A concise pathophysiological model of acute appendicitis against the background of the COVID-19 pandemic.

One of the most common clinical pictures has become the focus of attention during the COVID-19 pandemic: acute appendicitis with the associated diagnostics and therapy. The aim of the work is to show inconsistencies with regard to epidemiology, pathophysiology and therapy against the background of the pandemic with special attention to the conditions for children and to explain the pathophysiological processes that are likely to underlie the disease based on scientifically plausible models.

The application of artificial intelligence methods to gene expression data for differentiation of uncomplicated and complicated appendicitis in children and adolescents - a proof of concept study.

BACKGROUND: Genome wide gene expression analysis has revealed hints for independent immunological pathways underlying the pathophysiologies of phlegmonous (PA) and gangrenous appendicitis (GA). Methods of artificial intelligence (AI) have successfully been applied to routine laboratory and sonographic parameters for differentiation of the inflammatory manifestations. In this study we aimed to apply AI methods to gene expression data to provide evidence for feasibility. METHODS: Modern algorithms from AI were applied to 56.666 gene expression data sets from 13 patients with PA and 16 with GA aged 7-17 years by using resampling methods (bootstrap). Performance with respect to sensitivities and specificities where investigated with receiver operating characteristic (ROC) analysis. RESULTS: Within the experimental setting a best performing discriminatory biomarker signature consisting of a set of 4 genes could be defined: ERGIC and golgi 3, regulator of G-protein signaling 2, Rho GTPase activating protein 33, and Golgi Reassembly Stacking Protein 2. ROC analysis showed a mean area under the curve of 84%. CONCLUSIONS: Gene expression based application of AI methods is feasible and represents a promising approach for future discriminatory diagnostics in children with acute appendicitis.

Diagnosis and classification of pediatric acute appendicitis by artificial intelligence methods: An investigator-independent approach.

Acute appendicitis is one of the major causes for emergency surgery in childhood and adolescence. Appendectomy is still the therapy of choice, but conservative strategies are increasingly being studied for uncomplicated inflammation. Diagnosis of acute appendicitis remains challenging, especially due to the frequently unspecific clinical picture. Inflammatory blood markers and imaging methods like ultrasound are limited as they have to be interpreted by experts and still do not offer sufficient diagnostic certainty. This study presents a method for automatic diagnosis of appendicitis as well as the differentiation between complicated and uncomplicated inflammation using values/parameters which are routinely and unbiasedly obtained for each patient with suspected appendicitis. We analyzed full blood counts, c-reactive protein (CRP) and appendiceal diameters in ultrasound investigations corresponding to children and adolescents aged 0-17 years from a hospital based population in Berlin, Germany. A total of 590 patients (473 patients with appendicitis in histopathology and 117 with negative histopathological findings) were analyzed retrospectively with modern algorithms from machine learning (ML) and artificial intelligence (AI). The discovery of informative parameters (biomarker signatures) and training of the classification model were done with a maximum of 35% of the patients. The remaining minimum 65% of patients were used for validation. At clinical relevant cut-off points the accuracy of the biomarker signature for diagnosis of appendicitis was 90% (93% sensitivity, 67% specificity), while the accuracy to correctly identify complicated inflammation was 51% (95% sensitivity, 33% specificity) on validation data. Such a test would be capable to prevent two out of three patients without appendicitis from useless surgery as well as one out of three patients with uncomplicated appendicitis. The presented method has the potential to change today's therapeutic approach for appendicitis and demonstrates the capability of algorithms from AI and ML to significantly improve diagnostics even based on routine diagnostic parameters.

Sonographic differentiation of complicated from uncomplicated appendicitis.

OBJECTIVE: This study aims to differentiate acute uncomplicated and complicated appendicitis, by investigating the correlation between sonographic findings and histological results in different types of paediatric appendicitis. METHODS: This is a retrospective study of 1017 paediatric patients (age < 18 years) who underwent ultrasound by paediatric radiologists before appendicectomy at our institution between 2006 and 2016. Histologically, uncomplicated appendicitis was primarily associated with transmural infiltration of neutrophil granulocytes, while complicated appendicitis was characterised by transmural myonecrosis. Logistic regression analyses were used to investigate the association between sonographic and histological findings. RESULTS: Out of 566 (56%) male and 451 (44%) female patients with a mean age of 10.7 years, uncomplicated appendicitis was histologically diagnosed in 446 (44%) children and complicated appendicitis was diagnosed in 348 (34%) cases. The following ultrasound findings were significantly associated with complicated appendicitis in multivariate regression: an increased appendiceal diameter (OR = 1.3, p < .001), periappendiceal fat inflammation (OR = 1.5, p = 0.02), the presence of an appendicolith (OR = 1.7, p = 0.01) and a suspected perforation (OR = 6.0, p < .001) by the pediatric radiologist. For complicated appendicitis, an appendiceal diameter of more than 6 mm had the highest sensitivity (98%), while a sonographically suspected perforation showed the highest specificity (94%). CONCLUSION: Abdominal sonography by paediatric radiologists can differentiate between uncomplicated and complicated appendicitis in paediatric patients by using an increased appendiceal diameter, periappendiceal fat inflammation, the presence of an appendicolith and a suspected perforation as discriminatory markers. ADVANCES IN KNOWLEDGE: This paper demonstrates expanded information on ultrasound, which is not only an essential tool for diagnosing appendicitis, but also a key method for distinguishing between different forms of appendicitis when performed by paediatric radiologists. Compared with previous studies, the crucial distinction features in our analysis are 1) the definition of gangrene and not primarily perforation as an acute complicated appendicitis enabling early decision-making by sonography and 2) a large number of patients in a particularly affected age group.

Correction to: Phlegmonous appendicitis in children is characterized by eosinophilia in white blood cell counts.

The original article can be found online.

Phlegmonous appendicitis in children is characterized by eosinophilia in white blood cell counts.

BACKGROUND: Phlegmonous and complicated appendicitis represent independent entities depending on hereditary immunological mechanisms. However, clinically there are no means to distinguish uncomplicated phlegmonous from complicated appendicitis. The ability to distinguish these two forms of appendicitis is relevant as current attempts are to treat both forms of the disease differently. The aim of the present study was to investigate differences in white blood cell counts (WBCs) in these conditions to identify areas of interest for future molecular studies. METHODS: White blood cell counts of patients aged between 7 and 14 years who underwent appendectomy from January 2008 to June 2016 were investigated with special reference to particular cellular subpopulations. RESULTS: A total of 647 children were included in the study. Within distinct inflammatory patterns, significant eosinophilia and basophilia were found in phlegmonous inflammation compared with complicated inflammation (0.11 ± 0.19 × 109/L vs. 0.046 ± 0.104 × 109/L, P < 0.0001, and 0.033 ± 0.031 × 109/L vs. 0.028 ± 0.024 × 109/L, P < 0.001). CONCLUSIONS: Compared with complicated disease, phlegmonous appendicitis seems to depend primarily on eosinophil inflammation. This observation is stable over time and indicates a direction for investigation of underlying genetic prerequisites.

Obstruction of peritoneal dialysis catheter is associated with catheter type and independent of omentectomy: A comparative data analysis from a transplant surgical and a pediatric surgical department.

BACKGROUND: Peritoneal dialysis (PD) catheter occlusion is a common complication with up to 36% of catheter obstructions described in the literature. We present a comparison of complications and outcome after implantation of PD catheters in a transplant surgical and a pediatric surgical department. METHODS: We retrospectively analyzed 154 PD catheters, which were implanted during 2009-2015 by transplant surgeons (TS, University Medical Center Hamburg-Eppendorf, Germany, n=85 catheters) and pediatric surgeons (PS, Charité University Medicine Berlin, Germany, n=69 catheters) in 122 children (median (range) age 3.0 (0.01-17.1) years) for acute (n=65) or chronic (n=89) renal failure. All catheters were one-cuffed or double-cuffed curled catheters, except that straight catheters were implanted into smaller children (n=19) by TS in Hamburg. RESULTS: Patient characteristics and operation technique did not differ between the departments. Peritonitis was the most common complication (33 catheters, 21.4%). Leakage (n=18 catheters, 11.7%) occurred more often in children weighing <10kg (p<0.001). The incidence of obstruction and dysfunction was significantly higher in catheters used in PS than catheters used in TS (30.4% vs. 11.8%, p=0.004). Omentectomy did not reduce the incidence of catheter obstruction (p=1.0). Perforation at the catheter tips was larger and appeared to be rougher in catheters used in PS than the catheters in TS. CONCLUSIONS: The type of catheter and presumably the type of perforation at the catheter tip may influence the incidence of peritoneal dialysis catheter obstruction.

TLR4 preconditioning is associated with low success of OK-432 treatment for lymphatic malformations in children.

PURPOSE: We have recently shown that the relative TLR4 expression on monocytes of low responding pediatric patients after OK-432 treatment is significantly reduced after stimulation with lipopolysaccharide (LPS) compared with high responding children. The aim of this study was to perform further analysis to explain this observation. METHODS: Monocytes from children with high (HR, n = 5) and low response (LR, n = 6) after previous OK-432 treatment were stimulated with LPS for 20 h and analyzed with fluorescence-activated cell sorting (mean fluorescence intensity, MFI; level of significance P ≤ 0.05). RESULTS: Mean MFI after LPS stimulation was comparable in both groups (HR 1142 ± 652 units, LR 839 ± 427 units, P = 0.85). Significant changes after LPS stimulation are explained by higher pre-stimulation values in the LR group compared with the HR group (950 ± 718 vs. 477 ± 341, P = 0.25) with considerable differences of the mean expression changes after LPS stimulation (HR 665 ± 683 vs. LR -111 ± 605, P = 0.08). CONCLUSION: The previously shown reduced TLR4 upregulation on monocytes after LPS stimulation in the LR group compared with the HR group can be primarily explained by TLR preconditioning. This observation implies the use of absolute values with definite thresholds.

Partial gastric pull-up in the treatment of patients with long-gap esophageal atresia.

BACKGROUND: This study was to analyze outcomes of long-gap esophageal atresia (LGEA) treated with partial gastric pull-up (PGP) into the thorax. METHODS: The medical records of all children who had undergone PGP for LGEA from 1999 to 2012 were reviewed. Preoperative data, initial postoperative course, complications, time to full oral nutrition, follow-up diagnostics and nutritional status were assessed. RESULTS: Nine children who had undergone PGP were followed up for a mean period of 6.2 ± 3.1 years. Their median gestational age was 37 ± 2 weeks, and mean birth weight 2462 ± 658 g. Eight children were primarily treated with a gastrostomy, their mean age at PGP was 11.4 ± 10.9 weeks and mean weight was 4484 ± 1966 g. Their mean operation time was 199 ± 51 minutes. Leakage was an early postoperative complication in three children, one of whom had a consecutive stricture resection. Late complications were stenosis (n=7) and gastro-esophageal reflux (n=5). The general status of the children was judged as "good" or "very good" on the last presentation. The median percentile of the body-mass-index was 25. Gastroscopy at 3.7 ± 3.2 years after the operation revealed a grade I esophagitis in two children. There was no death in this group of children. CONCLUSIONS: Because of its high complication rate, partial gastric pull-up cannot be recommended as an alternative for the treatment of LGEA at present. A final judgment could be made on the basis of a comparative study.

Dynamic Toll-like receptor expression predicts outcome of sclerotherapy for lymphatic malformations with OK-432 in children.

BACKGROUND: Sclerotherapy with OK-432 is recommended as a first-line treatment for lymphatic malformations. However, 40% of patients show poor response, defined by involution to <50% of the original size. It has been suggested that the OK-432 effect is highly dependent on the Toll-like receptor (TLR) 4-dependent expression of TLR7 in antigen-presenting cells. We hypothesized that the ability for TLR expression in monocytes after treatment with the TLR4-ligand lipopolysaccharide (LPS) can be used to predict successful OK-432 treatment. METHODS: Blood was taken from children with low responder (LR, n = 6) and high responder (HR, n = 5) of previous OK-432 treatment. Monocytes were stimulated with LPS for 20 h. TLR expression was analyzed with fluorescence-activated cell sorting (mean fluorescence intensity). The level of significance was P ≤ 0.05. RESULTS: The mean age of patients in the HR group was 1.4 ± 0.9 y and in the LR group 2.8 ± 2.9 y (P = 0.31). The mean TLR4 upregulation after LPS stimulation in the HR group was significantly higher than in the LR group (factor 3.6 versus factor 1 compared with nonstimulated controls; P = 0.037). The mean TLR7 expression did not show significant differences between the groups. CONCLUSIONS: Dynamic TLR4 expression represents most probably a predictive parameter for the treatment of lymphatic malformations with OK-432 and should be further investigated.

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.

Polysialic acid on neuropilin-2 is exclusively synthesized by the polysialyltransferase ST8SiaIV and attached to mucin-type o-glycans located between the b2 and c domain.

Neuropilin-2 (NRP2) is well known as a co-receptor for class 3 semaphorins and vascular endothelial growth factors, involved in axon guidance and angiogenesis. Moreover, NRP2 was shown to promote chemotactic migration of human monocyte-derived dendritic cells (DCs) toward the chemokine CCL21, a function that relies on the presence of polysialic acid (polySia). In vertebrates, this posttranslational modification is predominantly found on the neural cell adhesion molecule (NCAM), where it is synthesized on N-glycans by either of the two polysialyltransferases, ST8SiaII or ST8SiaIV. In contrast to NCAM, little is known on the biosynthesis of polySia on NRP2. Here we identified the polySia attachment sites and demonstrate that NRP2 is recognized only by ST8SiaIV. Although polySia-NRP2 was found on bone marrow-derived DCs from wild-type and St8sia2(-/-) mice, polySia was completely lost in DCs from St8sia4(-/-) mice despite normal NRP2 expression. In COS-7 cells, co-expression of NRP2 with ST8SiaIV but not ST8SiaII resulted in the formation of polySia-NRP2, highlighting distinct acceptor specificities of the two polysialyltransferases. Notably, ST8SiaIV synthesized polySia selectively on a NRP2 glycoform that was characterized by the presence of sialylated core 1 and core 2 O-glycans. Based on a comprehensive site-directed mutagenesis study, we localized the polySia attachment sites to an O-glycan cluster located in the linker region between b2 and c domain. Combined alanine exchange of Thr-607, -613, -614, -615, -619, and -624 efficiently blocked polysialylation. Restoration of single sites only partially rescued polysialylation, suggesting that within this cluster, polySia is attached to more than one site.

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.

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

BACKGROUND: The 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. METHODS: Twenty-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. RESULTS: VATS 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: 0.5-0.8) 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). CONCLUSIONS: The 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.

Perioperative analgesia strategies in fast-track pediatric surgery of the kidney and renal pelvis: lessons learned.

PURPOSE: Effective analgesia is essential for the success of fast-track (FT) pediatric surgery. Aim of the study was to achieve an optimal analgesia protocol for a comfortable postoperative course and early mobilization in children undergoing urological procedures. METHODS: A prospective study of two analgesia concepts in a series of children undergoing laparoscopic pyeloplasty (LP) and nephrectomy (LN) was performed. Thirty-six consecutive patients received analgesia according to the protocol "Fast Track I" (FT I), 23 patients according to the modified analgesia protocol "Fast Track II" (FT II). Differences between the protocols were frequency of pain measurement, more frequent use of local anesthesia and higher dosage of Acetaminophen. End points were pain intensity, use of opioids and side effects. Data derived from the German reimbursement system (G-DRG) regarding lengths of hospital stay were compared. RESULTS: On the day of operation (6:00 p.m.), pain scores of patients using FT II were significantly lower than those using the FT I protocol (1.51 + or - 0.4 FT II versus 3.8 + or - 0.4 FT I, p = 0.001). Pain scores still tended to be lower in the FT II group on the first postoperative day (8:00 a.m.), but from that point on, the difference was not significant (1.52 + or - 0.5 FT II versus 2.3 + or - 0.4 FT I, p = 0.186). Hospital stay was short, compared to the German average, in both groups. CONCLUSION: The modified analgesia protocol FT II leads to earlier and more effective pain reduction, lower use of opioids and fewer side effects than the previously used protocol FT I. Hence, modification of analgesia in pediatric urology is essential.

Fast-track concepts in routine pediatric surgery: a prospective study in 436 infants and children.

BACKGROUND AND AIMS: The aim of this study was to investigate fast-track concepts in routine pediatric surgery in a university clinic over 1 year. PATIENTS/METHODS: Fast-track concepts were established for procedures requiring hospital admission in patients up to 15 years of age. Patients were studied prospectively from June 2006 to June 2007. RESULTS: Out of a total of 436 potentially suitable patients, 155 (36%) were finally treated following the protocols. The mean intensity of pain in children younger than 4 years (CHIPPS, 0-10) was 1.3 +/- 1.5 the evening of the operation day and decreased to <1 at all other time points. The initial postoperative mean pain intensity in older children (Smiley/VAS, 1-10) was 3.7 +/- 2.2 and decreased constantly thereafter. The mean hospital stay of fast-track patients was significantly shorter compared with German diagnosis-related group data (4.6 +/- 2.9 versus 9.7 +/- 3.8, p < 0.01). There were four (3%) readmissions for minor complications. At follow-up after 2 weeks, 95% of patients and parents judged fast-track care as excellent. CONCLUSION: Fast-track concepts are feasible in one third of pediatric patients undergoing routine in-hospital surgery. Fast-track pediatric surgery achieves accelerated convalescence, minimal hospital stay, and high patient and parent satisfaction.

Carbon dioxide does not affect the methylation status of prognostic important oncogenes Rassf1A and DCR2 in neuroblastoma cells.

PURPOSE: The aim of the current study was to investigate effects of CO(2) atmosphere, mimicking conditions of the pneumoperitoneum during laparoscopy, on epigenetic conditions of Rassf1A and DCR2 oncogenes in neuroblastoma cells. METHODS: SH-SY5Y neuroblastoma cells were exposed to 100% CO(2) for 4 h. Cells were lysed 4, 8 and 168 h after exposure. After methylation analysis of Rassf1A and DCR2 with polymerase chain reaction, results were compared to those of physiologically incubated neuroblastoma cells. RESULTS: No significant changes were found after exposure to carbon dioxide compared to the control. Values of methylated Rassf1A were 12.6 +/- 1.1 versus 13.2 +/- 1.4 ng/microl in the controls, respectively (4 h after incubation), 12.6 +/- 1.2 versus 15.1 +/- 0.9 ng/microl (8 h) and 14.2 +/- 1.5 versus 11.7 +/- 1.3 ng/microl (168 h). DCR2 showed values of 4.6 +/- 0.5 versus 3.7 +/- 0.5 ng/microl (4 h), 3.8 +/- 0.5 versus 4.1 +/- 0.4 ng/microl (8 h) and 3.6 +/- 0.4 versus 3.8 +/- 0.5 ng/microl (168 h). CONCLUSION: Exposure of neuroblastoma cells to 100% CO(2) does not alter methylation of two prognostic relevant index genes. It seems therefore unlikely that effects on methylation levels within CO(2) pneumoperitoneum lead to epigenetic changes in neuroblastoma.

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.