Estudio de la función vesicular y resultados a largo plazo de la colecistolitotomía / Gallbladder motility and long term results in cholecystolithotomy

Objetivos. La extracción de la litiasis vesicular conservando la vesícula es una alternativa a la colecistectomía que se viene realizando en nuestro servicio desde hace 25 años. Nuestra revisión tiene como objetivo valorar la tasa de recidiva y la función vesicular. Material y Métodos. A los pacientes intervenidos en nuestro servicio desde 1989 con esta técnica se les realizó una encuesta de problemas digestivos y un estudio ecográfico pre y postprandial tras la ingesta de un alimento graso para identificar la recidiva y para estimar el volumen y la contracción vesicular. Resultados. De los 20 pacientes intervenidos, 10 pacientes participaron en el estudio. Se identificó un cálculo en una paciente intervenida hace 2 años que presenta dolor abdominal (10%). La mediana de volumen preprandial fue 18,37 cc y la postprandial 7,16 cc. La mediana del porcentaje de reducción de volumen, 57,63%. Conclusiones. Solo se identificó un cálculo residual en el postoperatorio temprano. Todos los pacientes conservaron una función vesicular adecuada (valores superiores al 30%) por lo que consideramos que, de forma correctamente seleccionada, la colecistolitotomía es una alternativa a la colecistectomía en el paciente pediátrico

Introduction. Gallstone removal preserving the gallbladder is a therapeutic option in cholelithiasis. Our group has made use of this technique for twenty-five years in pediatric patients. The purpose of this review was to study gallstone recurrence and gallbladder motility. Materials and Methods. Patients who had gallstones removed with this technique from 1989 until 2013 were contacted and asked about digestive or other health problems. A sonographic assessment was made in order to look for gallstone recurrence and to measure gallbladder volumes and contraction after fasting and after consuming a fatty meal. Results. All patients are asymptomatic except a girl who suffered colic pain and who had a gallstone recurrence. The median of volume after fasting was 18.37 cc and after consuming the meal 7.16 cc. The median of gallbladder contraction was 57.63%. Conclusions. A gallstone relapse was identified (10%). Long-term follow up showed an excellent gallbladder function and contraction (greater than 30%) without other recurrences. Based on our experience, in patients properly selected cholecistolithotomy is an interesting option in children

[Gallbladder motility and long term results in cholecystolithotomy]. / Estudio de la función vesicular y resultados a largo plazo de la colecistolitotomía.

INTRODUCTION: Gallstone removal preserving the gallbladder is a therapeutic option in cholelithiasis. Our group has made use of this technique for twenty-five years in pediatric patients. The purpose of this review was to study gallstone recurrence and gallbladder motility. MATERIALS AND METHODS: Patients who had gallstones removed with this technique from 1989 until 2013 were contacted and asked about digestive or other health problems. A sonographic assessment was made in order to look for gallstone recurrence and to measure gallbladder volumes and contraction after fasting and after consuming a fatty meal. RESULTS: All patients are asymptomatic except a girl who suffered colic pain and who had a gallstone recurrence. The median of volume after fasting was 18.37 cc and after consuming the meal 7.16 cc. The median of gallbladder contraction was 57.63%. CONCLUSIONS: A gallstone relapse was identified (10%). Long-term follow up showed an excellent gallbladder function and contraction (greater than 30%) without other recurrences. Based on our experience, in patients properly selected cholecistolithotomy is an interesting option in children.

Agenesia de vesícula biliar: una causa inusual de hipertransaminasemia / Absence of the gallbladder: an unusual cause of hypertransaminasemia

La agenesia de vesícula biliar es una entidad poco frecuente, y su presentación con algún síntoma asociado es más infrecuente aún. La mayoría de casos se detectan de forma accidental en el curso de una exploración quirúrgica ante un cuadro sugestivo de litiasis biliar. Se presenta el caso de una niña de 4 años de edad con elevaciones transitorias y recurrentes de enzimas hepáticas, en quien sólo se encontró, tras un estudio exhaustivo, una agenesia de vesícula biliar como posible causa de esta alteración. Es un cuadro que hay que tener en cuenta para evitar procedimientos invasivos en estos pacientes(AU)

Abscence of the gallbladder is a very rare malformation and its symptomatic presentation is even more unusual. Most of the cases reported were discovered while a surgical exploration of the abdomen was performed in patients with biliary symptoms. It is reported a rare paediatric case. The patient was 4 years old and presented recurrent and transient elevation of liver transaminases. After a complete study only absence of the gallbladder was found as cause. Awareness of this entity by clinicians and surgeons is important because invasive diagnostic procedures can be avoided(AU)

Linfohistiocitosis hemofagocítica familiar: hallazgos neurorradiológicos / Familial hemophagocytic lymphohistiocytosis: neuroradiological findings

La linfohistiocitosis hemofagocítica (LHH) es un síndrome clínico raro, posiblemente infradiagnosticado, más frecuente en niños. Pueden ser cuadros agresivos y evolucionar en poco tiempo a fallo multiorgánico letal. Simula cuadros de sepsis infecciosa, aunque con peor respuesta y evolución. Se debe sospechar ante un niño pequeño con fiebre persistente de origen desconocido, afectación del estado general, hepatoesplenomegalia, citopenias, aumento de triglicéridos y ferritina y disminución del fibrinógeno. En la resonancia magnética cerebral se puede apreciar un realce difuso leptomeníngeo y perivascular, áreas parcheadas hiperintensas en T2 en sustancia blanca de ambos hemisferios cerebrales y atrofia cerebral. La secuencia de difusión es útil en la estadificación de las lesiones. Presentamos un caso clínico de LHH familiar de evolución mortal y realizamos una revisión bibliográfica de las características clínicas, anatomopatológicas y radiológicas de esta entidad (AU)

Hemophagocytic lymphohistiocytosis (HL) is a rare syndrome, although more common in children, that may be underdiagnosed. The clinical presentation can be aggressive, and patients may rapidly develop lethal multiple organ failure.…HL simulates the presentation of infectious sepsis, although the response to treatment and evolution are worse. HL should be suspected in young children with persistent fever of unknown origin, general malaise, hepatosplenomegaly, cytopenia, elevated triglycerides and ferritin, and decreased fibrinogen. Brain MRI shows diffuse leptomeningeal and perivascular enhancement, patchy areas of hyperintensity in the white matter of both cerebral hemispheres on T2-weighted sequences, and cerebral atrophy. Diffusion-weighted sequences are useful for staging the lesions. We present a fatal case of familial HL and review the literature about the clinical, histological, and radiological characteristics of this disease (AU)

[Familial hemophagocytic lymphohistiocytosis: Neuroradiological findings]. / Linfohistiocitosis hemofagocítica familiar: hallazgos neurorradiológicos.

Hemophagocytic lymphohistiocytosis (HL) is a rare syndrome, although more common in children, that may be underdiagnosed. The clinical presentation can be aggressive, and patients may rapidly develop lethal multiple organ failure....HL simulates the presentation of infectious sepsis, although the response to treatment and evolution are worse. HL should be suspected in young children with persistent fever of unknown origin, general malaise, hepatosplenomegaly, cytopenia, elevated triglycerides and ferritin, and decreased fibrinogen. Brain MRI shows diffuse leptomeningeal and perivascular enhancement, patchy areas of hyperintensity in the white matter of both cerebral hemispheres on T2-weighted sequences, and cerebral atrophy. Diffusion-weighted sequences are useful for staging the lesions. We present a fatal case of familial HL and review the literature about the clinical, histological, and radiological characteristics of this disease.