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Liver transplantation (LT) for small infants6 months old is rare but becoming common as perioperative care improves. In Taiwan, living donor LT (LDLT) has expanded indications but is rarely performed for this age group because of unfavorable outcomes in the literature. We evaluated LDLT outcomes of patients6 months old.We identified infants6 months old undergoing LDLT between 2004 and 2019 at our hospital. Variables related to recipients, donors, surgeries, and outcomes were analyzed.Nine patients were identified. Indications for LT were biliary atresia (n = 2), Alagille syndrome (n = 1), protein C deficiency (n = 1), and acute liver failure (n = 5), including two patients with neonatal hemochromatosis, one with herpes simplex hepatitis, one with giant cell hepatitis with autoimmune hemolytic anemia, and one with hemophagocytic lymphohistiocytosis. Median age and weight at LT were 129 days and 4.8 kg, respectively. Graft types included left lateral segment (LLS, n = 4), hyper-reduced LLS (n = 4), and monosegment (n = 1). The median graft-to-recipient weight ratio was 4%. The median follow-up period was 14 months (range, 8 days to 127 months) with two mortalities, and two patients were totally weaned off immunosuppressants. Adjuvant therapies were required for patients with giant cell hepatitis and hemophagocytosis. Preoperative reconstructive imaging for estimating graft thickness facilitated surgical planning.Although LDLT is difficult to perform for small infants, outcomes are favorable and mainly dependent on underlying causes in addition to technical innovations.

Total laparoscopic donor right hepatectomy (TLDRH) for adult living liver donors has been reported by a few experienced centers, but with limited cases, its safety and feasibility remain controversial. We report our experience initiating TLDRH using a stepwise approach to gradually convert laparoscopy-assisted donor right hepatectomy (LADRH) to TLDRH. We retrospectively analyzed the data of 61 LADRHs, 56 conventional open donor right hepatectomies (CODRHs), and 3 TLDRHs performed between March 2014 and June 2018. There were no significant differences in perioperative outcomes between donors undergoing LADRH and CODRH, except for a slight elevations in the operative time (436.5 vs 392.9 min, p

Using "large-for-size" liver graft, graft-to-recipient weight ratio (GRWR) ≥4%, has been debated in pediatric liver transplantation due to possible graft compartment after abdomen closure. Meticulous preoperative evaluation with three-dimensional (3D) techniques may prevent these problems. This study compared the safety of large-for-size grafts in pediatric living donor liver transplantation (PLDLT) during the eras with or without 3D planning.We defined the 3D era was after November 2017 due to our first implication of 3D printing for surgical planning and subsequently developing a 3D simulation implanting model. From November 2004 to July 2021, we enrolled 30 PLDLT patients with body weight (BW)10 kg and categorized them into conventional group: GRWR ≥4% before the 3D era (n = 9), 3D group: GRWR ≥4% in the 3D era (n = 8), and control group: GRWR4% (n = 13). We followed and compared their clinical outcomes.The 3D group had the lowest BW and the highest graft volume reduction rate, with all receiving modified left lateral segments (LLS), such as reduced LLS (n = 2), hyperreduced LLS (n = 5), and segment 2 monosegment (n = 1). Overall postoperative complications were similar in conventional and control groups but significantly lower in the 3D group (OR 0.06, 95% CI 0.006-0.70, p = 0.025). However, all groups had similar graft and patient survival at 1, 2, and 4 years.Advanced preoperative 3D planning can decrease post-transplant complications and increase the safety of large-for-size grafts in PLDLT.Type of study: Retrospective comparative study; Evidence level: Level III.

Background Congenital tracheobronchial stenosis (CTBS) is a rare congenital condition characterized by complete cartilage rings covering varying lengths of the major airway. In this study, we reviewed the outcomes of patients with CTBS receiving surgical tracheoplasty in our institute. Methods We retrospectively analyzed the outcomes of consecutive patients with CTBS operated between 2006 and 2017 when extracorporeal membrane oxygenation (ECMO) was used perioperatively. Results In total, 11 patients (median follow-up period, 4.2 years; interquartile range, 1.6–5.4) were included. Seven were symptomatic in the neonatal period, 10 had cardiorespiratory anomalies, 7 required preoperative bronchoscopic balloon dilatation, and 1 required preoperative stent placement. Slide tracheoplasty (STP) was performed in 9 patients, and 2 underwent pericardial patch tracheoplasty. Seven patients required postoperative balloon dilatation, and 6 required postoperative stent placement. Early stenting provided immediate ventilatory improvement in all patients and facilitated successful extubation in a median of 4 days after stenting in 80% of the patients. Conclusions Under ECMO, severe CTBS could be successfully treated through a combination of tracheoplasty and bronchoscopic management. STP provided excellent results for solitary trachea stenosis with a minimum diameter of ≥ 3 mm. In selected patients, postoperative tracheobronchial stent placement was crucial in minimizing the ECMO duration and facilitating extubation. Level of Evidence IV

Background The medial segment as a mono-segmental graft was proposed to increase the donor pool for pediatric liver transplantation, but to date, there has been no published case. This study aims to revisit the feasibility of procuring the medial segment graft (MSG) by three-dimensional (3D) printing and ex vivo procedures performed on explanted diseased livers to overcome the gap between theory and clinical implementation. Methods From October 2004 to December 2016, we retrospectively analyzed preoperative computed tomography, magnetic resonance cholangiopancreatography, and intraoperative cholangiography images of our previous live liver donors and identified the indicated anatomy for the MSG, then materialized by 3D printing models to simulate the engraftment. Furthermore, we practiced the procurement procedures on selected explanted diseased livers. Results Among 291 analyzed livers, 96 livers (33%) met the arterial criteria for MSG, and two-thirds of them had ideal portal branches for reconstruction. The proposed right border of the MSG was the Cantlie's line, and the left edge was the right side of the umbilical fissure. The mean estimated volume of the MSG was 234 ± 54 ml. Besides, we suggest implanting the MSG as an auxiliary partial graft in an inverted vertical position or a standalone graft with right-side rotation in the right subphrenic space. Conclusion The procurement of the MSG is feasible based on our results. However, due to the novelty of the procedure, we suggest that the first attempted case of MSG should be implanted as an auxiliary partial graft to maximize patient safety. Level of Evidence Type of study: Case series with no comparison groups Evidence level Level IV.

We herein report the case of a girl with PRETEXT III hepatoblastoma (HB) developing recurrent lung metastases despite multiple chemotherapy regimens, aggressive tumor excision, multiple lung metastasectomies, and autologous peripheral blood stem cell transplantation. High tumor mutation burden (TMB) was identified through targeted next-generation sequencing, and pembrolizumab was administered post-operatively as a last resort. A complete and sustained response to the immune checkpoint inhibitor was achieved for 22 months. Although the majority of HB have a low TMB, immune checkpoint inhibitor therapy may be useful for patients with refractory HBs with a high TMB.

Background This study measures the first-pass arrival times in the hepatic artery and portal vein of the transplanted liver using contrast-enhanced ultrasound (CEUS) and assess its correlation with graft performance in the early posttransplant period. Methods This study evaluated 35 liver transplant recipients who underwent CEUS examination within 1 month of transplant surgery. CEUS under contrast-specific harmonic imaging mode were recorded for 60 seconds immediately after intravenous administration of microbubble ultrasound contrast medium (Sonazoid, GE Healthcare, Oslo, Norway). The recorded video clips were reviewed by 2 readers to determine the first-pass arrival times in the hepatic artery and portal vein, and the difference between the 2 was defined as the arterial-portal arrival interval (APAI). Laboratory data on the same date of CEUS examination were collected as indicators to correlate with APAI. Results The intra- and inter-rater reliability for APAI measurement were excellent, with intraclass correlation coefficients > .95. The mean APAI was 4.5 ± 1.8 seconds (range, 2.0-10.5 seconds). The APAI was positively correlated with the serum total bilirubin level (r = 0.357, P = .035) and negatively correlated with the platelet count (r = −0.354, P = .037). At the 5 second cutoff point, a total serum bilirubin of >8 mg/dL was reported in 5 of 11 patients (45.4%) with APAI of >5 seconds and in only 3 of 24 patients (12.5%) with APAI of Conclusions The APAI is a quantitative marker that links the hemodynamics and the clinical status of the liver graft.

Immunosuppressant-related acceleration of fibrosis has been documented in chronic hepatitis C (CHC) patients who receive organ transplantation (Tx), and sustained virological response (SVR) rates for these patients by pegylated interferon (IFN)-based therapy are generally poor and associated with unfavorable safety profiles. In addition, IFN treatment varies by patient and poses a high risk of post-renal Tx graft rejection. This study was aimed to investigate the efficacy and safety of all oral direct acting antivirals (DAAs) for CHC patients following organ Tx.A total of 32 organ Tx (liver: 17, kidney: 13, kidney then liver: 1, and heart: 1) patients with CHC on an oral DAA (paritaprevir/ritonavir, ombitasvir, and dasabuvir: 11, daclatasvir and asunaprevir: 4, sofosbuvir-based: 17) were enrolled in the study. DAAs regimen was based by genotype/subtype, patient characteristics, drug interaction profiles, and health insurance coverage.Mean patient age was 61.4 ± 9.5 years, 50.0% male, and 15.6% with cirrhosis. Fourteen (43.7%) patients experienced unsuccessful IFN treatment. Genotype distribution was as follows: 1a: 6, 1b: 17, 2: 7, 3: 1, and 6: 1. Mean time between Tx and DAAs therapy was 77.3 ± 11.0 months. Baseline HCV RNA before DAAs was 6.20 ± 0.19 log10 IU/mL. After DAAs, the distribution of week 2 HCV RNA was as follows:15 IU/mL (53.1%), 15 to 50 IU/mL (15.6%), 50 to 100 IU/mL (6.3%), and100 IU/mL (25.0%), respectively. The rates of undetectable HCV RNA (15 IU/mL) at week 4 and end-of-treatment were 93.8% and 100%, respectively. Subjective adverse events during therapy were generally mild, with no treatment terminations. After posttreatment follow-up, all 32 patients (100%) achieved SVR12.Highly responsive treatment and favorable tolerability were achieved by all oral DAAs in this difficult-to-treat patient population.

Background Antifibrosis therapy may prevent progressive liver fibrosis after successful Kasai portoenterostomy (KPE) in biliary atresia (BA) patients. The aim of this study is to evaluate the efficacy of antifibrosis therapy in a rat model of BA and KPE. Methods BA model was created on three-week-old Sprague–Dawley rats by intrabiliary alcohol injection as previously described, and KPE was performed at postoperative week (POW) 5 by cystoenterostomy. Liver biopsies were performed at the time of BA creation, during KPE, POW 9, and at sacrifice (POW 17). Prednisolone (0.1 mg/100 g/day, group 1, n = 20), Vitamin A (0.5 mg/100 g/day, group 2, n = 20), and ursodeoxycholic acid (UDCA, 1.5 mg/100 g/day, group 3, n = 20) were respectively given to three groups after KPE and continued daily until sacrifice. Histological evaluation of fibrosis and immunohistochemistry stains for 8 fibrosis markers were compared to the control group (without medication, n = 10). Results Among the four markers, namely ɑ-smooth muscle actin (ɑ-SMA), glial fibrillary acidic protein (GFAP), tumor growth factor β1 (TGFβ1) receptors 1 and 2, which showed persistently high expression after successful KPE in the examined 8 markers, only the expression of ɑ-SMA was significantly reduced in all treatment groups at POW17. However, the fibrosis grade at POW 17 was only significantly reduced in group 2 in comparison with the control group (Vitamin A vs. control group, Ishak score 3 vs. 1.8, p Conclusion In our rat model of BA with KPE, Vitamin A was effective in reducing liver fibrosis, and the mechanisms deserve further study. Level of evidence Basic science.

Background Endovascular intervention with stent placement to treat portal vein (PV) and hepatic vein (HV) stenosis after pediatric liver transplantation (LT) is still controversial in small children owing to the potential risk of functional stenosis after growth. The aim of this study is to evaluate the safety and efficacy of stent placement in this population. Methods Between 2004 and 2016, 6 children (all n = 2) and PV ( n = 4) stents placement among 46 pediatric LT patients at our institution. The clinical outcome and patency rate were followed. Morphologic changes of stents were assessed from plain films by a new index: the stent diameter ratio (SDR). Results The median age of the patients at LT was 8.9 months. The patency rate was 100% without functional stenosis during a median follow-up period of 65.5 months. The "stent growth" phenomenon was demonstrated by SDR with significant resolution of hourglass deformity 2 years after stent placement ( p for trend Conclusion Vascular stent placement is a safe and effective method for the management of PV and HV stenosis following pediatric LT because these stents will enlarge as children grow. Type of study Case Series with no Comparison Group Level of evidence Level IV.

Background There is a lack of consensus regarding the treatment of total colonic aganglionosis (TCA) with respect to perioperative morbidity, mortality, complications, and functional outcomes. The aim of this study was to review the results of surgical TCA treatment over a 26-year period and characterize the outcomes. Methods We retrospectively reviewed the clinical characteristics, surgical courses, and outcomes of TCA patients who underwent definitive pull-through operations from 1986 to 2012. Follow-up data were collected by chart reviews and telephone interviews using a standardized questionnaire. Results We identified nine infants with TCA (8.6%) from among 105 infants with Hirschsprung's disease treated during the 26-year period. Neither sex predominated (male/female ratio = 4:5). All infants underwent laparotomies and simultaneous enterostomies. All patients eventually underwent modified Duhamel pull-through procedures at a mean age of 179 days (range, 47–352 days). Two infants died of complications after surgery including heart failure and sepsis. The remaining infants recovered smoothly with antilaxative medications, and all but one was weaned off these medications. Although the surviving patients did not catch up on growth, they and their families were satisfied with the surgical results. Conclusion Infants with TCA had satisfactory outcomes after the modified Duhamel pull-through operation. Based on our experience, we suggest that the pull-through operation could be performed earlier, even when there are loose stools from the enterostomy.

The mechanisms of liver fibrosis in biliary atresia (BA) after a Kasai operation deserve studying to improve the clinical outcomes. This study aimed to create a rat model simulating BA after a Kasai operation.We inserted a polyethylene tube (PE10) into the common hepatic duct (CHD) and ligated the common bile duct (CBD) in 30 newborn rats and injected 95% ethanol into IHD at postoperative week-one (POW-1). The PE10 was removed at POW-3. The rats were sacrificed at POW-5. The CBD cystojejunostomy was performed on another 10 rats at POW-5.The IHD obliteration and CBD dilatation were noted at POW-3 cholangiography before removal of the PE tube. The gross findings at sacrifice in the rats without cystojejunostomy included biliary fibrosis, CBD cyst, and IHD obliteration. The microscopic findings of the liver were like BA. Seven of the 10 rats with CBD cystojejunostomy were jaundice-free at POW-8. The fibrosis grade at POW-8 of the rats with CBD cystojejunostomy was significantly lower in the jaundice-free rats (Ishak fibrosis score, 3.4 ± 0.9 and 1.5 ± 0.3 in the jaundiced rats and jaundice-free rats, respectively, p.05).Based on our study, CBD cystojejunostomy five weeks after CBD ligation with ethanol injection into the IHD in newborn rats can provide a model for investigating mechanisms and treatments of liver fibrosis in BA after a Kasai operation.

Hepatic-based metabolic disorders are characterized by an enzyme deficiency expressed solely or mainly in the liver. They are divided into cirrhotic or non-cirrhotic metabolic liver diseases (NCMLDs), and most of them can be treated by liver transplantation. Because the livers with NCMLDs are usually structurally and functionally normal, the primary aim of the liver graft is to support the deficient enzymes rather than maintaining liver functions. Hence, we hypothesize that the exchange of partial liver grafts by the technique of auxiliary partial orthotopic liver transplantation (APOLT) between patients with 2 different NCMLDs may be feasible to replace the deficient enzymes in each patient. This hypothesis is based on the following conditions: (i) the patients have no chance of undergoing timely liver transplantation, (ii) the symptoms of each NCMLD may be alleviated after exchanging partial liver grafts, and (iii) each graft is anatomically appropriate for APOLT. In addition, we evaluate it with a focus on selection of cases, designing of graft sizes, and surgical techniques for reciprocal APOLT.

A 24-yr-old man was diagnosed with HCU during neonatal screening and remained on a pyridoxine, vitamin B12, folic acid, and betaine regimen with dietary methionine restriction for more than 10 yr. He had normal mental development, marfanoid appearance, myopia because of lens dislocation, and recurrent ankle subluxation during adolescence. Thereafter, he was a poor adherent to the conventional diet-restrictive therapy, and LT was considered when he developed hypertension and multiple infarctions over the right cerebellum early in the second decade of his life despite taking aspirin as a prophylaxis from 17 yr of age. In November 2009, he received a deceased whole LT from a blood group compatible donor. Along with the success of the transplantation, he was completely disease free without dietary or nutritional control. To the best of our knowledge, this is the first case of LT intended to cure HCU, and with promising results. This case provides an insight into the role of LT for this congenital metabolic disease, for which the decision should be made by judging between the severity of the disease and the risk of the operation, as well as the life quality of the patient.

SummaryCholedochoduodenal fistula (CDF) complicated by peptic diseases or following surgical or endoscopic approaches of the common bile duct is not uncommon. However, it usually occurs without significant symptoms and can be well controlled with conservative treatment in normal immunized patients. Here we report a case involving a 58-year-old male patient with diabetic nephropathy, who received a choledocholithotomy for choledocholithiasis in November 2007 and renal transplantation in March 2008. The patient had recurring cholangitis during the 5 months following his renal transplantation. Cholangiography and liver biopsy revealed sclerosing cholangitis. The patient underwent liver transplantation (LT) in May 2009 because radiological and endoscopic procedures failed to control his jaundice. A proximal CDF was found during the LT procedures. We considered that the patient's advanced secondary sclerosing cholangitis was induced by this fistula. At the 16 months' follow-up, the patient was surviving well and the graft remained intact. To our knowledge, this is the first report of a renal transplantation recipient receiving LT because of uncontrolled cholangitis caused by a CDF.

Retrograde arterial flush (RGAF) of liver grafts, which prevents arterial intima injuries in living donor liver transplantation (LDLT), seemed to reduce post-transplantational cholestasis by our preliminary study. The aim of this study was to further confirm this effect of RGAF of grafts in LDLT by a prospective randomized study. The enrolled cases were randomly divided into two groups, RGAF (portal flush plus RGAF) and non-RGAF (portal flush only). The outcome measures included intra-operative hemodynamic changes, one-month post-transplantational liver function tests and acute cellular rejection, vascular and biliary complications, the length of postoperative hospital stay, and graft and patient survivals. The significant findings of results were lower postoperative serum bilirubin in the RGAF group until three wk after transplantation (days 1, 3, 7, 14, and 21 serum total bilirubin, mg/dL: 5.82±5.46, 3.95±4.28, 4.87±5.01, 3.62±5.32, and 3.63±6.91 vs. 8.41±5.49, 7.47±5.61, 10.0±10.1, 9.06±11.6, and 6.02±9.84, respectively, in RGAF and non-RGAF, p=0.017, 0.002, 0.014, 0.011, 0.109) and shorter postoperative hospital stay in the RGAF group (31.1±17.6 vs. 44.8±32.3 d, respectively, in RGAF and non-RGAF, p=0.035). In conclusion, the RGAF of liver grafts on a back table may ameliorate postoperative functional cholestasis in LDLT, which possibly reduces postoperative hospital stay.

Background To compare the incidence of residual high-grade vesicoureteral reflux (HVUR) (≥Grade III) in neurogenic bladder patients receiving augmentation cystoplasty alone or with simultaneous ureteral reimplantation. Furthermore, we also tried to find the risk factors of residual VUR and febrile urinary tract infection. Methods Between 1999 and 2009, urinary bladder augmentation was performed in 21 children with neurogenic bladder. Seventeen of these patients had VUR on preoperative voiding cystourethrography, of whom 11 patients (14 ureters) received augmentation alone (Group A) and 6 patients (8 ureters) received simultaneously ureteral reimplantation (Group B). Univariate logistic regression analysis and Fisher exact test were used for statistical analysis. Results Six patients (8 ureters) had residual HVURs in Group A, but none in Group B. The incidences of residual HVUR were 57.14% and 0%, respectively. Seven patients had febrile UTIs after operation, 6 of them had residual HVURs. In risk factor analysis, postoperative follow-up duration less than 12 months and lack of anti-reflux operation were significant risk factors for residual HVUR; the residual HVUR was the significant risk factor for febrile urinary tract infection. Conclusion Simultaneous ureteral reimplantation reduces postop HVUR significantly. We recommend augmentation and simultaneous ureteral reimplantation in children with HVUR and neurogenic bladder if technically feasible.

Liu C, Niu D-M, Hsia C-Y, Loong C-C, Lin N-C, Tsai H-L, Tsou M-Y, Chin T. Living donor liver transplantation using a graft from a donor with Dubin–Johnson syndrome. Pediatr Transplantation 2012: 16: E25–E29. © 2010 John Wiley & Sons A/S. Abstract: DJS is an autosomal recessive disorder that causes an increase in conjugated bilirubin without elevation of liver enzymes. Most patients are asymptomatic and have normal life spans, but to the best of our knowledge, their livers have never been reported to be grafts in liver transplantation. Herein, we report an infant patient with MMA that received a partial liver graft from his mother, who had DJS. A biliary anastomosis stricture was noted five months after transplantation and was successfully treated with radiological interventions. Otherwise, the patient’s liver functions were normal, and a liver biopsy showed a pathognomonic picture of DJS nine months after the transplantation. The patient was followed for one yr, and the results were satisfactory for an increase in oral intake and protein uptake, no recurrence of metabolic stroke and there was a gradual catch-up with regard to physical development despite having a persistently abnormal profile of amino acid analysis. From the experience of our case, we suggest that a liver from a donor with DJS can be used as a graft for liver transplantation, although long-term follow-up is mandatory to examine the grafted liver under the use of immunosuppressive medications.

This study was designed to evaluate the possibility of avoiding small-for-size syndrome (SFSS) in living donor liver transplantation (LDLT) by increasing the donor's body weight (BW) before liver donation.Nineteen participants, including 15 volunteers and 4 liver donors, were enrolled in this study to increase their BW by 1.5-5 kg within 3 months by eating a high-protein and high-carbohydrate diet according to a flexible formula to increase calorie intake.Fifteen participants, including 12 volunteers and 3 live liver donors, successfully increased their BW by 1.5-5 kg within 3 months (failure rate, 21%). The actual liver weight (LW) gain was more than the expected LW gain using the formula for calculating standard liver volume (2.77- to 8.94-fold; median, 4.49-fold; mean, 4.45-fold, P0.005) and using the ratio of liver weight to body weight (1.36- to 4.49-fold; median, 2.01-fold; mean, 2.06-fold, P0.005). The enlargement of the livers was symmetrical without significant fatty change. The graft-versus-recipient weight ratio increased 0.17%, 0.07%, and 0.08%, respectively, for the three live liver donors and successful LDLTs were performed.By having liver donors eat a high-protein, high-carbohydrate diet to increase their BW in a short period of time, the actual LW may increase more than the expected LW gain without fatty change. This method of increasing LW may be used in selected cases of LDLT to avoid SFSS.

We aimed to minimize the dose of tacrolimus in pediatric patients undergoing liver transplantation prospectively.Pediatric liver transplant recipients with stable graft function1year (transplant at1year of age), or 2years (transplant at1year of age) post transplant were screened. After baseline graft biopsy, patients were enrolled into our protocol for elective tacrolimus dose reduction. Patients were assessed by liver function test and protocol biopsy during and after tacrolimus dose reduction.From January 2011 to December 2012, 16 patients were recruited, of whom 15 completed follow-up at a mean 40.75±5.98months. Six patients were preliminarily weaned off tacrolimus, and five remained tacrolimus-free for more than 2years. Of the 10 patients who were not weaned off tacrolimus, six experienced seven episodes of clinical rejection. Five patients had a reduction in tacrolimus dosage to an undetectable trough level, another five to a trough level4ng/ml, including one patient who was off the study. At the last patient visit, all of the patients had normal liver function test results with no graft loss. Three patients had low-grade graft fibrosis. The patients with metabolic liver disease (p=0.039) and who were recruited earlier after transplantation (p=0.028) were more likely to be weaned off tacrolimus.Tacrolimus withdrawal is feasible in select pediatric liver transplant recipients, and long-term follow-up for these patients is suggested.

Background Esophageal atresia (EA), tracheoesophageal fistula (TEF), or both is a complicated problem. The purpose of this study was to evaluate the outcomes and postoperative complications in patients with EA/TEF who were admitted to our hospital. Methods In total, 15 patients were enrolled from 1994 to 2003, including 8 males and 7 females. Patient demographics, associated anomalies, and outcomes were analyzed. Results The most common variant was EA with a distal TEF (type C), which occurred in 12 patients (80%). The latter had associated congenital anomalies, and cardiac anomalies were the most frequent, occurring in 8 patients (53.3%). Of the 6 cases who had life-threatening anomalies, 4 (66.7%) died, and of the 9 cases who had no life-threatening anomalies, 2 (22.2%) died. Tracheomalacia and/or stenosis were diagnosed in 8 patients (66.7%) postoperatively. Though 3 of the 4 cases who suffered from dying spell received intratracheal stent implantation, 2 cases still died. Conclusion The survival rate of the patients with EA/TEF is influenced mainly by associated life-threatening anomalies. TMS combined with a history of dying spell may be the major fatal complication.

Haddad syndrome is characterized by congenital central hypoventilation syndrome (Ondine's curse) associated with segmental distal gut aganglionosis (Hirschsprung's disease). The prognosis of Haddad syndrome is very poor, and survival is often less than 2 years. Treatment of Hirschsprung's disease is usually influenced by the association with Ondine's curse. We report the case of a girl with Haddad syndrome who underwent redo pull-through with Duhamel's method because of persistent obstruction after primary transanal pull-through surgery. After 7 years of follow-up, the patient is alive and does not suffer from recurrent partial intestinal obstruction. She performs her daily activities with the support of a portable ventilator. Thus, aggressive surgical treatment for Hirschsprung's disease could have good outcome in terms of long-term survival in Haddad syndrome patients.

Delayed PV complications are not rare in pediatric liver transplantation. Although PTPV offers a treatment and minimizes surgical revision, in case of complete PV thrombosis (PVT), the failure rate of PTPV is high. Herein, we report a successful technique of PTPV in a case of complete PVT with a stent placement using a bidirectional approach in a child with living donor liver transplantation.

The manifestations of glycogen storage disease type 1a (GSD 1a) are usually so prominent in childhood that it is readily diagnosed by pediatricians. However, a mild form of the disease may only become apparent during adolescence or adulthood. We observed a brother and sister with subtle manifestations of the disease, which was discovered after the brother's son was diagnosed with typical GSD 1a. The adult siblings never suffered from hypoglycemia, had normal fasting blood glucose and liver transaminases at the time of diagnosis, and were taller than average for Chinese. Their only notable disease manifestations were recurrent gouty arthritis associated with hyperuricemia and hyperlipidemia during adolescence. When diagnosed, the brother had multiple benign and malignant hepatic tumors, and died of fulminant metastatic hepatocellular carcinoma 6 months after liver transplantation. p.M121V/p.R83H and p.M121V/p.M121V genotypic constellations of the G6PC gene were identified in this family. Both siblings were homozygous for the newly identified p.M121V mutation. The infant had compound heterozygous mutations, p.R83H and p.M121V. We recommend that mild GSD should be considered in the adolescents with unexplained hyperuricemia and hyperlipidemia, despite the presence of normal blood glucose levels. This report also reminds us that hepatocellular carcinoma could develop even in very mild GSD 1a patients.

Retrograde arterial flush (RGAF) of liver grafts, which prevents arterial intima injuries in living donor liver transplantation (LDLT), seemed to reduce post-transplantational cholestasis by our preliminary study. The aim of this study was to further confirm this effect of RGAF of grafts in LDLT by a prospective randomized study. The enrolled cases were randomly divided into two groups, RGAF (portal flush plus RGAF) and non-RGAF (portal flush only). The outcome measures included intra-operative hemodynamic changes, one-month post-transplantational liver function tests and acute cellular rejection, vascular and biliary complications, the length of postoperative hospital stay, and graft and patient survivals. The significant findings of results were lower postoperative serum bilirubin in the RGAF group until three wk after transplantation (days 1, 3, 7, 14, and 21 serum total bilirubin, mg/dL: 5.82±5.46, 3.95±4.28, 4.87±5.01, 3.62±5.32, and 3.63±6.91 vs. 8.41±5.49, 7.47±5.61, 10.0±10.1, 9.06±11.6, and 6.02±9.84, respectively, in RGAF and non-RGAF, p=0.017, 0.002, 0.014, 0.011, 0.109) and shorter postoperative hospital stay in the RGAF group (31.1±17.6 vs. 44.8±32.3 d, respectively, in RGAF and non-RGAF, p=0.035). In conclusion, the RGAF of liver grafts on a back table may ameliorate postoperative functional cholestasis in LDLT, which possibly reduces postoperative hospital stay.

Although the lateral segment (LS) from the split-liver of a deceased donor or a live donor can increase the organ pool of pediatric patients awaiting liver transplantation, the shortage of organ donation in Asia countries is still serious and results in high death rates of pediatric patients. The medial segment (MS) of the liver is sacrificed during the standard technique of splitting a whole liver into an LS and an extended right liver because the cutting sites of portal vein, hepatic artery and bile duct are all in the bifurcation of the liver hilum to have adequate length of vascular and biliary pedicles for easier grafting. However, the surgical techniques of vascular and biliary reconstructions for liver transplantation, particularly from the experiences of living donor liver transplantation, have been much improved in the last decade. Therefore it may be possible for an additional MS of the liver to be an isolated graft for a small recipient on the premise that grafts of right lobe (RL) and LS are minimally injured. In light of detailed reviews of anatomies of hepatic arteries, hepatic veins, portal veins and bile ducts, the dissection and reconstruction of vessels and bile ducts for the MS can possibly be performed if the extra-hepatic length of the artery to the MS is long enough. As the artery for the MS, middle hepatic artery (MHA), usually derives from a branch of the left hepatic artery and often in the liver parenchyma, the length is usually too short to be reconstructed. If the MHA for the MS is isolated and its extra-hepatic length is more than 1cm, triple liver grafts from a deceased whole liver, consisting of the RL, MS and LS may be possible. The anatomies of the hepatic artery in abdominal computed tomography (CT) or magnetic resonance imaging (MRI) for live liver donors in our institution were retrospectively analyzed. The results showed that three types of hepatic arterial anatomies could be considered for possible recovery of triple segments: type I is an accessory left gastric artery to feed the lateral segment; type II is an isolated MHA; type III is an early bifurcation of the left hepatic artery and MHA.

DJS is an autosomal recessive disorder that causes an increase in conjugated bilirubin without elevation of liver enzymes. Most patients are asymptomatic and have normal life spans, but to the best of our knowledge, their livers have never been reported to be grafts in liver transplantation. Herein, we report an infant patient with MMA that received a partial liver graft from his mother, who had DJS. A biliary anastomosis stricture was noted five months after transplantation and was successfully treated with radiological interventions. Otherwise, the patient's liver functions were normal, and a liver biopsy showed a pathognomonic picture of DJS nine months after the transplantation. The patient was followed for one yr, and the results were satisfactory for an increase in oral intake and protein uptake, no recurrence of metabolic stroke and there was a gradual catch-up with regard to physical development despite having a persistently abnormal profile of amino acid analysis. From the experience of our case, we suggest that a liver from a donor with DJS can be used as a graft for liver transplantation, although long-term follow-up is mandatory to examine the grafted liver under the use of immunosuppressive medications.

Formal hepatic arterial flush to preserve the liver graft in living donor liver transplantation (LDLT) is not recommended by most transplant centers because direct cannulation may injure the intima of the hepatic artery. The authors describe a method of retrograde arterial flush of the liver graft without arterial cannulation by hepatic venous outflow occlusion (HVOO) in LDLT. First, we proved no backflow of the hepatic artery without HVOO by portal flush to pig livers. Then we used HVOO on 15 LDLT cases (Group HVOO). The results were compared with those of 24 counterpart LDLT cases (Group non-HVOO) without hepatic artery flush. The two-week posttransplantational liver functions were not different between two groups except that the day-three and day-seven serum bilirubin levels were lower in Group HVOO (day-three total bilirubin: 4.99 +/- 4.04 mg/dl versus 7.65 +/- 4.33 mg/dl, p =.016; day-seven total bilirubin: 5.06 +/- 5.02 mg/dl versus 9.57 +/- 6.09, p =.005). The rates of vascular complications, six-month graft survival, and biliary anastomotic stricture in Group HVOO were 0, 93.3, and 13.3% respectively, which were not different from those of Group non-HVOO. In summary, to avoid intima injury, the retrograde arterial flush of liver graft by HVOO is safe in LDLT. The short-term results showed the effect of decreasing early functional cholestasis but the long-term benefits need further evaluation, especially with regards to biliary anastomotic complications.

Liu C, Niu D-M, Loong C-C, Hsia C-Y, Tsou M-Y, Tsai H-L, Wei C. Domino liver graft from a patient with homozygous familial hypercholesterolemia. Pediatr Transplantation 2010: 14:E30–E33. © 2009 John Wiley & Sons A/S. Abstract: HFH is a metabolic disease caused by a defect in the gene that encodes the synthesis of the cellular receptor for LDL-Rs. A high plasma level of cholesterol is present from birth and leads to severe atherosclerosis in childhood, and death from myocardial infarction usually occurs before the age of 20 yr. The liver contains approximately 50–75% of the total body LDL-Rs; therefore, liver transplantation has been carried out to treat this metabolic disorder effectively. The rationale for using an HFH liver for a domino graft is that the absence of functional LDL-Rs in the liver may be compensated for by the extra-hepatic LDL-Rs. Therefore, an HFH liver can possibly be used as a domino graft for a recipient with a normal plasma cholesterol level before transplantation. We herein report a domino liver transplantation using an HFH liver as a domino graft with successful results. To expand the donor pool, especially in Asian countries, domino grafts from HFH should be encouraged by careful selection of the domino recipient.

The feasibility of D-D biliary reconstruction in pediatric LDLT using left-lobe graft has been discussed in few reports. The use of a trans-anastomotic biliary tube seemed to be the favorable method to avoid the complications according to these reports. We had performed left-lobe LDLT for seven pediatric cases and D-D was done originally. Three cases were converted to R-Y hepaticojejunostomy due to radical resection of hepatoduodenal ligament (n = 1) and severe kinking of D-D (n = 2). Four cases received D-D using 6-0 PDS interrupted sutures without external stent tube. One D-D case died of intra-cerebral hemorrhage 10 days after operation with a functioning graft. There was one biliary leakage in a D-D patient who required PTCD stent for 4 months without any sequale. From our limited experience, D-D biliary reconstruction without external stent tube in left-lobe LDLT is feasible in certain pediatric cases having normal extra-hepatic bile ducts. In smaller recipient with larger graft, the use of a trans-anastomotic biliary tube can prevent anastomotic kinking although we suggest R-Y biliary reconstruction is better for this condition.

It is difficult to reconstruct the hepatic venous outflow in LDLT for the case of BCS, especially when the thrombosis extends to the retro-hepatic and supra-hepatic vena cava. We reported on a technique of diamond-shape venoplasty which successfully treated such a case. The venoplasty used autologous vein grafts to overcome the size discrepancy between the orifices of supra-diaphragmatic inferior vena cava and hepatic vein of the liver graft.

Background: Liver transplantation is a challenging procedure that is associated with perioperative morbidity and mortality, so it is justifiable to perform such a procedure in high-volume procedure centers. Organ shortage remains a major issue in Taiwan. Due to the difficulty in establishing a high-volume procedure center, it is important to review the overall outcome of patients undergoing liver transplantation at a small-volume procedure center to determine if performing such a procedure is justified. Methods: Between April 2001 and May 2005, 26 adults underwent deceased donor liver transplantation at Taipei Veterans General Hospital. The overall outcomes were reviewed in terms of 90-day mortality, 1-year and 3-year survival rates. In addition, the patients were divided into a hepatocellular carcinoma (HCC) group (n = 12) and a benign end-stage liver disease (ESLD) group (n = 14). The clinical demographics, 90-day mortality, 1-year and 3-year survival rates were reviewed and compared between the 2 groups. Results: The 90-day mortality was 15.3% in the whole series, 8.3% in the HCC group and 18.7% in the ESLD group. The overall 1-year and 3-year survival rates were 76.9% and 63.5%, respectively, for the whole series. For the 2 groups, the respective 1-year and 3-year survival rates were 83.3% and 71.4% in the HCC group, and 71.4% and 57.1% in the ESLD group. The survival difference was not significant (p = 0.319) between the 2 groups. In the HCC group, the 1-year and 3-year disease-free survival rates were 88.9% and 71.1%, respectively. Conclusion: The survival rates between ESLD and HCC patients undergoing liver transplantation at a small-volume procedure center were comparable. The results of the whole series were not satisfactory, but the results for the HCC group were acceptable. [J Chin Med Assoc 2008;71(4):186–190]

A case of proximal jejunal obstruction by a congenital band is reported. The band ran from the antimesenteric wall of the proximal jejunum just distal of the Treitz's ligament to the root of the mesentery. Lysis of the band and enterotomy were performed to make sure no intrinsic obstruction cured the patient. No recurrence was found after 3 months of follow-up. To the best of the authors' knowledge, this is the second case with an anomalous congenital band causing proximal jejunal obstruction reported in the English-language literature.

Background The Palmaz stent can relieve congenital malacia or stenosis of airway, but reports on the indications and results of stent removal are rare. The authors report their experience in removing Palmaz stents and discuss the indications for removal. Methods Thirteen stents in 12 patients were removed by a rigid bronchoscope for various reasons. The indications were expected recovery (n = 5), severe granulations and expected recovery (n = 2), stent collapse (n = 2), and stent migration and/or fracture (n = 4). Results The course after removal was smooth in 9 patients and complicated in 3. The indications for stent removal in these 3 complicated cases were all expected recovery. One of the 3 complicated cases needed emergent cardiopulmonary bypass and tracheostomy for a collapsed stent that occluded airway, 1 failed for intractable bleeding, and 1 failed for intense vagal reflex causing cardiac arrest. At 6 months of follow-up, satisfactory results were seen in all but one case that needed further procedures for an iatrogenic tracheoesophageal fistula. Conclusions Most Palmaz stents can be removed smoothly with a rigid bronchoscope; however, lethal complications can happen. The authors suggest that indications for stent removal should be intractable airway symptoms caused by the stent rather than expected recovery. Muscle relaxants should be avoided during anesthesia to maintain spontaneous breathing, and cardiopulmonary bypass should be on standby.

The contralateral internal inguinal rings of 333 children were examined with a laparoscope during repair of unilateral inguinal hernia. The nonpuncture laparoscopy technique was used so that no extra wound was created. The morphology of the rings was classified as follows: type 1, flat and covered tightly with peritoneum; type 2, shallow with a visible base under an elevated peritoneal fold; type 3, deep ring that represented a patent processus vaginalis. The incidence of types 1, 2, and 3 rings was 45%, 23%, and 32%, respectively. The incidence of type 1 internal rings was similar for different age groups. The incidence of type 2 rings increased with age, and that of type 3 decreased with age. This observation is compatible with autopsy studies and the experience of contralateral exploration of childhood inguinal hernias.

Buried penis is a result of penile skin deficiency and inadequate attachment of the skin to the Buck's fascia. A modified prepuce unfurling technique and the results are reported.Thirty-two boys with buried penis, aged 14 months to 12 years, underwent the surgical procedure. A circumferential incision is made at the junction of the outer and inner prepuce. The subcutaneous tissue is dissected from the inner prepuce and degloved from the Buck's fascia, so that a thin inner prepuce can be sutured directly to Buck's fascia. Reapproximation of the outer and the inner prepuce completes the procedure.Prolonged preputial edema was seen in two patients. No patient had skin necrosis. Most patients achieved satisfactory results.This procedure unfurls the inner prepuce to cover the penile shaft. Ideal cosmetic results could be achieved in selected patients if some details of the procedure are emphasized.

Background. Only a few studies on extrahepatic biliary atresia (BA) have reported that the morphological changes of bile canaliculi could predict the clinical outcome after portoenterostomy and provide differential diagnosis of neonatal jaundice. Aminopeptidase N (APN) is an ectoenzyme of bile canaliculi that is involved in bile secretion. In this study, we tried to see whether APN of bile canaliculi had a significant role in BA. Patients and methods. We used monoclonal antibody 9B2 to compare the expression of APN in livers with BA, neonatal hepatitis, and choledochal cysts, as well as in nontumorous portions of pediatric hepatic livers with tumors. The expression of APN in fetuses, preterm babies, and term neonates was also studied. Results. A high degree of 9B2 expression in BA was closely related to poor outcome. Cholestasis in choledochal cysts, rather than neonatal hepatitis, made 9B2 expression stronger. Increasing expression of 9B2 from fetuses to neonates was noted and the degree of 9B2 expression was similar between term neonates and nontumorous portions of pediatric livers with tumors. Interestingly, some cases of BA had 9B2 expression like that of preterm babies. Conclusions. APN of bile canaliculi progressively develops from fetuses to neonates and is well developed in neonates. APN can be induced to stronger expression by obstructive jaundice. The amount of expression of APN of bile canaliculi in BA is a predictor of clinical outcome and may be a tool for implicating the mechanism of BA.

The authors studied the preoperative Technetium 99m-dimercaptosuccinic acid renal scan (DMSA) of patients undergoing unilateral vesicoureteral antireflux surgery to compare the amount of renal scarring between the refluxing and the contralateral renal units. They sought to determine whether postoperative contralateral vesicoureteral reflux was preexistent or new onset.Sixty-eight patients who underwent unilateral vesicoureteral antireflux surgery and had preoperative DMSA and postoperative voiding cystourethrography (VCUG) examinations were studied. Preoperative DMSA results were analyzed to determine the amount of renal scarring in each kidney.Sixty-four (94.1%) ipsilateral refluxing renal units had renal scars. Of the 68 contralateral renal units, scars were noted in 28 (41.2%). The rate of nonscar was 4 of 68 (5.9%) in reflux kidneys, which was significantly lower than 40 of 64 (62.5%, excluding 4 with a history of resolved reflux) in nonreflux kidneys (P.001). Of 40 contralateral nonscarred kidneys, 1 of 40 (2.5%) had subsequent reflux, which was significantly lower than 5 of 28 (17.9%) of scarred kidneys (P.005). Six patients (8.8%) had contralateral reflux, and 1 of them had a history of resolved reflux. Of the 6 contralateral kidneys with severe scarring involving 3 poles or contracted, 4 of 6 (66.7%) had subsequent reflux.Scar in the contralateral kidney seen on DMSA scan seems to predict contralateral reflux after unilateral antireflux surgery. The contralateral reflux may be preexistent. Postoperative VCUG should be performed routinely for patients who have contralateral renal scars. In patients with a history of contralateral reflux or severe contralateral renal scar, simultaneous contralateral ureteral reimplantation should be considered.

Purpose: The authors describe the experience of a modified technique to correct primary vesicoureteral reflux in children. Methods: Twenty children (28 renal units) with primary vesicoureteral reflux (grade III to grade IV) were treated with this technique. The authors incised the bladder mucosa and muscle along the direction of the ureter using a right-angled probe as a guide. About 2 to 3 cm of extravesical ureter was freed and pulled into the bladder via the incision. The bladder muscle was closed under the ureter, and the mucosa was repaired over the ureter. Results: One patient had residual reflux. All other patients, who underwent follow-up with sonogram and voiding cystourethrogram from 14 to 42 months after the operation, had neither recurrent reflux nor urinary tract obstruction. Conclusions: The advantages of this technique are (1) anastomosis of the ureteral orifice to the urinary bladder is not needed, so that the risk of orifice stenosis is minimized; (2) the ureteral orifice remains unchanged after the procedure, so that ureteroscopic procedures are easier to perform in future if required; and (3) pelvic dissection is not needed as in extravesical detrusorrhaphy. However, this procedure may not be suitable for patients with severe hydroureter.