The outcomes of pediatric living donor liver transplantation using small-for-size grafts: experience of a single institute

Purpose

We aimed to evaluate patients who had undergone pediatric LDLT with small-for-size graft (SFSG) and identify risk factors of graft failure to establish a preoperative graft selection strategy.

Methods

The data was collected retrospectively. SFSG was used in 14LDLTs (5.7 %) of 245 LDLTs performed between May 2001 and March 2014. The mean patient age and body weight at LDLT were 12.6 ± 2.0 years and 40.5 ± 9.9 kg, respectively. The graft type was left lobe in six patients, left + caudate lobe in seven patients, and posterior segment in one patient.

Results

The graft survival rates in SFSG and non-SFSG groups were 78.9 and 93.1 %, respectively (p = 0.045). In the univariate analysis, bleeding volume during LDLT were an independent risk factors for graft failure (p = 0.011). Graft failure was caused by sepsis in all three patients and occurred at a median of 70 postoperative days 70 (range 14–88 days). Among them, two cases showed high preoperative PELD/MELD score (PELD; 19.4 and MELD; 22, respectively).

Conclusions

Pediatric LDLT using SFSG had poor outcome and prognosis, especially when it accompanies the surgical infectious complications with preoperative high PELD/MELD scores.

     

Transition of Spleen Volume Long After Pediatric Living Donor Liver Transplantation for Biliary Atresia

Purpose

After undergoing the Kasai procedure for biliary atresia (BA), most patients develop severe splenomegaly that tends to be improved by liver transplantation. However, fluctuations in splenic volume long after transplantation remain to be elucidated.

Maternal grafts protect daughter recipients from acute cellular rejection after pediatric living donor liver transplantation for biliary atresia

Some studies have found that gender mismatch between donors and recipients are related to poor graft prognosis after liver transplantation. However, few studies have investigated the impact of gender mismatch on acute cellular rejection (ACR) in pediatric living donor liver transplantation (LDLT). This retrospective study investigated the clinical significance of these factors in ACR after pediatric LDLT. Between November 2001 and February 2012, 114 LDLTs were performed for recipients with biliary atresia (BA) using parental grafts. We performed univariate and multivariate analyses to identify the factors associated with ACR. The donor–recipient classifications included mother donor to daughter recipient (MD; n = 43), mother to son (n = 18), father to daughter (FD; n = 33), and father to son (n = 20) groups. The overall incidence rate of ACR in the recipients was 36.8%. Multivariate analysis showed that gender mismatch alone was an independent risk factor for ACR (P = 0.012). The FD group had a higher incidence of ACR than the MD group (P = 0.002). In LDLT, paternal grafts with gender mismatch were associated with a higher increased incidence of ACR than maternal grafts with gender match. Our findings support the possibility that maternal antigens may have an important clinical impact on graft tolerance in LDLT for patients with BA.     

Long-term Outcomes of Hypofractionated Stereotactic Radiotherapy for the Treatment of Perioptic Nonfunctioning Pituitary Adenomas

The efficacy of stereotactic radiotherapy (SRT) has been well established for postoperative residual and recurrent nonfunctioning pituitary adenomas (NFPAs). However, the risk of visual impairment due to SRT for lesions adjacent to the optic pathways remains a topic of debate. Herein, we evaluated the long-term clinical outcomes of hypofractionated stereotactic radiotherapy (HFSRT) for perioptic NFPAs. From December 2002 to November 2015, 32 patients (18 males and 14 females; median age 63 years; range, 36–83 years) with residual or recurrent NFPAs abutting or displacing the optic nerve and/or chiasm (ONC) were treated with HFSRT. The median marginal dose was 31.3 Gy (range, 17.2–39.6) in 8 fractions (range, 6–15). Magnetic resonance imaging (MRI) and visual and hormonal examinations were performed before and after HFSRT. The median follow-up period was 99.5 months (range, 9–191). According to MRI findings at the last follow-up, the tumor size had decreased in 28 (88%) of 32 patients, was unchanged in 3 (9%), and had increased in 1 (3%). The successful tumor size control rate was 97%. Visual functions remained unchanged in 19 (60%) out of 32 patients, improved in 11 (34%), and deteriorated in 2 (6%). Two patients had deteriorated visual functions; no complications occurred because of the HFSRT. One patient developed hypopituitarism that required hormone replacement therapy. The result of this long-term follow-up study suggests that HFSRT is safe and effective for the treatment of NFPAs occurring adjacent to the ONC.     

Protocol liver biopsy is the only examination that can detect mid-term graft fibrosis after pediatric liver transplantation

AIM:To assessed the clinical significance of protocol liver biopsy(PLB)in pediatric liver transplantation(LT).METHODS:Between July 2008 and August 2012,89and 55 PLBs were performed in pediatric patients at two and five years after LT,respectively.We assessed the histopathological findings using the Metavir scoring system,including activity(A)and fibrosis(F),and we identified factors associated with scores of≥A1 and≥F1.Our results clarified the timing and effectiveness of PLB.RESULTS:The incidences of scores of≥A1 and≥F1 were 24.7%and 24.7%,respectively,at two years after LT and 42.3%and 34.5%,respectively,at five years.Independent risk factors in a multivariate analysis of a score of≥A1 at two years included≥2 h ofcold ischemic time,no acute cellular rejection and an alanine amino transaminase(ALT)level of≥20 IU/L(P=0.028,P=0.033 and P=0.012,respectively);however,no risk factors were identified for a score of≥F1.Furthermore,no independent risk factors associated with scores of≥A1 and≥F1 at five years were identified using multivariate analysis.A ROC curve analysis of ALT at two years for a score of≥A1 demonstrated the recommended cutoff value for diagnosing≥A1 histology to be 20 IU/L.The incidence of scores of≥A2 or≥F2 at two years after LT was 3.4%(three cases),and all patients had an absolute score of≥A2.In contrast to that observed for PLBs at five years after LT,the incidence of scores of≥A2 or≥F2 was 20.0%(11 cases),and all patients had an absolute score of≥F2.In all cases,the dose of immunosuppressants was increased after the PLB,and all ten patients who underwent a follow-up liver biopsy improved to scores of≤A1 or F1.CONCLUSION:PLB at two years after LT is an unnecessary examination,because the serum ALT level reflects portal inflammation.In addition,immunosuppressive therapy should be modulated to maintain the ALT concentration at a level less than 20 IU/L.PLB at five years is an excellent examination for the detection of early reversible graft fibrosis because no serum markers reflect this finding.     

Transumbilical portal venous catheterization: a useful adjunct in left lobe living donor liver transplantation

To improve the processes used for perfusion of the explanted graft and measuring the portal venous pressure (PVP) in adult living donor transplantation (LDLT), we performed transumbilical portal venous catheterization (TPVC) to reopen the umbilical vein and insert the catheter for seven adult patients undergoing left lobe LDLT. There were no major complications as a result of this procedure. This procedure prior to implanting the graft was derived from our experience and is a classic diagnostic technique used during liver surgery. It is a simple and effective procedure for perfusion and washout of the graft and for the safe monitoring of the intraoperative PVP. We hope that this technique for left lobe LDLT will be helpful to others using postoperative PVP monitoring, administration of therapeutic drugs through the portal vein, and temporal portal decompression by preparation of extracorporeal shunting in patients with a small‐for‐size graft.     

Successful living donor liver transplantation for liver failure due to maternal T cell engraftment following cord blood transplantation in X-linked severe combined immunodeficiency disease: Case report

Maternal T cells from perinatal transplacental passage have been identified in up to 40% of patients with severe combined immunodeficiency (SCID). Although engrafted maternal T cells sometimes injure newborn tissue, liver failure due to maternal T cells has not been reported. We rescued a boy with X-linked SCID who developed liver failure due to engrafted maternal T cell invasion following living donor liver transplantation (LDLT) following unrelated umbilical cord blood transplantation (UCBT). After developing respiratory failure 3 weeks postpartum, he was diagnosed with X-linked SCID. Pathological findings showed maternal T cells engrafted in his liver and hepatic fibrosis gradually progressed. He underwent UCBT at 6 months, but hepatic function did not recover and liver failure progressed. Therefore, he underwent LDLT using an S2 monosegment graft at age 1.3 years. The patient had a leak at the Roux-en-Y anastomosis, which was repaired. Despite occasional episodes of pneumonia and otitis media, he is generally doing well 6 years after LDLT with continued immunosuppression agents. In conclusion, the combination of hematopoietic stem cell transplantation (HSCT) and liver transplantation may be efficacious, and HSCT should precede liver transplantation for children with X-linked SCID and liver failure.