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.     

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.     

Pneumatosis intestinalis following pediatric live‐related liver transplant: A case report and successful conservative approach

PI has been rarely reported following pediatric live‐related liver transplantation. Such a disorder is characterized by accumulation of gas in the bowel wall. The cause of PI has not been yet established; however, it has been strongly linked with steroid therapy. In this report, we present a case of PI following pediatric live‐related liver transplantation that has been successfully managed conservatively.     

Tacrolimus‐related seizure after pediatric liver transplantation – A single‐center experience

To identify the risk factors for new‐onset seizures after pediatric LT and to assess their clinical implications and long‐term prognosis. The clinical and laboratory data of 27 consecutive children who underwent LT from January 2007 to December 2010 in our center were analyzed retrospectively. Patients were divided into seizures group and a non‐seizures group. Pre‐operative, intra‐operative, and post‐operative data were collected. Seizures occurred in four children, an incidence of 14.8%. All exhibited generalized tonic–clonic seizures within the first two wk after LT. Univariate analysis showed that the risk factors associated with seizures after pediatric LT included gender, pediatric end‐stage liver disease score before surgery, Child–Pugh score before surgery, serum total bilirubin after surgery, and trough TAC level. Multivariate analysis showed that trough TAC level was the only independent risk factor associated with the seizures. All children who experienced seizures survived with good graft function and remained seizure‐free without anti‐epileptic drugs over a mean follow‐up period of 33.7 ± 14.6 months. High trough TAC level was the predominant factor that contributed to seizures in the early post‐operative period after pediatric LT. High PELD and Child‐Pugh scores before LT and high post‐operative serum Tbil may be contributory risk factors for TAC‐related seizures.     

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.     

Centrilobular necrosis as a manifestation of venous outflow block in pediatric malnourished liver transplant recipients – case reports

Gibelli NEM, Tannuri ACA, Andrade WC, Ricardi LRS, Tannuri U. Centrilobular necrosis as a manifestation of venous outflow block in pediatric malnourished liver transplant recipients – case reports. Abstract: CLN is a frequent histological finding in biopsies after pediatric: LT, and its pathogenesis has not yet been fully clarified and has different causes. Among the vascular causes, VOB is sometimes difficult to diagnose, especially when technical variants such as split‐liver, reduced‐liver, or living‐related LT are utilized. Three liver‐transplanted malnourished children (ages 12, 20, and 28 months) developed altered LFTs and post‐operative ascites with right pleural effusion (two cases) and jaundice (one case). Doppler ultrasound examinations were normal and liver biopsies showed CLN interpreted as severe ACR. There were no responses to the medical treatment. Additional investigation with CT angiography suggested obstructed hepatic vein drainage, which was confirmed by interventional radiology and angioplasty of the anastomosis between the hepatic vein and the inferior vena cava, with clinical and histological resolution. It is concluded that in malnourished children undergoing LT with technical variations, in which the occurrence of severe ACR is usually less common because of the severity of the patient condition, the finding of CLN should raise the possibility of VOB, so that excessive immunosuppression and its consequences can be avoided.     

Diaphragmatic hernias after pediatric liver transplantation: Experience of a high‐volume transplant center

Diaphragmatic hernias (DHs) are rare complications after pediatric liver transplantation (PLT). It is now widely accepted that DHs after liver transplantation (LT) is a pediatric related condition. PLTs (under of age 18) performed between January 2013 and June 2019 at Malatya Inonu University Institute of Liver Transplantation were retrospectively scanned. Study group consisting DHs and a control group were compared. Among 280 PLTs, 8 of them were complicated with DHs (%2.9). Median age of the patients with DH was 3.0 (0.8‐9.5) years. Median graft recipient weight ratio was 2.5 (0.9‐4.4). Five patients were below 5th percentiles in terms of pediatric weight growth chart at the time of LT. Also, 6 patients were below 5th percentiles in terms of pediatric height growth chart. There was no statistical difference between study and control groups. There are many risk factors mentioned in literature that may be primarily responsible for DHs after PLT. These factors are left lobe and large‐for‐size grafts, malnutrition, trauma or diathermy of diaphragmatic nerve and vessels and immunosuppressants. In our study, we could not specify any reason that differs in DHs. In our aspect, narrow diaphragma and thorax are exposed to high intra‐abdominal pressure from abdomen. Large‐for‐size grafts, which are specific to children, also may contribute to this affect. Excessive diathermy and trauma to diaphragmatic collaterals may aggravate the risk of DH. More patients are needed to make an exact conclusion, in order to evaluate with comparable study on this aspect.     

Bilateral native nephrectomy reduces systemic oxalate level after combined liver‐kidney transplant: A case report

Primary hyperoxaluria type 1 (PH1) is a rare liver enzymatic defect that causes overproduction of plasma oxalate. Accumulation of oxalate in the kidney and subsequent renal failure are fatal to PH1 patients often in pediatric age. Combined liver and kidney transplantation is the therapy of choice for end‐stage renal disease due to PH1. Levels of plasma oxalate remain elevated for several months after liver transplantation, as the residual body oxalate is slowly excreted. Patients with persistent hyperoxaluria after transplant often require hemodialysis, and accumulation of residual oxalate in the kidney can induce graft dysfunction. As the native kidneys are the main target of calcium oxalate accumulation, we postulated that removal of native kidneys could drastically decrease total body oxalate levels after transplantation. Here, we report a case of bilateral nephrectomy at the time of combined liver‐kidney transplantation in a pediatric PH1 patient. Bilateral nephrectomy induced a rapid decrease in plasma oxalate to normal levels in less than 20 days, compared to the several months reported in the literature. Our results suggest that removal of native kidneys could be an effective strategy to decrease the need for hemodialysis and the risk of renal dysfunction after combined liver‐kidney transplantation in patients with PH1.     

Association of post‐transplant donor‐specific HLA antibody with liver graft fibrosis during long‐term follow‐up after pediatric liver transplantation

The aim of this study was to evaluate the significance of post‐transplant DSA as a predictor of liver fibrosis during long‐term follow‐up after pediatric LT. We evaluated the histological findings in 18 LT recipients who underwent liver biopsy after DSA screening. Liver fibrosis was scored based on the METAVIR fibrosis staging. Patients were divided into 2 groups based on histological findings, and clinical characteristics among patients with liver fibrosis were assessed. Of 18 patients, 7 were included in the fibrosis group. No significant between‐group differences were found regarding peritransplant characteristics, including age, sex, primary disease, ABO incompatibility, and immunosuppressive regimen. Episodes of acute rejection and non‐adherence to immunosuppressive drugs were comparable between both groups. The MFI for anti‐DR DSA and positive rate were significantly higher in the fibrosis group (1655 vs 216; P = .019, 86% vs 27%; P = .012, respectively). MFI for anti‐DQ DSA was higher in the fibrosis group, but non‐significantly (2052 vs 384; P = .46). Post‐transplant anti‐DR DSA is associated with graft fibrosis during long‐term follow‐up. This finding seems useful for the implementation of valid histological examinations of liver grafts for patients with higher MFI, especially for anti‐DR DSA, after pediatric LT.     

Tacrolimus-induced pain syndrome in a pediatric orthotopic liver transplant patient

Post-transplant complications are common among patients receiving immunosuppressive medications, including pain syndromes. Recently, a pain syndrome, calcineurin-inhibitor induced pain syndrome (CIPS) has been described. To our knowledge, this article is the second report of tacrolimus-associated CIPS, and the first report in the pediatric setting.     

Roux‐en‐Y loop varices in children with portal hypertension after liver transplantation: An unusual cause of “obscure” gastrointestinal bleeding

Chu J, Kerkar N, Miloh TA, Rodriguez‐Laiz G, Lewis B, Stangl A, Newton KP, Iyer K, Arnon R. Roux‐en‐Y loop varices in children with portal hypertension after liver transplantation: An unusual cause of “obscure” gastro intestinal bleeding.
Pediatr Transplantation 2011: 15: E156–E161. © 2010 John Wiley & Sons A/S. Abstract: PHALT may result from graft dysfunction, portal vein thrombosis, arterio‐venous fistulas and can lead to GIB, commonly from bleeding esophageal varices. We present three children with GIB requiring multiple blood transfusions that were diagnosed with RY Loop bleeding. Routine EGD, colonoscopy, and CE failed to reveal the bleeding source. However, enteroscopy revealed large varices at the site of hepaticojejunostomy anastomosis in all. Our experience demonstrates that RY loop varices in children with PHALT are a rare and treatable cause of obscure GI bleeding.     

Human herpes virus type 8‐associated Kaposi sarcoma in a pediatric liver transplant recipient

Çeltik C, Ünüvar A, Aydo?an A, Gökçe S, Öztürk G, Güllüo?lu M, Y?lmaz G, Türko?lu S, Anak S, Sökücü S, Durmaz Ö. Human herpes virus type 8‐associated Kaposi sarcoma in a pediatric liver transplant recipient.
Pediatr Transplantation 2011: 15: E100–E104. © 2010 John Wiley & Sons A/S. Abstract: Development of KS in pediatric liver transplant recipients is a rare entity and has dismal prognosis. Latent HHV‐8 infection, immunosuppression, and genetic predisposition are possible etiological factors. Decreasing the dose or cessation of immunosuppressive drugs, switching to sirolimus with antiproliferative and antitumor properties, and different chemotherapeutic regimens are the current therapeutic strategies. We herein report a pediatric liver transplant recipient who developed generalized KS at post‐transplant fifth month. The disease had an aggressive course despite the highly toxic chemotherapy. On the other hand, a prompt and durable response was provided by paclitaxel with tolerable side effects. The patient is now free of disease for at least 24 months and healthy with good graft function under sirolimus therapy as maintenance immunosuppression. Instead of highly toxic chemotherapy, paclitaxel can be used as therapeutic option in cases with generalized disease and in those who are unresponsive to conventional chemotherapy. However, new studies are needed to assess the efficacy of the paclitaxel therapy in KS in the liver transplant recipients.     

Success of chemotherapy and a liver transplant in a pediatric patient with hepatic angiosarcoma: A case report

Hepatic angiosarcoma is an extremely rare diagnosis in children, with fewer than 50 pediatric cases reported in the literature worldwide. This aggressive vascular sarcoma carries a very dismal prognosis and is known to be resistant to radiation, chemotherapy, and other vascular‐targeted agents. Complete surgical resection is felt to provide the best chance for long‐term survival. In patients with tumors not amenable to resection, a liver transplant can be considered. However, very few such transplants have been reported, given that they remain controversial due to high cancer recurrence and mortality post‐transplant. Herein, we report the unique case of a 2‐year‐old child with localized hepatic angiosarcoma not amendable to resection who successfully underwent a liver transplant and received chemotherapy with six cycles of doxorubicin, docetaxel, and ifosfamide.     

Percutaneous interventional management of biliary complications after pediatric liver transplantation: A 16‐year single‐institution experience

The aim of the study was to investigate the BiCx after the pediatric OLT and to assess the efficacy of the fluoroscopic‐guided PBI in the patients with BiCx as compared to the SR. A total of 340 OLTs were performed in 302 patients over the last 16 years. The inclusion criteria were the presence of BS or BL as a complication after OLT. The management of the BiCx was studied. Graft revision, graft loss, and survival were evaluated following PBI and SR. BiCx occurred in 17.1% (58/339) of the transplants; 6.2% (21/339) of transplants demonstrated BL and 12.7% (43/339) of the transplants had BS. Overall graft survival rates at 1 and 3 years in OLT with BL treated with PBI were 75.0% and 68.8% as compared with 75% and 66.7% in OLT treated with SR (P>.05). Overall graft survival rates at 1 and 3 years in OLT with BS treated with PBI were 70.6% and 54.5% as compared with 71.4% and 50% in OLT with SR or ERCP, respectively (P>.05). Based on the results, we conclude that PBI is as effective as SR in patients with the BL and BS after OLT.     

Anesthetic management of a pediatric patient with pulmonary arteriovenous fistula undergoing liver transplantation – a case report

For patients with HPS who require anesthesia for a procedure, HPV should be maintained to prevent worsening hypoxemia. Here, the case of a 9‐yr‐old girl who was scheduled for a living donor liver transplantation is presented. The patient suffered from end‐stage liver disease with HPS due to biliary atresia, which contributed to the development of a diffuse pulmonary AVF. Consequently, anesthetic management of this patient involved two different types of pulmonary shunt. It is important to maintain HPV, not only to prevent worsening of the hypoxia caused by HPS but also to inhibit an increase in PVR that could cause an increase of shunt flow through the pathological fistula. A TIVA technique was performed, and a nitrous oxide inhaler was prepared in case of a possible increase in PVR during the reperfusion period. There were no adverse events during the operation. Thus, anesthesiologists should be aware of the pathophysiological status of HPS and its potential to progress to a pulmonary AVF in order to meticulously determine an anesthesia plan that accounts for the hypoxia and PVR that are associated with HPS.     

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.     

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.     

Hypogammaglobulinemia in pediatric liver transplant recipients

Hypogammaglobulinemia has been reported after solid organ transplantation in adults, however immunoglobulin replacement [intravenous immunoglobulins (IVIG)] is only necessary in a minority of affected patients. We here present three pediatric patients with severe post-transplant hypogammaglobulinemia following liver transplantation (LTx) receiving a cyclosporine-based standard immunosuppression. Patient 1 was transplanted at the age of 10 months for biliary atresia. Eight weeks post-Ltx the serum IgG was 1.7 g/L. Patient 2 was transplanted at the age of 12 yr for acute liver failure. Four weeks post-Ltx the IgG dropped to 2.6 g/L. Patient 3 was transplanted at the age of 4 months for biliary atresia. Ten weeks post-Ltx severe hypogammaglobulinemia (IgG < 1.48 g/L) was diagnosed during a severe infectious complication. Patients 1 and 3 received a steroid bolus therapy for acute graft rejection. All patients had normal IgG concentrations prior to Ltx and lymphocyte subsets were post-operatively in the normal range. There was no extensive loss of protein by ascites. IGIV were replaced in the three patients monthly without further complications. In two of the patients (1 and 3) IVIG therapy was discontinued 8 and 10 months after Ltx when the immunosuppression has been reduced and serum IgG concentrations were found in the normal range without further immunoglobulin replacement. Severe hypogammaglobulinemia is a rare phenomenon following pediatric LTx and seems to be mainly caused by immunosuppressive drugs, however, the exact underlying mechanisms are unclear. A screening for hypogammaglobulinemia is useful after pediatric LTx, especially in patients with an intensified immunosuppression. Moreover, further immunologic research in affected patients is necessary.     

Parenthood in pediatric liver transplant patients

Liver transplantation has become a universally accepted treatment for numerous congenital and acquired hepatic disorders that cause liver failure. Without liver transplantation, patients in their reproductive years are afflicted with oligospermia or azoospermia in men and amenorrhea in women, with infertility being a consequence in both sexes. The aim of this study is to describe our experiences concerning the parenthood of pediatric individuals who are successful recipients of liver transplantations coming into the reproductive years of life. We retrospectively analyzed data of 207 pediatric liver transplanted patients (96 women, 111 men). Among them, three women conceived and delivered four babies, and two men admitted to paternity of two children after they all had been recipients of liver transplants. All female transplant recipients had received tacrolimus-based immunosuppression. Preterm delivery was the most clinically important complication among these patients. Only one of the female patients experienced hypercalcemia during the pregnancy. None had any other complications such as hypertension, preeclampsia, cholestasis, or diabetes. There was no graft insufficiency, rejection, or birth defect. We concluded that maternity and paternity in liver transplant patients show normal outcomes even though this procedure occurs in childhood, and pregnancy did not seem to impair graft function in patients receiving immunosuppressive drugs.     

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.