Oropharyngeal epignathus with partial facial duplication: report of a rare case

Herath, Manjula; Siriwardena, B. S. M. S.; Jayasinghe, Ruwan Duminda; Dias, D.K.

doi:10.1016/j.ijom.2017.08.011

Cited by 4 publications

(5 citation statements)

References 6 publications

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“…These tumors have high mortality associated with obstruction of the airway during, or shortly after, birth [1]. Their etiology is still unknown, however, there are a variety of theories with the most popular being that the epignathus can surge from totipotent cells from Rathke's pouch and grow in a disorganized manner [2].…”

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confidence: 99%

Surgical management of palatal teratoma (epignathus) with the use of virtual reconstruction and 3D models: a case report and literature review

et al. 2021

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Epignathus is a rare congenital orofacial teratoma that arises from the sphenoid region of the palate or the pharynx. It occurs in approximately 1:35,000 to 1:200,000 live births representing 2% to 9% of all teratomas. We present the case of a newborn of 39.4 weeks of gestation with a tumor that occupied the entire oral cavity. The patient was delivered by cesarean section. Oral resection was managed by pediatric surgery. Plastic surgery used virtual 3-dimensional models to establish the extension, and depth of the tumor. Bloc resection and reconstruction of the epignathus were performed. The mass was diagnosed as a mature teratoma associated with cleft lip and palate, nasoethmoidal meningocele that conditions hypertelorism, and a pseudomacrostoma. Tridimensional technology was applied to plan the surgical intervention. It contributed to a better understanding of the relationships between the tumor and the adjacent structures. This optimized the surgical approach and outcome.

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confidence: 99%

Surgical management of palatal teratoma (epignathus) with the use of virtual reconstruction and 3D models: a case report and literature review

et al. 2021

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“…4,13 Co-existence of epignathus teratoma with tongue choristoma is very rare, 10 based on our English PubMed literature searched we found only 2 cases. 14,15 There are only 13 cases of isolated oral epignathus teratoma reported in the literature from the first case report in 1930 to 2004. 7 The ages of the patients at diagnosis were within the prenatal period which ranges from birth to 5 months and majority of the cases reported were females (8 out of 13 cases).…”

Section: Discussionmentioning

confidence: 99%

Epignathi Teratoma Co-Existing with Tongue Choristoma and Cleft Palate in a Neonate: Report of a Rare Case and Review of Literature

Abdullahi

Bukar

Wapada

et al. 2020

Craniomaxillofacial Trauma & Reconstruction Open

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Background: Oral epignathus teratoma is an extremely rare neoplasm that occurs exclusively in neonates. The tumor can grow to a large size obstructing airways and it may prevent fusion of the nasal septum with the palatine processes, which may result in cleft palate formation. The rare tumor may also co-exist with other congenital malformations such as hamartoma and choristoma causing airway obstruction and death. Case report: We present a case of a one-day-old male neonate who presented with oral mass associated with difficulty in breathing and inability to suck breast milk. Computed Tomography (CT) scan revealed multiple masses involving the alveolar, palate, and tongue along with cleft palate. Emergency surgery was done to relieve the airway and histology confirmed epignathi teratoma with tongue choristoma. Conclusion: Epignathus teratoma is a rare condition that requires prenatal prompt diagnosis. However, in countries with underprivileged hospital facilities, early surgical intervention during the neonatal period is what is required to save the patient from airway obstruction. Association of the tumor with midline anomalies is mostly the norms, however, 5% of cases with malignant transformation have been reported and the tumor is commonly associated with endodermal sinus tumor. Therefore long term follow-up of patients with serum tumor markers is highly recommended.

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“…Including our patient, 8 had a type I/w-shaped mandibular duplication. 9 -12,21,23,26 All of these patients also had a cleft palate and accessory or lobulated tongue. 63% (5/8) of these patients had airway compromise requiring intervention.…”

Section: Discussionmentioning

confidence: 97%

“…63% (5/8) of these patients had airway compromise requiring intervention. 11,12,21,23 Only 2 patients with other types of mandibular duplication were reported to have airway issues. Another patient with a w-shaped mandible died on day of life 3 (cause not reported) 26 (Tables 1 and 2).…”

Section: Discussionmentioning

confidence: 99%

PLAST Classification of Mandibular Duplication and Associated Anomalies

Leberfinger

Murthy

2023

FACE

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Mandibular duplication is a rare subtype of craniofacial duplication. It is often associated with other anomalies including cleft palate, lingual duplication, and macrostomia. We present an 8-year-old female who had mandibular duplication presenting soon after birth with tongue-based airway obstruction. She was found to have cardiac abnormalities, split sternum, accessory tongue, cleft palate, and accessory submandibular gland. She underwent surgical correction including resection of the duplicated mandible, tongue, and submandibular gland as well as tongue lip adhesion at 12 days old. Post operatively, we were able to avoid tracheostomy and she was able to undergo successful correction of her cardiac anomaly. Literature review found 29 patients with mandibular duplication. Patients with w-shaped (type I) mandibular duplication are more likely to have airway compromise requiring intervention. Several associated anomalies were seen in patients with mandibular duplication. After analysis, we propose the PLAST classification to describe common findings: cleft Palate, Lip duplication, Airway obstruction, Salivary gland abnormalities, and Tongue duplication. Extra craniofacial anomalies such as cardiac and spinal anomalies were also noted in several patients.

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Oropharyngeal epignathus with partial facial duplication: report of a rare case

Cited by 4 publications

References 6 publications

Surgical management of palatal teratoma (epignathus) with the use of virtual reconstruction and 3D models: a case report and literature review

Surgical management of palatal teratoma (epignathus) with the use of virtual reconstruction and 3D models: a case report and literature review

Epignathi Teratoma Co-Existing with Tongue Choristoma and Cleft Palate in a Neonate: Report of a Rare Case and Review of Literature

PLAST Classification of Mandibular Duplication and Associated Anomalies

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