Shunt portosistémico, una causa poco frecuente de hepatitis colestásica en neonatología. A propósito de un caso / Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report

El shunt portosistémico congénito es una anomalía vascular venosa que comunica circulación portal y sistémica, por la que se deriva el flujo sanguíneo, salteando el paso hepático. Es una entidad poco frecuente, cuya incidencia varía entre 1/30 000 y 1/50 000 recién nacidos. Puede cursar de forma asintomática o presentarse con complicaciones en la edad pediátrica o, menos frecuente, en la edad neonatal. Ante el diagnóstico, se deberá definir la necesidad de intervención quirúrgica o intravascular para el cierre. Esta decisión depende de las características anatómicas de la malformación, de las manifestaciones clínicas y complicaciones presentes. Se presenta el caso de un paciente de un mes de vida derivado a nuestro centro para estudio de hepatitis colestásica neonatal, con diagnóstico de shunt portosistémico extrahepático. Se realizó cierre intravascular de la lesión con mejoría significativa posterior.

Congenital portosystemic shunt is a venous vascular abnormality that connects portal and systemic circulation, resulting in diversion of the blood flow, bypassing the hepatic passage. It is a rare malformation; its incidence varies from 1:30 000 to 1:50 000 newborns. It may be asymptomatic or present with complications in the pediatric age or, less frequently, in the neonatal age. Upon diagnosis, the need for a surgical or an intravascular intervention for closure should be defined. This decision depends on the malformation anatomical characteristics, clinical manifestations, and complications. We present the case of a 1-month-old patient referred to our center for the study of neonatal cholestatic hepatitis, with a diagnosis of extrahepatic portosystemic shunt. Intravascular closure of the defect was performed with significant subsequent improvement.

Cavernoma portal complicada con biliopatía secundario a enfermedad de Gaucher tipo 1: reporte de un caso peruano / Cavernoma complicated with biliopatia secondary to type 1 Gaucher disease: report of a Peruvian case

La enfermedad de Gaucher, es un trastorno autosómico recesivo de depósito lisosomal que se caracteriza por deficiencia de la beta-glucocerebrosidasa que lleva a la acumulación de glucosilceramida principalmente en células del sistema fagocítico mononuclear causando afectaciones sistémicas. Se presenta paciente varón de 20 años que cursa con dolor crónico en hipocondrio izquierdo con episodios de sangrados desde hace 3 años y sensación de alza térmica, al examen físico se identificó ictericia y esplenomegalia masiva, sin afectación neurológica. Como apoyo al diagnóstico se mostró osteoporosis severa, pancitopenia y como hallazgo inesperado la presencia de trombosis de vena porta con transformación cavernomatosa complicada con biliopatía portal simulando un tumor de klatskin, los estudios de médula y enzimáticos eran compatibles con enfermedad de Gaucher, por lo cual recibió tratamiento con imiglucerasa realizando seguimiento. Es un caso poco frecuente, de gran interés, heterogeneidad en sus manifestaciones clínicas e inéditas por su complicación, constituyendo un desafío llegar a su diagnóstico de esta enfermedad huérfana.

Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by deficiency of beta-glucosidase that would lead to the accumulation of glucosylceramide mainly in cells of the mononuclear phagocytic system causing systemic effectations. We present a patient of twenty years who is suffering from chronic pain in the left hypochondrium with episodes of bleeding for 3 years and sensation of thermal rise, physical examination revealed jaundice and massive splenomegaly, without neurological involvement. Severe osteoporosis, pancytopenia, and the presence of portal vein thrombosis with cavernomatous transformation complicated by portal biliopathy simulating a klatskin tumor, marrow and enzymatic studies were compatible with Gaucher disease, were shown as unexpected findings. he received treatment with imiglucerase, following up. It is a rare case, of great interest, heterogeneity in its clinical manifestations and unpublished by its complication, constituting a challenge to reach its diagnosis of this orphan disease.

Malformación de Abernethy: causa inusual de cianosis central en pediatría / Abernethy malformation: unusual cause of central cyanosis in pediatrics

El shunt porto sistémico congénito es una causa rara de hipoxemia y una patología muy poco frecuente con complicaciones severas si no es tratada. Fue descrito por primera vez por John Abernethy en 1793. Existen dos tipos: tipo I (shunt termino lateral) en el que hay ausencia total del flujo portal intrahepático y tipo II (shunt latero lateral) con flujo portal parcialmente conservado. Se presenta el caso de una niña de 6 años de edad con antecedente de hipoxemia crónica desde los 4 años y medio de vida, acompañado de disnea progresiva, quien fue referida a la unidad de neumología pediátrica con diagnóstico de cianosis central. Entre los estudios diagnósticos considerados se realizó ecografía doppler del sistema venoso portal, evidenciándose ausencia de vena porta principal; además se realizó angiotomografía del sistema arterio-venoso portal y mesentérico, confirmándose la agenesia de vena porta. Se completó el estudio con una porto-esplenografía que confirmó el diagnóstico de malformación de Abernethy tipo I b. La malformación de Abernethy tipo I es más frecuente en el sexo femenino, tiene varias formas de presentación y el tratamiento es el trasplante hepático. En la malformación de Abernethy tipo II la circulación portal es variable y tiene mejor pronóstico que la de tipo I. La disnea al ejercicio y la cianosis central es una forma de presentación que debemos tener en cuenta en el diagnóstico diferencial de la patología cardiorrespiratoria en la edad pediátrica.

The congenital portosystemic shunt is an uncommon disease with severe complications if not treated. This rare cause of hypoxemia was first described by John Abernethy in 1973. There are two types: type I (termino-lateral shunt), in which there is total absence of the intrahepatic portal flow, and type II (latero-lateral shunt), in which the portal flow is partially preserved. We present the case of a 6-year-old girl with chronic hypoxemia history since 4 and a half years of age, showing progressive dyspnea, who was referred to the Pediatric Pulmonary Division with the diagnosis of central cyanosis. An Echo-Doppler in the portal venous system was performed, reporting agenesis of the principal portal vein. This finding was corroborated by an angiography of the portal and mesenteric arteriovenous system. The study was completed with a portosplenography, which confirmed the diagnosis of type Ib Abernethy malformation. The type I Abernethy malformation is more common in females, shows up in different ways and is treated with liver transplantation. On the other hand, type II Abernethy malformation shows a variable portal circulation and has a better prognosis than type I. Dyspnea when exercising and central cyanosis should be considered to make a differential diagnosis of cardiorespiratory disease at a pediatric age.

Congenital intrahepatic portosystemic shunt diagnosed during intrauterine life / Shunt portossistêmico congênito intra-hepático diagnosticado na vida intrauterina

Abstract Objective: To report a patient with prenatal diagnosis of portosystemic shunt; a rare condition in humans. Case description: 17-Day-old female infant admitted for investigation of suspected diagnosis of portosystemic shunt, presumed in obstetric ultrasound. The hypothesis was confirmed after abdominal angiography and liver Doppler. Other tests such as echocardiography and electroencephalogram were performed to investigate possible co-morbidities or associated complications, and were normal. We chose conservative shunt treatment, as there were no disease-related complications and this was intrahepatic shunt, which could close spontaneously by the age of 2 years. Comments: Portosystemic shunt can lead to various complications such as hepatic encephalopathy, hypergalactosemia, liver tumors, and hepatopulmonary syndrome. Most diagnoses are done after one month of age, after such complications occur. The prenatal diagnosis of this patient provided greater security for the clinical picture management, as well as regular monitoring, which allows the anticipation of possible complications and perform interventional procedures when needed.

Resumo Objetivo: Descrever a história clínica de paciente com diagnóstico pré-natal de shunt portossistêmico, condição rara na espécie humana. Descrição do caso: Recém-nascido do sexo feminino internada aos 17 dias para investigação de suspeita diagnóstica de shunt portossistêmico, aventada na ecografia obstétrica. A hipótese foi confirmada após angiotomografia do abdome e ecodoppler hepático. Outros exames, como ecocardiograma e eletroencefalograma, foram feitos para investigação de possíveis comorbidades ou complicações associadas e tiveram resultados normais. Optou-se por tratamento conservador do shunt, já que não havia quaisquer complicações relacionadas à doença e tratava-se de shunt intra-hepático, que pode fechar espontaneamente até os dois anos de idade. Comentários: O shunt portossistêmico pode levar a diversas complicações, como encefalopatia hepática, hipergalactosemia, tumores hepáticos e síndrome hepatopulmonar. A maioria dos diagnósticos é feita a partir de um mês de vida, após tais complicações ocorrerem. O diagnóstico pré-natal dessa paciente possibilitou maior segurança para o manejo do quadro, bem como um acompanhamento periódico que permite antecipar possíveis complicações e adotar conduta intervencionista, se necessário.

Shunt porto-sistémico congénito en hígado no cirrótico como causante de síndrome confusional agudo reiterado: report of one case / Portosystemic shunt in a normal liver causing confusional states

Portosystemic shunts are rare vascularization disorders, and an uncommon cause of confusional states. We report an 87-year-old male with a previously normal cognitive status who was repeatedly admitted for sudden symptoms of disorientation and functional limitation. The patient had high ammonium levels which lead to the suspicion of the presence a portosystemic shunt, even in the absence of pre-existing liver disease. A contrast enhanced computed tomography of the abdomen confirmed the presence an abnormal communication of the right portal vein with the suprahepatic veins. The communication was embolized and the confusional states of the patient subsided.

Ausencia congénita de la vena porta hepática asociada a malrotación intestinal y a vena cava inferior izquierda: reporte de caso / Congenital absence of the hepatic portal vein associated to intestinal malrotation and left-sided inferior vena cava: a case report

La ausencia congénita de la vena porta hepática es una malformación en extremo rara, fue descrita por primera vez en 1793 por John Abernethy y a la fecha se han reportado sólo 101 casos. Afecta con mayor frecuencia a mujeres y determina que el drenaje venoso intestinal sea derivado hacia el territorio de las venas sistémicas. Es también conocida como derivación porto-sistémica extra hepática congénita (CEPS), por su sigla en inglés, y se asocia a otras alteraciones congénitas,incluyendo anomalías cardíacas, de las vías biliares, enfermedades metabólicas y retardo mental. En este trabajo presentamos el hallazgo de esta malformación en el cadáver de un niño de dos años de edad, donde la vena porta seguía un trayecto anómalo y se unía a la vena renal derecha. El confluente venoso "mesentérico-esplénico-renal" así formado presentaba un trayecto descendente, recibía a la vena gonadal derecha, y desembocando en la confluencia de las venas ilíacas comunes. Esto se asociaba a mal rotación intestinal, arteria hepática aberrante y a vena cava inferior izquierda, situación descrita sólo una vez en la literatura. El hallazgo de estas anomalías anatómicas en los cadáveres disecados con fines docentes en nuestro Departamento de Anatomía, tiene un valor formativo indiscutible para nuestros alumnos de pre y postgrado, quienes pueden valorar las implicancias de este conocimiento anatómico en la clínica diaria.

Congenital absence of the hepatic portal vein is an extremely rare malformation that was first described by John Abernethy in 1793. Only 101 cases had been described in the literature until 2015 and most affected females. In this anomaly, also known as congenital extrahepatic porto-systemic shunt (CEPS), intestinal venous drainage is derived towards the territory of the systemic veins and could be associated with other birth defects, including heart and biliary tract anomalies, metabolic diseases, mental retardation. We present the case of a 2-year-old boy who died because of multifocal pneumonia and necropsy showed anatomical findings consistent with this malformation as an incidental finding. The portal vein followed an anomalous course and joined the right renal vein, forming the "mesenteric-splenic-renal" venous collector, which then received the right gonadal vein and ended at the confluence of the common iliac veins. In our case this anomaly was associated to intestinal malrotation, aberrant hepatic artery and persistent left inferior vena cava, situation described once in the literature. The finding of these anatomical abnormalities in cadavers has a great teaching value for our undergraduate and graduate students who are learning anatomy and they can also assess the associated clinical.

N-Butyl Cyanoacrylate Embolization with Blood Flow Control of an Arterioportal Shunt That Developed after Radiofrequency Ablation of Hepatocellular Carcinoma

We present a case of a patient with rapid deterioration of esophageal varices caused by portal hypertension accompanied by a large arterioportal shunt that developed after radiofrequency ablation of hepatocellular carcinoma. We used n-butyl cyanoacrylate (NBCA) as an embolic material to achieve pinpoint embolization of the shunt, because the microcatheter tip was 2 cm away from the shunt site. Under hepatic arterial flow control using a balloon catheter, the arterioportal shunt was successfully embolized with NBCA, which caused an improvement in the esophageal varices.

Sonography of hepatic hemangioma accompanied by arterioportal shunt

No abstract available.

Avaliação morfométrica e hemodinâmica comparativa dos vasos envolvidos no shunt portossistêmico congênito em cães / Morphometric and haemodynamic evaluation of the vessels associated with the congenital portosystemic shunting in dogs

The morphometry and haemodynamic aspects of portal vein were studied in 20 normal dogs with less than 120 days of age and in 14 dogs presenting portosystemic shunting with ages between 90 and 360 days. In the control group the hepatic margins were seen 1.50cm to 3.00cm caudal to the costal margin. Collected data indicated that the mean diameter of portal vein (VP), caudal vena cava (VCC) and abdominal aorta (AO) measured respectively, 0.38cm, 0.37cm and 0.41cm. The VP/VCC and VP/AO mean ratios were respectively, 1.10 and 0.94. The average of VP, VCC and AO areas were respectively, 0.12cm², 0.11cm² and 0.14cm². The haemodynamic of portal vein was studied by ultrasound Doppler and the mean velocity of portal blood flow (VMFSP) measured was 17.76cm/s. It was verified that portal blood flow (FSP) average was 83.11ml/min/kg and the congestion index (IC) average was 0.006. In the group of animals presenting portosystemic shunting, the hepatic margins were seen 1.00cm to 2.00cm cranial to the costal margin. The morphometry of VP, VCC and AO presented a mean diameter of 0.40cm, 0.74cm and 0.56cm, respectively. The VP/VCC and VP/AO mean ratios were respectively, 0.54 and 0.69. The average of VP, VCC and AO areas were respectively, 0.14cm², 0.31cm² and 0.25cm². The haemodynamic study demonstrated that the VMFSP measured was 22.29cm/s and the IC average was 0.006.

Foram realizados o estudo morfométrico e o estudo hemodinâmico da veia porta em vinte cães clinicamente normais, de idade igual e inferior a 120 dias e em quatorze cães portadores de shunt portossistêmico, de idades entre 90 e 360 dias. Nos cães do grupo controle, as margens hepáticas apresentaram-se entre 1,50cm e 3,00cm caudalmente à margem costal. Os diâmetros médios da veia porta (VP), veia cava caudal (VCC) e aorta abdominal (AO) obtidas foram respectivamente, 0,38cm, 0,37cm e 0,41cm. As proporções entre os diâmetros médios VP/VCC e VP/AO apresentaram médias de 1,10 e 0,94, respectivamente. As médias das áreas da VP, VCC e AO resultaram respectivamente em 0,12cm² , 0,11cm² e 0,14cm². No estudo hemodinâmico da VP destes animais, utilizando-se o ultrassom Doppler, a velocidade média de fluxo sangüíneo portal (VMFSP) mediu 17,76cm/s. A média de fluxo sangüíneo portal (FSP) resultou em 83,11ml/min/kg. O índice de congestão (IC) apresentou média de 0,006. Para o grupo de cães portadores de shunt portossistêmico, o fígado apresentou redução de seu volume, sendo as margens hepáticas visibilizadas entre 1,00cm e 2,00cm cranialmente à margem costal. No estudo morfométrico, as médias dos diâmetros médios obtidos de VP, VCC e AO resultaram respectivamente em 0,40cm, 0,74cm e 0,56cm. As proporções entre os diâmetros médios VP/VCC e VP/AO resultaram respectivamente em 0,54 e 0,69. As médias das áreas de VP, VCC e AO resultaram respectivamente em 0,14cm², 0,31cm² e 0,25cm². Ao ultrassom Doppler a VMFSP mediu 22,29cm/s e a média do IC da VP obtido foi de 0,006.

Cavernoma de la vena porta. Descripción de casos clínicos pediátricos / Portal vein cavernoma, a description of pediatric clinical cases

La cavernomatosis portal es una patología poco frecuente causada por la trombosis de la vena porta. Es la principal causa de hipertensión portal en niños. La causa muchas veces no se identifica, pudiendo reconocerse factores predisponentes, entre los cuales los más frecuentes son el cateterismo de la vena umbilical y la onfalitis. Se manifiesta a través de sus complicaciones: hemorragia digestiva alta por várices esofágicas y esplenomegalia. El diagnóstico se confirma con ecografía abdominalcon Doppler. La terapéutica incluye el tratamiento médico-endoscópico de las várices esofágicas. La derivación quirúrgica porto-sistémica resuelve la obstrucción portal. Se realizó un estudio retrospectivo con el objetivo de describir las características clínicas de pacientes con cavernomatosis portal en el período enero de 1999 a abril de 2009 en el Hospital Pediátrico del Centro Hospitalario Pereira Rossell (HP-CHPR). Se identificaron ocho pacientes, con una media de edad al momento del diagnóstico de 2 años y 2 meses. La forma de presentación más frecuente fue la hematemesis. En la mayoría se constató esplenomegalia. En tres se identificaron factores predisponentes: cateterismo umbilical, cirugía abdominal. Todos tenían várices esofágicas al diagnóstico. Tres pacientes reiteraron hemorragia digestiva alta, uno en tres y otro en cuatro oportunidades. Cuatro pacientes requirieron escleroterapia o banding por sus várices esofágicas. Un paciente recibió cirugía derivativa. Esta patología no tuvo, en este período de tiempo, una alta prevalencia, pero alta morbilidad. Todos eran pequeños, la mayoría sin factores de riesgo y se presentaron a través de complicaciones de la enfermedad. La mayoría reiteró complicaciones en la evolución.

Intrahepatic Portosystemic Venous Shunt: Successful Embolization Using the Amplatzer Vascular Plug II

A 67-year-old woman presented with memory impairment and behavioral changes. Brain MRI indicated hepatic encephalopathy. Abdominal CT scans revealed an intrahepatic portosystemic venous shunt that consisted of two shunt tracts to the aneurysmal sac that communicated directly with the right hepatic vein. The large tract was successfully occluded by embolization using the newly available AMPLATZERTM Vascular Plug II and the small tract was occluded by using coils. The patient's symptoms disappeared after shunt closure and she remained free of recurrence at the 3-month follow-up evaluation.

RE: Endovascular Treatment of Congenital Intrahepatic Portosystemic Shunts with Amplatzer Plugs

No abstract available.

Congenital Intrahepatic Portosystemic Venous Shunt and Liver Mass in a Child Patient: Successful Endovascular Treatment with an Amplatzer Vascular Plug (AVP)

A congenital intrahepatic portosystemic shunt is a rare anomaly; but, the number of diagnosed cases has increased with advanced imaging tools. Symptomatic portosystemic shunts, especially those that include hyperammonemia, should be treated; and various endovascular treatment methods other than surgery have been reported. Hepatic masses with either an intra- or extrahepatic shunt also have been reported, and the mass is another reason for treatment. Authors report a case of a congenital intrahepatic portosystemic shunt with a hepatic mass that was successfully treated using a percutaneous endovascular approach with vascular plugs. By the time the first short-term follow-up was conducted, the hepatic mass had disappeared.

Embolization of congenital intrahepatic porto-systemic shunt by n-butyl cyanoacrylate.

Congenital intrahepatic portosystemic venous shunt (IHPSVS) is rare vascular anomaly. We present one case of a 14- month male child who presented with global developmental delay. Child had high ammonia levels with low glutamine and high bile salts on the previous investigations and had history of neonatal seizures since day 13 of life. On admission, serum ammonia levels were elevated to 112μmol/L. Other laboratory investigations including liver and renal function test, and electrolytes were normal. He was, diagnosed to have IHPSVS on the basis of Doppler and CT, and treated by embolization with n-butyl cyanoacrylate (glue). A brief review of diagnostic modalities and endovascular management for the IHPSVS is presented including the present case.

Radiological Findings in a Case of Multiple Focal Nodular Hyperplasia Associated with Portal Vein Atresia and Portopulmonary Hypertension

We present here the radiological findings of a rare case of multiple focal nodular hyperplasia that was associated with portal vein atresia and portopulmonary hypertension in a young woman. This case illustrates and supports the pathophysiological hypotheses that were previously proposed for the coexistence of these three abnormalities.

Necrose isquêmica hepática e diabete melito: relato de caso / Liver ischemic necrosis and diabetes mellitus: case report

JUSTIFICATIVA E OBJETIVOS: O infarto hepático é definido como necrose isquêmica do parênquima hepático envolvendo pelo menos dois ácinos. Trata-se de evento considerado raro devido ao duplo suprimento sangüíneo, arterial e venoso. O objetivo deste estudo foi relatar um caso de paciente não sabidamente diabética que desenvolveu extensas áreas isquêmicas de infarto hepático, após quadro de descompensação aguda da diabete. RELATO DO CASO: Paciente do sexo feminino, 67 anos, hipertensa, procurou o Pronto Socorro com queixas de polidipsia, poliúria, turvação visual, náuseas e vômitos, dificuldade para deambular, havia aproximadamente 10 dias. Ao exame físico foi observado desidratação, palidez cutânea, cianose periférica, hipotermia, taquicardia, hipotensão, dor abdominal leve e difusa. Exames laboratoriais mostraram: leucócitos: 16800, creatinina (Cr): 3,7, uréia (Ur): 167, Na: 133, K: 6.9, glicose: 561; gasometria arterial (cateter de oxigênio: 2 L/min): pH: 6.93, pCO2: 12.1, pO2: 107, BE: -28,8, HCO3: 2,4, Sat 91,3 por cento, lact: 79; urina I: pH: 6,0; leucócitos: 13; densidade: 1015; eritrócitos: 19; proteína: ++; glicose: +++; bilirrubina: negativa; corpos cetônicos: + denotando cetonemia. Eletrocardiograma com onda T apiculada, bloqueio de ramo direito. A paciente foi tratada com insulina, hidratação, bicarbonato de sódio e introduzido antibioticoterapia. Após o tratamento inicial, os exames laboratoriais mostraram: Cr: 2,2, Ur: 122, Na: 162, K: 4,3, Ca: 6,4, glicose: 504, pH: 7,01, HCO3: 7.1, BE: - 22. Um dia após, a paciente apresentou importante dor abdominal acompanhada de irritação peritoneal, além de sonolência e dificuldade para falar; exames laboratoriais mostraram: pH: 7,4, pCO2 : 31, pO2: 68, BE: -4,4, HCO3: 19, Sat.O2: 93,5 por cento; Ur: 95; Cr: 1,4, albumina: 2,4, Ca: 0,95, Na: 166, K:4, bilirrubina: 0,5, bilirrubina D/I: 0,2/0,3, amilase: 1157, Gama-GT: 56, AST 7.210, ALT: 2.470, VHS: 15, lipase: 84. Ultrasonografia abdominal...

BACKGROUND AND OBJECTIVES: Hepatic infarction is characterized by parenchyma ischemic necrosis involving at least two acinis. It is extremely uncommon due to the arterial and portal venous blood supply. We report a case of a patient not know to have diabetes who developed massive areas of ischemic infarcts of the liver after episode of acutely diabetes decompensated. CASE REPORT: A 67 year-old hypertensive female who has been presenting, for the last 10 days, polydipsia, high urinary volume, visual and gait impairment, nausea and vomiting was admitted to the emergency room (ER). During the physical examination it was observed dehydration, skin discoloration, peripheral cyanosis, hypothermia, tachycardia, hypotension and mild diffuse abdominal pain. Admissional laboratory exams demonstrated total leukocytes: 16.800, Cr: 3.7, Ur: 167, Na: 133, K: 6.9, glucose: 561; arterial gasometry (O2 catheter: 2 L/min): pH: 6.93, pCO2: 12.1, pO2: 107, B.E.: -28.8, HCO3: 2.4, Sat 91.3 percent, lactato: 79; urinalysis: pH: 6; leukocytes: 13; density: 1015; erythrocytes: 19; protein: ++; glucose: +++; bilirubin: negative; ketonic bodies: + denote ketonemia. EKG: sharp T wave, right branch block. Patient was treated with intravenous insulin, hydration, sodium bicarbonate and ceftriaxone. After initial treatment, the laboratory exams showed Cr: 2.2, Ur: 122, Na: 162, K: 4.3, Ca: 6.4, glucose: 504, pH: 7.01, HCO3: 7.1, B.E.: -22. One day after admission the patient presented with important abdominal pain and peritoneal irritation, followed by difficulty for talking and somnolence; routine laboratory exams showed arterial gasometry: pH: 7.4, pCO2: 31, pO2: 68, BE: -4.4, HCO3: 19, SatO2: 93.5 percent; Ur: 95,Cr: 1.4, albumin: 2.4, Ca: 0.95, Na: 166, K:4, bilirubin: 0.5, bilirubin D/I: 0.2/0.3, Amylase: 1157, Gamma-GT: 56, AST 7.210, ALT: 2.470, SR (sedimentation rate): 15, Lipase: 84. Abdominal ultrasound was unremarkable. Patient respiratory function and conscience...

Presentación de un caso de cavernomatosis de la porta en Pediatría / A case presentation of Porta´s cavernomatosis in Pediatrics

Transicional masculino, blanco, con antecedentes de distress respiratorio severo al nacer que necesitó de cateterización venosa umbilical y ventilación mecánica prolongada en la etapa neonatal precoz, con retardo del desarrollo psicomotor que ingresa en esta ocasión por vómitos y deposiciones de mala calidad que se interpreta como una enfermedad diarreica aguda (EDA) de etiología viral y que en el transcurso de la misma hace sangramiento digestivo profuso que le lleva a la anemia aguda y al shock hipovolémico, por lo que se envía de nuestro centro al servicio de Cirugía Pediátrica, donde se interviene de urgencia con el diagnóstico de cavernomatosis de la porta, luego de un postoperatorio tórpido, se recupera y egresa.

Masculine transitional,white,with antecedents of severe respiratory distress at birth who needed a umbilical venous catheterization and prolongued mechanic ventilation in the precocious neonatal stage with a delay in the psychomotor development that appear in this case through vomits and bad quality stool which is interpreted as a EDA of a viral ethiology and that during the course of it cause severe digestive bleeding which carry out an acute anaemia and a hypovolemic shock, that´s why it´s sent from our center to the pediatric surgery service, where he recieve special delivery operation with the diagnosis of Porta´s cavernomatosis, then after a post operated dormancy, he is recovered and depart.

Congenital intrahepatic porto-systemic venous shunt with galactosemia.

We report a 51-day-old infant with congenital intrahepatic porto-systemic venous shunt associated with galactosemia, who presented with cholestatic jaundice. He was treated with ursodeoxycholic acid, calcium supplements and galactose-free diet. The child was asymptomatic six weeks later.

Malrotation of the intestine and preduodenal portal vein associated with abdominal situs inversus: a case report

Abdominal situs inversus is a rare condition usually associated with malformations of asymmetric organs such as the heart, liver, spleen and malrotation of the intestines. A case of abdominal situs inversus with intestinal malrotation and preduodenal portal vein is reported. Patient underwent prophylactic Ladd's procedure and preduodenal portal vein was left undisturbed during surgery. This case highlights the importance of rigorous investigation of anatomic features prior to surgery in a patient with heterotaxia. The authors advocate radiological investigation of patients with heterotaxia and prophylactic Ladd's procedure in those with intestinal malrotation.

Predictors of esophageal varices in patients of liver cirrhosis

To identify non-endoscopic predictors of esophageal varices in patients with liver cirrhosis. This observational and analytical study was carried at GI and Liver Clinic, Saeed Anwar Medical Center, Dabgari Gardens, Peshawar from January 2006 to August 2006. Seventy-three patients with established cirrhosis and no history of variceal bleeding were evaluated for predetermined variables and underwent endoscopy to look for esophageal varices. Out of 73 patients, 51 [69.9%] were males and 22 [30.1%] were females. Forty-four [60.3%] patients were having esophageal varices on endoscopy and 29 [39.7%] patients were having no varices. Out of 44 patients, small varices were found in 28 [63.6%] patients while large varices were found in 16 [36.4%] patients. Platelet count <65 x 103/ mico L. serum albumin <2.2 g/dl and portal vein diameter> 13mm on ultrasound were found to have significant predictive value for large varices. Platelet count less than 65 chi 103/ micro L, serum albumin less than 2.2 g/dl and portal vein diameter more than 13 mm on ultrasound are independent and significant predictors of esophageal varices on endoscopy. Therefore screening endoscopy must be done in all patients with liver cirrhosis who have no history of GI bleeding but any of these predictors