Congenital cervicothoracic dissociation: report of two cases.

PURPOSE: Congenital cervicothoracic dissociation (CCTD) of the spine is a rare condition while having major impacts on stability and neurological function. Surgical treatment includes decompression and instrumented fusion. Only few cases of CCTD have been reported in children. This report intends to demonstrate the complexity of this condition and its surgical management options based on two cases. METHODS: Retrospective illustration of two cases with CCTD treated with instrumented occipito-thoracic fusion. Timing and options of surgical management are discussed. RESULTS: Two patients aged 9 and 12 were treated. Patient 1 presented with asymmetric chronic weakness of the upper extremity and unstable neurogenic bladder. Imaging showed a CCTD with severe distortion of the posterior facets and a dysplastic spinal cord. Patient 2 presented with mild spasticity and unilateral weakness of the upper extremity. A low energy trauma resulted in tetraplegia by increasing the amount of antero-posterior displacement. Both patients were treated with Halo vest application, followed by posterior decompression, and instrumented occipito-thoracic fusion. Additional upfront Halo traction was applied in patient 2 for distraction and sagittal alignment improvement. The last follow-up confirmed solid fusion and minor residual neurological impairment in both patients at 5, and 6 years, respectively. CONCLUSION: CCTD is a rare congenital condition, which can be successfully managed by posterior instrumented fusion. A sudden cervicothoracic displacement with neurological deterioration may respond to careful Halo traction with preoperative sagittal alignment correction. Timing of surgery depends on the actual instability in neurologically stable patients.

Effect of a multinutrient intervention after ischemic stroke in female C57Bl/6 mice.

Stroke can affect females very differently from males, and therefore preclinical research on underlying mechanisms and the effects of interventions should not be restricted to male subjects, and treatment strategies for stroke should be tailored to benefit both sexes. Previously, we demonstrated that a multinutrient intervention (Fortasyn) improved impairments after ischemic stroke induction in male C57Bl/6 mice, but the therapeutic potential of this dietary treatment remained to be investigated in females. We now induced a transient middle cerebral artery occlusion (tMCAo) in C57Bl/6 female mice and immediately after surgery switched to either Fortasyn or an isocaloric Control diet. The stroke females performed several behavioral and motor tasks before and after tMCAo and were scanned in an 11.7 Tesla magnetic resonance imaging (MRI) scanner to assess brain perfusion, integrity, and functional connectivity. To assess brain plasticity, inflammation, and vascular integrity, immunohistochemistry was performed after killing of the mice. We found that the multinutrient intervention had diverse effects on the stroke-induced impairments in females. Similar to previous observations in male stroke mice, brain integrity, sensorimotor integration and neurogenesis benefitted from Fortasyn, but impairments in activity and motor skills were not improved in female stroke mice. Overall, Fortasyn effects in the female stroke mice seem more modest in comparison to previously investigated male stroke mice. We suggest that with further optimization of treatment protocols more information on the efficacy of specific interventions in stroked females can be gathered. This in turn will help with the development of (gender-specific) treatment regimens for cerebrovascular diseases such as stroke. This article is part of the Special Issue "Vascular Dementia".

Fog as a fresh-water resource: overview and perspectives.

The collection of fog water is a simple and sustainable technology to obtain fresh water for afforestation, gardening, and as a drinking water source for human and animal consumption. In regions where fresh water is sparse and fog frequently occurs, it is feasible to set up a passive mesh system for fog water collection. The mesh is directly exposed to the atmosphere, and the foggy air is pushed through the mesh by the wind. Fog droplets are deposited on the mesh, combine to form larger droplets, and run down passing into a storage tank. Fog water collection rates vary dramatically from site to site but yearly averages from 3 to 10 l m(-2) of mesh per day are typical of operational projects. The scope of this article is to review fog collection projects worldwide, to analyze factors of success, and to evaluate the prospects of this technology.

Termination of atrial flutter by directed transesophageal atrial pacing during transesophageal echocardiography.

INTRODUCTION: The purpose of this study was to evaluate termination of atrial flutter (AFL) by directed rapid transesophageal atrial pacing (TAP) with and without simultaneous transesophageal echocardiography (TEE) performed using a novel TEE tube electrode. MATERIALS AND METHODS, AND RESULTS: A total of 16 AFL patients (age 63+/-12 years; 13 males) with mean AFL cycle length of 224+/-24 ms (n=12) and mean ventricular cycle length of 448+/-47 ms (n=12) were analyzed using either an esophageal TO electrode (n=10) or a novel TEE tube electrode consisting of a tube with four hemispherical electrodes that is pulled over the echo probe (n=6). AFL could be terminated by directed rapid TAP using an esophageal TO electrode, leading to induction of atrial fibrillation (AF) (n=6), induction of AF and spontaneous conversion to sinus rhythm (SR) (n=3), and with conversion to SR (n=1). AFL could also be terminated by directed rapid TAP using the TEE tube electrode, with induction of AF (n=3) or induction of AF and spontaneous conversion to SR (n=3). CONCLUSION: AFL can be terminated by directed rapid TAP with hemispherical electrodes with and without simultaneous TEE. TAP with the directed TEE tube electrode is a safe, simple, and useful method for terminating AFL.

Genetic and phenotypic analysis of dilated cardiomyopathy with conduction system disease: demand for strategies in the management of presymptomatic lamin A/C mutant carriers.

BACKGROUND: One-third of cases of dilated cardiomyopathy (DCM) is of familial aetiology. Several genes have been reported to cause the autosomal dominant form of DCM. AIMS: To analyze the lamin A/C gene (LMNA) in 31 unrelated patients with DCM and conduction system disease (CSD). METHODS: Patients and family members underwent physical examination, ECG/Holter-ECG, echocardiography, and selective coronary angiography. Genetic analysis of all coding exons of LMNA was performed using PCR and sequencing. RESULTS: Three different LMNA mutations (Arg377His, c.1397delA, c.424_425ins21nt) were identified in three families with autosomal dominant disease comprised of 39 individuals. 21 individuals were mutation carriers, of whom 12 were symptomatic. We observed a progressive and age-dependent form of DCM with CSD and arrhythmias. First, the patients developed a moderate left ventricular dilatation without symptoms. Later, systolic function declined progressively and the patients became symptomatic resulting in a high mortality due to sudden death and heart failure. CONCLUSIONS: Genetic screening leads to the identification of symptomatic and asymptomatic mutant carriers. The latter at a young age should be regarded as "presymptomatic" because of the age-dependent disease manifestation. New guidelines are required for the management of these individuals.