Diagnosis
Open lip schizencephaly
Findings
As included in Discussion
Discussion
Schizencephaly refers to a type of migrational anomaly in which
a cleft a extends from the ependyma of the ventricle to the pial
surface. The clefts are lined by gray matter and are filled with
cerebrospinal fluid. Open lip schizencephaly refers to clefts where
the walls are separated and is associated with a worse prognosis
than closed lip schizencephaly in which the walls are in
apposition. Patients typically present with intractable seizures
and developmental delay. The generally accepted pathogenesis of
schizencephaly is via early in-utero vascular insult. Associations
include other migrational anomalies, dysgenesis of the corpus
callosum and septo-optic dysplasia. Patients with schizencephaly
may also have varying degrees of microcephaly (abnormally small
head), mental retardation, hemiparesis or quadriparesis (partial or
complete paralysis), and reduced muscle tone (hypotonicity). Most
patients have seizures. Some may have hydrocephalus.
Treatment for individuals with schizencephaly generally consists
of physical therapy, treatment for seizures, and, in cases that are
complicated by hydrocephalus, a shunt (a surgically implanted tube
that diverts fluid from one pathway to another).
Barkovich AJ, Kjos BO. Schizencephaly: correlation of clinical
findings with MR characteristics. AJNR, Jan-Feb 1992;
13(1):p85-94.
Klingensmith WC, Cioffi-Ragan DT. Schizencephaly: diagnosis and
progression in utero. Radiology, Jun 1986; 159(3):p617-8.