Colpocephaly
Encyclopedia
Colpocephaly refers to an abnormal appearance of the brain in which there is asymmetric dilatation of its lateral ventricle occipital horns, but with normal caliber frontal horns. It is typically associated with microcephaly
and developmental delay. Specific symptoms include decreased IQ, visual impairment, locomotor abnormalities, muscle spasms, and seizure
s.
Colpocephaly is a nonspecific finding and can be associated with multiple neurologic syndromes, including agenesis
of the corpus callosum
and Chiari malformation. It is thought to be related to an intrauterine disturbance that occurs between the second and sixth months of pregnancy. The finding may be indirectly suggested on ultrasound by the so called lemon sign, which occurs due to depression of the calvarium at the bilateral frontal suture lines, giving the calvarium the appearance of a lemon.
Specific treatment depends on associated symptoms and the degree of dysfunction. Anticonvulsant
medications can be given to prevent seizure complications, and physical therapy is used to prevent contractures (shrinkage or shortening of muscles) in patients that have limited mobility. The prognosis for individuals with colpocephaly depends on the severity of the associated conditions and the degree of abnormal brain development. Some children may benefit from special education
.
The lemon sign refers to the shape of the fetal skull at ultrasonography (US) when the frontal bones lose their normal convex contour and appear flattened or inwardly scalloped. This gives the skull a shape similar to that of a lemon (Figs 1, 2). The sign is seen on transverse sonograms of the fetal cranium obtained at the level of the ventricles
Microcephaly
Microcephaly is a neurodevelopmental disorder in which the circumference of the head is more than two standard deviations smaller than average for the person's age and sex. Microcephaly may be congenital or it may develop in the first few years of life...
and developmental delay. Specific symptoms include decreased IQ, visual impairment, locomotor abnormalities, muscle spasms, and seizure
Seizure
An epileptic seizure, occasionally referred to as a fit, is defined as a transient symptom of "abnormal excessive or synchronous neuronal activity in the brain". The outward effect can be as dramatic as a wild thrashing movement or as mild as a brief loss of awareness...
s.
Colpocephaly is a nonspecific finding and can be associated with multiple neurologic syndromes, including agenesis
Agenesis
In medicine, agenesis refers to the failure of an organ to develop during embryonic growth and development due to the absence of primordial tissue...
of the corpus callosum
Corpus callosum
The corpus callosum , also known as the colossal commissure, is a wide, flat bundle of neural fibers beneath the cortex in the eutherian brain at the longitudinal fissure. It connects the left and right cerebral hemispheres and facilitates interhemispheric communication...
and Chiari malformation. It is thought to be related to an intrauterine disturbance that occurs between the second and sixth months of pregnancy. The finding may be indirectly suggested on ultrasound by the so called lemon sign, which occurs due to depression of the calvarium at the bilateral frontal suture lines, giving the calvarium the appearance of a lemon.
Specific treatment depends on associated symptoms and the degree of dysfunction. Anticonvulsant
Anticonvulsant
The anticonvulsants are a diverse group of pharmaceuticals used in the treatment of epileptic seizures. Anticonvulsants are also increasingly being used in the treatment of bipolar disorder, since many seem to act as mood stabilizers, and in the treatment of neuropathic pain. The goal of an...
medications can be given to prevent seizure complications, and physical therapy is used to prevent contractures (shrinkage or shortening of muscles) in patients that have limited mobility. The prognosis for individuals with colpocephaly depends on the severity of the associated conditions and the degree of abnormal brain development. Some children may benefit from special education
Special education
Special education is the education of students with special needs in a way that addresses the students' individual differences and needs. Ideally, this process involves the individually planned and systematically monitored arrangement of teaching procedures, adapted equipment and materials,...
.
The lemon sign refers to the shape of the fetal skull at ultrasonography (US) when the frontal bones lose their normal convex contour and appear flattened or inwardly scalloped. This gives the skull a shape similar to that of a lemon (Figs 1, 2). The sign is seen on transverse sonograms of the fetal cranium obtained at the level of the ventricles