Budd-Chiari syndrome refers to hepatic pathology secondary to diminished venous outflow, most commonly associated with venothrombotic disease. Clinically, patients with Budd-Chiari present with hepatomegaly, ascites, abdominal distension, and pain. On imaging, Budd-Chiari syndrome is hallmarked by occluded IVC and or hepatic veins, caudate lobe enlargement, heterogeneous liver enhancement, intr...

This article reports the largest familial aggregation of Chiari malformation in a single family to date as reported in the literature. This study is a retrospective case series of a family of whom five individuals have a confirmed case of Chiari malformation and three additional individuals have Chiari signs and symptoms. This contribution further supports the implication of genetics in the tra...

Chiari malformation describes a group of structural defects of the cerebellum, characterized by brain tissue protruding into the spinal canal. Chiari malformations are often associated with myelomeningocele, hydrocephalus, syringomyelia, and tethered cord syndrome. Although studies of etiology are few, an increasing number of specific genetic syndromes are found to be associated with Chiari mal...

Figure 1. Parasternal short-axis systolic frame at the level of the aortic valve. The Chiari network is located in the posterior right atrium. Although precise attachment sites could not be identified, real-time imaging suggested constrained motion near the interatrial septum. RA = right atrium; RV = right ventricle. pentine highly mobile reflective structure was noted in the right atrium (RA)....

Editor: In the Talal Amer and Omran M. El-Shmam review published in your journal,1 there are two types of classification for Chiari I malformation: type A, which is associated with syringomyelia and type B, which is not associated with syringomyelia. Hans Chiari (1851–1916) first reported Chiari malformation (1891) morphologically as a cerebellar tonsillar herniation with occasional fourth vent...

Chiari type I malformation (CMI) is a congenital hindbrain anomaly characterized by downward displacement of the cerebellar tonsils through the foramen magnum. Chiari type I malformation often presents with a complex clinical picture and can be sporadic or linked to a variety of genetic conditions. We report on a girl in whom Chiari type I malformation was associated with hypoglycemia, headache...

BACKGROUND Budd-Chiari syndrome, which is a rare complication of Behcet's disease, carries a high mortality rate. OBJECTIVES The aim of the study was to present our long-term follow up experience with patients suffering from Budd-Chiari syndrome due to Behcet's disease. METHODS The records of 402 patients with Behcet's disease were evaluated retrospectively. To facilitate detection of the l...