Chiari malformation is a congenital anomaly that primarily involves the downward displacement of the cerebellar tonsils through the foramen magnum and elongation of forth ventricle and lower brainstem. Patients with Chiari I (congenital or acquired) malformation are asymptomatic or may present with neurologic signs and symptoms. It is always a question of safety of neuraxial anesthesia in these...

Background Numerous studies suggest a non-chance association among Chiari, syringomyelia, and scoliosis, though the relationship remains a mystery. Studies suggest that more than sixty percent of patients receiving decompression surgery had no change (14%) or a worsening (48%) of their scoliosis symptoms. Curve reduction following Chiari treatment through the utilization of conservative scolios...

RIEGER (1935) gave the accurate and self-explanatory name "dysgenesis mesodermalis corneae et iridis" to an inherited malformation involving the angle of the anterior chamber, iris stroma, and peripheral cornea. The basic feature of this abnormality was the presence of strands of iris stroma which passed from the iris root across the angle. to the trabecular meshwork and to a congenital corneal...

A 7-year-old girl with a history of headaches and Gorham disease was surgically treated in infancy for Chiari I malformation. Subsequent investigation revealed that her cerebellar tonsillar ectopia was due to a long-standing spinal CSF-lymphatic fistula causing intracranial hypotension. Percutaneous fistula closure was performed several times, resulting in transient symptomatic improvement.

The frequencies of cleft lip with or without cleft palate (CL(P)) and isolated cleft palate (CP) have been estimated in France to be 0.082% and 0.035%, respectively, after exclusion of malformation syndromes. A genetic and epidemiological study has been carried out on 468 patients with CL(P) and 163 with CP. The results are given in detail and some specific points are discussed: the apparently ...

A case of simultaneous trigeminal and vagoglossopharyngeal neuralgia is described. No microvascular compression was seen at the trigeminal complex while the origin of vagoglossopharyngeal pain could have been due either to Chiari malformation or microvascular compression of the IX-X nerve complex. Decompressive surgery was ineffective. The patient could completely block his facial painful fits ...

BACKGROUND The Hirschsprung's disease Anorectal malformation QoL questionnaire (HAQL) is a disease-specific quality of life (QoL) questionnaire for patients with Hirschsprung's disease (HD) or anorectal malformations (ARM). It was originally proposed in Dutch and is currently being translated into other languages to obtain an internationally standardized instrument. In this work we validate a F...