Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation

Osorio MJ, Bonow A, Bond GJ, Rivera MR, Vaughan KG, Shah A, Shneider BL. Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation.
Pediatr Transplantation 2011: 15: E149–E151. © 2010 John Wiley & Sons A/S. Abstract: A seven‐yr‐old boy presented with persistent oxygen requirement following a respiratory infection. Physical exam was remarkable for orthodeoxia and digital clubbing. Laboratory evaluation showed elevated A‐a oxygen gradient of 48 mmHg and mildly elevated transaminases. Sonography showed a 13 cm multilobulated liver mass and a biopsy revealed histological findings consistent with focal nodular hyperplasia. MAA scan revealed 23% right to left shunting. Abdominal CTA and MRV demonstrated the absence of the intrahepatic portal vein with an extrahepatic portocaval shunt. Abernethy malformation is a rare anomalous intra‐ or extrahepatic communication between portal blood flow and systemic venous return. In rare cases, Abernethy malformation results in HPS. Ours is the sixth case report to describe the co‐existence of these two entities. Surgical correction of anomalous hepatic vasculature or liver transplant is imperative to restoration of lung function and also to prevent progression of possible malignant liver tumors. We describe the second patient with Abernethy and HPS who underwent liver transplant with complete resolution of HPS.     

Incidence,impact, and treatment of portal and hepatic venous complications following pediatric liver transplantation: A single‐center 12 year experience

Heffron TG, Pillen T, Smallwood G, Henry S, Sekar S, Casper K, Solis D, Tang W, Fasola C, Romero R. Incidence, impact, and treatment of portal and hepatic venous complications following pediatric liver transplantation: A single‐center 12 year experience.
Pediatr Transplantation 2010: 14:722–729. © 2010 John Wiley & Sons A/S. Abstract: PVT or PVS and HVOO are known causes of graft and patient loss after pediatric liver transplantation. Increased incidences of these complications have been reported in partial livers including DDSLT or LDLT. From 1997 to 2008, 241 consecutive pediatric patients received 271 hepatic grafts at a single center. Median follow‐up is 1856 days. Surgical technique, demographics, lab values, and radiologic imaging procedures were obtained utilizing OTTR^® to evaluate the relationship of portal and hepatic complications with risk factors, patient and graft survival. Grafts were composed of 115/271 (42.4%) partial livers of which 90 (33.2%) were DDSLT and 25 (9.2%) LDLT. Of 271 patients, 156 (57.6%) received whole‐sized grafts. There were six PVC in five patients with one patient requiring retransplantation (0.34%) and no patient deaths. Utilizing all three hepatic vein orifices on the recipient hepatic vena cava and the donor hepatic vein cut short enables a wide hepatic outflow tract unlikely to twist. None of the 241 patients developed early or late complications of the hepatic vein. None of the last 128 consecutive patients who received 144 grafts over seven and a half yr have developed either early or late complications of the hepatic or portal vein. Partial‐graft actuarial survival was similar to whole‐graft survival (87.2% vs. 85.3% at one yr; 76.6% vs. 80.2 at three yr; p = 0.488). Likewise, patient survival was similar between partial grafts and whole grafts (93.8% vs. 93.1% at one yr; 89.8% vs. 87.2% at three yr; p = 0.688) with median follow‐up of 1822 (±1334) days. Patients receiving partial livers were significantly younger and smaller than patients receiving whole livers (p < 0.001). Portal and hepatic venous complications may have negative effects on patient or graft survival after pediatric liver transplantation. In our series, there was one graft and no patient loss related to portal or hepatic venous complications after pediatric liver transplantation over 12 yr.     

Thrombosed congenital extrahepatic portal vein aneurysm in an infant

Extrahepatic portal vein aneurysm is extremely rare in infants. We report an infant with thrombosed congenital extrahepatic portal vein aneurysm demonstrated on US and CT. Follow-up US after 16 months showed complete regression of the portal vein aneurysm.     

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

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

Combination of tissue expansion and porcine mesh for secondary abdominal wall closure after pediatric liver transplantation

Lafosse A, de Magnee C, Brunati A, Bayet B, Vanwijck R, Manzanares J, Reding R. Combination of tissue expansion and porcine mesh for secondary abdominal wall closure after pediatric liver transplantation. Abstract: We report the case of a two and a half yr boy hospitalized in our Pediatric Transplantation Unit for portal vein thrombosis following liver transplantation. After performing a meso‐Rex shunt, abdominal wall closure was impossible without compressing the portal flow. A combination of two techniques was used to perform the reconstruction of the muscular fasciae and skin layers. The association of tissue expanders and porcine mesh (Surgisis^®) allowed complete abdominal wall closure with good functional and esthetic results. Use of both techniques is a useful alternative for difficult abdominal closure after liver pediatric transplantation.     

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.     

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

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

Meso‐Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: Review and perspectives

de Ville de Goyet J, Lo Zupone C, Grimaldi C, D’Ambrosio G, Candusso M, Torre G, Monti L. Meso‐Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: Review and perspectives. Abstract: Direct portal revascularization can be achieved by interposing a vascular graft between the SMV and the Rex recessus (left portal vein system): the MRB. To review indications and results of the procedure in the setting of pediatric liver transplantation, reports were selected from the English literature. Previously reported series were updated to analyze long‐term outcome. A new series was added and analyzed as a complementary set of cases. A total of 51 cases were analyzed. With a 96% overall patient survival rate and a 100% long‐term patency rate when the IJV is used for the bypass, MRB achieves a very successful physiologic cure of chronic portal hypertension and restores the portal flow into and through the liver graft. It also has been used successfully for primary revascularization of liver grafts, as well as for managing early acute portal vein thrombosis episodes. The use of this procedure in conjunction with other strategies and techniques might be of interest for transplant surgeons, particularly those caring for children.     

Liver transplantation in children with metabolic diseases: The studies of pediatric liver transplantation experience

Arnon R, Kerkar N, Davis MK, Anand R, Yin W, González‐Peralta RP for the SPLIT Research Group. Liver transplantation in children with metabolic diseases: The studies of pediatric liver transplantation experience.
Pediatr Transplantation 2010: 14:796–805. © 2010 John Wiley & Sons A/S. Abstract: Metabolic diseases are the second largest indication for LT in children after BA. There are limited data on the long‐term post‐transplant outcome in this unique group of patients. Therefore, our aim was to assess post‐liver transplant outcomes and to evaluate risk factors for mortality and graft loss in children with metabolic disorders in comparison to those with non‐metabolic diagnoses. We reviewed all patients enrolled in the SPLIT registry. Between 1995 and 2008, 446 of 2997 (14.9%) children enrolled in SPLIT underwent liver transplant for metabolic diseases. One‐yr and five‐yr patient survival for children with metabolic diseases was 94.6% and 88.9% and for those with other diseases 90.7% and 86.1% (log‐rank p = 0.05), respectively. One‐yr and five‐yr graft survival for children with metabolic disorders was 90.8% and 83.8%, and for those with other diseases 85.4% and 78.0% (log‐rank p = 0.005), respectively. Children with metabolic diseases were less likely to experience gastrointestinal complications (5.6% vs. 10.7%, p = 0.001), portal vein thrombosis (2.9% vs. 5.2%, p = 0.04), and reoperations within 30 days post‐transplant (33.4% vs. 37.8%, p = 0.05) than those with other indications. In conclusion, children who underwent liver transplant for metabolic disease had similarly excellent patient survival as, and better graft survival than, those who received a liver allograft for other indications.     

Portal hypertension after combined liver and intestinal transplantation,a diagnostic and therapeutic challenge?

Monbaliu D, Vandersmissen J, De Hertogh G, Van Assche G, Hoffman I, Knops N, Debbaut C, Heye S, Pirenne J, Maleux G. Portal hypertension after combined liver and intestinal transplantation, a diagnostic and therapeutic challenge?
Pediatr Transplantation 2012: 00: 000–000. © 2012 John Wiley & Sons A/S. Abstract: A widely accepted technique to transplant the liver‐bowel bloc is first to perform a piggyback anastomosis of the donor suprahepatic vena cava to the recipient vena cava; second to restore the arterial blood supply through an aortic interposition graft; and third to ensure venous drainage of the native foregut. The venous drainage of the native foregut can be restored through an end‐to‐end portocaval anastomosis between the donor infrahepatic vena cava and the recipient portal vein. Stenosis of this anastomosis can lead to portal hypertension presenting with upper GI congestion, bleeding, and hypersplenism. We report the successful treatment of this complication using an e‐PTFE‐covered stent inserted following balloon angioplasty.     

Multidetector CT findings of splenic artery aneurysm in children with chronic liver disease

BACKGROUND: Splenic artery aneurysm (SAA) is a well-known complication of chronic liver disease and portal hypertension in adults. The incidence of SAA in children undergoing selective hepatic angiography prior to liver transplantation is reported as 4%, but there are few systematic studies. OBJECTIVE: To investigate the SAAs detected by multidetector CT angiography (MDCTA) among children with chronic liver disease. MATERIALS AND METHODS: A total of 124 children (71 girls, 53 boys; mean age 118 months; age range 5 days to 204 months) with chronic liver disease underwent MDCTA to display the vascular anatomy and any vascular complications during the pretransplantation period. Of these children, 23 also underwent coeliac angiography. The digital subtraction angiography (DSA) and MDCTA findings were compared. RESULTS: SAAs were detected in 13 children (10.4%); none was detectable by US. All patients had more than one aneurysm; ten patients had more than three. In all except one patient, the SAAs were located only in the intraparenchymal branches of the splenic artery; in one patient they were located in the intraparenchymal segment and in the distal third of the splenic artery. The mean size of the aneurysms was 6.5 mm (range 2.5-18 mm). All patients with aneurysms had splenomegaly and vascular collaterals. Nine of the children with SAAs had portal vein pathologies (two occlusions, two stenoses, five dilatations). A statistically significant difference existed with regard to the size of spleen (P < 0.05) and patient age (P < 0.05) between children with SAAs and children without SAAs. There was an increased risk of SAAs in patients with portal vein pathologies. In 19 patients without SAAs on MDCTA, no SAAs were seen on DSA. CONCLUSIONS: It is likely that the incidence of SAA in children with chronic liver disease will increase with improved survival of children with long-standing portal hypertension and chronic liver disease. MDCTA with multiplanar reconstruction is a noninvasive and effective means of imaging paediatric patients with SAAs, especially during the peritransplantation period, which is considered to be a time of significant risk for SAA rupture in this patient population.     

Congenital portocaval shunt

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

Decreased portal vein flow during Kawasaki disease in a liver transplant patient

Abnormalities of liver function tests are frequently documented in patients with Kawasaki disease, but the mechanism responsible for this has not yet been established. Described herein is the case of a 1‐year‐10‐month‐old girl who underwent liver transplantation at 11 months of age. Eleven months after transplantation the patient was diagnosed with Kawasaki disease, which was associated with some portal flow reduction, and received i.v. immunoglobulin, after which fever abated with improvement of portal flow to its pre‐fever level. Abnormalities of liver function tests in Kawasaki disease patients may occur as a result of inflammation of both the biliary and portal systems. There are no reports on the potential relationship between Kawasaki disease and the portal vein, and accumulation of further data is necessary to better examine this relationship.     

A review of abdominal organ transplantation in cystic fibrosis

Lu BR, Esquivel CO. A review of abdominal organ transplantation in cystic fibrosis.
Pediatr Transplantation 2010: 14:954–960. © 2010 John Wiley & Sons A/S. Abstract: With advances in medical treatments, patients with CF are having improved quality of life and living longer. Although pulmonary disease is still the leading cause of morbidity and mortality, this longevity has allowed for the development of other organ dysfunction, mainly liver and pancreas. This review discusses the abdominal organ complications and the role of abdominal organ transplantation in CF. Liver failure and portal hypertension complications are the most common indicators for liver transplantation in CF, and five‐yr survival for isolated liver transplantation is >80%. Deficiency of pancreatic enzymes is almost universal and up to 40% of patients with CF can develop insulin‐dependent diabetes, although the role of pancreas transplantation is less clear and needs further research. Finally, the need for lung transplantation should always be assessed and considered in combination with liver transplantation on a case‐by‐case basis.     

Follicular carcinoma of thyroid following successful liver transplantation – A report

Dantuluri S, Urs A, Karthik SV. Follicular carcinoma of thyroid following successful liver transplantation – A report. Abstract: Follicular carcinoma of the thyroid is a relatively rare malignancy in childhood even in paediatric solid organ transplant recipients. The risk of developing de novo malignancies after liver transplantation is higher compared to the general population. We report an 18‐yr‐old girl who had successfully undergone liver transplantation five yr earlier for neonatal sclerosing cholangitis complicated by the development of dysplastic nodules. Baseline immunosuppression was with tacrolimus and prednisolone. Mycophenolate mofetil was later added in view of steroid‐resistant episodes of graft rejection. She subsequently suffered from marked obesity and essential hypertension needing antihypertensive medication. Five yr after liver transplantation, she presented with a right‐sided thyroid swelling that was rapidly progressive with no associated lymphadenopathy and normal systemic examination. Ultrasound of her neck revealed a solid lesion in the right lobe of the thyroid gland with ill‐defined margins, and a diagnostic right thyroid lobectomy confirmed the diagnosis of follicular carcinoma with focal capsular and vascular invasion. She underwent total thyroidectomy and currently remains well on thyroxine supplements. Our report highlights the need for high level of suspicion and prompt investigation into any abnormal lesion in the long‐term follow‐up of solid organ transplant recipients.     

Primary HHV 6 infection after liver transplantation with acute graft rejection and multi‐organ failure: Successful treatment with a 2.5‐fold dose of cidofovir and reduction of immunosuppression

Dohna‐Schwake C, Fiedler M, Gierenz N, Gerner P, Ballauf A, Breddemann A, Läer S, Baba H. A, Hoyer P. F. Primary HHV 6 infection after liver transplantation with acute graft rejection and multi‐organ failure: Successful treatment with a 2.5‐fold dose of cidofovir and reduction of immunosuppression.
Pediatr Transplantation 2011: 15: E126–E129. © 2010 John Wiley & Sons A/S. Abstract: HHV type 6 has been reported with enhanced pathogenicity in immunocompromised patients. Herein, we report about a two‐yr‐old girl who experienced primary HHV 6 infection after liver transplantation. She clinically presented with graft rejection and necrotic hepatitis as well as high fever, pneumonitis with respiratory failure and a rash. Therapy with cidofovir of 5 mg/kg per wk did not show improvement, so that a full pharmacokinetic profile of cidofovir was performed. It demonstrated enhanced body weight normalized clearance of cidofovir and cidofovir dosage was augmented to 12 mg/kg per wk to reach adequate drug exposure. With additional reduction of immunosuppression, the patient dramatically improved and liver function stabilized.     

Development of liver size and perfusion after reduced-size liver transplantation in children

The technique of segmental liver transplantation (s-LTx) provides a method to overcome the shortage of suitable livers for small recipients. Patient survival rates are parallel to those obtained with whole liver transplantation (w-LTx). For long-term rehabilitation, adaptive liver growth and adequate perfusion is crucial; however, morphometric and hemodynamic parameters in growing children with s-LTx are not available. Seventeen children who received a s-LTx and 25 with a w-LTx who had follow-up evaluation 1 and 2 yr after LTx were studied. Mean age at time of transplantation was 4.3 +/- 3.5 yr for s-LTx and 10.3 +/- 6.0 yr for w-LTx, mean height 98 +/- 21 cm and 122 +/- 30 cm respectively. At follow-up evaluation mean values for liver enzymes, bilirubin and prothrombin time were in the normal ranges for both groups. Liver dimensions were measured by gray scale ultrasound, and hemodynamic parameters by Doppler sonography in the portal vein and hepatic artery using an Acuson 128 machine. Maximal (Vmax), minimal (Vmin) and time-average velocity (TAV) were measured and the resistive index (RI) calculated. We found that 1 and 2 yr after LTx liver dimensions were at a mean in the upper normal range of healthy controls. Spleen size was above the normal range and did not show any tendency towards regression. Mean Vmax in the hepatic artery in s-LTx and w-LTx was 48 cm/sec vs. 28 cm/sec after 1 yr and 30 cm/sec vs. 35 cm/sec after 2 yr, the RI 0.66 vs. 0.55 and 0.59 vs. 0.73, respectively (p for all parameters > 0.05). Maximal portal vein flow was 25 cm/sec in s-LTx vs. 29 cm/sec in w-LTx. Blood flow calculated by vessel diameter and TAV showed no statistical difference between both groups. In conclusion, liver size after s-LTx and w-LTx was increased to the upper normal range, and portal vein blood flow velocities were within the normal range. Vmax in the hepatic artery was reduced in s-LTx; however, the reduction was to the same extent as in w-LTx. In the view of long-term functional adaptation, s-LTx is not inferior to w-LTx.     

Liver transplantation in a child with liver failure due to chronic graft-versus-host disease after allogeneic hematopoietic stem cell transplantation from the same unrelated living donor

Englert C, Ganschow R. Liver transplantation in a child with liver failure due to chronic graft‐versus‐host disease after allogeneic hematopoietic stem cell transplantation from the same unrelated living donor. Abstract: We report a case of a six‐yr‐old boy who developed chronic GVHD of the liver, intestines, and skin following allogeneic hematopoietic SCT. The boy received an allogeneic hematopoietic stem cell transplant at the age of two yr because of early recurrence of ALL. Chimerism analysis showed complete chimerism. In the following year, he developed GVHD despite adequate immunosuppressive therapy. Liver biopsy showed liver GVHD resulting in liver cirrhosis by the age of five yr. LTx was performed with a left liver lobe from the unrelated donor from whom the stem cells had been taken. Immunosuppressive therapy consisted of low‐dose steroids and low‐dose cyclosporine. The postoperative course was uneventful. Graft function was excellent, and we performed protocol biopsies at seven days and three wk as well as three, six, and nine months after transplantation; none of these showed any signs of rejection or GVHD. Immunosuppressive therapy was discontinued nine months after LTx. Three yr after transplantation, the boy is in good condition with normal graft function. To our knowledge, this is the first report on LTx following allogeneic hematopoietic SCT from the same unrelated living donor.     

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

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