Bado III Monteggia in children - treatment options and outcome: an ultrasound control study.

The Bado III Monteggia lesion is an uncommon fracture in children. Radial head dislocation can occur due to intra- or extra-articular fractures of the olecranon. In extra-articular ulnar fractures, heterogeneous operative methods are proposed in different case reports. The objective of this study is to determine if closed reduction of the ulnar fracture, either without osteosynthesis or by using TEN, is a safe treatment option. In a multicenter study, we retrospectively analyzed 26 children who were treated for an extra-articular proximal metaphyseal ulnar fracture with dislocation of the radial head. These patients underwent a follow-up examination after an average of four years. The investigation included a physical examination, visualization of the elbow anatomy, and specifically, the radial head retention in the joint by ultrasound. Of the 26 patients, 18 were treated by closed reduction and intramedullary osteosynthesis (TEN), 7 were treated by closed reduction and cast immobilization without osteosynthesis, and 1 child was treated by open reduction of the ulna and plate osteosynthesis. In the follow-up examination, 25 children showed an excellent outcome, as well as inapparent sonography. Although this is a retrospective study with different types of treatment, closed reduction of the ulna with or without osteosynthesis appears to be effective. We believe that the correct primary reduction is the key to an excellent long-term outcome. Regarding the invasiveness of the treatment, reduction without or with TEN osteosynthesis should be the preferred approach in extra-articular pediatric Bado III fractures.

Epidemiology and injury morphology of traumatic hip dislocations in children and adolescents in Germany: a multi-centre study.

OBJECTIVE: Traumatic hip dislocations are very rare in childhood and adolescence. The aim of this multi-centre study is to analyse the current epidemiology and injury morphology of a large number of traumatic hip dislocations in children. This can provide a better understanding of childhood hip dislocations and contribute to the development of a therapeutic approach in order to prevent long-term impacts. METHODOLOGY: This retrospective, anonymised multi-centre study included patients, aged up to 17 years, with acute traumatic hip dislocations and open growth plates. The patients came from 16 German hospitals. Exclusion criteria included insufficient data, a positive history of hip dysplasia, or an association with syndromal, neurological or connective tissue diseases predisposing to hip dislocation. An analysis was carried out on the patients' anthropometric data and scans (X-ray, MRI, CT), which were collected between 1979 and 2021. Gender, age at the time of dislocation, associated fractures, mechanism of injury, initial treatment including time between dislocation and reduction, method of reduction, treatment algorithm following reduction and all documented complications and concomitant injuries were evaluated. RESULTS: Seventy-six patients met the inclusion criteria. There were two age peaks at 4-8 years and 11-15 years. There was an increased incidence of girls in the under-eight age group, who had mild trauma, and in the group of over-eights there were more boys, who had moderate and severe trauma. Dorsal dislocation occurred in 89.9% of cases. Mono-injuries dominated across all age groups. Concomitant injuries rarely occurred before the age of eight; however, they increased with increasing ossification of the acetabulum and appeared as avulsion injuries in 32% of 11-15-year-olds. Of the 76 patients, 4 underwent a spontaneous, 67 a closed and 5 a primary open reduction. A reduction was performed within 6 h on 84% of the children; however, in around 10% of cases a reduction was not performed until after 24 h. Concomitant injuries needing intervention were identified in 34 children following reduction. Complications included nerve irritation in the form of sensitivity disorders (n = 6) as well as avascular necrosis (AVN) of the femoral head in 15.8% of the patients (n = 12). CONCLUSIONS: Traumatic hip dislocations are rare in childhood and adolescence and have high complication rates. The most severe complication, femoral head necrosis, occurred in 16% of cases. Minor injuries, especially in younger children, are enough to cause a dislocation. Posterior dislocation was more frequent and primarily occurred as a mono-injury; however, concomitant injuries must be considered with increasing age. Children continue to experience delayed reductions. The length of time until reduction, age and the severity of the concomitant injury play a role in the development of femoral head necrosis; however, this topic requires additional investigation.

Greenstick fractures of the proximal metaphyseal tibia: a retrospective multicenter study on the outcome after non-surgical or surgical treatment and the occurrence of posttraumatic tibia valga.

PURPOSE: This study investigates the occurrence of (progressive) posttraumatic valgus deformity after proximal metaphyseal greenstick fractures of the tibia in young children, and whether non-surgical or surgical treatment influences the outcome. METHODS: A retrospective multi-center study was conducted including surveys and X-rays of patients < 12 years of age with a fracture of the proximal tibia. In patients with greenstick fractures, the medial proximal tibia angle (MPTA; defined as the angle of the tibial axis and the joint-line of the knee) was measured at trauma, short-term follow-up (st-FU), and long-term FU (lt-FU) as defined for the 2 groups of non-surgically and surgically treated patients. RESULTS: Of a total of 322 fractures, 91 were greenstick fractures. Of these, 74 were treated non-surgically and 17 were treated surgically. The mean MPTA at trauma of non-surgically treated patients was 91.14°, and of surgically treated patients was 95.59° (p = 0.020). The MPTA in non-surgically treated patients significantly increased from the timepoint of trauma to st-FU (92,0°; p = 0.030), and lt-FU (92,66°, p = 0.016). In surgically treated patients, the MTPA improved after trauma to st-FU (94.00°; p = 0.290), and increased again to lt-FU (96.41°; p = 0.618). CONCLUSION: Progressive valgus deformity after greenstick fractures of the proximal tibia occurred in both non-surgically and surgically treated patients. In non-surgically treated patients, this was of statistical, but not clinical significance. In surgically treated patients, progressive valgus was observed after metal removal for an unknown reason. Therefore, surgery for proximal greenstick fractures of the tibia in this age group has only limited effect and may be indicated only in selected cases. Further studies are needed to explain the responsible mechanisms. LEVEL OF EVIDENCE: III, retrospective analysis.

[Clavicle shaft fractures in childhood and adolescence : Consensus report of the Pediatric Traumatology Section of the German Society for Trauma Surgery]. / Claviculaschaftfrakturen im Kindes- und Jugendalter : Konsensusreport der Sektion Kindertraumatologie in der Deutschen Gesellschaft für Unfallchirurgie.

INTRODUCTION: Clavicle shaft fractures are among the most common fractures in childhood and adolescence. In the past they were almost exclusively treated conservatively but in recent years there has been an increase in surgical treatment. Nevertheless, exact recommendations for the choice of diagnostics and for the treatment regimen do not yet exist. MATERIAL AND METHODS: Therefore, our aim was to develop a consensus within the 7th scientific working meeting of the section for pediatric traumatology in the German Society for Trauma Surgery based on expert opinion. RESULTS: Single-plane radiographic imaging is considered the gold standard diagnostic tool. Children younger than 10 years are primarily treated conservatively, and the type of immobilization is secondary. In girls older than 12 years and boys older than 14 years, fractures dislocated by more than the shaft width and shortened by > 2 cm should be treated by open reduction and stabilized by osteosynthesis, followed by free-functional follow-up treatment. CONCLUSION: In addition to X­rays, diagnostics using ultrasound must be further established. Treatment continues to be primarily conservative, but surgical treatment is also important, especially in adolescents. If the indications are correct, a good outcome can be expected regardless of the choice of treatment.

[Intraoperative imaging of children and adolescents, for selected fractures and in follow-up after conservative and operative treatment : Part 2 of the results of a nationwide online survey of the Pediatric Traumatology Section of the German Trauma Society]. / Bildgebung bei Kindern und Jugendlichen intraoperativ, bei ausgewählten Frakturen und im Verlauf nach konservativer und operativer Behandlung : Teil 2 der Ergebnisse einer bundesweiten Online-Umfrage der Sektion Kindertraumatologie der Deutschen Gesellschaft für Unfallchirurgie.

The indication for radiographic examinations in pediatric and adolescent trauma patients should follow ALARA (as low as reasonably achievable). Because of the effect of radiation on the growing sensitive tissues of these young patients, a strict indication should always be given for radiation use and during controls after fracture repair. METHODS: An online survey by the Pediatric Traumatology Section (SKT) of the German Trauma Society (DGU) from Nov. 15, 2019, to Feb. 29, 2020, targeting trauma, pediatric, and general surgeons and orthopedic surgeons. RESULTS: Participants: 788. Intraoperative applications: Collimation 50% always, postprocessing for magnification 40%, pulsed x-ray 47%, and 89% no continuous fluoroscopy; 63% osteosynthesis never directly on image intensifier. Radiographic controls after implant removal never used by 24%. After operated supracondylar humerus fracture, controls are performed up to 6 times. After distal radius greenstick fracture, 40% refrain from further X-ray controls, after conservatively treated clavicular shaft fracture, 55% refrain from further controls, others X-ray several times. After nondisplaced conservatively treated tibial shaft fracture, 63% recommend radiographic control after 1 week in two planes, 24% after 2 weeks, 37% after 4 weeks, and 32% after 6 weeks. DISCUSSION: The analysis shows that there is no uniform radiological management of children and adolescents with fractures among the respondents. For some indications for the use of radiography, the benefit does not seem evident. The ALARA principle does not seem to be consistently followed. CONCLUSION: Comparing the documented results of the survey with the consensus results of the SKT, differences are apparent.

[Imaging after trauma in clinics and practice for children and adolescents : Part 1 of the results of a nationwide online survey of the Pediatric Traumatology Section of the German Trauma Society]. / Bildgebung nach Unfall in Klinik und Praxis bei Kindern und Jugendlichen : Teil 1 der Ergebnisse einer bundesweiten Online-Umfrage der Sektion Kindertraumatologie der Deutschen Gesellschaft für Unfallchirurgie.

BACKGROUND: The indication for radiography should strictly follow the ALARA (as low as reasonably achievable) principle in pediatric and adolescent trauma patients. The effect of radiation on the growing sensitive tissue of these patients should not be disregarded. QUESTION: The Pediatric Traumatology Section (SKT) of the German Trauma Society (DGU) wanted to clarify how the principle is followed in trauma care. METHODS: An online survey was open for 10 weeks. Target groups were trauma surgeons, pediatric surgeons, general surgeons, and orthopedic surgeons. RESULTS: From Nov. 15, 2019, to Feb. 29, 2020, 788 physicians participated: branch office 20.56%, MVZ 4.31%, hospital 75.13%; resident 16.62%, senior 38.07%, chief 22.59%. By specialist qualification, the distribution was: 38.34% surgery, 33.16% trauma surgery, 36.66% special trauma surgery, 70.34% orthopedics and trauma surgery, 18.78% pediatric surgery. Frequency of contact with fractures in the above age group was reported as 37% < 10/month, 27% < 20/M, 36% > 20/M. About 52% always request radiographs in 2 planes after acute trauma. X-ray of the opposite side for unclear findings was rejected by 70%. 23% use sonography regularly in fracture diagnosis. In polytrauma children and adolescents, whole-body CT is never used in 18%, rarely in 50%, and standard in 14%. DISCUSSION: The analysis shows that there is no uniform radiological management of children and adolescents with fractures among the respondents. CONCLUSION: Comparing the results of the survey with the consensus findings of the SKT recently published in this journal, persuasion is still needed to change the use of radiography in primary diagnosis.

[Shaft fractures of the lower limb in adolescents]. / Frakturen im Schaftbereich der unteren Extremität bei Adoleszenten.

Shaft fractures of the lower extremities are rare during adolescence. Conservative treatment is no longer used for the upper leg and the significance is declining for the lower leg, as only minor axis deviations and malrotations of the shaft are acceptable and conservative therapy is associated with severe impairments in the daily routine; however, conservative treatment is still an option for femoral fractures in children < 3 years of age and for stable fractures of the lower leg. Various osteosynthesis procedures can be used depending on the body weight and epiphyseal maturation. Elastic stable intramedullary nailing is the first choice, followed by solid adolescent femoral or tibial nailing for higher body weights and external fixation for open or multifragmentary fractures. Plate osteosynthesis is an established alternative. Following correct surgical treatment, all fractures have movement and at least partial load-bearing stability and the results are satisfactory. Regular controls are recommended up to the end of the growth period.

Centralization of Biliary Atresia: Has Germany Learned Its Lessons?

INTRODUCTION: The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade. MATERIALS AND METHODS: In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany. RESULT: Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001-2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001-2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001-2005 [p = 0.5]). Compared with the 2001-2005 analysis, all criteria showed improvement but the differences are statistically not significant. CONCLUSION: Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction.

[X-ray diagnostics of fractures in childhood and adolescence-Consensus report of the scientific working group of the pediatric traumatology section of the German Society for Trauma Surgery (DGU)]. / Röntgendiagnostik bei Frakturen im Kindes- und Jugendalter ­ Konsensusbericht des Wissenschaftlichen Arbeitskreises der Sektion Kindertraumatologie der DGU.

For decades projection radiography has been the standard in the diagnostics of fractures and injuries, including patients in childhood and adolescent; however, each examination with X­rays should also be based on an individual assessment of the benefits and risks. The pediatric traumatology section of the DGU has developed a consensus paper on various aspects of imaging for diagnostics and assessment of progression, on setting options for intraoperative imaging, on X­ray imaging of the opposite side, on polytrauma and computed tomography (CT), on postoperative radiological checks and the use of sonography after an accident.

Lack of Mucosal Cholinergic Innervation Is Associated With Increased Risk of Enterocolitis in Hirschsprung's Disease.

BACKGROUND & AIMS: Hirschsprung's disease (HSCR) is a congenital intestinal motility disorder defined by the absence of enteric neuronal cells (ganglia) in the distal gut. The development of HSCR-associated enterocolitis remains a life-threatening complication. Absence of enteric ganglia implicates innervation of acetylcholine-secreting (cholinergic) nerve fibers. Cholinergic signals have been reported to control excessive inflammation, but the impact on HSCR-associated enterocolitis is unknown. METHODS: We enrolled 44 HSCR patients in a prospective multicenter study and grouped them according to their degree of colonic mucosal acetylcholinesterase-positive innervation into low-fiber and high-fiber patient groups. The fiber phenotype was correlated with the tissue cytokine profile as well as immune cell frequencies using Luminex analysis and fluorescence-activated cell sorting analysis of colonic tissue and immune cells. Using confocal immunofluorescence microscopy, macrophages were identified in close proximity to nerve fibers and characterized by RNA-seq analysis. Microbial dysbiosis was analyzed in colonic tissue using 16S-rDNA gene sequencing. Finally, the fiber phenotype was correlated with postoperative enterocolitis manifestation. RESULTS: The presence of mucosal nerve fiber innervation correlated with reduced T-helper 17 cytokines and cell frequencies. In high-fiber tissue, macrophages co-localized with nerve fibers and expressed significantly less interleukin 23 than macrophages from low-fiber tissue. HSCR patients lacking mucosal nerve fibers showed microbial dysbiosis and had a higher incidence of postoperative enterocolitis. CONCLUSIONS: The mucosal fiber phenotype might serve as a prognostic marker for enterocolitis development in HSCR patients and may offer an approach to personalized patient care and new therapeutic options.

Failure analysis and recommendations for treatment of posttraumatic non-unions of the distal humerus during childhood.

PURPOSE: Non-unions of the distal humerus are rare complications of common children's fractures such as radial condyle fractures and supracondylar fractures. The aim of this paper was to update the knowledge about etiology, reasons, management, and results of these troublesome, and sometimes debilitating entities. METHODS: The sparse literature concerning nonunions following condylar or supracondylar fractures was analyzed together with the presentation of some typical clinical cases. RESULTS: In most of the cases, non-unions were induced by neglect, unstable fixation, too early implant removal, too much revision surgery, and an inconsequent transfer of follow-up algorithms, or combinations of the above. Treatment of non-union should start as early as possible because the effort of required surgery increases with time that the nonunion has been neglected. Often a combination of stable fixation of the pseudarthrosis and correction of the elbow axis are necessary to achieve a satisfying outcome. CONCLUSION: In pediatric traumatology, qualified and consequent care for children's fractures of the distal humerus can prevent rare complications such as non-unions in almost any situation. If such a disturbance of healing is noticed, immediate and adequate, i.e. children specific surgical consequences achieve best results.

Treatment of nonunion after forearm fractures in children: a conservative approach.

PURPOSE: Nonunions are a very rare complication after surgical treatment of displaced forearm fractures in children, but can occur in both the radius or ulna. The aim of this study is to evaluate predisposing factors for non-unions and to discuss treatment options. METHODS: We conducted a retrospective analysis of all patients who were treated primarily or secondary at our institution for nonunion after forearm fractures and operative treatment. Therefore, we retrospectively reviewed the medical charts and radiographs of affected patients from 1990 to 2020. We analysed demographic and fracture-related data as well as treatment options. RESULTS: Twenty-seven cases were identified. Median age was 12 years (7-16 years). The ulna was affected in 20 cases and the radius in 7. The most common location for ulna nonunion was the middle third of the shaft, and for the radius the mid and distal shaft. In 21 cases an open reduction was required. In 9 cases technical problems were identified. In most cases (26/27) a hypertrophic nonunion occurred. 10 of 27 (37%) nonunions healed without any surgical management after 9 (7-15) months. The median age of the conservatively managed group was 10 (6-13) years, younger than in the group with managed with reoperation [median 13 (7-16) years]. Indications for surgical intervention were increasing deformity, pain and limitation of movement. CONCLUSION: Open reduction and technical problems seem to be influencing factors. A conservative approach can be adopted in asymptomatic patients, otherwise an operative management, constituting plate osteosynthesis with or without bone grafting, should be performed.

[Diaphyseal and metaphyseal femoral fractures in childhood]. / Dia- und metaphysäre Femurfrakturen im Kindesalter.

Femoral fractures in childhood are rare and are usually the result of severe trauma. Conservative treatment (overhead extension, hip spica cast) can be used in children up to 3 years of age and beyond that elastic stable intramedullary nailing (ESIN) is the method of choice. The prognosis is very good if the surgical technique is adequate. In adolescents > 50 kg in body weight solid nails implanted via the greater trochanter are an alternative (adolescent lateral femoral nail, ALFN). External fixators and plate osteosynthesis are indicated in special situations. Femoral neck fractures are very seldom seen in this age group. Besides Kirschner (K)-wires and screw osteosynthesis a special pediatric hip plate is available in this situation. In the distal metaphysis mostly stable fractures occur in disabled children, which can usually be treated conservatively. In addition, pathological fractures occur in juvenile bone cysts and complex injuries in adolescents, which need stabilization by descending ESIN or with plates. At the distal growth plate relevant growth disturbances are possible.

Cholinergic Signaling Attenuates Pro-Inflammatory Interleukin-8 Response in Colonic Epithelial Cells.

Infants affected by Hirschsprung disease (HSCR), a neurodevelopmental congenital disorder, lack ganglia of the intrinsic enteric nervous system (aganglionosis) in a variable length of the colon, and are prone to developing severe Hirschsprung-associated enterocolitis (HAEC). HSCR patients typically show abnormal dense innervation of extrinsic cholinergic nerve fibers throughout the aganglionic rectosigmoid. Cholinergic signaling has been reported to reduce inflammatory response. Consequently, a sparse extrinsic cholinergic innervation in the mucosa of the rectosigmoid correlates with increased inflammatory immune cell frequencies and higher incidence of HAEC in HSCR patients. However, whether cholinergic signals influence the pro-inflammatory immune response of intestinal epithelial cells (IEC) is unknown. Here, we analyzed colonic IEC isolated from 43 HSCR patients with either a low or high mucosal cholinergic innervation density (fiber-low versus fiber-high) as well as from control tissue. Compared to fiber-high samples, IEC purified from fiber-low rectosigmoid expressed significantly higher levels of IL-8 but not TNF-α, IL-10, TGF-ß1, Muc-2 or tight junction proteins. IEC from fiber-low rectosigmoid showed higher IL-8 protein concentrations in cell lysates as well as prominent IL-8 immunoreactivity compared to IEC from fiber-high tissue. Using the human colonic IEC cell line SW480 we demonstrated that cholinergic signals suppress lipopolysaccharide-induced IL-8 secretion via the alpha 7 nicotinic acetylcholine receptor (a7nAChR). In conclusion, we showed for the first time that the presence of a dense mucosal cholinergic innervation is associated with decreased secretion of IEC-derived pro-inflammatory IL-8 in the rectosigmoid of HSCR patients likely dependent on a7nAChR activation. Owing to the association between IL-8 and enterocolitis-prone, fiber-low HSCR patients, targeted therapies against IL-8 might be a promising immunotherapy candidate for HAEC treatment.

Pediatric aseptic lower leg fracture nonunion.

PURPOSE: Lower leg nonunion in pediatric patients is a rarity. Therefore, eight European pediatric trauma units retrospectively analyzed all patients younger than 18 years suffering lower leg fractures resulting in aseptic nonunion. METHODS: Thirteen children and adolescents less than 18 years old (2 girls and 11 boys) diagnosed with aseptic nonunion of the tibia and/or fibula were evaluated. In all patients, epidemiological data, mechanism of injury, fracture configuration, and the initial treatment concept were assessed, and the entire medical case documentation was observed. Furthermore, potential causes of nonunion development were evaluated. RESULTS: The mean age of patients was 12.3 years with the youngest patient being seven and the oldest being 17 years old. Open fractures were found in six out of thirteen patients (46%). Nonunion was hypertrophic in ten and oligotrophic in three patients. Mean range of time to nonunion occurrence was 7.3 ± 4.6 months. Nonunion healing resulting in complete metal removal was found in 12 out of 13 patients (92%), only in one case of a misinterpreted CPT type II osseous consolidation could not be found during the observation period. Mean range of time between surgical nonunion revision and osseous healing was 7.3 months as well. CONCLUSION: If treatment principles of the growing skeleton are followed consistently, aseptic nonunion of the lower leg remains a rare complication in children and adolescents. Factors influencing the risk of fracture nonunion development include patient's age, extended soft tissue damage, relevant bone loss, and inadequate initial treatment.

Multicenter Analysis of Acquired Undescended Testis and Its Impact on the Timing of Orchidopexy.

OBJECTIVE: To assess whether late orchidopexy for undescended testis represents delayed treatment of primary undescended testis or later-occurring acquired undescended testis. STUDY DESIGN: We examined boys undergoing orchidopexy for cryptorchidism regarding age at surgery and entity of undescended testis. We characterized differences between primary undescended testis and acquired undescended testis and evaluated the knowledge regarding the diagnosis and management of acquired undescended testis among practicing physicians. We conducted an observational study using a mixed-method multicenter cross-sectional design. A total of 310 consecutive boys undergoing orchidopexy for undescended testis at 6 pediatric medical centers in Germany between April 2016 and June 2018 were investigated regarding testicular position at birth and age at surgery. In addition, a survey on acquired undescended testis management was carried out in 1017 multidisciplinary physicians and final-year medical students. RESULTS: Only 13% of all patients were operated on in their first year of life. Among patients with known previous testicular position (67%), primary undescended testis (n = 103) and acquired undescended testis (n = 104) were equally frequent. More than one-half (56%) of orchidopexies performed after the first year of life were due to acquired undescended testis. Remarkably, only 15% of physicians considered acquired undescended testis as an indication for late surgery. CONCLUSIONS: Acquired undescended testis is more common than previously perceived and accounts for a significant proportion of "late" orchidopexies in patients with undescended testis. Acquired undescended testis needs to be better recognized in clinical practice and screening should continue in older children with previously descended testes. TRIAL REGISTRATION: German Clinical Trials Registry: DRKS00015903.

[Current Treatment of Esophageal Atresia with Tracheoesophageal Fistula - Updated Guidelines of the German Society of Pediatric Surgery]. / Behandlung der Ösophagusatresie mit unterer tracheoösophagealer Fistel ­ Zusammenfassung der aktuellen S2K-Leitlinie der DGKCH.

Esophageal atresia (EA) is a congenital anomaly that entails an interrupted esophagus with or without tracheoesophageal fistula (TEF). Depending on the distance of the two esophageal pouches a "short-gap" is distinguished from a "long-gap" variant. Up to 50% of newborns have additional anomalies. EA is prenatally diagnosed in 32-63% of cases. Recently, the interdisciplinary care in these children underwent substantial changes. Therefore, we summarize the current guideline of the German society of pediatric surgery for the treatment of patients with EA and distal TEF (Gross Type C). Controversies regarding the perioperative management include surgical-technical aspects, such as the thoracoscopic approach to EA, as well as general anesthesia (preoperative tracheobronchoscopy, intraoperative hypercapnia and acidosis). Moreover, postoperative complications and their management like anastomotic stricture are outlined. Despite significant improvements in the treatment of EA, there is still a relevant amount of long-term morbidity after surgical correction. This includes dysmotility of the esophagus, gastroesophageal reflux disease, recurrent respiratory infections, tracheomalacia, failure to thrive, and orthopedic complications following thoracotomy in the neonatal age. Therefore, close follow-up is mandatory to attain optimal quality of life.