Neonatal dural arteriovenous fistula at the confluence presenting with paralysis of the orbicularis oris muscle.

A male neonate presented a dural arteriovenous fistula (DAVF) at the confluence with paralysis of the orbicularis oris muscle. The interesting features in our case were the clinical symptoms (orbicularis oris muscle paralysis at birth), angioarchitecture (high-flow arteriovenous shunts at the confluence) and the size and hemodynamic flow (mid-sized venous pouch) of the fistula. Additionally, the embolization technique (i.e., occipital artery approach, closing shunts with pure glue) automatically resulted in the immediate and complete closure of accessory feeders without any additional treatment, and the midterm clinical outcome was good. We succeeded improving the symptoms of a neonate with a congenital high-flow DAVF by closing a fistula using a small amount of glue.

Inhaling gas with different CT densities allows detection of abnormalities in the lung periphery of patients with smoking-induced COPD.

STUDY OBJECTIVES: To establish a novel method allowing detection of regional abnormalities in gas distribution at the acinar level by high-resolution CT (HRCT). PARTICIPANTS: Nonsmoking control subjects (n = 28) and patients with smoking-induced COPD (n = 47). MEASUREMENTS AND RESULTS: Changes in lung CT densities were examined by HRCT while the subjects inhaled a gas mixture consisting of 21% O(2) in SF(6) or 21% O(2) in He. HRCT images of the right upper and lower lung fields were obtained at the end of inspiration and expiration of the second and 60th breaths after the start of each gas. Introducing mean lung density (MLD) and relative area with low CT attenuation (%LAA), we analyzed the differences in acinar SF(6) and He distribution in the early phase (second breath) and in the equilibrium state (60th breath). We found that the differences in inspiratory MLD between the SF(6) and He images at the 60th breath were qualitatively consistent with the differences predicted from the physical properties of these gases. However, the differences in inspiratory MLD between the SF(6) and He images taken at the second breath were smaller than those at the 60th breath, especially in the smoking group with COPD. These differences in second-breath inspiratory MLD in the smoking group were smaller in the upper lung field than in the lower lung field. The differences in MLD between the two gases were not detected at end-expiration at the time of either the second or 60th breaths. The %LAA values did not differ between the SF(6) and He images in either the nonsmoking group or the smoking group. CONCLUSIONS: SF(6)/He-associated HRCT images obtained at end-inspiration, but not at end-expiration, in the early breathing phase are useful for predicting acinar gas distribution abnormalities in patients with COPD.

A family affected by branchio-oto syndrome with EYA1 mutations.

Branchio-oto (BO) syndrome is complicated with congenital preauricular fistulae, branchial fistulae (cysts), and hearing loss (sensorineural, conductive or mixed). As well as branchio-oto-renal (BOR) syndrome. it is known to be an autosomal dominant hereditary disorder. Since mutations in the EYA1 gene have been identified in both BO and BOR syndromes, mutation screening of this gene has been drawing attention as a genetic test to diagnose BOR/BO syndromes. In this study, we genetically investigated the presence of EYA1 mutations in a BO syndrome family in which we observed congenital preauricular fistulae, branchial fistulae (cysts) and hearing loss in four generations. Whereas there was a variety of phenotype expressions in this family, all subjects tested had a nonsense mutation (R264X) in exon 8 of the EYA1 gene. The present report adds further examples to support the usefulness of molecular genetic testing for the diagnosis of patients with BO syndrome.

Inhomogeneities of ventilation and the diffusing capacity to perfusion in various chronic lung diseases.

Although impairment of gas exchange caused by ventilation-perfusion (VA/Q) mismatch has been extensively analyzed, there have been no systematic studies focused on determining the distributions of diffusion properties in dose connection with those of VA/Q. We attempted to clarify the simultaneous distributions of VA/Q and diffusion capacity to perfusion (D/Q) in patients with idiopathic pulmonary fibrosis (IPF) or chronic obstructive pulmonary disease (COPD). To assess pathologic determinants causing functional abnormalities, we compared VA/Q and D/Q distributions with the findings on high-resolution computed tomography. O2, CO2, and CO together with six foreign inert gases were used as indicator gases. We transformed the measured data on indicator gases in arterial blood into a continuous distribution of Q in the VA/Q-D/Q field. In IPF, active alveolitis or acinitis played a major role in producing low D/Q regions impeding gas exchange via a diffusion limitation, whereas extensive fibrosis with minimal inflammation accounted for low D/Q as well as low VA/Q regions. In COPD, no regions with low D/Q ratios were observed, but an abnormality in the VA/Q distribution with low or high VA/Q ratios was identified. Emphysematous lesions produced high VA/Q regions, whereas peripheral airway involvement yielded low VA/Q regions. These findings suggest that hypoxemia in patients with IPF is caused by inhomogeneous distributions of D/Q in combination with those of VA/Q. Hypoxemia in patients with COPD is attributable primarily to inhomogeneities in VA/Q rather than in D/Q distributions.

[Normal predicted values of CT indices reflect emphysematous alterations in the lung].

In order to obtain normal values and 95% confidence limits of various CT indices, healthy adult subjects with no history of smoking (n = 36) underwent CT scanning under a variety of conditions. By then applying the normal limits thus obtained to CT images of COPD patients (n = 45), we examined the sensitivity for detecting abnormal emphysematous changes in the lung fields. To measure emphysematous alterations, we used the average value of lung CT densities (ROI), the maximally appearing value in a CT histogram (Hist. Peak), the relative area with low CT densities below -910 HU (%LDA) and the total cross-sectional area (Area) in each lung section. Regardless of the section thickness (10 mm or 1 mm), the lung volume level at which the breath was held or the site from which CT images were taken (upper, middle or lower lung field), no significant correlation was observed between the CT indices associated with emphysematous changes and the subjects' age. This allowed us to define, independently of the subjects' age, normal values and 95% confidence limits for the CT indices. Among the CT indices surveyed, %LDA was found to be the most sensitive indicator for detecting emphysematous abnormalities. In so far as the extent of emphysema may be determined by lung CT density, classical CT images of 10-mm section thickness appear to have a sufficiently high sensitivity for the detection of emphysematous abnormalities, such that high-resolution CT may be unnecessary.

[Indices allowing early detection of chronic pulmonary emphysema].

To establish criteria allowing early detection of pathologically significant alterations in pulmonary emphysema caused by smoking, pulmonary-function tests and high-resolution computed tomography were done in 104 subjects categorized into three groups: nonsmoking healthy adults, smokers with a normal FEV1%, and smokers with a low FEV1% (cross-sectional analysis). Fifty-six of the 104 patients underwent pulmonary-function testing and high-resdution computed fomography once per year for 3 years (longitudinal analysis). Cross-sectional and longitudinal analyses showed that abnormalities in functional residual capacity, in single-breath diffusing capacity for carbon monoxide, and in the average tomographic density of sections in the lower lung fields obtained after a deep inspiration could be used to predict whether the disease would reach an advanced stage, even if the patients had no significant symptoms at the time of testing. Relative areas of low-attenuation regions, which were alleged to directly reflect the size of emphysematous areas, appear not to be useful for early detection of pathological emphysema.

[Abnormalitis in the distributions of ventilation-perfusion ratios and diffusing capacity-perfusion ratios in three types of chronic obstructive pulmonary disease].

To assess whether diffusion-limited gas exchange plays a significant role in hypoxemia in various types of chronic obstructive pulmonary disease(COPD), we analyzed the distribution of ventilation-perfusion (VA/Q) ratios and of diffusing capacity-perfusion (G/Q) ratios. We compared VA/Q and G/Q distribution in patients with three basic types of COPD: emphysematous changes, bronchiolar involvement, and airway hypersecretion, which were classified based on symptoms and on findings of high-resolution CT. The results were that 1) hypoxemia was not caused by diffusion-limited gas exchange with low G/Q regions in any type of COPD, that 2) hypoxemia was not caused by inhomogeneities in VA/Q distribution, that 3) emphysematous changes and bronchiolar involvement were associated with high and low VA/Q regions, respectively, and that 4) either hypersecretion itself or related airway abnormalities may cause low VA/Q regions to form.

[Diagnosis of sinusitis in children with allergic diseases--comparison of waters projection radiographs and CT scans].

Sinusitis is common in children with allergic diseases, and the relationship between sinusitis and reactive airway diseases involving asthma has been reported. Most pediatricians and physicians base their diagnosis of sinusitis on findings from plain radiographs of the sinuses, especially Waters projection radiographs. We compared the diagnoses made by 11 pediatric allergists using 56 Waters projection radiographs with transverse CT findings. The ratio for the two diagnosis being consistent (normal plain radiographic findings and normal CT findings, or abnormal plain radiographic findings and abnormal CT findings) was approximately 60%. Sinusitis in children is often misdiagnosed on the basis of findings from Waters projection radiographs alone. Therefore, the use of CT findings for the diagnosis of sinusitis together with Waters projection radiographs is recommended.

Enhancement of topoisomerase I-mediated unwinding of supercoiled DNA by the radioprotector WR-33278.

The radioprotector WR-33278, the disulfide of WR-1065 (N-(2-mercaptoethyl)-1,3-diaminopropane), is shown to stimulate eukaryotic topoisomerase I unwinding of negatively supercoiled DNA. This observation suggests the possibility that some protection may be conferred to DNA either by a decrease in its supercoiled state or by altering directly other enzymatic processes. This is the first report of a radioprotective compound stimulating an enzyme involved in DNA structure and synthesis.

[3 cases of ectopic ureteral orifice terminating in the seminal vesicle; efficacy of CT].

We present three cases of ectopic ureteral orifice terminating in the seminal vesicle. The enlarged seminal vesicle, ipsilateral renal agenesis or hypoplasia and the absence of normal ureteral orifice are the triad of CT findings in this disease.

The definition of vascular skin territories with prostaglandin E1--the anterior chest, abdomen and thigh-inguinal region.

The vascular skin territories over the anterior chest, the abdomen and thigh-inguinal region in man have been defined in vivo using selective angiography and the intra-arterial injection of Prostaglandin E1. With this method it was clear that the vascular skin territories represented the area of axial patterns. The technique had significant advantages over other conventional methods of definition of skin vascular territories. The survival area of axial pattern flaps was often far greater than the precise vascular skin territory of its axial vessels and the extent of this phenomenon appeared to depend mainly on the vascular pattern of the adjoining territories.

The definition of cutaneous vascular territories over the back using selective angiography and the intra-arterial injection of prostaglandin E1: some observations on the use of the lower trapezius myocutaneous flap.

The lower trapezius myocutaneous flap based on the descending branch of the transverse cervical artery is particularly useful in the repair of defects over the back, shoulder and scalp region. Three illustrative examples are given of the use of this particular flap. The precise definition of the vascular territory in the overlying skin can be shown by selective angiography and intra-arterial injection of PGE 1. An axial vascular territorial map has been constructed to show the anatomical distribution of the various dominant areas that supply the skin over the back.