Marc Reismann

Normalization of serum bile acids after partial external biliary diversion indicates an excellent long-term outcome in children with progressive familial intrahepatic cholestasis.

BACKGROUND/PURPOSE The surgical treatment for patients with progressive familial intrahepatic cholestasis (PFIC) is either liver transplantation (LTX) or partial external biliary diversion (PEBD). Both procedures achieve a good short-term outcome. However, the treatment strategy for these children remains controversial because the long-term outcome after PEBD is unknown. The aim of our study was to assess the long-term outcome and complications after PEBD in our institution. METHODS We retrospectively analyzed the characteristics of all patients with PFIC undergoing PEBD in our department from 1994 to 2008. The course of serum bile acids, pruritus, and liver enzymes was assessed in a regular follow-up. RESULTS Twenty-four patients underwent PEBD. Thirteen patients (54%) improved significantly, with a normalization of serum bile acids (P < .001 vs postoperatively) and lessened pruritus (P < .05 vs preoperatively) at 12 months after PEBD. None of these patients showed progression of cholestasis during a median follow-up of 9.8 years (range, 1.6-14.3 years). Partial external biliary diversion failed to normalize bile acids in 11 patients, of whom 9 required secondary LTX at a 1-year follow-up, with a median interval of 1.9 years (range, 0.5-3.8 years). All 7 patients (100%) with liver cirrhosis at the time of PEBD and 2 of 17 patients without cirrhosis (12%) required secondary LTX (P < .001). CONCLUSIONS Clinical improvement with normalization of serum bile acids within 1 year was associated with an excellent long-term outcome in patients with PEBD. The presence of liver cirrhosis at the time of PEBD indicated an unfavorable outcome. Thus, we recommend primary LTX only in PFIC patients with liver cirrhosis.

Excision of the Dilated Pelvis is not Necessary in Laparoscopic Dismembered Pyeloplasty

PURPOSE The surgical treatment of choice for significant hydronephrosis is dismembered pyeloplasty. While in open surgery, extensive resection of the dilated pelvis is common practice, laparoscopically usually only a sparing resection is performed. We compared the treatment results of both techniques to investigate whether extensive resection is necessary or not in dismembered pyeloplasty procedures. METHODS To obtain comparable renal units, matched pairs according to age and relative kidney uptake as shown by (123)J-orthoiodohippurate renography were selected out of a total of 76 patients who underwent dismembered pyeloplasty between 2000 and 2007. Twenty-four patients complied with the criteria for inclusion in the study. Changes in urinary drainage preoperatively and at three months postoperatively were compared between both groups. RESULTS The mean age in the sparing resection group was 3.8 years (range 0.3 to 14 years); in the extensive resection group it was 3.4 years (range 0.5 to 10 years). Mean urinary drainage improved significantly in both groups from 35.1 +/- 10.7 % to 75.2 +/- 13.2 % (sparing resection) vs. 45.1 +/- 23.7 % to 70.2 +/- 22 % (extensive resection). There were no differences between the groups (p > 0.05). CONCLUSIONS We conclude that extensive resection of the renal pelvis is not necessary in dismembered pyeloplasty procedures since there were no differences in the renographic outcome of comparable patients treated by the different surgical methods.

Carbon dioxide, Hypoxia and Low pH Lead to Overexpression of c-myc and HMGB-1 Oncogenes in Neuroblastoma Cells

PURPOSE It has been reported that CO (2) gas, used to establish a pneumoperitoneum during laparoscopy, affects the behavior of tumor cells. The proto-oncogenes C-MYC and HMGB-1 mediate aggressive behavior of neuroblastomas. We studied whether exposure to CO (2), hypoxia or acidosis affects the expression of C-MYC and HMGB-1 in neuroblastoma cells. METHODS SH-SY5Y cells were incubated with 100% CO (2), 95% helium/5% CO (2) or pH 6.2 for 2 h. The expression of C-MYC and HMGB-1 was measured by Western blot test immediately, 3 h and 6 h after incubation. Additionally, we measured apoptosis after incubation using fluorometric measurements of caspase 3 and 7 activity. RESULTS C-myc (160+/-26%, p=0.007 and 138+/-16% vs. control, p=0.04) and HMGB-1 proteins (140+/-13% and 136+/-11%, both p=0.037) were found to be significantly upregulated 6 h after incubation with CO (2) and helium. There was early upregulation of both oncogenes 3 h after CO (2) incubation (251+/-79%, p=0.04 and 292+/-136, p=0.037). Correspondingly, pH 6.2 led to significant overexpression. Levels of apoptosis were reduced. CONCLUSIONS Exposures mimicking conditions of CO (2) pneumoperitoneum lead to significant overexpression of C-MYC and HMGB-1 in neuroblastoma cells with decreased apoptosis. These results point to a negative influence and potentially increased malignancy of tumor cells.

Thoracoscopic Biopsy in Children With Diffuse Parenchymal Lung Disease

Lung biopsy is necessary for establishing the diagnosis in patients with otherwise unclassified diffuse or localized parenchymal lung disease. This study aimed to assess the safety and accuracy of video‐assisted thoracoscopic (VATS) lung biopsy in children with diffuse parenchymal lung disease (DPLD). In addition we aimed to evaluate the value of this technique with respect to the spectrum of diseases encountered, correlating histological diagnosis with treatment decisions and subsequent clinical outcome. Data from all patients (n = 21) who underwent surgical lung biopsy for suspected DPLD between March 2001 and August 2006 were collected prospectively. Median age was 3 years, 8 months (range 11 days to 15 years, 2 months). All lung biopsies were performed by VATS under general anesthesia. Median operative time was 45 min (range 25–100 min). Conversion to minithoracotomy due to cardiorespiratory difficulties was necessary in two young infants. There were no further intraoperative complications. In 8/21 children, a chest tube was inserted postoperatively for a median of 2 days (range 1–5 days). In one patient, prolonged air‐leakage was managed thoracoscopically on postoperative day 9. There were no other postoperative complications. The specimens were of adequate volume and quality and a histopathological diagnosis was obtained for all patients. There was a broad spectrum of different diagnoses which led to specific therapeutic decisions. Subsequent medical treatment was beneficial in the majority of the patients. In conclusion, VATS is a safe and effective procedure for diagnosis of children with suspected DPLD. Diagnostic accuracy is high, morbidity rates are low, and patients may benefit from avoiding thoracotomy. Pediatr Pulmonol. 2008; 43:992–996.

Implementation of Fast-Track Pediatric Surgery in a German Nonacademic Institution without Previous Fast-Track Experience

Fast-track concepts in pediatric surgery were established in a university hospital in consecutive studies within several years. They significantly reduced the length of hospitalization compared with German institutions without fast-track protocols. The aim of this study was to assess the implementation process of fast-track in a German nonacademic department of pediatric surgery without previous fast-track experience. All patients undergoing four types of operations (appendectomy, hypospadias repair, pyloromyotomy, and fundoplication) from February 2011 to January 2012 were included in this prospective study. Fast-track included detailed clinical pathways and specific pain treatment protocols using validated pain scales according to age. Mobilization and oral nutrition were started 2 hours postoperatively and documented with established scores. The length of hospital stay was compared with data from other hospitals with conventional treatment using information from the German reimbursement system (German diagnosis-related groups [G-DRG]) and with the hospital stay of patients from the corresponding university hospital undergoing fast-track treatment for the same procedures during the same study period. Two weeks after discharge, a questionnaire was completed by the patients/parents. A total of 143 patients with a mean age of 7.9 ± 5.0 years underwent fast-track treatment. The mean pain intensity during the immediate postoperative period was 1.7 ± 2.1 in patients < 4 years and 2.3 ± 2.1 in patients ≥ 4 years on a 10-point scale. Full mobilization was reached after a mean duration of 2.3 ± 2.0 days while full oral nutrition was completed after a mean duration of 1.8 ± 1.4 days. There were no complications associated with fast-track. The mean hospital stay was 5.8 ± 3.4 days which was not significantly different compared with G-DRG data from other hospitals without fast-track. This was in contrast to the mean hospital stay of patients from the corresponding university hospital (5.6 ± 3.0 days vs. G-DRG 6.9 ± 3.2 days, p < 0.05). After 2 weeks, patients/parents were highly satisfied with fast-track (mean score of 8.6 ± 1.4 on a 1-10-point scale) and 95.2% claimed that they would choose it again. Fast-track concepts can be applied in a nonacademic department of pediatric surgery without previous fast-track experience and with excellent patient/parent satisfaction. However, the G-DRG system interferes with concepts of early discharge of patients. Modifications of the reimbursement modalities within the German health care system seem to be mandatory.

Thoracoscopic resection of congenital pulmonary malformations in infants: is the feasibility related to the size of the lesion?

BackgroundThe size of congenital pulmonary malformation (CPM) in infants might interfere with the feasibility of thoracoscopic resection. This study was undertaken to evaluate the impact of the size of CPM on the applicability of video-assisted thoracic surgery (VATS) in infants.MethodsTwenty-two infants were operated on for CPM from November 2000 to June 2009. The intra- and postoperative course was analyzed retrospectively from patient charts. Preoperative scans were evaluated blindly by a radiologist to calculate the relation between the maximum size of the lesion and the thoracic diameter in VATS and open procedures.ResultsVATS was performed in 14 (64%) of the 22 patients and thoracotomy in 8. VATS was successfully performed in 11 (79%) of the 14 patients, whereas VATS was converted to thoracotomy due to lack of overview in 3 (21%). The mean relative size of CPM at preoperative imaging was 0.34 ± 0.05 (range: 0.3–0.4) in patients who received successful VATS, 0.57 ± 0.06 (range: 0.5–0.6) in converted cases, and 0.68 ± 0.10 (range: 0.5‥8) in infants who underwent thoracotomy. The relative CPM size was significantly lower in successful VATS than in cases of conversion (P<0.01) and thoracotomy (P<0.01).ConclusionsThe relative size of CPM at preoperative imaging might be useful information for a decision-making on the use of VATS in infants. A relative CPM size below 0.5, which is less than half of the thoracic diameter, indicates a good feasibility for thoracoscopic resection of CPM. A larger size may indicate that VATS might be technically difficult.