Congenital contractural arachnodactyly

Congenital contractural arachnodactyly (CCA, Beals syndrome) is a connective tissue disorder characterized by multiple flexion contractures, arachnodactyly, severe kyphoscoliosis, abnormal pinnae and muscular hypoplasia.

Encephalocele

A neural tube defect characterized by sac-like protrusions of the brain and the membranes that cover it through openings in the skull.


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(per page)
PMID (PMCID)
30579083
 MALE Child
Beals syndrome with middle and inner ear dysplasia and encephalocele: A case report and review of imaging findings.
Weidman EK, Morgenstern PF, Phillips CD, Greenfield JP, Schwartz TH, Heier LA.
Int J Pediatr Otorhinolaryngol. 2019;117:26-29.
A 10-year-old male with history of Beals syndrome presented with hearing loss and was found to have middle and inner ear dysplasia and left temporal encephalocele on imaging.
30579083
 MALE Child
Beals syndrome with middle and inner ear dysplasia and encephalocele: A case report and review of imaging findings.
Weidman EK, Morgenstern PF, Phillips CD, Greenfield JP, Schwartz TH, Heier LA.
Int J Pediatr Otorhinolaryngol. 2019;117:26-29.
Beals syndrome with middle and inner ear dysplasia and encephalocele: A case report and review of imaging findings.