2013 Volume 59 Issue 3 Pages 187-191
Sakoda complex is as a distinctive disease entity characterized by sphenoethmoidal meningoencephalocele, agenesis of the corpus callosum, and cleft lip, cleft palate, or both. The etiology remains unclear. However, this characteristic congenital malformation complex may be explained by embryological restriction of the cephalic neural crest and improper separation between the neurectoderm and the surface ectoderm after neural tube formation. We describe a patient with anomalies consistent with the Sakoda complex. The patient's extraoral findings included bilateral orbital proptosis, left exotropia, a right accessory auricle, and incomplete medial cleft lip, and intraoral findings included medial cleft jaw and upper labial frenum. MR imaging of the brain showed enlargement of the posterior horns and an Azygos-type anterior cerebral artery. He had life-threatening symptoms such as obstructive respiratory problems, but showed improvement in accordance with growth. About 7 months after birth, we performed an operation to close the cleft lip. During and after the operation, the infant had transient abnormalities of thermoregulation due to a pituitary disorder, but the condition was not so serious. In patients with a good prognosis, lip plasty and palatoplasty are necessary not only to improve aesthetic problems and feeding disorders, but also to protect the meningoencephalocele.
