Enfermedades quísticas del pulmón: hallazgos en la tomografía computarizada de alta resolución / Cystic diseases of the lung: high-resolution computed tomography findings

No disponible

[Contrast-enhanced sonourethrography versus conventional miction cystourethrography in the assessment of the urethra: preliminary study]. / Uretrocistosonografía contrastada versus cistouretrografía miccional convencional para el estudio de la uretra: estudio preliminar.

BACKGROUND AND AIMS: Voiding cystourethrography (VCUG) is the first choice imaging modality for assessing the urethra, but this technique exposes patients to ionizing radiation. Cystosonography with echocontrast (CS) has proved to be a reliable technique to detect and grade vesicoureteral reflux (VUR) without exposing patients to ionizing radiation, but its capacity to adequately study the urethra has yet to be demonstrate in large series of patients. The aim of this study is to demonstrate the reliability of contrast-enhanced CS for assessing the urethra by comparing the results with those of the VCUG. MATERIAL AND METHODS: 108 patients were studied with ultrasound (US) using a galactose-based contrast agent. This exam was always followed by VCUG. Basal and voiding urethral US studies were performed with. Patients were studied in supine decubitus position. Girls were examined by longitudinal translabial approach, with the probe (a 7.5 MHz liner array transducer) positioned longitudinally at the introitus, to evaluate the bladder neck and urethra. In boys the transducer was initially placed longitudinally in the escrotum at ventral root of the penis to assess the bladder neck and proximal bulbar urethra, and then displaced distally toward the penile urethra. On basal study the echogenic urethral mucosa and the collapsed sonolucent urethral lumen were indentified and measured when distended. The patients were asked to void with the probe in place. During voiding attention was focused on elasticity and distention of urethral walls, as well as in the caliber of the entire urethra. Patients unable to void during either CS or VCUG were excluded. RESULTS: The bladder neck and the entire urethra were well demonstrate with CS. All females and 43 males showed a normal urethra both in CS and VCUG. Four patients were dignoses of posterior urethral valves (PUV) with CS and confirmed at VCUG, one patient had anterior urethral valves and 5 showed urethral stenosis at both techniques. Three patients with a vesicosphincteric dysinergia, 14 with resected PUV and one with a resected syringocele were adequately evaluated. Twelve girls showed vaginal reflux. The information provided by CS was equivalent to the VCUG in all patients but two with a syringocele (only seen on VCUG). CONCLUSIONS: CS is a reliable imaging modality sufficiently sensitive and specific to study the urethra, adding dynamic information to VCUG and can be used as a complement to VCUG.

Imagen radiológica de las anomalías congénitas de las costillas. Parte I. Anomalías de número y fusión.Costillas finas / Radiologic imaging of congenital anomalies of the ribs: number and fusion anomalies thin ribs

Las anomalías congénitas del tórax son una causa significativa de morbilidad en neonatos, lactantes, niños e incluso en la edad adulta. La evaluación de los pacientes afectados requiere frecuentemente múltiples modalidades de imagen para diagnosticarlas y, en su caso, planear la corrección quirúrgica. Este artículo analiza e ilustra aspectos prácticos de ciertas anomalías congénitas comunes e infrecuentes que afectan a las costillas, con énfasis particular en las manifestaciones radiológicas. Se evalúa, asimismo, la utilidad de las diferentes modalidades de imagen en el diagnóstico y manejo de estas alteraciones. Las alteraciones descritas incluyen anomalías de número y fusión (ausencia e hipoplasia de costillas y costillas supernumerarias) y costillas finas (neurofibromatosis, síndrome de la trisomía 13, síndrome de la trisomía 18 y osteogénesis imperfecta congénita). Las bases embriológicas y patológicas de los distintos hallazgos radiológicos se discuten en los casos apropiados. Se incluye también el diagnóstico diferencial de algunos de estos procesos (AU)

Resultados de la derivación mesentérico-cava con injerto autólogo de yugular en la hipertensión portal presinusoidal en el niño / Mesenteric-cava shunt's results with autologous yugular vein graft in children with presinusoidal portal hipertension

En el niño la hipertensión portal de origen presinusoidal (HPP) cursa sin daño funcional hepático, y con el tiempo tiende a compensarse mediante la creación de shunts espontáneos portosistémicos; no obstante, algunos sufren episodios de hemorragia gastrointestinal (HGI) que por su severidad o frecuencia, obliga a plantear la posibilidad de tratamiento quirúrgico. Objetivo. Valorar los resultados de la derivación mesocava (shunt meso-cavo, SMC) con injerto autólogo de vena yugular en niños con HPP.Material y métodos. De una serie de 32 niños con HPP tratados en nuestro hospital en los últimos 7 años, 10 sufrieron episodios de HGI que obligó a plantear una derivación quirúrgica. El tipo de shunt fue esplenorrenal distal en tres casos y mesocavo en siete; estos últimos constituyen el material de este estudio. La HPP fue ocasionada por transformación cavemomatosa de la porta (TCP) en seis y fibrosis hepática congénita (FHC) en uno. Previamente a la cirugía, la media de episodios de HGI fue de 9 (rango: 2-15); todos precisaron transfusión de hemoderivados y esclerosis de varices, y sonda con balón en dos; cinco fueron tratados con somatostatina y propranolol. La exploración con Doppler pulsado antes de la cirugía puso de manifiesto una intensa circulación colateral hepatófuga en todos los casos. Resultados. El flujo a través del shunt fue adecuado en todos excepto uno, que precisó de dilatación percutánea con balón. A excepción de éste, ninguno de los otros 6 niños ha vuelto a sufrir episodios de HGI. Los signos de hiperesplenismo regresaron o mejoraron en los siete casos, así como la circulación colateral en el seguimiento con Doppler pulsado, observándose velocidades de flujo adecuadas a través del shunt en todos los niños menos en uno. En los cuatro casos en los que se determinó la presión en el territorio esplácnico; ésta descendió alrededor del 50 por ciento de la original tras la apertura del shunt. En ninguno se ha observado encefalopatía, y sólo el enfermo con fibrosis hepática congénita muestra signos bioquímicos leves de disfunción hepática. El tiempo medio de evolución post-shunt es de 32 meses (rango: 8 meses-6 años).Conclusiones. El SMC evita la HGI en los casos de HPP que no responden al tratamiento conservador; su eficacia está en relación con una adecuada permeabilidad a través del injerto, y al menos en los casos de cavemomatosis portal (los más frecuentes de HPP en el niño), no produce disfunción hepática. El Doppler pulsado proporciona una información muy precisa de la situación postcirugía, siendo un método excelente de seguimiento de estos enfermos. (AU)

[Mesenteric-cava shunt's results with autologous jugular vein graft in children with pre-sinusoidal portal hypertension]. / Resultados de la derivación mesentérico-cava con injerto autólogo de yugular en la hipertensión portal presinusoidal en el niño.

UNLABELLED: Presinusoidal portal hypertension (PPH) in children evaluates without functional hepatic damage, and with the time, trends to compensate through the creation of spontaneous portosystemic shunts. Nevertheless, some patients suffer episodes of gastrointestinal bleeding (GIB) that because of its frequency or severity, force to propose the change of surgical treatment. AIM: To evaluate the results of the mesocaval shunt (MCS) with autologous jugular vein in children with PPH. MATERIAL AND METHODS: Among the 32 children with PPH treated in our Hospital in the last 7 years, 10 had episodes of GIB that forced to perform a surgical shunt. The types of shunt were distal splenorenal in 3 patients and mesocaval in 7. These 7 cases are the material of this study. The origin of the PPH was a cavernomatosis transformation of the portal vein in 6 cases and a congenital hepatic fibrosis in 1. Before the surgery the average number of episodes of GIB was 9 (range 2-15); all the patients needed transfusion of blood products and variceal sclerosis. In 2 cases a tamponade with the Sengtaken balloon was required and 5 patients were treated with somatostatin and propranolol. The Doppler ultrasounds revealed and intense hepatofugal collateral circulation in all the cases. RESULTS: The initial flow through the shunt was adequate in all the patients except one who required a percutaneous balloon dilatation. Only this patient has suffered an episode of GIB. The hyperesplenism signs disappeared or improved in all the seven cases and the collateral circulation was significantly reduced. The pressure in the splenic territory decreased around 50% in the 4 patients that was measured. There were no cases of encephalopasty and only one child with congenital hepatic fibrosis shows signs of mild hepatic disfunction. The medium follow up post-shunt is 32 months (range 8 m-6 years). CONCLUSIONS: The MCS prevents the GIB in the PPH not responsive to the conservative treatment; its effectiveness is related with an adequate permeability though the graft and at least in the cases with portal cavernomatosis (the most frequent in children) doesn't produce hepatic dysfunction. Doppler ultrasounds give a very precise information about the post-surgical situation and are an excellent method of follow up.

Vesicoureteral reflux: diagnosis and grading with echo-enhanced cystosonography versus voiding cystourethrography.

PURPOSE: To evaluate the usefulness of echo-enhanced cystosonography compared with voiding cystourethrography (VCUG) for detecting and grading vesicoureteral reflux (VUR). MATERIALS AND METHODS: Two hundred sixteen pediatric patients underwent cystosonography enhanced with SH U 508A, a galactose-based echo-enhancing agent. Sonograms of the kidneys and bladder were obtained before filling, during bladder filling, and during voiding. This examination was followed immediately with VCUG. Each kidney or portion of a kidney with its own complete collecting system was considered separately, for a total of 440 kidney units. RESULTS: VUR was detected in 123 kidney units at cystosonography and in 104 at VCUG. In 401 kidney units, there was concordance between results at cystosonography and at VCUG regarding the presence or absence of VUR. Ninety-four kidney units showed VUR with both methods. Seventy-seven of the 94 refluxing units were depicted with the same grade of VUR with both modalities, and in 17 the VUR grade was greater at cystosonography than at VCUG. Twenty-nine of the 94 units showed VUR at only cystosonography, and 10 units at only VCUG. The McNemar test showed that cystosonography depicted a significantly (P = .003) higher number of units with VUR. By patient, VUR was depicted with both studies in 67 and with only one study in 25. VUR was seen at only cystosonography in 16 patients and at only VCUG in nine. The McNemar test for patients showed no significant difference between the two tests in detection of VUR. CONCLUSION: Cystosonography with SH U 508A appears comparable to VCUG in the depiction of VUR.

Hipertensión portal prehepática como complicación tardía del trasplante hepático pediátrico / Prehepatic portal hypertension after pediatric liver transplantion

Algunos niños sometidos a trasplante hepático (TH) desarrollan a largo plazo hipertensión portal prehepática (HPP) y plantean problemas hasta ahora poco conocidos; muchas de las lecciones aprendidas con el manejo de estos enfermad pueden tener además aplicación fuera del trasplante. Objetivos: 1. Analizar la incidencia y factores de riesgo de HPP tras TH. 2, Valorar los resultados con los diferentes tratamientos utilizados. Material y métodos. Estudio retrospectivo sobre 164 niños que sobrevivieron más de un año tras TH. Análisis univariante de posibles factores de riesgo asociados a multivariante (regresión logística) para aquéllos que en el análisis univariante tuvieron significación. Se analizan otros factores asociados y las indicaciones y resultados de dos tipos de tratamiento: dilatación neumática percutánea y shunt quirúrgico (esplenorrenal y meso-portal o shunt de Rex). Resultados. Nueve niños desarrollaron Hpp sintomática (hemorrágica en 8, ascitis en 1), asociada en 2 a trastorno linfoproliferativo postrasplante y a estenosis biliar anastomótica en uno. La edad al primer trasplante (menores de un año), peso (menores de 10 Kg) y necesidad de retrasplante fueron en el análisis univariante las variables asociadas con riesgo incrementado de HPP. Fueron casi significativas el diagnóstico (atresia biliar), y el grado de urgencia del TH. En el análisis multivariante, la necesidad de retrasplantes es la única variable independiente que incrementa el riesgo (riesgo relativo; 4,5 intervalo de confianza 95 por ciento: 1,29-18,87). Al diagnóstico, tres caos mostraron estenosis portal, y ausencia de permeabilidad con trasformación cavernomatosa de la porta en cinco. La hPP fue ocasionada en un caso por desconexión de vena esplénica ( que de momento no requiere tratamiento); los tres casos de estenosis portal fueron dilatados con éxito por abordaje percutáneo, y los dos de los 5 casos de trombosis portal han sido derivados quirúrgicamente; uno mediante shunt esplenorrenal y otro mediante shunt de Rex (primer caso realizado en España); los restantes tres casos están estables y pendientes de solución quirúrgica. La función hepática es normal en los 9 casos. Conclusiones. La HPP puede complicar a largo plazo en pronóstico del TH pediátrico. Diagnosticada en fase de estenosis portal, la dilatación neumática es el tratamiento de elección, debiendo ser tratados los restantes quirúrgicamente. En estos casos, el shunt más fisiológico y la mejor opción es el de Rex (AU)

Anomalías congénitas de la uretra: diagnóstico radiológico / Congenital anomalies of the uretra: radiological diagnosis

Este artículo revisa una serie de anomalías congénitas frecuentes e infrecuentes que afectan a la uretra, e ilustra la utilidad de la urografía, cistografía miccional y ecografía en el diagnóstico y manejo de estas alteraciones. Las anomalías de la uretra incluyen válvulas de uretra posterior, duplicación uretral, divertículos uretrales, megalouretra y fístula uretral. Se incluye, así mismo, la malformación de la cloaca y el seno urogenital. Las bases embriológicas y patológicas de las diferentes patologías se discuten en cada apartado (AU)

Anomalías congénitas del uréter y de la vejiga: diagnóstico radiológico / Congenital anomalies of the ureter and the bladder: radiological diagnosis

La evaluación de los pacientes con anomalías congénitas del tracto urinario inferior requiere frecuentemente múltiples modalidades de imagen para el diagnóstico y el planteamiento de la corrección quirúrgica. Este artículo revisa una serie de anomalías congénitas frecuentes e infrecuentes que afectan al uréter dista¡ y a la vejiga, e ilustra la eficacia de las placas simples, urografía, cistografía miccional y ecografía en el diagnóstico y manejo de estas alteraciones. Las anomalías del uréter distal incluyen ureteroceles simples, ureteroceles ectópicos y uréteres ectápicos en múltiples localizaciones. En las anomalías vesicales incluimos divertículos, duplicación vesical tanto en el plano frontal como sagital y anomalías del uraco tales como persistencia del uraco, quiste uracal y divertículo uracal. Las bases embriológicas y patológicas de los diferentes hallazgos radiográficos se discuten en los casos apropiados (AU)

[Pre-hepatic portal hypertension as a late complication of liver transplantation in children]. / Hipertensión portal prehepática como complicación tardía del trasplante hepático pediátrico.

UNLABELLED: In the long-term after liver transplantation (LT), some children develop prehepatic portal hypertension (PPH) and raise problems not very well known yet; many of the lessons learned with the management of these patients may be useful outside the LT. AIM: 1. To analyze the incidence and risk factors of PPH after LT. 2. To evaluate the results with the different treatments used. METHODS: Retrospective study over 164 children surviving more than 1 year after LT. Univariant analysis of possible risk factors associated and multivariant (logistic regression), for those that had significance in the univariant analysis. Other factors associated are analyzed as well as the indications and results of two types of treatment: percutaneous pneumatic dilatation and surgical shunt (splenorenal and Rex shunt). RESULTS: 9 children developed symptomatic PPH (hemorrhage in 8, ascites in 1), associated to lymphoproliferative post-LT disease in 2, and to anastomotic biliary stricture in 1. The age at first LT (children under 1 year old), weight (below 10 kg), and need of retransplantation (reLT) were in the univariant analysis the associated variables with increased risk of PPH. The diagnosis (biliary atresia) and the emergency status of the LT were almost significative. In the multivariant analysis, the need of reLT is the only independent variable that increases the risk (relative risk: 4.5, confidence interval 95%: 1.29-18.87). At diagnosis 3 cases showed portal estenosis, and 5 showed absence of permeability with cavernomatous transformation. The PPH was caused in one case because of the esplenic vein disconnection (treatment not required at the moment); the three cases of portal estenosis were dilated percutaneously with success, and 2 of the 5 cases with portal thrombosis have been surgically shunted: one by an splenorenal shunt and the other by a Rex shunt (first case done in Spain); the other 3 cases are stable waiting for a surgical solution. The hepatic function is normal in the 9 cases. CONCLUSIONS: The PPH can complicate the prognostic of the pediatric LT in the long term. The treatment depends on the permeability of the portal trunk. Whenever possible, percutaneous dilatation should be attempted; should surgery be required, the Rex shunt is the best option.

Diagnóstico del reflujo vesicoureteral mediante ecocistografía. Estudio preliminar / Diagnosis of vesicoureteral reflux with cystosonography. Preliminary study

La primera modalidad de imagen para diagnosticar el reflujo vesicoureteral es la cistouretrografía miccional fluoroscópica (CUMS) cuya principal limitación es que expone al paciente a las radiaciones ionizantes.Propósito: El propósito de este estudio fue analizar la utilidad de la ecografía realzada con un medio de contraste potenciador de la señal ecográfica (ecocistografía) para diagnosticar el reflujo vesicoureteral (RVU), comparando los resultados de esta nueva modalidad de imagen con los de la cistouretrografía miccional fluoroscópica convencional.Material y métodos: 72 pacientes pediátricos con sospecha de reflujo vesicoureteral fueron sometidos a una cistografía realizada con un agente ecopotenciador bajo control ecográfico (ecocistografía), seguida de una cistouretrografía miccional fluoroscópica convencional durante una misma sesión diagnóstica. El medio de contraste utilizado para la ecocistografía es una suspensión de galactosa y ácido palmitico (Levograf) diluida en suero salino. Treinta y tres pacientes eran varones y 39 niñas, con edades comprendidas entre 3 días y 17 años. Para el análisis estadístico de los resultados el riñón izquierdo y el derecho se contabilizaron separadamente, obteniéndose un total de 146 unidades renales.Resultados: En 134 (91.7 por ciento) de las 146 unidades renales hubo concordancia entre la ecocistografía y la CUMS para diagnosticar o excluir la existencia de RVU. 102 unidades renales (69.8 por ciento) fueron normales y 32 (21.9 por ciento) mostraron RVU en ambas pruebas. En 5 unidades (7 por ciento) el reflujo diagnosticado en la ecocistografía no se observó en la CUMS, y en 2 unidades (4.2 por ciento) se observó RVU únicamente en la CUMS.Conclusión: La ecocistografía con contraste es una modalidad de imagen fiable y suficientemente sensible y específica para detectar el reflujo vesicoureteral sin exponer al paciente a las radiaciones ionizantes, ofreciendo información simultánea del tamaño renal y la cicatrización parenquimatosa (AU)

[Pediatric liver transplantation: now the patients are different and the problems are different as well]. / Trasplante hepático pediátrico: los enfermos son ahora diferentes y los problemas también son distintos.

AIM: 1. To show how in a program of pediatric liver transplantation (PLT) with 12 years of experience, the continuous use of technical innovations has allowed to improve the results and to treat the most complex cases. 2. To point out that when the immediate results of the transplant improve and the evolution time get longer, the late complications become the main source of concern. MATERIAL AND METHODS: The graft survival and the incidence of early surgical complications was compared between the first 100 PLT and the last 100 PLT (Total 235 PLT). The patients survival was also compared between the first and the last 5 years. In every case it was analyzed the age and weight of the children at the time of the transplant and the type of graft (full or reduced liver). The incidence of late complications in the global series was also analyzed, especially the posttransplant lymphoproliferative disease (PTLD) and the late biliary and vascular complications. RESULTS: There are significant differences between the first 100 PLT and the last 100 PLT in relation with the age of the patients (6.8 +/- 0.6 vs 4.2 +/- 0.5 years), the number of children younger than 1 year (4 vs 28), weight (22.4 +/- 1.2 vs 16.9 +/- 1.7 kg), number of PLT in children under 10 kg (7 vs 43), use of reduction techniques (7 vs 49), rate of hepatic artery thrombosis (12% vs 3%), and rate of early biliary complications (13% vs 4%). The 5 years survival of the graft was 51% in the first 100 PLT vs 65% in the last 100 PLT, and the 5 years patients survival was 70.1% in the first 5 years of the program vs 79.6% in the last 5 years. Among the late complications in the global series, 13 cases of PTLD (2 deaths) have special relevance, 6 cases of late anastomotic biliary stricture, 4 cases of portal stenosis, 1 arterial thrombosis, 1 death due to a fulminant sepsis and another death because of a colitis with multiple hepatic abscess. CONCLUSIONS: 1. The result of PLT improve, despite of the fact that in the last years the difficulty of the surgical procedures have increased (the patients are younger, with less weight and the reduction techniques are used more frequently). 2. The late complications have a significant influence in the prognosis, being that influence not completely established yet.

Congenital anomalies of the small intestine, colon, and rectum.

Congenital anomalies of the gastrointestinal tract are a significant cause of morbidity in children and, less frequently, in adults. These abnormalities include developmental obstructive defects of the small intestine, anomalies of the colon, anomalies of rotation and fixation, anorectal anomalies, and intestinal duplications. Neonates with complete high intestinal obstruction do not usually require further radiologic evaluation following radiography, whereas those with complete low obstruction should undergo a contrast material enema examination. An upper gastrointestinal series must be performed in all patients with incomplete intestinal obstruction because management is different in each case. In low intestinal obstruction, ultrasonography (US) may help differentiate between small bowel obstruction and colonic obstruction. In addition, US can help correctly identify meconium ileus and meconium peritonitis and is useful in the diagnosis of enteric duplication cysts. In malrotation and anorectal anomalies, computed tomography (CT) and magnetic resonance (MR) imaging can provide superb anatomic detail and added diagnostic specificity. Intestinal duplications manifest as an abdominal mass at radiography, contrast enema examination, or US. At CT, most duplications manifest as smoothly rounded, fluid-filled cysts or tubular structures with thin, slightly enhancing walls. At MR imaging, the intracystic fluid has heterogeneous signal intensity on T1-weighted images and homogeneous high signal intensity on T2-weighted images. Familiarity with these gastrointestinal abnormalities is essential for correct diagnosis and appropriate management.

Congenital anomalies of the upper gastrointestinal tract.

A wide spectrum of congenital anomalies may affect the upper gastrointestinal tract, including anomalies of the esophagus (e.g., atresia, fistulas, webs, duplications, vascular rings), stomach (e.g., congenital gastric outlet obstruction, duplications), and duodenum (e.g., atresia, annular pancreas, duplications, malrotation). The evaluation of affected patients can require multiple imaging modalities for diagnosis and surgical planning. Radiography is often diagnostic and specific and can usually provide important clues to help determine the optimal diagnostic procedure. Neonates with complete gastric or upper intestinal obstruction do not usually require further radiologic evaluation after radiography: Barium studies are usually contraindicated, and complementary procedures (e.g., ultrasound [US], computed tomography [CT]) are not usually helpful and may even delay surgery, resulting in death. Nevertheless, US has become important in the evaluation of the pediatric gastrointestinal tract and is being used in an increasing number of applications. CT and magnetic resonance imaging are unsuitable for general screening but provide superb anatomic detail and added diagnostic specificity. They are especially useful in demonstrating esophageal duplications and vascular rings as well as associated abnormalities. However, the decision to perform a given imaging examination should be considered carefully to avoid inconvenience or unnecessary radiation exposure to the patient or delays in surgical correction. Quality control programs should be in place to ensure safe, effective radiologic practice through use of up-to-date equipment and good imaging technique.

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

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.

Late biliary complications in pediatric liver transplantation.

PURPOSE: The aim of this study was to review the biliary complications occurring in late follow-up after liver transplantation in children. METHODS: The medical records of 135 children who received orthotopic liver transplantations (OLT) and had graft survival of more than 1 year were reviewed. Technical variants using a reduced-size graft were applied in 32 (23.7%). For biliary reconstruction, 15 patients had choledochocholedochostomy and 120 a Roux-en-Y loop. Biliary reoperation in the early post-OLT period was needed in 24 patients (17.7%). Routine checking of liver function and duplex Doppler ultrasonography (DDS) were performed during the follow-up period, which averaged 58 months. Late biliary complication was defined as that occurring after the first hospital discharge. RESULTS: Late biliary complications occurred in 18 children (13.3%); 16 showed symptoms or analytical disturbances in liver function tests. The Diagnoses included uncomplicated cholangitis (n = 6), anastomotic biliary stricture (n = 7), ischaemic damage of the biliary tree (n = 3) including one late (28 months) hepatic artery thrombosis leading to an intrahepatic biloma. and bile leak after T-tube removal (n = 2). The six children with uncomplicated cholangitis had no repeat episodes in follow-up despite persistent aerobilia. Six patients affected by anastomotic strictures were treated successfully with percutaneous dilatation and, if present, stone removal. Persisting dysfunction and cholangitis occurred in one case affected by ischaemic biliary disease. Biliary leaks after T tube removal settled spontaneously. Risk factors for late biliary complications were determined. There was no relation to the cold ischaemia time, type of graft or biliary reconstruction, or previous early post-OLT biliary reoperation. Aerobilia (affecting 21.5% of OLT patients) was related to cholangitis (P = .001). CONCLUSIONS: Anastomotic strictures, reflux of intestinal contents via the Roux-en-Y loop, and residual ischaemic damage led to late biliary complications in 12% of paediatric OLT patients. Evidence of biliary dilatation on DDS may be delayed in anastomotic strictures; in these cases the results of percutaneous treatment were excellent. Children with aerobilia have and increased risk of cholangitis.

Long-term follow-up of patients with biliary atresia successfully treated with hepatic portoenterostomy. The importance of sequential treatment.

The outcome of 18 biliary atresia (BA) patients (5 male, 13 female; age range 10.7-22.5 years; mean 15.4+/-0.7 years) treated with hepatic portoenterostomy (HPE) and jaundice-free for more than 10 years without liver transplantation (LT) is analyzed retrospectively. Eight of these patients subsequently required LT (age at LT 12. 8+/-0.5 years, range 10.5-15.2 years); 3 children (aged 11.6, 13.2 and 14.1 years, respectively) had episodes of gastrointestinal variceal bleeding associated with other signs of severe disease and are now candidates for LT; and among the 7 asymptomatic patients (age range 11.2-22.5 years; mean 15.9+/-2.1 years), 5 had sonographic and biochemical signs of moderate portal hypertension (PH). In order to analyze whether the age at transplantation influences the survival of children transplanted for BA, we also reviewed the outcome of 71 BA patients transplanted at our hospital between 1986 and 1996. All the children older than 10 years at the time of LT were alive; only patients younger than 10 years died following LT (n = 15). We conclude that the natural outcome of extrahepatic BA is toward PH, fibrosis, and cirrhosis, even in those cases successfully treated with HPE. In our experience, the results of sequential treatment with HPE and LT were excellent.

Syndrome of Alagille: radiological and sonographic findings. A review of 37 cases.

The objective of this study was to evaluate the association of intrahepatic cholestasis, pulmonary stenosis, and hyperlipemia with osseous and renal abnormalities in Alagille's syndrome. A total of 37 patients (25 males and 12 females) with ages ranging between 8 months and 27 years were included. Clinical records as well as radiological and ultrasonographic studies were reviewed. Chronic cholestasis was observed in all patients. Thirteen had favorable clinical outcome and normal hepatic sonograms. Clinical and sonographic evidence of portal hypertension was found in 13 patients and of cirrhosis in 8 patients. The remaining 3 patients required hepatic transplantation due to severe cholestasis. Hyperlipemia correlated with hepatic malfunction. Pulmonary stenosis and renal hypoplasia were the most frequently associated abnormalities. All patients showed a peculiar facies. Abnormal "butterfly" vertebrae were present in 18 patients and ulnar or phalangeal shortening in 11 patients. Ultrasonography allowed hepatobiliary disease assessment and helped to establish indications for hepatic transplantation. Renal and osseous abnormalities were not specific but in the appropriate clinical setting reinforced the diagnosis.

US and color Doppler imaging of ocular and orbital disease in the pediatric age group.

Ultrasonography (US) is a rapid, accurate, noninvasive method of evaluating ocular and orbital disease. A wide spectrum of pediatric ocular and orbital disorders can be demonstrated with US: ocular congenital malformations, vitreoretinal disorders, primary and metastatic ocular and orbital neoplasms, inflammatory and congestive disease (focal or systemic), cystic disease, trauma, and foreign bodies. US may be the method of choice for studying ocular congenital anomalies and vitreoretinal disorders owing to its high definition and noninvasiveness. In ocular neoplasms, US demonstrates the morphology of the lesion, its solid or liquid nature, and the presence of calcifications. Its use is limited when the tumor extends outside the globe; computed tomography (CT) or magnetic resonance (MR) imaging must then be performed. In ocular trauma, US precisely demonstrates the presence of hemorrhage, retinal and choroidal detachment, and foreign bodies. CT and MR imaging are superior in evaluation of orbital disease. Color Doppler US often allows differential diagnosis of vascular, inflammatory, and neoplastic disease.