Pediatric vascular anomalies in Austria. Where are we at? A survey among primary care pediatricians.

Introduction: Vascular anomalies (VAs) are rare conditions and affected patients often experience a difficult patient journey. Timely diagnosis is only possible if primary caregivers are aware of the anomalies and are connected with dedicated specialists. Aim of our survey was to investigate the knowledge about diagnostic and therapeutic possibilities for children with VAs, and the existing networking among primary pediatric caregivers in Austria. Methods: Primary care pediatricians in Austria were invited to complete an online questionnaire consisting of 28 questions focusing on pediatric VAs. Results: Out of 373 invited pediatricians 93 (25%) returned the questionnaires, 86 of which were complete. Most physicians (39/93 42%) answered that they see between 15 and 30 patients with infantile hemangiomas per year. Vascular malformations are rarely treated in the primary care setting; most primary care pediatricians (58/86, 67%) reported that they currently treat fewer than 5 patients with such type of VAs. There was unequivocal agreement among the participants (84/86, 98%) on the need to establish a network of specialists and a registry dedicated to pediatric VAs. Conclusions: This survey represents the first study shedding light on the awareness of VAs among Austrian pediatricians and can serve as a basis for future investigations and advances in the management of these conditions in Austria and other countries with a similar healthcare setting.

Probiotic OMNi-BiOTiC® 10 AAD Reduces Cyclophosphamide-Induced Inflammation and Adipose Tissue Wasting in Mice.

Cancer therapy is often associated with severe side effects such as drug induced weight loss, also known as chemotherapy-induced cachexia. The aim of this study was to investigate the effects of a multispecies probiotic (OMNi-BiOTiC® 10 AAD) in a chemotherapy mouse model. A total of 24 male BALB/c mice were gavage-fed with the probiotic formulation or water, once a day for 3 weeks. In the third week, the mice received intraperitoneal cyclophosphamide. At euthanasia, the organs were dissected, and serum was sampled for cytokine analysis. Tight junction components, myosin light chain kinase, mucins, and apoptosis markers were detected in the ileum and colon using histological analyses and qRT-PCR. Lipolysis was analyzed by enzymatic activity assay, Western blotting analyses, and qRT-PCR in WAT. The fecal microbiome was measured with 16S-rRNA gene sequencing from stool samples, and fecal volatile organic compounds analysis was performed using gas chromatography/mass spectrometry. The probiotic-fed mice exhibited significantly less body weight loss and adipose tissue wasting associated with a reduced CGI58 mediated lipolysis. They showed significantly fewer pro-inflammatory cytokines and lower gut permeability compared to animals fed without the probiotic. The colons of the probiotic-fed animals showed lower inflammation scores and less goblet cell loss. qRT-PCR revealed no differences in regards to tight junction components, mucins, or apoptosis markers. No differences in microbiome alpha diversity, but differences in beta diversity, were observed between the treatment groups. Taxonomic analysis showed that the probiotic group had a lower relative abundance of Odoribacter and Ruminococcus-UCG014 and a higher abundance of Desulfovibrio. VOC analysis yielded no significant differences. The results of this study indicate that oral administration of the multispecies probiotic OMNi-BiOTiC® 10 AAD could mitigate cyclophosphamide-induced chemotherapy side effects.

Beneficial Perioperative Aspects Favor the Use of Percutaneous Crossed Pinning over Antegrade Nailing in Pediatric Supracondylar Fractures-A Retrospective Comparative Study.

(1) Background: Displaced supracondylar humeral fractures in pediatric patients can be treated by either antegrade nailing (AN) or percutaneous crossed pinning (PCP). The aim of this study was to compare the intra- and perioperative management, complications and outcome of AN and PCP. (2) Methods: This retrospective study enrolled 271 individuals (median age 5 years, IQR 4-7 years) who underwent AN (n = 173) or PCP (n = 98). Patient history was analyzed for incidence of nerve injuries, postoperative treatment, postoperative malrotation, time of hospital stay, time to implant removal and revision rate. Operative procedures were investigated for duration and radiation exposure. (3) Results: PCP was associated with a significantly lower radiation exposure (dose area product: PCP mean 20.1 cGycm2 vs. AN mean 34.7 cGycm2, p < 0.001; fluoroscopy time: PCP mean 1.1 min, range 0.1-8.1 min, vs. AN mean 1.5 min, range 0.1-7.1 min, p < 0.001), duration of surgery (PCP mean 32.2 min vs. AN mean 48.3 min, p < 0.001) and time to implant removal (PCP mean 37 days vs. AN mean 113 days, p < 0.001). Cast removal was performed earlier in the AN group (PCP mean 30.2 days vs. AN mean 20.4 days, p < 0.001) and there were fewer iatrogenic nerve lesions (PCP: 24% vs. AN: 8%, p < 0.001). (4) Conclusions: In the investigated study population, the analyzed parameters seem to favor the use of PCP. The advantages of AN should be weighed against its drawbacks. For special indications, AN remains a relevant technique in supracondylar fracture treatment, and surgeons should be familiar with this procedure.

Outcome after surgical treatment of venous malformations of the hand in childhood.

OBJECTIVE: Surgical treatment of venous malformations (VMs) of the hand is challenging. The hand's small functional units, dense innervation, and terminal vasculature can be easily compromised during invasive interventions like surgery or sclerotherapy, leading to an increased risk of functional impairment, cosmetic consequences, and negative psychological effects. METHODS: We have conducted a retrospective review of all surgically treated patients diagnosed with VMs of the hand between 2000 and 2019 and evaluated their symptoms, diagnostic investigations, complications, and recurrences. RESULTS: Twenty-nine patients (females, n = 15) with a median age of 9.9 years (range, 0.6-18 years) were included. Eleven patients presented with VMs involving at least one of the fingers. In 16 patients, the palm and/or dorsum of the hand was affected. Two children presented with multifocal lesions. All patients presented with swelling. Preoperative imaging was done in 26 patients and consisted of magnetic resonance imaging in nine patients, ultrasound in eight patients, and both modalities in nine patients. Three patients underwent surgical resection of the lesions without any imaging. Indications for surgery were pain and restriction of function (n = 16), and when lesions were preoperatively evaluated as completely resectable (n = 11). In 17 patients, a complete surgical resection of the VMs was performed, whereas in 12 children, an incomplete resection of VM was deemed due to nerve sheath infiltration. At a median follow-up of 135 months (interquartile range, 136.5 months; range, 36-253 months), recurrence occurred in 11 patients (37.9%) after a median time of 22 months (range, 2-36 months). Eight patients (27.6%) were reoperated because of pain, whereas three patients were treated conservatively. The rate of recurrences did not significantly differ between patients presenting with (n = 7 of 12) or without (n = 4 of 17) local nerve infiltration (P = .119). All surgically treated patients who were diagnosed without preoperative imaging developed a relapse. CONCLUSIONS: VMs in the region of the hand are difficult to treat, and surgery is associated with a high recurrence rate. Accurate diagnostic imaging and meticulous surgery may contribute to improve the outcome of the patients.

Acute appendicitis manifests as two microbiome state types with oral pathogens influencing severity.

Mounting evidence suggests that acute appendicitis (AA) is not one but two diseases: complicated appendicitis, which is associated with necrosis leading to perforation or periappendicular abscess, and uncomplicated appendicitis, which does not necessarily result in perforation. Even though AA is the most frequent cause of surgery from abdominal pain, little is known about the origins and etiopathogenesis of this disease, much less regarding the different disease types. In this study, we investigated the microbiome (inter-domain amplicon and metagenome sequencing) of samples from the appendix, rectum and peritoneum of 60 children and adolescents with AA to assess the composition and potential function of bacteria, archaea and fungi. The analysis of the appendix microbial community revealed a shift depending on the severity of the AA. This shift was reflected by two major community state types that represented the complicated and uncomplicated cases. We could demonstrate that complicated, but not uncomplicated, appendicitis is associated with a significant local expansion of oral, bacterial pathogens in the appendix, most strongly influenced by necrotizing Fusobacterium spp., Porphyromonas and Parvimonas. Uncomplicated appendicitis, however, was characterized by gut-associated microbiomes. Our findings support the hypothesis that two disease types exist in AA, which cannot be distinguished beyond doubt using standard clinical characterization methods or by analysis of the patient's rectal microbiome. An advanced microbiome diagnosis, however, could improve non-surgical treatment of uncomplicated AA.

Therapy preference of 131 parents confronted with a pediatric femoral fracture.

Background: The management of femoral fractures in children between 3 and 5 years of age is still vividly debated. Therefore, we aimed to assess the basic attitude of parents if confronted with a hypothetical femoral fracture of their toddler. Materials and methods: Parents of children aged between 12 and 36 months were asked for their preference after receiving detailed information on conservative and surgical treatment of femoral shaft fractures. Furthermore, we obtained information regarding the parents' gender, marital status, medical background, highest level of education and profession in a leading or non-leading position and if any of their children already had undergone any operations. The Freiburg Personality Inventory (FPI-R) questionnaire was used to assess parents' personality traits. Results: In total, 131 participants were included in this study. The vast majority (n = 116, 88.5%) preferred surgical treatment. The most frequently mentioned reasons for this decision were lack of acceptance, followed by faster reconvalescence, shorter hospital stay, less deformity or growth disorders and less stress on the child. The only reason stated against surgical treatment was the need of general anesthesia. A significantly higher rate of conservative procedures was noticed in self-employed participants and stress was found to significantly influence the treatment decision of the parents toward conservative treatment. Conclusion: The majority of parents confronted with a hypothetical femoral fracture of their child questioned in this study opted for a surgical approach with elastic stable intramedullary nailing (ESIN). This corresponds with trends toward surgery in these cases in major trauma centers in Europe.

Standalone Axial Malrotation after Pediatric Supracondylar Fracture Does Not Seem to Be an Indication for Immediate Postoperative Revision Surgery.

Rotational spurs as evidence for post-surgical malrotation are frequently observed when treating pediatric supracondylar humeral fractures (SCHFs). This study aimed to investigate the long-term outcome of a pediatric cohort with unrevised axial malrotation and to discuss the indication for revision surgery. Postoperative radiographs of children treated for SCHFs over eight years were retrospectively analyzed. Children with radiological signs of malrotation (von Laer malrotation quotient) were invited for a follow-up clinical and radiological examination. Among 338 treated children, 39 (11.5%) with a mean age of 5.3 years (range 1.8-11.7 years) showed radiological signs for postoperative malrotation and were not revised and therefore invited to participate in the study. Twelve patients (31%) with a mean age of 11.3 years (range 8.8-13.8 years) took part in the follow-up examination after a mean of 7.1 years (range 5.4 to 11.3 years). The mean postoperative van Laer malrotation quotient was 0.15 (range 0.11-0.2). At follow-up, the range of motion of the elbow joint was not significantly different compared to the contralateral side. Apart from the humeral ulnar angle (p = 0.023), there were no significant differences in the radiological axes. The Flynn criteria were excellent and good in 90% of the cases. The mean was 1.7 points indicating excellent subjective results. Standalone postoperative malrotation did not lead to an adverse long-term outcome in a small cohort of pediatric patients with SCHFs and did not indicate immediate postoperative revision surgery. However, further investigations with larger cohorts should verify whether additional criteria such as stability of the osteosynthesis and signs for increasing valgus or varus displacement in the follow-up radiographs should get more importance in decision making.

Chylous content might determine the optimal surgical approach for mesenteric lymphatic malformations in childhood.

BACKGROUND: Mesenteric lymphatic malformations (LMs) represent rare congenital anomalies that can include chylous or nonchylous content. The pathologic mechanisms explaining this phenomenon are poorly understood and not yet described. Furthermore, the current management approach does not consider the contents of the mesenteric LMs. In the present study, we have defined the relationship between the lymphatic mesenteric cyst content and the histologic evidence of LMs within the bowel wall. METHODS: We retrospectively investigated all patients with mesenteric LMs treated surgically at our department from 1999 to 2018. RESULTS: A total of 11 patients (6 girls and 5 boys) were included in our analysis. Seven patients had presented with LMs located in the jejunal mesentery, three in the ileocecal region, and only one in the mesocolon transversum and omentum. Of the 11 children, 7 had had LMs with nonchylous content and 4 had presented with chylous content LMs. Intestinal resection was performed in all 4 patients with chylous content LMs and 4 patients with nonchylous content LMs. Histopathologic evaluation of the surgical specimens determined that only the LMs with chylous content displayed malformed lymphatic channels throughout the bowel wall. The resected small bowel of four patients with nonchylous content showed no LM extension throughout the intestinal wall. CONCLUSIONS: LMs with chylous content seem to develop from malformed lymphatic channels within the bowel wall. In such cases, segmental intestinal resection is mandatory. In contrast, mesenteric LMs with nonchylous content can potentially be treated without bowel resection if the blood supply can be preserved. This finding is, to the best of our knowledge, reported in the present study for the first time.

The Financial Burden of Surgery for Congenital Malformations-The Austrian Perspective.

Neonatal "surgical" malformations are associated with higher costs than major "non-surgical" birth defects. We aimed to analyze the financial burden on the Austrian health system of five congenital malformations requiring timely postnatal surgery. The database of the Austrian National Public Health Institute for the period from 2002 to 2014 was reviewed. Diagnosis-related group (DRG) points assigned to hospital admissions containing five congenital malformations coded as principal diagnosis (esophageal atresia, duodenal atresia, congenital diaphragmatic hernia, gastroschisis, and omphalocele) were collected and compared to all hospitalizations for other reasons. Out of 3,518,625 total hospitalizations, there were 1664 admissions of patients with the selected malformations. The annual mean number was 128 (SD 17, range 110-175). The mean cost of the congenital malformations per hospital admission expressed in DRG points was 26,588 (range 0-465,772, SD 40,702) and was significantly higher compared to the other hospitalizations (n = 3,516,961; mean DRG 2194, range 0-834,997; SD 6161; p < 0.05). Surgical conditions requiring timely postnatal surgery place a significant financial burden on the healthcare system. The creation of a dedicated national register could allow for better planning of resource allocation, for improving the outcome of affected children, and for optimizing costs.

Trauma in pediatric urology.

In pediatric trauma, the kidney is the most commonly injured organ of the urinary tract. Renal trauma occurs in 10% to 20% of all pediatric blunt abdominal trauma cases. The vast majority of renal injuries can be treated conservatively. However, cases associated with hemodynamic instability require operative interventions. Injuries to the ureter, bladder or urethra are almost exclusively encountered in polytraumatized children. The aim of this article is to give an overview on traumatic injuries to the pediatric urinary system.

Laparoscopic Treatment of Gastroesophageal Reflux Disease in Children: How We Do It.

Over decades now, laparoscopic Nissen fundoplication represents the treatment of choice for symptomatic children with gastroesophageal reflux disease (GERD) unresponsive to medication. Although the basic principles of Nissen's technique are still essential today, academic studies of long-term results, complications, and patients benefits have fostered distinct modifications. Identification of surgical factors for wrap migration, dysphagia, and recurrent GERD led to recommendations for "short and floppy" wraps with minimal dissection of the phrenoesophageal membranes. This report summarizes up-to-date information from experts in the field on "how to wrap it right" followed by a critical discussion about long-term benefits for children with GERD and future developments of laparoscopic Nissen fundoplication.

Update on Metabolic Bariatric Surgery for Morbidly Obese Adolescents.

Despite worldwide public attention and intense medical efforts, the prevalence of severe morbid obesity in children and adolescents is still rising. Similar to adults, excess adipose tissue triggers multiple immunological and metabolic pathways leading to serious co-morbidities such as impaired glucose tolerance or even type 2 diabetes (T2D), dyslipidemia, arterial hypertension, non-alcoholic fatty liver disease, and hyperuricemia. The management of severe childhood obesity requires a life-long multidisciplinary approach with a combination of lifestyle changes, nutrition, and medications. Standardized life-style intervention programs remain the first-line treatment for morbid obese children and adolescents, but unfortunately reveal limited long-term success. In such cases, metabolic bariatric surgery (MBS) has evolved from being a controversial issue to being included in distinct recommendations. According to the American Society for Metabolic and Bariatric Surgery (ASMBS) Pediatric Committee, indications for bariatric surgery in adolescence must follow very strict criteria. Adolescents with class II obesity (BMI > 120% of the 95th percentile) and a diagnosed co-morbidity or with class III obesity (BMI ≥ 140% of the 95th percentile) should be considered for MBS. These interventions represent high-risk operations, and adolescents should be treated in specialized, multidisciplinary high-volume obesity centers with long-term follow-up programs. The Roux-en-Y gastric bypass (RYGB) remains the gold standard of all malabsorptive procedures. Laparoscopic sleeve gastrectomy (LSG), which the authors pioneered as a stand-alone procedure in morbidly obese adolescents in 2008, has become the most commonly performed operation in morbidly obese adolescents at present. Recent literature proves that MBS is safe and effective in morbidly obese adolescents. Mid-term data have revealed significant improvement or even resolution of major co-morbidities. Thus, MBS for the treatment of morbidly obese adolescents has evolved from being a controversial issue to being included in distinct recommendations by several medical societies as a therapeutic strategy to reduce severe co-morbidities potentially causing end-organ damage in adulthood.

Interdisciplinary Radical "En-Bloc" Resection of Ewing Sarcoma of the Chest Wall and Simultaneous Chest Wall Repair Achieves Excellent Long-Term Survival in Children and Adolescents.

Introduction: Ewing sarcomas of the chest wall, historically known as "Askin tumors" represent highly aggressive pediatric malignancies with a reported 5-year survival ranging only between 40 and 60% in most studies. Multimodal oncological treatment according to specific Ewing sarcoma protocols and radical "en-bloc" resection with simultaneous chest wall repair are key factors for long-term survival. However, the surgical complexity depends on tumor location and volume and potential infiltrations into lung, pericardium, diaphragm, esophagus, spine and major vessels. Thus, the question arises, which surgical specialties should join their comprehensive skills when approaching a child with Ewing sarcoma of the chest wall. Patients and Methods: All pediatric patients with Ewing sarcomas of the chest wall treated between 1990 and 2020 were analyzed focusing on complete resection, chest wall reconstruction, surgical complications according to Clavien-Dindo (CD) and survival. Patients received neo-adjuvant chemotherapy according to the respective Ewing sarcoma protocols. Depending on tumor location and organ infiltration, a multi-disciplinary surgical team was orchestrated to perform radical en-bloc resection and simultaneous chest wall repair. Results: Thirteen consecutive patients (seven boys and six girls) were included. Median age at presentation was 10.9 years (range 2.2-21 years). Neo-adjuvant chemotherapy (n = 13) and irradiation (n = 3) achieved significant reduction of the median tumor volume (305.6 vs. 44 ml, p < 0.05). En-bloc resection and simultaneous chest wall reconstruction was achieved without major complications despite multi-organ involvement. Postoperatively, one patient with infiltration of the costovertebral joint and laminectomy required surgical re-intervention (CD IIIb). 11/13 patients were treated with clear resections margins (R1 resection in one patient with infiltration of the costovertebral joint and marginal resection <1 mm in one child with multiple pulmonary metastases). All patients underwent postoperative chemotherapy; irradiation was performed in four children. Two deaths occurred 18 months and 7.5 years after diagnosis, respectively. Median follow-up for the remaining patients was 8.8 years (range: 0.9-30.7 years). The 5-year survival rate was 89% and the overall survival 85%. Conclusion: EWING specific oncological treatment and multi-disciplinary surgery performing radical en-bloc resections and simultaneous chest wall repair contribute to an improved survival of children with Ewing sarcoma of the chest wall.

Nasal glial heterotopia: A rare interdisciplinary surgical challenge in newborns.

Nasal Glioma (NG) represents a rare congenital abnormality of the neonate, which can be associated with skull defects or even a direct communication to the central nervous system. MRI serves valuable information for differentiation from encephalocele, dermoid cyst and congenital hemangioma. Complete resection remains the treatment of choice. We present two cases of NG, which were both suspected during prenatal ultrasound and MRI. In the first case, postnatal MRI showed a transcranial continuity. Mass excision was performed and the defect was covered by a glabellar flap allowing a good cosmetic result. Postnatal MRI excluded a trans-glabellar communication in the second case. After surgical excision, the resulting skin defect was covered with a full thickness skin graft harvested from the right groin. In cases of NGs complete resection and cosmetic appealing results can be achieved and might necessitate a multidisciplinary approach.

Rapidly involuting congenital hemangioma of the liver in a newborn with incomplete Pentalogy of Cantrell: description of a new association.

The perinatal management of newborns with giant omphaloceles requires careful suspicion concerning 'unexpected' associated malformations. We describe a newborn with a combination of incomplete Pentalogy of Cantrell consisting of giant omphalocele, anterior congenital diaphragmatic hernia (CDH) and pericardial absence complicated by a hepatic rapidly involuting congenital hemangioma (RICH). A giant omphalocele was detected prenatally. Postnatally, staged closure of the omphalocele was planned. A mass of the liver was noted and (mis-)taken for a hematoma. In the further course, the baby developed cardiorespiratory insufficiency due to a central CDH which was excised. Subsequently, staged closure of the omphalocele became impossible. Specific workup revealed a mass in the left liver lobe. The mass was resected and the abdominal wall defect repaired. Histology confirmed the diagnosis of an RICH. The surgical treatment of newborns with giant omphaloceles requires a multidisciplinary neonatal support and an elaborate pediatric surgical armamentarium to cope with additional malformations.

Loop Ileostomy in Europe's Tiniest Male Newborn for Meconium-Related Ileus.

With the advances of neonatology, the survival rate for "live-born periviable fetuses" weighing < 300 g, a subgroup of extremely low birth weight (BW) infants, has improved over the past 10 years. Meconium-related ileus (MRI) represents an early postnatal hazard, and, if medical evacuation fails, a surgical challenge in such immature babies. We report the interdisciplinary management of surgically treated MRI in a newborn with a BW of 273 g. According to the worldwide database held by the University of Iowa, he is registered as the tiniest male newborn in Europe. The boy was born in the 25th gestational week by cesarean section after a triplet pregnancy with twin-twin transfusion syndrome, him being the donor. He had a BW of 273 g, whereas his brothers had a BW of 740 g and 722 g. Cardiopulmonary stabilization and ventilation were successful. He developed MRI unresponsive to medical treatment. On day 14 of life, a minilaparotomy was performed in the right lower quadrant to externalize a loop of the distal ileum in a no-touch technique. Despite the small diameter of only 2 mm, a standard loop ileostomy could be fashioned. There were no intra- or postoperative abdominal complications. Bowel function and weight gain were adequate and the ileostomy was closed electively 5 months later at a body weight of 3.5 kg. In summary, minilaparotomy and loop ileostomy placement were effective to treat surgical MRI in Europe's tiniest male newborn. With the advances of neonatology, pediatric surgery reaches new frontiers as well.

Eukaryotic Translation Initiation Factor 4AI: A Potential Novel Target in Neuroblastoma.

Neuroblastoma (NB) is the most common extracranial pediatric solid tumor. Children suffering from high-risk and/or metastatic NB often show no response to therapy, and new therapeutic approaches are urgently needed. Malignant tumor development has been shown to be driven by the dysregulation of eukaryotic initiation factors (eIFs) at the translation initiation. Especially the activity of the heterotrimeric eIF4F complex is often altered in malignant cells, since it is the direct connection to key oncogenic signaling pathways such as the PI3K/AKT/mTOR-pathway. A large body of literature exists that demonstrates targeting the translational machinery as a promising anti-neoplastic approach. The objective of this study was to determine whether eIF4F complex members are aberrantly expressed in NB and whether targeting parts of the complex may be a therapeutic strategy against NB. We show that eIF4AI is overexpressed in NB patient tissue using immunohistochemistry, immunoblotting, and RT-qPCR. NB cell lines exhibit decreased viability, increased apoptosis rates as well as changes in cell cycle distribution when treated with the synthetic rocaglate CR-1-31-B, which clamps eIF4A and eIF4F onto mRNA, resulting in a translational block. Additionally, this study reveals that CR-1-31-B is effective against NB cell lines at low nanomolar doses (≤20 nM), which have been shown to not affect non-malignant cells in previous studies. Thus, our study provides information of the expression status on eIF4AI in NB and offers initial promising insight into targeting translation initiation as an anti-tumorigenic approach for NB.

Volatile Organic Compounds, Bacterial Airway Microbiome, Spirometry and Exercise Performance of Patients after Surgical Repair of Congenital Diaphragmatic Hernia.

The aim of this study was to analyze the exhaled volatile organic compounds (VOCs) profile, airway microbiome, lung function and exercise performance in congenital diaphragmatic hernia (CDH) patients compared to healthy age and sex-matched controls. A total of nine patients (median age 9 years, range 6-13 years) treated for CDH were included. Exhaled VOCs were measured by GC-MS. Airway microbiome was determined from deep induced sputum by 16S rRNA gene sequencing. Patients underwent conventional spirometry and exhausting bicycle spiroergometry. The exhaled VOC profile showed significantly higher levels of cyclohexane and significantly lower levels of acetone and 2-methylbutane in CDH patients. Microbiome analysis revealed no significant differences for alpha-diversity, beta-diversity and LefSe analysis. CDH patients had significantly lower relative abundances of Pasteurellales and Pasteurellaceae. CDH patients exhibited a significantly reduced Tiffeneau Index. Spiroergometry showed no significant differences. This is the first study to report the VOCs profile and airway microbiome in patients with CDH. Elevations of cyclohexane observed in the CDH group have also been reported in cases of lung cancer and pneumonia. CDH patients had no signs of impaired physical performance capacity, fueling controversial reports in the literature.

Radioulnar interphalangeal joint angles in children and adolescents aged 0 to 19 years.

The purpose of this study is to determine the normal ranges of radioulnar (i.e. medial-lateral) finger deviations during growth. We retrospectively measured radioulnar interphalangeal joint angles in 6236 properly aligned thumbs and fingers in trauma radiographs of 4720 patients aged 0 to 19 years. The mean interphalangeal joint angle of the thumb was 0.2° (standard deviation 1.5°). The average proximal interphalangeal joint angles were ulnar deviation of 2.5° (1.7°) for the index, ulnar deviation 1.7° (1.5°) for the middle, radial deviation 1.3° (1.8°) for the ring, radial deviation 2.0° (2.8°) for the little fingers. The distal interphalangeal joint angles were ulnar deviation of 2.5° (1.7°), ulnar deviation 2.1° (1.7°), radial deviation 2.1° (1.7°), radial deviation 5.1° (2.8°) from index to the little fingers. Thumbs were typically straight, whereas the index and middle fingers deviated ulnarly, and ring and little fingers radially. There were no relevant differences in sex or laterality.