Outcome of duct‐to‐duct vs. Roux‐en‐Y hepaticojejunostomy biliary anastomoses in below 15‐kg pediatric liver transplant recipients

The best type of biliary anastomosis to use in lower weight pediatric liver transplant recipients is debatable. In this study, we share a single center's experience comparing the rate of anastomotic biliary complications based on the type of biliary anastomosis performed in this population of patients. A retrospective review of pediatric liver transplants for recipients weighing <15 kg from 11/2003 till 12/2011 was performed. Patients were grouped based on the type of biliary anastomosis into two groups: duct‐to‐duct (d‐d) and Roux‐en‐Y hepaticojejunostomy (h‐j) anastomoses. A total of 24 patients (12 males, 12 females) with a mean age of 26 ± 20 months and a mean weight of 9.27 ± 2.63 kg (range = 5.3–13.9 kg) were studied. All anastomotic complications occurred in patients who received left lateral segments. No statistical differences were found in the post‐operative biliary (p = 0.86) or vascular (p = 0.99) complications between the two groups. Acknowledging the limited sample size, our data suggest that duct‐to‐duct anastomosis can be performed safely in pediatric liver transplantation recipients weighing below 15 kg.     

Intrahepatic cholangiojejunostomy for complex biliary stenosis after pediatric living‐donor liver transplantation

The treatment of biliary stenosis after pediatric LDLT is challenging. We describe an innovative technique of peripheral IHCJ for the treatment of patients with complex biliary stenosis after pediatric LDLT in whom percutaneous treatment failed. During surgery, the percutaneous biliary drainage is removed and a flexible metal stylet is introduced trough the tract. Subsequently, the most superficial aspect of the biliary tree is recognized by palpation of the stylet's round tip in the liver surface. The liver parenchyma is then transected until the bile duct is reached. A side‐to‐side anastomosis to the previous Roux‐en‐Y limb is performed over a silicone stent. Among 328 pediatric liver transplants performed between 1988 and 2015, 26 patients developed biliary stenosis. From nine patients requiring surgery, three patients who had received left lateral grafts from living‐related donors due to biliary atresia were successfully treated with IHCJ. After a mean of 45.6 months, all patients are alive with normal liver morphological and function tests. The presented technique was a feasible and safe surgical option to treat selected pediatric recipients with complex biliary stenosis in whom percutaneous procedures or rehepaticojejunostomy were not possible, allowing complete resolution of cholestasis and thus avoiding liver retransplantation.     

Roux‐en‐Y intussusception after pediatric liver transplant – A case report

Small bowel obstruction in a pediatric patient following liver transplant often results from adhesions, hernias, or post‐transplant lymphoproliferative disease. Here, we present an unusual and previously unreported entity – Roux‐en‐Y intussusception in an eight‐yr‐old female several years after liver transplantation. Although a rare complication, Roux‐en‐Y intussusception should be considered as a potential etiology in the patient presenting with bowel obstruction, with specific attention to acute presentation accompanying jaundice.     

Duct-to-duct biliary reconstruction in pediatric living donor liver transplantation

The results of duct-to-duct biliary reconstruction in six pediatric patients who received a living donor liver transplant aged from 2 months to 11 yr old are reported. The graft was either entire or a part of the left lateral segments. The orifice of the bile duct of the graft was anastomosed to the recipients' hepatic duct in an end-to-end fashion by interrupted suture using 6-0 absorbable material. A transanastomotic external stent tube (4 Fr) was passed through the stump of the recipients' cystic duct. Mean time for reconstruction was 24 min. All the recipients survived the operation and reinitiated oral intake on postoperative day 3. There were no early biliary complications. One 5-yr-old boy suffered from an anastomotic stenosis 9 months after transplantation. He underwent re-anastomosis by Roux-en Y (R-Y) procedure and recovered uneventfully. Duct-to-duct anastomosis in pediatric living donor liver transplantation has benefits while the complication rate is comparable to R-Y reconstruction.     

Foregut atresias and bile duct anomalies: rare,infrequent or common?!

The association of foregut atresias and bile duct anomalies is reportedly rare. We encountered five referrals within 2 years where the secondary diagnosis was missed at operation. Four patients initially presented on antenatal scans as a foregut atresia whereas the fifth presented at nine years with abdominal pain due to a choledochal cyst. The biliary anomalies (cholecysto-hepatic duct, liver cyst and choledochal cysts) in the first four presented as postoperative jaundice during infancy whereas the fifth patient developed subacute intestinal obstruction due to congenital duodenal stenosis at fifteen years. In the patients with duodenal atresia neither did the preoperative X ray reveal any distal bowel gas nor did the subsequent intraoperative cholangiograms reveal bifid common bile duct or pancreato-biliary malunion. Atresias were corrected by primary repair (duodenoduodenostomy for congenital duodenal obstruction in four patients and disconnection/ligation of tracheo-oesophageal fistula with oesophageal anastomosis in one patient). The biliary anomalies were corrected by excision of the abnormal bile ducts (choledochal cyst/liver cyst/cholecystectomy) with Roux en Y hepaticojejunostomy. All patients are asymptomatic and liver function and biliary dilatation has normalised. The association of foregut atresias and bile duct anomalies is not as rare as previously reported. Antenatal ultrasound suggesting either a foregut or a biliary anomaly should alert one to the association. Full radiological and/or imaging investigation may be indicated prior to corrective surgery of the primary anomaly.     

Unusual venous collateral pathways allow for reperfusion of the intrahepatic portal venous system in children with portal vein thrombosis after split liver transplantation: Clinical relevance and management implications

PVT is the most frequent vascular complication after LT in small children, and a higher incidence has been observed in those transplanted for biliary atresia or with a LLSG. Thrombosis of the PV causes extrahepatic portal hypertension and is associated with splenomegaly and the development of venous neo‐collaterals, including gastro‐oesophageal varices and splenorenal shunts. It has also been incidentally suggested in the literature that patients who have had a Roux‐en‐Y loop for a biliary reconstruction may present with a cavernomatous transformation of the distal portion of the loop. In this study, 13 children with CEPH caused by thrombosis of the PV after LT were analysed. The study evidenced the development of two types of hepatopetal venous networks: (a) a large cavernoma along the Roux loop and around the biliary anastomosis, and (b) a network of neo‐collaterals in the gastro‐duodeno‐pancreatic area that connected to the intrahepatic portal branches directly through the liver capsule. These hepatopetal venous networks between the venous system of the surrounding organs or the omentum and the intrahepatic portal branches can be identified by radiologists. The relevance for the transplanting physician and the transplant surgeon is discussed.     

Feasibility of duct-to-duct biliary reconstruction in pediatric living related liver transplantation: report of three cases

Feasibility of duct-to-duct biliary reconstruction in adult living related liver transplantation (LRLTx) has been recently reported; however, little has been known of its surgical outcome in children. To assess the feasibility and safety of duct-to-duct biliary reconstruction in children, the surgical outcomes of duct-to-duct biliary reconstruction were retrospectively analyzed. The subjects were three children who underwent LRLTx in our hospital each utilizing allografts with a right lobe, a left robe and a lateral segment, respectively. The cause of end-stage liver disease in each of them was fulminant Wilson's disease, fulminant hepatic failure and unresectable hepatoblastoma. Duct-to-duct anastomosis was performed in younger patients and adolescents with interrupted and continuous sutures, respectively. The diameter of bile duct in allografts was from 4 to 6 mm and 12 or 13 stitches were required for anastomosis. Post-operative choledochography from the external tube showed neither stenosis nor leakage and the tube was evacuated within 3 months after LRLTx. No biliary complications were observed with the median follow-up of 28 months. In conclusion, our results show that duct-to-duct biliary reconstructions in pediatric LRLTx seemed to be feasible and safe. Further studies are required to elucidate its real impact on pediatric LRLTx.     

45例腹腔镜辅助胆总管囊肿根治术

目的本研究对腹腔镜先天性胆总管囊肿切除，肝管空肠Roux-Y吻合术进行探讨。方法从2001年6月至2003年9月，共收治先天性胆总管囊肿患儿45例，年龄2个月到12岩。其中42例为囊肿型，平均囊肿直径3.8cm（2-18cm）；其余3例为梭形，直径分别为1．5、2．0和2．2cm。本组45例患儿均经腹腔镜行先天性胆总管囊肿切除，肝管空肠Roux-Y吻合术。结果本组45例患儿手术全部成功，手术时间平均为4.3h（3．5～7．6h），术中出血量约5～10ml。本组8例患儿合并肝管狭窄，术中同时行腹腔镜胆总管囊肿切除及肝管成形；6例患儿合并共同管内蛋白栓，术中通过腹腔镜导入肠道或插管冲洗清除。其中1例患儿于术后第1d发生胆漏，术后第26d时自愈；其余44例患儿术后恢复顺利，住院时间3～6d。术后随访3～30个月，无肠粘连梗阻和吻合口狭窄等术后并发症发生。结论经腹腔镜先天性胆总管囊肿切除，肝管空肠吻合术是一种安全可靠的方法，术中胆道造影全面了解胆道的结构，清晰的肝门暴露，准确的囊肿分离和熟练的缝合技术是手术成功的关键。     

Roux‐en‐Y enterolith leading to obstruction and ischemic necrosis after pediatric orthotopic liver transplantation

Biliary complications are a common cause of morbidity after liver transplantation, with biliary stone formation being a known occurrence generally upstream of a stricture. A 12‐year‐old boy, who underwent an orthotopic liver transplantation at 11 months of age for biliary atresia, presented acutely with fever and abdominal pain. Cross‐sectional imaging revealed Roux‐en‐Y limb dilatation and thickening. He was explored and was found to have an ischemic Roux limb secondary to an obstructing enterolith. A segmental bowel resection and revision of his hepaticojejunostomy was performed. While rare, biliary enteroliths may present as either a bowel obstruction or cholangitis and should be considered in the differential diagnosis of a patient following biliary reconstruction. Additionally, anatomic etiologies should be considered and potentially surgically corrected.     

Outcomes after discontinuation of routine use of transanastomotic biliary stents in pediatric liver transplantation at a single site

Routine use of transanastomotic biliary stents (RTBS) for biliary reconstruction in liver transplantation (LT) is controversial, with conflicting outcomes in adult randomized trials. Pediatric literature contains limited data. This study is a retrospective review of 99 patients who underwent first LT (2005–2014). In 2011, RTBS was discontinued at our center. This study describes biliary complications following LT with and without RTBS. 56 (56%) patients had RTBS. Median age at LT was 1.9 yr (IQR 0.7, 8.6); 55% were female. Most common indication for LT was biliary atresia (36%). Most common biliary reconstruction was Roux‐en‐Y choledochojejunostomy (75% with RTBS, 58% without RTBS, p = 0.09). Biliary complications (strictures, bile leaks, surgical revision) occurred in 23% without significant difference between groups (20% with RTBS, 28% without RTBS, p = 0.33). Patients with RTBS had routine cholangiography via the tube at 6–8 wk; thus, significantly more patients with RTBS had cholangiograms (91% vs. 19%, p < 0.0001). There was no difference in the number of patients who required therapeutic intervention via endoscopic or percutaneous transhepatic cholangiography (11% with RTBS, 19% no RTBS, p = 0.26). Routine use of RTBS for biliary reconstruction in pediatric LT may not be necessary, and possibly associated with need for costlier, invasive imaging without improvement in outcomes.     

Hepatobiliary scintigraphy for the assessment of biliary strictures after pediatric liver transplantation

Abstract: HBS is used in the management of liver transplantation, a significant complication of which is biliary stricture. Strictures may be intraparenchymal within segments and main duct (non‐anastomotic) or at the biliary‐enteric anastomosis (anastomotic). Strictures are definitively diagnosed, and often managed, by PTC. This is invasive, technically challenging, and requires general anesthesia in young children. HBS may allow early detection of these complications and is non‐invasive. The aim of this study was to review the scintigraphic pattern of biliary strictures using ^99mTcDISIDA HBS following pediatric orthotopic liver transplantation, and to assess its role in the diagnostic algorithm of suspected biliary strictures. All available hepatobiliary studies performed post‐transplant in 101 episodes of liver transplantation in 92 pediatric patients were reviewed. Twenty‐three (23%) patients had known biliary strictures. Twenty‐two patients had adequate studies available for review; five had intrahepatic (non‐anastomotic) strictures alone, nine had a stricture of the anastomosis alone, and eight had both intrahepatic and anastomotic strictures. HBS patterns (either segmental or global changes) correlated very highly with clinically significant biliary strictures. All patients with known strictures had abnormal HBS; hence, in patients with abnormal liver function tests post‐liver transplant, a normal HBS makes strictures very unlikely. We propose that HBS can thus be used to determine if further investigation is required.     

Placement of an internal–external biliary drain through a bilio‐enteric fistula in a neonate to re‐establish antegrade bile flow after liver transplantation

Three‐month‐old baby girl with history of post‐liver transplant hepatic artery dissection treated with ligation after take down of the biliary anastomosis and placement of a surgical external common bile duct drain. There was persistent malfunction of this drain. A bilio‐enteric fistula was noted during the later placement of an image guided percutaneous external drain. Subsequently, an internal–external biliary drain was successfully placed through this fistula. An excellent clinical and functional result was achieved.     

Recanalization of post‐transplant late‐onset long segmental portal vein thrombosis with bidirectional transhepatic and transmesenteric approach

PV complications are the most frequent vascular complications in pediatric LT. We have experienced a case with chronic postoperative PVT that necessitates combined transhepatic and transmesenteric approach and have confirmed mid‐term patency. An eight‐yr‐old boy had successful LDLT with a left lateral segment graft at the age of two months for HBV‐related acute liver failure. Seven years after transplantation, the patient suddenly showed a melena with hypovolemic shock. Doppler ultrasound and CT revealed intrahepatic bile duct dilatation and main PVT with collateral formation at hepatic hilus and mesenterium of the Roux‐en‐Y jejunal loop. Urgent splenic artery embolization was performed to control the bleeding and was temporarily effective. Therefore, recanalization of PVO was attempted. Because of long segmental PVO and steep angle between the intrahepatic PV and the portal trunk, bidirectional transhepatic and transmesenteric approach was selected and resulted in deploying three metallic stents necessitating additional infusion thrombolytic therapy. The patient is now followed as an outpatient with patent stents for two yr since the procedure. For the rescue of these patients, recanalization of obstructed PV trunk with bidirectional approach would be feasible with better graft survival and less invasiveness than conventional surgical interventions.     

Isolated liver transplantation in children with cystic fibrosis – An Australian experience

Nightingale S, O’Loughlin EV, Dorney SFA, Shun A, Verran DJ, Strasser SI, McCaughan GW, Jermyn V, Van Asperen P, Gaskin KJ, Stormon MO. Isolated liver transplantation in children with cystic fibrosis – An Australian experience.
Pediatr Transplantation 2010: 14:779–785. © 2010 John Wiley & Sons A/S. Abstract: CF liver disease is an uncommon indication for pediatric LT. Determining optimal timing and type (isolated liver versus multi‐organ) of transplantation for those with severe liver disease can be challenging and involves consideration of the extent of liver disease (PHT, synthetic dysfunction) and extrahepatic factors such as pulmonary function. We present the experience of isolated LT for CF at our center. Eight children received one allograft each (3.9% of all grafts). One‐ and four‐yr survivals are both 75%. The two deaths occurred within the first two months after LT, and in both cases, invasive fungal infections were implicated, one following treatment for acute severe rejection. All had significant PHT, and six had synthetic dysfunction. All had roux‐en Y biliary anastomoses and none developed long‐term biliary complications. Seven had pulmonary colonization with Pseudomonas aeruginosa and six with fungus at time of transplantation. Mean pre‐LT FEV1 was 80% (range 59–116%) predicted, and lung function post‐LT was stable. Isolated LT in children with CF is successful in those with relatively preserved pulmonary function, which does not appear to deteriorate as a consequence. Roux‐en Y biliary anastomosis and antifungal prophylaxis should be a part of management of these patients.     

Biliary reconstruction before clamp removal to avoid portal vein thrombosis in pediatric living‐donor liver transplantation using hyper‐reduced left lateral segment grafts: A novel technical strategy

LT has become the treatment of choice for children with end‐stage liver disease. The scarcity of donors and the considerable mortality on waiting lists have propelled the related living‐donor techniques, especially in small children. This population need smaller and good quality grafts and are usually candidates to receive a LLS from a related donor. Many times this grafts are still large and do not fit in the receptor's abdomen, so a further hyper‐reduction may be required. Despite all advances in LT field, vascular complications still occur in a considerable proportion remaining as a significant cause of morbidity, graft loss, and mortality. Technical issues currently play an essential role in its genesis. The widely spread technique for biliary and vascular reconstruction in living donor LT (LDLT) nowadays implies removal of the portal vein (PV) clamp after the venous anastomosis, then the arterial reconstruction is done, followed by the biliary reconstruction. However, due to the posterior location of the LLS bile duct, for its reconstruction, a rotation of the liver is required risking a potential transient PV occlusion leading to thrombosis afterward. We describe a new technique that involves performing biliary reconstruction after the PV anastomosis and before removing the vascular clamp, thus allowing to freely rotate the liver with less risk of PV occlusion and thrombosis.     

Roux‐en‐Y hepatico‐jejunostomy for a left segmental graft: Do not twist the loop,stick it!

Biliary complications remain a major challenge for long‐term success after LT, as it is, as a rule, the most common technical – early and late – complication that occurs, and because these complications contribute to a significant number of late graft losses and retransplantations. In the pediatric age group, both biliary atresia, as the patient's condition, and the use of a left liver graft, obtained by a liver division technique, make it necessary for the use of a Roux‐en‐Y jejunal loop for the biliary reconstruction in the majority of cases. A slight modification of the technique is presented, consisting of a straight positioning along the cut surface (rather than the conventional position that results in a harpoon shape). A favorable outcome in terms of a technical complication and graft survival was observed. This way of doing this is an interesting variation and adds to the surgical armamentarium.     

Simultaneous surgical and interventional radiological approach to treat complicated biliary strictures after pediatric liver transplantation

Post-transplantation biliary strictures occur in 5-15% of the pediatric liver transplant patients and are conventionally managed by interventional radiological techniques. Failure of this treatment leads to reoperation and sometimes to retransplantation. Herein, we describe a surgical approach and interventional radiologic approach to manage biliary strictures that failed the conventional radiologic treatment, in order to avoid retransplantation. Included in the study were eight children who underwent liver transplantation at our center or referred to our institution for evaluation of the biliary strictures that failed radiological treatment. Biliary strictures were confirmed by a narrowing of the biliary anastomosis on the percutaneous transhepatic cholangiogram. At surgery, a guide wire was introduced into the distal bile system through the use of an enterotomy in Roux limb. Over the guide wire, the stricture was ballooned and the diameter of the biliary tree was determined. A pigtail catheter was introduced on the biliary tree across the abdominal wall, the liver, the stricture and the anastomosis into the enterotomy. A final cholangiogram confirmed the positioning of the catheter. Mean follow-up was 39.8 +/- 20.8 months. All patients had their strictures successfully treated and survived the procedure. Three patients were readmitted to the hospital with fever. It was necessary to revise the hepaticojejunostomy in three patients because of cholangitis and/or recurrence of biliary stricture. Of the eight patients of this study, two required retransplantation and one died. We conclude that an aggressive combined surgical and radiologic approach can avoid retransplantation in patients with complicated post-transplant biliary strictures.     

Sclerosing cholangitis associated to cryptosporidiosis in liver-transplanted children

Three children of a series of 461 pediatric liver transplant recipients developed diffuse cholangitis associated with intestinal cryptosporidium carriage. All three received immunosuppression consisting of tacrolimus and prednisone. Cryprosporidium carriage was treated with paramomycin, while immunosuppression was decreased according to graft tolerance. No other infectious pathogens were found, and no vascular problems were detected. Bile duct anastomosis was reoperated in all three, but biliary cirrhosis developed in one patient, requiring retransplantation. All three patients are alive and well, and free of intestinal parasites on follow-up. Conclusion Cryptosporidium intestinal infection may play a role in some cases of otherwise unexplained cholangiopathies in pediatric liver transplant recipients. This may lead to significant morbidity, including need for retransplantation. Received: 29 June 1999 and in revised form: 10 September 1999 / Accepted: 21 September 1999     

Simultaneous or sequential gastrectomy in pediatric liver transplant recipients

The association between LT and gastrectomy is not common. Only two studies reported the gastrectomy/LT association in children. Here, we report three children who underwent LT who required a concomitant or sequential gastrectomy for different reasons. Patient 1, a 16‐yr‐old boy, during the LT, underwent a partial gastrectomy due to extensive injury to the duodenum. He had a previous and unusual portoenterostomy performed in the duodenum. Bowel reconstruction was performed using an intestinal loop that was first used for the bilio‐enteric anastomosis and then connected to the gastric stump. Patient 2, a 22‐month‐old female child, underwent a partial gastrectomy with a Roux‐en‐Y reconstruction during a retransplantation. She had a large perforated gastric ulcer blocked by the allograft liver. Patient 3, a 26‐month‐old male child, five yr after living donor LT, was submitted to a partial gastrectomy because of gastric outlet obstruction. The histopathology was compatible with eosinophilic gastritis. The association between LT and gastrectomy in the pediatric population is extremely rare. Appropriate knowledge of the previous transplantation technique is very important. Further studies are required to assess the outcomes of the different types of gastric reconstruction in pediatric recipients.     

Comparison of Histidine‐Tryptophan‐Ketoglutarate and University of Wisconsin preservation solutions in pediatric liver transplantation

UW and HTK solutions are the two primary organ preservation solutions most used in the United States. This study analyzes use of the two solutions in all pediatric liver transplants performed at a single center between 2001and 2017. Outcome measures included early graft function, as well as graft and patient survival. Bile duct complications were reviewed. Operative technique, immunosuppressive protocols, and donor acceptance criteria remained uniform among participating surgeons throughout the study period. There were 104 pediatric liver transplants with complete data during the study period, 75 preserved with HTK (68%) and 29 with UW (26%). Demographics were similar. Cold and warm ischemia times were similar. Peak ALT post‐transplant was higher in the UW group at both peak and post‐transplant day 3. The peak TB levels were similar. Bile duct strictures were more common in the UW group (44% vs 16%, P < .01). Early graft survival was statistically similar at 7‐, 90‐ and 365‐days post‐transplant. Cox regression graft survival was similar at 10‐years. This study suggests that use of HTK in pediatric liver transplantation is safe with outcomes similar to UW, though bile duct stricture rates may be lower with HTK.