Percutaneous removal of biliary stones post‐liver transplant in a pediatric patient: Case report and review of the literature

This case report describes an 8‐year‐old girl who underwent a segmental LT for a primary diagnosis of citrullinemia at the age of 12 months. She presented with cholangitis secondary to stenosis of the biliary‐enteric anastomosis. MRI revealed dilatation of intrahepatic bile ducts associated with multiple stones. An endoscopic approach failed to decompress the bile ducts and remove the stones. A percutaneous approach was then undertaken. After placement of a temporary external biliary drain for 12 days, a 26 French sheath was placed to access the bile ducts. Using a 14Fr flexible cystoscope, 80%‐90% of the biliary stones were removed. This was followed by antegrade balloon dilatation of the biliary‐enteric anastomosis. Two months later, the procedure was repeated, resulting in complete clearance of the biliary stones. An internal‐external biliary drain was maintained in placed for 10 months. The patient has been asymptomatic, with no evidence of stone recurrence for 13 months after drain removal. Percutaneous biliary stone removal is commonly performed in adults with non‐transplanted livers, especially in complex cases, and has also been shown to be successful in the pediatric population. However, it is rarely reported in transplanted livers in adults, and to the best of our knowledge, no pediatric cases have been reported. This case illustrates that this technique can be successfully utilized in pediatric LT patients.     

Castleman disease in a pediatric liver transplant recipient: a case report and literature review

Castleman disease is a rare hematologic disorder, closely linked to the HHV-8, and most commonly observed in immunocompromised individuals. Thirteen months following a liver transplant for CPS-1 defect, a 15-month-old boy presented with fevers, anemia, and growth retardation. Abdominal CT scan showed splenomegaly and generalized lymphadenopathy. Histology of chest wall lymph nodes revealed a mixed CD3+ T-cell and CD20+ B-cell population with atretic germinal centers consistent with multicentric Castleman disease. Qualitative DNA PCR detected HHV-8 in the resected lymph node and in the blood, supporting the diagnosis. Immunosuppression was tapered, and he was transitioned from tacrolimus to sirolimus. His graft function remained stable, and repeat imaging showed regression of the lymphadenopathy. The child is living one yr after Castleman disease diagnosis with a well-functioning graft. Castleman disease is a potential complication of solid organ transplant and HHV-8 infection. Reduction in immunosuppression and switch to sirolimus may be an effective strategy to treat this condition.     

Central pontine myelinolysis following pediatric living donor liver transplantation: A case report and review of literature

CPM is one of the most serious neurological complications that can occur after OLT and is characterized by symmetrical demyelinization in the basis pontis. The etiology of CPM remains unclear, although the rapid correction of the serum sodium and CNI concentrations may be associated with the development of CPM. With recent advances in MRI technology, early diagnosis of CPM has become possible. Here, we present the case of a five‐yr‐old female who developed CNI‐associated CPM after undergoing LDLT. A decreased level of consciousness and dysphasia was noted one wk after LDLT, and MRI revealed findings compatible with a diagnosis of CPM. The patient fully recovered from the neurological deficits related to CPM following the switch from the CNI to sirolimus. We propose MRI to be promptly considered for patients with abnormal neurological findings, together with the substitution of CNI with an mTOR inhibitor as a management regimen for CNI‐related CPM.     

Antithrombotic management and thrombosis rates in children post–liver transplantation: A case series and literature review

Thrombosis is a major postoperative complication in pediatric liver transplantation. There is marked heterogeneity in prophylactic antithrombotic therapies used, without established guidelines. This review summarizes current worldwide incidence of thrombotic events and compares antithrombotic therapies in children post–liver transplant, with comparison to our institution's experience. Of the twenty‐three articles with sufficient detail to compare antithrombotic regimens, the overall incidence of thrombosis ranged from 2.4% to 17.3%. Incidence of HAT ranged from 0% to 28.1%, of HVT from 0% to 4.7%, of PVT from 1.5% to 11.2%, and of IVC thrombosis from 0% to 2.8%. Re‐transplantation due to thrombosis ranged from 0% to 4.8%. Prophylactic antithrombotic therapies varied between studies, and bleeding complications were infrequently reported. Since 2010, 96 children underwent 100 liver transplants at our institution with thrombosis incidence comparable to international literature (HAT 6%, PVT 5%, IVC 1%, and HVT 0%). Re‐transplantation due to thrombosis occurred in 2% and major bleeding occurred in 10%. The prophylactic antithrombotic therapies used post–liver transplantation in children remain varied. Low rates of thrombosis have been reported with antiplatelet use both with and without anticoagulation. Standard definitions and consistent reporting of bleeding complications are required, in addition to thrombosis rates, so that true risk‐benefit assessment of reported regimes can be understood.     

Renal graft outcome after combined liver and kidney transplantation in children: UCLA and UNOS experience

de la Cerda F, Jimenez WA, Gjertson DW, Venick R, Tsai E, Ettenger R. Renal graft outcome after combined liver and kidney transplantation in children: UCLA and UNOS experience.
Pediatr Transplantation 2010: 14:459–464. © 2010 John Wiley & Sons A/S. Abstract: Although it has been described in adults that renal grafts in the context of CLKT have a lower number of AR episodes and improved renal allograft survival, this has never been examined in pediatrics. We performed a single center retrospective case–control study examining 10 patients aged 10 ± 6 yr with a CLKT that survived the post‐surgery period of six months, and compared outcomes to a group of 20 KO transplants matched for age, era, and immunosuppression. We observed a significant reduction in the incidence of AR episodes in the CLKT group. To evaluate whether or not this experience was reproducible nationally, we performed an analysis of the 1995–2005 UNOS database. As of March 2007, 111 CLKT and 3798 KO transplants were identified from the OPTN/UNOS data. There was a significant improvement in the late kidney graft survival at five yr post‐transplant in the CLKT group. These findings support the concept that liver transplantation is immunologically protective of the kidney allograft in CLKT.     

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.     

Liver transplantation for hereditary tyrosinemia type I: analysis of the UNOS database

Patients with HT-1 can develop progressive liver disease and have a high incidence of HCC. LT is indicated in patients with fulminant liver failure, HCC or decompensated chronic liver disease refractory to NTBC. To determine the need for LT and outcomes after LT in children with HT-1. Children with HT-1 who had LT between 10/1987 and 5/2008 were identified from the UNOS database. Of 11,467 children in the UNOS database, 125 (1.1%) required LT secondary to HT-1. Mean age at LT was two and half yr (s.d. ± 3.6 yr). Mean age at LT during the first 10 yr of the study (1.82, s.d. ± 2.86 yr) was significantly lower than in the last decade (3.70, s.d. ± 4.42 yr), p = 0.01. Nearly half of the patients (58, 46.4%) were transplanted between 1988 and 1992. Overall, one- and five-yr patient survival was 90.4% and 90.4%, respectively. LT is a valuable option for children with HT-1 with fulminant liver failure or when medical treatment fails. The rate of LT for children with HT-1 has decreased and age at transplant increased over the last decade most probably reflecting the effect of early diagnosis and treatment with NTBC.     

Diaphragmatic hernia in infants following living donor liver transplantation: report of three cases and a review of the literature

DH is a rare complication following LT. This report presents three cases of right-sided DH after LT using a left-sided graft. All of the patients were younger than one yr of age, and they were critically ill owing to their original disease, characterized by biliary atresia, progressive familiar intrahepatic cholestasis, and acute liver failure. DH occurred with sudden onset within three months after LT. All of the cases were promptly diagnosed and treated. A literature review of 24 cases of DH identified four factors associated with DH: left-sided graft, right-sided DH, relatively delayed onset of DH, and age-specific chief complaint. DH following LT should be considered as a potential surgical complication when a left-sided graft is used, especially in small infants with coagulopathy and malnutrition.     

Arterial anastomosis in a pediatric patient receiving a right extended split liver transplant: a case report

Abstract:  We report a case of a pediatric patient who received a right-extended liver transplant. The size of the recipient hepatic artery did not match with the donor right hepatic arterial stump. Moreover, recipient arterial anatomy made the direct anastomosis difficult or at increased risk for complications. The recipient's splenic artery was then mobilized, divided and anastomosed to the donor's right hepatic artery. The spleen was preserved and revascularization through collaterals is demonstrated by Angio CT Scan. Doppler US of the transplanted liver demonstrated good flow through the liver and the patient was discharged with perfect liver function. Splenic artery is perfectly suited for hepatic artery anastomosis. The use of splenic artery is favored in particular situations as in the case of a pediatric recipient receiving a right-extended liver graft with small caliber artery.     

Surgical anterior cavoplasty for managing a case of early acute outflow obstruction after liver transplantation

HVOO following liver transplantation is rarely treated surgically because it tends to debut subacutely. However, acute HVOO is a surgical emergency that compromises the viability of the graft. We report a case of HVOO diagnosed intra‐operatively during surgical revision for a suspected arterial thrombosis in a 10‐month‐old male recipient of a second graft (segments II–III) for familial intrahepatic cholestasis. HVOO was related to a stenosis at the first transplant hepato‐caval anastomosis, left in place to obtain longer venous cuffs for retransplantation. An anterior cavoplasty was necessary to resolve the issue. The new anastomosis was created under total vascular exclusion after gaining control of the supradiaphragmatic vena cava, because the inferior vena cava was unsuitable for further surgery. This approach (normally used as a means to avoid sternotomy in patients with hepatic or renal tumours associated with venous thrombosis) allows adequate vascular control and, in selected cases, offers a surgical alternative for treating HVOO.     

Hepatitis C viral recurrence in a pediatric patient following liver transplantation

Hepatitis C virus (HCV) infections are known to have a more benign course in children than in adults. Although the natural history of HCV recurrence after liver transplantation has been well studied in adult patients, much less is known about HCV recurrence after liver transplantation in pediatric patients. Herein, we report a case of a pediatric patient with HCV presumably acquired through vertical transmission. She underwent liver transplantation at 14 yr of age. The first three yr after liver transplantation were uneventful. However, in the past 12 months she has been hospitalized twice after developing ascites, hematemesis and esophagogastroduodenoscopy (EGD)-documented esophageal varices. Post-transplant biopsy has demonstrated chronic inflammation complicated with active hepatitis C and stage 2-3 scarring. This case report demonstrates the need for further epidemiologic studies to study the natural history of the rate of HCV recurrence after liver transplantation in the pediatric population.     

A case of pediatric live‐donor liver transplantation with a left lateral segment reduction by a linear stapler after reperfusion

In pediatric LDLT, graft reduction is sometimes required because of the graft size mismatch. Dividing the portal triad and hepatic veins with a linear stapler is a rapid and safe method of reduction. We herein present a case with a left lateral segment reduction achieved using a linear stapler after reperfusion in pediatric LDLT. The patient was a male who had previously undergone Kasai procedure for biliary atresia. We performed the LDLT with his father's lateral segment. According to the pre‐operative volumetry, the GV/SLV ratio was 102.5%. As the patient's PV was narrow, sclerotic and thick, we decided to put an interposition with the IMV graft of the donor between the confluence and the graft PV. The graft PV was anastomosed to the IMV graft. The warm ischemic time was 34 min, and the cold ischemic time was 82 min. The ratio of the graft size to the recipient weight (G/R ratio) was 4.2%. After reperfusion, we found that the graft had poor perfusion and decided to reduce the graft size. We noted good perfusion in the residual area after the lateral edge was clamped with an intestinal clamp. The liver tissue was sufficiently fractured with an intestinal clamp and then was divided with a linear stapler. The final G/R ratio was 3.6%. The total length of the operation was 12 h and 20 min. The amount of blood lost was 430 mL. No surgical complications, including post‐operative hemorrhage and bile leakage, were encountered. We believe that using the linear stapler decreased the duration of the operation and was an acceptable technique for reducing the graft after reperfusion.     

The role of liver transplantation for hepatic adenomatosis in the pediatric population: Case report and review of the literature

Wellen JR, Anderson CD, Doyle M, Shenoy S, Nadler M, Turmelle Y, Shepherd R, Chapman WC, Lowell JA. The role of liver transplantation for hepatic adenomatosis in the pediatric population: Case report and review of the literature.
Pediatr Transplantation 2010: 14: E16–E19. © 2009 John Wiley & Sons A/S. Abstract: Hepatic adenomas are benign lesions often found in young women during childbearing age. These tumors are often solitary but can also be multiple in which case this is referred to as hepatic adenomatosis (HA). HA is defined as having greater than or equal to ten adenomas within an otherwise normal liver. We present a case of a teenager with HA who underwent an orthotopic liver transplant for complications of her HA. To date there are only four reports of teenagers, without an underlying glycogen storage disease, who have undergone a liver transplant for HA. Liver transplantation within the pediatric population is an acceptable treatment for HA that are deemed unresectable.     

Embolization of congenital portosystemic shunt presenting after pediatric liver transplantation: Case report and literature review

This case report describes a 13‐year 10‐month‐old girl who underwent a deceased‐donor split LT for primary diagnosis of biliary atresia at the age of 12 months, who presented with a lower GI bleed. Ultrasound and CT revealed a venous vascular anomaly involving the cecum and ascending colon, with communication of the SMV and pelvic veins consistent with a CEPS. Associated varices were noted in the pelvis along the uterus and urinary bladder. These findings were confirmed by trans‐hepatic porto‐venography, which was diagnostic and therapeutic as a successful embolization of the CEPS was performed using micro‐coils. There were no complications following the procedure and no further GI bleeding occurred, illustrating the efficacy of this treatment option for CEPS. We discuss the literature regarding the presenting complaint of GI bleeding post–LT, CEPS as a rare cause of GI bleeding and its association with PV, and the classification and treatment of CEPS.     

Pediatric liver transplantation in Iran: evaluation of the first 50 cases

LT is nowadays accepted as the definitive therapy for end-stage liver disease. We report our experiences with pediatric LT using grafts from living related and DD. From April 1999 to March 2006, 50 infants and children who underwent LT were studied for pretransplantation status, medical and surgical complications and survival rate. There were 33 (66%) boys and 17 (34%) girls. The mean age of patients was 9.9 +/- 4.8 yr (range: 0.9-17.7) with a mean weight of 33.4 +/- 18.4 kg (range: 7.5-80). The main indications were cryptogenic cirrhosis (30%), autoimmune cirrhosis (24%), followed by biliary atresia (22%), Wilson disease (14%), progressive familial intrahepatic cholestasis (4%), fulminant hepatitis (4%) and tyrosinemia (2%). We used living-related donor in 14 (28%) and split liver in 5 (10%) cases and other patients received whole liver from DD. The mean follow-up of patients was 24.7 +/- 22.6 months (range: 1-72). The main postoperative complications were acute cellular rejection (44%) and infections (30%), whereas chronic rejection was seen in 26% of cases. The mortality rate was 24%. Overall mean survival (76% alive) was 63.5 +/- 5.7, 95% CI: 52.3-74.6. Our results demonstrate that pediatric LT is a feasible undertaking in Iran. Organ shortage in our area led to liberal use of living related and split liver techniques. The overall results of the pediatric LT in Iran are encouraging.     

Lobar displacement following pediatric heart-lung transplantation: case report and review of literature

Lobar displacement (LD) after heart-lung transplantation (HLT) has been reported in adults, but there are no reported pediatric cases. Its occurrence may cause vascular compromise of the displaced lung segment leading to necrosis, infection and bronchiectasis, as well as compression of contralateral lobes. We report two cases of LD in children following HLT, treated differently and with different outcomes. Assessment of pulmonary perfusion and weighing the risk of surgical repair may be considered for optimal patient management of this condition.     

De novo cholangiocarcinoma after liver transplantation in a pediatric patient

Channabasappa N, Johnson‐Welch S, Mittal N. De novo cholangiocarcinoma after liver transplantation in a pediatric patient
Pediatr Transplantation 2010: 14:E110–E114. © 2009 John Wiley & Sons A/S. Abstract: To date, no child has been reported to develop de novo CCA after liver transplantation although patients with transplants have a significantly higher risk of malignancy than the general population. CCA is extremely rare in the pediatric age group, seen mostly in patients with a history of choledochal cysts, Caroli’s disease, or PSC. We report the first case of pediatric de novo CCA in the liver allograft 12 yr after liver transplantation.     

Early recurrence of primary disease after pediatric renal transplantation: two case reports and a review of the literature

Recurrence of primary diseases such as FSGS or HUS is known to cause early graft dysfunction after pediatric renal transplantation. We report the unusual occurrence of early graft dysfunction following kidney transplant in two pediatric cases. Both subjects had biopsy proven recurrence of CGN in less than a week after transplantation. We were able to sustain the renal function in one of them following aggressive treatment. Hence, early recurrence of CGN should be considered in the differential diagnosis of early graft dysfunction.