Urgent distal splenorenal shunt in low-body weight patients

The authors report two patients with extrahepatic portal hypertension and repeated massive␣bleeding from esophageal varices who underwent urgent distal splenorenal shunting (DSRS) after having proved refractory to medical treatment, endoscopic sclerotherapy, and ligation of esophageal varices. Their ages and weights were 18 months/10 kg, and 11 months/6.4 kg, respectively. The splenic veins were 6 and 4 mm in diameter. During follow-up of 4 and 3 years, respectively, the shunts have remained patent in both patients as demonstrated by Doppler Ultrasound. Gastrointestinal hemorrhage has not recurred, splenomegaly regressed, and platelet and white blood cell counts increased gradually. Nither patient developed a significant encephalopathy, and liver function tests showed no significant changes throughout the observed period. The authors considered the shunting feasible despite the relatively narrow splenic veins. In both patients the anatomic position of the splenic vein – more caudal to the posterior wall of the pancreas – facilitated its isolation. In the smaller infant, the inferior mesenteric vein was sutured to gain a satisfactory segment of splenic vein. The successful use of a DSRS to control actively bleeding varices in a child weighing 6.4 kg has not been previously reported. Accepted: 16 February 2001     

Serum hyaluronan as a marker reflecting the severity of cirrhosis and portal hypertension in postoperative biliary atresia

The majority of patients with biliary atresia (BA) develop progressive liver disease and serious complications of portal hypertension, including esophageal varices and ascites, despite successful Kasai operation. The aim of this study was to evaluate whether serum hyaluronan (HA) reflects the severity of cirrhosis and its complications in patients with postoperative BA. Fifty-four postoperative BA patients aged 1 to 19 years were recruited into this study. Serum HA was determined by ELISA-based assay, and serum HA levels were compared among groups of patients according to Childs classification as well as the presence of esophageal varices and ascites. Mean serum HA concentration was significantly increased in patients with Child C (534.35±573.02 ng/ml) in comparison to patients with Child B (97.67±104.22 ng/ml, p=0.002) and Child A (36.27±71.41 ng/ml, p<0.001). Mean serum HA concentration was also significantly higher in patients who had ascites or esophageal varices than in those who did not suffer from these complications. Furthermore, patients who had variceal hemorrhage had significantly higher mean serum HA levels than those without bleeding. Thus, serum HA levels may reflect the severity of cirrhosis and its complications, and may be useful in monitoring the clinical outcomes of patients with postoperative BA.     

Clinical significance of plasma endothelin levels in patients with biliary atresia

Biliary atresia (BA) is the end-result of a destructive inflammatory process that affects intra- and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tracts with the development of biliary cirrhosis and portal hypertension (PH). Endothelins (ET) are 21-amino-acid peptides of endothelial origin with potent vasoconstrictor activity that bind to various cells of the liver. Nothing is presently known about plasma ET levels in BA. The aim of this study was to determine the clinical significance of plasma ET levels in patients with BA after hepatic portoenterostomy (Kasai's procedure) and to correlate these with liver function tests (LFT) and PH. We measured plasma concentrations of ET in 19 patients with BA (5 boys and 14 girls; mean age 11.6 ± 5.5 years) after portoenterostomy and 10 age-matched controls. Patients were grouped according to outcome based on LFT: group A consisted of 9 patients with an ‘‘unfavorable outcome” and Group B 10 patients with a “favorable outcome”. The plasma ET levels were measured using a highly sensitive and specific enzyme immunometeric assay (EIA). No patient had ascites or hepatorenal syndrome. Plasma ET levels were significantly higher in patients with BA than in controls (3.42 ± 0.42 vs 1.75 ± 0.39 pg/ml, respectively; P < 0.01) and in patients in group A than in group B. (3.75 ± 0.25 vs 3.06 ± 0.23 pg/ml, respectively; P < 0.01). In group A, plasma ET levels were higher in patients with PH (n = 4) than in those without PH (n = 5) (3.99 ± 0.06 vs 3.64 ± 0.22 pg/ml, respectively; P < 0.05). We conclude that plasma ET levels are high in patients with BA, especially those with severe biliary cirrhosis, and that ET may partially contribute to development of PH in BA. The results of the present study also suggest that plasma ET concentrations may be a useful marker in the follow-up of patients with BA. Accepted: 12 September 1997     

Magnetic resonance angiography versus endoscopy for the assessment of gastroesophageal varices in biliary atresia

This study was undertaken to compare magnetic resonance angiography (MRA) and gastrointestinal tract endoscopy (ENDO) for detecting varices in postoperative biliary atresia (BA) patients. Thirty-four BA patients were divided into groups according to age and liver function; group 1: 1–4 years old; group 2: 5–9 years old; group 3: over 10 years old; group A: normal liver function; and group B: moderate liver dysfunction. All subjects had MRA and ENDO. ENDO showed esophageal or gastric varices in 16 of 34 subjects. MRA depicted gastroesophageal vessels in 12 of 16 cases with varices on ENDO (sensitivity: 75%). Two subjects appeared to have varices on MRA that could not be confirmed on ENDO. Sixteen subjects had no varices on ENDO and normal MRA (specificity: 88.9%). Sensitivities and specificities of MRA for detecting varices were 20 (1/5) and 100% (6/6) in group 1 (n = 11), 100 (6/6) and 66.7% (4/6) in group 2 (n = 12), 100 (5/5) and 100% (6/6) in group 3 (n = 11), 100 (8/8) and 100% (9/9) in group A (n = 17), 50 (4/8) and 77.8% (7/9) in group B (n = 17), respectively. From these findings, MRA was not accurate in BA patients less than 10 years old or with moderate liver dysfunction. Therefore, periodic endoscopic examination is recommended for the accurate assessment and follow-up of varices in postoperative BA patients.     

Custom-made covered transjugular intrahepatic portosystemic shunt (TIPS) in an infant with trisomy 22 and biliary atresia

We report an 8-month-old girl with portal hypertension secondary to biliary atresia. The decision to treat with TIPS was made at the age of 8 months due to recurrent variceal bleeding. The procedure was carried out with a 6-mm bare stent due to her small size. Radiological follow-up with Doppler US showed gradual stenosis and finally occlusion of the stent 80 days after implantation. Revision was performed with placement of an additional 6-mm expanded polytetrafluoroethylene (e-PTFE) stent-graft that had remained patent for 9 months, proving that in small children with a portal vein diameter less than 8 mm, the combination of a bare stent and stent-graft can provide excellent results.     

Helicobacter pylori infection in postoperative patients with biliary atresia A benign colonization?

The prevalence of Helicobacter pylori infection in postoperative patients with biliary atresia (BA) was investigated in relation to esophageal varices, portal-hypertensive gastropathy (PHG), and peptic ulcer disease (PUD) in 25 Japanese patients (10 boys and 15 girls) aged from 16 months to 20 years. Gastric biopsy specimens obtained during endoscopy were used for both the rapid urease test and modified Giemsa staining. The patients were classified into three groups according to liver function: 15 in group A (total bilirubin [TB] < 1.0 mg/dl), 7 in group B (1.0 ≤ TB < 2.0 mg/dl), and 3 in group C (TB ≥ 2.0 mg/dl). Esophageal varices were found in 19 patients (60% of group A and all patients in groups B and C) and PHG in 3 group B patients. However, no gastric or duodenal ulcers were found in any case. Only 2 patients (8%) had H. pylori colonization of the gastric mucosa. Both, however, belonged to group A, and the degree of chronic neutrophilic infiltration of the mucosal layer was as mild as that of the other patients. The prevalence of H. pylori infection and PUD in postoperative patients with BA was quite low, and a pathological relationship with the severity of liver disease could not be determined in these patients. Accepted: 2 December 1998     

Portal hypertensive enteropathy in biliary atresia

This paper describes a rare case of biliary atresia (BA) in which massive postoperative bleeding developed due to portal hypertensive enteropathy. A 15-year-old boy had been doing well after Kasai's operation for BA. At around the age of 6 years he developed recurrent episodes of esophageal variceal bleeding and underwent esophageal transection, splenectomy, sclerotherapy, and embolization of the left gastric vein. At the age of 15 years he suddenly developed abdominal pain, hematemesis, and massive tarry stools. His hemoglobin level was 6.3 g/dl. Endoscopy showed several small, polypoid lesions in the jejunum beyond Treitz' ligament. The source of the bleeding was one of these lesions located in the proximal jejunojejunostomy of the Roux-en-Y loop. Because he had repeated episodes of melena, he underwent partial resection of the jejunum under endoscopic guidance. He has since been free of gastrointestinal (GI) bleeding. From this experience, we conclude that the polypoid lesions of an enteropathy may be a cause of massive GI bleeding in the postoperative portal hypertension of BA. Accepted: 17 April 1997     

Changes in portal vein hemodynamics after hepatic portoenterostomy in biliary atresia

Portal vein (PV) shrinkage sometimes eliminates the possibility of liver transplantation in biliary atresia patients after hepatic portoenterostomy. To determine the factors leading to PV shrinkage, we performed a serial sonographic study of the portal venous system in 21 children. Cross-sectional PV area and mean portal venous velocity (PVV) were reduced in patients with refractory cholangitis and those with gastroesophageal varices and cholangitis. Although the reduction in cross-sectional PV area was greater in patients with four-time laparotomy than single laparotomy, the mean PVV was not reduced by repeated laparotomy. Patients with varices were lower in age, weight, mean PVV, cross-sectional PV area for age, and had higher serum total bilirubin levels. In conclusion, refractory cholangitis is a significant factor in shrinking the PV. With active bile drainage, varices spontaneously regress, the PV increases in both caliber and total length per unit hepatic volume, and PVV normalizes. It is suggested that pulsed Doppler PV sonography can help to determine the optimal time for liver transplantation referral in biliary atresia patients with progressive cirrhosis.This work was supported by a Grant for Pediatric Research (63-A-05) from the Ministry of Health and Welfare. Offprint requests to: K. Tanaka     

Long-term outlook in biliary atresia

The oldest survivors from the Kasai portoenterostomy originate in Sendai, Japan and are approaching their 60th birthday. These represent the tip of an expanding cohort of adults born with this previously fatal condition. Increasingly transition to adult-biased hepatologists and physicians will be the expectation of many with this condition. However unlike their usual patients with alcohol, drugs, virally mediated liver disease these are different with different expectations of health and quality of life. Cure is not on the cards for most of these and they survive still with impaired bile flow and increased liver fibrosis and cirrhosis with the threat of cholangitis and portal hypertension still apparent.We review the reported statistics on long-term survival essentially from Japan and Western Europe (such as the UK and France) and the range of complications that may still beset this group.     

肠系膜上静脉门静脉左支分流术治疗肝外门脉高压

目的 探讨肠系膜上静脉门静脉左支分流术治疗肝外门静脉梗阻的效果.方法 2008年10月至2010年2月对12例肝外门脉梗阻并门脉高压患儿实施肠系膜上静脉门静脉左支分流术(mesenteric-to-left portal vein bypass,MLPVB;又称Rex Shunt,RS手术).2例因门静脉左支闭锁,无法吻合,实施远端脾肾分流术(Warren手术).RS手术患儿年龄1.6～12岁,平均(5.6±3.3)岁.男8例,女2例.7例患儿有反复呕血、黑便史,输血史,输血量800～2400 ml;另外3例患儿表现巨脾、脾功能亢进、进行性贫血.8例患儿术前红细胞、白细胞、血小板降低;2例AST轻度升高,1例TBIL轻度升高,其他患儿各项指标正常;无脑病表现.术前影像学检查提示:10例表现食道胃底静脉曲张,门脉海绵样变;脾脏增大,脾脏平均长径(n=8)(12.4±0.8)cm,厚度(5.1±0.57)cm.手术采用上腹肋缘下横切口,测定肠系膜上静脉压力,并行门脉造影.分离矢状部肝组织,游离肝门静脉左支,穿刺测压并造影,确认门静脉左支通畅,阻断钳阻断并纵行劈开矢状部.根据门脉造影及探查情况选择游离胃冠状静脉、或脾静脉或移植一段合适的回肠静脉.然后以6-0 Prolene线将胃冠状静脉、脾静脉与门静脉左支行端侧吻合,移植血管一端与门静脉左支矢状部吻合另一端与门静脉远端吻合.再次测定肠系膜上静脉压力,并行血管造影.术后随访4～20个月.结果 10例患儿均成功实施手术,平均手术时间(220±14.7)min,出血约10～50 ml,分流完成后造影显示分流血管均通畅无狭窄.分流前门脉平均压力(38.3±7.2)cm H2O,分流后降为平均(27.2±5.3)cm H2O,(P=0.001).平均住院时间(10±1.9)d.随访期间无患儿再出现呕血,影像学检查分流血管通畅,肝功能各项指标正常;血小板、红细胞血红蛋白、白细胞正常;脾脏较术前缩小,脾脏平均长径(n=8)(10.2±0.5)cm,较术前缩小(P=0.00),平均厚度(3.6±0.2)cm,较术前缩小(P=0.000),门静脉左支直径增大.结论 Rex分流术治疗门静脉海绵样变性安全、可行、效果良好.     

Control of bleeding in extrahepatic portal hypertension — the reverse splenorenal shunt and portal-azygos disconnection

Abstract Variceal bleeding in children with extrahepatic portal hypertension is best controlled by an effective decompressive shunt. Both the standard and central splenorenal shunts require splenectomy which is undesirable in childhood, and alternative shunts such as the mesocaval shunt and the distal splenorenal shunt are also technically difficult with a similar risk of shunt thrombosis.
The reverse splenorenal shunt first described in 1962 by Erlik uses the proximal portion of the left renal vein which was initially anastomosed to the portal vein. It can be anastomosed to any of the major tributaries of the portal system. In the technique described, the proximal end of the renal vein is joined to the side of the splenic vein in continuity. It is preferable for this to be done as an elective planned procedure. Massive continuing haemorrhage which is not responding to conservative management can be controlled by portal-azygos disconnection with gastric transection. This procedure will control major variceal haemorrhage for several years and allow a decompressive shunt to be performed under optimal conditions and after appropriate preoperative investigations.     

Ultrasonographic assessment of the gallbladder in 21 children with portal vein thrombosis

Background: Portal vein thrombosis (PVT) is one of the most frequent causes of portal hypertension (PH) during childhood. Portal systemic collateral vessels occur at several locations, including the gallbladder (GB). Objective: To evaluate the GB in patients with PVT using US to assess GB wall thickness and its function, and the incidence of lithiasis and varices. Materials and methods: A prospective study was done on 21 children and young adults whose ages ranged from 17 months to 20 years and 10 months (mean age: 11 years and 7 months). A control group was matched for age and sex. All of the patients and controls fasted for at least 6 h prior to the US examination. The GB measurements included anterior wall thickness. These measurements were obtained before the ingestion and then 30 and 60 min after the ingestion of a meal containing at least 25 g of fat. The rate of GB contractility was calculated based on these results. The presence of varices in the GB wall was detected by the characteristic serpentine shape of the intramural vessels and by the venous flow using pulse duplex and color Doppler imaging. The presence of biliary lithiasis was confirmed by shadowing. The chi-square test, the exact Fisher test and the Mann–Whitney test were used to compare the results. Results: Biliary lithiasis occurred in 3 (14.2%) of the 21 patients. The GB wall was thickened in 13 (61.9%) of the 21 patients, which corresponded with the number of patients with GB varices. The wall dimensions of all the controls were within normal limits. In patients with PVT; GB contractility was lower than in the 21 patients used as control and resulted in a significant difference in all of the measurements. Conclusion: GB varices are very common in children with PVT, and it is noted especially in patients whose GB wall was thickened and in whom the GB contractility was reduced. Lithiasis could be a consequence of the decreased contractility of GB.     

Acute intrahepatic portal vein thrombosis complicating cholangitis in biliary atresia

A 2-year-old boy with a Kasai portoenterostomy had systemic features suggestive of cholangitis and acute portal vein obstruction. The rapid rise in portal pressure caused a transient, severe decompensation of hepatic function. A superior mesenteric arterial angiogram confirmed the presence of diffuse intrahepatic portal vein thrombosis. Conclusion This case provides clinical and radiological evidence supporting an association between ascending cholangitis and acute intrahepatic portal vein thrombosis. Received: 7 May 1996 / Accepted: 3 September 1996     

Surgical intervention in children with portal hypertension

Over a 5-year period 252 children with bleeding oesophageal varices secondary to portal hypertension were treated by endoscopic injection scletherapy. Surgical treatment was restricted to patients with bleeding complications following sclerotherapy and the occasional problem of symptomatic splenomegaly (2 children). Sclerotherapy failure was due to the development of varices not accessible to sclerotherapy (gastric fundus 7, duodenum 1, jejunum 1, unidentified site in small bowel 1) and to continued bleeding from oesophageal ulcers in 2 children. There were 5 children with Child's grade A cirrhosis. Fifteen children (4.4 % of the total; median age 8.7 years, range 1.5 – 18 years) underwent 16 operations during this period, which included 13 porto-systemic shunts (8 lieno-renal and 5 meso-caval) and 3 oesophageal transections/devascularisations. Sclerotherapy was not attempted in 1 of the 2 children treated for continued bleeding from oesophageal varices. Two lieno-renal shunts thrombosed and 1 of these patients underwent a meso-caval shunt. There were no deaths in this group of patients during a median follow-up of 40 months. One child with biliary atresia has developed a mild intermittent encephalopathy. The use of injection sclerotherapy for bleeding oesophageal varices with surgical intervention limited to patients failing treatment resulted in low morbidity and no mortality in a large group of children with portal hypertension. Correspondence to: N. D. Heaton     

The relationship between procollagen-III-peptide and the severity of esophageal varices in children with biliary atresia after Kasai operation

Biliary atresia (BA) is a common cause of infantile cholestasis. Disease progression leads to intra hepaticfibrosis, and thus to the development of PH and EV. Our objective has been to study the relationship between procollagen-III-peptide (PIIIP) and the severity of EV in children with BA after Kasai operation. Children below 15 years of age (n=29) with BA after a Kasai operation were evaluated for EV by endoscopy. Healthy (n=26) children of the same age and sex distribution who participated in the hepatitis B vaccination program served as the controls. Serum PIIIP was determined by radioimmunoassay. The BA patients were classified on the basis of severity of EV (Paquet's classification) into three groups: group 1 (n=15) had grade 0, group 2 (n=8) grade 1–2, and group 3 (n=6) grade 3–4 EV. In group 3, serum PIIIP (2.9 ± 1.3 IU/ml) was significantly higher than in group 2 (1.5 ± 0.4 IU/ml) (P < 0.05). Serum PIIIP levels were increased in group 2 compared with group 1 (1.2 ± 0.4 IU/ml) and in group 1 compared with the control group (1.2 ± 0.2 IU/ml), but this difference was not significant. PIIIP levels increased with severity of the EV in the BA patients. Hence, high PIIIP levels may serve as a non invastive indicator of EV developing in postoperative BA patients. Accepted: 8 January 2001     

Extrahepatic biliary atresia associated with trisomy 18

A case of extrahepatic biliary atresia (EBA) associated with trisomy 18 is presented. A 1-month-old boy was suspected to have Alagille syndrome with obstructive jaundice, a systolic heart murmur, growth retardation, and a small, pointed chin. However, surgery and chromosomal analysis revealed EBA associated with trisomy 18. Chromosomal examination must be performed in patients with jaundice and congenital anomalies. It is possible that EBA in trisomy 18 syndrome is due to a chromosomal disorder. Accepted: 20 April 1998     

肠门分流（Rex手术）治疗肝外型门静脉高压的初步研究

目的探讨肠系膜上静脉门静脉左支分流术（Rex手术）治疗门静脉海绵样变的临床效果：方法2008年10月至2009年9月本院共收治6例门静脉海绵样变性并门脉高压患儿。年龄20～82个月,均为男性。4例有反复上消化道出血、呕血、输血史,输血量800～2400mL;2例表现为巨睥、脾功能亢进、进行性贫血。5例患儿术前红细胞、白细胞、血小板不同程度降低;肝功能各项指标均正常。术前影像学检查提示：食管胃底静脉曲张,门脉海绵样变;脾脏增大。手术采用上腹肋缘下横切口,测定肠系膜上静脉压力,并行门静脉造影。游离胃冠状静脉达食管裂孔水平,离断。游离肝门静脉左主支,阻断并纵行劈开矢状部,然后以6-0Prolene线将胃冠状静脉与门静脉左支行端侧吻合。再次测定肠系膜上静脉压力,并行血管造影。术后随访3～11个月。结果6例均成功实施手术,手术时间200．240min,出血约20～30mL,分流完成后造影显示分流血管均通畅无狭窄。分流前门静脉压力39～53cmH2O,分流后降为29～32cmH2O。术后住院时是7～12d：随访期间患儿未再出现呕血,影像学检查显示分流血管通畅,肝功能各项指标正常;血小板、红细胞、血红蛋白、白细胞正常;脾脏较术前缩小,厚度3．5～4．0cm。结论Rex分流术治疗门静脉海绵样变性安全、可行,效果良好。     

An ovarian dysgerminoma in a girl with biliary atresia

We present a case of an ovarian dysgerminoma in an 11-year-old girl who had had a hepatic porto-jejunostomy for biliary atresia (BA) when she was 1 month old. Although the tumor was successfully treated with surgery followed by radiotherapy, she died of hepatic failure 12 months after operation. The incidence of malignant disease in patients with BA is extremely low, and the etiologic correlation between the two diseases still remains unclear.     

胆管闭锁与十二指肠乳头异位的关系探讨

目的 胆管闭锁的病因目前尚不清楚,本研究对胰胆的解剖进行造影研究.方法 对221 例胆管闭锁患儿中 47 例(21.3%)进行胆管造影,根据胆囊管和胆总管及十二指肠显影清晰影像进行研究,观察胆总管、胰管、胰胆合流共同管及其开口部位.以同期收治的 29 例非胆管闭锁性黄疸婴儿的胆管造影结果 为对照.结果 对照组29例中7例(24.1%)十二指肠乳头开口于十二指肠降段以远,而胆管闭锁组 47 例中 24 例(51.1%)十二指肠乳头开口于十二指肠降段以远,后者较前者明显增高(P<0.05).胆管闭锁组和对照组合并胰胆合流异常的发生率分别为95.7%(45/47)和82.8%(24/29),二者差异无显著性意义(P>0.122).胆管闭锁患儿胰胆合流共同管的平均长度指数为0.418±0.152,比对照组(0.236±0.122)明显延长,P<0.05.胆管闭锁组胆总管的平均直径指数为0.078±0.043,对照组为0.199±0.086,前者较后者明显细小,P<0.05,而共同管和胰管的直径差异不显著(P>0.05).结论 十二指肠乳头远端开口异位和胰胆合流共同管延长是胆管闭锁影像学改变特点,提示此畸形的病因与胚胎早期肝憩室发育异常有关.     

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.