Pulmonary Hypertension with Coexisting Portal Hypertension

A 10-year-old boy who had portal hypertension secondary to portal fibrosis/intrahepatic biliary atresia developed syncopal episodes related to strenuous activity. A work-up excluded a metabolic or neurologic etiology and cardiac catheterization demonstrated significant pulmonary hypertension. Six months later he died and an autopsy revealed pulmonary plexogenic arteriopathy without microemboli. Previous reported cases of this symptom complex are reviewed. In addition, he had a history of nonspecific colitis, ulcerative stomatitis, and conjunctivitis responsive to steroid therapy. The possible relationship of these manifestations to the portal pulmonary pathology is discussed.     

Pulmonary Hypertension Associated with Portal Hypertension in a Child with Williams Syndrome—a case Report

A 14-year-old white female with Williams syndrome and portal hypertension presented in shock; at autopsy she was found to have grade II to VI vascular changes of pulmonary hypertension. This case demonstrates the association of portal hypertension and pulmonary hypertension in a pediatric patient.     

小儿肝前型门脉高压症治疗方式的探讨

为了对小儿肝前型门脉高压症不同的治疗方法进行评价，寻找比较合理的治疗方案，对本院２０年来收治的２７例小儿肝前型门脉高压症患儿的资料进行回顾性分析和随访。其中行断流术者１４例，分流术８例，单纯切脾和大网膜包肝包肾术５例。随访１６例，５例有再出血史。随年龄增长出血逐渐停止，全组无死亡。结论：小儿肝前型门脉高压症主要是由于门静脉主干或脾静脉梗阻、栓塞而致门静脉压力增高，而肝脏功能、结构均正常，预后好。自然侧支循环形成为本症最好的结果。因此提倡做能促进侧支循环建立的手术，如大网膜包肝包肾、脾肺固定术。断流术主要用于救治大出血者及反复多次出血者。分流术及脾切除的目的在于降低门脉系统压力避免静脉曲张，并能刺激自然侧支分流。所以分流术以远端分流较好，并只主张行巨脾切除。     

Long-term follow-up of portal hypertension after liver transplantation in children

Abstract:  We aimed to describe the long-term changes in the imaging and clinical features of PHALT in children. A retrospective review was undertaken of consecutive children undergoing their first liver transplant between 1993 and 2003. Details of clinical progress and ultrasound imaging were recorded at one-yr post-transplantation and at last follow-up. Data were extracted on 83 children (median age at transplant 1.7 yr, range one month to 17.5 yr, 44 girls) who underwent 89 transplants. Four of these children died at a mean 5.6 yr (range 3.8–6.9 yr) after transplantation. Of the survivors, follow-up at one yr (n = 83) and at last follow-up (n = 71, median 4.3 yr post-transplant) revealed imaging evidence of splenomegaly in 46% and 44%, ascites in 6% and 4%, and portal systemic collaterals in 12% and 14%, respectively. Gastrointestinal hemorrhage associated with portal hypertension had occurred in no children at one yr and in four (6%) at latest follow-up. Features of portal hypertension on ultrasound scan are common in children before liver transplantation. An important minority of children will suffer clinically significant complications of PHALT during long-term follow-up, caused by both vascular and parenchymal disease.     

不同术式对肝前型门脉高压症门脉压力影响的实验研究

目的 为了对肝前型门脉高压症的术式选择提供依据。方法 应用缩窄门静脉方法进行动物（犬）的肝前型门脉高压症的模型制作。将已形成肝前型门脉高压症犬１８只分为４组,分别进行脾切除术、脾切除＋贲门周转血管离断术、肠腔分流术及联合手术。手术前后测定门脉压力进行对比。结果 与术前相比,脾切除有一定降低门脉压的作用（Ｐ〈０．０５）;肠腔分流术降压效果最佳（Ｐ〈０．００１）;脾切除＋离断术无明显降压作用;联合手术     

Effectiveness of Rex shunt in children with portal hypertension following liver transplantation or with primary portal hypertension

Abstract:  Portal vein thrombosis can occur as a result of primary anomalies, after liver transplantation, and for other reasons. It may result in severe complications secondary to portal hypertension, such as bleeding from esophageal or gastric varices, hypersplenism, or impaired somatic growth. In this retrospective study, we analyzed the outcome of 25 children who underwent a Rex shunt procedure. The following venous grafts were used as the shunt: the autologous internal or external jugular vein (n = 17) or a cryopreserved graft (n = 5); in three patients the umbilical vein was recanalized. The median follow up time was 109 months (range 18 days–146 months). The best results were achieved in patients in whom an autologous jugular vein segment was used as a vascular graft for the Rex shunt (shunt patency of 88%). In patients with a functioning shunt no further lower or upper gastrointestinal bleeding occurred. And in the entire study population hypersplenism syndrome improved after surgery. In our large cohort of pediatric patients, the Rex shunt has shown to be an effective method to eliminate portal hypertension and to revascularize the liver and thereby prevents the possible consequences of long-term portosystemic shunting.     

Portal flow modulation in auxiliary partial orthotopic liver transplantation

APOLT is a suitable technique of liver transplantation in patients with ALF and some types of MLD. Portal venous steal is a problem with this procedure that leads to graft dysfunction and failure. Modulation of the portal flow to the graft and native liver can help in preventing this problem. We discuss the pathophysiology of this complication, review available literature regarding its management, and describe our results using the technique of graded hemiportal banding to achieve adequate perfusion for the graft and native liver.     

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.     

Determinants of large drain losses early after pediatric liver transplantation

The goal of this study was to evaluate postoperative ascites to correlate it with graft dysfunction and other complications. We therefore reviewed the files of patients transplanted between 2009 and 2014 to correlate drain losses with indication, patient and organ size, PELD, graft type, GRWR, NRBW, NGWD, cold ischemia time, histologically proven graft dysfunction, and surgical complications. Of 120 LTs in 104 patients, 48 (40%) were complicated by graft dysfunction, 43 (36%) by surgical complications, and 25 (21%) by cellular rejection. Large drain losses correlated with younger age (P=.05), graft dysfunction (P<.01), surgical complications (P<.01), chylous ascites (P=.05); there was no association with PELD, GRWR, NRBW, or NGWD. Graft dysfunction was predicted by >20 mL/kg/d of ascites at age 0‐2 years (AUROC 0.671), and >10 mL/kg/d above 2 years (AUROC 0.710). The measurement of drain losses after pediatric LT could be used as a non‐invasive marker of graft dysfunction. Younger recipients tend to develop larger amounts of ascites, and its persistence is associated with early complications.     

儿童门脉高压25例病因分析

目的探讨儿童门脉高压病因、分类及临床特点。方法总结25例确诊病例临床资料。结果门静脉海绵样变性是肝前性门脉高压的主要病因;特发性门脉高压所占比例较高;肝硬化门脉高压并不少见,病因包括感染性肝炎、遗传代谢病、胆道梗阻及原因不明者。结论儿童门脉高压症确诊并不困难,更重要的是明确病因,这与疾病的预后密切相关,需要不断从病原学、影像学、组织病理学等方面提高诊断水平,早期诊断、早期治疗,以提高疗效。     

Management of late‐onset portal vein complications in pediatric living‐donor liver transplantation

The purpose of this study was to evaluate retrospectively the results of PTA for late‐onset PV complications after pediatric LDLT and to assess whether a meso‐Rex shunt is a viable option for treating restenosis of the PV after PTA in selected cases. Seventy‐five children who underwent adult‐to‐child LDLT were included in this study, and there were six late‐onset PV complications (8.0%). The initial therapeutic approach was PTA, with or without stent: PTA with balloon dilation for three children, PTA with stent placement for one child, and failure to cannulate the occluded PV for two children. A meso‐Rex shunt was performed in the two children after failed PTA: One suffered complete obstruction of the main PV, and the other, restenosis with total thrombosis after PTA with stent. The PTA was a technical and clinical success in four with PV stenosis of the six patients (66.7%), and successful application of a meso‐Rex shunt in the other two children resulted in restoration of PV flow. In conclusion, PTA is a safe and effective procedure for treating late‐onset PV stenosis after pediatric LDLT. However, in growing pediatric recipients with restenosis of the PV after PTA or chronic PV thrombosis, a meso‐Rex shunt may be a better choice for late‐onset PV complications.     

Decreased portal vein flow during Kawasaki disease in a liver transplant patient

Abnormalities of liver function tests are frequently documented in patients with Kawasaki disease, but the mechanism responsible for this has not yet been established. Described herein is the case of a 1‐year‐10‐month‐old girl who underwent liver transplantation at 11 months of age. Eleven months after transplantation the patient was diagnosed with Kawasaki disease, which was associated with some portal flow reduction, and received i.v. immunoglobulin, after which fever abated with improvement of portal flow to its pre‐fever level. Abnormalities of liver function tests in Kawasaki disease patients may occur as a result of inflammation of both the biliary and portal systems. There are no reports on the potential relationship between Kawasaki disease and the portal vein, and accumulation of further data is necessary to better examine this relationship.     

波生坦治疗儿童肺动脉高压的研究进展

波生坦是一种非选择性的内皮素受体拮抗剂,在儿童肺动脉高压的治疗中具有重要作用。波生坦能改善肺动脉高压儿童的运动耐量、降低肺血管阻力、改善心功能,且不良反应较成人更少。与其他药物联用能改善患儿的生活质量。波生坦的远期效果、最佳治疗时程及药物联合应用仍有待于进一步研究。     

Portal vein reconstruction using vein grafts in pediatric living donor liver transplantation: Current status

PV reconstruction is an important aspect of LDLT, with post‐transplant outcomes depending on PV reconstruction methods. However, it is unclear whether the preferential selection of these techniques is dependent on preoperative recipient characteristics. This retrospective study assessed whether preoperative recipient factors differed in pediatric patients who did and did not receive VGs for PV reconstruction. Of 113 pediatric patients who underwent LDLT from January 2010 to July 2015, 31 (27%) underwent PV reconstruction with VGs and the other 82 (73%) without VGs. The presence of collateral vessels (P<.0001) and ascites (P=.02); PV size (P<.001), thrombosis (P=.01) and the direction of flow (P=.01), Child‐Pugh class A vs B/C liver function (P=.01), Alb concentration (P=.02), primary diagnosis: BA vs non‐BA (P=.03), and previous abdominal surgery (P<.005) differed significantly in patients who did and did not receive VGs for PV reconstruction. PV complications, patient survival, and graft survival did not differ significantly in patients with and without VGs at 1‐year follow‐up. VGs should be harvested for recipients with pretransplant hypoplastic PV, intense collaterals, hepatofugal flow, poor liver status, or previous abdominal surgery.     

Impact of liver transplantation on rate-corrected QT interval and myocardial function in children with chronic liver disease*

Prolonged QTc interval (>440 ms) is a common abnormality in adult patients with CLD and has been reported to predict patient survival. In this study, 88 children who underwent evaluation for LT, including a 12-lead electrocardiogram and echocardiogram included to determine the frequency of QTc prolongation and related factors in children with CLD and the effect of LT on these factors. Sixty-nine healthy, age- and sex-matched children served as controls. QTc interval was prolonged in 40 CLD patients (45.4%). It was found to be related to PELD score and presence of portal hypertension. Mean QTc was higher in patients who died prior to LT than in the survivors without LT. Mortality risk was increased 3.66-fold in patients with prolonged QTc (p = 0.001, 95% CI: 2-7.2). Cox regression analysis showed that only PELD score was an independent predictor of survival (p = 0.001, beta = -0.41, 95% CI: 5.58-1.82). Five of 48 transplanted children died within three months post-transplant; QTc was not related to post-transplant survival (p = 0.27). QTc normalized in 63.8% patients after LT. After LT, LAD, LVEF, and LVPWT decreased. In conclusion, QTc prolongation is common in children with CLD and associated with high mortality. It may be useful for assessment of the severity of CLD and for the timing for transplantation.     

Pathology of the Pediatric Regio Inguinalis: Mysteries of the Hernia Sac Exposed

Inguinal hernia repair is one of the most common surgeries performed on children. However, the value of routine histologic examination of hernia sac tissues continues to be debated. Although the surgical pathology of herniorrhaphy tissues is usually simple, occasional examples have unexpected findings that potentially lead to inappropriate management or that have added clinical implications. These along with surgical-quality assurance issues need to be considered in cost-benefit arguments. This article reviews basic histology, common potential pitfalls, and significant unexpected conditions encountered in the surgical pathology of the inguinal hernia sac in children. Received March 22, 2000; accepted June 30, 2000.