Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: Pulmonary hypertension and pulmonary vascular dilatation

Law YM, Mack CL, Sokol RJ, Rice M, Parsley L, Ivy D. Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: Pulmonary hypertension and pulmonary vascular dilatation.
Pediatr Transplantation 2011: 15: E162–E168. © 2010 John Wiley & Sons A/S. Abstract: HPS and PPHTN are unusual and challenging pulmonary manifestations of liver disease. We report two pediatric cases in association with heterotaxy polysplenia syndrome and congenital absence of the portal vein. Both patients were symptomatic and hemodynamically compromised and required aggressive medical therapy. One patient with PPHTN alone achieved a successful liver transplant. The second child presented with combined HPS and PPHTN and exhibited a different evolution of pulmonary vascular disease. These cases illustrate associations that must be entertained in the setting of heterotaxy syndrome, cyanosis, or pulmonary hypertension and how strategic medical combined with surgical management can provide a good outcome.     

Extrahepatic portosystemic shunt in congenital absence of the portal vein depicted by time-resolved contrast-enhanced MR angiography

Congenital absence of the portal vein is a rare malformation in which mesenteric and splenic venous flow bypasses the liver and drains into various sites in the systemic venous system via an extrahepatic portosystemic shunt. In an 11-year-old girl with congenital absence of the portal vein, the detailed anatomy of the extrahepatic portosystemic shunt is demonstrated by time-resolved contrast-enhanced MR angiography. Electronic supplementary material The online version of this article (doi: ) contains supplementary material, which is available to authorized users.     

Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings

We describe a 10-year-old girl with congenital absence of the portal vein (CAPV) and multiple hyperplastic nodules in the liver. MRI appearances of the liver lesions and the portocaval anastomosis between the inferior mesenteric vein and internal iliac veins are presented. In addition, the relevance of CAPV and nodular lesions of the liver is reviewed.     

Congenital absence of the portal vein in a boy

Congenital absence of the portal vein (CAPV) is a malformation that is generally thought to be limited to females. We encountered an 11-year-old boy with this malformation. In 17 previously reported cases of CAPV, 2 were male. Three male patients, including our case, were Abernethy type Ib malformation. They had no associated liver tumour and two had no additional congenital abnormality. Conversely, 13 of the 15 female patients had congenital abnormalities and 6 had liver mass lesions. Received: 8 January 1998 Accepted: 24 September 1998     

64层CT在儿童门静脉海绵样变性手术治疗中的价值

目的 探讨64层CT在门静脉海绵样变性术前病情判断及手术方式选择中的作用.方法 情况,与患儿手术治疗方式对比分析.结果 12例患儿中5例食管胃底静脉曲张,行胃底及食管下段血管断流术和脾切除术,4例仅行脾切除术,3例病变累及肝内门静脉行活体肝移植.自发性脾/胃-肾静脉分流8例,腹膜后Retzius静脉丛(RV)开放4例,术中保留.术后1例肝移植出现门静脉血栓死亡,其余无明显并发症.结论 64层螺旋CT血管成像可清楚显示门静脉及分支,侧支循环建立情况,有助于术前手术方案选择.     

Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome)

Absence of the portal vein with systemic visceral venous return was demonstrated in an 8-year old girl with oculoauriculovertebral dysplasia (Goldenhar syndrome) during preoperative evaluation of a liver mass. Congenital absence of the portal vein is a rare malformation of potential clinical significance.     

Meso‐Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: Review and perspectives

de Ville de Goyet J, Lo Zupone C, Grimaldi C, D’Ambrosio G, Candusso M, Torre G, Monti L. Meso‐Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: Review and perspectives. Abstract: Direct portal revascularization can be achieved by interposing a vascular graft between the SMV and the Rex recessus (left portal vein system): the MRB. To review indications and results of the procedure in the setting of pediatric liver transplantation, reports were selected from the English literature. Previously reported series were updated to analyze long‐term outcome. A new series was added and analyzed as a complementary set of cases. A total of 51 cases were analyzed. With a 96% overall patient survival rate and a 100% long‐term patency rate when the IJV is used for the bypass, MRB achieves a very successful physiologic cure of chronic portal hypertension and restores the portal flow into and through the liver graft. It also has been used successfully for primary revascularization of liver grafts, as well as for managing early acute portal vein thrombosis episodes. The use of this procedure in conjunction with other strategies and techniques might be of interest for transplant surgeons, particularly those caring for children.     

儿童门静脉海绵样变性的临床特点及手术治疗策略CSCD

目的探讨儿童门静脉海绵样变性的临床特点及手术治疗策略。方法回顾性分析湖南省儿童医院2010年5月至2022年1月收治的27例儿童门静脉海绵样变性患儿临床资料,其中男12例,女15例;年龄1岁7个月至11岁11个月。患儿均经腹部超声及CT检查确诊为儿童门静脉海绵样变性,并经数字减影血管造影(digital subtraction angiography,DSA)评估肝内外门静脉通畅情况,根据病变情况选取手术方式。结果27例患儿均实施手术。其中Rex手术19例,行Rex手术的患儿术后门静脉系统压力均明显下降;其中2例利用粗大肝外侧支静脉与门静脉矢状部行侧侧吻合,肝内门静脉获得满意灌注量;1例行巨脾切除后利用脾静脉行经典Rex手术;2例搭桥血管血栓形成,经抗凝治疗后失败,血管闭塞。3例因严重门静脉海绵样变性和反复消化道出血实施肝移植手术,其中1例早期发生肝动脉血栓,经抗凝治疗后再通。3例实施远端脾肾分流手术,分流血管通畅。2例实施近端脾肾分流手术,其中1例出现脾静脉附壁血栓,经抗凝治疗后好转。结论儿童门静脉海绵样变性常常以门脉高压相关症状为临床特点,手术方式应根据肝内外门静脉发育和代偿情况综合分析后进行选择,搭桥血管可以根据患儿自身血管特点进行选择。首选Rex手术的原因在于其能够恢复门静脉系统正常解剖通道和生理作用,改善肝脏灌注和生长发育,避免肝性脑病的发生。     

Congenital absence of the portal vein: Case report and MR demonstration

Congenital absence of the portal vein (CAPV) is a rare anomaly that results from aberrant venous development in early embryonic life. The intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava (IVC), the left renal vein, or the left hepatic vein. We describe a case where prenatal ultrasonography demonstrated an unusual C-shaped vessel between the umbilical vein and a dilated IVC, and failed to show a portal vein. Ultrasonography and magnetic resonance imaging and magnetic resonance angiography at 4 months of age again found no portal vein. The superior mesenteric vein drained into the left renal vein.     

Pathology of Noncirrhotic Portal Hypertension: Clinicopathologic Study in Pediatric Patients

From 1995–2002, 14 patients with predominantly prehepatic, noncirrhotic portal hypertension were evaluated. At presentation, the eight females and six males had a mean age of 9 years (range 2–18). Seven were admitted with gastrointestinal, mostly esophageal bleeding, three with splenomegaly, three with hepato-pulmonary syndrome, and one with hyperammonemia. Imaging studies showed portal vein obstruction in six patients and nonobstructed but frequently anomalous vascular patterns, including hypoplasia of the portal vein, in the remaining eight patients. At the onset, liver function was marginally abnormal in all patients, but thrombocytopenia of approximately 100 × 10⁹/L was consistently observed, probably reflecting chronic mild consumption coagulopathy and hypersplenism. The most striking and frequent histopathologic finding in 25 liver samples, was the presence of hypoplastic portal triads with collapsed portal vein radicles. In contrast, other triads showed markedly distended and misshapen portal vein radicles and likely lymphatics. These two patterns of collapse and distention presumably reflect areas of impaired versus overloaded intrahepatic portal venous flow. Some of the biopsies showed variable portal/sinusoidal fibrosis. Four patients (two with intestinal bleeding, two with hepato-pulmonary syndrome) required liver transplants and are doing well. Eight patients are doing well clinically after surgical or spontaneous vascular shunting. Two patients with intestinal bleeding and hepato-pulmonary syndrome, respectively) who had congenital dyskeratosis, underwent bone marrow transplantation and died of nonhepatic-related complications. It is possible to suggest prehepatic causes of portal hypertension even in needle biopsies when collapsed portal vein radicles are present in portal triads, but more than one biopsy sample with larger bore bioptomes may be required to see the changes. Conversely, identifying these changes may suggest to the clinicians the need to work-up a patient for portal hypertension. This study was presented in abstract form at the annual meeting of the Society for Pediatric Pathology, Chicago, Illinois, USA, February 2002.     

Agenesis of the Ductus Venosus and its Correlation to Hydrops Fetalis and the Fetal Hepatic Circulation: Case Reports and Review of the Literature

Under normal conditions about 50% of the placental venous return bypasses the liver through the ductus venosus. This blood flow is preferentially directed toward the foramen ovale and provides optimum oxygenation to the fetal heart and brain. Absence of the ductus venosus is a rare vascular anomaly, the significance of which has been disputed. We distinguish the pattern in which the liver is entirely bypassed, a manifestation of a fundamental malformation in the umbilical venous system, from the pattern in which the ductus venosus is absent despite a normal course of the umbilical vein. We review the literature regarding the latter and report eight new cases. Three of the four previously reported cases showed associated malformations and two of them suffered from portal congestion and hydrops. Among our eight cases three showed severe malformations in the cardiovascular system. Three cases presented themselves with hydrops fetalis and disturbance in the portal circulation, and two cases expressed signs of intrauterine asphyxia. The absence of the ductus venosus might be a minor vascular maldevelopment resulting in an early disturbance in the portal circulation. Our findings suggest that this anomaly might induce hydrops fetalis.     

Congenital absence of the portal vein in a child with Turner syndrome

Congenital absence of the portal vein (CAPV) is a rare malformation associated with hepatic encephalopathy and liver function abnormalities. We report a case of a 2-year-old with Turner syndrome, CAPV, and congenital heart malformations.     

小儿门静脉分型及肝脏体积测量的数字化研究

目的 应用海信计算机辅助手术系统(海信CAS)研究小儿门静脉解剖变异并测量相应的流域体积,为实施小儿精准肝切除术提供理论依据.方法 应用海信CAS将106例儿童上腹部增强CT进行肝脏及血管三维重建,分析小儿肝脏门静脉主干解剖变异并进行分型,按照年龄分组,自动测量右前叶、右后叶及左叶的流域体积.结果 海信CAS能够立体、清晰地显示小儿肝脏门静脉主干的解剖走行,并自动测量各流域体积.门静脉分型:a型82例(77.4％),b型12例(11.3％),c型8例(7.6％),d型2例(1.9％),e型1例(0.9％),f型1例(0.9％).根据门静脉右前支、右后支及左支供血区域实现个体化肝段划分.各年龄段组间肝脏体积差异有统计学意义(P＜0.05);肝脏总体积与年龄、身高、体重、体表面积均呈正相关(年龄:R=0.889,P＜0.05;身高:R=0.914,P＜0.05;体重:R=0.919,P＜0.05;体表面积:R=0.931,P＜0.05).结论 海信CAS能准确立体的显示出肝内血管走行并自动测量流域体积,为小儿门静脉分型及个体化肝段划分提供可靠依据,对小儿精准肝切除术的实施具有重要意义.     

Study of the Malformation of Ductal Plate of the Liver in Meckel Syndrome and Review of Other Syndromes Presenting with This Anomaly

Meckel syndrome (MIM 249.000) is an autosomal recessive disorder with a variable spectrum of anomalies. Since the first reports of this syndrome, very broad diagnostic criteria have been proposed, but the process of defining them continues. It is probable that at least two of three manifestations, including cystic kidney dysplasia, occipital encephalocele or other anomaly of the central nervous system, and postaxial polydactyly occur in most cases. Arrest of the development of intrahepatic bile ducts at the stage of the bilaminar plate formation or ductal plate malformation is considered of high diagnostic value in Meckel syndrome, but there is no complete agreement in the literature about its occurrence. The aims of this investigation were to study the prevalence and morphologic patterns of ductal plate malformation of the liver in Meckel syndrome by evaluating the dilatation of primitive biliary structures and the increase in connective tissue of the portal tract. Archival data files from four German centers (Berlin, Freiburg, Heidelberg, Mainz) were reviewed. Liver sections of 30 well-studied fetuses with Meckel syndrome were immunostained with antibodies against cytokeratins (intermediate filaments of the cytoskeleton) and factor VIII (an endothelial cell marker) and were evaluated both qualitatively and quantitatively. Cystic kidney dysplasia, occipital encephalocele, and postaxial polydactyly were found in 100%, 90%, and 83.3% of the fetuses, respectively. Ductal plate malformation of the liver was a constant anomaly in Meckel syndrome, seen as frequently as renal lesions. We observed essentially two kinds of hepatic lesions: 23 cases showed mainly a cystic dilatation of primitive biliary structures with little portal fibrosis, while 7 cases showed mainly rings of interrupted curved lumina around a central fibrovascular axis and pronounced portal fibrosis. In these seven cases an abnormal pattern of the portal vein, with many small and closely spaced branches of the portal vein (the so-called pollard willow pattern), was also seen. With respect to other fetal developmental anomalies, no difference between the two types of lesions was found. We also provide a potentially useful comprehensive review of other genetic syndromes in which ductal plate malformations may occur. Received May 14, 1999; accepted October 11, 1999.     

Liver abscesses in children: a single center experience in the developed world

OBJECTIVES: The aim of this study was to investigate the clinical and radiologic features, predisposing risk factors, and complications of children with pyogenic liver abscess (PLA) referred to a tertiary pediatric hepatology center. METHODS: We analyzed our database of all children referred to our unit over a 10 year period and performed a case note review of all patients with a radiologically proven PLA. RESULTS: PLA was diagnosed in 15 children (7 boys), 0.5% of all referrals. They presented at a median age of 10 years (range 2 months-15 years). In three children (2 boys), PLA was the first manifestation of chronic granulomatous disease. Among the others, five had radiologic evidence of other intra-abdominal pathology (1 with subsequently proven appendicitis), and four developed portal vein thrombosis with portal hypertension. The commonest isolated pathogen was Staphylococcus aureus. Combined treatment with guided aspiration and prolonged intravenous antibiotics was successful in all patients. CONCLUSION: PLA is a rare diagnosis in children in the developed world. It may be caused by primary neutrophil disorders even in the absence of a previous history of infection. Co-existent appendicitis, intra-abdominal sepsis, and ascending pylephlebitis must be sought because these children are at risk of developing portal vein obstruction and portal hypertension. Prolonged intravenous antibiotic treatment guided by microbiologic sensitivities is highly effective.     

Congenital absence of the portal vein associated with congenital hepatic fibrosis

The radiological features of a 7-year-old boy with congenital absence of the portal vein, pathologically proven congenital hepatic fibrosis, double inferior vena cava, ventricular septal defect, vertebral anomalies, crossed fused renal ectopia, and facial anomalies with pathological correlation are reported. This association between congenital absence of the portal vein and congenital hepatic fibrosis is unique.     

Nonsystemic treatment of metastatic tumors of the liver—a review

Hepatic metastasis is usually quite resistant to conventional systemic chemotherapy. Nonsystemic treatment of metastatic tumors of the liver include surgical resection; infusion of chemotherapeutic agent(s), either via hepatic artery or portal vein; ligation of hepatic artery; radiotherapy; and other more investigative approaches (isolation perfusion, cryosurgery, liver transplantation, etc). The relative applicability, results, and limitations of each of these therapeutic modalities are reviewed.     

Hepatoblastoma with congenital absence of the portal vein - a case report.

A 17-month-old girl who had been followed up as an extremely-low-birth-weight infant presented with hepatoblastoma in the right lobe of her liver. Preoperative angiography revealed an absence of the portal vein, and the visceral venous return was through the left renal vein into the inferior vena cava. No liver dysfunction and no jaundice were found; however, a marked elevation of the alpha-fetoprotein level was noted. She underwent a typical right hepatic lobectomy successfully after chemotherapy and has no evidence of recurrence 6 months after surgery.     

Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation

Osorio MJ, Bonow A, Bond GJ, Rivera MR, Vaughan KG, Shah A, Shneider BL. Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation.
Pediatr Transplantation 2011: 15: E149–E151. © 2010 John Wiley & Sons A/S. Abstract: A seven‐yr‐old boy presented with persistent oxygen requirement following a respiratory infection. Physical exam was remarkable for orthodeoxia and digital clubbing. Laboratory evaluation showed elevated A‐a oxygen gradient of 48 mmHg and mildly elevated transaminases. Sonography showed a 13 cm multilobulated liver mass and a biopsy revealed histological findings consistent with focal nodular hyperplasia. MAA scan revealed 23% right to left shunting. Abdominal CTA and MRV demonstrated the absence of the intrahepatic portal vein with an extrahepatic portocaval shunt. Abernethy malformation is a rare anomalous intra‐ or extrahepatic communication between portal blood flow and systemic venous return. In rare cases, Abernethy malformation results in HPS. Ours is the sixth case report to describe the co‐existence of these two entities. Surgical correction of anomalous hepatic vasculature or liver transplant is imperative to restoration of lung function and also to prevent progression of possible malignant liver tumors. We describe the second patient with Abernethy and HPS who underwent liver transplant with complete resolution of HPS.