ABSTRACT
Tuberous sclerosis is a rare autosomal dominant neurocutaneous syndrome characterized by the presence of benign congenital tumors in multiple organs. The diagnosis is usually established on the basis of diagnostic criteria applied to physical or radiologic findings. Because the classical triad of epilepsy, mental retardation, and adenoma sebaceum is uncommonly seen at clinical examination, radiologic examinations can play an important role in the diagnosis of tuberous sclerosis and in treatment. Cardiac rhabdomyoma, renal angiomyolipoma, and neurologic involvement encompassing cortical or subependymal tubers and white matter abnormalities are the common radiologic findings. Detection of these entities can be strong evidence for suspecting tuberous sclerosis. The presence of pulmonary lymphangioleiomyomatosis, multifocal micronodular pneumocyte hyperplasia, or multiple renal cysts also raises suspicion of tuberous sclerosis. Moreover, tuberous sclerosis can involve bone, liver, and the alimentary tract. The clinical course and patient prognosis depend on the sites of manifestations. Familiarity with the clinical and radiologic findings in various organs is crucial in diagnosis and treatment.
Subject(s)
Multiple Organ Failure/diagnosis , Tomography, X-Ray Computed/methods , Tuberous Sclerosis/diagnosis , Adolescent , Adult , Female , Humans , Infant , Male , Viscera , Young AdultABSTRACT
A 71-year-old woman who had undergone a total abdominal hysterectomy and pelvic irradiation for cervical cancer and fecal diversion for adhesive ileus was referred to us for a "left" ureteral stone and intermittent gross hematuria. Bilateral ureteral stents had been indwelled because of lower ureteral strictures for a long time. Hematuria continued after the removal of the ureteral stone, and she once went into hypovolemic shock at the time of exchange of the right ureteral catheter. Selective arteriography revealed a "right" ueteroarterial fistula. Endovascular management alone failed to resolve the fistula, but a subsequent transurethral metal coil embolization was effective, and the hematuria was relieved. She is still free from disease at 7-month followup. As far as we know, there has been no previous report of a transurethrally managed ureteroarterial fistula.
Subject(s)
Embolization, Therapeutic/methods , Fistula/therapy , Iliac Artery , Ureteral Diseases/therapy , Urinary Fistula/therapy , Aged , Female , Follow-Up Studies , Hematuria/etiology , Hematuria/therapy , Humans , Treatment Outcome , UrethraABSTRACT
INTRODUCTION/OBJECTIVE: Various pathological changes have been attributed to leukoaraiosis. Some investigators have suggested that increase in interstitial water may partly contribute to leukoaraiosis. We hypothesized that leukoaraiosis may be attenuated by compression to the cerebral hemisphere if interstitial water may partly contribute to leukoaraiosis. We retrospectively reviewed patients with unilateral chronic subdural hematomas (CSDHs) to investigate the difference in appearance of leukoaraiosis between both cerebral hemispheres. METHODS AND MATERIAL: Leukoaraiosis on T2-weighted magnetic resonance (MR) images at the levels of the centrum semiovale and those of the frontal horns of both cerebral hemispheres in five contiguous patients with unilateral CSDHs were scored. The difference in the leukoaraiosis scores on the ipsilateral side and contralateral side of the CSDH was analyzed. RESULTS: Leukoaraiosis was less prominent on the ipsilateral side of the CSDHs than on the contralateral side of the CSDHs, both at the level of the centrum semiovale (P=0.02) and that of the frontal horns (P=0.03). DISCUSSION AND CONCLUSION: Our results support the theories that interstitial water may partly contribute to the appearance of leukoaraiosis on MR images.
