Epignathus (Palatal teratoma):A case report.

BACKGROUND: A case of a 2.7kg term baby with a large epignathus, a rare congenital teratoma arising from the palate is presented. Birth was by spontaneous vaginal delivery (SVD). The baby survived following a timely multidisciplinary approach to her management. This is the first reported case in Ghana.

Congenital peripheral developing odontoma accompanied by congenital teratomatous fibroma in a 9-month-old boy: a case report.

Peripheral odontoma is rare, and only two cases of congenital peripheral odontoma have been reported. Congenital oral fibroma is also rare. We describe a unique case of congenital peripheral developing odontoma accompanied by congenital teratomatous fibroma in an infant. Both tumors were difficult to detect on radiography. Two small masses were seen in the median anterior portion of the palatal mucosa of a 9-month-old boy. The masses had been present since birth and were surgically removed at age 28 months, when one of the masses had grown to a diameter of 8 mm. Histopathologic examination showed a fibrous lesion and a tooth germ-like rounded lesion composed of dental papilla, enamel organ, dentin, and cementum. Although congenital odontoma is rare, it should be considered when selecting appropriate treatment, as early radiographic detection is difficult.

Congenital hairy polyp of the soft palate.

Hairy polyp is an unusual developmental malformation that is most frequently seen as a pedunculated tumor in the neonate. They are benign lesions containing elements of both ectodermal and mesodermal origin. The symptoms of hairy polyps relate both to their location and their size. Larger lesions produce symptoms due to feeding difficulties and airway obstruction while smaller lesions cause intermittent symptoms resulting from a ball-valve type of obstruction. We present two cases of a soft palate hairy polyp causing respiratory and feeding difficulties and review the literature.

Report of rare palatal expression of Nevus of Ota with amendment of Tanino's classification.

Nevus of Ota, a benign melanocytic pigmentary disorder with rare malignant transformation potential, affects 0.014-0.034% of the Asian and Black population and has a strong predilection for females. It occurs in the area innervated by the first and second division of the trigeminal nerve. Oral manifestation is rarely noted with only 14 cases reported till date. This report documents a case of Nevus of Ota with the infrequently noted oral involvement, in an Indian lady. Since oral manifestation is not acknowledged in Tanino's classification, the authors propose a modification to the same.

Mushroom-shaped teratoma of the soft palate in a neonate: case report.

Teratomas of the head and neck are uncommon congenital lesions, which are rarely seen in the soft palate. We here reported a male neonate with a soft palate teratoma associated with soft palate cleft. The most common presenting symptom of oral teratoma is feeding problems. The tumour looks like a mushroom, and was successfully treated with surgery. Feeding returned to normal by removing the mass, and the patient is under follow up for selective operation of the cleft palate. This case is unique because teratoma in a mushroom shape was not reported before.

Reconstruction of a "double pathology" on a soft palate: hairy polyp and cleft palate.

Hairy polyps of the head and neck are rare congenital tumors that may cause upper aerodigestive tract impairment in newborn. Although cleft palate is a common congenital anomaly, hairy polyp with cleft palate is extremely rare in the newborn. We aimed to present the planning of reconstruction in a patient who has an incomplete cleft palate and defect due to hairy polyp excision on soft palate in this report. A 9-month-old child was admitted to our clinic due to cleft palate. In the clinical examination, incomplete cleft palate plus a defect on the posterior side of the left soft palate was observed. On the history, the patient had been operated urgently at the newborn period for a hairy polyp localized on the left soft palate which had obstructed the airway. Wardill-Kilner-Veau technique was modified for the reconstruction of the defect on the soft palate and incomplete cleft palate. No complication was seen on postoperative period. The speech ability was evaluated on the late postoperative controls after 3 years and adequate speech intelligibility was obtained. A case of hairy polyp of nasopharynx with incomplete cleft palate in a neonate and its reconstruction techniques are discussed. The etiology of hairy polyp, hamartoma, teratoma, dermoid cyst and epignathus, their nomenclature, histopathology, clinical features, and management in neonates are also discussed briefly.

Hemipalate deformity caused by a teratoma.

Congenital intraoral tumors derived from embryological germ layers are very rare. These lesions include teratomas, dermoids, epignathi, and teratoid tumors. These embryological neoplasms may interfere with normal development and elevation of the palatal shelves, resulting in a cleft palate. We report the case of a patient with an intraoral teratoma associated with a hemipalate deformity and describe the treatment that was carried out. The classification and sometimes the confusing terminology associated with these lesions are discussed, as are the principles of managing them.

A large teratoma of the hard palate: a case report.

Congenital teratoma is a rare malformation, and few papers have been published about it. We present a large teratoma that arose from the hard palate in a neonate. The obstructive mass caused maternal polyhydramnios and was identified prenatally by ultrasonography. The mother went into labour at 35 week's gestation at home. The child was in respiratory distress as a result of airway obstruction, and a tracheostomy was done when she was 4hours old. She also had major cardiac abnormalities. The palatal mass was removed successfully at 4 weeks of age. The typical components of a teratoma were identified including immature neural glial tissue.

Congenital osteolipoma associated with cleft palate: a case report.

Congenital tumors of the oral cavity are uncommon. Teratoid tumors account for most of the reported cases and are infrequently associated with a cleft of the soft palate. Of the remaining tumors associated with pediatric cleft palate, congenital lipoma is rare. Lipomas of the oral cavity may show cartilaginous or osseous changes. These changes have not been reported in lipoma associated with cleft palate. A rare case of congenital osteolipoma associated with cleft palate and showing osseous change is presented here. The pathogenesis of the lesion and the osseous metaplasia are described. The present case is the second case of congenital lipoma associated with cleft palate in the literature.

Management of a prenatally diagnosed huge teratoma arising from the soft palate.

Teratomas arising from the oral cavity are relatively rare and can cause life-threatening airway obstruction immediately after birth. We report a case of a huge teratoma arising from the soft palate detected prenatally. To save the patient, a caesarean section and ex utero intrapartum treatment (EXIT) were planned at 29 weeks of gestation. However, during EXIT, the patient's cardiopulmonary status deteriorated due to unstable cord blood flow secondary to uterine contractions. EXIT was abandoned, the patient was delivered and an emergency tracheotomy performed. The tumour was successfully excised 4 hours after tracheotomy. The tumour weighed 1,591 g and was 20 x 22 x 12 cm. The patient, a female, weighed 715 g. Histopathology showed Grade II teratoma. The postoperative course was uneventful and she is now 5 years old with no neurological sequelae. EXIT is indicated in patients who have a high risk for airway obstruction immediately after birth. However, if EXIT must be abandoned, as in this case, urgent tracheotomy is mandatory. Planned prevention of airway obstruction at delivery is indispensable for successful outcome and requires excellent coordination among obstetricians, neonatologists, anaesthesiologists and paediatric surgeons.

Epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma: report of a case.

A case of epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma is reported. A newborn female presented with a large skin-covered mass arising from the palate, cleft palate, and lobulated tongue with a soybean-sized lingual lesion. The palatal and lingual masses were removed on the 9th day after birth. A cystic lesion was also found on the nasal septum after removal of the palatal mass; this lesion was resected at its base. Glossoplasty was performed after excision of the lingual lesion. Histologically, the palatal mass was diagnosed as epignathus. The cystic lesion was suspected to be meningothelial tissues on the basis of hematoxylin-eosin (HE) and immunohistochemical staining: Vimentin and epithelial membrane antigen were positive, and factor VIII was negative. The mass of the tongue dorsum was diagnosed as hamartoma. No recurrence of epignathus or evidence of meningocele have been observed after 5 years of follow up.

Reconstruction of a congenital midpalatal hairy polyp.

Hairy polyps (dermoids or oronasopharyngeal teratomas) are a type of congenital tumour that contain elements of bigerminal origin (ectoderm and mesoderm). We describe a baby who presented with a hairy polyp of the hard palate protruding from the mouth. This was not causing respiratory distress but she had feeding difficulties. She was successfully operated on the age of 5 days, the defect was reconstructed with hairless skin of the mass and she was able to suck after a month.

Cleft palate with congenital midline teratoma.

A 4-year-old girl presented with hypernasal speech. On examination, in addition to velopharyngeal incompetence noted by speech examination, a palatal tumor was found in between the cleft palate. The computed tomographic and magnetic resonance imaging examinations were suspicious for midline teratoma. Total excision of the nasopalatal tumor was performed, and the pathology revealed benign teratoma. After 6 months of follow-up, no recurrence was noted. A two-flap palatoplasty with a superior-based pharyngeal flap was then performed to reconstruct the palatal defect and to correct the velopharyngeal incompetence. An anterior oronasal fistula developed after the operation, but a tongue flap was transferred to cover the defect successfully. The purpose of this case report is to present the relationship between a congenital midline nasopalatal tumor and cleft palate.

Lipoma of the cleft soft palate: a case report of a rare congenital anomaly.

OBJECTIVE: Congenital tumors of the oral cavity are extremely uncommon. The teratoid tumors (epiganthi, dermoid, and hairy polyps) account for almost all of the reported cases and are not infrequently associated with cleft of the soft palate. Of the remaining tumors in the pediatric cleft palate population, infantile lipoma of the oral cavity is exceedingly rare. A case of a congenital lipoma associated with a cleft of the soft palate is presented. The review of the literature, the description of the lesion, the diagnosis, and the management of this finding are outlined.

Antenatal sonographic diagnosis of epignathus at 15 weeks of pregnancy.

Epignathus is a rare, benign, congenital teratoma of the hard palate. Most of these teratomas are unidirectional and protrude through the mouth. Hence, the prognosis depends on the size of the tumor and the degree of face distortion and airway obstruction that it causes. However, some epignathi protrude bidirectionally, involving and destroying the brain tissue, resulting in a poor prognosis. This report presents a case of ultrasonographic detection of a bidirectional epignathus at 15 weeks of pregnancy.

Duplication of pituitary gland.

We present a case of duplication of the pituitary gland with associated clefts of the hard palate and body of the sphenoid and a midline palatine dermoid. This is an extremely rare malformation with only six previous case reports in the literature. Imaging findings are discussed and literature reviewed for possible morphogenesis, including its association with partial twinning and midline cleft face syndrome.

Oral teratoma (epignathus) with intracranial extension: a report of two cases.

Epignathi are unusual congenital tumours presenting as oropharyngeal masses, often resulting in rapid asphyxia following birth. Occasionally, intracranial extension of the tumour is present, and two such cases are described. The presence of this complication, diagnosable by ultrasound examination, indicates that aggressive surgical treatment is inappropriate.