Carmen Dingemann

Long-term health-related quality of life after complex and/or complicated esophageal atresia in adults and children registered in a German patient support group

BACKGROUND Health-related quality of life (HRQoL) after esophageal atresia (EA) repair is postulated to be good. However, little is known about the long-term results after repair of complex and/or complicated EA regarding HRQoL. We investigated long-term HRQoL after delayed anastomosis, esophageal replacement, major revisions, or multiple dilatations in patients registered in a support group. METHODS Patients registered in the German patient support group database (KEKS) were enrolled and allocated to subgroups according to surgical treatment and age. HRQoL was evaluated using validated questionnaires (GIQLI, WHO-5, KIDSCREEN27). RESULTS Complete follow-up (mean 14.5 ± 9.8 years) was available for 90/92 patients. Patients were allocated to subgroups delayed anastomosis (n=28), esophageal replacement (n=27), major revisions (n=15), and multiple dilatations (n=20). Adult patients presented with impaired well-being according to WHO-score and gastrointestinal function (GIQLI). In contrast, HRQoL of children was comparable to controls in most KIDSCREEN27-dimensions. Delayed anastomosis was associated with most-favourable HRQoL. Regarding physical well-being, these children scored significantly better than controls [64.01 ± 10.40 vs. 52.36 ± 8.73;p=0.0011], children after replacement [51.40 ± 5.70;p=0.008], revisions [52.04 ± 6.97;p=0.026], and multiple dilatations [50.22 ± 9.67,p=0.04]. CONCLUSIONS HRQoL after complex and/or complicated EA is excellent in children registered in a patient support group. In adults, disease-specific symptoms negatively affect HRQoL. Our data indicate that saving the esophagus may achieve the best HRQoL.

Early complications after esophageal atresia repair: analysis of a German health insurance database covering a population of 8 million.

The treatment of esophageal atresia is not centralized in Germany. Therefore, high numbers of departments are involved. Data on the results of esophageal atresia repair from Germany are lacking. The aim of this study was to evaluate the early postoperative results after repair of esophageal atresia based on unbiased data of a German health insurance. We aimed to determine whether characteristics of the departments had an impact on outcome and compared the results from this study with the literature data from centers with a high caseload. Data of a German health insurance covering ∼10% of the population were analyzed. All patients who had undergone esophageal atresia repair from January 2007 to August 2012 were included. Follow-up data of 1 year postoperatively were analyzed. The potential impact of various characteristics of the treating surgical institutions was assessed. Results were compared with the latest international literature. Seventy-five patients with esophageal atresia underwent reconstructive surgery in 37 departments. The incidences of anastomotic leak (3%) and recurrent tracheoesophageal fistula (7%) were comparable with the literature (both 2-8%). Anastomotic stricture required dilatation in 57% of patients (mean 5.1 ± 5.6 dilatations) comparing unfavorably to most, but not all international reports. During 1-year follow-up, 93% of the patients were readmitted at least once (mean 3.9 ± 3.1 admissions). The incidence of complications did not correlate with any of the characteristics of the treating institutions such as academic affiliation, the number of consultants, beds, and preterm infants treated per year (all P > 0.05). Based on unbiased data, postoperative results after repair of esophageal atresia in Germany are comparable with recently published reports from international single centers. A correlation between the complication rate and characteristics of the treating institutions was not identified.

Thoracoscopic procedures in pediatric surgery: what is the evidence?

INTRODUCTION Video-assisted thoracoscopic surgery (VATS) has gained enormous acceptance among pediatric surgeons. However, most studies on advantages of VATS do not reach a high level of evidence. According to a recent classification of the Oxford Centre for Evidence-Based Medicine (CEBM), studies can be classified into Levels 1 to 5 in order of descending quality. We aimed to identify comparative studies investigating VATS versus open procedures in pediatric surgery and to classify publications according to the CEBM criteria. MATERIALS AND METHODS Systematic review of comparative studies were identified using PubMed. Only studies published in English, comparing pediatric VATS with the corresponding open operation were included. End points were advantages and disadvantages of VATS as compared with the open procedure. Levels of evidence were determined using the recent CEBM criteria. RESULTS A total of 3 meta-analysis (MA) and 18 retrospective comparative studies (RCS) investigating 5 different VATS procedures (repair of congenital diaphragmatic hernia [CDH], repair of esophageal atresia/tracheoesophageal fistula (EA/TEF), lung resection, treatment of pneumothorax, and resection of neuroblastoma) were included in this study. No studies of CEBM Level 1 or Level 2 were identified. All studies were classified as CEBM Level 3. The advantages of VATS were less postoperative pain (CDH repair, EA/TEF repair, and pneumothorax repair), shorter hospital stay (CDH repair, EA/TEF repair, lung resection, and pneumothorax), shorter time of ventilation and lower Pco 2 (CDH repair), shorter duration of chest drain (lung resection), and less blood loss (resection of neuroblastoma). However, disadvantages such as higher recurrence rates (CDH repair), higher Pco 2 (EA/TEF repair), and longer operative times (CDH and EA/TEF repair) were also identified. CONCLUSION Only RCS on pediatric VATS are available. Therefore, the best available evidence is Level 3. Randomized controlled trials comparing VATS and the corresponding open procedure are mandatory to obtain the highest possible evidence.

Thoracic Wall Reconstruction for Primary Malignancies in Children: Short- and Long-term Results

AIM Primary thoracic wall malignancy is a rare and diverse entity in children. Surgical treatment commonly involves major chest wall resection causing large defects requiring complex reconstruction. In adults, the use of alloplastic and/or xenogenic materials and muscle flap repair is well established. However, literature provides only little information on procedures in children. We report our experience in 8 consecutive children who underwent chest wall resection and reconstruction with regard to surgical treatment and outcome. PATIENTS AND METHODS Retrospective study of all children with primary malignant chest wall tumors requiring rib resection and reconstruction with prosthetic material performed in our institution between November 2002 and April 2010. Endpoints were postoperative complications and long-term results, focusing on scoliosis defined radiologically by the Cobb angle. RESULTS 8 children (7 male, 1 female) with a median age of 10.6 (4.1-18.9) years underwent resection of thoracic wall tumors. A mean number of 3 (1-5) ribs were resected. Stability was obtained using rigid prosthetic material (STRATOS™ titanium bar) in 2 patients and/or non-rigid prosthetic material (Goretex® patch in 6 patients, Vicryl® patch in 3 patients, Tutopatch® in 1 patient). A muscular flap was added in 5 patients. Postoperative complications included superficial wound infection (n = 2) and dislocation of a titanium bar necessitating removal in 1 patient. No infections of the prosthetic material were observed. No perioperative mortality occurred. At a mean follow-up of 37.5 (4-97) months, 6 patients were alive. 2 patients died due to early tumor recurrence. Mild scoliosis (Cobb angle 10-20°) was detected in 2 of the surviving patients (33%). CONCLUSION Surgical reconstruction after resection of malignant thoracic wall tumors using non-rigid prosthetic material is safe and effective in pediatric patients, whereas rigid prosthetic material might dislocate. Scoliosis represents a long-term complication after chest wall reconstruction and should be monitored during routine follow-up.

Thoracoscopic repair of oesophageal atresia: results of a selective approach.

INTRODUCTION The repair of oesophageal atresia (OA) and tracheoesophageal fistula (TOF) may be routinely performed via thoracoscopy. However, data on the feasibility and efficacy of thoracoscopic OA/TOF repair are scarce and some authors reported a high rate of anastomotic leak. The aim of this study was to evaluate our concept of OA/TOF repair which includes specific selection of patients and a selective surgical approach. PATIENTS AND METHODS The study was approved by the Institutional Review Board and written informed consent was obtained from all guardians for anonymized data analysis. All patients undergoing OA/TOF repair from June 2001 to December 2011 were included in this analysis. Thoracoscopy was used in cardiorespiratory stable newborns with OA/TOF and a birthweight of ≥ 2000 g. In case of any intraoperative adverse events, lack of progress for ~15 minutes, or anastomosis under tension, the procedure was converted to open thoracotomy. Study endpoints were conversion rate and postoperative complications. RESULTS A total of 44 newborns with OA/TOF were operated, of whom 22 (6 females, 16 males) met our criteria for a thoracoscopic approach. The mean birthweight of thoracoscopically operated patients was 2760 (2020 to 3960) g, 7 were < 36 weeks of gestation. The mean operative time was 142 (75 to 220) minutes. Thoracoscopy was converted to thoracotomy in eight patients due to problems with exposition (n = 2), ventilation (n = 3), anastomosis under tension (n = 2), or bleeding (n = 1, no transfusion). There was no anastomotic leak in the group of patients who underwent successful thoracoscopic repair, but one recurrence of TOF. Two anastomotic leaks emerged in patients in whom the operation was converted. A total of 7 out of the 22 children required endoscopic dilatation. There was no mortality during a mean follow-up of 5.5 years (43 days to 10.6 years). CONCLUSION Our approach included meticulous patient selection and prompt conversion in case of any adverse events. With this approach, thoracoscopic repair of OA/TOF can be safely performed achieving excellent results and a low rate of complications.

Our Experience with Single Lung Ventilation in Thoracoscopic Paediatric Surgery

INTRODUCTION Data on the feasibility and effects of single lung ventilation (SLV) in children are scarce. We conducted a retrospective study on the feasibility of SLV during video-assisted thoracoscopic surgery (VATS) in children and adolescents undergoing major thoracic procedures. METHODS A retrospective chart review of all records from patients who underwent VATS at our institution from 2000 to 2010 was done. Patients receiving SLV were analysed in detail. Endpoints of the analysis were conversion to open thoracotomy (frequency and reasons), postoperative duration of ventilation, and pulmonary complications such as radiologically confirmed atelectasis and pneumonia. RESULTS 74 out of 305 patients (24%, 43 boys, 31 girls) with a mean age of 9.4 years (56 days-18 years) and mean weight of 34 kg (4.5-76 kg) had SLV. Lung resection was done in 43 (58%), pleural surgery in 17 (23%), a combination of both in 7 (9%), and mediastinal procedures in 7 (9%). 11 patients (15%) required conversion of VATS to open surgery, mostly because of problems with exposure of the operative field (73%). 32 patients (43%) were extubated immediately after the operation, whereas 8 (11%) required ventilation for more than 24 h. The mean intensive care unit stay was 1.6 days. 18 patients (24%) developed radiologically confirmed atelectasis, and 1 patient (1%) required bronchoscopic clearance. Pneumonia occurred in 1 case (1%) and was successfully treated with antibiotics. CONCLUSION SLV is feasible in children and adolescents undergoing VATS for a broad spectrum of procedures. However, despite SLV, the conversion rate in our series was 15%. The main reason for conversion was problems with exposure of the operative field. The complication rate for SLV was low. Atelectasis developed in every fourth patient but usually resolved spontaneously, and intervention to achieve ventilation was rarely indicated.

Minimally invasive repair of esophageal atresia: an update.

We reviewed the available literature on the role of minimally invasive techniques for esophageal atresia (EA)/tracheoesophageal fistula (TEF) repair. No prospective studies have been published to date. According to the recent classification of the Oxford Centre for Evidence based Medicine, the best available evidence for studies comparing the minimally invasive versus open approach for EA/TEF repair is Level 3 (a, b). Similar postoperative results of the thoracoscopic versus conventional repair are confirmed in four retrospective comparative studies and one meta-analysis. However, the available data on complications and postoperative esophageal function are derived from series operated by experienced surgeons in specialized centers. More data on the impact of the learning curve are mandatory before a recommendation on a widespread use can be made. Patient selection and a low threshold for conversion may further improve results.

Development and pilot-testing of a condition-specific instrument to assess the quality-of-life in children and adolescents born with esophageal atresia

The survival rate of children with esophageal atresia has today reached 95%. However, children are at risk of chronic morbidity related to esophageal and respiratory dysfunction, and associated anomalies. This study describes the pilot testing of a condition-specific health-related quality-of-life instrument for children with esophageal atresia in Sweden and Germany, using a patient-derived development approach consistent with international guidelines. Following a literature review, standardized focus groups were conducted with 30 Swedish families of children with esophageal atresia aged 2-17 years. The results were used for item generation of two age-specific pilot questionnaire versions. These were then translated from Swedish into German with considerations of linguistic and semantical perspectives. The 30-item pilot questionnaire for children aged 2-7 years was completed by 34 families (parent report), and the 50-item pilot questionnaire for children aged 8-17 years was completed by 52 families (51 child report, 52 parent report), with an overall response rate of 96% in the total sample. Based on predefined psychometric criteria, poorly performing items were removed, resulting in an 18-item version with three domains (Eating, Physical health and treatment, Social isolation and stress,) for children aged 2-7 years and a 26-item version with four domains (Eating, Social relationships, Body perception, and Health and well-being) for children aged 8-17 years. Both versions demonstrated good internal consistency reliability and acceptable convergent and known-groups validity for the total scores. The study identified specific health-related quality-of-life domains for pediatric patients with esophageal atresia, highlighting issues that are important for follow-up care. After field testing in a larger patient sample, this instrument can be used to enhance the evaluation of pediatric surgical care.

Single- and double-lung ventilation in infants and children undergoing thoracoscopic lung resection.

INTRODUCTION Video-assisted thoracoscopic surgery (VATS) has gained wide acceptance for the pediatric population. Single-lung ventilation (SLV) has been suggested for thoracoscopic lung resection to provide better surgical exposure, but its role and sequelae compared with double-lung ventilation (DLV) have not been determined. The aim of this study was to investigate the feasibility and effects of SLV and DLV in infants and children undergoing thoracoscopic lung resection. PATIENTS AND METHODS Written informed consent from all guardians for anonymized data analysis and approval by the Institutional Review Board were obtained. A retrospective study on a consecutive series of infants and children who underwent thoracoscopic lung resection during an 11 years period was performed. SLV was selected mainly in lesions localized in the upper, middle, and/or central lung for reasons of surgical exposure. Patients with lower lobe lesions and those who underwent atypical resections were preferably operated under DLV. End points were conversion rate, duration of postoperative ventilation, and perioperative complications, such as, atelectasis or pneumonia. RESULTS Of 114 pediatric patients (58 female and 56 male; ratio 1.04:1) with a mean age of 7.1 years (3 days to 18.1 years), 62 patients underwent DLV and 52 patients underwent SLV for thoracoscopic lung resection. There were no significant differences between the two groups for conversion rate (DLV 8.1 vs. SLV 6.1%; p = 0.53), prompt extubation (DLV 50 vs. SLV 34.6%; p = 0.14), and postoperative atelectasis (DLV 35.5 vs. SLV 25%; p = 0.32). No major cardiorespiratory events, such as bleeding or pneumonia, were observed. No perioperative mortality occurred. CONCLUSIONS This is the first study on safety, effectiveness, and outcome of SLV and DLV in pediatric patients undergoing thoracoscopic lung resection. Our data suggest that both SLV and DLV can be safely performed with similar low rate of surgical complications, when specific selection criteria are applied.

Surgical Management of Congenital Abdominal Wall Defects in Germany: A Population-Based Study and Comparison with Literature Reports

Introduction We aimed to analyze for the first time the characteristics, treatment modalities, and outcomes in infants with congenital abdominal wall defects (CAWDs) in Germany and to compare the results with current literature reports. Patients and Methods Data of a health insurance covering approximately 10% of the German population were analyzed. Patients who had undergone CAWD closure during a period of nearly 6 years were included. Surgical approach was categorized into primary versus secondary closure. Complications were defined as any reintervention within 1 year after initial treatment. Results Patients with gastroschisis were treated in 24 centers, newborns with omphalocele in 34 centers. There was no mortality, and the type of surgical approach had no significant impact on the incidence of complications in both gastroschisis and omphalocele. Out of 39 patients with gastroschisis, 72% had undergone primary closure being associated with a shorter duration of ventilation (p = 0.003) and hospitalization (p < 0.001). Out of 54 patients with omphalocele, 54% had undergone secondary closure, whereas modality of management did not affect duration of ventilation and hospitalization. Although heterogeneous, data of the current literature were comparable to those of this study. Conclusion Unbiased data demonstrate for the first time that the quality of the current surgical management of newborns with CAWD across Germany is excellent. There was no correlation of complications with the method of closure in gastroschisis and omphalocele.