Abstract
Sakoda complex is a rare but distinct combination of birth defects consisting of a basal cephalocele, agenesis of the corpus callosum, and midline cleft lip/palate. It has been reported in association with ophthalmologic abnormalities, cognitive deficits and severe epilepsy. Here we describe both prenatal and postnatal MRI findings of a classic case of Sakoda complex in a child with characteristic findings on fetal MRI; prenatal findings have not been described in the literature. Diagnosis of this entity has important implications for prenatal counseling and perinatal management, as is demonstrated in this case.
Similar content being viewed by others
References
Sakoda K, Ishikawa S, Uozumi T (1979) Sphenoethmoidal meningoencephalocele associated with agenesis of the corpus callosum and median cleft lip and palate. J Neurosurg 51:397–401
Dempsey MA, Torres-Martinez W, Walsh LE (2007) Two cases further delineating the Sakoda complex. Am J Med Genet A 143:370–376
Ehara H, Kurimasa A, Ohno K, Takeshita K (1998) New syndrome with the Sakoda complex, bilateral anophthalmia, and cortical dysgenesis. Pediatr Neurol 18:445–451
Hope-Ross M, Johnston SS (1990) The morning glory syndrome associated with sphenoethmoidal encephalocele. Ophthalmic Paediatr Genet 11:147–153
Richieri-Costa A, Guion-Almeida ML (2004) The syndrome of frontonasal dysplasia, callosal agenesis, basal encephalocele, and eye anomalies — phenotypic and aetiological considerations. Int J Med Sci 1:34–42
Okano S, Tanaka R, Okayama A et al (2017) Congenital basal meningoceles with different outcomes: a case series. J Med Case Rep 11:359
Webb EA, AlMutair A, Kelberman D et al (2013) ARNT2 mutation causes hypopituitarism, post-natal microcephaly, visual and renal anomalies. Brain 136:3096–3105
Kantarci S, Ragge NK, Thomas NS et al (2010) Donnai-Barrow syndrome (DBS/FOAR) in a child with a homozygous LRP2 mutation due to complete chromosome paternal isodisomy. Am J Med Genet A 42:113–121
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflicts of interest
None
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Nagaraj, U.D., Moudgal, R., Hopkin, R.J. et al. Prenatal evaluation of the Sakoda complex. Pediatr Radiol 49, 1843–1847 (2019). https://doi.org/10.1007/s00247-019-04491-5
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-019-04491-5