Chinsu Liu

Thromboelastography-guided transfusion decreases intraoperative blood transfusion during orthotopic liver transplantation: randomized clinical trial.

OBJECTIVE To test in a prospective randomized study the hypothesis that use of thromboelastography (TEG) decreases blood transfusion during major surgery. MATERIAL AND METHODS Twenty-eight patients undergoing orthotopic liver transplantation were recruited over 2 years. Patients were randomized into 2 groups: those monitored during surgery using point-of-care TEG analysis, and those monitored using standard laboratory measures of blood coagulation. Specific trigger points for transfusion were established in each group. RESULTS In patients monitored via TEG, significantly less fresh-frozen plasma was used (mean [SD], 12.8 [7.0] units vs 21.5 [12.7] units). There was a trend toward less blood loss in the TEG-monitored patients; however, the difference was not significant. There were no differences in total fluid administration and 3-year survival. CONCLUSION Thromboelastography-guided transfusion decreases transfusion of fresh- frozen plasma in patients undergoing orthotopic liver transplantation, but does not affect 3-year survival.

Esophageal Atresia with Tracheoesophageal Fistula: Ten Years of Experience in an Institute

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.

A longitudinal cohort study of incidence rates of inguinal hernia repair in 0- to 6-year-old children.

BACKGROUND/PURPOSE This study provides epidemiologic data on the incidence of inguinal hernia repair in preschool children using the Taiwan National Health Insurance Research Database. We believe that the data on hernia repair in said database provide a close approximation of the true incidence of inguinal hernia in young children. METHOD A cohort of 1,073,891 deidentified individuals was randomly selected from an insured population of 23 million. Subjects born during the period 1997-2004 were followed from birth to 6 years. The chi-square test and logistic regression modeling were used for statistical analyses. RESULT A total of 92,308 individuals were born during the study period. Of these individuals, 3881 underwent hernia repairs. The cumulative incidence of hernia repair in children aged 0 to 6 years was 4.20%/7 years. The boy/girl ratio was 4.27:1 and the unilateral/bilateral ratio was 3.77:1. The incidence of hernia repair among boys was highest during the first year of life, but then decreased with age. In contrast, the incidence among girls remained stable during the first 6 years of life. Boys younger than 1 year had more bilateral repairs than boys in other age groups (p<0.0001) and girls had significantly more bilateral repairs than boys (p<0.0001). Subjects with a history of preterm birth also had a higher incidence of hernia repair than subjects who were born at full term (odds ratio=2.34, p<0.0001). CONCLUSION Yearly incidence of hernia repair was obtained from a nationwide database. Some of the observations have not been reported elsewhere.

Domino liver graft from a patient with homozygous familial hypercholesterolemia.

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.

Venoplasty of hepatic venous outflow with a venous patch in domino liver transplantation.

The domino donor (DD) was a 16-year-old boy with homozygous familial hypercholesterolemia. He received a whole liver graft from a 9-year-old deceased donor. We designed the hepatic outflow to be reconstructed by side-to-side cavocaval anastomosis for the first liver transplantation, so the DD hepatectomy was performed with preservation of the inferior vena cava (IVC) and the stumps of 3 hepatic veins were preserved as long as possible.

Surgical treatment of chylothorax caused by cardiothoracic surgery in children

Four pediatric cases of chylothorax after cardiothoracic surgery, which were managed surgically, are reviewed retrospectively. All patients underwent right thoracotomy and mass ligation of the right thoracic duct without detecting the true site of leakage. Although 1 patient died from heart failure the day after operation, the other 3 recovered quickly without sequelae. Based on our limited experience, we suggest that right thoracotomy with mass ligation of the right thoracic duct can successfully cure chylothorax on either side, particularly if identification of the site of leakage is considered too risky because of severe adhesion from previous cardiothoracic surgery.

Acute life-threatening arrhythmias caused by severe hyperkalemia after induction of anesthesia in an infant with methylmalonic acidemia.

Methylmalonic acidemia (MMA) is a very rare genetic disease of metabolism that progressively leads to neurological and renal sequelae. This report describes an unusual case of a patient with MMA who developed severe hyperkalemia and severe dysrhythmia during anesthesia. A 13-month-old male infant with MMA underwent urgent insertion of a port-a-cath under general anesthesia. A life-threatening arrhythmia suddenly occurred, with severe hyperkalemia (up to 7.4 mmol/L), immediately following induction of anesthesia. Emergent resuscitation was successfully carried out, with a complete neurological recovery after 7 days after surgery. Although MMA is a rare complication, the possibility of severe hyperkalemia should be considered in the differential diagnosis of patients with MMA presenting with wide QRS complex tachycardia. The management and intraoperative complications of this disorder are reported here, and the available literature is reviewed.

Renal Cell Carcinoma in Children and Young Adults

Renal cell carcinoma (RCC) is a relatively uncommon tumor in childhood. Its biologic behavior and prognostic factors have rarely been documented. We report treatment and survival of 4 children (aged 8, 9, 11, and 14 years) who had RCC, along with a review of the literature to analyze the frequency of major symptoms, clinical stage, and prognostic factors based on 130 published cases of RCC in individuals younger than 20 years of age. Two of our cases had renal tumors detected by ultrasound screening, and all 4 cases were followed for a considerable length of time and were alive and free of disease after treatment. An analysis of these 130 published cases of pediatric RCC showed tumor staging and cell type to be the factors that affected patient survival. Tumors composed of granular cells or mixed cells, or at advanced stages, had a poor prognosis. Age, sex, tumor size, symptom duration, and cellular pattern were not related to patient prognosis. Children older than 10 years of age, who have an abdominal mass, flank pain, and/or hematuria should alert clinicians to consider the possibility of RCC. The importance of early diagnosis of renal tumors, using ultrasound as a tool of screening, is emphasized, since surgical treatment leads to a favorable prognosis only in the early stage of RCC.

Second hernia repairs in children—a nationwide study

BACKGROUND Second inguinal hernia repairs may be needed either owing to contralateral metachronous hernia (MH) or ipsilateral recurrent hernia (RH). In this study, we estimated the incidence rates of MH and RH from a large nationwide database. METHODS The information was obtained from the National Health Insurance Database (with 23 million insurants). Subjects with hernia repairs were identified, and information such as age, gender, unilateral/bilateral procedures in the first and second hernia repairs were analyzed. RESULTS Among the 92,308 newborns observed from their births to the end of 6th year, 3881 had first hernia repairs. Among the 3068 subjects with first unilateral repairs, 307 had second repairs, and among the 813 with first bilateral repairs, 15 had second repairs. The incidence of second repairs was 10.85% (13.71% if <1year old) after first unilateral repairs and was 1.23% (0.63% if <1year old) after first bilateral repairs. CONCLUSIONS The incidence of RH (estimated from second hernia repairs after first bilateral repairs) was 1.23%. The incidence of MH (from second repairs after first unilateral repairs) was 9.62%. These incidence rates are consistent with other published reports.

Surgical outcomes of total colonic aganglionosis in children: A 26-year experience in a single institute

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 Hirschsprungs 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.