We report a rare case of progressive myelopathy caused by intracranial dural arteriovenous fistula with venous drainage into the spinal perimedullary veins. A 45-yr-old man developed urinary and fecal incontinence and muscle weakness in the lower limbs. Magnetic resonance imaging revealed brainstem edema and dilated veins of the brainstem and spinal cord. Cerebral angiography showed a dural arteriovenous fistula fed by the neuromeningeal branch of the left ascending pharyngeal artery. Occlusion of the fistula could be achieved by embolization after a diagnostic and subsequent therapeutic delay. There was no improvement in clinical condition. For the neurologic outcome of these patients it is important that fistula must be treated before ischemic and gliotic changes become irreversible.
Spinal Cord Diseases/*etiology ; Middle Aged ; Male ; Magnetic Resonance Imaging ; Humans ; Dura Mater/*blood supply ; Arteriovenous Fistula/*complications/diagnosis

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.
Adolescent ; Anoxia ; Arteriovenous Fistula/etiology ; Biliary Atresia/*diagnosis/etiology ; Cyanosis/complications ; Dyspnea/complications ; Echocardiography, Transesophageal ; End Stage Liver Disease/complications/*surgery ; Female ; Hepatic Artery/abnormalities ; Hepatopulmonary Syndrome/*diagnosis/ultrasonography ; Humans ; *Liver Transplantation ; Osteoarthropathy, Secondary Hypertrophic/complications

With the increased temporal resolution available in dynamic computed tomography (CT) and magnetic resonance imaging (MRI), hepatic arterioportal shunts are now more frequently encountered than in the past. The condition occurs in various hepatic diseases in which portal or hepatic venous flow is compromised. The underlying mechanism and the degree of shunt affect its appearance at dynamic imaging. The dynamic CT and MRI findings have been summarized as early enhancement of peripheral portal veins, and wedge-shaped transient parenchymal enhancement during the hepatic arterial phase. Recognition of arterioportal shunt can suggest the presence of a previously unsuspected disorder and avoids false-positive diagnosis or overestimation of a hepatic disease. Familiarity with the pathophysiology of arterioportal shunt also allows investigation of the hepatic hemodynamic changes occurring in various hepatic diseases.
Arteriovenous Fistula/*diagnosis/etiology/physiopathology ; Carcinoma, Hepatocellular/complications ; Chemoembolization, Therapeutic/adverse effects ; *Hepatic Artery ; Human ; Liver Circulation/physiology ; Liver Diseases/complications ; Liver Neoplasms/complications ; *Magnetic Resonance Imaging ; Portal System/physiology ; *Portal Vein ; *Tomography, X-Ray Computed

Arteriovenous Fistula ; complications ; diagnosis ; diagnostic imaging ; Child ; Contrast Media ; Coronary Angiography ; Coronary Vessels ; pathology ; Female ; Humans ; Superior Vena Cava Syndrome ; diagnosis ; diagnostic imaging ; etiology ; Tomography, X-Ray Computed ; Vena Cava, Superior ; abnormalities ; diagnostic imaging

This study was designed to identify the causes of the development of carpal tunnel syndrome (CTS) associated with end stage kidney disease (ESKD). A total of 112 patients with ESKD, 64 on hemodialysis (HD) and 48 on peritoneal dialysis (PD), were enrolled. The duration of ESKD and dialysis, the site of the arteriovenous (A-V) fistula for HD, laboratory data such as blood urea nitrogen, creatinine, and beta-2-microglobulin were determined. Clinical evaluation of CTS and electrophysiological studies for the diagnosis of CTS and peripheral neuropathy were performed. The electrophysiological studies showed that the frequency of CTS was not different in the HD and PD groups (P = 0.823) and the frequency of CTS was not different in the limb with the A-V fistula compared to the contralateral limb (P = 0.816). The frequency of HD and PD were not related to beta-2-microglobulin levels, an indicator of amyloidosis. The frequency of CTS did not increase as the severity of the peripheral neuropathy and the duration of ESKD and dialysis increased (P = 0.307). The results of this study do not support that microglobulin induced amyloidosis or placement of an A-V fistula are associated with an increase in CTS.
Adult ; Aged ; Amyloidosis/complications ; Arteriovenous Fistula/complications ; Blood Urea Nitrogen ; Carpal Tunnel Syndrome/*complications/*diagnosis ; Creatinine/blood ; Electrophysiological Phenomena ; Female ; Humans ; Kidney Failure, Chronic/*complications/*therapy ; Male ; Middle Aged ; Peritoneal Dialysis/adverse effects ; Polyneuropathies/*complications/*diagnosis ; Renal Dialysis/adverse effects ; beta 2-Microglobulin/blood