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The occurrence of teratoma is 1 in 4,000 live births. Head-and-neck region teratoma (epignathus) is rare. These patients present with facial disfigurement, respiratory problems, and difficulty in feeding. It warrants early excision to avoid any morbidity or mortality due to airway obstruction. We describe the management of a patient with pedunculated epignathus with cervical lymphangioma.

The prenatal diagnosis of epignathus presents a unique challenge for physicians. Differential diagnosis is usually based on the anatomic location of the tumor. Typical prenatal ultrasound characteristics of epignathus include a mixed solid and cystic lesion with vascularity in the solid component, originating from the hard or soft palate, and it is often associated with other anomalies such as craniofacial clefts or trans‐sphenoidal intracranial extension. Herein, we present a case of prenatal diagnosis of epignathus with rare ultrasonographic findings, prenatal management requiring collaborative efforts of a multidisciplinary team, and a well‐planned innovative ex utero intrapartum treatment procedure. In addition, this report highlights the evolving postnatal diagnosis of the rare developmental anomaly, duplication of the pituitary gland‐plus syndrome, which includes various midline craniofacial, central nervous system, spinal, and endocrine abnormalities. [ABSTRACT FROM AUTHOR]

Epignathus is an extremely rare teratoma found in the oral cavity or oropharyngeal region of newborns, whose pathogenesis is poorly understood. We describe a giant epignathus arising from the oropharynx in a newborn. The giant tumor completely obstructed the airway of the newborn resulting in death. Histological and radiological examination of the tumor reveals the presence of a remarkably well-developed skeleton of the head and neck. A row of teeth, the axis and atlas, thyroid and salivary glands, trachea, and cerebral tissue are all detected within the tumor. These findings suggest that the epignathus is fetus-in-fetu which is considered a type 0 germ cell tumor in accordance with current literature. [ABSTRACT FROM AUTHOR]

An epignathus is caused by a continuous spectrum of masses of the oral cavity or oropharynx ranging in its entity from mature teratoma to the exceedingly rare fetus-in-fetu. Due to its location, regardless of the entity, the occurrence of an epignathus is frequently associated with life threatening airway obstruction. Here we demonstrate a case of a fetus-in-fetu presenting as an epignatus. We describe its successful management and review the available literature. Early diagnosis and knowledge of the preoperative workup are essential to enable a multidisciplinary management. Once the airway is secured, surgical excision is the treatment of choice often resulting in a good clinical outcome and prognosis.

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Abstract Background Epignathus is a rare, benign, congenital teratoma of the hard palate with an estimated incidence of 1 in 200,000 live births. Epignathus frequently leads to a high mortality (80–100%) due to airway obstruction in the neonatal period. Case presentation We report a case of successful management of a rare oropharyngeal teratoma in a African newborn girl who was referred to our institute with a large protruding intraoral mass, combined with cleft palate, causing some respiratory compromise. The palatal mass was removed on the fifth day after birth, and a palatoplasty performed on day 30. Conclusion Epignathus is a life-threatening condition at the time of delivery. Appropriate management begins with securing the airway, followed by complete resection of the tumor.

This article, published in the Malaysian Journal of Pathology, reports on a case of congenital orofacial teratoma, also known as epignathus, in a 22-week-old fetus. The mother, a 34-year-old woman, presented for a routine examination and an ultrasound revealed a solid-cystic facial tumor in the fetus. The pregnancy was terminated and the stillborn fetus was examined, revealing a large tumor protruding from the oral cavity and an intracranial tumor. Microscopic examination showed immature teratomas in both tumors. Congenital orofacial teratoma is rare and can be associated with malformations such as cleft palate. Early detection is important for intervention to prevent airway obstruction. [Extracted from the article]

Teratomas are benign tumours containing tissues derived from ectoderm, endoderm and mesoderm Epignathus is a rare congenital teratoma and originates from oropharyngeal region. We present a case of giant epignathus arising from tonsillar region in a neonate. A male neonate that was born with a 38-week cesarean section presented with a pedunculated mass from left tonsilla palatina and protruding outside the mouth. The patient did not have any airway problem. Magnetic resonance imaging and computed tomography scan showed no intracranial extension. The patient was operated on the postpartum 3rd day and the mass was excised successfully. After histopathological examination, mature teratoma was diagnosed. During post-operative 6 months control visit, there was no recurrence. Epignathus is a rare congenital oropharyngeal teratoma, it should be diagnosed in the fetus as early as possible. Teratomas of the tonsilla palatina are extremely rare. In such cases, the mass may cause airway obstruction and feeding difficulties so complete resection is curative in most cases during the early neonatal period.

We report a rare case of epignathus (oropharyngeal teratoma) in a neonate, who presented with a midline mass covered with skin and multiple hairs protruding from the Palate and associated with bifid tongue and ranula. With the characteristic presentation, diagnosis of oro/oropharyngeal teratoma was made and a massive internet search revealed very few reported cases of "epignathus". It is unfortunate that the survival of such neonates is only moderate. Prenatal scans and follow up in an institution can prepare the multidisciplinary team to save the child. EXIT procedure to excise the mass or secure the airway, with future repair of the palate is the treatment option available. This case report emphasizes the rare clinical presentation of the disease and the prenatal diagnosis of such a condition can help in prompt decision making and management. [ABSTRACT FROM AUTHOR]

We report a case of neonatal respiratory distress caused by an epignathus. This rare tumour, arising from the basisphenoid and protruding from the mouth, is associated with high perinatal mortality. We highlight unique clinico-radiological findings and multidisciplinary approach for a successful outcome. We review oropharyngeal teratomas reported in the literature with current management approaches. [ABSTRACT FROM AUTHOR]

Background: Epignathus is a rare, benign, congenital teratoma of the hard palate with an estimated incidence of 1 in 200,000 live births. Epignathus frequently leads to a high mortality (80-100%) due to airway obstruction in the neonatal period.Case Presentation: We report a case of successful management of a rare oropharyngeal teratoma in a African newborn girl who was referred to our institute with a large protruding intraoral mass, combined with cleft palate, causing some respiratory compromise. The palatal mass was removed on the fifth day after birth, and a palatoplasty performed on day 30.Conclusion: Epignathus is a life-threatening condition at the time of delivery. Appropriate management begins with securing the airway, followed by complete resection of the tumor. [ABSTRACT FROM AUTHOR]

Teratomas are benign tumours containing tissues derived from ectoderm, endoderm and mesoderm Epignathus is a rare congenital teratoma and originates from oropharyngeal region. We present a case of giant epignathus arising from tonsillar region in a neonate. A male neonate that was born with a 38-week cesarean section presented with a pedunculated mass from left tonsilla palatina and protruding outside the mouth. The patient did not have any airway problem. Magnetic resonance imaging and computed tomography scan showed no intracranial extension. The patient was operated on the postpartum 3rd day and the mass was excised successfully. After histopathological examination, mature teratoma was diagnosed. During post-operative 6 months control visit, there was no recurrence. Epignathus is a rare congenital oropharyngeal teratoma, it should be diagnosed in the fetus as early as possible. Teratomas of the tonsilla palatina are extremely rare. In such cases, the mass may cause airway obstruction and feeding difficulties so complete resection is curative in most cases during the early neonatal period. [ABSTRACT FROM AUTHOR]

Presence of intraoral pathology especially in neonates poses a great challenge during airway management. Epignathus is a rare form of teratoid tumour that arises from the oropharyngeal region. We hereby report a case of a 7-day-old neonate who presented with feeding difficulty secondary to swelling arising from the hard palate. Surgical excision was decided to overcome feeding difficulty and to enable the child to thrive better. In view of anticipated difficult airway, the child was induced with sevoflurane, maintaining spontaneous breathing and intubated with Airtraq optical laryngoscope size 0. The further intraoperative and postoperative period was uneventful.

Abstract Background Epignathus is a rare congenital orofacial teratoma infrequently associated with intracranial extension. Intracranial extension of an epignathus indicates a poor prognosis; however, only a small number of such cases have been reported. While there have been some studies reporting cases of epignathus expanding directly into the cranium, others have reported no communication between an epignathus and an intracranial tumor. Case presentation A fetus at gestational week 27 was suspected of having an epignathus with intracranial tumor as shown by ultrasonographic and magnetic resonance imaging. The fetus was stillborn and an autopsy was performed. An epignathus measuring 12 × 6 × 6 cm and weighing 270 g protruded from the mouth, with its base on the soft palate. An intracranial tumor weighing 14 g was located at the middle intracranial fossa and connected to the epignathus through the right side of the sella turcica. The intracranial tumor was encapsulated, and there was no invasion into the brain. Histologically, both the epignathus and intracranial tumor were immature teratomas, with neural and pulmonary components that were especially immature as compared to those of the internal organs and brain tissues of the fetus. Conclusion There have been several reports of epignathus and intracranial tumors that did not communicate; therefore, careful evaluation is needed when a fetus is suspected of having an epignathus extending into an intracranial lesion. Our case supports the findings that an epignathus can directly expand into the cranium. Moreover, this is a rare case of an epignathus in which the intracranial lesion was encapsulated and did not invade the brain. These rare but important findings will provide additional, potential therapeutic strategies for gynecologists, neurosurgeons, and pathologists.

Background: Atypical presentation of an atypical abnormality makes the diagnosis challenging. Oropharyngeal teratoma (epignathus) is a rare, potentially life-threatening neonatal tumor. Its atypical presentation may cause a delay in diagnosis and increase morbidity and mortality. Case presentation: A newborn girl with oropharyngeal teratoma and cleft palate presented with feeding difficulty. The airway was patent. The tumor was tongue-shaped, smooth-walled, displacing the native tongue, and prevented the fusion of two palatine halves resulting in cleft palate. On the 4th day of life, complete excision was done transorally. Histopathology revealed a mature teratoma. There was no sign of recurrence at three months of follow-up. Conclusion: Presentation of the oropharyngeal teratoma varies according to its site, size, and extension. Timely diagnosis is essential to avoid life-threatening respiratory obstruction. Complete excision of the mass is usually associated with a good prognosis.

Epignathus is a rare congenital orofacial teratoma. Teratomas are tumors that originate from all three germs cell layers. Tumor size is an important prognostic factor, and we describe the case of massive epignathus identified by sonography at 25 weeks. Our case was a 35-year-old pregnant woman that was subjected to a routine ultrasound at 25 weeks of gestation, and epignathus was diagnosed. Labor pain started in the 28th week of the pregnancy, so the dead fetus was aborted, and curettage was conducted. A pathologic sample was sent to the laboratory, and benign teratoma was diagnosed. Because fetal epignathus has a wide range of outcomes, early prenatal diagnosis is essential for optimal management. [ABSTRACT FROM AUTHOR]

Epignathus or enigmatic teratoma is rare congenital tumor, arising from the sphenoid region of the palate or pharynx. A 30-year-old pregnant woman presented at 34 weeks + 2 days' gestation with a well-defined solid mass measuring 6.47 cm × 5.7 cm arising from the fetal oral cavity. The color flow mapping showed that the mass was well vascularized, especially in its center, and its blood supply was originated from the fetal oral cavity. The newborn has no chromosomal abnormalities or other associated anomalies and/or intracranial extension immediately and successfully managed after delivery to highlight the importance of prenatal diagnosis and the importance of multidisciplinary team management. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to evaluate the indications and the clinical outcomes of the fetuses managed with ex utero intrapartum treatment (EXIT) procedures.Methods: We retrospectively reviewed the medical records of all fetuses who underwent EXIT procedures between 2003 and 2018.Results: EXIT procedures were performed in nine cases. The prenatal diagnosis of the neonates was congenital high airway obstruction syndrome in four cases, the neck masse in five cases. Although the airway management under the EXIT procedure was successful in eight cases, the airway management failed in one case. During the EXIT procedures, the airway was managed by endotracheal intubation in two cases, whereas six cases underwent tracheostomy. Six cases with fetal airway obstruction survived to discharge, whereas three cases died due to airway management failure or complications of the underlying disease. A case with a cervical teratoma underwent tumor resection the day after birth due to rapid enlargement of the neck mass. Long-term survival was achieved in five cases.Conclusions: We concluded that the EXIT procedure was effective and could be performed safely in the airway management of fetuses with suspected airway obstruction. The treatment strategy for the neck masses should be planned before birth. [ABSTRACT FROM AUTHOR]

Congenital teratoma that arises in the oropharyngeal region is called epignathus. Here, we report a case in which large epignathus extending from the pharynx to the oral cavity was resected. A female fetus was found to have a mass extending on her face to the pharyngeal region during a sonographic examination performed at 33 weeks' gestation. On the 9th day after birth, the tumor extending from the left nostril (10 cm) was excised by the pediatric surgeons. The residual tumor protruded from the oral cavity day by day. Pediatric and oral surgeons resected the tumor (14.5 cm). A bifid uvula was noted and submucosal cleft palate was suspected. No recurrence has been seen after 4 years' follow‐up. [ABSTRACT FROM AUTHOR]

Epignathus is an extremely rare oral teratoma which leads to high mortality in the early neonatal period. Various theories have been put forward for the genesis of such a tumor, though none is completely convincing. A genetic basis is not well established for the tumor. Microdeletions/duplications, as well as single gene disorders, have been known to cause epignathus, all with additional malformations. Evidence of single gene involvement in an isolated epignathus is lacking. We present a case of a 19-week-fetus with oro-pharyngeal teratoma detected on the level II ultrasound. The couple was counseled regarding the grave prognosis of the fetal condition following which they opted for termination of pregnancy and fetal autopsy. The autopsy revealed fetus-like body attached to the tumor. Genetic testing including a whole genome microarray did not reveal any significant variant. An explanation for the fetus-like body maybe a common origin of the teratoma and the additional fetus-like bodies due to an erroneous process of early embryonic development. Another possibility is of an acardiacus acranius twin masquerading as a fetus-like body. Thus, we conclude that in the absence of an associated malformation, an epignathus is unlikely to have a genetic etiology. This study highlights the importance of performing a fetal autopsy as a part of deep phenotyping to ascertain the etiology, as it identified additional fetal-like body which was not detected on the antenatal ultrasound. [ABSTRACT FROM AUTHOR]

Congenital teratomas arising from the oropharynx, also known as epignathus, are exceedingly rare tumours occurring in 1:35,000–200,000 live births. Although histologically benign, these tumours are often difficult to diagnose antenatally and can cause life-threatening airway obstruction in the newborn. We present a case of a full-term female infant presenting at birth with respiratory distress and stridor. Clinical examination and magnetic resonance imaging showed a large multilobulated nasopharyngeal mass causing severe airway compromise. The infant underwent excision biopsy with partial debulking of the tumour surrounding the airway, and was successfully extubated thereafter to room air. Histopathological examination confirmed the diagnosis of mature teratoma. The pathophysiology of epignathus is thought to be aberrant proliferation of pluripotent cells in the notochord during early embryogenesis. Antenatally, elevated maternal alpha-fetoprotein or polyhydramnios may be clue to diagnosis. The mainstay of treatment is airway clearance with surgical resection, which can be curative or at least minimizes the risk of malignancy. This case highlights the importance of early recognition and multidisciplinary management of the neonatal airway, in order to improve outcome. • Congenital oropharyngeal teratomas are benign but can be life-threatening. • Early airway stabilization and surgical resection are the mainstay of treatment. • Multidisciplinary follow-up is important to support feeding and development. [ABSTRACT FROM AUTHOR]

Presence of intraoral pathology especially in neonates poses a great challenge during airway management. Epignathus is a rare form of teratoid tumour that arises from the oropharyngeal region. We hereby report a case of a 7-day-old neonate who presented with feeding difficulty secondary to swelling arising from the hard palate. Surgical excision was decided to overcome feeding difficulty and to enable the child to thrive better. In view of anticipated difficult airway, the child was induced with sevoflurane, maintaining spontaneous breathing and intubated with Airtraq optical laryngoscope size 0. The further intraoperative and postoperative period was uneventful. [ABSTRACT FROM AUTHOR]

Epignathus also known as oropharyngeal teratoma is a very rare tumor in newborns, which has a female predominance. The treatment is simple excision of the mass surgically which should not be delayed. We operated a male newborn due to intraoral mass causing episodic deglution and respiration problems. The histopathology was reported as epignathus and this case is discussed based on the current literature because of its rarity. [ABSTRACT FROM AUTHOR]

The authors describe a history case of severe congenital dentofacial anomaly with congenital cleft lip, palate and alveolar ridge, associated with somatic disease. The diagnosis was confirmed histologically. The surgical treatment was performed after the careful presurgical preparation due to severe general condition of the child. The indication to the surgical treatment was a significant enlargement of the tumor mass since the child’s birth. The tumor filled the oral cavity and blocked oropharynx almost completely.

Fetuses with upper airway obstruction have a high mortality rate if proper airway management is not immediately administered after delivery. The EXIT (ex utero intrapartum treatment) procedure is a new technique that establishes the fetal airway while uteroplacental circulation is still maintained. The prognosis of such neonates has much improved after the introduction of this procedure. We report two neonates with prenatal diagnosis of upper airway obstruction; they were born smoothly via the EXIT procedure. The first was a case of epignathus, a rare type of nasopharyngeal or oropharyngeal teratoma derived from all three germ cell layers with variable maturity. The second was a case of giant lymphangioma that resulted from localized malformations in the development of the lymphatic system. Furthermore, compared with routine cesarean section, the short-term maternal outcomes were not different with regard to infection rate, estimated blood loss, wound complication, and postpartum hospital stay.

O teratoma congênito de orofaringe é o tipo mais raro de teratoma, compreendendo apenas 2% desses tumores fetais. O diagnóstico deve ser realizado o mais precocemente possível, preferencialmente durante o pré-natal. O prognóstico irá depender do tamanho e localização da lesão, da velocidade de crescimento desta, do envolvimento de estruturas intracranianas e da ressecção adequada do tumor com equipe multidisciplinar. Relatamos o caso de uma paciente que teve diagnosticado durante a gestação feto com teratoma congênito de orofaringe (epignathus) por meio de ultra-sonografia. O feto evoluiu para óbito intra-uterino na 29ª semana de gestação, sendo então induzido o parto por via vaginal. O exame anatomopatológico revelou feto do sexo feminino, compatível com 27-28 semanas, teratoma orofaríngeo e outras malformações congênitas.Oropharyngeal teratoma is the most rare type of teratoma, with only 2% of fetal teratomas. The diagnosis must be established as early as possible, preferably during the prenatal period. The prognosis will depend on the size and location of the lesion, growth rate of the lesion, degree of intracranial spread, its resectability, and immediate care at birth by a multisciplinary team. We report aparticular case of congenital oropharyngeal teratoma (epignathus). The diagnosis was made during the prenatal period by ultrasound, and the fetus evolved to intrauterine death at the 29th week. The anatomopathological examination revealed a female fetus, compatible with 27-28 weeks, oropharyngeal teratoma and congenital malformations.

Epignathus es una forma infrecuente de teratoma de localización orofaríngea que protruye por la boca y se asocia a una alta tasa de mortalidad causada por obstrucción severa de la vía aérea en el período neonatal. La clave del éxito del manejo de la obstrucción es a través del diagnóstico prenatal precoz y la ejecución controlada del EXIT (ex-utero intrapartum treatment), procedimiento utilizado para asegurar la vía aérea fetal. Describimos un caso de epignathus gigante y la aplicación del EXIT para el manejo de la vía aérea a través de la preservación del intercambio gaseoso uteroplacentarioEpignahtus is an uncommon form of teratoma, located in the oropharynge, which protrudes through the mouth and that is associated with a high mortality during the neonatal period. The key to a successful outcome in managing of the airway obstruction is early prenatal diagnosis and the controlled execution of the EXIT (ex-utero intrapartum treatment) to secure the fetal airway. We report a case of a giant epignathus and the application of EXIT procedure, in which time to secure an airway is provided while preserving uteroplacental gas exchange

Foetal oral teratoma or epignathus is a rare benign condition that originates in the oropharyngeal region. The term ‘teratoma’ refers to a mass composed of poorly organized tissues derived from each of the three germ layers. Epignathus may occur when one twin ceases development during gestation and becomes vestigial to the fully formed dominant twin. The reported incidence of epignathus is approximately 1:35,000 to 1:200,000 live births. A unique case of partial facial duplication with gross histological and radiological evidence of partial duplication of the facial elements and organs is presented herein. The affected newborn baby underwent urgent surgery due to breathing difficulties. The mass was excised successfully and the infant made an uneventful recovery. The resected specimen included two developing faces, each composed of a developing mandible and maxilla with developing tooth buds. Tissues from all three germ layers were present. In such cases, the treatment option is exclusively surgical, and complete resection is curative in most cases during the early neonatal period. [ABSTRACT FROM AUTHOR]

Epignathus is an extremely rare oropharyngeal teratoma that commonly arises from the palate, leading to a high mortality (80-100%) due to airway obstruction in the neonatal period. We present a case of epignathus immature teratoma with fetiform features, originating from basisphenoid in a 28-week preterm male baby, who succumbed to death immediately after birth. Since epignathus is a life-threatening condition at the time of delivery, a prenatal diagnosis is essential to coordinate the treatment and appropriate management by securing the airway, either by endotracheal intubation or tracheostomy followed by complete resection of the tumor.

Background Oropharyngeal teratoma may occur by itself or together with other craniofacial malformations, most commonly cleft palate. Oropharyngeal teratoma may be also seen in association with frontonasal dysplasia and/or various degrees of craniofacial duplication. The nosology of these sporadic disorders is poorly defined. Case and Review We report on a 22-week fetus with a protruding nasopharyngeal teratoma, partial oral duplication, anencephaly, multiple costo-vertebral segmentation defects, and cervical diplomyelia. A review of the literature identified 48 patients published from 1931 to 2013 with co-existing clefting and duplication anomalies of the cephalic pole. Thoracic and abdominal midline anomalies were reported 13 times. Conclusion The term 'craniofacial teratoma syndrome' is introduced to define this phenotype as a recognizable developmental field defect of the cephalic pole. Developmental pathogenesis is discussed with a focus on pleiotropy and stereotaxis. The observation of midline findings suggestive of holoprosencephaly in a few previously reported cases suggests a role for the sonic hedgehog signaling pathway in this malformation pattern. Birth Defects Research (Part A) 103:554-566, 2015. © 2014 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Epignathus is a rare congenital head and neck tumor (1 in 35,000-200,000 live births) with many associated complications. We report a rare association of giant epignathus associated with disseminated intravascular coagulation. To our knowledge, only one case of this association has been reported so far. A review of different clinical presentations of epignathus, classification, its reported associations and available management strategies is included. [ABSTRACT FROM AUTHOR]