Journal of Clinical Neonatology

CASE REPORT
Year
: 2018  |  Volume : 7  |  Issue : 3  |  Page : 177--180

Cyclopia-otocephaly-agnathia-synotia-astomia complex: A case report


Kanwar Singh, Shruti Sharma, Khushboo Agarwal, Abhimanyu Kalra 
 Department of Paediatrics, J. L. N. Medical College, Ajmer, Rajasthan, India

Correspondence Address:
Dr. Kanwar Singh
A.211, Budhvihar, Near Power House, Alwar, Rajasthan
India

Agnathia is an extremely rare condition. Disorder has also been termed agnathia-holoprosencephaly spectrum, agnathiaotocephaly complex, agnathia-astomia-synotia, or cyclopia-otocephaly association. The spectrum of agnathia ranges from isolated agnathia or virtual absence of the mandible to otocephaly. Agnathia-otocephaly can occur alone or in association with other abnormalities, Holoprosencephaly being the most common. A severe form of holoprosencephaly resulting in children being born with Cyclopia. It results from failure of the cerebral hemisphere to separate during fetal development. It is incompatible with life. Majority result in miscarriages or stillbirths. In this report we present a male preterm baby with cyclopia, otocephaly, agnathia, astomia and synotia (Kanwar Syndrome). As it is a lethal syndrome, knowledge of every variant of this is important for prenatal diagnosis and prognostication. To the best of our knowledge, this unique combination is the first case reported by us in the world.


How to cite this article:
Singh K, Sharma S, Agarwal K, Kalra A. Cyclopia-otocephaly-agnathia-synotia-astomia complex: A case report.J Clin Neonatol 2018;7:177-180


How to cite this URL:
Singh K, Sharma S, Agarwal K, Kalra A. Cyclopia-otocephaly-agnathia-synotia-astomia complex: A case report. J Clin Neonatol [serial online] 2018 [cited 2020 Aug 15 ];7:177-180
Available from: http://www.jcnonweb.com/article.asp?issn=2249-4847;year=2018;volume=7;issue=3;spage=177;epage=180;aulast=Singh;type=0