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Background-In recent studies, coma, cerebral hemorrhage, older age, and infectious origin have been identified as prognostic factors in cerebral venous thrombosis (CVT). However, no studies of the prognosis of CVT have evaluated hemodynamic factors. However, it is conceivable that the presence or absence and the efficiency of venous collaterals, as well as recanalization, may have an impact on brain tissue damage and hence on the prognosis of acute CVT. Methods-Twenty-six patients with acute CVT (mean age, 40Ϯ15 years) were recruited prospectively. All patients were treated with intravenous heparin, followed by oral anticoagulation for 12 months, except for 2 patients who were lost to follow-up after hospital discharge. Neurological deficits were graded on the National Institute of Health Stroke Scale on admission, at hospital discharge, and at 90Ϯ14 days after admission. The functional clinical outcome was graded on the modified Rankin Scale on day 90 after admission. All patients received a venous transcranial duplex sonography (TCCS) on admission and were followed up in case of a pathological result until normalization was recorded (mean follow-up, 316Ϯ395 days; range, 13 to 1180 days). Results-Initial TCCS was pathological in 18 of 26 patients (69%). Four distinct venous drainage types were identified: increased drainage to the cavernous sinus and to the deep cerebral veins, flow reversal in the basal veins, and either compensatory increased or reversed flow in the transverse sinus. Initially normal venous TCCS or normalized TCCS within 90 days was significantly related to favorable outcome. Conclusions-TCCS can be used to evaluate venous drainage patterns in acute CVT. Furthermore, initially normal and normalization of initially pathological venous TCCS within 90 days is related to a favorable outcome in this disease.

IntroductionSevere traumatic brain injury (TBI) (Glasgow Coma Scale < 8) occurs in 60% of polytraumatized children after car accidents or child abuse, and it is associated with a high mortality and morbidity [1,2]. The primary therapeutic aim is to maintain an adequate cerebral blood flow (estimated from cerebral perfusion pressure) and brain oxygenation. Intensive care management of severe head injury in cases of refractory R133 CBF = cerebral blood flow; CEO 2 = cerebral extraction rate for oxygen; CT = computed tomography; ICP = intracranial pressure; SEP = somatosensory evoked potentials; TBI = traumatic brain injury. AbstractIntroduction Severe traumatic brain injury (TBI) in childhood is associated with a high mortality and morbidity. Decompressive craniectomy has regained therapeutic interest during past years; however, treatment guidelines consider it a last resort treatment strategy for use only after failure of conservative therapy. Patients We report on the clinical course of six children treated with decompressive craniectomy after TBI at a pediatric intensive care unit. The standard protocol of intensive care treatment included continuous intracranial pressure (ICP) monitoring, sedation and muscle relaxation, normothermia, mild hyperventilation and catecholamines to maintain an adequate cerebral perfusion pressure. Decompressive craniectomy including dura opening was initiated in cases of a sustained increase in ICP > 20 mmHg for > 30 min despite maximally intensified conservative therapy (optimized sedation and ventilation, barbiturates or mannitol). Results In all cases, the ICP normalized immediately after craniectomy. At discharge, three children were without disability, two children had a mild arm-focused hemiparesis (one with a verbal impairment), and one child had a spastic hemiparesis and verbal impairment. This spastic hemiparesis improved within 6 months follow-up (no motor deficit, increased muscle tone), and all others remained unchanged. Conclusion These observational pilot data indicate feasibility and efficacy of decompressive craniectomy in malignant ICP rise secondary to TBI. Further controlled trials are necessary to evaluate the indication and standardization of early decompressive craniectomy as a 'second tier' standard therapy in pediatric severe head injury.

This study confirms that endosonography is highly effective in staging laryngeal cancer. Therefore, the latter may constitute a complementary diagnostic tool in these tumors and additionally may assist in choosing an adequate treatment.

X-linked lissencephaly with abnormal genitalia (XLAG) is a rare disorder caused by mutations in the aristaless-related homeobox (ARX) gene. We report on the clinical data of a boy with a 1-bp deletion (790 delC) resulting in a frame shift in the ARX gene and prolonged survival until age 18 months. Similar to other patients, the boy showed postnatal microcephaly, hypothalamic dysfunction, intractable neonatal seizures, and chronic diarrhoea. In addition, he suffered from exocrine pancreatic insufficiency and renal phosphate wasting became apparent from age 5 months, both of which have not been described previously in XLAG. This allows us to speculate that the phenotype of XLAG is more complex than hitherto known and may include renal phosphate wasting which might not have been observed in other patients due to early death.