Consuelo Prieto

Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review.

Congenital extrahepatic portosystemic shunt (CEPS) is a rare condition in which the portomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt. Most often, the diagnosis is made primarily with Doppler ultrasonography. Computed tomographic angiography and magnetic resonance angiography are used for further classification of the shunt and assessment of accompanying anomalies. Conventional angiography is necessary when results of the other tests disagree or are inconclusive. CEPS is classified into two types according to the pattern of anastomoses between the portal vein and systemic vein. In type 1, intrahepatic portal venous supply is absent; in type 2, intrahepatic portal venous supply is preserved. Type 1 usually occurs in girls with associated malformations, such as situs ambiguous with polysplenia and congenital heart defects. Associated anomalies are less frequent in type 2, and symptoms usually develop later without a gender preference. Hepatic encephalopathy and liver dysfunction are possible complications of both types and usually develop during adulthood. Both types are also associated with regenerative hepatic nodules. The clinical setting and imaging appearance of these nodules can help one avoid misdiagnosis. Definitive treatment of CEPS is determined by the type of shunt. Liver transplantation is the only effective treatment for symptomatic type 1 CEPS; surgical closure or embolization of the shunt is the therapeutic approach for type 2.

Complete duplication of bladder and urethra in the coronal plane in a girl: case report and review of the literature.

Abstract An unusual case of complete duplication of the bladder and urethra in the coronal plane associated with left ectopic kidney, duplication of the clitoris and a chondrolipomatous malformation in the pelvic region in a girl is reported. The accessory bladder was located posterolateral to the normal bladder and a ureteric opening into the accessory bladder was found. The malformation was initially identified by US and cystography and confirmed by cystoscopy.

Heterotopic gastric mucosa involving the gallbladder and biliary tree

Abstract. A case of heterotopic gastric mucosa in the common bile duct, cystic duct and gallbladder is reported in a 3-year-old girl with abdominal pain and jaundice. Abdominal US and CT showed dilatation of the biliary tree and a well-defined mass in the common bile duct that narrowed its lumen. The gallbladder was contracted in both examinations. The common bile duct and the gallbladder were resected and a choledochojejunostomy was performed. Although gastric heterotopy has been described throughout the entire length of the gastrointestinal tract, it is a very uncommon finding in the gallbladder and extremely rare in the biliary tree. A firm diagnosis of gastric heterotopia is based on the presence of fundal mucosa replete with parietal and chief cells. To our knowledge, this is the fifth reported case of heterotopic gastric tissue within the common bile duct, and the first to describe the US and CT findings. A relevant literature review and brief outline of the histological and radiological features are included in the discussion.

Multiple congenital ectatic and fusiform arterial aneurysms associated with lower limb hypoplasia

Aneurysmal disease is uncommon in children, and its presence often leads to suspicion of genetic disorders (Loeys-Dietz syndrome, Marfan syndrome, Ehlers-Danlos syndrome, tuberous sclerosis), trauma, and infection. We describe the case of a newborn boy with generalized left lower limb hypoplasia associated with diffuse areas of arteriectasis combined with areas of stenosis and fusiform aneurysms of the iliac, femoral, and popliteal arteries. No additional vascular territories were affected. The patient was asymptomatic, and no therapeutic intervention has been considered. Numerous complementary imaging and laboratory examinations failed to establish a definitive diagnosis. This collection of findings has not been previously reported.

Utilidad del Doppler color y pulsado en la valoración de los shunts portosistémicos quirúrgicos en la edad pediátrica

Los shunts portosistemicos se realizan para paliar la hipertension portal sintomatica o para descomprimir la vascularizacion hepatica en pacientes con sindrome de Budd-Chiari. La mayoria de los shunts por-tosistemicos quirurgicos pueden estudiarse adecuadamente mediante ecografia complementada con Doppler color y pulsado si se compren-de la hemodinamica de los procedimientos quirurgicos. Este articulo muestra la utilidad y limitaciones de la ecografia duplex Doppler en la evaluacion de los shunts portosistemicos realizados a pacientes pedia-tricos. El Doppler pulsado proporciona informacion sobre la naturaleza y direccion del flujo sanguineo y el Doppler color es capaz de mostrar directamente el shunt y, en la mayoria de los casos, permite localizar las anastomosis. Los tipos de shunts que se muestran incluyen espleno-renal proximal y distal, portocava y mesocava. Se ilustran los tipos de conexiones vasculares y se muestra la direccion posquirurgica espera-ble del flujo en los vasos sanguineos implicados. Se discute la tecnica ecografica y los criterios para determinar la permeabilidad vascular. Asi mismo se enfatizan las ventajas, limitaciones y dificultades diag-nosticas de las distintas modalidades del Doppler.

Ultrasound Imaging and Colour Doppler Studies in Familial Nephropathy Associated with Hyperuricemia (FNAH)

FNAH is an inherited disease of unknown pathogenesis. By means of clearance techniques we have demonstrated that diminished renal plasma flow and increased renal vascular resistance may be primary pathogenic events in FNAH (1). Ultrasonography and two-dimension echo-color-doppler (2D ECD) are two methods that have been applied to the study of renal morphology and the intrarenal vascular bed, respectively. Sonography is routinely used for the study of kidney size (2). In addition, sonography allows the assessment of the parenchymal thickness and the cortico-medular echogenicity (3). Twodimensional ultrasound imaging and blood flow detection with color doppler are accepted methods to quantify intrarenal vascular resistance (4). In this study we have examined renal morphology and the intrarenal vascular bed in patients with FNAH.

Hidronefrosis leve en neonatos y lactantes: ¿puede predecirse la presencia de reflujo vesicoureteral?

Objetivo El proposito de este estudio fue determinar el valor de la hidronefrosis leve o moderada, detectada mediante ecografia, en recien nacidos y lactantes como indicador de reflujo vesicoureteral (RVU) y evaluar la necesidad de realizar cistouretrografia miccional (CUMS) en estos pacientes. Material y metodos Se revisaron las CUMS de 237 pacientes (174 ninos y 63 ninas, con edades comprendidas entre 0 y 18 meses) con hi-dronefrosis leve detectada por ecografia, para valorar la presencia de RVU primario. Los pacientes con reflujo secundario o los que presen-taban anomalias que imposibilitaban la graduacion exacta del reflujo se excluyeron; sin embargo, cuando se observo reflujo en un rinon normal, contralateral al rinon con hidronefrosis, se incluyo en el estudio. Para el analisis estadistico de los datos, cada rinon se considero por se-parado, sumando un total de 474 rinones. Se diagnostico hidronefrosis leve cuando se observo un diametro anteroposterior de la pelvis renal de 0,5-1,5 cm. Para la graduacion del reflujo en la CUMS se utilizo el sistema internacional. Resultados De las 474 unidades renales evaluadas, 306 mostraron hidronefrosis leve en la ecografia. Solo 98 de estas (32%) presentaron reflujo (14 grado I, 50 grado II, 32 grado III y 2 grado IV). La ecogra-fia fue normal (no mostro hidronefrosis) en 52 unidades renales con reflujo (31%), contralaterales al rinon hidronefrotico, a pesar de que 38 de estas unidades tenian reflujo grado II o superior. La ecografia mos-tro hidronefrosis leve en 208 rinones que no tenian reflujo en la CUMS (68%). Conclusiones No hubo diferencias significativas en la incidencia de RVU entre los rinones con o sin hidronefrosis. La hidronefrosis leve se correlaciona escasamente con la presencia y el grado de reflujo en neonatos y lactantes y, por tanto, no debe considerarse una indicacion para realizar CUMS.