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Object Many ophthalmological abnormalities are described in conjunction with hydrocephalus. The results of visual field diagnosis remain a matter of further discussion. The aim of this study was to investigate visual field deficits in children with shunt-treated hydrocephalus. Methods All children over 6 years of age treated for hydrocephalus at the authors' institute between December 2007 and December 2008 were included in the study. The children underwent an ophthalmological investigation for strabismus and binocular function, ophthalmoscopy, visual acuity, and refraction. The special focus was the visual field diagnosis, which the authors established in all children with cognitive conditions. The investigation was made by using the Goldmann visual field examination (kinetic perimetry). Children with and without visual field defects were compared concerning age at the time of ophthalmological examination, genesis of hydrocephalus, and frontooccipital horn ratio measured on current CT or MR images. Results Complete investigations were undertaken in 56 children (24 girls and 32 boys, mean age 15.1 years). The following orthoptic pathological entities were diagnosed: 29 children have a strabismus in 29 cases, 17 of these have an exotropia, 12 an esotropia, 4 children a hypotropia, 2 a hypertropia and 3 children a heterophoria. A nystagmus was found in 10 children. The ocular fundus investigation showed 13 children with an optic nerve atrophy. A visual field diagnosis was possible in 44 of the 56 patients and was incomplete in 12 patients with cognitive deficits or inadequate compliance. In 24 of 42 children there was a concentric visual field constriction between 10° and 50° out of the center. Children with visual field deficits were older than those with a normal visual field (p = 0.051). Nine of 10 children with postmenigitic hydrocephalus had a visual field defect (p = 0.025). In children with visual field defects the frontooccipital horn ratio was significantly higher (p = 0.013). Conclusions The results suggest that children with shunt-treated hydrocephalus have a higher risk of having ophthalmological abnormalities. Visual field deficits are often a problem in these patients. A diagnostic visual field examination can complete the ophthalmological monitoring in patients with hydrocephalus, especially in patients with large ventricles. Children with postmeningitic hydrocephalus should be ophthalmologically monitored more frequently and intensively.

Methode und Ergebnisse Von 01/2001–06/2007 wurden 59 Kinder und Jugendliche mit der gesicherten Diagnose Kindesmisshandlung im Kinderzentrum der Universitat Leipzig stationar betreut (36-mal korperliche Misshandlung, 11 Vernachlassigungsfalle, 6-mal sexueller Missbrauch, 5 Kombinationsformen von Vernachlassigung und korperlicher Misshandlung, 1-mal Munchhausen-by-proxy-Syndrom). Deren Daten wurden retrospektiv mit einem Datenerfassungsbogen erhoben und mit Hilfe deskriptiver Statistik ausgewertet. Beide Geschlechter waren etwa gleich haufig betroffen, das Alter variierte stark. Bei 17% der misshandelten Kinder lag eine Entwicklungsstorung vor. In >91% (54 von 59) wurden keine regelmasigen Fruherkennungsuntersuchungen dokumentiert. Alkohol- oder andere Drogenprobleme bestatigten 17% der Elternteile. Alleinerziehend waren 53% der Mutter und 10% der Vater. Jeweils 7% der Vater und Mutter sowie 5% der Geschwister der misshandelten Kinder waren chronisch krank. Bei den 59 stationar behandelten Patienten wurden 108 konsiliarische Beurteilungen durchgefuhrt, nur in 20% wurde eine Fallkonferenz dokumentiert.

Congenital diaphragmatic hernia (CDH) occurs sporadically with an incidence of 1 : 2 500 live births. Despite the progress in neonatal intensive care, CDH remains associated with a mortality of at least 30 % in isolated cases. The in essence surgically correctable defect of the diaphragm en-ables the prenatal herniation of abdominal organs into the thoracic cavity. The resulting abnormal development of the airways and pulmonary vessels causes neonatal respiratory insufficiency and persistent pulmonary hypertension. The condi-tion can be diagnosed prenatally and the degree of pulmonary hypoplasia, which determines the postnatal course, can be measured to make an -individual prognosis. In severely affected patients, prenatal surgery may improve neonatal outcome by reversing pulmonary hypoplasia. This is currently implemented by percutaneous fetoscopic endoluminal tracheal occlusion (FETO) to trigger fetal lung growth. Although there are no maternal complications, preterm rupture of the membranes remains the major drawback of the procedure (20 % < 34 weeks). However, as compared to historical controls of a similar severity, survival as well as early neonatal morbidity are significantly improved by FETO. As a consequence, a multicentre randomised-controlled trial in fe-tuses with mod-erate hypoplasia on FETO com-pared to expectant management has been started ( www.totaltrial.eu ). Primary outcome measure is survival without chronic lung disease (i. e., with-out bronchopulmonary dysplasia). A trial in severely affected -fetuses with survival as main outcome is cur-rently under review by ethics committee. A standardised neonatal management en-ables optimal treatment and multicentre com-patibility. It remains to be proven if fetoscopic surgery can maintain a solid position in the prenatal treatment of CDH to improve both mortality and morbidity of the affected children.

Object During recent years, several biomarkers have been introduced for use in the diagnosis of traumatic brain injury (TBI). The primary objective of this investigation was to determine if S100B (or S100 calcium-binding protein B) and neuron-specific enolase (NSE) serum concentrations can effectively be used to discriminate between symptomatic and asymptomatic children with minor head trauma. Methods The authors conducted a prospective clinical study that involved patients age 6 months to 15 years who had sustained minor head trauma. Children with concomitant extracranial injuries were excluded. Blood samples were obtained within 6 hours of injury to measure S100B and NSE levels in serum. The authors defined 2 diagnostic groups: a mild TBI group (patients with Glasgow Coma Scale [GCS] scores of 13–15) in whom there were clinical signs of concussion (short loss of consciousness, amnesia, nausea, vomiting, somnolence, headache, dizziness, or impaired vision) and a head contusion group (patients with a GCS score of 15) in whom symptoms were absent. Both S100B and NSE concentrations were compared between the 2 groups. Secondary end points were defined as follows: correlation of S100B/NSE and a) the presence of scalp lacerations, b) GCS score, c) age, and d) correlation between S100B and NSE. Results One hundred forty-eight patients were enrolled (53 in the contusion group, 95 in the mild TBI group). After adjusting for differences in age and time of injury to blood sample withdrawal, there was no significant difference in S100B or NSE between patients in the 2 groups. Scalp lacerations and GCS score had no affect on posttraumatic S100B or NSE concentrations. The correlation between S100B and NSE was significant. Both markers showed a significant negative correlation with age. Conclusions The authors demonstrated that S100B and NSE do not discriminate between symptomatic and asymptomatic children with minor head injury. There seem to be limitations in marker sensitivity when investigating pediatric patients with mild TBI.

Study Type – Therapy (RCT) Level of Evidence 1b OBJECTIVE To evaluate the efficacy of different modes of combined therapy in children with monosymptomatic nocturnal enuresis (MNE). PATIENTS AND METHODS A randomized prospective study was performed to compare the order of two types of combined therapy in children with MNE. Group A was treated with primary desmopressin treatment that was combined with alarm treatment after 3 months, while group B was treated with primary alarm treatment that was combined with desmopressin after 3 months. RESULTS Within a period of 18 months, 43 previously untreated children fulfilled the inclusion criteria. Thirteen children achieved dryness after initial monotherapy or discontinued the study. Group A consisted of 16 children and group B of 14 children. After the standardized treatment course of 6 months, 11/16 children in group A and 11/14 children in group B became dry (

Bardet-Biedl syndrome (BBS) is a rare genetic disorder characterized by a wide range of phenotypic variability and associated with the development of life-threatening obesity. Birth weight tends to be normal, but rapid weight gain begins after the first year, probably due to polyphagia rather than abnormalities in energy metabolism. A morbidly obese 16-year-old male patient with BBS was referred to our institution, after nonsurgical methods of weight control had failed, for surgical treatment of his obesity. His preoperative body mass index (BMI) was 52.28 kg/m(2) (height, 1.84 m; weight, 177 kg) and was above the 99th centile for age and gender. The patient underwent laparoscopic Roux-en-Y gastric bypass (RYGBP). The postoperative period was uneventful. Three and a half years after the operation, the patient's weight has decreased to 118 kg (BMI, 34.85 kg/m(2)), while significant improvement in his hypertension, hyperuricemia, and mobility has been noted. In our BBS patient, RYGBP proved to be safe and effective; nevertheless, longer follow-up is required to evaluate the weight loss durability and to assess the lasting beneficial effect of surgical intervention on genetically determined co-morbidities.

Wir berichten uber ein Neugeborenes mit schwerer Schadel-Hirn-Verletzung nach Verkehrsunfall und konsekutiver Hypophyseninsuffizienz. Es entwickelte sich 5 Tage nach dem Trauma ein Syndrom der inadaquaten Sekretion antidiuretischen Hormons (SIADH) mit einer erheblichen Hyponatriamie, gefolgt von mehreren polyurischen Phasen mit einer hypernatriamischen Dehydratation. Des Weiteren wurden eine sekundare Hypothyreose, ein Hypokortisolismus sowie eine Hyperprolaktinamie diagnostiziert. Wir behandelten mit Desmopressin, L-Thyroxin und Hydrokortison. Innerhalb von 10 Monaten wurde eine vollstandige Erholung der Hypophysenfunktion beobachtet.

Liver transplantation is still the only treatment for end-staged liver diseases in children. However, donor organ shortage and immunosuppression are major limitations. Thus, approaches of hepatocyte transplantation are under investigation. Using cells might permit mass expansion, cryopreservation, and the ex vivo genetic modification of cells. For the development of cell-transplantation techniques, the use of three-dimensional scaffolds as carrier was shown to be advantageous. Polymeric matrices permit the formation of a neo-tissue and stimulation by the modification of the matrix surface. Another important issue is to define the right cell type for transplantation. Adult hepatocytes have a limited growth and differentiation potential. In contrast, fetal liver cells (FLC) possess an enormous growth and a bipotential differentiation potential. Thus, these cells may be very attractive as a cell resource for developing cell-based liver replacement. A third major issue in this approach is the neo-vascularization. Therefore, the transplantation in a recently developed model using a microsurgically created arterioveno-venous (AV) loop as a central vessel for the neo-tissue was used for transplantation of FLC in a fibrin-matrix. Initial results indicated that the transplantation of FLC using the AV-loop transplantation model may be promising for the development of highly vascularized in vivo tissue-engineered liver support systems.

ZusammenfassungDie positive Entwicklung der Überlebensrate extrem unreifer Frühgeborener sowie bei schweren Fehlbildungen ist das Ergebnis medizinischer Fortschritte und Neuerungen. Diese geht aber oftmals mit einer Zunahme von therapiebedürftigen Folgeerkrankungen und einer ausgeprägten psychosozialen Belastung mit späteren Verhaltensauffälligkeiten einher (1). Die Notwendigkeit begleitender psychosozialer Hilfs- und Unterstützungsangebote für diese Kinder und Eltern findet bisher nur an wenigen Perinatalzentren Beachtung. Das Defizit besteht einerseits in einer noch unzureichenden Verknüpfung der pflegerischen, medizinischen und psychosozialen Behandlungen, andererseits stellt sich durch das Fehlen eines funktionierenden Bindeglieds zwischen Kinderklinik und nachstationärer Betreuung eine große Versorgungslücke dar (2). In der vorliegenden Arbeit sind im Rahmen der psychosozialen Begleitung von Eltern Frühgeborener und kranker Neugeborener an unserem universitären Perinatalzentrum im Sinne eines Case-Management-Ansatzes von 2005–2007 Daten erhoben worden. Diese werden in der vorliegenden Arbeit analysiert und diskutiert. 718 Patienten von insgesamt 606 betreuten Familien wurden während des Studienzeitraums erfasst. Die psychosoziale Begleitarbeit umfasst die individuell auf die Familien abgestimmte Betreuung auf Station, sozialrechtliche Beratungen und Fallkonferenzen. Durch die Häufigkeit der Inanspruchnahme kann ihre Effizienz für die Familien belegt werden. Demzufolge sollte an allen Perinatalzentren die psychosoziale Betreuung von Eltern nach dem Case-Management-Ansatz gesetzlich vorgeschrieben werden.

ZusammenfassungKinderschutz ist ein gesellschaftlicher Auftrag und grundsätzliches Anliegen des Menschen. ÄrztInnen und viele psychosoziale Berufsgruppen, die Kinder und Jugendliche betreuen, sind Teil eines Systems, das für Früherkennung sowie für frühe Intervention und Hilfen ausgewiesen ist und heute innerhalb unserer Gesellschaft zur Verfügung steht. Prävention bedeutet im Kontext von Kinderschutz die Verhinderung von Kindesmisshandlung, Missbrauch und Vernachlässigung. In der Kinderheilkunde und in der Jugendmedizin spielt das Thema Prävention eine vorrangige Rolle. Die Bedeutung von Impfungen, Neugeborenenscreening-Untersuchungen (Stoffwechsel -screening und Hörscreening) zur Verhinderung bzw. frühzeitigen Erkennung und Behandlung von Krankheiten ist unbestritten. Psychosoziale Risikofaktoren, die eine positive Entwicklung behindern oder unmöglich machen, sind weitestgehend bekannt. Präventions-und Hilfsstrategien sind entwickelt worden und sollten überall in Deutschland zur Verfügung stehen. Einen wichtigen Stellenwert nehmen dabei die Neugeborenenscreening-und die Vorsorgeuntersuchungen U 1−J 1 ein. Die Teilnahme an diesen Untersuchungen muss verpflichtend sein. Die Umsetzung dieser Forderung kann durchaus unter den heutigen rechtlichen Bedingungen (Datenschutz, Schweigepflicht) erfolgen. Darüber hinaus sind die Verknüpfung von Melde-, Sozial- und Gesundheitsdaten sowie die daraus folgende Verpflichtung zur aufsuchenden Hilfe nachdrücklich weiter zu diskutieren.

Fetal tracheal occlusion (TO) is offered to fetuses with severe pulmonary hypoplasia due to congenital diaphragmatic hernia (CDH). TO induces lung growth, but even when performed minimally invasive, there is a risk for iatrogenic preterm delivery. Whenever this is anticipated, maternal glucocorticoids (GC) may be given to enhance lung maturation. The pulmonary effects of GC in fetuses with CDH that underwent TO are yet poorly defined. Therefore, we conducted a placebo-controlled study in the nitrofen (NF) rat model for CDH. Pregnant rats were gavage fed NF or olive oil (OO) on ED9.5. At ED19.0, fetuses were either assigned to TO or left untouched. Maternal betamethasone (BM) or saline (PLAC) was administered on ED20. Necropsy was done on ED21.5 to obtain lung-to-body-weight ratio (LBWR), and perform quantitative RT-PCR and fluorescent immunostaining for Ki-67 and proliferating cell nuclear antigen (PCNA) in fetal lungs. CDH fetuses had a lower LBWR than normal fetuses, but comparable pulmonary PCNA and Ki-67 expression levels. TO increased LBWR, irrespective of maternal BM or PLAC. However, BM but not PLAC inhibited proliferation in TO and unoperated fetuses. Rats with NF-induced CDH have hypoplastic lungs with normal proliferation indices. TO triggers proliferation, an effect countered by BM.

Neuroblastoma is a biological, genetic and morphological heterogeneous tumor with a variable clinical course. NCAM is a cell adhesion molecule belonging to the immunoglobulin superfamily with structural similarities to cell adhesion molecule L1. The aim of this study was to determine the expression of NCAM in neuroblastoma and to compare the results to the findings of a previous study which examined L1 expression in the same group of patients. NCAM expression was investigated on a tissue array with 66 surgically resected neuroblastoma samples by immunohistochemistry with a monoclonal antibody clone 1B6 and peroxidase method. Strong expression of NCAM was detected in all of the 66 (100%) neuroblastoma tumors in contrast to L1 which was not expressed in all tumors. In contrast to L1, which was found to predict favorable outcome, NCAM is universally expressed in neuroblastoma. Therefore NCAM represents a marker for neuroblastomas irrespectively of their stages whereas L1 as an indicator for developing neuronal cells seems to identify more mature stages of this tumor. The high grade of NCAM expression might present a prerequisite for establishment of antibody-based therapies.

The CCN3 protein (Nov, Nephroblastoma overexpressed) is a member of the CCN family (Cyr61, CTGF, Nov) of growth regulators and exerts antiproliferative properties. We show here that the tumor suppressor p53 transcriptionally upregulates the CCN3 gene. p53 is an important transcription factor contributing to cell cycle arrest and apoptosis after cell damage through the regulation of numerous target genes. We show that CCN3 mRNA and protein are upregulated following p53 expression. A DNA binding-deficient p53 mutant fails to regulate CCN3. CCN3 protein is located in the perinuclear space after induction and is also exported to the extracellular matrix. Furthermore, the CCN3 promoter is inducible by p53 and the response element is located in the first exon of the CCN3 gene. Chromatin immunoprecipitations show that p53 binds to the CCN3 promoter in vivo. As CCN3 was shown to inhibit cell growth, transcriptional induction by p53 may serve as an antiproliferative signal in the extracellular matrix. Furthermore, CCN3 depletion was also reported to reduce collagen type IV-dependent adhesion of melanocytes. Thus, elevated levels of CCN3 protein regulated by p53 might influence cell adhesion.

The molecular mechanisms underlying the regulation of vas deferens (VD) motility and semen emission are still poorly understood. Interstitial cells of Cajal (ICC), which harbour the c-kit receptor (CD117), provide the basis of coordinated gut motility. We investigated whether c-kit receptor-positive cells also exist in the normal human VD. Enzyme and fluorescence immunohistochemical techniques were applied on serial sections of human proximal, middle, and distal VD segments (n=49) employing 13 different monoclonal and polyclonal antibodies recognizing the c-kit receptor. The c-kit receptor was detected in either round- or spindle-shaped cells. On account of their antigenic profile, the round- and oval-shaped c-kit receptor-positive cells were identified as mast cells (MC) occurring in all layers of the VD except the epithelium. In contrast, two distinct populations of exclusively c-kit receptor-positive spindle-shaped cells were found within the lamina propria and, rarely, in the inner and outer smooth muscle layers, as well as within the epithelium. Different shaped c-kit receptor-positive MC and IC were present in all layers of the human VD. Our findings demonstrate the presence of different c-kit receptor-positive cells also in the human VD. Their rather ubiquitous distribution within the lamina propria and muscle layers suggests that IC and MC may modulate the neuromuscular transmission and the propagation of electrical signals in multiple systems involved in the draining of fluids. The importance of the c-kit receptor-positive interepithelial cells remains unclear.

Neuroblastoma (NBL) is the most common solid tumor in children. Tumors in advanced stage or with positive risk factors still have a poor prognosis. Thy1 (CD90) is a membrane glycoprotein expressed in thymus, retinal ganglionic cells, and several types of stem cells. The aim of this study was to assess Thy1 expression in NBL and analyze the correlation with clinical outcome. Sixty-three specimens of NBL were stained for Thy1 on a tissue microarray by immunohistochemistry. Fresh frozen tumor tissues were used for RNA isolation, and RT-PCR analysis for Thy1-mRNA expression was performed. Patients’ survival data were correlated with Thy1 status using a log rank test and a Cox regression multivariate analysis. Thy1 was expressed on 51 (81%) of the tumors. Kaplan–Meier survival analysis showed a significantly impaired survival in patients with NBL missing Thy1 (P

ZusammenfassungAufgrund der statischen, der dynamischen und der schützenden Funktion der Wirbelsäule für das Rückenmark besitzen Wirbelsäulenverletzungen eine große klinische Bedeutung. Kräfte auf die Wirbelsäule wirken am Gelenkkapsel- und Bandapparat oder treten als direkte Kontusionen auf. Verletzungen, die zu Frakturen führen, betreffen vorrangig die Brustwirbelsäule (BWS) und die Lendenwirbelsäule (LWS). Beteiligungen der Halswirbelsäule (HWS) sind rar. Die Ursache für eine vermehrte BWS-Verletzung im Kindesalter liegt in der hohen Elastizität des Thoraxbereiches.Als Unfallursachen im Kindesalter dominieren Verkehrsunfälle und Stürze aus der Höhe sowie der Fall von schweren Gegenständen auf das Kind. Zwei Drittel aller Wirbelsäulenverletzungen des Kindesalters sind Kompressionsfrakturen von Wirbelkörpern mit Keilbildungen unter 10°, die eine konservative Therapie nach sich ziehen. Eine operative Behandlung ist selten.Von 1980–2005 behandelten wir 244 Patienten mit Wirbelsäulen (WS)-Kontusionen und 65 Patienten mit WS-Frakturen. Begleitverletzungen traten bei 21 Patienten (6,7%) auf. Alle Verletzungen der Patienten mit Kontusionen der WS heilten unter konservativer Behandlung aus. Nur 5 Patienten (7,7%) mit WS-Frakturen mussten einer operativen Korrektur unterzogen werden.

ZusammenfassungLängendefizite und Wachstumsdifferenzen der Extremitäten lassen sich mittels operativer Eingriffe korrigieren. Dazu stehen verschiedene Möglichkeiten zur Verfügung. Abhängig von der individuellen Situation und der Behandlungsstrategie kommen der monolaterale Fixateur externe, der dreidimensionale Ringfixateur und in letzter Zeit zunehmend der nach dem Hexapoden-Prinzip arbeitende Taylor-Spatial-Framezum Einsatz. Dreidimensionale Korrekturen erfordern eine aufwändige Planung und den Einsatz spezieller Computerprogramme. Besonders im Bereich der statisch belasteten unteren Extremitäten gewährleisten diese Techniken gute Ergebnisse für die Patienten.Im Zeitraum von 2004–2006 wurden an der Klinik und Poliklinik für Kinderchirurgie des Universitätsklinikums Leipzig AöR 7 Kinder mit Längendefiziten und Deformitäten der unteren Extremitäten mit dem Taylor-Spatial-Frame erfolgreich behandelt.

ZusammenfassungSowohl Prävalenz als auch Ausmaß der Adipositas haben im Kindes- und Jugendalter in den letzten Jahrzehnten drastisch zugenommen. Im Gegensatz zu Erwachsenen gibt es für die operative Behandlung der morbiden Adipositas im Kindes- und Jugendalter kaum Erfahrungen. Sie lehnen sich hauptsächlich an die bereits etablierten Techniken in der Allgemeinchirurgie an. Am häufigsten wird das Magenbanding, die Roux-en-Y-Bypassoperation, die Schlauchmagenplastik oder die Platzierung eines Magenballons durchgeführt. Ziel der Behandlung ist neben einer Verbesserung der Lebensqualität vor allem die Besserung bereits vorhandener metabolischer, kardiovaskulärer und orthopädischer Begleiterkrankungen (arterieller Hypertonus, Typ-2-Diabetes mellitus, Fettstoffwechselstörungen, Hyperurikämie, Gelenkprobleme oder Schlafapnoe). Die publizierten klinischen Studien zeigen eine hohe Effektivität für die postoperative Gewichtsabnahme, wobei langfristige Ergebnisse bei Kindern noch nicht vorliegen. Aufgrund der präoperativ bestehenden Sekundärerkrankungen und des massiven Übergewichts ist die Adipositaschirurgie allerdings mit spezifischen Risiken behaftet. Alle Patienten bedürfen daher neben einer sorgfältigen, postoperativen Evaluierung und Risikostratifizierung einer interdisziplinären Betreuung in einem spezialisierten Zentrum.

ZusammenfassungDie laparoskopische (minimal-invasive) Chirurgie hat sich bei vielen ablativen und rekonstruktiven Eingriffen in der Kinderchirurgie etabliert. Minimal-invasive Techniken sind im Vergleich zu offenen Operationen gleichermaßen effektiv und weisen eine geringere Morbidität und bessere Kosmetik auf. Nun breitet die minimal-invasive Chirurgie ihren Einfluss zunehmend auch in der Kinderurologie aus: Laparoskopische Operationen des Hodenhochstandes und Nephrektomien (laparoskopisch oder retroperitoneoskopisch) sind nicht nur akzeptierte Alternativen zu den konventionellen Operationsverfahren, sondern haben sich zum „Goldstandard“ entwickelt. Technisch aufwändigere bzw. rekonstruktive Verfahren wie die Pyeloplastik, Heminephrektomie und Adrenalektomie werden in zunehmend großen Serien publiziert und zeigen bei kürzerem Krankenhausaufenthalt vergleichbare Operationszeiten und Raten an Komplikationen. Initiale Erfahrungen mit der laparoskopischen Ureterneuimplantation und der rekonstruktiven Blasenchirurgie sind vielversprechend. Es bedarf allerdings weiterer kontrollierter, randomisierter Studien, um die tatsächliche Überlegenheit laparoskopischer Verfahren zu beweisen.

ZusammenfassungDas Krankheitsbild der ECF ist charakterisiert durch eine Lösung und langsam zunehmende Dislokation der proximalen Femurepiphyse (Hüftkopfgleiten) im Jugendalter. Die Erkrankung tritt einseitig, nicht selten mit einem Zeitintervall beidseits auf. Ätiologisch werden mehrere Möglichkeiten diskutiert. Eine Ursache ist die mechanische Instabilität der Knorpelsubstanz der Epiphysenfuge durch endokrine Regulationsstörungen.Die Diagnostik umfasst neben der Klinik das konventionelle Röntgenbild in zwei Ebenen, bei unklaren Befunden die MRT-Untersuchung. Die Behandlung erfordert eine Epiphyseodese mittels Kirschnerdrähten oder Schrauben der erkrankten und prophylaktisch auch der Gegenseite. Das eingebrachte Material wird erst nach sicherem Fugenschluss wieder entfernt.Im Zeitraumvon 1999–2005 behandelten wir 41 Patienten. Im Behandlungsverlauf wurden 7 Korrekturosteotomien erforderlich, darunter bei 5 Patienten nach einem akuten Abrutsch und ausgeprägter Dislokation bzw. nach Ausbildung einer Hüftkopfnekrose.

Next generation sequencing techniques are currently revealing novel insight into the microbiome of the human gut. This new area of research seems especially relevant for neonatal diseases, because the development of the intestinal microbiome already starts in the perinatal period and preterm infants with a still immature gut associated immune system may be harmed by a dysproportional microbial colonization. For most gastrointestinal diseases requiring pediatric surgery there is very limited information about the role of the intestinal microbiome. This review aims to summarize the current knowledge and outline future perspectives for important pathologies like necrotizing enterocolitis (NEC) of the newborn, short bowel syndrome (SBS), and Hirschsprung’s disease associated enterocolitis (HAEC). Only studies applying next generation sequencing techniques to analyze the diversity of the intestinal microbiome were included. In NEC patients intestinal dysbiosis could already be detected prior to any clinical evidence of the disease resulting in a reduction of the bacterial diversity. In SBS patients the diversity seems to be reduced compared to controls. In children with Hirschsprung’s disease the intestinal microbiome differs between those with and without episodes of enterocolitis. One common finding for all three diseases seems to be an overabundance of Proteobacteria. However, most human studies are based on fecal samples and experimental data question whether fecal samples actually represent the microbiome at the site of the diseased bowel and whether the luminal (transient) microbiome compares to the mucosal (resident) microbiome. In conclusion current studies already allow a preliminary understanding of the potential role of the intestinal microbiome in pediatric surgical diseases. Future investigations could clarify the interface between the intestinal epithelium, its immunological competence and mucosal microbiome. Advances in this field may have an impact on the understanding and non-operative treatment of such diseases in infancy.

In children, fractures have a huge impact on the health care system. In order to develop effective prevention strategies exact knowledge about the epidemiology of fractures is mandatory. This study aims to describe clinical and epidemiological data of fractures diagnosed in infants.A retrospective analysis of all infants (children1 year of age) presenting with fractures in an 11 years period (2001-2011) was performed. Information was obtained regarding the location of the fractures, sites of the accident, circumstances and mechanisms of injury and post-injury care.248 infants (54% male, 46% female) with a mean age of 7 months presented with 253 fractures. In more than half of the cases skull fractures were diagnosed (n=151, 61%). Most frequently the accidents causing fractures happened at home (67%). Falls from the changing table, from the arm of the care-giver and out of bed were most commonly encountered (n=92, 37%). While the majority of skull fractures was caused from falls out of different heights, external impacts tended to lead to fractures of the extremities. 6 patients (2%) were victims of maltreatment and sustained 10 fractures (2 skull fractures, 4 proximal humeral fractures, 2 rib fractures, and 2 tibial fractures).Falls from the changing table, the arms of the caregivers and out of bed caused the majority of fractures (especially skull fracture) in infants. Therefore, awareness campaigns and prevention strategies should focus on these mechanisms of accident in order to decrease the rate of fractures in infants.

In recent years, the use of robotic-assisted surgery (RAS) has expanded within pediatric surgery. Although increasing numbers of pediatric RAS case-series have been published, the level of evidence remains unclear, with authors mainly focusing on the comparison with open surgery rather than the corresponding laparoscopic approach. The aim of this study was to critically appraise the published literature comparing pediatric RAS with conventional minimally invasive surgery (MIS) in order to evaluate the current best level of evidence.A systematic literature-based search for studies comparing pediatric RAS with corresponding MIS procedures was performed using multiple electronic databases and sources. The level of evidence was determined using the Oxford Centre for Evidence-based Medicine (OCEBM) criteria.A total of 20 studies met defined inclusion criteria, reporting on five different procedures: fundoplication (n=8), pyeloplasty (n=8), nephrectomy (n=2), gastric banding (n=1), and sleeve gastrectomy (n=1). Included publications comprised 5 systematic reviews and 15 cohort/case-control studies (OCEBM Level 3 and 4, respectively). No studies of OCEBM Level 1 or 2 were identified. Limited evidence indicated reduced operative time (pyeloplasty) and shorter hospital stay (fundoplication) for pediatric RAS, whereas disadvantages were longer operative time (fundoplication, nephrectomy, gastric banding, and sleeve gastrectomy) and higher total costs (fundoplication and sleeve gastrectomy). There were no differences reported for complications, success rates, or short-term outcomes between pediatric RAS and conventional MIS in these procedures. Inconsistency was found in study design and follow-up with large clinical heterogeneity.The best available evidence for pediatric RAS is currently OCEBM Level 3, relating only to fundoplication and pyeloplasty. Therefore, higher-quality studies and comparative data for other RAS procedures in pediatric surgery are required.

In 1989, Sauer introduced a technique to treat patients with total colonic aganglionosis (TCA) where he preserved the ileo-cecal valve and the right hemicolon to retain water and electrolyte absorption. This report examines the long-term outcome of patients who underwent this technique. All TCA patients treated between 1981 and 2005 according to Sauer were invited in 2013 to participate in a questionnaire survey to assess their long-term outcome focusing on bowel function and quality of life (QoL), using standardized scores. Of eight TCA patients (2 females, 6 males), seven participated in the follow-up survey (median follow-up time 13.5 years (range 8–31.6). Early postoperative complications involving bouts of enterocolitis in 3 and anal strictures in 5 patients ceased with age. Bowel movements ranged from median 3–4 times a day (range 1–8). Bowel-function score (BFS) was reduced in 6 patients (median 16 points, range 8–19, max 20), who also reported soiling incidences with the need for night-time protective aids in two. QoL assessment, however, documented a good outcome with a median score of 10 points (range 7–13, max 13). This long-term investigation of TCA patients treated according to Sauer’s technique documented a good QoL despite a reduced BFS.

BACKGROUND Neonates with congenital diaphragmatic hernia and large defects often require patch closure. Acellular collagen matrices (ACM) have been suggested as an alternative to synthetic durable patches as they are remodeled by the host or could also be used for tissue engineering purposes. MATERIALS AND METHODS 2.0x1.0 cm diaphragmatic defects were created in 6-weeks old New-Zealand white rabbits. We compared reconstruction with a purpose-designed cross-linked ACM (Matricel) to 4-layer non-cross-linked small intestinal submucosa (SIS) and a 1-layer synthetic Dual Mesh (Gore-Tex). Unoperated animals or animals undergoing primary closure (4/0 polyglecaprone) served as age-matched controls. 60 (n = 25) resp. 90 (n = 17) days later, animals underwent chest x-ray and obduction for gross examination of explants, scoring of adhesion and inflammatory response. Also, uniaxial tensiometry was done, comparing explants to contralateral native diaphragmatic tissue. RESULTS Overall weight nearly doubled from 1,554±242 g at surgery to 2,837±265 g at obduction (+84%). X-rays did show rare elevation of the left diaphragm (SIS = 1, Gore-Tex = 1, unoperated control = 1), but no herniation of abdominal organs. 56% of SIS and 10% of Matricel patches degraded with visceral bulging in four (SIS = 3, Matricel = 1). Adhesion scores were limited: 0.5 (Matricel) to 1 (SIS, Gore-Tex) to the left lung (p = 0.008) and 2.5 (Gore-Tex), 3 (SIS) and 4 (Matricel) to the liver (p

Aim Fluorescence diagnosis is gaining clinical importance for the detection of malignancies in various medical disciplines. The technique relies on the specific metabolic capacity of a lesion to produce a fluorescent compound. It is still unknown whether pediatric solid tumors like hepatoblastoma (HB) are susceptible to this technique as well. Methods Human HB (3 × 10 6 ) cells were laparoscopically injected (4 mm scope, 18-G needle) underneath the peritoneum of 7 nude rats (mean weight, 198 g). Tumor growth was allowed for 7 weeks. Photosensitization was induced by peritoneal lavage with aminolevulinic acid (3%). After 3 hours, the animals were investigated with white light laparoscopy (WL) and laparoscopic fluorescence diagnosis (LFD), applying the Storz PDD system. Every suspicious lesion was analyzed by spectrometry and harvested for histology. Results Every tumor seen in WL also demonstrated strong fluorescence during LFD (100%). One micrometastasis, almost invisible in WL, was obviously illuminating in LFD. Spectrometry demonstrated the specific peak of the aminolevulinic acid metabolite protoporphyrin IX at 635 nm and a 6.34-fold increase of the fluorescence intensity. Histology revealed HB in all specimens. Conclusion Human HB can be detected with LFD in a rat model. This finding opens a wide spectrum of experimental and clinical investigations to evaluate the impact of fluorescence diagnosis for pediatric oncology.

BACKGROUND/PURPOSE: The transanal endorectal pull-through technique (TERPT) for Hirschsprung's disease (HD) exercises considerable traction on the anorectal tissue during dissection. So the question arises as to whether TERPT impairs the integrity of the anorectal sphincter. Computerised 8-channel vector manometry allows a segmental, 360 degrees analysis of muscular defects along the anal canal. Such data after TERPT are not available yet. METHODS: Between 2002 and 2004, 7 children underwent primary TERPT for HD of the rectosigmoid. All could be recruited for follow-up examinations. Stooling pattern, rectal examination, conventional 4-channel and computerised 8-channel vector manometry were assessed; the anal sphincter pressure at rest (ASPR), rectoanal inhibitory reflex (RIR), anal canal length (ACL), high pressure zone (HPZ), maximal segmental pressure (max SP), segmental/total asymmetric index (SAI/TAI), vector volume at rest (VV) were studied. Mean pre- and postoperative values were compared (pre/post). RESULTS: After a mean of 14 months (range 3-21 months) all children had spontaneous bowel movements, with no complaints of encopresis or constipation. 4-channel manometry revealed an unchanged ASPR (48.1/49.2 mmHg). RIR was present in 1/7. Computerised 8-channel comparison revealed no changes for ACL (15.4/16 mm), HPZ (60/53.19 % of ACL), SAI (17.6/18.63 %) and TAI (35.8/35.63 %). A postoperative increase was noted for max SP squeeze (141.4/178.7 mmHg) and VV (38 161/46 680 mmHg/cm (2)). In conclusion, the TERPT for HD preserves the functional integrity of the anorectal sphincter complex and has a favourable clinical and manometric outcome.

ZusammenfassungDie hypertrophe Pylorusstenose ist eine der häufigsten chirurgischen Erkrankungen im Säuglingsalter. Seit der Erstbeschreibung der Operation vor fast einem Jahrhundert wird sie erfolgreich mit einer Pyloromyotomie behandelt. Über die letzten 15 Jahre hat sich die laparoskopische, minimalinvasive Technik dieser Operation etabliert. Hierbei wird die Operation über kleine, unauffällige Hautschnitte mit speziellen Instrumenten durchgeführt. Vorteile dieser Methode für den Patienten sind das bessere kosmetische Ergebniss, eine Verringerung des Gewebeschadens und eine schnellere postoperative Erholung.

Background Instilling perfluorooctyl bromide (PFOB) into the fetal lung may lead to alveolar distension. Objective The aim of the study was to evaluate the safety of PFOB instillation into fetal lungs and to determine the radiographic distribution and tissue concentration of PFOB in New Zealand white rabbits. Methods Sibling fetuses of pregnant (day 27) New Zealand white rabbits were randomized to intratracheal instillation of 1 mL PFOB with tracheal ligation, instillation without ligation, and unmanipulated controls. The maternal animals were killed directly after instillation, at 3 or 6 hours (n = 10 each). For each study cohort, we determined fetal lung/body weight (FLBW) ratios, the radiographic distribution of PFOB, as well as pulmonary PFOB and water content by tissue distillation. PFOB concentrations in maternal and fetal tissues were assessed by gas chromatography. Results The relative amount of fetal lung PFOB recovered by fractional distillation was highest in ligated (25%) and lower in unligated lungs (9%). Extrapulmonary PFOB was found in the fetal brain (2.0 ± 0.7 ppm), but not in any other fetal or maternal tissues. Mean FLBW ratios were highest in ligated fetuses, followed by unligated fetuses and controls. PFOB partially displaced fetal lung water. PFOB was visible in the lungs of all treated fetuses. Fetal survival between manipulated and unmanipulated fetuses did not differ. Conclusions After prenatal intrapulmonary instillation, some PFOB remains in the lung, even if the trachea is not ligated, and may exert distending pressure on the alveoli.

Buts: Le developpement rapide des techniques laparoscopiques met le chirurgien pediatre face a des challenges difficiles concernant son habilite et sa competence. De ce fait, l'entrainement doit realiser unepreparation parfaite. L'etude experimentale presentee explore comment les residents en chirurgie realisent une biopsie laparoscopique de l'intestin et reparent le defect, apres qu'ils se soient entraines sur un pelvitrainer ou sur un lapin. Methodes: Des lapins White New Zealand, des instruments 3 mm, une camera 5 mm et une suture au Prolene 6/0 sont utilisees. Douze residents en chirurgie etaient divises en deux groupes. Le but de leur travail etait de realiser une biopsie laparoscopique seromusculaire de l'intestin et de reparer ce defect avec une suture. Le groupe I s'etait entraine 8 fois avec un pelvitrainer (PT), le groupe Il s'etait entraine de la meme facon sur un lapin (RM). Chaque participant realisait un test final sur le lapin. Le temps de l'operation, le temps de la suture et l'eventuelle perforation de l'intestin etaient analyses. Resultats: Dans le groupe PT, le temps moyen de l'operation diminuait de 11,18 (′ 5,04) minutes a 4,91 (′ 0,89) minutes (p < 0,01), bien que le test final sur le lapin demandait 9,62 (′5,11) minutes. Dans le groupe RM, le temps moyen de l'operation diminuait de 10,04 (′3,39) minutes a 6,38 (′ 1,40) minutes (p < 0,01) durant les huit operations d'entrainement. Dans ce groupe, l'operation finale prenait 5,45 (′0,67) minutes et etait significativement plus rapide que le groupe PT 9,62 (′ 5,11 ) minutes (p < 0,1). Le temps de suture etait equivalent. Une difference significative concernant le taux de perforations n'etait pas trouve. Conclusions: L'entrainement repete chez le lapin permet une meilleure preparation pour les interventions chez l'homme. Dans les centres de Chirurgie Pediatrique avec des interventions laparoscopiques, le modele animal doit etre considere comme une etape importante de l'entrainement et contribue a une amelioration pour le patient.

Hodenhochstand oder Maldescensus testis gehort zu den haufigsten Storungen der mannlichen Genitalorgane. Aufgrund potenzieller Schaden bei verzogerter Behandlung ist eine Korrektur innerhalb der ersten 12–18 Lebensmonate unbedingt erforderlich. Der Artikel gibt eine Ubersicht uber gangige osterreichische und europaische Leitlinien, uber die aktuellen Behandlungsmoglichkeiten sowie uber die Prognose nach erfolgter Operation.

Der gastroosophageale Reflux (GOR) ist ein haufiges Phanomen im Kindesalter. Eine Indikation zur Chirurgie ergibt sich bei erfolglosen oder nicht Erfolg versprechenden konservativen Therapieversuchen eines klinisch relevanten GOR. Die Chirurgie ist eine kausale Therapie mit einer Erfolgsquote von >90%. Sie besteht aus einer Hiatusplastik und einer Fundoplicatio, gelegentlich zusatzlich einer Gastropexie oder Anlage eines Gastrostomas. Das minimal invasive Vorgehen ist wegen der geringeren postoperativen Morbiditat und der besseren Kosmetik in der Regel dem offenen Vorgehen vorzuziehen, das speziellen Indikationen vorbehalten ist. Schwere psychomotorische Retardierung und Zustand nach Osophagusatresie sind haufig mit einem ausgepragten GOR assoziiert und bedingen gleichzeitig schlechtere postoperative Ergebnisse. Wahrend im Normalfall die Hemifundoplicatio bevorzugt wird, sollte bei diesen Patientengruppen eine lockere 360°-Fundoplicatio erwogen werden. Alternative Operationsverfahren kommen nur sehr selten zur Anwendung.

Background Wound dressing changes after heat injuries expose the patient to repeated painful and frightening procedures in short intervals. Safe, adequate, and easily administered analgesia and sedation are required. The goal of this study was to evaluate the off-label use of rectally administered S(+)-ketamine and Midazolam by paediatric surgeons during repeated outpatient dressing changes for paediatric burns and scalding. Patients and methods A total of 47 dressing changes of 30 children with I - IIa degrees burns were evaluated. Vital signs, side-effects, complications, anxiolysis, and analgesia were recorded during the procedure and for the following two hours. Patients were assessed by a discharge scoring system and an age-appropriate pain scoring system at regular intervals. Before discharge, parents were interviewed on their level of satisfaction with the protocol. Results Adequate sedation and analgesia was achieved in 44 procedures (94 %). No complications and, in particular, no compromise of breathing, ventilatory, and cardiovascular functions were recorded. The discharge scoring system indicated a return to baseline function 30 minutes after the procedure in all patients. The parents were generally very satisfied with the protocol. All children old enough to be questioned were found to have an anterograde amnesia for the duration of the procedure. Conclusion Conscious sedation with rectally applied S(+)-ketamine and Midazolam allows safe and painless dressing changes after heat injuries in children.

Background: The present study was performed to investigate the differences in lymphocyte subpopulations and HLA-DR expression on monocytes as markers for the systemic immune response in children undergoing either minimal invasive surgery (MIS) or conventional (open) surgery. Methods: In a prospective study we compared 20 children admitted for fundoplication or chest wall correction. The surgical access was decided based upon the individual anatomy. Postoperative inflammatory immune response was assessed for the MIS versus open group by measuring the level of C-reactive protein (CRP) and lymphocyte subpopulations (CD3, CD4, CD8, NK cells, CD25, and HLA-DR on monocytes) by a flow cytometer. Blood samples were collected preoperatively and 12, 24, 72 h, and 7 days after surgery. Results: Postoperatively in the open surgery group a significantly greater increase of CRP was recorded. Open surgery, but not MIS, was found to cause a significant reduction of all lymphocyte subpopulations and HLA-DR on monocytes. A...

Vorliegende Studie pruft Effektivitat und Sicherheit einer rektal verabreichten Analgosedierung im Sinne einer „Conscious Sedation“ in der ambulanten Kinderchirurgie. In 50 Sitzungen zur Durchfuhrung kleiner diagnostischer und therapeutischer Eingriffe wurden bei 38 Patienten vor der Intervention je 0,75 mg/kg KG Ketamin und 0,4 mg/kg KG Midazolam rektal appliziert. Die Vitalparameter, Anxiolyse und Schmerzempfindung wurden dokumentiert, sowie aufgetretene Nebenwirkungen und unerwunschte Komplikationen protokolliert. Die Zufriedenheit von Patienten und Eltern wurde speziell evaluiert. Unter Berucksichtigung der adaquaten Indikationsstellung, der Verwendung von etablierten Medikamenten in standardisierten Dosierungen und geregelten organisatorischen Rahmenbedingungen ist die Durchfuhrung einer Analgosedierung im Sinne einer „Conscious Sedation“ durch den behandelnden Arzt, auch in Abwesenheit eines Anasthesisten, sicher und komplikationslos moglich. Der Vorteil ist ein kooperativer Patient mit stabilen Vitalparametern. Die Patienten und deren Eltern profitieren von einer erheblichen Stressreduktion durch Amnesie und Schmerzfreiheit.

The concept that fetal tissue transplants enjoy an immunologic privilege grounds on the primary immaturity of major histocompatibility complex (MHC) expression. However, experiences in human organ transplantation reveal that the immunogenicity of any graft could be modified by external factors such as ischemia. Consequently, the question arises, whether the process of transplantation modifies the immunogenicity of fetal grafts. In a syngeneic rat model (Lewis), fetal adrenal glands were transplanted into the greater omentum of adult hosts. After harvesting the grafts sequentially, the immunogenicity was evaluated by analyzing the expression and distribution of the MHC classes I and II and were compared with untreated organs of equivalent age. The untreated fetal adrenal gland depicted little immunogenicity. However, compared with age-matched untreated control organs, at 2 wk after transplantation, the grafts demonstrated an increased expression of MHC I and II, upregulated throughout the entire adrenal cortex. No signs of MHC-mediated rejection were found. The upregulation of MHC persisted until the eighth week after transplantation. At 3 months after transplantation the expression of MHC I and II returned to the normal pattern of untreated controls. As this study used a purely syngeneic model, the immunologic changes observed could not be induced by a graft vs. host incompatibility, instead they were caused by experimental factors. The expressions of MHC class I and II was increased at 2 wk, but these proteins did not induce a T-cell mediated rejection or cellular infiltration. In conclusion, these findings question the concept of an immunologic privilege of fetal tissue transplants. Instead, experimental factors may modify the tissue's primary immaturity of its MHC. Further investigations must evaluate, whether the increase in MHC expression will have an impact on the rejection of fetal adrenal grafts in allogeneic hosts.

ZusammenfassungFremdkörperingestionen sind im Kindesalter häufig. Eine potenziell lebensbedrohliche Komplikation ist vermeidbar, wenn verschluckte Gegenstände rechtzeitig erkannt und je nach ihrer Beschaffenheit und Lage adäquat behandelt werden. Im Ösophagus retinierte Knopfbatterien sind eine Indikation zur sofortigen endoskopischen Entfernung, während andere Gegenstände in der Speiseröhre innerhalb von 24 Stunden geborgen werden sollten. Fremdkörper, die kaudal des Zwerchfelles gelegen sind, werden in der überwiegenden Mehrzahl der Fälle mit dem Stuhl ausgeschieden. Falls nötig, ist die flexible Endoskopie das Standardverfahren zur Extraktion von Fremdkörpern im Verdauungstrakt. Das Risiko einer Fremdkörperingestion kann durch entsprechende Aufklärung der Sorgeberechtigten verringert werden.

Objective: Infants with gastroschisis (GS) still face severe morbidity. Prenatal closure may prevent gastrointestinal organ damage, but intrauterine GS repair (GSR) has not been established yet. Methods: In New Zealand White rabbits we developed and compared GS versus GSR: creation of GS was achieved by hysterotomy, right-sided laparotomy of the fetus and pressure on the abdominal wall to provoke evisceration. GSR was accomplished by careful reposition of eviscerated organs and a running suture of the fetal abdominal wall. For study purposes, 18 animals were divided equally into 3 groups: GS, GS with GSR after 2 h, and unmanipulated controls (C). Vitality was assessed by echocardiography. After 5 h all animals were sacrificed. Results: GSR inflicted no increased mortality, because all fetuses survived GS or GS with GSR. All fetuses with GS demonstrated significant evisceration of abdominal organs. In contrast, the abdominal wall of the fetuses from GSR was intact. Conclusion: The present animal model demonstrated the technical feasibility and success of an intrauterine repair of GS for the first time. However, further long-term studies (leaving GS and GSR in utero for several days) will be necessary to compare survival rates and intestinal injury, motility or absorption. The clinical application of GSR in utero remains a vision so far.

The essential role of major histocompatibility complex (MHC) class I and II in the process of rejection has been documented, and some studies suggest that fetal transplants could enjoy an organ-specific immunologic privilege. However, little is known as to when these antigens develop in fetal organs and which tissues mainly present them. This study investigated the dynamics of immunogenicity in the developing transplant organs of rats. The study focused on the classic transplant organs including lung, heart, liver, pancreas, intestine, and kidney. Fetal organs (14th and 20th day of gestation), organs at the 3rd, 7th, 10th, 14th and 28th days postpartum (pp), 2 and 3 months pp, and adult organs were taken and snap-frozen in liquid nitrogen. MHC expression was analyzed applying the APAAP technique on serial cryosections by two well-defined monoclonal antibodies (mAb) generated against rat MHC class I (Ox 18) and class II (Ox 6). Immunoreactivities were compared to those of different monoclonal markers against endothelial cells (HIS52, CD 31), histiocytes (ED 1, ED 2), dendritic cells (Ox-62), granulocytes (HIS48), B-cells (RLN-9D3), T-cells (Ox-52), CD 4 (Ox-35), CD 8 (Ox-8a), natural killer cells (10/78), and CD 45 (Ox-1, leukocyte common antigen). A non specific mAb (MR 12/53) served as a negative control. In all stages of organ maturation, MHC I expression was found predominantly on immunocompetent cells, endothelial cells, and certain parenchymal cells, whereas MHC II was almost entirely restricted to dendritic cells. In organ development, the onset of MHC I expression and the number of MHC II-positive cells varied in a time-dependent manner. However, between the 2nd and 3rd month pp the expression pattern was comparable to adult organs. The study indicates that each organ carries a variable immunologic burden, that matures heterogeneously. Consequently, the variable content of MHC I/II in organ maturation needs to be considered for any transplantation model.

Since the first description in 1998, the minimally invasive repair of pectus excavatum has gained increasing acceptance. The aim of this survey is to report on the experiences of eight European centres with this technique. 172 patients with a mean age of 15.1 (+/- 4.6) years were treated and evaluated, 35.5 % were symptomatic. 45.3 % of the patients had an asymmetric configuration of the chest, 74.3 % had a CT index above 3.25. Mean duration of the operative procedure was 76 minutes. Major complications, including dislocation of the bar or stabiliser, pneumonia, atelectasis, local infection, pleural and pericardial effusion and liver injury occurred in 11.1 % of the patients. Minor complications, such as self-resolving pneumothorax, atelectasis and subcutaneous emphysema were reported in another 8.1 %. Early cosmetic results were excellent or good in 81.5 %. Although the surgical procedure is simple, blood-sparing and short, consideration of some important technical details, proper patient selection and knowledge of the limitations is of vital importance. Long-term results are still lacking.

Autologous serological screening of a cDNA expression library (SEREX) derived from childhood neuroblastoma led to the identification of 10 different antigens, including 6 novel gene products. The novel antigen 018INX was derived from a small open reading frame in a region of α-internexin mRNA that was previously described as 3′ untranslated region. 018INX thus represents a novel type of tumor antigen. Five novel gene products were derived from NNP-1 (NNP3) and Hu genes (HuC-L, HuD3, HuDY, HuD1proc). As indicated by sequence analysis, these antigens were generated by alternative splicing and/or alternative promoter usage or allelic polymorphism. mRNA expression analyses revealed different tissue restrictions of novel compared to known HuD and NNP-1 transcripts in normal and malignant tissues. The expressions patterns of distinct transcripts indicated potential clinical meanings as diagnostic and/or prognostic tissue markers. When kinetics of serum antibody titres against SEREX-defined antigens were compared to tumor load over time in our patient with neuroblastoma, we found 100-fold increases of anti-Hu and anti-018INX antibody titres preceding the clinical diagnosis of recurrent tumor growth after 2 years. When sera of pediatric patients with cancer (30) and healthy controls (30) were tested for humoral responses to SEREX-defined neuroblastoma antigens, we detected antibodies against all known antigens and NNP3 with low frequencies and titres in control sera, while anti-018INX and anti-Hu antibodies were found in cancer patients only. Our findings indicate that SEREX-defined tumor antigens might provide novel tools for understanding and treatment of this aggressive childhood malignancy. © 2002 Wiley-Liss, Inc.

Purpose The aim of the present study is to present the outcome of a cohort of adolescent patients with trochlear dysplasia and elevated tibial tuberosity trochlear groove (TTTG) distance suffering from recurrent patellar dislocation. Treatment consisted of medial patellofemoral ligament (MPFL) reconstruction and a modified Grammont procedure. Methods MRI examinations were obtained pre- and postoperatively. Trochlear dysplasia was classified according to Déjour, and TTTG was measured on MRI. The Tegner Activity Scale and the Kujala Knee Score were assessed preoperatively and at follow-up. The Kujala Knee score and the IKDC 2000 knee score were documented at follow-up (median 50, range 20–61 months; SD 16.6). Results Seven knees of six patients (median age 16.5 years, range 14–17 years) with trochlear dysplasia and elevated TTTG distance (median 17 mm, range 16.1–21.9 mm; SD 2.8) were treated. Trochlear dysplasia was classified as Déjour type A in 1, type B in 5, and type C in 1 knee. The Kujala Knee Score significantly increased from values of 55 (range 17–88; SD 25.9) to 94 (range 73–100; SD 9.1) at follow-up (p = 0.028). TAS improved from preoperative 2 (range 0–7; SD 2.5) to 5 (range 4–9; SD 1.8) at follow-up (p = 0.034). Median IKDC 2000 Knee Score at follow-up was 89 (range 61–100, SD 13.4). No re-dislocations were encountered. Conclusion In selected adolescents with recurrent patellofemoral instability, MPFL reconstruction in combination with a modified Grammont technique yields excellent functional outcome and could, therefore, help to avoid major procedures, such as osteotomies. Level of evidence Therapeutic, Level IV.

Objective To assess the effect of neuroblastoma (NB) on the intestinal microbiome, metabolism, and inflammatory parameters in a murine model. Materials and Methods Athymic Hsd:Fox1nu mice received subperitoneal implantation of human NB cells (MHH-NB11) (tumor group, TG) or culture medium (sham group). Following 10 weeks of tumor growth, all animals were sacrificed to collect total white adipose tissue (WAT). Luminex assays were performed for gut hormone and inflammation marker analysis. Bile acids were measured by high-performance liquid chromatography–mass spectrometry in feces and serum. The microbiome of the ileal content was determined by 16S rDNA next-generation sequencing. Results At 10 weeks, tumors masses in the TG reached a mean weight of 1.10 g (interquartile range 3.45 g) associated with a significant reduction in WAT. Furthermore, in the TG, there was a marked reduction in leptin and an increase in glucagon-like peptide 1 serum levels. Moreover, the TG mice displayed a pro-inflammatory profile, with significant increases in monocyte chemotactic protein 1, tumor necrosis factor alpha, and interleukin-10. Lithocholic acid, deoxycholic acid, and ursodeoxycholic acid were significantly decreased in the stool of TG mice. Significant alterations of the intestinal microbiome were found in the ileal contents of the TG. Conclusions The present study provides a first glimpse that human NB in a murine model induces tumor cachexia associated with alterations in metabolic and inflammatory parameters, as well as changes in the intestinal microbiota. Since the intestinal microbiome is known to contribute to the host's ability to harvest energy, a favorable modulation of the intestinal microbiome in tumor patients could potentially represent a novel therapeutic target to prevent tumor-associated cachexia.

Introduction: Venous malformations in the bowel are extremely rare in children. A few case reports recommend the laparoscopic assisted mobilization of the lesion and conversion to an external resection and anastomosis. However, in infants with large tumors of the descending and sigmoid colon, this strategy would require a laparotomy. Case Description/Technique Description: A 2-year-old girl presented with painless rectal bleeding and anemia. Ultrasonography and magnetic resonance imaging (MRI) revealed a 5 3 3-cm angiodysplastic lesion of the distal bowel. Colonoscopy verified a vascular malformation of the sigmoid with exophytic growth. Performing a 4-port laparoscopy (3–5 mm), we identified the lesion along with grossly distended blood vessels in the sigmoid colon. After hitching it to the anterior abdominal wall, we carefully mobilized the lesion. To avoid a laparotomy of equivalent size or significant bleeding during externalization, the mass was resected laparoscopically using the LigaSure device (Covidien, Mansfield, Massachusetts). Finally an all-in laparoscopic anastomosis was fashioned (4–0 Vicryl, interrupted stitches; Ethicon, Somerville, New Jersey). The inspection of both remaining colonic margins showed no macroscopic evidence of the disease. The specimen was placed in a bag and morcellated with forceps through one slightly extended port site until it could be extracted. Operative time was 269 minutes. Histology described a venous malformation. The postoperative course was uneventful, and after a follow-up of more than 1.5 years, the girl remains free of symptoms. Conclusion: An all-in laparoscopic resection of a vascular malformation of the colon can be performed successfully and with excellent cosmetic results in children and even infants.

The formation and environmental release of highly toxic organohalogen compounds associated with informal recycling of waste electric and electronic equipment (e-waste) is a growing problem at e-waste dumps/recycling sites (EWRSs) in many developing countries worldwide. We chose a cross-sectional study design to measure the internal exposure to polychlorinated dibenzo-p-dioxins and dibenzofurans (PCDD/Fs) as well as polychlorinated biphenyls (PCBs) of individuals working on one of the largest EWRSs of Africa, located at Agbogbloshie, Accra, Ghana, and in controls from a suburb of Accra without direct exposure to EWRS activities. In whole blood samples of 21 age matched male exposed individuals (mean age: 24.7 years, SD 6.0) and 21 male controls (mean age: 24.4 years, SD 5.7) 17 PCDD/F congeners were determined. Moreover three indicator PCB congeners (#138, #153 and #180) were measured in blood of 39 exposed (mean age: 27.5 years, SD 11.7) and 19 non-exposed (mean age: 26.8 years, SD 9.7) patients. Besides a health examination, biometric and demographic data, residential and occupational history, occupational exposures and working conditions were recorded using a standardized questionnaire. In the exposed group, median PCDD/F-concentrations were 6.18 pg/g lipid base WHO2005-TEq (range: 2.1–42.7) and significantly higher compared to the control group with 4.60 pg/g lipid base WHO2005-TEq (range: 1.6–11.6). Concentrations were different for 2,3,7,8-TetraCDD, three HexaCDD and all 10 PCDF congeners, indicating a combustion pattern. Using a multivariate regression analysis exposure to EWRS activities was the most important determinant for PCDD/F exposure. Median PCB levels for the indicator congeners #138, #153 and #180 were 0.011, 0.019 and 0.008 μg/l whole blood (ranges: 0.002–0.18, 0.003–0.16, 0.002–0.078) in the exposed group and, surprisingly, significantly higher in the controls (0.037, 0.062 and 0.022; ranges: 0.005–0.46, 0.010–0.46, 0.004–0.21). In a multivariate regression approach e-waste related activities had no positive influence on internal PCB exposure, but rather the time living in Accra. The internal PCB exposure is in particular notable for a country where PCBs have historically never been produced or used. The impact of EWRS activities on organohalogen compound exposure of individuals working at and living in the surroundings of the Agbogbloshie EWRS, and the surprisingly high PCB exposure of people living in Accra not involved in e-waste activities require further investigation. Keywords: Polychlorinated dibenzo-p-dioxins and dibenzofurans (PCDD/Fs), Polychlorinated biphenyls (PCBs), Human blood, E-waste, Agbogbloshie, Ghana

BACKGROUND: We aim to report a 5-year retrospective multicentric European survey about the outcome of laparoscopic partial nephrectomy in infants and children with duplex kidneys. METHODS: The data of fifty-two children underwent laparoscopic partial nephrectomy (42 upper-pole nephrectomies and 10 lower-pole nephrectomies) in six European centers of Pediatric Surgery, were collected and analyzed. Median age at surgery was 5.1 years (range 6 months-9.7 years). There were 32 girls and 20 boys. In 37 patients, the left side was affected and in 15 patients the right side. For the right side, 4 trocars were used and for the left side 3/4 trocars. Special hemostatic devices were used for dissection and parenchymal section in all centers. We assessed intraoperative and postoperative morbidity. RESULTS: Median length of surgery was 166.2 min (70-215 min). No conversion to open surgery nor intraoperative bleeding was reported. Mean hospitalization was 3.5 days. We recorded 10/52 complications (4 urinomas, 2 recurrent UTIs, 4 prolonged urinary leakage), all managed conservatively. Reoperation rate was 0%. No loss of renal function on the residual kidney moiety was recorded in all operated patients. CONCLUSIONS: Laparoscopic partial nephrectomy remains a technically challenging procedure performed only in pediatric centers with high experience in minimally invasive surgery. Although the median operative time was higher than 2 h, we recorded no conversions in our series. The complication rate remains high (10/52-19.2%). All were II grade complications according to Clavien-Dindo classification and were treated conservatively without the need of other surgical procedures.