| Background: Occult spinal dysraphism (OSD) represents a spectrum
of congenital anomalies that are characterized by skincovered
lesions without exposed neural tissue, which can cause
rostrocaudal traction on the spinal cord. Prophylactic surgery has
a high likelihood of changing the natural history in which a
gradual loss of function may be replaced by clinical stability.
Detection of OSD in infants is difficult because an abnormal
neurological examination is often not apparent until the child
becomes ambulatory, or even later. Cutaneous signs are often the
initial markers of congenital spine abnormalities and investigation
of skin lesions in these patients may lead to earlier diagnosis of
OSD. This study was conducted to investigate the frequency and
type of cutaneous stigmata in different forms of OSD.
Methods: In a prospective study, 39 patients with OSD were referred
to our hospital from August 23, 2001 to March 20, 2010.
Results: A total of 39 patients, 15 (38.5%) males and 24 (61.5%)
females, with the age range of 4 months to 41 years (mean=
12.8 y) were included in this study. Skin stigmata were present in
66.66% of patients and most frequent findings were hypertrichosis
followed by lipoma.
Conclusions: The discovery of a midline skin lesion in an
otherwise well, asymptomatic neonate or child often prompts
a search for OSD using imaging modalities. Cutaneous markers
in a high percentage accompany spinal malformations. They
can aid the clinician in further diagnostic and therapeutic work.
The knowledge of these skin lesions can guide the clinician to
underlying spinal pathology.
Key Words: spinal dysraphisms, occult spinal dysraphism, skin
lesions
(Neurosurg Q 2014;24:63–66)
Raphe is a line in which fusion between symmetrical
embryonic structures occurs in midline. Lack of the
mentioned fusion would lead to dysraphism.1 Spinal
dysraphism is a term referring to all kinds of developing
disorders in back midline, from the outer skin to the
vertebrae and the spinal cord.2 Spinal dysraphism consists
of a spectrum of congenital anomalies that occur due to
the incomplete closure of neural tube in early stages of
embryo formation.3,4 Spinal dysraphisms are divided into
2 groups of open or uncovered and occult; this classification
is based on the presence or absence of normal
skin over lesions.
Occult spinal dysraphism (OSD) defects include a
spectrum of congenital anomalies causing clinical symptoms
and spinal cord injury by rostrocaudal traction on
the spinal cord.
These patients tend to have dermatologic and orthopedic
disorders.2 OSD is difficult to be diagnosed in
infants, because in most cases it is asymptomatic at birth5
and neurological symptoms are not often diagnosable
until the child starts walking.4 Cutaneous marks are often
the initial sign of congenital spinal disorders.6 Consequently,
paying attention to skin lesions is crucial for
diagnosis and early treatment of OSD and can prevent
complications in these patients. |