Occlusion of liver veins (Budd-Chiari syndrome) in childhood: a case report.

The Budd-Chiari syndrome is a rare cause of portal hypertension during childhood. We report on a 2-year-old boy suffering from liver congestion and ascites. No cause for the occlusion of the liver veins was found. There was an almost complete occlusion of the subdiaphragmatic vena cava due to compression by a hypertrophic lobus caudatus. Normal venous pressure could be demonstrated below this subtotal occlusion while numerous venous collaterals into the areas of the venae azygos and hemiazygos were encountered. A mesocaval shunt operation, therefore, seemed to be appropriate. There was, however, no decrease in production of ascites postoperatively. Repeat cavography now showed an elevation of venous pressure caused by the additional inflow of portal blood which could not, as expected, be compensated by caval collaterals. Therefore, a mesoatrial shunt was performed eight days after the first operation. However, even this additional shunt did not decrease the enormous production of ascites, and the child finally died. Hemodynamic and lymphodynamic pathways of the Budd-Chiari syndrome are discussed. Possibly a mesoatrial shunt in the first place, followed by a mesocaval shunt, would have been the better operative strategy.     

Living donor liver transplantation with alternative porto‐left gastric vein anastomosis in patients with post‐Kasai extrahepatic portal vein obstruction

EPVO is a common cause of prehepatic portal hypertension in pediatric patients and sometimes results in cavernous transformation of the PV. We herein present the cases of two patients who underwent LDLT for EPVO with post‐Kasai biliary atresia. PV reconstruction was performed with a porto‐left gastric vein anastomosis. The patient who underwent PV reconstruction using an interposition vein graft is doing well without surgical complications, whereas PV anastomotic stenosis was detected three months after LDLT in the patient who did not receive an interposition vein graft. The availability of vein grafts is limited in the LDLT setting. In such cases, performing PV reconstruction with varicose veins using interposition vein grafts is a feasible and valuable alternative option for obtaining a sufficient portal blood flow. Our experiences suggest that using interposition vein grafts may be appropriate for preventing the anastomotic stenosis caused by the fragility of varicose veins.     

Congenital absence of portal vein presenting as hepatopulmonary syndrome

A 9-year-old girl presented with cyanosis and decreased exercise tolerance. Chest X-ray, lung function and echocardiogram were normal. Contrasted echocardiogram showed intrapulmonary right to left shunt and computerized tomography of the thorax showed dilated pulmonary vasculature. There was no arteriovenous malformation. Computerized tomography of the abdomen revealed absence of the intrahepatic portion of the portal vein. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained into the right atrium. We concluded that the patient had hepatopulmonary syndrome secondary to absence of the portal vein. This is the first report of hepatopulmonary syndrome in a female paediatric patient with a congenital absence of the portal vein. As all portal blood entered directly into systemic circulation, the condition was equivalent to congenital portosystemic shunt. Cases of congenital portosystemic shunt complicated by hepatopulmonary syndrome were also reviewed.     

Ileo-caecal arterio-venous malformation associated with extrahepatic portal hypertension: a case report

This paper is a case report describing a boy with Down syndrome and a novel combination of multiple vascular anomalies: extrahepatic portal hypertension, an arterio-venous malformation (AVM) at the ileo-caecal junction, and caval/iliac vein anomalies and developing anal bleeding. We considered that the ileo-caecal AVM would be one of the causes of the repeated hematochezia. The patient underwent ileo-caecal resection with the AVM, and anastomosis of the left external iliac vein and the jejunal branch vein because of the stenosis of the superior mesenteric vein (Clatworthy mesocaval shunt). Intraoperative portal pressure measurement at the site of the right colic vein showed a moderate pressure reduction (42.5–31.5 cm H₂O). On the fourth month after operation, gastrointestinal fiberscopy showed no existence of esophageal varices. One year after operation, the patient was doing well without bleeding.     

Reno‐portal anastomosis as an approach to pediatric kidney transplantation in the setting of inferior vena cava thrombosis

In pediatric renal transplantation in the setting of IVC thrombosis, the retrohepatic IVC or gonadal veins are often used for outflow. However, if use of systemic venous outflow is unsuccessful, options become limited. We report the use of the portal vein for venous outflow in kidney retransplantation in the setting of IVC thrombosis. The patient is a 19‐month‐old male who developed end‐stage renal failure at seven months of age secondary to hypotension after spontaneous rupture of an accessory renal vein. The IVC was occluded during emergent laparotomy, and the patient developed extensive IVC thrombosis. The first two transplant attempts used the retrohepatic IVC for venous outflow. Despite good initial flow, in both instances the renal vein thrombosed on post‐operative day 1. In an unsuccessful salvage attempt of the second transplant, a reno‐portal anastomosis was performed. With few options for vascular access, a third transplant was attempted. The reno‐portal stump from the second transplant was used for outflow. The patient recovered well from his third transplant (creatinine 0.6 mg/dL 35 months post‐surgery), demonstrating that the portal vein can be used for outflow in cases of extensive IVC thrombosis.     

Therapeutic coil embolization of dominant shunt in hepatopulmonary syndrome enhances post‐liver transplant respiratory recovery

Coil embolization of the atypical enlarged pulmonary artery/arteriole with visible shunting may improve hypoxemia in patients with hepatopulmonary syndrome (HPS). When used selectively in cases with large shunts, either pre‐ or post‐liver transplantation (LT), it can aid an early recovery and reduce morbidity. We present a case where a large intrapulmonary shunt was embolized preoperatively to improve hypoxemia associated with HPS and enhance post‐operative recovery.     

Creation of a percutaneous mesocaval shunt to control variceal bleeding in a child

Although transjugular intrahepatic portosystemic shunt (TIPS) placement is the standard procedure for the treatment of portal hypertension, it is often impossible to perform in patients with extrahepatic portal vein occlusion. In these patients, options for decompressing the liver are few. In this report, we present a novel solution for managing gastro-esophageal hemorrhage in a child with portal hypertension and extrahepatic portal vein occlusion, through the creation of a percutaneous mesocaval shunt.     

门静脉主干-门静脉右支搭桥治疗门静脉海绵样变

目的 Rex手术(肠系膜上静脉与门静脉左支矢状部搭桥分流手术)是治疗肝外门静脉海绵变导致门静脉高压的最流行术式,但是有大约14％的患者合并有门静脉矢状部闭锁或发育不良不能行此分流手术,并且此手术后的失败率在4％～28％,这限制了这种符合生理性分流手术的应用.本研究的目的是探索向肝内门静脉右支分流建立恢复门静脉血流的新途径.方法 2015年11月至2016年6月,对4例门静脉海绵样变性行Rex分流手术后门脉高压复发患儿行门静脉右支分流手术.患几年龄3.5～8.9岁,平均6.1岁,分别于1.1～2.5年前在我院行Rex手术,其中采用扩张的胃冠状静脉与肝内门静脉矢状部搭桥3例,扩张的门静脉主干分支与肝内门静脉矢状部搭桥1例,术后再发出血和脾功能亢进,经保守治疗无效.术前影像学检查提示:食管胃底静脉曲张,门静脉海绵样变存在;原搭桥血管显示不清,肝内门静脉血流少;脾脏增大.手术采用原上腹肋缘下横切口,首先游离肝门的了解原搭桥血管的形态;然后将胆囊从胆囊床上游离;在右侧肝门的后方切开Glisson氏鞘,显露门静脉右支,将其游离足够的长度;然后切开肝十二指肠韧带,游离扩张门静脉主干足够的长度;根据肝内门静脉右支至肝外门静脉主干之间的距离,截取肠系膜下静脉作为搭桥血管:血管壁外观正常,直径≥5 mm;以7-0 Prolene线将间置静脉的近心端和远心端分别与门静脉右支和肝外门静脉的主干行端侧吻合.术后1个月、3个月及6个月定期随访.结果 4例患儿中3例门静脉右支发育好、通畅,直径5～6 mm,成功实施门静脉右支搭桥手术;另外1例门静脉右支纤细放弃搭桥.手术时间2.3 h(1.9～3.5 h),术中出血约50 ml(30～80 ml),无一例需要输血.取肠系膜下静脉间置3例;搭桥后门静脉的平均压力由33 cmH2 O(26～45 cmH2 O)下降至18.7 cmH2O(14～24 cmH2O).术中发现肝内门静脉右支与左支延续相通,而与肝外门静脉主干中断不通;Glisson鞘内沿着胆管周围大量增生的小静脉;原搭桥血管呈条索状,一致性狭窄或闭锁.术后随访6～18个月,无患儿再出现呕血,影像学检查分流血管增粗通畅,肝内门静脉增粗,门静脉海绵样改变消失,肝功能各项指标正常;血小板、红细胞血红蛋白、白细胞正常.结论 门静脉主干与肝内门静脉右支分流是治疗肝外型门静脉高压的有效新方法,适用于门静脉左支发育不良或Rex手术失败的病例,目前该方法尚未见文献报告.     

Portosystemic Shunt with Polysplenia and Hypoplastic Left Heart Syndrome

We report a unique case of portosystemic shunt with polysplenia and hypoplastic left heart syndrome. The abnormal vessel connected the right renal vein and the splenic vein. The shunt flow was from the systemic vein to the portal vein before the Fontan-type operation, but it reversed and ran from portal vein to systemic vein after the Fontan-type operation.     

Idiopathic hypereosinophilic syndrome in a case with ABO‐incompatible liver transplantation for biliary atresia complicated by portal vein thrombosis

Yamada Y, Hoshino K, Shimojima N, Shinoda M, Obara H, Kawachi S, Fuchimoto Y, Tanabe M, Kitagawa Y, Morikawa Y. Idiopathic hypereosinophilic syndrome in a case with ABO‐incompatible liver transplantation for biliary atresia complicated by portal vein thrombosis.
Pediatr Transplantation 2010: 14:e49–e53. © 2009 John Wiley & Sons A/S. Abstract: Idiopathic HES is characterlized by prolonged eosinophilia without an identifiable underlying cause and multiple‐organ dysfunction. We report a case of a LDLT for a 12‐yr‐old Japanese girl with BA accompanied by HES. Histological examination of the resected liver showed biliary cirrhosis with dense eosinophilic infiltration of portal tracts and the lobules of the liver. She developed portal vein thrombosis on post‐operative day 10 and the histopathological findings of the thrombus revealed dense eosinophilic deposition, suggesting that HES might have influenced the formation of this thrombus. Liver graft biopsies also demonstrated the presence of activated eosinophilils with biliary damage. Blood chemistry findings suggested liver dysfunction as a result of the eosinophilic infiltrations. Prednisolone treatment improved the liver dysfunction. Four years after LDLT, she remains clinically well on prednisolone at 0.3 mg/kg/day, with an eosinophil count ranging from 10 to 15%. A literature review has not shown any previous reports of HES with BA. This case demonstrates the possibility of an association between eosinophilic infiltration and liver dysfunction during follow‐up for BA and after LDLT.     

移植血管间置、门静脉主干-门静脉肝内左支分流术在治疗小儿肝外门静脉高压中的应用

目的 探讨移植血管间置、门静脉主干-肝内门静脉左支分流术在治疗小儿肝外门静脉高压中的效果和预后.方法 回顾性分析2010年1月至2014年12月9例(男6例,女3例)因“呕血、黑便”等上消化道出血表现入院,入院后完善腹部CT、超声及门静脉血管B型超声检查,诊断为门静脉海绵样变、门静脉高压和脾大患儿的临床资料.所有患儿均行移植血管间置、门静脉主干-肝内门静脉左支分流术治疗,术中造影发现胃冠状静脉直径较细或长度不够,不能行胃冠状静脉-门静脉左支分流术治疗,遂采用移植自体血管的方式,将其两端分别与门静脉主干和肝内门静脉左支吻合.其中采用移植空肠静脉方法治疗2例,回肠静脉4例,肠系膜下静脉3例.患儿术后均获随访,随访时间1～60个月,平均24.6个月.随访期间,采用超声评估脾大小及分流血管通畅情况.记录血常规、血生化和血氨,评估脾功能亢进情况和肝功能.结果 所有患儿均成功行移植血管间置、门静脉主干-门静脉肝内左支分流术治疗.手术时间105～360 min,平均218.3 min.其中3例住院期间有输血记录.术后住院时间6～10d,平均7.2d.术后复查B型超声示脾长径为9.6～14.7 cm,厚径为3.4～5.4 cm;较术前(长径10.4～17.6 cm,厚径3.2～6.8 cm)明显降低.血常规、生化和血氨均恢复正常.随访B型超声可见分流血管通畅,血管直径为0.5～0.7 cm,平均0.6 cm;分流血管入肝血流速度为0.14～0.18 m/s,平均0.16 m/s.1例患儿复发,采用保守治疗有效.结论 移植血管间置、门静脉主干-门静脉肝内左支分流术是治疗小儿肝外门静脉高压的有效方式之一,在患儿不能采用胃冠状静脉-门静脉左支分流术治疗时可考虑采用该方法治疗.     

Imaging the Rex vein preoperatively using wedged hepatic venous portography

Background  

Mesentericoportal bypass, the Rex shunt, restores the physiological hepatic portal flow and reduces the clinical sequelae of portal hypertension in children with extrahepatic portal vein obstruction (EHPVO). The preoperative evaluation includes an accurate assessment of the portal venous inflow and outflow. The former is readily assessed by ultrasound and MRI, while the outflow intrahepatic portal vein is harder to assess.     

Unusual shunt for symptomatic portal vein thrombosis after liver transplantation - Clatworthy revisited

PV thrombosis is not an uncommon occurrence following pediatric LT. Symptomatic PHT following PV thrombosis is treated medically, surgical portosystemic shunting (mesorex, splenorenal, and mesocaval) being reserved for refractory cases. A 10-yr-old boy suffered recurrent malena and hemorrhagic shock because of chronic PV thrombosis following LT nine yr ago (1999). Extensive work-up failed to localize the bleeding source. The liver function remained normal. Initial attempts at surgical shunts failed owing to thrombosis (mesocaval 2001, splenorenal, inferior mesenteric-left renal vein, splenic-left external iliac vein 2008). In this situation, we performed a Clatworthy shunt by anastomosing the divided lower end of the LCIV to the side of SMV. There was a single, large caliber anastomosis. Post-operatively, the malena stopped completely, and clinically, there was no lower limb edema or encephalopathy. Doppler USG revealed persistence of hepatopetal flow within the portal collaterals. Follow-up at two yr reveals stable hepatic function with a patent shunt. To the best of our knowledge, we are not aware of a Clatworthy shunt being performed in a transplant setting. We reviewed the literature pertaining to this shunt in non-transplant patients with PHT.     

Congenital portocaval shunt

Several congenital anomalies of the portal vein and inferior vena cava (IVC) have been reported; however, reports of a congenital communication between the portal vein and IVC are few. We report a patient who was found to have a natural shunt between the extrahepatic portal vein and the IVC that was discovered when she underwent a right hepatic lobectomy for an undifferentiated liver sarcoma. The patient also had agenesis of the right kidney. We have not been able to find a similar case reported in English literature.     

Portosystemic shunt as a cause of congenital hyperinsulinemic hypoglycemia

Congenital hyperinsulinemic hypoglycemia (CHH) is characterized by the inappropriate secretion of insulin from pancreatic beta cells in the presence of hypoglycemia. We herein describe the case of a 5‐month‐old boy with CHH due to congenital portosystemic shunt (CPSS). Insulin secreted from pancreatic beta cells flows into the portal vein and is first metabolized in the liver. First‐pass elimination of insulin in the liver leads to great decrease in insulin concentration by approximately 40–80% in humans. CPSS accounts for a large quantity of insulin delivery into the systemic circulation due to the lack of hepatic first‐pass elimination. Hypoglycemia can result from consistently high levels of insulin after reaching normal glucose level. CPSS therefore should be considered as a rare cause of CHH, especially in the case of post‐prandial hyperinsulinemic hypoglycemia.     

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.     

Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia,ventricular septal defect and intrapulmonary shunting

Abstract: Congenital portosystemic shunts are the anomalies in which the mesenteric venous drainage bypasses the liver and drains directly into the systemic circulation. This is a report of a rare case of LDLT in a four‐yr old male child suffering with biliary atresia (post‐failed Kasai procedure) associated with (i) a large congenital CEPSh from the spleno‐mesentric confluence to the LHV, (ii) intrapulmonary shunts, (iii) perimembranous VSD. The left lobe graft was procured from the mother of the child. Recipient IVC and the shunt vessel were preserved during the hepatectomy, and the caval and shunt clamping were remarkably short while performing the HV and portal anastomosis. Post‐operative course was uneventful; intrapulmonary shunts regressed within three months after transplantation and currently after 18 months following transplant child is doing well with normal liver functions. CEPSh has been extensively discussed and all the published cases of liver transplantation for CEPSh were reviewed.     

Severe gastric variceal bleeding successfully treated by emergency splenic artery embolization

Bleeding from gastric varices due to splenic vein obstruction is extremely rare in children, but it can be catastrophic. Reported herein is the case of a teenager with splenic vein thrombosis and chronic decompensated liver disease from autoimmune hepatitis who presented with massive gastric variceal bleeding. Standard medical management did not control the bleeding. Due to decompensated liver disease and continuous active bleeding, emergency partial splenic artery embolization was preferred over splenectomy or a shunt procedure. Bleeding was successfully controlled by partial splenic artery embolization by decreasing the inflow of blood into the portal system. It is concluded that emergency partial splenic artery embolization is a safer alternative life‐saving procedure to manage severe gastric variceal bleeding due to splenic vein obstruction in a patient with high surgical risk. To our knowledge, only one other patient with similar management has been reported in the pediatric age group.     

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.     

Jejunal varices diagnosed by capsule endoscopy in patients with post‐liver transplant portal hypertension

Portal hypertension secondary to portal vein obstruction following liver transplant occurs in 5%‐10% of children. Jejunal varices are uncommon in this group. We present a case series of children with significant GI blood loss, negative upper endoscopy, and jejunal varices detected by CE. Case series of patients who had CE for chronic GI blood loss following liver transplantation. Three patients who had their initial transplants at a median age of 7 months were identified at our institution presenting at a median age of 8 years (range 7‐16 years) with a median Hgb of 2.8 g/dL (range 1.8‐6.8 g/dL). Upper endoscopy was negative for significant esophageal varices, gastric varices, and bleeding portal gastropathy in all three children. All three patients had significant jejunal varices noted on CE in mid‐jejunum. Jejunal varices were described as large prominent bluish vessels underneath visualized mucosa, one with evidence of recent bleeding. The results led to venoplasty of the portal vein in two patients and a decompressive shunt in one patient with resolution of GI bleed and anemia. CE is useful to diagnose intestinal varices in children with portal hypertension and GI bleeding following liver transplant.