Effect of postoperative chemotherapy on the serum alpha-fetoprotein level in hepatoblastoma

Purpose

It is generally accepted that postoperative chemotherapy does not affect the serum alpha-fetoprotein (AFP) level. The authors report on 3 patients who supposedly showed chemotherapy-related changes in their AFP levels after operation.

Methods

This study included 3 patients with hepatoblastoma (1 case of PRETEXT III and 2 cases of PRETEXT IV).

Results

One patient with PRETEXT III underwent a complete tumor resection, and the postoperative AFP level decreased until it reached the normal range. However, he consistently exhibited a transient, 2- to 3-fold increase in the AFP after each course of chemotherapy for 3 courses. The chemotherapy regimen had to be stopped because of drug-induced encephalopathy, but he has been followed up for 5 years without any evidence of recurrence, and his AFP level has also remained stable and in the normal range. Two patients with PRETEXT IV, who underwent a curative tumor resection, also showed similar chemotherapy-related changes in AFP levels. Both of these cases were observed only after the administration of routine postoperative chemotherapy instead of administering further high-dose chemotherapy. The AFP level remained stable for 17 months and 7 months after the cessation of chemotherapy in 2 cases, respectively.

Conclusions

Regarding the postoperative chemotherapy of hepatoblastoma, we have to pay close attention to both the AFP status during chemotherapy as well as the absolute AFP level.     

Association between surgical margins and long-term outcome in advanced hepatoblastoma

Background

Many patients with hepatoblastoma present with unresectable disease. Neoadjuvant therapy has improved resectability rates to as high as 70% to 90%. Despite this improvement, many patients will be left with tumors that are of borderline resectability. The authors hypothesize that favorable outcomes may be achieved even with resection margins less than 1 cm thus sparing the need for liver transplantation.

Methods

Between January 1981 and March 2003, 23 patients age less than 16 years with a diagnosis of hepatoblastoma undergoing surgical resection were identified. The clinical characteristics, pathologic resection margins, and survival status were reviewed.

Results

Eighteen (78%) of the patients were alive with no evidence of recurrence at last follow-up. Thirteen (56.5%) had ≥1 cm resection margins, whereas 10 (43.5%) had resection margins less than 1 cm. Eleven (47.8%) presented with PRETEXT III tumors. There was no significant difference in survival rate between resection margins less than 1 cm and ≥1 cm (P = .13; 95% CI 0.91 to 2.61). Thirteen patients (56.5%) presented with synchronous pulmonary metastatic disease, where survival was significantly worse (P = .04; 95% CI 1.10 to 2.50). Subgroup analysis confirmed that margins less than 1 cm did not significantly affect survival after controlling for pulmonary metastatic disease (P = .56; 95% CI 0.71 to 3.61).

Conclusions

Surgical resection with margins less than 1 cm are associated with survival that is equivalent to resection with margins ≥1 cm. Our findings suggest it is preferable to preserve key structures with a small resection margin and therefore spare the need for liver transplantation in patients with advanced hepatoblastoma.     

Successful liver transplant for unresectable hepatoblastoma

Background

Treatment of children with stage III and IV hepatoblastoma has shown little improvement with 5-year survival rates of 64% and 25%, respectively (J Clin Oncol 2000;18:2665-75). A timely and organized treatment program including preoperative chemotherapy combined with living donor liver transplantation and postoperative chemotherapy has been used seeking improved long-term survival in stage III and IV cases.

Methods

A retrospective review of 8 patients with stage III and IV hepatoblastoma unresectable by conventional resection were treated with complete hepatectomy and transplantation. Approval was obtained from our institutional review board.

Results

Since August of 2001, we have treated 6 patients with stage III hepatoblastoma and 2 patients with initial stage IV hepatoblastoma. These patients (age, 23 months-9 years) had all received extensive chemotherapy or prior resections. After chemotherapy, none had gross tumor documented outside of the liver at time of transplantation. All underwent hepatectomy including vena cava resection, in selected cases, with living donor orthotopic liver transplantation. All patients had at least 2 cycles of postoperative chemotherapy. Of 8 patients, 6 are alive and well with normalized alpha-fetoprotein levels. There were 2 late deaths from recurrent disease. Length of follow-up ranged from 7 to 53 months.

Conclusion

Complete hepatectomy with living donor liver transplantation provides optimal surgical treatment in unresectable stage III and initial stage IV disease confined to the liver at resection. This series indicates that children tolerate complete hepatectomy, transplantation, and postoperative chemotherapy well. Referral to a transplant center during the first 3 cycles of chemotherapy appears to offers the best opportunity for long-term survival.     

Improved survival outcome for hepatoblastoma based on an optimal chemotherapeutic regimen--a report from the study group for pediatric solid malignant tumors in the Kyushu area

Background/purpose

The survival outcome for patients with hepatoblastoma normally depends on the resectability of the tumor. In Japan, the pre and/or postoperative chemotherapy protocol using a combination of cisplatin (CDDP) and tetrahydropyranyl-Adriamycin (THP-ADR) has been the standard treatment since 1991. This study aims to assess exactly what influence the establishment of this chemotherapy protocol has had on both the tumor resectability and the outcome of patients with hepatoblastoma.

Methods

From 1982 to 1997, 60 patients with hepatoblatoma were treated in the Kyushu area, Japan. Based on the pretreatment extent of disease (PRETEXT), the outcome and tumor resectability were compared between group A (1982 to 1990, n = 27, PRETEXT I:5, II:8, III:6, IV:8) and group B (1991 to 1997, n = 33, PRETEXT I:9, II:9, III:5, IV:10).

Results

The 5-year survival rates (group A and group B) were 33% and 73% for all cases (P < .01), 100% and 89% for PRETEXT I, 38% and 89% for II (P < .05), 17% and 80% for III (P < .01), and 0% and 40% for IV (P < .01), respectively. The 5-year survival rates for patients with metastases were 0% for group A (n = 5) and 57% for group B (n = 7; P < .01). The rates of a complete resection of primary tumor were 48% for group A and 67% for group B. In particular, a significant difference was found regarding the complete resection rate between groups A and B in the patients with PRETEXT III (17% for group A and 80% for group B; P < .01). In the patients with an incomplete tumor resection (14 for group A, 11 for group B), the 5-year survival rates were 0% for group A and 45% for group B (P < .01).

Conclusions

The optimal chemotherapeutic regimen of CDDP and THP-ADR was thus found to greatly contribute to the improved survival rate of hepatoblastoma patients. Preoperative chemotherapy resulted in an increased resectability of the tumor, whereas postoperative chemotherapy played an important role in the increased cure rate of cases with either an incomplete tumor resection or metastasis. However, refractory cases with PRETEXT IV or metastasis may still require the development of an even more effective treatment modality, including the use of blood stem cell transplantation.     

Defining hepatoblastoma responsiveness to induction therapy as measured by tumor volume and serum α-fetoprotein kinetics

Purpose

Hepatoblastoma is commonly unresectable at presentation, necessitating induction chemotherapy before definitive resection. To refine the paradigm for timing of resection, we questioned whether a plateau in hepatoblastoma responsiveness to neoadjuvant therapy could be detected by calculating tumor volume (TV) and serum α-fetoprotein (sAFP) kinetics.

Methods

To calculate TV and sAFP as measures of treatment responsiveness over time, infants having initially unresectable epithelial-type hepatoblastomas were identified at a single institution (1996-2008). Effects of therapy type, therapy duration, and lobe of liver involvement on TV, sAFP, margin status, and toxicity were analyzed.

Results

Of 24 infants treated for epithelial-type hepatoblastoma during this interval, 5 were resected primarily, and 15 had complete digital films for kinetics analysis. Both TV and sAFP decreased dramatically over time (P < .0001). No statistically significant difference in mean TV or sAFP was detected after chemotherapy cycle 2. Left lobe tumors had greater presenting levels of and significantly slower decay in sAFP compared with right lobe tumors (P = .005), although no statistically significant differences in TV existed between liver lobes. Resection margins did not change with therapy duration.

Conclusions

Measuring TV and sAFP kinetics accurately reflects hepatoblastoma responsiveness to induction therapy. Treatment toxicities may be reduced by earlier resection and tailoring of chemotherapeutic regimens.     

Efficacy of complete resection for high-risk neuroblastoma: a Children's Cancer Group study

Background/purpose

Previous reports indicate that complete resection of high-risk neuroblastoma improves outcome but may entail high surgical complication rates. The authors evaluated the effect of complete primary site resection on event-free survival (EFS), overall survival (OS), and complication rates in patients entered on a high-risk neuroblastoma treatment protocol.

Methods

A total of 539 eligible patients with high-risk neuroblastoma were entered on protocol CCG-3891. Patients were assigned randomly to continuation chemotherapy or autologous bone marrow transplantation. Surgical resection was performed at diagnosis or after induction chemotherapy. Surgeons assessed resection as complete (CR), minimal residual (<5%, MR), or partial (PR). Incomplete resections received secondary resection or 10 Gy of external beam radiation. Patients were evaluated for EFS, OS, and complications of surgery based on completeness of overall best resection.

Results

The proportion of patients resectable at diagnosis was 27% for CR and 14% for MR. This improved after chemotherapy to 45% and 25%. Complication rates based on completeness of resection were 29%, 38%, and 36% for CR, MR, and PR, respectively. Estimated 5-year EFS rate was 30% ± 3% for patients who achieved CR (n = 210) compared with 25% ± 3% (P = .1010) for those with less than CR (n = 258).

Conclusions

Resectability improved after neoadjuvant chemotherapy. Complete resection did not increase complications. There was a small survival benefit for complete resection. This study suggests that complete resection may still be important in the current era of intense chemotherapy and transplant.     

Utility of PET, CT, and EUS to identify pathologic responders in esophageal cancer

Background

This study evaluates the utility of positron emission tomography (PET), endoscopic ultrasonography (EUS), and computed tomographic (CT) scans to predict pathologic response and survival following preoperative chemoradiation (CRT) in esophageal cancer.

Methods

One hundred three sequential patients with locoregionally advanced esophageal cancer, who were treated with CRT and esophageal resection between May 2001 and November 2003 at the University of Texas M.D. Anderson Cancer Center, were retrospectively reviewed. PET, EUS, and CT were performed before (pre) or after (post) CRT and before surgical resection. PET standardized uptake value (SUV) was defined as maximal uptake in primary tumor.

Results

Most patients were male (91 [88%]) with adenocarcinoma (90 [87%]). Pretreatment clinical stages were: IIA (42 [41%]), IIB (5 [5%]), III (50 [49%]), and IVA (6 [6%]). At the time of surgery, 58 patients (56%) had a pathologic response to CRT (≤10% viable cells). Post-CRT measurements that correlated with pathologic response were: CT esophageal wall thickness (13.3 vs 15.3 mm, p = 0.04), EUS mass size (0.7 vs 1.7 cm, p = 0.01) and PET SUV (3.1 vs 5.8, p = 0.01). Post-CRT PET SUV equal to or greater than 4 had the highest accuracy for pathologic response (76%). Univariate and multivariate Cox regression analysis demonstrated that a post-CRT PET SUV equal to or greater than 4 was an independent predictor of survival (HR, 3.5, p = 0.04).

Conclusions

The FDG-PET SUV is the most accurate noninvasive test to predict long-term survival after preoperative CRT and before surgical resection. Post-CRT FDG-PET cannot, however, rule out residual microscopic disease so esophagectomy should remain a therapeutic option even if the post-CRT imaging modalities are normal.     

Long-term survival after resection of primary adenoid cystic and squamous cell carcinoma of the trachea and carina

Background

Tracheal resection for primary carcinoma may extend survival. We evaluated survival after surgical resection or palliative therapy to identify prognostic factors.

Methods

We conducted a retrospective study of patients diagnosed with primary adenoid cystic carcinoma (ACC) or squamous cell carcinoma (SCC) of the trachea between 1962 and 2002. Laryngotracheal, tracheal, or carinal resection was performed when distant metastasis and invasion of adjacent mediastinal structures were absent and tumor length permitted. Radiotherapy was administered after operation (54 Gy), except in superficial tumors, or as palliation (60 Gy).

Results

Of 270 patients with ACC or SCC (135 each), 191 (71%) were resected. Seventy-nine were not resected due to tumor length (67%), regional extent (24%), distant metastasis (7%), or other reasons (2%). Overall operative mortality was 7.3% (14/191) and improved each decade from 21% to 3%. Tumor in airway margins was present in 40% (17/191) of resected patients (ACC 59% versus SCC 18%) and lymph node metastasis in 19.4% (37/191). Overall 5- and 10-year survival in resected ACC was 52% and 29% (unresectable 33% and 10%) and in resected SCC 39% and 18% (unresectable 7.3% and 4.9%). Multivariate analysis of long-term survival found statistically significant associations with complete resection (p < 0.05), negative airway margins (p < 0.05), and adenoid cystic histology (p < 0.001), but not with tumor length, lymph node status, or type of resection.

Conclusions

Locoregional, not distant, disease determines resectability in primary tracheal carcinoma. Resection of trachea or carina is associated with long-term survival superior to palliative therapy, particularly for patients with complete resection, negative airway margins, and ACC.     

Liver transplantation for childhood hepatic malignancy: a review of the United Network for Organ Sharing (UNOS) database

Background

Orthotopic liver transplantation (OLT) is the only treatment option for unresectable hepatoblastoma (HB) and hepatocellular carcinoma (HCC) in children. Aggregated outcomes of OLT for these hepatic malignancies have not been evaluated in the United Network for Organ Sharing national database.

Purpose

The purpose of this study was to evaluate graft and patient survival in pediatric OLT recipients with HB and HCC.

Methods

Data from the United Network for Organ Sharing Standard Transplant and Research Files were analyzed and included pediatric (<18 years) OLT recipients with HB or HCC from 1987 to 2004. The effects of diagnosis on pretransplant variables were evaluated using analysis of variance methods or χ² tests, as appropriate. Actuarial survival and effect of diagnosis on survival were determined using Kaplan-Meier methods and log-rank tests.

Results

Since 1987, 152 OLTs have been performed in 135 pediatric patients for HB and 43 OLTs in 41 pediatric patients for HCC. Respective 1-, 5-, and 10-year patient survival after OLT was 79%, 69%, and 66% for HB and 86%, 63%, and 58% for HCC (P = .73). The primary cause of death for both groups was metastatic or recurrent disease, accounting for 54% of deaths in the HB group and 86% in the HCC group (P = .338). Patients with hepatoblastoma were younger (mean age, 2.9 ± 2.5 vs 10.4 ± 4.8 years for the HCC group; P < .001) and more likely to receive a living donor organ (16% vs 4%, P = .03). A greater proportion of the patients with HB had previous abdominal surgery than patients with HCC (63% HB vs 37% HCC, P = .04). Pretransplant medical condition and transplant era were associated with graft and patient survival on univariate and multivariate analysis (all P < .05).

Conclusions

Orthotopic liver transplantation remains a viable option for pediatric patients with unresectable primary hepatic malignancies and results in good long-term survival. Pretransplant medical condition is an important predictor of outcome. Thus, in conjunction with better chemotherapy regimens, earlier evaluation for OLT in patients with unresectable HB and HCC may result in yet further improved long-term survival.     

Surgical resection of pulmonary metastatic lesions in children with hepatoblastoma

Background

Although the most common site of metastasis for hepatoblastoma is the lung, the role of thoracotomy and surgical resection of pulmonary metastasis remains uncertain. In this study, we aimed to determine the long-term outcome of patients with hepatoblastoma who have a surgical resection of pulmonary metastatic lesions.

Methods

Patients with hepatoblastoma in the Children's Oncology Group INT-0098 were reviewed. Detailed reports enabling comprehensive review were available for 175 of 181 eligible patients. Prognostic factors (histology, tumor margin, surgical complications, α-fetoprotein) were also reviewed.

Results

Thoracotomy for initial pulmonary metastasis: 38 patients presented with pulmonary metastasis, Children's Oncology Group stage IV. Nine of these 38 underwent thoracotomy and pulmonary metastectomy either before (2), simultaneous (5), or after (2) resection of their primary liver tumor. Eight of these 9 patients with metastectomy were long-term survivors. Thoracotomy for tumor relapse: 20 patients who had previously achieved complete tumor clearance experienced subsequent pulmonary relapse of their tumor (11 stage I or III, 9 stage IV). All 20 patients with pulmonary relapse had salvage chemotherapy; 13 also had thoracotomy and pulmonary metastectomy (8) or thoracotomy and biopsy (5). Only 4 of 13 were long-term survivors: 2 were stage I and 2 were stage IV.

Conclusions

As pediatric surgeons, we varied tremendously in our timing and surgical approach to the management of pulmonary metastasis in hepatoblastoma. This large multicenter review suggests that thoracotomy should be used cautiously in the management of pulmonary relapse and perhaps more aggressively in the management of metastases present at diagnosis that persist after neoadjuvant chemotherapy.     

The use of positron emission tomography in detecting hepatoblastoma recurrence--a cautionary tale

Purpose

The use of positron emission tomography (PET) with [18F] fluorodeoxyglucose (FDG) in the detection of recurrences has been well established in many tumor types. Here the authors present their experience using this modality in the evaluation of posttreatment hepatoblastoma patients.

Methods

The authors conducted a retrospective review on patients with hepatoblastoma diagnosed from 1996 to 2003. FDG-PET imaging was performed together with measurement of alpha-fetal protein (AFP) during posttreatment follow-up.

Results

Sixteen patients (8 boys and 8 girls) were identified in this series. The mean age was 23.5 months (range, 5 months to 4 years). Three posttreatment patients had PET results suggestive of tumor recurrence. One of these patients had normal AFP level and suspected recurrence in the caudate lobe. Radiologic-guided biopsy was performed 3 times, and there was no evidence of tumor. The other 2 patients underwent further liver resections because of mildly raised AFP levels. The histology of these showed regenerative liver tissue only with no hepatoblastoma recurrence.

Conclusions

Although PET has been gaining popularity as a tool in the detection of tumor recurrences worldwide, it has been shown in this series that PET may not be useful in hepatoblastoma patients, and caution must be taken in the interpretation of positive results.     

Liver transplantation is an alternative treatment of hepatocellular carcinoma beyond the Milan criteria

Background

The decision to perform liver transplantation (LT) or liver resection (LR) for patients with hepatocellular carcinoma (HCC) who are beyond the Milan criteria remains controversial.

Methods

We retrospectively analyzed outcome data for 179 patients with HCC beyond the Milan criteria who were treated with LR (n = 135) or LT (n = 44). Univariate and multivariate Cox proportional hazard models were established. Kaplan-Meier survival curves were generated, and a log-rank test was performed to compare group survival status.

Results

Patients who underwent LR group were significantly older, had a lower TNM stage, and were more likely to have unilateral disease and noncirrhotic liver. Significantly more patients in the LR group had recurrence (53.3% vs 29.5%) or died (61.5% vs 43.2%) than patients in the LT group. Recurrence-free survival rates were 11.9% for the LR group and 61.5% for the LT group. The median overall survival duration showed no statistically difference between the LR group (28.0 months) and the LT group (50.0 months).

Conclusions

LT may be the better choice for patients with HCC beyond the Milan criteria.     

Autosomal-Dominant Polycystic Kidney Disease and Kidney Transplantation: Experience of a Single Center

Background

Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disease that frequently leads to end-stage renal disease and is a common indication for kidney transplantation. We sought to evaluate the demographic characteristics, graft and patient survival, and some posttransplantation complications among ADPKD recipients.

Methods

This retrospective study included 445 renal transplant recipients, among whom 48 had ADPKD. We excluded patients with pretransplantation diabetes mellitus. We evaluated patient and graft survivals as well as posttransplantation complications.

Results

There was no difference between the 2 groups with respect to demographic or transplant characteristics, except for older age among the ADPKD group (51.2 ± 8.6 years vs 44 ± 13.1 years; P < .001). We also observed no significant difference with regard to immediate graft function, immunological graft, or patient survival. Although not significant, there was a lower incidence of proteinuria and a greater number of acute rejections among ADPKD patients. As for posttransplantation complications, there was no difference regarding the prevalence of hypertension, but there was more erythrocytosis among the ADPKD group. The incidence of posttransplantation diabetes mellitus was significantly greater in ADPKD patients (33.3% vs 17.1%; P = .009), and remained significant after adjusting for confounding variables by multivariate analysis with an adjusted odds ratio of 2.3 (95% confidence interval, 1.008-5.136; P = .048).

Conclusion

Our results suggested that ADPKD patients display a greater incidence of diabetes mellitus posttransplantation; ADPKD emerged as an independent predictor for this complication.     

Anemia after kidney transplantation in adult recipients: prevalence and risk factors

Objective

To evaluate the prevalence of anemia and appraise its risk factors at 6 months after renal transplantation.

Materials and Methods

This retrospective study was performed between 2008 and 2010 in 2713 adult kidney transplant recipients to determine the prevalence of posttransplantation anemia. Anemia was defined as hemoglobin concentration of 12 g/dL or less in women and 13 g/dL or less in men.

Results

The prevalence of posttransplantation anemia was 52.7%, with severe anemia (hemoglobin ≤11 g/dL) detected in 24.4% of patients. Impaired renal function was the only risk factor associated with anemia (odds ratio, 3.6; P = .047). However, severe anemia after kidney transplantation was correlated with female sex (P = .001), renal allograft dysfunction (P = .00), and cytomegalovirus infection (P = .002).

Conclusion

The present study demonstrated a quite high prevalence of posttransplantation anemia, in particular associated with impaired renal allograft function. Severe anemia was correlated with female sex, degree of kidney graft dysfunction, and cytomegalovirus infection.     

Factors affecting postoperative course and survival after en bloc resection for esophageal carcinoma

Background

To identify factors affecting postoperative course and survival after esophagectomy for cancer and reasons for improved survival over time.

Methods

Complete esophageal resection was attempted for middle and lower third esophageal carcinomas in 386 consecutive patients between January 1982 and January 2002. Two study periods were analyzed: 1982 to 1993 and 1994 to 2002. Prognostic factors were identified by multivariate analysis and the two periods compared.

Results

Hospital mortality rate decreased from 5.4% to 2.9% (p = 0.245). Both anastomotic leakage and pulmonary complications rates decreased from 9.8% to 2.2% (p = 0.001) and 24.1% to 19.3% (p = 0.295), respectively. An increased proportion of patients had R0 resection in the latter period, 78.5% versus 67.0%, (p = 0.028). Five-year survival rate after R0 resection increased from 29% to 46% (p = 0.001), with a decreased recurrence rate from 65.8% to 44.3% (p = 0.002). Three favorable prognostic factors were identified: low pT stage, pN0 stage, and operation during the 1994 to 2002 study period.

Conclusions

Short-term outcome and survival of patients with resected esophageal cancer have improved over time. Advances in perioperative technique, staging methods, and surgical management combined with higher patient selection and use of neoadjuvant chemoradiation may be responsible for this progress.     

Resection of pulmonary metastases in pediatric patients with Ewing sarcoma improves survival

Background

Ewing sarcoma (ES) is the second most common bone tumor in children, and survival of those with metastatic ES has not improved. Previous studies have shown a survival benefit to whole lung irradiation in patients with pulmonary metastases and may be given either before, after, or instead of surgical pulmonary metastasectomy (PM). The contribution of surgery compared with irradiation in ES has not previously been studied.

Methods

A retrospective review of patients younger than 21 years (median age, 16 years) treated at a single institution (1990-2006) was performed. Kaplan-Meier survival curves were compared using log-rank test and a multivariate Cox proportional hazards model. P ≤ .05 was regarded as significant.

Results

Eighty patients with ES were identified. Of these, 31 (39%) had pulmonary metastases. Nine patients had incomplete details of their full treatment regimen, but the following groups could be defined from the remainder: resection alone (n = 5), radiation alone (n = 3), radiation and resection (n = 3), or chemotherapy alone (n = 11). There were 24 deaths overall, with a median overall survival (OS) of 2.7 (95% confidence interval [CI], 1.7-5.2) years. Patients who had PM had the best OS (80%), whereas those who underwent radiation to the lung without PM compared with chemotherapy only for pulmonary metastasis both had similar OS of 0% at 5 years (P = .002). Patients who had radiation followed by PM for lung metastasis had a 5-year OS of 65%. Patients with PM had a longer OS compared with those without lung resection (P < .0001).

Conclusion

These data suggest a possible benefit for ES patients who undergo surgical resection of lung metastases.     

A population-based study of congenital diaphragmatic hernia outcome in New South Wales and the Australian Capital Territory, Australia, 1992-2001

Purpose

The aim of the study was to describe the incidence and survival of infants born with congenital diaphragmatic hernia (CDH) in the state of New South Wales (NSW) and the Australian Capital Territory (ACT), Australia.

Methods

A population-based cohort study of all infants inclusive up to 1 month of age diagnosed with CDH in NSW and the ACT between 1992 and 2001 was conducted. Data sources were the NSW and ACT Neonatal Intensive Care Data Collection (Neonatal Intensive Care Units Study), NSW Birth Defects Register, Population Health Research Centre of ACT Health, and NSW Midwives Data Collection.Individual risk factors for mortality were assessed using the χ² test with P < .05 being considered statistically significant. Multivariate analysis was performed using logistic regression to adjust for potential confounding variables.

Results

From the databases used, the incidence of CDH in NSW and the ACT was 1 per 3800 births. Of 242 infants identified with CDH, 8% underwent termination of pregnancy, 10% were stillborn after 20 weeks' gestation, and 82% were liveborn.Most liveborn infants (70%) were delivered at term with a 64% survival, whereas 30% were preterm with a 35% survival. For liveborn infants, the overall preoperative mortality was 35% with 56% surviving to discharge.Logistic regression identified a low 5-minute Apgar score, prematurity, and air leak as independent risk factors for mortality.

Conclusions

This population-based study of CDH provides us with baseline data for our states. Mortality is high in preterm infants and in the preoperative period. Avoiding preterm delivery and improving preoperative stabilization are the measures most likely to improve survival.     

Excellent Outcome Following Liver Transplantation for Hepatoblastoma Using an Extensive En Bloc Hepatectomy Technique

BackgroundHepatoblastoma is a rare malignancy but the most common primary hepatic malignancy in childhood. Pediatric liver transplantation (LT) offers the possibility to achieve a complete resection in otherwise unresectable tumors. Almost no data are available regarding specific surgical technique of LT in children with hepatoblastoma.MethodsWe analyzed all children with hepatoblastoma and LT between 2007 and 2012. Special regard was given to the surgical technique and long-term follow-up.ResultsOverall 7 children were transplanted with the diagnosis of hepatoblastoma (5 male, 2 female). Thereof, 4 children (median age was 11 months, range, 6–31 months) underwent “primary” LT for hepatoblastoma Pretreatment Extent of Disease III to IV. A 4-year-old boy received “salvage” LT for recurrent hepatoblastoma 2.5 years after successful liver resection. Another 15-year-old boy was transplanted as a prophylactic treatment after repeated liver resection for hepatoblastoma due to the high recurrence risk. A 14-year-old boy underwent LT due to complications following liver resection for hepatoblastoma during infancy. In all children, extensive en bloc hepatectomy was performed together with resection of the adjoining retroperitoneal tissue and regional lymphadenectomy. Actually, all children are alive without tumor recurrence median 7.1 years after LT (range, 5.7–10.7 years).ConclusionOur data show an excellent long-term outcome in selected children with hepatoblastoma undergoing standardized en bloc hepatectomy for “primary” and “rescue” LT with 100% overall and recurrence-free survival.     

Extracorporeal membrane oxygenation in pediatric cardiac transplantation

Background

We reviewed a single institution experience with extracorporeal membrane oxygenation (ECMO) in the perioperative management of cardiac transplantation.

Methods

Of all pediatric cardiac transplant candidates (1984-2003), patients requiring ECMO pretransplantation/posttransplantation were identified, with particular attention to use of ECMO as a bridge to transplantation. Parameters reviewed included proportionate survival, incidence of pre-ECMO cardiac arrest, ECMO duration, and United Network for Organ Sharing list time.

Results

Three hundred patients were listed for transplantation. Twenty-nine required ECMO: 18 pretransplant, 3 pretransplant and posttransplant, 6 posttransplant, and 2 for delayed acute rejection. There were 21 bridge-to-transplant candidates, of which 10 eventually transplanted with 60% survival; 11 not transplanted had no survivors (P = .004). Thirteen of 21 had cardiac arrest pre-ECMO with 1 (8%) survivor; 8 of 21 had no arrest with 5 (63%) survivors (P = .014). Mean ECMO duration and United Network for Organ Sharing list times between transplanted and not transplanted were not significant. Nine received ECMO posttransplantation for cardiopulmonary support; 5 (56%) of 9 survived. Two patients supported with ECMO for rejection-related cardiovascular collapse survived.

Conclusion

ECMO can bridge children to cardiac transplantation. Survival is significantly impaired in bridge-to-transplant candidates stratified by pre-ECMO cardiac arrest. ECMO can also help transition from cardiopulmonary bypass after transplantation and provide effective support during acute rejection.