Prenatal evaluation of the Sakoda complex

Clinical Applications of Fetal MRI in the Brain

“…An example of this is the Sakoda complex, which is a rare condition described as a triad of abnormalities including agenesis of the corpus callosum, midline cleft lip and palate, and a basal encephalocele. This basal encephalocele cannot be reliably identified on prenatal US alone; however, if diagnosed on fetal MRI, it can be an indication for ex utero intrapartum treatment (EXIT) to airway procedure, as the cephalocele has the potential to obstruct the nasopharyngeal and oropharyngeal airways in the neonate [46] (Figure 6). cephaly (c) demonstrates complete absence of normal separation of the cerebral hemispheres with a monoventricle (arrow).…”

An Endoscopic Endonasal Attempt at Pediatric Meningoencephalocele Repair

“…Meningoencephalocele is a rare congenital condition with an incidence of 1 in 5000 live births with the typical presentation of headaches, seizures, nasal obstruction, meningitis, and CSF rhinorrhea, sometimes associated with the rare Sakoda complex. 1,2 Depending on the location of the meningoencephalocele, it can be visualized via sonography and classified as occipital, frontonasal, or basal, with different surgical approaches for each classification. 3,4 The occipital meningoencephalocele is at the posterior fontanel, the frontonasal herniation is between the frontal and nasal bones and the basal protrusion is internally located along the skull base.…”

“…Rarely, this condition can present as a part of the Sakoda complex, a rare combination of congenital defects including corpus callosum agenesis, cleft lip/palate, optic disc dysplasia, cortical dysgenesis, and anterior skull base encephaloceles. 1 Typically, meningoencephaloceles that protrude through bony defects in the cribriform plate, sphenoid sinus, or ethmoid sinus can be treated with the endoscopic transsphenoidal approach by otolaryngologists and neurosurgeons. We now report a case of an 18-month-old female who presented with a Sakoda complex meningoencephalocele and attempted endoscopic resection that ultimately required an open craniotomy.…”