Biliary tract diseases have always been one of the important indications for liver transplantation. These biliary tract diseases include congenital atresia of biliary duct, biliary diseases with tendency of malignant transformation, malignant biliary diseases, primary biliary cirrhosis, and secondary biliary cirrhosis. The right time to consider liver transplantation for the above biliary diseases includes situations when transplantation is the only approach to treat the disease, the only effective approach to treat the disease, or one of the effective approaches to treat the disease. Currently there is still no commonly accepted pre-operative evaluation standard for biliary disease-related liver transplantation. However, it is important to obey the following principles. First, the diagnosis should be confirmed; secondary, the necessity or urgency of the liver transplantation should be clarified; and third, the patient and his/her family should understand the whole procedure of transplantation the risk and advantage of the surgery, and the potential life-long intake of immunosuppressors. This article will briefly review all these relavant issues based on the authors' personal experience and clinical cases.
Biliary Atresia ; surgery ; Biliary Tract Diseases ; surgery ; Humans ; Liver Cirrhosis, Biliary ; surgery ; Liver Transplantation ; Living Donors

Bile Duct Diseases ; Biliary Atresia/surgery* ; Cysts ; Humans ; Infant ; Liver Failure ; Portoenterostomy, Hepatic ; Retrospective Studies

OBJECTIVETo summarize experience of pediatric intensive care and explore the incidence of complications, the involved pathogens among liver recipients to determine the effective strategies for preventing complications.

METHODSBetween June 2006 and July 2009, 35 children under the age of 14 yr received 35 liver transplantations (LTs) performed at the center. A retrospective review of 22 infants weighing 8.8 kg or less underwent 23 transplants was conducted. Indication for transplantation was biliary atresia. Central venous pressure and arterial blood pressure were monitored continuously and fluid monitoring was performed every 2 hours in the first postoperative week. Blood loss, ascites, and intraoperative transudate loss were primarily replaced with 5% albumin and crystalloids to maintain a central venous pressure between 4 and 6 cm H(2)O. Oral food intake was allowed as soon as possible. To identify vascular or biliary complications, liver doppler ultrasound was performed intraoperatively immediately after reperfusion and after closure of the abdominal wall and postoperatively, twice daily during the first week after surgery. Immunosuppression was initially cyclosporine based, in combination with steroids. Cyclosporine was begun one day prior to transplantation at a dose of 10 mg/(kg·d) divided into two doses, except for cases with hepatic encephalopathy and severe infection. The subsequent doses were adjusted on the basis of recommended trough blood concentrations at different stages. Steroids were eventually discontinued at a time point exceeding 6 months after transplantation. The diagnosis of rejection was confirmed by histology on needle biopsy specimens. Acute graft rejection episodes were treated with a 3-day scheme of IV methylprednisolone 10 mg/(kg·d) followed by recycling doses during the following 3 days (7.5, 5 and 2.5 mg/(kg·d).

RESULTSThe most common postoperative complications were infections (18 cases), gastrointestinal bleeding (3 cases), and vascular complications (4 cases). Rejection occurred in 25% of patients. There was one perioperative death from primary graft non-function. The most common isolated bacteria of the pathogen spectrum were Staphylococcus epidermidis. The median length of stay (LOS) in the PICU for 22 patients (23 transplants) was 10 days (range 5 - 21) and the mean length of stay in the hospital was (18.5 ± 116) days (range, 11 - 48 days). Mean requirement for artificial ventilation was 37.6 h. Mean use of dobutamine, prostaglandin E1 and dopamine was 3.3, 7.5 and 8.8 days, respectively. Preoperatively, 3 children had gastrointestinal bleeding, 18 had ascites, 2 had encephalopathy, 22 had jaundice, and 16 had coagulopathy. There were multiple early operative complications in these infants, including one graft with primary non-function (4.5%). Two patients (9.1%) returned for a total of three times for gastrointestinal bleeding or intra-abdominal hematoma. Three patients (13.6%) had early postoperative intestinal perforations related to adhesions or enterotomy, one was associated with a bowel obstruction. There were 26 episodes of bacterial or fungal infections in 18 (81.8%) patients in the early postoperative period, and infection was the direct/contributing cause of death in one infant. These infections included pneumonia, intra-abdominal abscess or sepsis. All of the bacterial and fungal infections were successfully treated with the appropriate antibacterial and antifungal agents, except for one patient who developed overwhelming sepsis after small bowel perforation. Four (18.2%) patients developed five episodes of acute allograft rejection during the first 15 days after LT. Three of the four patients who developed rejection were transplanted before 2007. All episodes of rejection were treated successfully with intravenous steroid pulse and optimization of cyclosporine levels or FK506 conversion. Of the 20 survivors beyond the perioperative period, two cases (10%) had hypertension requiring therapy.

CONCLUSIONSLiver transplantation in infants with biliary atresia appears technically demanding but acceptable. There should be essentially no age or size restriction for infants and transplantation can be performed with good outcome, although the frequency of complications is much higher than that seen in older children. The improvement in medical and nursing expertise in this group of very sick infants is based on judicious preoperative donor and recipient selection, meticulous surgical technique (vascular reconstruction and abdominal closure), immediate detection and prompt intervention of complications, and keen postoperative surveillance, which reflect a learning curve for both the technical aspects of liver transplantation and post-operative care of these very small patients in our institution. Liver transplantation for infants can be technically challenging.

Biliary Atresia ; surgery ; therapy ; Child, Preschool ; Critical Care ; methods ; Humans ; Infant ; Liver Transplantation ; Living Donors ; Parenteral Nutrition ; Postoperative Care ; methods ; Retrospective Studies ; Treatment Outcome

Objective: To compare the clinical efficacy of split liver transplantation (SLT) and living donor liver transplantation(LDLT) in the treatment of children with biliary atresia. Methods: The clinical data of 64 children with biliary atresia who underwent SLT and 44 children who underwent LDLT from June 2017 to May 2022 at Liver Surgery & Liver Transplantation Center,the Third Affiliated Hospital of Sun Yat-sen University were retrospectively analyzed. Among the children who received SLT, there were 40 males and 24 females. The median age at transplantation was 8 months (range:4 to 168 months). Among the patients who received LDLT, there were 24 males and 20 females. The age at transplantation ranged from 4 to 24 months,with a median age of 7 months. Sixty-four children with biliary atresia were divided into two groups according to the SLT operation time: 32 cases in the early SLT group(June 2017 to January 2019) and 32 cases in the technically mature SLT group (February 2019 to May 2022). Rank sum test or t test was used to compare the recovery of liver function between the LDLT group and the SLT group,and between the early SLT group and the technically mature SLT group. The incidence of postoperative complications was compared by χ² test or Fisher exact probability method. Kaplan-Meier method and Log-rank test were used for survival analysis. Results: The cold ischemia time(M (IQR)) (218 (65) minutes), intraoperative blood loss(175 (100) ml) and graft-to-recipient body weight ratio (3.0±0.7) in the LDLT group were lower than those in the SLT group(500 (130) minutes, 200 (250) ml, 3.4±0.8) (Z=-8.064,Z=-2.969, t=-2.048, all P<0.05). The cold ischemia time(457(158)minutes) and total hospital stay ((37.4±22.4)days) in the technically mature SLT group were lower than those in the early SLT group(510(60)minutes, (53.0±39.0)days).The differences were statistically significant (Z=-2.132, t=1.934, both P<0.05).The liver function indexes of LDLT group and SLT group showed unimodal changes within 1 week after operation. The peak values of ALT, AST, prothrombin time, activeated partial thromboplasting time, international normalized ratio, fibrinogen and creatinine all appeared at 1 day after operation, and the peak value of prothrombin activity appeared at 3 days after operation. All indicators returned to normal at 7 days after operation. The 1-,2-,and 3-year overall survival rates were 95.5% in LDLT group and 93.5% in the technically mature SLT group, and the difference was not statistically significant. The 1-,2-,and 3-year overall survival rates were 90.2% in the early SLT group and 93.5% in the technically mature SLT group, and there was no significant difference between the two groups(P>0.05). The main complications of the early SLT group were surgery-related complications(28.1%,9/32), and the main complications of the technically mature SLT group were non-surgery-related complications(21.9%,7/32). There were 5 deaths in the SLT group,including 4 in the early SLT group and 1 in the technically mature SLT group. Conclusion: The survival rate of SLT in the treatment of biliary atresia is comparable to that of LDLT.
Adolescent ; Biliary Atresia/surgery* ; Child ; Child, Preschool ; Creatinine ; Female ; Fibrinogen ; Humans ; Infant ; Liver Transplantation/methods* ; Living Donors ; Male ; Postoperative Complications/epidemiology* ; Prothrombin ; Retrospective Studies ; Treatment Outcome

PURPOSE: The purpose of this study was to define the role of cyclooxygenase-2 inhibitors (COX-2i) in reducing hepatic fibrosis in pediatric patients with chronic liver disease. MATERIALS AND METHODS: From September 2009 to September 2010, patients over 2 years old who visited our outpatient clinic for follow-up to manage their chronic liver disease after Kasai portoenterostomy for biliary atresia, were included in this study. Volunteers were assigned to the study or control groups, according to their preference. A COX-2i was given to only the study group after obtaining consent. The degree of hepatic fibrosis (liver stiffness score, LSS) was prospectively measured using FibroScan, and liver function was examined using serum analysis before and after treatment. After 1 year, changes in LSSs and liver function were compared between the two groups. RESULTS: Twenty-five patients (18 females and 7 males) were enrolled in the study group. The control group included 44 patients (26 females and 18 males). After 1 year, the least square mean values for the LSSs were significantly decreased by 3.91±0.98 kPa (p=0.004) only in the study group. Serum total bilirubin did not decrease significantly in either group. CONCLUSION: COX-2i treatment improved the LSS in patients with chronic liver disease after Kasai portoenterostomy for biliary atresia.
Biliary Atresia/complications/enzymology/*surgery ; Child ; Child, Preschool ; Chronic Disease ; Cyclooxygenase 2 Inhibitors/*therapeutic use ; Female ; Humans ; Liver Cirrhosis/etiology/pathology/*prevention & control ; Male ; *Portoenterostomy, Hepatic ; Thiazines/*therapeutic use ; Thiazoles/*therapeutic use

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.
Adolescent ; Anoxia ; Arteriovenous Fistula/etiology ; Biliary Atresia/*diagnosis/etiology ; Cyanosis/complications ; Dyspnea/complications ; Echocardiography, Transesophageal ; End Stage Liver Disease/complications/*surgery ; Female ; Hepatic Artery/abnormalities ; Hepatopulmonary Syndrome/*diagnosis/ultrasonography ; Humans ; *Liver Transplantation ; Osteoarthropathy, Secondary Hypertrophic/complications

INTRODUCTIONThis study aimed to quantify and investigate factors affecting the health-related quality of life (HRQoL) in children with biliary atresia (BA) living with their native livers.

MATERIALS AND METHODSA cross-sectional study on the HRQoL using the PedsQL4.0 generic core scales in children with BA aged between 2 to 18 years followed up at the University Malaya Medical Centre (UMMC) in Malaysia was conducted. Two groups, consisting of healthy children and children with chronic liver disease (CLD) caused by other aetiologies, were recruited as controls.

RESULTSChildren with BA living with their native livers (n = 36; median (range) age: 7.4 (2 to 18) years; overall HRQoL score: 85.6) have a comparable HRQoL score with healthy children (n = 81; median age: 7.0 years; overall HQRoL score: 87.4; P = 0.504) as well as children with CLD (n = 44; median age: 4.3 years; overall score: 87.1; P = 0.563). The HRQoL of children with BA was not adversely affected by having 1 or more hospitalisations in the preceding 12 months, the presence of portal hypertension, older age at corrective surgery (>60 days), a lower level of serum albumin (≤34 g/L) or a higher blood international normalised ratio (INR) (≥1.2). Children who had liver transplantation for BA did not have a significantly better HRQoL as compared to those who had survived with their native livers (85.4 vs 85.7, P = 0.960).

CONCLUSIONHRQoL in children with BA living with their native livers is comparable to healthy children.

Adolescent ; Age Factors ; Biliary Atresia ; complications ; physiopathology ; psychology ; surgery ; Case-Control Studies ; Child ; Child, Preschool ; Chronic Disease ; Cross-Sectional Studies ; Female ; Health Status ; Humans ; Hypertension, Portal ; etiology ; physiopathology ; psychology ; Liver Diseases ; physiopathology ; psychology ; Liver Transplantation ; Malaysia ; Male ; Quality of Life ; Serum Albumin