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
Similar Articles with Keyword 'developmental'
Downloads: 1
Comparative Studies, Pediatrics, India, Volume 10 Issue 12, December 2021
Pages: 534 - 537Long 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
Downloads: 1 | Weekly Hits: ⮙1 | Monthly Hits: ⮙1
Analysis Study Research Paper, Pediatrics, India, Volume 12 Issue 3, March 2023
Pages: 473 - 475To Study the Association between Severity of Perinatal Asphyxia and Multiorgan Dysfunction
Yendeti Bharath Vyass | Veedha Sree Jasti | Gokul Krishnan R. | Kedarnath Reddy T.