We describe a 38 year-old woman with polycythemia vera who presented with fulminant hepatic failure due to acute Budd-Chiari syndrome. She had a history of abdominal pain and distention for 4 days. Laboratory and clinical findings showed fulminant hepatic failure due to acute Budd-Chiari syndrome. Diagnosis was confirmed with abdominal ultrasonography and doppler ultrasonography showing ascites...

Myelomeningocele (MMC) is the most frequent congenital defect of the central nervous system for which there is no satisfactory alternative to postnatal treatment. On the contrary prenatal MMC surgery is conducting before birth and is aimed at protecting from Chiari II malformation. The main goal of fetal MMC repair is to improve development and life quality of children with Chiari II malformati...

Type I Chiari malformations consist of herniation of the cerebellar tonsils through the foremen magnum and present with a variety of symptoms and signs described previously but hemiathrophy of extremities and hypothalamic hypogonadism are not characteristic findings. Hemiathrophy of extremities has been reported in just one case, and to our knowledge hypothalamic hypogonadism has not been repor...

The etiology of vocal cord paralysis is highly variable. Neurological, birth trauma, surgical procedure and idiopathic are notable causes. Clinical findings are different according to type of paralysis. Vocal cord paralysis can be seen unilateral or bilateral. Bilateral vocal cord paralysis are more severe clinical findings than according to unilateral paralysis. We report a 9-month child. He w...

SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decomp...

Sleep apnea is a rare but well-known clinical feature of disorders of the craniocervical junction. It may be obstructive or central in nature, and rarely presents without other neurological symptoms. We report the cases of two children, presenting with isolated central sleep apnea leading to a diagnosis of type I Chiari malformation. Surgical treatment resulted in a dramatic improvement in resp...

In a patient with the Wolff-Parkinson-White syndrome, Ebstein's anomaly of the tricuspid valve, a right atrial Chiari net and a patent foramen ovale two unusual complications developed after surgical epicardial dissection combined with cryoablation of the anomalous pathway. The first complication was that ablation of the right atrial wall led to changes in interatrial pressure gradients and the...

The report concerns a 21-year-old woman who, after having taken oral contraceptives for three months, developed the Budd-Chiari syndrome as the results of a widespread chronic obliterative process involving the intrahepatic venous system. The diagnosis was established by liver biopsy. She was treated with supportive measures and completely recovered after 28 days of hospitalization. The literat...