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We describe a rare association of hypoplastic left heart syndrome, trismony 18, and epignathus in a 970 g female twin born at 37 weeks of gestational age. She died at 24 hours of age. Neonatologists and obstetricians should be aware of this rare association for a thorough prenatal counselling.

Abstract: Teratomas are congenital malformations that are rarely located in the head and neck region. We report a case of congenital teratoma of the oral cavity, which was causing an airway obstruction and was treated at the time of birth. This teratoma was discovered at 27 gestational weeks by ultrasonography. A multidisciplinary team was consulted for antenatal diagnosis; the options of therapeutic abortion or management of the birth with the prevention of respiratory distress were debated. However, preterm labour at 32 gestational weeks accelerated the parental and the medical decisions. The parents agreed to the birth. The various disciplines coordinated their work, and the predefined treatment plan for clearing the airway obstruction was applied to manage the birth. The reestablishment of patency of the airway was performed during delivery and removal of the tumour was performed immediately afterwards. The follow-up of this case over 3 years is also presented. [Copyright &y& Elsevier]

Epignathus is a very rare fetal tumor. We report a case of fast-growing giant epignathus with severe distortion of the right part of the face and orbit. A thorough prenatal work-up was performed by the association of Magnetic Resonance Imaging and Ultrasonography. A multidisciplinary approach was crucial to assess the operability and provide careful counseling to help parents understand and reach decision. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: To present our experience in the perinatal management of fetuses with large oropharyngeal tumors by ex utero intrapartum treatment (EXIT). Methods: We performed a retrospective chart review of all patients with congenital oropharyngeal tumor who underwent an EXIT procedure between May 2006 and June 2012. Results: Four patients were included in the series, three females and one male. The diagnoses were epignathus (n =2) and congenital epulis (n =2). Three EXITs were done at term and one at late preterm due to premature rupture of membranes. Median maternal time under anesthesia was 185min (range: 166–281) and median maternal operative time was 99min (range: 85–153). Median maternal blood loss was 550ml (range: 350–2000); one mother required a blood transfusion. Mean maternal hospital stay was 4days. Median hysterotomy-to-cord clamp time was 24min (range: 18–66). Mean fetal birth weight was 2.7kg (range: 2.4–3). The airway was successfully accessed and secured under placental circulation in all cases. In the two patients with congenital epulis the tumors were resected at the base of their pedicles and the airway accessed via direct laryngoscopy before the umbilical cord was clamped. One patient with epignathus underwent a retrograde tracheal intubation under placental circulation and had the tumor resected thereafter. The second patient with epignathus had a tracheostomy done under placental circulation and then had tumor debulking immediately after the EXIT. The maternal morbidity was minimal and there were no mortalities. Conclusions: We conclude that the EXIT procedure is the ideal delivery strategy for fetuses with prenatally diagnosed oropharyngeal tumors and potential airway obstruction at birth. Patients with prenatally diagnosed oropharyngeal tumors should be promptly referred to a fetal treatment center with a dedicated multidisciplinary team and EXIT capabilities. [Copyright &y& Elsevier]

A case of sudden cardiopulmonary arrest in a 3-month-old girl is presented. The patient had barely recovered from hypoxic encephalopathy when she presented with repeated respiratory distress. Computed tomography and endoscopic analysis revealed a shiny polyp in the lateral wall of the nasopharynx, and this polyp was suspected to be the main cause of respiratory distress. After referral to our hospital, surgical removal was performed, and the histopathological diagnosis was hairy polyp. Hairy polyp is a rare congenital benign tumor that sometimes induces respiratory distress. This polyp can potentially induce a life-threatening event. In a systematic review of 40 reported cases, polyps of ≤3.0 cm in diameter have a higher risk of respiratory distress than do those >3.0 cm in diameter ( P = 0.01). Small hairy polyps may be lethal because of delayed diagnosis. To locate small hairy polyps, physicians should not hesitate to perform further examination because there is the possibility of oversight with only physical examination. [ABSTRACT FROM AUTHOR]

We report a female infant with presentation of epignathus teratoma involving duplication of both the mandible and tongue. Epignathus with duplication of the mandible has rarely been reported in the literature thus far. The location and extent of the tumor, as well as the involvement of adjacent structures, resulted in trismus and upper airway obstruction at birth. Thus, staged operations including debulking and correction of anatomical anomaly were performed on this patient after life-saving tracheostomy. As a result, we not only prevented morbidity associated with the anomaly but also refined the patient's appearance and improved her quality of life. [ABSTRACT FROM AUTHOR]

Epignathus of a fetus is an extremely rare type of an oropharyngeal teratoma that commonly arises from the palate and often grows in the prenatal period, leading to polyhydramnios due to difficulty in swallowing before birth and a high mortality due to the airway obstruction after birth. We report here a very rare case of a fetus with an epignathus decreasing in size naturally in the prenatal period with a favorable prognosis. In contrast to previously reported cases of epignathus that grew rapidly in the prenatal period, color Doppler fetal ultrasound showed a hypovascular tumor in our case. Moreover, histopathologic examination revealed an epignathus with necrotic areas inside. Based on our experience, a hypovascular epignathus demonstrated by color Doppler fetal ultrasound may be an indicator of a favorable prognosis. [ABSTRACT FROM AUTHOR]

A congenital teratoid tumor arising and protruding through the mouth is classified as epignathus or fetus in fetu. On review of literature, we found various reports of midline mandibular and lower lip cleft associated with flexion contracture of neck, midline cervical cord, but there is only one report of association with midline dermoid. We present an unusual case of midline cleft of mandible with an epignathus. A 2.3-kg male child, delivered transvaginally in the 38th gestational week, was referred to us for management of a large irregular growth hanging outside the mouth. On examination, he had a wide median cleft of the mandible with tongue adherent to the "V"-shaped defect in the area of lower lip. A midline irregular mass of size 12 x 8 x 5 cm with variegated consistency was arising in the midline from the floor of the mouth between the tongue and lower lip. X-ray and computed tomography scan showed a rounded soft tissue mass arising from the alveolus with multiple calcifications within it along with a large triangular calcification and absence of hyoid bone. The mass was excised by mobilizing the tip of tongue. Staged repair was planned for the defect in the mandible. Unfortunately, the baby succumbed postoperatively to complex congenital heart disease. Histopathology was suggestive of epignathus. We discuss hereby the embryology and current management strategies of the problem. [ABSTRACT FROM AUTHOR]

Teratomas are benign tumors containing cells from ectodermal, mesodermal and endodermal layers. They occur in about 1 in every 4,000 births and most commonly in the sacrococcygeal region, followed by the ovaries. Congenital epignathus teratomas are rare embryological neoplasms localised in the region of head and neck. An epignathus is found in approximately 1:35,000-1:200,000 live births. This accounts for 2-9% of all teratomas. Size and location of the neoplasm in the oronasopharynx is variable. Teratomas are partly undiagnosed at the time of birth. They may exist with an intracranial extension or as small polyps. Large epignathi can lead to difficult management during and after birth. We present a case of a female infant with a combination of nasopharyngeal teratoma and a cleft palate with successful multidisciplinary management via a staged surgical approach. The epignathus presented as a huge mass extending out of the mouth of the infant girl. On the day of birth debulking of the extraoral portion of the tumor, followed by intraoral extirpation and cleft palate repair at a later stage, was performed. The results of the histopathological examination confirmed diagnosis of a congenital teratoma. [ABSTRACT FROM AUTHOR]

Abstract: We present a case of a female infant that was diagnosed on prenatal ultrasound with an oral mass, and subsequently underwent a planned EXIT procedure to secure the airway. Ultimately the epignathus teratoma was excised at 3 days of age. The number of cases in which neonates have survived epignathus teratomas has increased in the past decade due to improved prenatal imaging and the development of the EXIT procedure. This case demonstrates the importance of a multidisciplinary approach that must be established when a prenatal diagnosis raises concern for airway compromise, and the thorough work-up required for definitive management. [Copyright &y& Elsevier]

The article presents a case report of a 16-week-old fetus suspected to have epignathus during ultrasound examination because of the finding of a persistently open mouth. Postmortem MRI and pathologic examination of the fetus at 20 weeks confirmed the diagnosis. The article discusses epignathus, a rare form of benign congenital teratoma, with intracranial extension on a 16-week old fetus.

Introduction: The aim of the study was to produce a systematic review about etiology, pathology, diagnosis, prognosis and clinical management regarding oral and cervical teratomas. Materials and methods: A systematic review of Pubmed/Medline using the following keywords was made: epignathus, cervical teratoma, fetus, oral teratoma, prenatal diagnosis, prognosis, treatment, ultrasound. Conclusion: The following clinical conclusions can be reached: (1) teratomas are rare, usually benign congenital tumors which recognized multifactorial etiology; (2) prenatal ultrasound diagnosis can be made early in pregnancy (15–16 weeks); (3) 3D ultrasound and MRI may enhance the accuracy of the antenatal diagnosis (location, extension and intracranial spread) and may aid in the selection of patients requiring treatment; (4) prenatal karyotype and search for associated abnormalities is mandatory in all teratomas; (5) delivery should involve elective Cesarean section with ex utero intrapartum treatment procedure or resection of the tumor mass, which may be performed on placental support operation on placental support procedure to increase the chances of postnatal survival. [ABSTRACT FROM AUTHOR]

Abstract: Teratomas are embryonal tumours composed of diverse tissues from three germinal layers with variable levels of maturity. The authors report a female patient with a large immature epignathus. Prenatal diagnosis permitted a caesarean section and tracheotomy to be planned under ex utero intrapartum treatment for airway obstruction. The tumour was successfully controlled, even though it was impossible to distinguish from normal tissue because it lacked a pedicle and capsule, using multidisciplinary therapy, including a series of surgical treatments and adjuvant chemotherapy. This case suggested that the level of serum alpha fetoprotein might be a useful indicator after surgery. At present, after 4 years, no regrowth has been observed and the patient has no problems with respiration or swallowing. [ABSTRACT FROM AUTHOR]

A prenatal ultrasound diagnosis of epignathus in a dichorionic-diamniotic twin pregnancy is reported. A complex mass protruding from the fetal face was seen at week 19. Amniocentesis resulted in a 46,XX fetus with elevated alphafetoprotein (a-FP). An increase in tumor size and severe polyhydramnios ensued. Selective feticide performed at 22 weeks led to untreatable uterine contractions with iatrogenic abortion and early neonatal mortality of the healthy cotwin. Without development of polyhydramnios and tumor growth, weekly scan and transvaginal cervical assessment would have been carried out and cesarean section planned at around 32 weeks. Necroscopy and histology aided the ultrasound-based prenatal diagnosis. [ABSTRACT FROM AUTHOR]

Introduction: epignathus is a congenital teratoma of the oropharyngeal cavity, is presented as a mass of tissue is exposed on the outside through the oral cavity, the histology is benign and can cause severe obstruction of the airway and / or digestive tract. At birth it is essential to secure the airway and then removed the tumor. We present this case because it is very rare. Case report: A newly born female, obtained via cesarean for prenatally detected tumor apparently chin, the mouth showed tumor protrusion. He secured the airway with Guedel airway. Establishing a diagnosis of intraoral teratoma: Epignatus. The histopathological report was intraoral teratoma. The evolution to date is satisfactory with 6 months follow up. Discussion: epignatus is a teratoma of the oral cavity is rare and protrudes through the mouth. It is a benign, but mortality is high because it causes airway obstruction at birth. Prenatal diagnosis can be established with USG or MRI, and treatment is surgical resection. Because of its benign histology is virtually free from recurrence. [ABSTRACT FROM AUTHOR]

We present a case of epignathus (oral teratoma) with midline intracranial associated malformations. This is a rare report of epignathus diagnosed in utero with fetal MRI description of these midline brain malformations, including duplication of the pituitary gland. This case is an example of how these intracranial findings led to the correct diagnosis of a small fetal oral mass. [ABSTRACT FROM AUTHOR]

The article presents the case of a 26-year-old obese woman who was diagnosed with polyhydramnios and a large encephalocele at almost seven months of gestation. She was admitted into preterm labor, resulted in cesarean section, and born a female infant. The infant passed though a physical examination and revealed epignathus or a large mass protruding from her mouth that attached to the palate.

Epignathus is an oropharyngeal teratoma, which has an incidence of 1:35000 to 1:200000 in those born alive. This oropharyngeal teratoma arises from the base of the skull or of the posterior wall of the pharynx and it extends inferiorly causing partial or complete obstruction of the airway and digestive passage. That is the reason why prenatal diagnosis is important to establish a suitable and immediate handling plan to improve the survival of the newborn. A case of epignathus in a woman in her first pregnancy and in the 32nd week of gestation reported and to which the diagnosis was made prenatally through an ultrasound and corroborated at birth. [ABSTRACT FROM AUTHOR]

Teratomas are complex lesions composed of diverse tissues from all 3 germinal cell layers and may exhibit variable levels of maturity. Head and neck teratomas are most commonly cervical with the oropharynx (epignathus) being the second commonest location. In this article, clinical presentation, behaviour and associated significance of head and neck teratomas have been highlightened. Because of their obscure origin, bizarre microscopic appearance, unpredictable behaviour and often dramatic clinical presentation, a dental surgeon or a dental specialist should be aware of their clinical presentation and pathology. [ABSTRACT FROM AUTHOR]

Background: Epignathus is a rare form of congenital teratoma, originating from the base of the skull, most commonly the hard palate, or mandible. It has been associated with a poor prognosis due to complications including polyhydramnios and respiratory compromise at birth as a consequence of upper airway obstructions. It is usually not associated with chromosomal aberrations. We present a case of prenatally diagnosed epignathus associated with a gonosomal pentasomy 49,XXXXY. Case: A 34-year-old gravida 1, para 0 was referred to our unit with a sonographically suspected gastroschisis at 26+6 weeks’ gestation. A detailed ultrasound scan revealed a large mixed echogenic mass seen in continuation with the mouth in the midline. Based on the appearance, an epignathus was suspected. No other fetal anomalies were detected. Karyotyping showed a 49,XXXXY karyotype of the fetus. The couple decided to continue the pregnancy after detailed counseling about results and prognosis. A cesarean section was necessary and performed at 29+0 weeks’ gestation due to a pathological Doppler and cardiotocogram. Because of the enormous epignathus intubation of the newborn was not possible. A tracheostomy was performed for ventilation and oxygenation, which failed and the newborn died 30 min after birth. Conclusion: Prenatal diagnosis by ultrasound has improved perinatal management. This should include assessment of the tumor size and spread in order to establish an accurate prognosis and to anticipate likely problems which are to be encountered during pregnancy or at the time of delivery. To our knowledge, this is the first reported case of a prenatally diagnosed epignathus with a gonosomal pentasomy 49,XXXXY. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

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. [Copyright &y& Elsevier]

Malignant cervical teratoma (MCT) usually appears in newborns as an enlarging mass of the neck that causes respiratory distress, requiring prompt airway control. We report a case of MCT in an infant electively delivered at 32 weeks to prevent airway impairment. At first, the preoperative diagnosis was hygroma of the neck, and a surgical excision was performed when the newborn was 9 days old. Diagnosis was benign extragonadic immature teratoma, but it was changed in MCT when cervical metastases appeared and the alpha-fetoprotein (AFP) level increased. Subsequent surgical procedures and chemotherapy were necessary. The child has been free from disease and healthy for 7 years since the last surgery. The preoperative diagnosis of MCT is difficult because of its rarity and non-specific clinical findings. Surgical excision is required for an adequate cure and airway repair; a long-term follow-up is mandatory to promptly treat any recurrence. [ABSTRACT FROM AUTHOR]

Recent attempts at predelivery management of obstructed fetal airways have focused on the EXIT (ex-utero Intrapartum treatment) procedure, which allows sufficient time to secure the fetal airway through preservation of utero placental gas exchange. We report a fetus with an exophytic oral tumor noted at 34 weeks of gestation. In this case, three-dimensional (3D) ultrasound allowed a complete and interactive evaluation of the tumor and related facial anatomy, and confirmed that access to the fetal airway was unlikely during delivery. Fetal magnetic resonance imaging (MRI) further demonstrated that the upper originated in the nasopharynx and obstructed the upper airway. Both imaging results led to a final decision to offer an EXIT procedure for the neonate. At 36 weeks' gestation, a successful EXIT procedure was performed to reduce the risk of respiratory distress immediately after birth. This report highlights the value of 3D ultrasound and MRI as essential prerequisites for optimization of the triage process in selecting EXIT candidates. [ABSTRACT FROM AUTHOR]

AbstractAn epignathus is an extremely rare form of teratoma that arises from the oral cavity, most commonly from the palate. We describe a case identified sonographically at 17 weeks’ gestation after a raised maternal serum alpha-fetoprotein was recorded. This pregnancy was terminated but we review the literature over the last 10 years and describe the management options available in such cases. [ABSTRACT FROM AUTHOR]

Teratomas are tumors formed by pluripotential primordial germ cells which constitute heterogeneous types of tissues foreign to the organ from which they originate. Rarely however, an extragonadal presentation occurs. Origin in the gonads is most common. An infant with an incomplete cleft palate, and a 20 × 10 × 10 cm mass protruding from the mouth is presented. The lesion had a pedicle 20 cm in length, its base being attached to the hard palate. Soon after birth, surgical excision was performed. Histopathological examination revealed a teratoma containing mature elements from all 3 germinal layers without evidence of malignant change. The pathogenesis and the prognosis of this rare tumor are reviewed. [ABSTRACT FROM AUTHOR]

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Abstract: A male infant was born to a 24-year-old mother (gravida 1 para 1) by cesarean delivery at 33 1/7 weeks of gestation. The physical examination revealed a large mass protruding from the baby''s mouth, which appeared to be attached to the palate. Tracheostomy was performed immediately in the delivery room. A partial surgical excision was performed on the second postnatal day, removing most of the teratoma (epignathus), which was attached to the back of the pharynx and protruding from the baby''s mouth measuring 13×11×9 cm and weighing 545 g. The final pathological diagnosis was “malignant epignathus with nephroblastoma component.” According to our knowledge, this is the first case that have malignant epignathus including nephroblastoma component in the literature. [Copyright &y& Elsevier]

Epignathus is a rare congenital orofacial teratoma. We present a case of a fast-growing tumor, where early prenatal diagnosis was made and where fetopathological examination revealed the reason of the remarkable ultrasonographic signs and underlined the expected poor prognosis. Ultrasonographic examination at 18 weeks' gestation showed that there was a growing tumor protruding from the fetus's mouth. The fetal stomach could not be seen and extreme polyhydramnios was also detected. After counseling, the couple opted for a termination of pregnancy. Fetopathological examination showed that the tumorosus mass was not only protruding from the mouth, but also inexplicably grew downwards, was connected to the hard palate and the periosteum of the vertebral corpus, making an airway and esophageal obstruction, causing the ultrasonographic findings. Postnatal treatment and surgical removal of this tumor seemed to be impossible. In case of an early detection of a fast-growing fetal epignathus, pregnancy termination should be considered. [ABSTRACT FROM AUTHOR]

Abstract: We report two original cases of association of cleft palate and lateral cervico-facial teratoma. We discuss the embryological explanation. The first child presented a cleft palate associated with two cervico-facial localisations of teratoma. The other had Pierre Robin sequence associated with lateropharyngeal teratoma with an extra sub maxillary localisation. Most reported cases were of midline teratomas, leading different authors to advance a mechanical origin to the cleft. Our cases are different: we could hardly find reported cases of associate lateral tumours and cleft palate, which would suggest two different embryologic mechanisms, or at least a combination of more complicated phenomenons. [Copyright &y& Elsevier]

Teratoma of the maxilla is a rare entity. Congenital intraoral teratoma occurs in 1:4000 births. It is a benign tumor, although malignancy has been described in adults. A 10-year-old male child with this condition is described in this report. This case illustrates a huge mass on the right side of the maxilla. The mass was excised under general anesthesia. Histopathologically, it consisted of all three layers of embryonic elements with predominantly fibrous tissue. Postoperative result was uneventful and no recurrence was detected after 2 years. [ABSTRACT FROM AUTHOR]

Summary: Nasopharyngeal teratoma is a rare teratoma arising anywhere from the oronasal cavity, regarded as an expanding, cavity-filling lesion, with a high mortality rate due to severe airway obstruction, especially in the neonatal period. We report a patient with a single, firm, hairy swelling arising from vomer, protruding into the oral cavity associated with cleft palate and an inguinal hernia. Excision of the lesion and reconstruction of the cleft palate was successfully performed in a single stage. To the best of our knowledge, this is the first report of a child with a nasopharyngeal teratoma, cleft palate and an inguinal hernia. [ABSTRACT FROM AUTHOR]