epignathus
MONDO:0015386
Epignathus is a very rare and life threatening intraoral teratoma, usually arising from the maxilla, mandible, palate or base of skull and invading the cranium, nasopharynx or oral cavity. Epignathus is more commonly seen in females, and presents with various manifestations (depending on the tumor size) including obstructive polyhydramnios in the prenatal period and dyspnea, cyanosis, cough, difficulty in sucking and swallowing, and rarely vomiting (due to swallowing difficulties) postnatally. When large, they can lead to airway obstruction, asphyxia and death in the neonatal period.
Research intelligence
Directly mapped studies only · unique clinical trialsCurrent registry observations. Phase counts may overlap. Study start dates are not registry registration dates. Investigational interventions are not approved-treatment recommendations.
Medications under investigation
Verified disease and drug mappings that co-occur within the same study; not evidence of effectiveness.
MONDO disease family
Recruiting studies by city
Trial status
Trial phase
Study start year
Investigated drug interventions
Epignathus
Epignathus is a rare teratoma of the oropharynx. Epignathus is a form of oropharyngeal teratoma that arises from the palate and, in most cases, results in death. The pathology is thought to be due to unorganized and uncontrolled differentiation of somatic cells leading to formation of the teratoma; sometimes it is also referred to as fetus in fetu, which is an extremely rare occurrence of an incomplete but parasitic fetus located in the body of its twin. This tumor is considered benign (non-cancerous) but life-threatening because of its atypical features (size, location, and rate of development) and high risk of airway obstruction, which is the cause of death in 80–100% of the cases at the time of delivery. Despite the high mortality rate, the most important factor in improving survival probability is to detect and diagnose the lesion before birth using ultrasound and MRI scans. If undetected prenatally, the epignathus will be apparent immediately after birth, but prognosis will be poor due to lack of preparation and treatment plans. Most babies with epignathus have a poor prognosis due to late diagnosis and, subsequently, complications in securing the airway. However, with early detection and multidisciplinary healthcare teams, an adequate treatment plan to secure the baby's airway and surgically remove the lesion may improve the prognosis. Treatment options for this rare condition prioritize managing the risk of asphyxiation prior to deciding on an appropriate plan for the teratoma resection.
Background only. Canonical identity remains MONDO; clinical-trial relationships come from ClinicalTrials.gov.Drugs being studied
Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.