Hans-Joachim Kirschner

Acute traumatic posterior elbow dislocation in children

Traumatic posterior dislocation of the elbow is often associated with significant morbidity and incomplete recovery. The aim of this study was to retrospectively analyse the outcome of 33 children (median age 10.8 years). Patients underwent reduction and assessment of stability under general anaesthesia. Pure dislocations (n=10) were immobilized, whereas unstable fractures (n=23) were stabilized. Refixation of ligaments was performed if stability was not achieved by fracture stabilization alone. Immobilization was continued for 26 (pure dislocations) or 35 days (associated injuries), respectively. Results were excellent (n=9) or good (n=1) after pure dislocation. Results were excellent (n=15), good (n=7) or poor (n=1) in children with associated injuries. Accurate diagnosis, concentric stable reduction of the elbow as well as stable osteosynthesis of displaced fractures are associated with good results in children with acute posterior elbow dislocations.

IGF2/H19 hypomethylation is tissue, cell, and CpG site dependent and not correlated with body asymmetry in adolescents with Silver-Russell syndrome

BackgroundSilver-Russell syndrome (SRS) is characterized by severe intrauterine and postnatal growth failure and frequent body asymmetry. Half of the patients with SRS carry a DNA hypomethylation of the imprinting center region 1 (ICR1) of the insulin-like growth factor 2 (IGF2)/H19 locus, and the clinical phenotype is most severe in these patients. We aimed to elucidate the epigenetic basis of asymmetry in SRS and the cellular consequences of the ICR1 hypomethylation.ResultsThe ICR1 methylation status was analyzed in blood and in addition in buccal smear probes and cultured fibroblasts obtained from punch biopsies taken from the two body halves of 5 SRS patients and 3 controls. We found that the ICR1 hypomethylation in SRS patients was stronger in blood leukocytes and oral mucosa cells than in fibroblasts. ICR1 CpG sites were affected differently. The severity of hypomethylation was not correlated to body asymmetry. IGF2 expression and IGF-II secretion of fibroblasts were not correlated to the degree of ICR1 hypomethylation. SRS fibroblasts responded well to stimulation by recombinant human IGF-I or IGF-II, with proliferation rates comparable with controls. Clonal expansion of primary fibroblasts confirmed the complexity of the cellular mosaicism.ConclusionsWe conclude that the ICR1 hypomethylation SRS is tissue, cell, and CpG site specific. The correlation of the ICR1 hypomethylation to IGF2 and H19 expression is not strict, may depend on the investigated tissue, and may become evident only in case of more severe methylation defects. The body asymmetry in juvenile SRS patients is not related to a corresponding ICR1 hypomethylation gradient, rendering more likely an intrauterine origin of asymmetry. Overall, it may be instrumental to consider not only the ICR1 methylation status as decisive for IGF2/H19 expression regulation.

Late-onset volvulus without malrotation in extremely preterm infants - a case–control-study

BackgroundVolvulus without malrotation in preterm infants is a rare but potentially life-threatening event of unknown aetiology. Confusion with necrotising enterocolitis may delay surgical intervention thereby aggravating morbidity and mortality.We aimed at elucidating potential risk factors for, and characteristic clinical signs of, volvulus without malrotation in preterm infants.MethodsRetrospective, single-centre case-control study (2007-2011). For every index patient, five infants of similar gestational age, birth weight and birth year were evaluated. Additionally, all 9 cases of necrotising enterocolitis occurring during the above period were evaluated. Data are presented as median (interquartile range).ResultsFive extremely premature infants suffering from volvulus without malrotation were identified (gestational age at birth 24.4 (23.6-25.5) weeks, birth weight 480 (370-530) g). All were small for gestational age and female; three out of five died. Volvulus occurred several weeks after birth, whereas necrotising enterocolitis occurred significantly earlier. Beyond that, no striking differences in clinical or laboratory presentation of volvulus without malrotation and necrotising enterocolitis were found. Infants with volvulus had significantly more frequent manipulations with rectal tubes for flatulence, but there were no differences in the frequency of enemas, abdominal massage or defecation. In infants with volvulus, nasal high-frequency oscillation was used more frequently for respiratory support, and PEEP-level tended to be higher.ConclusionsIn extremely premature infants volvulus without malrotation represents a life-threatening event that occurs typically several weeks after birth with an acute abdomen and seems to affect predominantly girls. Infants requiring intensive non-invasive respiratory support might be at highest risk.

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.

Epidemiological study on intestinal volvulus without malrotation in VLBW infants

Background We conducted a monthly epidemiological survey in Germany to detect the prevalence of volvulus without malrotation (VWM) in very low birthweight (VLBW) infants and to identify factors for a better distinction between this rare and life-threatening event and other acute abdominal diseases in preterm infants. Methods Throughout 2014 and 2015, every paediatric department in Germany was asked to report cases of VWM in infants with birth weights <1500 g to the Surveillance Unit for Rare Paediatric Conditions in Germany. Hospitals reporting a case were asked to return an anonymised questionnaire and discharge letter. Results Of 36 cases reported, detailed information was submitted on 29, with 26 meeting entry criteria. With 9896 and 10 140 VLBW infants born in Germany in 2014 and 2015, respectively, we estimated a prevalence of 1.52/1000 VLBW infants for 2014 and 1.08/1000 in 2015. No specific early symptom could be determined. 10% died, and 21% of infants developed short bowel syndrome. Calculated from all verified VWM arise death in 8% and a short bowel syndrome in 15%. Conclusion VWM is a rare source of acute abdomen in VLBW infants. No specific signs and symptoms potentially facilitating an early recognition of VWM could be found from this survey. Because the rates of death and short bowel syndrome are high, VWM should always be considered early in an acute abdomen in a VLBW infant.

ERCP in infants, children and adolescents is feasible and safe: results from a tertiary care center

Background Indications for endoscopic retrograde cholangiopancreatography (ERCP) in children differ from adults. Paucity of data and concerns about potential lower effectiveness and more side effects limit its use even in high volume centers. We retrospectively analyzed indications, success rates, limitations, and side effects of ERCPs in children <18 years. Methods From January 2012 to March 2015, 54 ERCPs were performed in 31 children (median age 11 (0–17) years; median weight 22 (3.3–142.7) kg) with suspected choledocholithiasis (n = 13 interventions in 9 patients), post-transplantation anastomotic stenosis (10/4), malignancy (10/5), chronic pancreatitis (7/1), biliary atresia (6/6), anomaly (2/2), leak (4/3), or primary sclerosing cholangitis (PSC) (2/1). All patients were followed up as inpatients. Results Thirty-six therapeutic and 18 diagnostic procedures were performed by adult ERCP expert endoscopists. Successful intervention was achieved in 90.7% of cases. Failed cannulation (n = 4) was associated with lower body weight (p = 0.023). In children younger than 1 year, ERCP was significantly more often diagnostic than in patients >1 year (p < 0.001). In three of six infants with suspected atresia, surgical exploration was avoided. Five complications were recorded (9.3%), and included four episodes of mild pancreatitis (7.4% post-ERCP pancreatitis (PEP) rate) and one cholangitis in PSC. A trend towards a protective effect of pancreatic stents on PEP was observed. All complications were managed conservatively. No complications were attributed to mechanical stress on the gastrointestinal tract. Conclusions ERCP in newborns, infants, and adolescents can be safely performed with high technical and clinical success. Endoscopists must be aware of differing spectrum of pediatric diseases. Failed cannulation was associated with lower body weight of young children. Complications were similar to rates reported in adults.

Bilateral single-system ectopic ureter associated with choledochal cyst: report of 2 cases

Ectopic ureters in children are associated with symptoms related to an abnormal localization or structure of the ureteric orifice including reflux or obstruction. Approximately 20% to 25% of ectopic ureters drain a nonduplicated renal system. Associated malformations are common in these cases. We treated 2 girls with bilateral single-system ectopic ureter associated with choledochal cysts. In both cases, ureteral reimplantation and resection of the choledochal cyst with hepaticojejunostomy were carried out. The coincidence of bilateral single-system ectopic ureter with choledochal cyst without any other severe malformation is not described in the literature.