International Journal of Science and Research (IJSR)

International Journal of Science and Research (IJSR)
Call for Papers | Fully Refereed | Open Access | Double Blind Peer Reviewed

ISSN: 2319-7064

Downloads: 122 | Views: 187 | Weekly Hits: ⮙6 | Monthly Hits: ⮙6

Case Studies | Pediatrics | Nigeria | Volume 8 Issue 5, May 2019

Otocephaly: Agnathia Microstomia Synotia Syndrome - A Case Report

Onyesoh Chinyeaka.R | Okoye John.O | Nduji Oluchi. J | Okorie Elizabeth-Martha.C | Udeozor Lucy.C

Abstract: Otocaphaly is rare lethal malformation seen in 1: 70000 births and is characterized by the association of agnathia (mandibular agenesis) / mandibular hypoplasia, melotia (ventromedial/ auricular malposition) with or without auricular fusion (synotis) and microstomia. It is thought to be a consequence of first pharyngeal arch malformation resulting from failure of migration of neural crest cells from the hindbrainat4 weeks gestational age. Ventilatory difficulties typically lead to demise shortly after birth in affected babies. Prenatal diagnosis of Otocephaly and other rare congenital malformations relies on the availability of both expertise in imaging studies and the availability of 3D ultrasonography machines/MRI all of which are not readily available. Prenatal diagnosis is very important as it will facilitate appropriate parental counseling and enhance decision regarding intra-partum and immediate post-partum intervention. In resource-poor setting, even when these diagnostic facilities are available, it is not affordable by the majority of patients. Until universal health insurance is available to all, cost will continue to be a major hindrance to appropriate diagnosis and treatment in these settings. To the best of these authors knowledge, no case of Otocephaly has been reported in Nigeria and West Africa. In this report we present a preterm female with Otocephaly-Agnathia Microstomia Synotia syndrome.

Keywords: Otocephaly Agnathia, developmental malformation, Prenatal diagnosis, Port-Harcourt

Edition: Volume 8 Issue 5, May 2019,

Pages: 1605 - 1607

How to Download this Article?

Type Your Email Address below to Download the Article PDF

Text copied to Clipboard!
Onyesoh Chinyeaka.R, Okoye John.O, Nduji Oluchi. J, Okorie Elizabeth-Martha.C, Udeozor Lucy.C, "Otocephaly: Agnathia Microstomia Synotia Syndrome - A Case Report", International Journal of Science and Research (IJSR), Volume 8 Issue 5, May 2019, pp. 1605-1607,

Similar Articles with Keyword 'developmental'

Downloads: 1

Comparative Studies, Pediatrics, India, Volume 10 Issue 12, December 2021

Pages: 534 - 537

Long Term Outcomes of Children Born by ICSI in Comparison to Spontaneously Conceived Children: A Retrospective Observational Study

Dr. Sujitha .N | Prof. Dr. L. Umadevi | Prof. Dr. P. Radhapandiyan

Share this Article

Downloads: 1 | Weekly Hits: ⮙1 | Monthly Hits: ⮙1

Analysis Study Research Paper, Pediatrics, India, Volume 12 Issue 3, March 2023

Pages: 473 - 475

To Study the Association between Severity of Perinatal Asphyxia and Multiorgan Dysfunction

Yendeti Bharath Vyass | Veedha Sree Jasti | Gokul Krishnan R. | Kedarnath Reddy T.

Share this Article