Constriction rings syndrome

Constriction rings syndrome is a congenital limb malformation disorder with an extremely variable clinical presentation characterized by the presence of partial to complete, congenital, fibrous, circumferential, constriction bands/rings on any part of the body, although a particular predilection for the upper or lower extremities is seen. Phenotypes range from only a mild skin indentation to complete amputation of parts of the fetus (e.g. digits, distal limb). Compression from the rings may lead to edema, skeletal anomalies (e.g. fractures, foot deformities) and, infrequently, neural compromise.

Tethered cord

During normal embryological development, the spinal cord first occupies the entire length of the vertebral column but goes on to assume a position at the level of L1 due to differential growth of the conus medullaris and the vertebral column. The filum terminale is a slender, threadlike structure that remains after the normal regression of the distal embryonic spinal cord and attaches the spinal cord to the coccyx. A tethered cord results if there is a thickened rope-like filum terminale which anchors the cord at the level of L2 or below, potentially causing neurologic signs owing to abnormal tension on the spinal cord.


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PMID (PMCID)
11423777
MALE Infant, Newborn
Open thoracic meningocele associated with amniotic band syndrome.
Jackson T.
Pediatr Neurosurg. 2001;34(5):252-4.
Although there have been several case reports of encephalocele, anencephaly, closed meningocele and a case of tethered cord associated with amniotic band syndrome (ABS), there has yet to be a report of an open meningocele associated with ABS.