Left Lateral Segment Liver Transplant Research Articles

P.408: Left lateral segment liver transplantation in the cirrhotic setting with hepatocellular carcinoma: A case report.

P.408: Left lateral segment liver transplantation in the cirrhotic setting with hepatocellular carcinoma: A case report.

Percutaneous Transhepatic Treatment for Obstructing Retained Surgical Biliary Stents After Pediatric Liver Transplantation Without Placement of Biliary Drain

Percutaneous Transhepatic Treatment for Obstructing Retained Surgical Biliary Stents After Pediatric Liver Transplantation Without Placement of Biliary Drain

A220 AN UNEXPECTED CAUSE OF ACUTE JAUNDICE IN A POST-TRANSPLANT PATIENT

Abstract Background Complications post liver transplantation are common. These may include thromboses, primary graft nonfunction, biliary complications, chylothorax, infection, and rejection. Hepaticojejunostomy with Roux-en-Y limb is a common technique for pediatric liver transplantation. Complications of hepaticojejunostomy include small bowel obstruction due to incarceration of the Roux-en-Y limb within an internal hernia. Acquired diaphragmatic hernia has previously been reported in liver transplant patients. Here we present a unique case of acute biliary obstruction secondary to incarceration of a Roux limb within an acquired diaphragmatic hernia. Aims To describe a unique case of incarcerated Roux-en-Y limb in a diaphragmatic hernia leading to acute jaundice in a pediatric liver transplant patient. Methods Retrospective chart review and case review with the surgical and medical teams. Results A 14 month old girl with unresectable hepatoblastoma received a living donor, left lateral segment liver transplant using standard venous reconstruction, microvascular plastics arterial reconstruction, and the recipient common hepatic duct was anastomosed to the donor left hepatic duct. Serial ultrasounds showed persistent intrahepatic duct dilatation with cholestasis. Given the concern of biliary obstruction, 10 days post-transplant, she underwent repeat laparotomy and was found to have a dilated common bile duct with a proximal obstruction. A Roux-en-Y hepaticojejunostomy was performed to establish adequate biliary drainage. Three months post-transplant she developed lung metastases that required wedge resection and chemotherapy. 7 months post-transplant, while admitted for routine chemotherapy she acutely developed jaundice, pruritus, and transaminitis. Liver ultrasound showed an apparent fluid collection at the cut surface of the liver and biliary dilatation. Extrinsic compression of the biliary tree was suspected, and so a percutaneous biliary drain was placed to drain the collection and decompress the biliary system. Despite improvement in the transaminitis and cholestasis, several days later, she developed severe abdominal pain and associated grunting respirations. An MRI with MRCP to reassess the previous findings and rule out a new intraabdominal cause for her symptoms was completed and unexpectedly showed herniation of her Roux-en-Y limb into a diaphragmatic hernia. She had urgent diaphragmatic hernia repair with reduction of the incarcerated bowel loop, with resolution of her pain and jaundice. Conclusions Post liver transplant Roux-en-Y herniation is uncommon, but can occur with internal hernias and in diaphragmatic hernias. This uncommon complication should be considered for the post liver transplant patient with hepaticojejunostomy in the setting of acute jaundice, particularly if accompanied by acute onset abdominal pain and/or increased work of breathing. Funding Agencies None

A Novel Surgical Technique to Assure Donor and Recipient Safety: Pyloric to Segment 4 Arterial Reconstruction.

Background.Strategies to extend the pool of organs include and promote the use of segmental liver grafts. While performing a living donor left lateral segment (LLS) liver transplant and in split procedures, the hepatic artery´s division becomes critical when a dominant segment 4 artery (S4A) emerges from the left hepatic artery (LHA). We aim to describe a novel technique that consists of performing microsurgical reconstruction from the pyloric artery (PA) to S4A.Case Reports.A 45-y-old living donor was evaluated to use his LLS as a graft for a pediatric recipient. During the procedure, a dominant S4A born from the LHA was dissected. To obtain an appropriate LHA length and diameter for the recipient, it was necessary to transect it. An extended right lobe split graft was used in a 61-y-old patient. The S4A born from LHA had to be sectioned during the split procedure. In both cases, segment 4 remained incompletely perfused. The PA was dissected with enough length to be rotated, to perform a microsurgical anastomosis to the S4A, recovering parenchyma’s color and Doppler signal while vascular permeability was demonstrated using CT scan. There was no biliary or cut surface complication.Conclusions.PA to S4A reconstruction is a simple and novel technique that can be used for LLS and extended right lobe split graft and might contribute to increase donor selection and reduce living donor and recipient S4A-related complications.

Pediatric living donor left lateral segment liver transplantation for biliary atresia: Doppler ultrasound findings in early postoperative period.

To analyze hepatic hemodynamic parameters detected by Doppler ultrasound (DU) of uncomplicated children with biliary atresia who underwent left lateral segment living donor liver transplantation (LLS-LDLT), explore its normal change trend over time and determine the normal reference interval. We retrospectively involved the data from 227 biliary atresia patients (100 Males,127 Females). Hemodynamic parameters include peak systolic velocity (PSV), end-diastolic velocity (EDV), resistivity index (RI), and pulsation index (PI) of the hepatic artery (HA), portal vein velocity (PVV), portal vein flow (PVF) and hepatic vein velocity (HVV) during intra-operative and on the 1st, 3rd, 5th and 7th day after operation were collected. Repeated measures analysis of the variance and Friedman test were used to analyze the changing trend of hemodynamic parameters over time in the first week after the operation. PSVHA and EDVHA showed a similar changing tendency at one week after surgery, with an overall decrease-rise trend;RIHA and PIHAalso changed similarly with an overallrise-decrease trend. The HVV and PVV at surgery were lower than at all time points after surgery. As for PVF, the value of POD5 was the highest and then decreased. Additionally, this study provided the normal reference interval of hemodynamic parameters for LLS-LDLT patients, which were PSVHA: 18.4-98.3cm/s, EDVHA: 0-43.3cm/s, RIHA: 0.41-1.0, PIHA: 0.51-2.0, PVV: 19.0-83.7cm/s, HVV: 19.4-68.0cm/s, and PVF:99.5-500.0ml/min/100g at intraoperation. Within the first postoperative week: PSVHA: 21.0-97.7cm/s, EDVHA: 0-32.7cm/s, RIHA: 0.47-1.0, PIHA: 0.62-2.0, PVV: 23.0-92.0cm/s, HVV: 19.7-86.0cm/s, and PVF: 100.0-513.0ml/min/100g. The hepatic hemodynamic of post-transplanted children detected by DU had specific changing trends and normal ranges, which provides valuable reference values for ultrasonologists and pediatric transplant clinicians.

Biliary restoration using a combined endoscopic-percutaneous approach following “orphan duct syndrome” after pediatric liver transplantation

A 2-year-old male with arginosuccinate lyase deficiency underwent left lateral segment liver transplantation complicated by “orphan duct syndrome (Celik et al., 2019) [1]” and biliary leak. After revision of the Roux-en-Y anastomosis, biliary drainage was still impaired. The excluded bile duct was diagnosed and a biliary restoration (or neo-duct) using a combined endoscopic and percutaneous approach was created by Gastroenterology and Interventional Radiology. To our knowledge, this case report represents the first combined endoscopic-percutaneous biliary restoration procedure performed in a pediatric patient.

A97 TACROLIMUS-ASSOCIATED HEMOLYTIC UREMIC SYNDROME IN A PEDIATRIC LIVER TRANSPLANT RECIPIENT

Abstract Background Tacrolimus associated hemolytic uremic syndrome (HUS) has been reported in renal and pediatric cardiac transplantation. Furthermore, in adults receiving liver transplant, there are reports of multiorgan failure, death, and end stage renal failure secondary to tacrolimus induced HUS [J NEPHROL 2003; 16: 580–585]. Although tacrolimus is commonly used in pediatric liver transplantation, there are no reports of the same entity. Aims To describe a case of tacrolimus induced HUS in a pediatric liver transplant patient and subsequent re-initiation of tacrolimus. Methods The patient was followed during his hospital admission and after discharge. Results An 8-year-old male with Progressive Familial Intrahepatic Cholestasis (PFIC) Type III, diagnosed at age 2, presents for living unrelated donor liver transplantation. He was previously well compensated but began to deteriorate in the months leading up to transplant with rising liver enzymes, ascites, and mild encephalopathy after presumed viral gastroenteritis. He receives a left lateral segment liver transplantation and is started on tacrolimus immunosuppression post liver transplant as per protocol. On initiation of tacrolimus, the patient’s hemoglobin trends downward to a trough of 59 g/L four days post transplantation. Concurrently, his haptoglobin is <0.10g/L, total bilirubin peaks at 708 umol/L, creatinine rises to a peak of 71 umol/L, urea peaks at 23.0 mmol/L. Platelets reach a nadir at 27x10^9/L, seven days post transplantation. Direct antibody test was negative, and blood cultures remained negative. Given the constellation of hemolytic anemia, thrombocytopenia, and acute kidney injury following initiation of tacrolimus, a diagnosis of atypical HUS is made. Tacrolimus is discontinued, intravenous methylprednisolone is started and the patient is switched to cyclosporine immunosuppression. Both the hemolysis and kidney injury resolve gradually. The patient develops side effects from cyclosporine including hypertension and excessive hair growth. One month post discontinuation of tacrolimus, he is started on sirolimus, while weaning steroids and cyclosporine. Seven weeks post discontinuation of tacrolimus, he is restarted on tacrolimus and is currently tolerating dual immunosuppression with no signs of HUS recurrence. Conclusions Atypical HUS secondary to tacrolimus post liver transplant in children is a rare entity. In the acute period, discontinuation of tacrolimus is essential to preserve renal function given potentially devastating outcomes seen in adults. Cyclosporine is an alternative immunosuppressive agent used in pediatric solid organ transplantation, although it has also been implicated in causing HUS. Funding Agencies None

Transjugular Liver Biopsy Following Left Lobe or Lateral Segment Transplantation in Pediatric Patients

To evaluate the efficacy and safety of transjugular liver biopsy in pediatric patients with left lobe or lateral segment liver transplant. This was a retrospective study of data from 104 transjugular liver biopsies (57 children) using an 18- or 19-G biopsy needle. Transjugular liver biopsy was selected due to coagulopathy and/or ascites in 56 (54%) procedures. The median patient age, body weight, and interval from liver transplantation were 56months, 16kg, and 2months, respectively. Technical success was defined as adequate liver specimens yield determined by the operator at the time of procedure. Complications, the longest length of obtained liver specimens according to needle type, and adequacy for histological diagnosis were analyzed. Biopsy using a biopsy needle was successfully achieved in 103 procedures (99%). In one procedure, liver samples were obtained using biopsy forceps following unsuccessful attempts with a biopsy needle. Major complication occurred in one procedure following biopsies using both a biopsy needle and biopsy forceps. The patient received transfusion to manage decreased blood pressure and hemoglobin levels although post-biopsy venography did not show extravasation. The median longest length of liver specimens using 18-G (n = 63) and 19-G (n = 40) needles were 10mm (range, 5-20) and 10mm (range, 5-20), respectively (p = 0.704). Liver specimens were adequate for histological diagnosis in all procedures. Transjugular liver biopsy using a biopsy needle appears to be a safe and effective method to obtain liver specimens for histological diagnosis in pediatric patients with left lobe or lateral segment liver transplant. Level 4, case series.

Right diaphragmatic hernia after liver transplant in pediatrics: A case report and review of the literature

Diaphragmatic hernias (DH) are an unusual complication after pediatric liver transplantation; however, they have been reported with increased frequency in the past few years. DHs are responsible for nearly half of the small bowel obstructions requiring surgical intervention in this patient population. It has been suggested that the use of a left lobe liver graft, surgical trauma, malnourishment, elevated intra-abdominal pressures, and mTor inhibitors may predispose to development of DH. The use of a segmental graft may increase the recognition of diaphragmatic hernia because the surgically damaged right hemi-diaphragm often remains exposed to underlying viscera, instead of being covered by the right hepatic lobe. Treatment is surgical reduction, with up to 20% of the patients requiring resection of the herniated intestine. Herein we describe a case of DH after left segmental liver transplant in a two- yr-old boy that presented one month post left lobe split liver transplant with abdominal pain, anorexia, and respiratory distress. Just like in the majority of the reported cases, an urgent laparotomy with primary repair was performed. No resection of the herniated segment of intestine was required. For pediatric patients with otherwise unexplained respiratory or gastrointestinal symptoms after a left lateral segment liver transplant, right-sided diaphragmatic hernias should always be high in the differential diagnosis.

The reduced left lateral segment in pediatric liver transplantation: An alternative to the monosegment graft

Tailoring graft size to small paediatric recipients is a challenge. We have developed a reduced left lateral segment as an alternative to monosegment transplantation for small size recipients. Since November 2000, 89 children have been transplanted with 100 deceased donor liver grafts in our unit. Our median patient and graft survival is 89% and 88% respectively. Four of these cases were performed using a new technique of creating a small donor graft by reducing the left lateral segment. The median weight of the reduced liver graft was 264 g (range: 165-390 g). The median blood transfusion requirement was 101 mL/kg body weight (range 69-167 mL/kg). The median values of peak ALT were 1473 IU/L, INR 2.2 and bilirubin 293 micromol/L in the first two wk following surgery. One neonatal recipient died five days after transplantation from a massive intracranial haemorrhage despite satisfactory graft function. Another recipient with excellent graft function died 10 months later from primary pulmonary hypertension and secondary cardiac failure. Hepatic artery thrombosis occurred in one patient with successful revascularization but he was retransplanted three months later for chronic rejection. No biliary or venous outflow complications occurred in this group. This technique of reduced left lateral segment liver transplantation is an alternative to the monosegment graft and allows small recipients to be successfully transplanted with few technical complications related to graft preparation.

Liver transplant in a four‐month‐old child with biliary atresia, unilateral pulmonary agenesis, and diaphragmatic hernia: First case report

Bilateral pulmonary agenesis (PA) is a rare embryological defect incompatible with life. Unilateral PA has a wide range of clinical presentations: its prognosis depends on the presence and severity of other associated anomalies. Fetal biliary atresia has been associated with a number of congenital anomalies, but the etiology is still not understood. An unusual case of a child with right PA, right diaphragmatic hernia, and delayed diagnosed biliary atresia leading to liver failure is presented herein. At the age of 4 months the patient was referred to the Transplant Department at Children Healthcare of Atlanta at Egleston with cholestasis and failure to thrive. With a rapidly progressive liver insufficiency, this child was evaluated for liver transplantation. In the absence of any respiratory symptom, the patient received a deceased donor size-matched left lateral segment liver transplant, which covered the diaphragmatic defect, with no further repair required. Twenty-seven months post-transplant, the patient has good graft function, a normal Z-score and is thriving. In spite of the increased physiological and surgical challenges (absence of right lung tissue, hemi-diaphragm, and ectopic position of the liver in the right chest), liver transplantation was performed with positive outcome in this high-risk child. Whether PA, may have developmentally contributed to expression of biliary atresia will need further investigation.

Cadaveric Orthotopic Auxiliary Split Liver Transplantation and Kidney Transplantation: An Alternative for Type 1 Primary Hyperoxaluria

Liver transplantation (LTX) corrects the enzymatic defect responsible for type 1 primary hyperoxaluria (PH1). It has been advocated in combination with kidney transplantation (KTX) in patients with renal failure from PH1 because KTX alone can result in early graft loss. A 58-year-old male patient with PH1 on hemodialysis underwent resection of the left lateral segment of the liver followed by orthotopic auxiliary left lateral segment liver transplantation and kidney transplantation from a deceased donor. The serum oxalate dropped from 34.8 micromol/L before transplant to 3.6-8.3 in the first months posttransplant to <1 micromol/L (normal range 0.4-3.0). One year after posttransplant, the patient has an iothalamate glomerular filtration rate of 58 ml/min. Orthotopic auxiliary LTX is an alternative to whole LTX in PH1. By using a split deceased donor liver, it does not deprive the donor pool and protects the recipient from liver failure in case of graft loss.

Mycophenolate Mofetil in the Management of Alloimmune Hemolytic Anemia in ABO‐Compatible but Non‐Identical Pediatric Liver Transplantation

Mycophenolate Mofetil in the Management of Alloimmune Hemolytic Anemia in ABO‐Compatible but Non‐Identical Pediatric Liver Transplantation

Transjugular Intrahepatic Portosystemic Shunt in a Living Donor Left Lateral Segment Liver Transplant Recipient: Technical Considerations

The technical aspects of placing transjugular intrahepatic portosystemic shunts (TIPS) in liver transplant recipients with full allografts have well been described. In the era of live related hepatic donors, and the growing population of their recipients, it is likely that TIPS shunts will be placed in failing transplant lobes/segments. Growing allografts that are initially undersized can have an unconventional orientation of the hepatic and portal veins, which may also change with remodeling and rotation of the graft during their growth. The authors review the technical differences for TIPS procedures in transplants, particularly split grafts. They describe a technically successful TIPS procedure in an undersized and remodeled left lateral segment liver recipient and the additional difficulty this may pose.

Rapid response to rituximab in a pediatric liver transplant recipient with post‐transplant lymphoproliferative disease and maintenance with sirolimus monotherapy

A 12-yr-old girl with end-stage renal disease secondary to primary hyperoxaluria type I received a living related (left lateral segment) liver transplant from her brother as the first step of a staged liver and kidney transplant. Renal transplantation was planned for a later date from the same donor. Nine weeks after transplantation she developed polymorphic PTLD of the tonsils and adenoids. Initial treatment with surgical resection and withdrawal of immunosuppression was insufficient as she developed recurrence of the PTLD lesion 1 wk after surgical resection and reduction of immunsuppression. Treatment with the chimeric monoclonal anti CD20 antibody, rituximab (Mabthera, Hoffman-La Roche AG, Grenzach-Whylen, Germany), resulted in quick response and complete recovery from PTLD within 2 wk, with no recurrence up to 8 months after treatment. Rejection prophylaxis was successfully achieved with Sirolimus (Rapamune, Wyeth Pharmaceuticals Inc., Philadelphia, PA, USA) monotherapy, with no episodes of acute rejection.

Balloon Dilatation of Biliary–Enteric Strictures in Children

The objective of our study was to evaluate the efficacy and safety of balloon dilatation in the treatment of anastomotic strictures in children with liver transplants. For a period of 7 years, we treated 20 consecutive biliary-enteric strictures in 19 children (age range, 13 months to 17.9 years, mean, 7.3 years) with balloon dilatation. Dilatation was performed between 30 days and 8.4 years (mean, 2.6 years) following surgical creation of the biliary-enteric anastomosis. Thirteen patients had left lateral segment liver transplant grafts, one patient had a split-liver, left-lobe graft, and five patients had whole liver grafts. Technical success was 100% and there were no procedure-related complications. One patient with a patent anastomosis underwent repeat transplantation 183 days after the procedure for chronic rejection. In 58% (11/19) of the remaining procedures, balloon dilatation resulted in biliary-enteric patency at one year, and continued patency ranges from 1.4 to 5.4 years (mean, 3.6 years). In 40% (8/20) of the procedures, the biliary-enteric stricture persisted after balloon dilatation, and these patients eventually underwent surgical revision, retransplantation, or endobiliary metallic stent placement. Balloon dilatation is a safe and effective treatment for biliary-enteric strictures following pediatric liver transplantation.

Is Ketamine a Safe Anesthetic for Percutaneous Liver Biopsy in a Liver Transplant Recipient Immunosuppressed with Cyclosporine?

A 6-yr-old male patient underwent live related left lateral segment liver transplant for cryptogenic cirrhosis with portal hypertension. One month after the liver transplant the patient had an isolated liver transaminases increase. He was posted for percutaneous liver biopsy for suspected graft rejection under general anesthesia. The patient was administered ketamine 7 mg/kg along with glycopyrrolate 0.01 mg/kg IM in the preoperative area. He developed generalized tonic clonic seizures just before the biopsy and was treated with IV midazolam 1 mg and thiopental 60 mg. Percutaneous liver biopsy was obtained once the convulsions subsided. Both ketamine and cyclosporine have been implicated as having proconvulsant properties and may have been responsible for the seizures in our patient. Our experience prompted us to suggest that ketamine in a patient immunosuppressed with cyclosporine may not be safe and that alternative anesthetics may need to be considered for such procedures.

HOW MUCH LIVER TISSUE IS NECESSARY TO COMPENSATE THE DEFECT IN TYPE 1 HYPEROXALURIA?

A170 Aims: Liver transplantation (LTX) corrects the enzymatic defect responsible for primary hyperoxaluria. It has been advocated in combination with kidney transplantation (KTX) in patients with renal failure, since KTX alone can result in early graft loss. Methods: Case presentation of a patient with primary hyperoxaluria type 1. Pre- and posttransplant data were collected prospectively and reviewed in a retrospective fashion. Results: We present in a 58-year-old male patient with primary hyperoxaluria type 1, confirmed by genetic testing, on hemodialysis, who underwent resection of the left lateral segment of the liver followed by an orthotopic auxiliary left lateral segment liver transplantation and kidney transplantation from a deceased donor. The larger part of the donor liver was transplanted in another patient. The postoperative course was marked by an episode of biliary leakage from both the cut surface of the liver and the biliary duct-to-duct left hepatic anastomosis, which was converted to a Roux-en-Y 8 days posttransplant. The serum oxalate dropped from 34.8 before transplant to 3.6-8.3 μmol/L in the first months after transplant to less than 1 μmol/L (normal range 0.4-3.0 μmol/L). One year after transplantation the patient has good renal function, with an iothalamate GFR of 58 ml/min. Conclusions: Orthotopic auxiliary LTX is a good alternative to whole LTX in primary hyperoxaluria. By using a split deceased donor liver, it does not deprive the cadaveric donor pool and protects the recipient from liver failure in case of graft loss. This approach is also attractive in the living donor setting.

Living related liver transplantation in biliary atresia with absent inferior vena cava.

The success of the triangulation technique for hepatic venous anastomosis in left lateral segment liver transplantation has led to standardization of this procedure. We report a case of syndromic biliary atresia with absent inferior vena cava in which we constructed a neo cava to implant a living related left lateral segment graft by using the triangulation technique.

Characterization and treatment of biliary anastomotic stricture after segmental liver transplantation

Background/Purpose: Biliary anastomotic strictures (BAS) after left lateral segment liver transplantation (LLST) may cause graft dysfunction, sepsis, and patient mortality. A review of the authors' experience was performed to better characterize the risk factors and corrective management. Methods: The medical records of 9 children who underwent a LLST in whom a BAS developed from 1989 to the present were reviewed retrospectively. Results: Seventy-five of 199 liver transplants (38%) at the authors' institution since 1989 have been LLST. BAS developed in 12% of these cases. BAS were diagnosed less than 12 months after transplantation in 4 children (mean, 7.5 months; range, 5 to 11 months) and greater than 12 months in 5 children (mean, 37 months; range, 14 to 72 months). Early strictures (<12 months) were associated with hepatic artery thrombosis (n = 1), and posttransplant bile leak (n = 1) and ducts from segment II and III exiting separately from the left lateral segment (n = 2). The diagnosis of BAS was heralded by episodes of liver biopsy-proven cholangitis in all patients and confirmed radiographically. Seven children underwent successful biliary exploration and revision of the hepaticojejunostomy. Two of these children ultimately required retransplantation secondary to chronic graft rejection. Conclusions: BAS in LLST are a source of significant morbidity and should be considered in children after LLST who present with cholangitis. Surgical correction is possible in most cases. J Pediatr Surg 35:940-942. Copyright © 2000 by W.B. Saunders Company.