Treatment of unstable forearm fractures at the metaphyseal-diaphyseal junction in children: antegrade ESIN vs. transepiphyseal intramedullary K-wire fixation.

BACKGROUND: Treatment of unstable forearm fractures in the metaphyseal-diaphyseal junction (MDJ) zone is still a matter of debate. Major drawbacks of all types of fixations include either invasiveness, technical impracticality, or lack of acceptance by patients. This study reports results after antegrade ESIN (a-ESIN) compared to transepiphyseal intramedullary K-wire (TIK) for unstable MDJ forearm fractures. METHODS: The MDJ of the forearm was defined as the square over the joints of both forearm bones subtracted with the square over the metaphysis of the radius alone. The data of 40 consecutive patients < 16 years of age who were treated either by a-ESIN (later treatment period) or TIK (early treatment period) for an unstable MDJ forearm fracture at a single high-volume pediatric trauma center were retrospectively analyzed. RESULTS: The average age was slightly lower in the first group (TIK = 7.42 years; a-ESIN = 10.5 years). An additional ulna fracture was found in 50% of cases and was treated with a classic antegrade ESIN in 10/20 (TIK) and 6/20 cases (a-ESIN). Additional plaster cast immobilization was performed in all cases with TIK and in three cases with a-ESIN. After TIK, no complication, malalignment, or functional limitation occurred. After a-ESIN, 19/20 patients had an event-free course with stable retention and healing without axial malalignment. In one case, a temporary sensor dysfunction occurred. The same patient suffered a refracture two months after the original trauma, which required a closed reduction. Metal removal was performed after 84 days (TIK) and 150 days (a-ESIN). The outcome in all patients was good. CONCLUSION: Both a-ESIN and TIK are minimally invasive procedures that are technically easy to perform. Both methods are safe and lead to a complete restoration of the forearm's range of motion. The decisive advantage of a-ESIN is the possibility of postoperative immobilization-free rehabilitation.

Incidence, treatment techniques, and results of distal humeral coronal shear fractures in children and adolescents-a multicenter study of the German Section of Pediatric Traumatology (SKT).

PURPOSE: Distal humeral coronal shear fractures (CSF) are uncommon and may be challenging to treat due to their size, location, and intraarticular nature. The purpose of this study was to analyze treatment concepts of this rare entity in the growing age. METHODS: Based on a multi-center data analysis we retrospectively reviewed patients below 16 years of age with CSF treated at 13 high-volume pediatric trauma centers. RESULTS: Data from 51 patients with a CSF treated between 01/2012 and 12/2021 were analyzed. The mean age was 12.9 years (10-15), and there was a trend towards male patients (30: 21). The initial diagnostic approach was conventional X-ray in all cases. In addition, a CT scan (n = 33), MRI scan (n = 9), or both (n = 3) were performed. All fractures except two showed relevant displacement. Consequently, only two cases received conservative treatment consisting of plaster immobilization. Surgical treatment was performed in 49 cases consisting of open or mini-open reduction and metal/resorbable screw osteosynthesis (n = 39), plates (n = 4), K-wire pinning (n = 6), and others (n = 6), as well as combinations. In 1 case open reduction without osteosynthesis was performed. Postoperative additional plaster immobilization was performed in 40 cases (for a mean of 19 days (2-42)), physiotherapy was initiated in 29 cases, and metal removal was performed in 28 cases (after a mean of 18.1 weeks (4-44)). After a mean follow-up of 9.9 months (2-25), elbow axial deviation (5° valgus) was observed in one case and mild loss of elbow ROM in six cases (11.7%). Complications included revision of the osteosynthesis because of insufficient articular reconstruction (n = 4), removal of a free joint body (n = 1), an osteonecrosis (n = 1), and a cartilage defect (n = 1). CONCLUSION: In pediatric patients CSFs start to occur at the age of 10, but are typically observed at the age of 13 and older. Because of their intraarticular nature and predilection toward displacement, these fractures are frequently treated operatively. The surgical strategy requires open reduction and anatomic reconstruction of the articular surface. Stable internal fixation, most often achieved by screws, permits early mobilization and leads to good outcomes in most cases. This is presumably due to the fact that mostly simple fractures occur in children and mostly complex injuries in older adults. LEVEL OF EVIDENCE: III, retrospective analysis.

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.

Thoracoscopic Resection of Lung Nodules following CT-Guided Labeling in Children and Adolescents with Solid Tumors.

Resection of lung metastases in children with solid tumors is regularly hampered by limited intraoperative detectability and relevant operative trauma of the open surgical access. The aim of this study was to analyze thoracoscopic resection of lung metastases in children following CT-guided labeling with coil wires. We retrospectively analyzed data of children and adolescents undergoing this approach at our institution between 2010 and 2022 with regard to technical aspects as well as surgical and oncological data. Within this period, we performed this procedure on 12 patients wherein we resected 18 lesions (1-5 per patient). The median age of patients was 178 months (51-265). The median duration of coil wire placement was 41 min (30-173) and the median surgery time was 53 min (11-157). No conversions were necessary and no intraoperative complications occurred. Complete microscopic resection (R0) was achieved in all labeled lesions and malignant tumor components were found in 5/12 patients. Our study shows that with a careful patient selection, thoracoscopic resection of lung metastases after coil wire labeling is a safe and reproducible procedure in children. Using this approach, lesions that are expected to have a reduced intraoperative detectability during open surgery become resectable. Patients benefit from the minimally invasive surgical access and reduced operative trauma.

Clinical Follow-Up without Radiographs Is Sufficient after Most Nonoperatively Treated Distal Radius Fractures in Children.

Distal forearm fractures are common in children and are usually treated nonoperatively. No consensus has been reached on how to perform clinical and radiographic follow-up of these fractures. Our aim was to study whether radiographic and clinical follow-up is justified. We included 100 consecutive patients with non-operatively treated distal forearm fractures who were treated at Oulu University Hospital in 2010-2011. The natural history of the fractures during the nonoperative treatment was analyzed by measuring the potential worsening of the alignment during the follow-up period. The limits of acceptable fracture position were set according to the current literature using "strict" or "wide" criteria for alignment. We determined the rate of worsening fracture position (i.e., patients who reached the threshold of unacceptable alignment). In relation to splinting, we evaluated how many patients benefited from clinical follow-up. Most of the fractures (98%) preserved acceptable alignment during the entire follow-up period when wide criteria were used. The application of stricter criteria for alignment in radiographs showed loss of reduction in 19% of the fractures. Worsening of the alignment was recognized at a mean of 13 days (range 5-29) after the injury. One in three (32%) patients needed some intervention due to splint loosening or failure. Radiographic follow-up of nonoperatively treated distal forearm fractures remains questionable. Instead, clinical follow-up is important, as 32% of patients needed their splints fixed.

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.

Results after skin traction for femur shaft fractures in children below the age of four years.

PURPOSE: Nonsurgical management has been identified as the treatment of choice for femoral shaft fractures in children below four years of age. For various reasons, the surgical approach has become increasingly popular in recent years. The aim of this study is to report results after vertical skin traction and analyze the benefits of this technique as well as to point out advantages compared with surgery in this age group. METHODS: The authors performed a retrospective data analysis, including all patients with femoral shaft fractures below the age of four who were treated with vertical skin traction at our institution between January 2006 and December 2016. RESULTS: Skin traction for a femoral shaft fracture was performed for 36 patients (mean age 1.6 years; 1 day-3.5 years). The mean duration of traction was 18.5 days (14-30). Complications included soft tissue affections (n = 5), which all healed spontaneously. Consolidation was observed in all fractures. Initial axial deviations and shortening did not change during traction until consolidation (p > 0.05), and no relevant torsion deformity occurred (p = 0.01). Patients gained full weight-bearing within 12.3 days (7-40) following end of traction. At the final follow-up, after a mean of 29.3 months (12-192), leg-length discrepancy (mean 7.1 mm; 5-20) was found on radiograms in nine cases, and axial deviations (mean 7.7°; 5-25) were documented in seven cases. None of the patients had limitations in daily activities or sports. CONCLUSION: Skin traction is a technically easy, safe, and non-invasive treatment modality for femoral shaft fractures in children below the age of four years. Strong results are obtained benefited from a high potential of growth-related correction, and in principle no anesthesia is needed. A prolonged hospitalization and socio-economic factors maintain the ongoing debate in comparison with the surgical approach. LEVEL OF EVIDENCE: Level III, retrospective.

Gastric Transposition for Repair of Long-Gap Esophageal Atresia: Indications, Complications, and Outcome of Minimally Invasive and Open Surgery.

BACKGROUND: Gastric transposition (GT) is a possible option for esophageal replacement in long-gap esophageal atresia (LGEA). The present study aims to report and compare indications and outcome of laparoscopic-assisted GT (LAGT) versus open (OGT) GT for LGEA repair. METHODS: Retrospective single-center analysis of all LGEA patients undergoing GT between 2002 and 2021. RESULTS: Thirty-one children with LGEA underwent GT. Of these, 19 underwent LAGT (mean weight at surgery 5.6 kg; mean age 167 days) and 12 underwent OGT (6.1 kg; 233 days). Indications for OGT were previous surgery (n = 7), associated severe cardiac malformations (n = 4), and a simultaneous resection of a choledochal cyst (n = 1). The conversion rate was 1. The two procedures (LAGT/OGT) differed in anesthetic time (308/350 min), duration of ventilation (5.1/5.3 days), hospital stay (34/32 days), and complications (22/15). None of the differences reached statistical significance. Outcome was also comparable: completely oral nutrition uptake in 66%/73%, slow weight gain in the low centiles in both groups, no patient developed dumping syndrome, symptomatic reflux was seen in 1 patient after OGT. CONCLUSION: In our cohort, LAGT for repair of LGEA provided similar outcomes as open surgery. The minimally invasive approach preserves thoracal structures, prevents additional thoracotomy or laparotomy, and is faster. To realize LAGT, a postpartal treatment concept including gastrostomy placement via a microincision to minimize adhesions is essential. The open surgical approach should be considered in cases of previous extensive surgical attempts of EA correction causing severe adhesions as well as associated anomalies or genetic syndromes causing hemodynamic instability.

Fractures of the proximal radius in children: management and results of 100 consecutive cases.

INTRODUCTION: Pediatric radial neck and head fractures are rare, accounting for only 1% of all fractures in children. The aim of this study is to describe the management and results of the respective fracture types and different injury characteristics. MATERIALS AND METHODS: This study performs a retrospective data analysis of 100 consecutive patients with a fracture of the proximal radius treated in a single high-volume pediatric trauma center. RESULTS: One hundred patients [mean age 7.5 years (1-15)] were documented with a fracture of the proximal radius between 3/2011 and 12/2019. The gender distribution was 62 girls and 38 boys. Twenty-seven patients had concomitant injuries. Conservative treatment was performed in 63 patients (Judet I = 27; II = 30; III = 6; Mason I = 2) using an above-the-elbow cast for 21 days (6-35). Surgical treatment was performed in 37 patients (Judet II = 3; III = 22; IV = 5; V = 7) using elastic stable intramedullary nailing (ESIN). Open reduction was necessary in five cases, and additional immobilization was performed in 32 cases. Six complications occurred: loss of implant stability (n = 2), healing in malalignment, pseudarthrosis, radioulnar synostosis, and a persisting hypoesthesia at the thumb. As a result, two ESIN osteosynthesis were revised, and one radial head resection was performed. Loss of movement was seen in 11% of cases, overall Mayo elbow performance index (MEPI) was 99.8 (90-100), and none of the patients experienced negative impacts on activities of daily life. CONCLUSIONS: Proximal radial fractures occur predominately without dislocation. Good results are obtained with conservative treatment throughout. In cases with displacement exceeding growth-related correction, ESIN is the undisputed treatment of choice. Open surgery and long immobilization periods should be avoided whenever possible.

Implant removal associated complications after ESIN osteosynthesis in pediatric fractures.

PURPOSE: ESIN (elastic stable intramedullary nailing) is considered the gold standard for various pediatric fractures. The aim of this study was to analyze the incidence and type of complications during or after TEN (titanium elastic nail) removal. METHODS: A retrospective data analysis was performed. Metal removal associated complications and preoperative extraosseous length/outlet angle of TENs as possible causes of complications were assessed. RESULTS: The complication rate in 384 TEN removals was 3.1% (n = 12). One major complication (rupture of M. extensor pollicis brevis) was documented. One refracture at the forearm occurred, however, remodeling prior TEN removal was completed. Ten minor complications were temporary or without irreversible restrictions (3 infections, 5 scaring/granuloma, 2 temporary paraesthesia). In 38 cases (16 forearms, 10 femora, 9 humeri, 3 lower legs), intra-operative fluoroscopy had to be used to locate the implants. In patients with forearm fractures, extraosseous implant length was relatively shorter than in cases without fluoroscopy (p = 0.01), but outlet angle of TENs was not significantly different in these two groups (28.5° vs 25.6°). In patients with femur fractures, extraosseous implant length and outlet angle were tendentially shorter, respectively, lower, but this did not reach statistical significance. CONCLUSION: Removal of TENs after ESIN is a safe procedure with a low complication rate. Technically inaccurate TEN implantation makes removal more difficult and complicated. To prevent an untimely removal and patient discomfort, nail ends must be exactly positioned and cut. Intraoperative complications may be minimized with removal of TENs before signs of overgrowth. EVIDENCE: Level III, retrospective.

Changes in the management of pediatric medial humeral epicondyle fractures with and without associated elbow dislocation.

Purpose The optimal management of medial humeral epicondyle fractures continues to be debated since decades. This single center study analyzes changes and optimizations of treatment over an observation period of 16 years and reports the results. Materials and Methods Retrospective analysis of all patients treated with a medial humeral epicondyle fracture between 2005 and 2020 at our institution. Results Ninety-six patients (mean 9.3 years, range 1 - 15) were included in the study. In 25 cases (26 %), the fracture was associated with an elbow dislocation. Most patients received surgical treatment (83.3 %), whereas 17.7 % were treated nonoperatively. Surgical treatment consisted of open reduction and fixation with compression screw (n = 44 steel, n = 2 absorbable), K-wire (n = 30), a combination of screw/K-wire (n = 2), or a PDS suture (n = 1). Compression screws have been used significantly more often in the latter half of the study period (p = 0.006). Patients were immobilized in a long arm cast for 29 days (range 11 - 50). Eleven surgically treated patients were early mobilized in an elbow orthosis. After a mean follow up of 7.6 months [2 - 61), Mayo elbow performance index (MEPI) outcome was excellent in all 96 patients. Loss of elbow movement (LOM) was found to be mild in 30 and moderate in 15 patients. LOM was found to be associated with surgical treatment (p = 0.001), and with concomitant elbow dislocations (p = 0.29). One pseudarthrosis occurred after conservative treatment. A persistence of ulnar nerve palsy or recurrent joint instability has not been described. Conclusion Most children with medial humeral epicondyle fractures nowadays undergo surgery. Screw osteosynthesis represents the increasingly preferred method in order to prevent joint instability or non-union, and to allow shorter immobilization duration. Overall results after medial epicondyle fractures are good.

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.

Functional outcome after laparoscopic assisted gastric transposition including pyloric dilatation in long-gap esophageal atresia.

BACKGROUND/PURPOSE: Among the options for esophageal replacement in long-gap esophageal atresia (LGEA), gastric transposition (GT) is accessible for an endoscopic approach. Here we report a novel technique and functional results after laparoscopic-assisted gastric transposition (LAGT), including pyloric dilatation in patients with LGEA. METHODS: Retrospective analysis of 14 children undergoing LAGT. Surgical steps included the release of the gastrostomy, transumbilical ante-situ section of the stomach including pyloric balloon-dilation, and laparoscopically controlled transhiatal retromediastinal blunt dissection followed by LAGT for cervical anastomosis to the proximal esophagus. RESULTS: The median age at LAGT was 110 days (33-327 days), bodyweight 5.3 kg (3.1-8.3 kg). Operation time was 255 min (180-436 min); one conversion was necessary. The duration of ventilation was 4 days (1-14 days). Postpyloric feeding was started after 2 days, and oral feeding after 13 days. Complications were recurrent pleural effusion or pneumothorax and transient Horner syndrome or transient incomplete paresis of the recurrence nerve. After a median follow-up of 60 months (13-240 months), all children have a patent upper GI tract, show weight gain, and are fed without delayed gastric emptying, dumping, or reflux. Severe (n = 1) or mild (n = 2) anastomotic or pyloric (n = 5) stenosis was resolved with endoscopic dilatations. CONCLUSIONS: Functional outcome after LAGT in patients with LGEA is good. The laparoscopic retromediastinal dissection preserves thoracal structures and increases patients' safety. The technique of pyloric dilatation might also prevent dumping syndrome. TYPE OF STUDY: Case Series with no Comparison Group. LEVEL OF EVIDENCE: Level IV.

[The treatment of elbow dislocations in children and adolescents : Consensus report of the pediatric trauma section of the German Trauma Society (DGU)]. / Die Behandlung der Ellenbogenluxation im Kindes- und Jugendalter : Konsensusbericht der Sektion Kindertraumatologie der DGU.

Dislocations of the elbow during growth are rare but because of associated fractures a range of therapeutic methods are employed. The postoperative care is even more varied, so the scientific working group of the section for pediatric trauma of the DGU developed the following recommendations for the treatment of elbow dislocations in children and adolescents based on own experience and a review of the literature. Diagnosis is made from radiographs with at least one (preferably lateral) view showing the dislocation. Reduction is considered urgent and should be done as atraumatically as possible with the patient under general anesthesia. After reduction, mobility and stability of the elbow are assessed and associated fractures have to be ruled out by fluoroscopy while the patient is still under anesthesia. Any fractures requiring operative treatment should be addressed at this time. A short-term immobilization should be ordered according to the degree of injury and the stability of any osteosynthesis. The aim should always be to initiate mobilization as soon as possible with immobilization lasting not longer than 3 weeks in any case. Mobilization is initially limited to the activities of daily life at first. 6 weeks after reduction physiotherapy may be initiated as warranted and patients can participate in sports again. An MRI of the elbow may be necessary if there are persistent problems. Even if a measurable loss of range of motion is often recorded, prognosis regarding a good functional outcome is favorable.

Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome.

BACKGROUND: Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung's disease occurring in 3-10% of the cases. Only few studies reported the long-term clinical and metabolic outcomes of patients with TCA. The aim of this study was to evaluate the functional and metabolic long-term outcomes of children undergoing surgical treatment for TCA. METHODS: A 15-year retrospective study was performed. Blood chemistry tests and stool analysis performed at the last follow-up visit were recorded. Height and weight development were assessed using the corresponding percentiles for age. Faecal continence and quality of life were evaluated using a detailed questionnaire. RESULTS: Eleven patients were included in the study. The median age at surgery was 6 months (range: 3-72 months). After histological confirmation, all patients underwent a total colectomy. Ileoanal anastomosis (n = 6), ileorectal anastomosis (n = 1), J-pouch (n = 1) and Duhamel procedure (n = 3) were performed. Temporary ileostomy was closed after a median of 8 weeks in 10/11 patients. After a median follow-up of 78 months (range: 27-199 months), all evaluated patients were continent. Height and weight were appropriate for age in only 5 patients. Vitamin B12 and folic acid serum levels were normal in all examined patients. Ten patients had normal hemoglobin serum levels. Seven patients had low transferrin saturation in serum. Hemoccult tests were negative in all examined patients. Despite complex postoperative courses in some cases, patients and parents showed good overall satisfaction in terms of quality of life. CONCLUSION: The majority of patients reported a good quality of life. This can result from the adaptation of the patients to certain disease states. The failure to thrive seems to be related with the extent of aganglionosis. The inclusion of these patients in interdisciplinary long-term follow-up care, in which pediatric surgeons, gastroenterologists, and dieticians are involved, is essential.

Outcome of primary repair in extremely and very low-birth-weight infants with esophageal atresia/distal tracheoesophageal fistula.

PURPOSE: The optimal surgical management of extremely (ELBW) and very low-birth-weight (VLBW) neonates with esophageal atresia and distal tracheoesophageal fistula (EA/TEF) (Gross type C) is still debated. The aim of this study was to evaluate the surgical outcome of primary repair in these patients and compare it to ≥1500g neonates. METHODS: Medical records of neonates with repaired EA from 2002 to 2016 were reviewed. RESULTS: 4 ELBW, 7 VLBW, and 24 ≥1500g infants had type C EA/TEF and underwent primary repair. Anastomotic leakage occurred in 0% ELBW, 0% VLBW and 8.3% ≥1500g patients and anastomotic stricture in 25% ELBW, 28.5% VLBW and 37.5% ≥1500g patients. 50% ELBW, 14.2% VLBW and 20.8% ≥1500g patients underwent secondary fundoplication. One patient of the VLBW group and one patient of the ≥1500g group died postoperatively of causes not related to EA/TEF. CONCLUSIONS: In extremely and very low-birth-weight neonates with type C EA/TEF surgical outcome after primary repair is comparable to the outcome in ≥1500g neonates. Primary repair can be performed in most of these patients and staged repair can be restricted to unstable patients. LEVEL OF EVIDENCE: Treatment study level III.

Experiences and challenges of thorcoscopic lung surgery in the pediatric age group.

BACKGROUND: We report a single-center study of minimally invasive thoracoscopic lung surgery with pediatric patients. METHODS: We performed a retrospective analysis of patients who underwent thoracoscopic lung surgery between 2004 and 2013. The procedures were divided into anatomic and non-anatomical lung resections. RESULTS: Seventy-six patients with a mean age of 6.5 years (range: 7 days-17 years) and a mean weight of 11 kg (range: 2.6-56.0 kg) received thoracoscopic lung surgery for tumor metastases (n = 20), sequestration/congenital adenomatoid malformation (19), cysts (12), aspergillomas (7), bullae (5), middle lobe syndrome (3), bronchiectasis (3), emphysema (2), and other reasons (5). Twenty-nine anatomical lung resections (Group I: lobectomies, segmentectomies) and 47 non-anatomical lung resections (Group II: wedge resections, lung tissue-sparing surgery) were performed. In 6 cases, preoperative CT-guided coiling was used to localize the lung lesions. Specimen removal was achieved using a widened (2 cm) trocar site. The operating times of Group I patients were longer compared than those of Group II patients (means: 154 and 68 min, respectively); conversion rates (8 versus 2), chest tube insertion rates (100% versus 51%), and postoperative ventilation (48% versus 13%) also differed. CONCLUSION: Thoracoscopic anatomical lung resections appear to be safe and effective in infants and children. In congenital lung diseases, the key to success is the intraoperative destruction of space-occupying lesions. Limitations exist in cases with infectious adhesions. Non-anatomical lung resections are technically easier and should remain standard in pediatric surgery. Limitations exist in cases of metastases, which are deep within the parenchyma and are not visible on the lung surface.

The role of BH3-mimetic drugs in the treatment of pediatric hepatoblastoma.

Pediatric hepatoblastoma (HB) is commonly treated by neoadjuvant chemotherapy and surgical tumor resection according to international multicenter trial protocols. Complete tumor resection is essential and survival rates up to 95% have now been achieved in those tumors classified as standard-risk HB. Drug resistance and occurrence of metastases remain the major challenges in the treatment of HB, especially in high-risk tumors. These conditions urgently require the development of alternative therapeutic strategies. One of those alternatives is the modulation of apoptosis in HB cells. HBs regularly overexpress anti-apoptotic proteins of the Bcl-family in comparison to healthy liver tissue. This fact may contribute to the development of chemoresistance of HB cells. Synthetic small inhibitory molecules with BH3-mimetic effects, such as ABT-737 and obatoclax, enhance the susceptibility of tumor cells to different cytotoxic drugs and thereby affect initiator proteins of the apoptosis cascade via the intrinsic pathway. Besides additive effects on HB cell viability when used in combination with cytotoxic drugs, BH3-mimetics also play a role in preventing metastasation by reducing adhesion and inhibiting cell migration abilities. Presumably, including additive BH3-mimetic drugs into existing therapeutic regimens in HB patients might allow dose reduction of established cytotoxic drugs and thereby associated immanent side effects, while maintaining the antitumor activity. Furthermore, reduction of tumor growth and inhibition of tumor cell dissemination may facilitate complete surgical tumor resection, which is mandatory in this tumor type resulting in improved survival rates in high-risk HB. Currently, there are phase I and phase II clinical trials in several cancer entities using this potential target. This paper reviews the available literature regarding the use of BH3-mimetic drugs as single agents or in combination with chemotherapy in various malignancies and focuses on results in HB cells.

BH3-mimetic drugs prevent tumour onset in an orthotopic mouse model of hepatoblastoma.

Drug resistance and metastasis remain major challenges in the treatment of high-risk hepatoblastoma (HB) and require the development of alternative therapeutic strategies. Modulation of apoptosis in HB cells enhances the sensitivity of these cells towards various drugs and has been discussed to enforce treatment. We investigated the impact of apoptosis sensitisers, BH3-mimetics, on the interaction between the host and HB to reduce tumour growth and dissemination while enhancing immunity. BH3-mimetics, such as obatoclax and ABT-737, enhanced the apoptosis-inducing effect of TRAIL and TNF-α resistant HB cells (HepT1 and HUH6). Tumour cell migration was inhibited by ABT-737 and more markedly by obatoclax. In an orthotopic model of HB, tumour uptake was reduced when the cells were pretreated with low concentrations of obatoclax. Only 1 of 7 mice developed HB in the liver, compared with an incidence of 0.8 in the control group. In summary, our study showed that apoptosis sensitisers had broader effects on HB cells than expected including migration and susceptibility to cytokines in addition to the known effects on drug sensitization. Sensitising HB to apoptosis may also allow resistant HB to be targeted by immune cells and prevent tumour cell dissemination.