agnathia-otocephaly complex
Sign in to saveAlso known as agnathia-holoprosencephaly-situs inversus syndrome, dysgnathia complex agnathia-holoprosencephaly, holoprosencephaly-agnathia, otocephaly, AGOTC, AGNATHIA-OTOCEPHALY COMPLEX; AGOTC
Otocephaly, also known as agnathia–otocephaly complex, is a very rare and lethal cephalic disorder characterized by the absence of the mandible (agnathia), with the ears fused together just below the chin (synotia). It is caused by a disruption to the development of the first branchial arch. It occurs in every 1 in 70,000 embryos.
Research
101 papers- Re-focusing on Agnathia-Otocephaly complex.Clinical oral investigations · 2021
- Agnathia-otocephaly complex diagnosed by prenatal ultrasound: a case report.Translational pediatrics · 2021
- First-Trimester Diagnosis of Agnathia-Otocephaly Complex: A Series of 4 Cases and Review of the Literature.Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine · 2019
- Agnathia-microstomia-synotia syndrome (otocephaly).Autopsy & case reports · 2020
- Cyclopia: isolated and with agnathia-otocephaly complex.BMJ case reports · 2017
via PubMed
Wikidata facts
- Subclass of
- disease
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- Otocephaly.png
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- health specialty
- medical genetics
- exact match
- www.orpha.net/ORDO/Orphanet_990
- ICD-9-CM
- 759.89
- on focus list of Wikimedia project
- WikiProject Medicine
- Commons category
- Otocephaly
Sources (2)
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Encyclopedic overview
8 sectionsContents
- Signs and symptoms
- Grades
- Cause
- Development
- Prognosis
- History
- References
- External links
Otocephaly, also known as agnathia–otocephaly complex, is a very rare and lethal cephalic disorder characterized by the absence of the mandible (agnathia), with the ears fused together just below the chin (synotia). It is caused by a disruption to the development of the first branchial arch. It occurs in every 1 in 70,000 embryos.
== Signs and symptoms == thumb|Otocephaly with cyclopia and a proboscis|237x237px
Excerpted from Wikipedia’s “agnathia-otocephaly complex” article, available under the CC BY-SA 4.0 licence.