[Management and outcome of vein of galen malformation in childhood]. / Management und Outcome der Vena-Galeni-Malformation im Kindesalter.

The vein of Galen malformation (VGM) is a rare congenital cerebral vascular malformation. In most cases it already has been diagnosed prenatally. The impact of heart failure due to the arteriovenous shunt is relevant for the prognosis.Prognosis of neonatal symptomatic VGM with early heart failure is severe. Therefore an interdisciplinary cooperation is necessary and it is important to treat patients in a clinic with a perinatal center and a neuroradiology and neurosurgery that is experienced in treating such patients. The genesis, the pathophysiology, the diagnostic agents, the therapeutic agents and the prognosis of VGM will be described.

[Evaluation of syncope in children with tilt table test]. / Synkopenabklärung bei Kindern mittels Kipptischuntersuchung.

Tilt table testing is a well approached examination for differentiating unexplained syncope and control trial after therapy. It is evaluated at children and is fast and reliable in spite of high personality effort. We present the indication of examination, course of examination, additional procedures like transcranial doppler sonography, reaction models during the tilt table examination and treatment features for neurocardiogenic syncope.

[Value of transcranial Doppler sonography during tilt table test in children]. / Wertigkeit der transkraniellen Dopplersonographie in der Kipptischuntersuchung bei Kindern.

AIM: Transcranial Doppler sonography was used to asses time relation and characteristically changes in middle cerebral artery blood flow velocity before syncope during tilt table test in children. METHOD: We performed tilt table tests in 40 pediatric patients with a medical history for syncope after exclusion of neurologic and cardiologic diseases. Examination was done in a 70 degrees postural position under control of electrocardiogram, blood pressure measurement and measurements of blood flow velocity of middle cerebral artery by transcranial Doppler with 3-MHz probe. RESULTS: In 13 children syncope occurred after 5 to 45 min (average 23.3). 10 of them were neurocardiogenic syncopes. With it diastolic and medium blood flow velocity of middle cerebral artery initial ever increased while resistance and pulsatility index decreased. Just before syncope the diastolic blood flow velocity decreased dramatically thus no diastolic blood flow could be measured, respectively. Almost we could measure no diastolic blood flow just before syncope occurred. CONCLUSION: Patients with neurocardiogenic syncope do have characteristically blood flow velocity changes of middle cerebral artery just before loosing consciousness. Transcranial Doppler is a usefully examination during tilt table. Clear transcranial Doppler results can help to prevent children from the impending syncope.

Nephrotic syndrome, hypertension, and adrenal failure in atypical Cockayne syndrome.

This report describes a boy with an atypical severe from of Cockayne syndrome type II manifesting in infancy. He developed nephrotic syndrome at the age of 4.7 years and a hypertensive crisis with hemiparesis at 5.4 years. Renal biopsy revealed focal segmental glomerulosclerosis, which was confirmed at autopsy. Adrenocortical failure was also present. The course was characterized by frequent infections and an episode of myocarditis. The boy died at the age of 6.0 years after rapid neurological deterioration accompanied by renal insufficiency. Autopsy disclosed cerebral leukodystrophy compatible with Cockayne syndrome.

[High frequency oscillation in meconium aspiration and bronchopulmonary dysplasia]. / Hochfrequenzoszillation (HFO) bei Mekoniumaspiration und bei Bronchopulmonaler Dysplasie.

Within one year 3 newborns with meconium-aspiration and 4 infants with bronchopulmonary dysplasia (BPD) were treated with HFP, synchronous with conventional ventilation (CMV). The entrance criteria were insufficient oxygenation (PO2/FiO2 < 50 mmHg) and/or CO2-elimination (> 60 mmHg), respectively peak inspiratory pressure Pi > 40 mmHg and mean airway pressure MAP > 20 mbar during CMV. All three cases of meconium-aspiration have shown a striking improvement in oxygenation and ventilation, in one case starting from a disastrous situation with PCO2 > 90 mmHg, PO2 30 mmHg (FiO2 100%). After a HFO period of 9 to 10 hours Pi, MAP and CMV-frequency could be reduced. The patients could be extubated after 1-2 weeks. In severe BPD only in one case continuous improvement and extubation in the 4. week of life were possible. Here the pulmonary artery pressure in doppler-echocardiography slightly was elevated (30-35 mmHg). In a further case extubation was possible after several trials with HFO. Indeed chronic respiratory insufficiency, progredient pulmonary emphysema on x-ray and clearly elevated pulmonary artery pressure (> 40 mmHg) persisted. In 2 further cases there was no longstanding improvement of ventilation. One child died after 8 months, one after 6 months. In both cases there was a right-to-left shunt over foramen ovale and pulmonary artery pressures at systemic level. HFO led to an improvement in oxygenation and ventilation in all three cases of meconium-aspiration and probably prevented a fatal outcome in one case. The effect seems to depend on improved secretolysis and gas exchange.(ABSTRACT TRUNCATED AT 250 WORDS)