Confluens-sinuum-Obstruktion durch Langerhanszell-Histiozytose

Die Langerhanszell-Histiozytose, bis vor wenigen Jahren als Histiozytose X bezeichnet, umfast 3 verschiedene Syndrome, namlich das eosinophile Granulom, die Hand-Schuller-Christian-Krankheit sowie die Abt-Letterer-Siwe-Krankheit, und gehort zu den seltenen Erkrankungen des Kindesalters. Wir berichten uber einen 10 jahrigen Jungen, der mit einer Schwellung am Hinterkopf und beginnender Hirndrucksymptomatik zur stationaren Aufnahme kam. Klinik, konventionelle radiologische Diagnostik und Computertomographie liesen differentialdiagnostisch ein eosinophiles Granulom als wahrscheinlich erscheinen. MRT und MRT-Angiographie zeigten jedoch die akute Bedrohung des Patienten durch das intrakraniale W…

Survivors of childhood cancer for more than twenty years.

Present health status, complications, and development of long-term survivors of childhood cancer followed for more than 20 years in a single institution were reviewed. The departmental database was searched to identify patients diagnosed with childhood cancer and consequently treated between 1965 and 1978. A total of 124 (77%) long-term survivors participated on a voluntary basis in the study. A semi-standardized interview consisted of measures evaluating the present health condition, sequelae of treatment, second malignancies, intellectual development and presence of offspring of the former patients. The majority of patients were treated with chemotherapy (82%). 67% received radiotherapy a…

Male fertility in long-term survivors of childhood ALL.

A study of fertility was conducted in postpubertal male patients who had been treated for acute lymphoblastic leukemia (ALL) during childhood or adolescence between 1970 and 1980. Thirteen men (age 18 to 35 years) participated on a volunteer basis. Their age at diagnosis was between 2 and 15 years. Therapy followed the protocol ''Memphis VII (Pinkel)." Interview, physical examination, andrological studies (ejaculate), and hormone status (luteinizing hormone, follicle-stimulating hormone, and testosterone) were performed at least 5 years after completion of therapy. No normozoospermia was achieved; 10 patients were identified with asthenozoospermia and 3 patients with azoospermia. With respe…

Fatal Late Vitamin K-Deficiency Bleeding After Oral Vitamin K Prophylaxis Secondary to Unrecognized Bile Duct Paucity

Paediatric urological malignancies

Verschluß einer Gaumenspalte bei einer Patientin mit Ebstein-Anomalie unter Hochfrequenzoszillationsbeatmung

Hintergrund: Es wird uber den zunachst nicht erfolgreichen Versuch der konventionellen Beatmung wahrend eines kieferchirurgischen Eingriffs bei einer Patientin mit Ebstein-Anomalie berichtet. Verminderter pulmonaler Blutflus und entsprechend verminderter linksventrikularer Auswurf fuhrten zu einer arteriellen Hypotonie mit Sauerstoffsattigungsabfall. Unter Spontanatmung normalisierte sich der kritische Zustand rasch. Operation: Der Einsatz eines Hochfrequenzoszillationsbeatmungsgerats (SensorMedics 3100A) mit gleichbleibendem mittlerem Atemwegsdruck und Vermeiden inspiratorischer Spitzendrucke ermoglichte 2 Jahre spater eine adaquate Ventilation und Oxygenierung der Patientin, so das die Op…

Cerebral spinal fluid flow, venous drainage and spinal cord compression in achondroplastic children: impact of magnetic resonance findings for decompressive surgery at the cranio-cervical junction

In order to investigate the diagnostic properties of MRI of the brain and spine in achondroplastic children with regard to decompressive surgery, 25 patients were examined by conventional morphological and by "functional" imaging of CSF flow and magnetic resonance angiography (MRA) of the veins and sinuses at the cranial base following a special protocol. The results were compared to those from age-matched controls and were correlated with each other and retrospectively with the neurological findings. Measurements of distances and angulations at the cranio-cervical junction (CCJ) from MR scans showed similar values to those from conventional radiographs and CTs and thus can be used without …

Clinical aspects and prognosis of ependymoma in infants and children

Thirty-two patients (22 boys and 10 girls) with a histologically confirmed diagnosis of ependymoma were treated between 1972 and 1999. A total macroscopic resection was achieved in 16 of these patients, whereas 15 resections were classified by the surgeon as subtotal. In 1 patient a ventriculostomy was created as part of a palliative strategy. All children over 3 years old were treated with postoperative radiotherapy. Chemotherapy consisted of procarbazine, ifosfamide, etoposide, methotrexate, cisplatin and cytosine arabinoside. There was 1 perioperative death. Twenty children developed a relapse of disease within 2 months to 13 years and 1 month after the initial therapy. A maximal number …

Orbital rhabdomyosarcoma in childhood

Background: Rhabdomyosarcoma is the most common malignant orbital tumor in children. Treatment modalities (individualized therapy or study protocol) have been changed radically. Surgery was supplemented by radiation therapy and chemotherapy. The objective of our retrospective analysis was to define the prognosis in correlation to changes of treatment in an unselected patient group of a single institution. Patients and methods: Between 1954 and 1995, 18 patients (age at presentation 1 month to 17 years, 11 male and 7 females) with orbital rhabdomyosarcoma were diagnosed and treated at our institution. Results: The 5-year survival rate was 76 %. Primary exenteration was replaced by tumor rese…