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Background/aim Hepatic hemangiomas (HH) can show an aggressive course with significant complications. Prognostic markers that identify an aggressive course are entirely absent. Since we have showed that Hedgehog signaling is overexpressed in aggressive hemangiomas of the skin. Here, we hypothesize that it is also altered in aggressive HH. Materials and methods Immunohistological staining for GLUT1 and quantitative PCR was performed in seven specimens with aggressive HH. For comparison, we included specimens of kaposiform hemangioendothelioma (KHE), skin hemangioma and normal liver tissue. Results Overexpression of the Hedgehog signaling components SHH and GLI2 and its target gene FOXA2 in HH were similar to those found in aggressive skin hemangioma and KHE, their expression being significantly higher than in mild skin hemangioma. High expression levels of SHH and FOXA2 positively correlated with HH, but not with normal liver tissue. Conclusion Hedgehog signaling is up-regulated in aggressive HH. This finding may lead to a biomarker allowing early intervention.

We report on four female adolescents, who presented with inflammatory symptoms. Extensive diagnostic workup revealed tumors on different locations. After surgical removal, clinical and laboratory signs of inflammation disappeared rapidly. On histology, the tumors showed a mixture of inflammatory cells characteristic of inflammatory pseudotumors in three of the patients.In patients with unclear inflammatory symptoms, inflammatory pseudotumor should be added to the differential diagnosis.• The inflammatory pseudotumor (IPT) is a mostly benign myofibroblastic tumor of the soft tissue and causes inflammatory symptoms. What is new: • IPTs have may wider than hitherto defined histologic features. Removal of IPT is curative.

Chirurgische Erkrankungen der Schilddruse im Kindesalter sind selten und sollten daher in entsprechenden Zentren behandelt werden.

Chirurgische Erkrankungen der Trachea betreffen osophagotracheale Fisteln, Tracheomalazien und Stenosen. Die Anlage eines Tracheostomas ist eine der haufigsten kinderchirurgischen Eingriffe an der Trachea und dient der Sicherung des Atemwegs.

Fehlbildungen des Halses betreffen von den Kiemengangen und dem Ductus thyreoglossus ausgehend Fisteln und Zysten sowie die auserst selten auftretenden medianen Halsspalten und Flugelfellbildungen (Pterygium colli). Pathologien der Gefassysteme werden in dieser Region haufiger gesehen und imponieren als Gefastumoren, wie Hamangiome, und kongenitale Gefasmalformationen, wie Lymphangiome und Blutgefasdysplasien.

Entzundliche Veranderungen betreffen uberwiegend Lymphknoten; hier sei insbesondere auf den Befall mit atypischen Mykobakterien hingewiesen.

Infantile hemangioma is a vascular neoplasm and is one of the most common tumors diagnosed in young children. Although most hemangiomas are harmless and involute spontaneously, some show severe progression, leading to serious complications, such as high-output cardiac failure, ulcerations, compression of the trachea or deprivation amblyopia, depending on their size and localization. However, the pathogenesis and cause of hemangioma are largely unknown to date. The goal of this study was to identify markers that could predict hemangiomas with aggressive growth and severe progression that would benefit from early intervention. By using a PCR-based screening approach, we first confirmed that previously known markers of hemangioma, namely FGF2 and GLUT1, are highly expressed in hemangioma. Nevertheless, these genes did not show any differential expression between severely progressing tumors and mild tumors. However, transcriptional upregulation of several Hedgehog signalling components, comprising the ligand Sonic Hedgehog (SHH), the transcription factor GLI2 and its target gene FOXA2 were detected in extremely aggressive hemangioma specimens during the proliferation phase. Notably, GLI2 was even overexpressed in involuting hemangiomas if they showed an aggressive growth pattern. In conclusion, our data suggest that overexpression of the Hedgehog components SHH, GLI2 and FOXA2 might be used as markers of an aggressive hemangioma that would benefit from too early intervention, while FGF2 and GLUT1 are more general markers of hemangiomas.

BACKGROUND Oral propranolol has been used to treat complicated infantile hemangiomas, although data from randomized, controlled trials to inform its use are limited. METHODS We performed a multicenter, randomized, double-blind, adaptive, phase 2-3 trial assessing the efficacy and safety of a pediatric-specific oral propranolol solution in infants 1 to 5 months of age with proliferating infantile hemangioma requiring systemic therapy. Infants were randomly assigned to receive placebo or one of four propranolol regimens (1 or 3 mg of propranolol base per kilogram of body weight per day for 3 or 6 months). A preplanned interim analysis was conducted to identify the regimen to study for the final efficacy analysis. The primary end point was success (complete or nearly complete resolution of the target hemangioma) or failure of trial treatment at week 24, as assessed by independent, centralized, blinded evaluations of standardized photographs. RESULTS Of 460 infants who underwent randomization, 456 received treatment. On the basis of an interim analysis of the first 188 patients who completed 24 weeks of trial treatment, the regimen of 3 mg of propranolol per kilogram per day for 6 months was selected for the final efficacy analysis. The frequency of successful treatment was higher with this regimen than with placebo (60% vs. 4%, P

Only 1 % of all hemangiomas are in the anogenital region. Their main complication is ulceration due to the macerating influence of urine and stool. Ulcerated hemangiomas are painful, may become infected, and have a tendency to heal slowly. Prevention of ulceration is the most important aim in these cases. Active therapy with laser is possible but may induce ulceration. A new possibility to treat such hemangiomas is the application of s-blocker as a systemic drug therapy.

Es wird uber 3 Patienten im Alter von 12, 14 Wochen und 4 Jahren mit Atemnot berichtet, bei denen die thorakale Sonografie eine bronchogene Zyste aufdeckte. In allen 3 Fallen zeigte die Thoraxaufnahme nur eine unspezifische Uberblahung beider Lungen mit nur fraglicher Weichteildichte. Die laterale Thoraxaufnahme zeigte eine Verdrangung von Osophagus und Trachea in 2 Fallen, wahrend thoraxsonografisch in allen 3 Fallen eine echoarme, glatt begrenzte Struktur ohne Dopplerflus zwischen Pulmonalarterie und Aorta descendens darstellbar war. Diese Struktur wurde im Computertomogramm (CT) bestatigt. Bronchoskopisch war eine Vorwolbung der Pars membranacia typisch. Es erfolgte die chirurgische Resektion der Zysten, die histologisch als bronchogene Zysten bestatigt wurden. In 1 Fall trat 3 Monate postoperativ wieder Atemnot auf. Bronchoskopisch und mittels NMR wurde ein Rezidiv der bronchogenen Zyste festgestellt. Es erfolgte die erfolgreiche Abtragung eines Rezidivs der Zyste mittels Lasertechnik uber die Bronchoskopie. Schlusfolgerung: Bei Atemwegsobstruktion ist an eine bronchogene Zyste mit der Moglichkeit einer Darstellung durch die Sonografie zu denken. Postoperativ kommen Rezidive vor, die einer Laserbehandlung zuganglich sind.

Die zahnarztliche Betreuung und Behandlung von Kindern sollte fruhzeitig erfolgen, da die Kiefer- und Gesichtsentwicklung eng mit der Gebissentwicklung verknupft ist. Der Erhalt der Milchzahne bis zum physiologischen Ersatz durch bleibende Zahne ist aus diesen Grunden das Ziel der padiatrischen Zahnheilkunde.

Hamangiome sind im Sauglings- und Kleinkindesalter die haufigsten gutartigen proliferativen Weichteiltumore und betreffen etwa 2% aller normalgewichtigen Sauglinge. Sie mussen von vaskularen Malformationen unterschieden werden, die angeboren sind und weder Wachstums- noch Regressionssphasen [7] aufweisen. Etwa 2/3 sind im Kopfbereich lokalisiert und konnen damit optisch ein Problem werden. Mit 65%–70% ist das weibliche Geschlecht uberproportional haufig betroffen. Hamangiome weisen in der Regel eine typische Entwicklung in drei Phasen auf: 1. Wachstumsphase: 0 — maximal 6 Monate 2. Stagnationsphase: 6 – 12 Monate 3. Remissionsphase: 1 – 10 Jahre (je nach Endgrose) Funktionelle Probleme konnen Hamangiome im Augenlidbereich (Amblyopie) und in der Trachea verursachen. Hier sind eventuell schnelle Interventionen erforderlich.

For airway obstruction caused by subglottic hemangiomas, tracheostomy is still regarded by some as the only established therapy, despite numerous other therapeutic options. Resection with lasers was also reported, but subglottic scar formation may occur, and different laser types may have advantages over others. The charts of 46 consecutive patients over 26 years were reviewed. Until 1986, therapy involved systemic steroids or tracheostomy. Thereafter, a Neodym-Yag and after 1995 a CO2 laser was used. Mean initial stenosis was 61.0% in the first (n=15), 85.8% in the Neodym-Yag (n=14), and 86.7% in the CO2 period (n=17). Tracheostomy rates could be reduced from 76.9% to 46.9% with the Neodym-Yag and to 30.8% with the CO2 laser, and to 22.2% in children not intubated before referral. One tracheostomy obstruction resulted in severe neurological damage; granulomas required resection in 37.5%. Secondary subglottic stenosis was found in 15.4% with the Neodym-Yag, but not with the CO2 laser. With tracheostomy, 12.5% were symptom-free at age 2-3 years, vs. 25.0% in the Neodym-Yag and 41.6% in the CO2 laser period. Speech development was delayed in 75.0% with tracheostomy, and parental anxiety lessened in only 18.8% before the second birthday (68.8% without tracheostomy). Since the end of the retrospective analysis, we treated a further 21 patients (mean stenosis, 83.3%) with the CO2 laser, with only one tracheostomy (4.8%). Compared to steroids and tracheostomy, a significant reduction in morbidity and speech developmental delay, and an improved quality of life, were achieved with CO2 laser resection, and this approach was superior to the Neodym-Yag laser.

In this report we describe an idiopathic hypertrophic cardiomyopathy in a 21 month old infant girl; who died shortly after a small surgical intervention for cardiovascular failure. Fine structural investigation disclosed an extreme increase of often abnormally structured and enlarged mitochondria and a great loss of myofibrils in the heart muscle cells. Furthermore, mitochondrial hyperplasia was observed focally in all skeletal muscles investigated. The pathogenesis of this mitochondriopathy in heart and skeletal muscle is unknown. It might be a consequence of a functional mitochondrial defect with compensatory hyperplasia of mitochondria. Differential diagnosis of this very rare infantile cardiomyopathy from the myopathies of storage diseases, typical hypertrophic cardiomyopathy and carnitine defiency is discussed, as is the distinction from the oncocytic or so-called histiocytic transformation of heart muscle cells. Methological hints for diagnostic procedures are given.