan desapercibido puesto que los síntomas pueden manifestarse hasta en edades adultas y puede pasarse por alto. A propósito de un caso clínico, presentamos una revisión de este tema, que es importante tomar en cuenta para darles a estos pacientes un seguimiento y tratamiento adecuados.

Abstract As other congenital syndromes are considered rare based on their low incidence, the diagnosis of scimitar síndrome is based on the presence of an abnormal right venous pulmonary drainage, accompanied in many cases with other pulmonary and cardiac malformations. The vast majority of them has been undiagnosed, because the symptoms present later in adulthood. With regard to a clinical case, we present a review of this topic, which is important to take into account to give this patients a follow-up and adequate treatment.

Angiotomografia com múltiplos detectores no diagnóstico de drenagem venosa pulmonar anômala: experiência inicial / Multidetector-row computed tomography angiography for the diagnosis of anomalous pulmonary venous drainage: an initial experiment

OBJETIVO: Demonstrar que a angiotomografia computadorizada com múltiplos detectores é um método efetivo e não invasivo para o diagnóstico de drenagem venosa pulmonar anômala. MATERIAIS E MÉTODOS: Estudo retrospectivo de 2.905 angiotomografias computadorizadas com múltiplos detectores cardíacas realizadas em nosso serviço no período de julho de 2003 a novembro de 2007. Destas, 393 foram destinadas para avaliar cardiopatias congênitas e as 2.512 restantes, para analisar as artérias coronárias. RESULTADOS: Foram encontrados 21 casos de drenagem venosa pulmonar anômala, sendo 7 (33,3 por cento) do tipo total e 14 (66,7 por cento) do tipo parcial. Das drenagens venosas pulmonares anômalas totais, três foram do tipo supracardíaco, três do tipo infracardíaco e uma do tipo cardíaco. CONCLUSÃO: A angiotomografia computadorizada com múltiplos detectores tem demonstrado fundamental importância no diagnóstico destas anomalias, principalmente por se tratar de método não invasivo capaz de analisar estruturas intra e extracardíacas e por permitir um estudo completo da anatomia torácica, contribuindo sobremaneira na conduta cirúrgica e, consequentemente, no prognóstico destes pacientes, especialmente por diagnosticar malformações não suspeitadas clinicamente.

OBJECTIVE: We aimed to determine if multidetector-row computed tomography angiography is an effective and non-invasive method for diagnosing anomalous pulmonary venous drainage. MATERIALS AND METHODS: We performed a retrospective review of 2,905 cases from July 2003 to November 2007 in which cardiac multidetector-row computed tomography angiography was used. Of the cases, 393 evaluated congenital cardiopathy, with the others (2,512) evaluating the coronary arteries. RESULTS: Anomalous pulmonary venous drainage was found in 21 cases, with 7 (33.3 percent) from total anomalous pulmonary venous drainage (3 supracardiac, 3 infracardiac and 1 cardiac), and 14 (66.7 percent) from partial anomalous pulmonary venous drainage. CONCLUSION: Multidetector-row computed tomography angiography can be useful for diagnosing the described anomalies because of its non-invasiveness, and its ability to evaluate intra- and extra-cardiac structures. The technique allows a thorough study of the thoracic anatomy and contributes to surgical conduct, consequently improving patient prognosis, in particular by allowing the diagnosis of clinically unsuspected malformations.

A Case of an Anomalous Opening of the Common Bile Duct and Pancreatic Duct into the Duodenal Bulb, and the Patient Presented with Acute Cholangitis and Recurrent Duodenal Ulcer / 대한소화기내시경학회지

There are various congenital anomalies of the biliary system, but an ectopic opening of the common bile duct into the duodenal bulb is an extremely rare finding. Despite the recent improvement in the diagnosis and management of pancreatobiliary lesions, the general lack of knowledge and understanding about these variations is undoubtedly responsible for many of the problems that occur during the medical and surgical management of these lesions. We report here on a case of a 65-year-old man who had an ectopic opening of the common bile duct into the duodenal bulb, and this cause acute cholangitis with bile duct sludge, and also recurrent duodenal ulcer. In this case, we used abdomen CT, MRCP, duodenoscopy, EUS and ERCP for making the diagnosis. The cholangitis and duodenal ulcer was improved with medical therapy, and the patient was discharged without any surgical procedure. We report here on this unusual case and we include a review of the relevant medical literature.

A Case of Anomalous Drainage of the Common Bile Duct into the Duodenal Bulb with Pancreatic Duct Anomaly / 대한소화기내시경학회지

The common bile duct normally enters the posteromedial aspect of the second part of the duodenum. However, anomalous drainage of the common bile duct into the stomach, pyloric channel, duodenal bulb and fourth portion of the duodenum has been reported in the literature. An anomalous pancreatic duct, with ectopic drainage of the common bile duct, is particularly rare. Herein, we report the case of a 69-year-old woman who showed pancreatic ductal variation and anomalous drainage of the common bile duct into the duodenal bulb. The patient required a cholecystectomy and choledochoenteric anastomosis to relieve the obstructive jaundice and abdominal pain.

A Case of Anomalous Drainage of the Common Bile Duct into the Duodenal Bulb Presenting with Obstructive Jaundice / 대한소화기내시경학회지

The common bile duct classically enters the posteromedial aspect of the second part of the duodenum through an oblique, 1 to 2 cm long intramural tunnel. Some case reports of the common bile duct emptying into other sites including the fourth part of the duodenum, the pyloric canal, stomach and duodenal bulb have appeared in the literature. We report a case of a 40-year-old man who showed anomalous drainage of the common bile duct into the duodenal bulb presenting with obstructive jaundice and duodenal ulcer. This patient required choledochoenteric anastomosis to relieve obstructive jaundice and abdominal pain. We report the case with a review of other cases in Korean literature.

Anomalous Drainage of the Common Bile Duct and Pancreatic Duct into the Duodenal Bulb / 대한소화기내시경학회지

While anomalies of the pancreaticobiliary system are not uncommon, drainage of the common bile duct into the bulb of the duodenum has rarely been reported. The awareness of ectopic drainage of the CBD is stressed to prevent surgical damage and improve medical management in this area. We reported one case of anomalous drainage of the CBD and pancreatic duct associated with nonvisible gallbladder or agenesis of gallbladder and cystic duct diagnosed by ERCP, ultrasonography and upper abdominal CT.

Anomalous Drainage of the Common Bile Duct and Pancreatic Duct into the Duodenal Bulb / 대한소화기내시경학회지

While anomalies of the pancreaticobiliary system are not uncommon, drainage of the common bile duct into the bulb of the duodenum has rarely been reported. The awareness of ectopic drainage of the CBD is stressed to prevent surgical damage and improve medical management in this area. We reported one case of anomalous drainage of the CBD and pancreatic duct associated with nonvisible gallbladder or agenesis of gallbladder and cystic duct diagnosed by ERCP, ultrasonography and upper abdominal CT.