Limited dorsal myeloschisis: a distinctive clinicopathological entity.
BACKGROUND
Limited dorsal myeloschisis (LDM) is a distinctive form of spinal dysraphism characterized by 2 constant features: a focal "closed" midline defect and a fibroneural stalk that links the skin lesion to the underlying cord. The embryogenesis is hypothesized to be incomplete disjunction between cutaneous and neural ectoderms, thus preventing complete midline skin closure and allowing persistence of a physical link (fibroneural stalk) between the disjunction site and the dorsal neural tube.


OBJECTIVE
To illustrate these features in 51 LDM patients.


METHODS
All patients were studied with magnetic resonance imaging or computed tomography myelography, operated on, and followed for a mean of 7.4 years.


RESULTS
There were 10 cervical, 13 thoracic, 6 thoracolumbar and 22 lumbar lesions. Two main types of skin lesion were saccular (21 patients), consisting of a skin-base cerebrospinal fluid sac topped with a squamous epithelial dome, and nonsaccular (30 patients), with a flat or sunken squamous epithelial crater or pit. The internal structure of a saccular LDM could be a basal neural nodule, a stalk that inserts on the dome, or a segmental myelocystocele. In nonsaccular LDMs, the fibroneural stalk has variable thickness and complexity. In all LDMs, the fibroneural stalk was tethering the cord. Twenty-nine patients had neurological deficits. There was a positive correlation between neurological grade and age, suggesting progression with chronicity. Treatment consisted of detaching the stalk from the cord. Most patients improved or remained stable.


CONCLUSIONS
LDM is a distinctive clinicopathological entity and a tethering lesion with characteristic external and internal features. We propose a new classification incorporating both saccular and flat lesions.
Department of Pediatric Neurosurgery, University of California, Davis, Davis, California, USA. PangTV@aol.com
Dachling
D
Pang
John
J
Zovickian
Angelica
A
Oviedo
Greg S
GS
Moes
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology; D009436:Neural Tube Defects / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D009460:Neurologic Examination; D012189:Retrospective Studies; D012871:Skin Diseases / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D016135:Spinal Dysraphism / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome; D055815:Young Adult