Percutaneous recanalization of an occluded hepatico-jejunostomy, using Colapinto needle, in a two-yr-old patient after partial liver transplantation

We report one case of percutaneous recanalization of an hepatico-jejunostomy, using Colapinto needle, in a two-yr-old patient after partial liver transplantation and occlusive anastomotic biliary stricture non-crossable with conventional interventional radiology techniques. The procedure was successfully performed and followed by biliary trans-anastomotic catheter placement. Later, the patient underwent multiple session of conventional percutaneous balloon dilatation of the anastomosis as a good flow of contrast from bile ducts to bowel loops was achieved. The patient is now without percutaneous biliary catheter since 10 months in good general conditions and with good liver function tests.     

Portosystemic shunt as a bridge to liver transplantation in infants: A comparison of two techniques

Portosystemic shunts can serve as a bridge to liver transplantation in patients with end‐stage liver disease by providing portal decompression to treat life‐threatening variceal bleeding and prevent recurrent episodes until an organ becomes available. The conventional TIPS procedure, however, is technically challenging to perform in infants due to the small size of their intrahepatic vasculature. We report two cases of emergent creation of portosystemic shunts as a bridge to liver transplantation in infants with life‐threatening variceal bleeding using a conventional TIPS technique in the first case and a percutaneous DIPS technique in the other. Both procedures were successful at reducing the portosystemic pressure gradient and preventing further variceal bleeds until a liver transplant could be performed. The novel percutaneous DIPS procedure is a valuable alternative to the conventional TIPS in infants, as it is better suited for small or challenging intrahepatic vascular anatomy.     

Hemodynamic failure as an indication to urgent liver transplantation in infants with giant hepatic hemangiomas or vascular malformations--report of four cases

Abstract:  The aim of this study was to present acute hemodynamic failure as a rare indication for liver transplantation in neonates and infants with liver hemangiomatosis. We report four patients aged one to six months with giant liver hemangiomas, with huge arterio-venous shunting within these malformations. In three, many skin hemangiomas were found. All children developed right ventricular failure. In two, a trial of pharmacological reduction was attempted with corticosteroids and cyclophosphamide. In one patient, the arterio-venous fistulas were embolized without any improvement in hemodynamic status. Two children underwent rescue hepatic artery surgical ligation, which did not prevent heart and then multiorgan failure including liver failure. After unsuccessful conventional therapy, all infants were considered for urgent liver transplantation; in three cases, it was performed with a living-related donor, and in one case with a deceased donor. All patients are alive and well with the follow-up between nine and 37 months after transplantation. Liver transplantation should be considered as a rescue treatment in children with hepatic vascular malformations leading to hemodynamic insufficiency when conventional therapy is unsuccessful and multiorgan failure develops.     

CT in the staging of children with malignant tumours

The application of CT whole-body scanning as an adjunct in the staging investigations was evaluated in 56 children with various types of extracranial solid malignant tumours. CT proved to be superior to conventional films in the detection of pulmonary or pleural dissimination. The abdominal lymph nodes were difficult to evaluate by CT, and abnormal nodes could only be diagnosed when they were unequivocally enlarged. The role of CT in the detection of liver metastases could not be established in this study due to the rare occurrence of liver involvement. CT seemed to be a valuable method in the prediction of the operability of the primary tumour, but seemed to have an inability to show local invasiveness of the tumour.CT is an important supplement to conventional, noninvasive methods in the staging of these patients because CT is able to detect otherwise undetectable dissimination and this results in an improved therapeutic approach in individual cases.     

Gaucher's disease

We report our observations made by conventional radiography, ultrasound, computerized tomography (CT), and magnetic resonance imaging (MRI) on a 3 1/2-year-old girl with Gaucher's disease. The interest of the case consists in the exceptional lungs involvement, the demonstration by MRI of the bone marrow involvement and the necrosis and fibrosis of the liver, as shown by CT. This liver complication has been previously reported only once.     

Meso-Rex shunt for immediate portal revascularization in pediatric liver transplantation: first report

We describe the case of a 13-month-old girl transplanted for biliary atresia with PV hypoplasia. She received the left liver lobe of her mother and presented intraoperative portal thrombosis. Because of technical reasons, the opportunity to have conventional PV reconstruction using the donor left PV stump was lost. Immediate conversion to a meso-Rex shunt, using the recipient jugular vein as a bridge between the superior mesenteric vein and the graft Rex recessus, allowed excellent portal revascularization of the transplant. We suggest that synchronous meso-Rex shunt may constitute a valid alternative to truncal PV anastomosis during pediatric LT.     

Graft reduction using a powered stapler in pediatric living donor liver transplantation

Large‐for‐size syndrome is defined by inadequate tissue oxygenation, which results in vascular complications and graft compression after abdominal closure in living donor liver transplantation recipients. An accurate graft reduction that matches the optimal liver volume for the recipient is essential. We herein initially present the feasibility and safety of graft reduction using a powered stapler to obtain an optimal graft size. From October 1996 to October 2015, a total of eight graft reductions were performed using a powered stapler (group A; n=4) or by the conventional method using a cavitron ultrasonic surgical aspirator and portal triad suturing (group B; n=4). The background, intraoperative findings and the post‐operative outcomes of these eight patients were retrospectively investigated. There were no statistically significant differences in the background of the patients in the two groups. Graft reduction was successfully achieved without any intraoperative complications in group A, whereas intraoperative complications, such as bleeding and bile leakage, occurred in two patients of group B. No post‐operative surgical complications were detected on computed tomography; moreover, the serum aspartate aminotransferase level normalized significantly earlier in group A (P<.05). In summary, graft reduction using a powered stapler was feasible and safe in comparison with the conventional method.     

Living related liver transplant following bone marrow transplantation from same donor: Long‐term survival without immunosuppression

Granot E, Loewenthal R, Jakobovich E, Gazit E, Sokal E, Reding R. Living related liver transplant following bone marrow transplantation from same donor: Long‐term survival without immunosuppression.
Pediatr Transplantation 2012: 16: E1–E4. © 2010 John Wiley & Sons A/S. Abstract: We report long‐term (seven yr) immunological tolerance in a 16‐yr‐old boy, to a liver allograft donated by his father following a bone marrow transplant at age 2.5 yr from the same donor. The bone marrow transplant was complicated by severe GVHD leading to liver failure and the ensuing need for a liver transplant, performed under planned avoidance of immunosuppression. At one wk post‐transplant, although a liver biopsy was histologically compatible with acute rejection, favorable clinical and biochemical evolution precluded initiating immunosuppressive therapy, thus highlighting the need for caution when interpreting early histological changes so that administration of unnecessary immunosuppression can be avoided. Induction of tolerance in transplant recipients remains an elusive goal. In those patients who had received conventional bone marrow transplants and had endured the consequences of GVHD, development of macrochimerism may allow immunosuppression‐free solid organ transplantation from the same donor.     

Considering complementary and alternative medicine alternatives in cases of life-threatening illness: applying the best-interests test

In this article we explore decision-making about treatment when a child faces a life-threatening illness but conventional treatment presents substantial risk and uncertain benefit. When is it acceptable for parents to decide to use complementary and alternative medicine as an alternative, rather than a complement, to conventional care? We use the example of a young child suffering from progressive glycogen storage disease, for whom liver transplant offers the only prospect of a cure. Without a liver transplant, the disease usually results in death within a few years. However, experience using transplant to treat this illness has been limited, success is far from ensured, and the risks (including death and continued progression of the disease) are substantial. The child's parents, who are first-generation immigrants, consider the risks of the transplant unjustified because it still does not offer good prospects for a healthy future. They believe that traditional Chinese medicine could help remediate their daughter's disease. In the article we (1) review parents' obligation to make treatment decisions in the best interests of their child, (2) explain limits on parents' decision-making authority, (3) explore how "best interests" are determined, focusing on cases of serious illness for which conventional treatment is risky and benefit is possible but uncertain, (4) explain the standard of care that physicians must meet in advising about treatment, and (5) outline factors that clinicians and parents should take into account when making decisions.     

The value of cholinesterase activity after Kasai operation

Cholinesterase (ChE) is an enzyme synthesized in the liver. The aim of this study was to determine the value of ChE as an index of liver function. We measured the ChE activity as well as the values of bilirubin, alkaline phosphatase, gamma-glutamyl transpeptidase, aminotransferases and albumin before and 7 days after Kasai operation in 25 infants with biliary atresia. The increased activity of ChE in plasma after Kasai operation was accompanied by a decrease of other measured values (P<0.0001), except for albumin. We can conclude that the increase of ChE activity together with the decrease of bilirubin, alkaline phosphatase and gamma-glutamyl transpeptidase show early improvement of liver function after Kasai operation. ChE activity can be used to assess liver function in terms of synthesis.     

Pre- and post-transplant assessment of liver function in paediatric liver transplantation

The pre-operative risk of paediatric liver transplantation candidates (n=41) was assessed in a prospective study by means of clinical symptoms, conventional static and liver blood flow dependent dynamic liver function tests. Nine patients died during the 365-day waiting period. The data were subjected as covariates to a survival analysis in the Cox proportional hazards model. There was a significant relationsship between the results of mono-ethylglycinexylidide (MEGX) formation and ICG test and the 365-day survival rate. In the stepwise analysis, none of the remaining parameters improved the predictive ability when added to the dynamic liver function test results. The assessment of post-transplantation liver function was studied in 27 patients during the first 28 postoperative-day period. In addition, liver function was studied in a cross-sectional study 1–7 years after successful liver transplantation in children with complete or partial rehabilitation. In the early postoperative period severe organ damage was indicated by both static and dynamic liver function tests. In the later course after transplantation no deterioration of liver function measured with MEGX formation was to be observed. These findings demonstrate the usefulness of dynamic liver function tests in the pre- and post-transplant assessment of liver function.     

Liver involvement in Langerhans cell histiocytosis

Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient.     

Neonatal liver abscesses due to Candida infection effectively treated with caspofungin

Candidiasis is relatively frequent in neonatal and pediatric intensive care units (ICUs), particularly in preterm infants less than 28 weeks of gestational age. Neonatal candidiasis shows high mortality and is often associated to poor neurodevelopmental prognosis in survivor patients. Amphotericin B and fluconazole are the first choice drugs for the treatment of neonatal candidiasis. Caspofungin is an alternative antifungal agent, which is recommended for invasive candidiasis in adults, but has been poorly experienced in neonates and infants as far as now. We report the first two infants with Candida liver abscesses treated with caspofungin. In the first infant bloodstream and liver lesions were cleared by combination therapy with fluconazole, liposomal amphotericin and caspofungin, while in the second one by caspofungin alone.
Conclusion: Our observations confirm the efficacy and tolerability of caspofungin in the treatment of neonatal candidiasis refractory to conventional antifungal drugs. More extensive data are recommended in order to asses a specific neonatal schedule.     

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.     

Primary and secondary amenorrhea associated with spironolactone therapy in chronic liver disease.

Women with severe liver disease often have amenorrhea that resolves as liver disease abates. We describe three patients with mild to moderate chronic liver disease and amenorrhea. In each case amenorrhea resolved when spironolactone therapy was discontinued. We suggest that spironolactone, an androgen inhibitor, may also cause reversible amenorrhea.     

Reversal of parenteral nutrition-associated liver disease in two infants with short bowel syndrome using parenteral fish oil: implications for future management

Here we report the reversal of cholestasis in 2 infants with intestinal failure and parenteral nutrition-associated liver disease. Treatment involved the substitution of a conventional intravenous fat emulsion with one containing primarily omega-3 fatty acids. Biochemical tests of liver function improved significantly. One child was removed from the liver transplantation list because of improved hepatic function, and the second child had complete resolution of cholestasis while solely on parenteral nutrition. This suggests that fat emulsions made from fish oils may be an effective means of treating and preventing this often-fatal condition. A randomized, controlled trial is necessary to study the efficacy of this new approach to parenteral nutrition-associated liver disease.     

A TECHNIQUE FOR THE ENZYMATIC DIAGNOSIS OF GLYCOGEN STORAGE DISEASE ON VERY SMALL TISSUE SPECIMENS

The difficulties to obtain an exact diagnosis in cases of glycogen storage disease are mentioned. New diagnostic methods, utilizing blood cells, have not allowed settling a definite diagnosis in all cases. Analyses on liver tissue, consequently, are still needed in many cases. However, the methods up to now used for obtaining a piece of liver tissue (surgery or large-diameter needle-biopsy) involve dangers to the patient. Because of this, a less risky biopsy procedure, such as can be performed with a fine-needle, was thought preferable. To be able to utilize very small amounts of liver tissue, as obtainable with a fine-needle, for the diagnosis of glycogen storage disease, methods were worked out, allowing the analysis of glucose-6-phosphatase, phosphorylase, amylo-1,6-glucosidase, α-glucosidase, glycogen and protein on 0.5-2 mg fragments of liver tissue. The metnods involved incubation of very small volumes, assay of phosphorus by a newly described, highly sensitive method and of glucose by the hexokinase-glucose-6-phosphate dehydrogenase method with fluorimetric measurement of the NADPH. Protein and glycogen could be assayed in a more conventional way. The stability of the enzyme activities and the precision of the methods is discussed. Results on liver biopsy specimens from seven controls and on biopsy and autopsy specimens from liver in eight cases of four different types of glycogen storage disease are given and compared to the results on these specimens with earlier described methods.     

Hepatic cell transplantation in childhood. State of the art

To date, children who suffer from a certain type of illness such as hepatic failure, could benefit with a non conventional functional replacement as an alternative to liver transplantation. Deterioration and death of patients on waiting list encourage the search for alternatives methods within transplantation. Liver cell transplantation has become a potential alternative treatment whose validation as an alternative or as a bridge until the donor appears, will probably contribute to improve the quality of life and survival of the patients. The aim of this review is to describe the state of the art of hepatocyte transplantation in pediatric patients.     

Hepatitis B virus DNA. Detection with polymerase chain reaction in liver tissue of children with chronic hepatitis B]

BACKGROUND: Detection of hepatitis B virus DNA is a reliable evidence of the presence of the viral agent and its replication. Conventional hybridization techniques are limited to detect about 30,000 virions. With the polymerase chain reaction it became possible to extend the sensitivity by amplification of viral sequences. In our study we intended to test whether viral sequences could be found in liver tissue specimens negative for hepatitis B virus DNA by conventional hybridization techniques. METHODS: Hepatitis B virus DNA was detected by PCR in liver tissue of 37 children with chronic hepatitis B, negative for hepatitis B virus DNA by Southern blot hybridization. PCR was performed in a thermal cycler using Taq-polymerase and oligonucleotide primers within the hepatitis B core region. Hepatitis B virus DNA was visualized by ethidium bromide staining and subsequent Southern blot hybridization. RESULTS: 20 patients were HBeAg- and 17 anti-HBe-seropositive. Viral sequences were present in each of the 20 HBeAg positive HBsAg carriers and in 10 patients with anti-HBe. No hepatitis B virus DNA could be found in 7 children, all of them positive for anti-HBe. CONCLUSIONS: Our results confirm polymerase chain reaction to be a more sensitive method to detect hepatitis B virus DNA in the liver compared with conventional hybridization techniques. Every HBeAg positive carrier as well as the majority of anti-HBe positive patients present viral DNA in their liver. Polymerase chain reaction will be suitable to monitor viral replication in spontaneous course and treated patients.     

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

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