Article
Cystic spinal dysraphism of the cervical and upper thoracic region
Registro en:
SALOMÃO, J. Francisco et al. Cystic spinal dysraphism of the cervical and upper thoracic region. Child's Nervous System, Berlin, v. 22, n. 3, p. 234-242, 2006.
0256-7040
10.1007/s00381-005-1161-1
Autor
Salomão, José Francisco Manganelli
Cavalheiro, Sérgio
Matushita, Hamilton
Leibinger, René D.
Bellas, Antonio R.
Vanazzi, Elide
Souza, Luiz A. M. de
Nardi, Andréa G.
Resumen
Background:Cystic dys-raphic lesions of the cervical and
upper thoracic region are rare and
only a few series have been published
about the topic. These malformations
can be divided into categories that
include both myelocystoceles and the
so-called cervical meningoceles or
myelomeningoceles.Methods:A
retrospective study of 18 patients was
conducted. Results:In 17 patients a
squamous or a cicatricial epithelium
of variable thickness covered the
dome of the lesions, while the base
was covered with full-thickness skin.
In one case the skin was entirely
normal. Four patients displayed asso-ciated CNS malformations and three
more had systemic congenital
anomalies. All patients underwent
surgical exploration and the length
of time between birth and surgery
ranged from 6 h to 9 months. The
most frequent surgical finding, seen in
14 patients, was a stalk connecting the
dorsal surface of the spinal cord to the
cyst. In three patients the findings
were consistent with myelocystocele.
Only in one case was a true menin-gocele found. Hydrocephalus and
Chiari II malformation were not as
consistently associated as in myelo-meningoceles. Neurological signs and
symptoms were not so marked as in
myelomeningoceles and were found
in the follow-up of four patients. In
two of them there was a non-pro-gressive deficit, probably expressing
an imperceptible involvement of the
nervous system in the first year of life.
The histopathological findings were
of three types: neuroglial stalks,
fibrovascular stalks and myelocysto-celes. Conclusions:Cystic dysraph-isms of the cervical and upper
thoracic region differ clinically and
structurally from meningomyelocele
and have a more favorable outcome.
We believe that these malformations
have not been properly labeled and
propose a classification based on the
structures found inside the cyst.