Nasopharyngeal Dermoids and Cleft Palate.

Nasopharyngeal dermoids associated with cleft palate present as intraoral protruding masses. Only 5 cases of nasopharyngeal dermoids associated with cleft palate have been reported in the literature. We are reporting 4 such cases encountered by us in the last 10 years in our series of 900 cleft palate surgeries. Imaging studies were done to know the extension of dermoid and to look for any associated congenital intracranial anomalies. We observed that nasopharyngeal dermoids are usually nonmalignant and can be easily managed by complete local excision followed by palatal closure after 6 months.

An unusual cause of inspiratory stridor in the newborn: congenital pharyngeal teratoma--a case report.

BACKGROUND: Neonatal inspiratory stridor is an important examination finding that requires immediate and adequate evaluation of the underlying etiology. Depending on the severity of the airway obstruction and the presence or absence of associated symptoms such as respiratory distress and feeding problems, early initiation of a complete diagnostic workup can be crucial. The most common cause of neonatal inspiratory stridor is laryngomalacia, however, several differential diagnoses need to be investigated. More rare causes include oral or laryngeal masses. Teratomas of the head and neck region are one of the most unusual causes of respiratory distress during the neonatal period. We present a case of a mature teratoma in the oropharynx presenting with airway obstruction in a newborn infant. CASE PRESENTATION: A four-day-old female Caucasian infant was admitted to the neonatal intensive care unit of our hospital because of inspiratory stridor and profound desaturations while feeding. Diagnostic workup by ultrasound, magnetic resonance imaging and flexible endoscopy revealed a pediculated lesion in the pharyngeal region causing intermittent complete airway obstruction. The mass was surgically removed by transoral laser resection on the seventh day of life. Histological evaluation was consistent with a mature teratoma without any signs of malignancy. The further hospital course was uneventful, routine follow-up examinations at 3, 6 and 9 months of age showed no evidence of tumor recurrence. CONCLUSION: Neonatal stridor is a frequent symptom in the neonatal period and is mostly caused by non-life-threatening pathologies. On rare occasions, however, the underlying conditions are more critical. A careful stepwise diagnostic investigation to rule out these conditions, to identify rare causes and to initiate early treatment is therefore warranted.

Prenatal diagnosis and treatment of craniomaxillofacial anomalies.

Many advances in health care are built on the evolution of technology. An entirely new patient has emerged in fetal medicine, with these advances in prenatal imaging allowing one to see and diagnose disease not previously appreciated. Clinicians can better plan for the delivery of the neonate, with identified anomalies being optimally managed and the impact on the neonate's health minimized. The oral and maxillofacial surgeon offers expertise in the management of craniomaxillofacial anomalies, including congenital tumors, facial clefts, craniosynostosis, micrognathia, and other congenital abnormalities. The techniques for perinatal care of the patient with craniofacial abnormalities continue to evolve as the technology improves. The authors describe their experience and some of the more common abnormalities with their management considerations that may be encountered by the oral and maxillofacial surgeon on the fetal diagnosis and treatment team.

Extragonadal teratomas presenting at birth and originating from 3 different sites--a report of 3 cases.

Three cases of extragonadal teratomas presented at birth and all the cases arisen from three separate sites are reported in the present study. A huge sacrococcygeal teratoma is being reported and the other two retroperitoneal and nasopharyngeal teratomas, are reported for the rarity of their location in neonatal period. Teratomas of all three babies were mature benign on histopathology and surgical removal sufficed as mode of treatment. No recurrence was noted on follow-up.

Prenatal diagnosis and management of fetal pharyngeal teratoma: a case report and review of the literature.

We present a case of a fetal pharyngeal teratoma, which was diagnosed at 21 weeks' gestation. At the time of examination, a mass of mixed echogenicity was detected that protruded through the mouth. During a second examination 3 weeks later, the tumor had increased in size, and a severe polyhydramnios had developed. Intrauterine death of the fetus was detected at 27 weeks' gestation. Labor was induced with misoprostol, and a 1,015-g stillborn female neonate was delivered. Postmortem examination confirmed the diagnosis of a pharyngeal teratoma.

Lymphatic malformation of the parapharyngeal space.

Lymphatic malformations are rare benign congenital lesions. A 28-month-old girl presented with a parapharyngeal lymphatic malformation. A cystic lesion had been diagnosed at 18 weeks' gestation by ultrasonography and she had been followed up conservatively until her referral to our department. We used a transcervical approach to excise the parapharyngeal lesion and 1 year later, there was no sign of recurrence.

Teratomas of the head and neck region.

BACKGROUND: Teratomas are exceptionally rare malformations in the head and neck region. They are mostly benign but as a direct result of their rarity, most clinician's experience of these tumours is very limited, and consequently most of the associated literature consists of single case reports. In this paper, however, all the cases managed by a major Craniofacial Unit (the Australian Craniofacial Unit) were reviewed to attempt to identify common problems encountered in their management. MATERIAL AND METHODS: All cases managed by the Australian Craniofacial Unit over the last 25 years were reviewed. In total a series of nine cases was identified, but two were seen and operated on in overseas centres and the data in these cases were incomplete, and have been excluded from the study. Case note, radiology and pathology review was undertaken to collect data. RESULTS: In total a series of seven cases was identified as suitable for inclusion in this study. Six of these have had a minimum of 9 years follow-up, three having completed growth. CONCLUSION: The initial and subsequent management demonstrates that these tumours when benign can be successfully removed, but depending on the affected site may require continued multidisciplinary management until growth has finished.

Teratoma of the tongue in neonates: report of a case and review of the literature.

A female newborn presented with a huge mass protruding from the mouth. She had no respiratory distress but had difficulty swallowing. The mass originated from the tongue and was completely extirpated on the day of birth. The histologic diagnosis was mature teratoma. Three days later, another mass measuring 1 cm in diameter was found attached to the upper pharyngeal wall and was also completely extirpated 20 days after birth. The histologic diagnosis was also mature teratoma. The infant had a complete cleft palate, but no other malformation was found. Two years after surgery there is no sign of recurrence.

Perinatal management of a lingual teratoma.

BACKGROUND: Teratomas of the head and neck are rare and occur almost exclusively in neonates. Prenatal diagnosis of these tumors allows for a carefully planned delivery that maintains an open airway and potentially improves perinatal outcome. We report the perinatal management of a huge intrapharyngeal and intra-oral teratoma that had a broad connection to the base of the tongue. CASE: An anterior neck mass (5 x 5 cm) in an otherwise normal-appearing fetus was detected at 19 weeks' gestation. The mass increased in size over the next 10 weeks to 8 x 6.8 x 4.3 cm. Marked fetal head deflexion was noted along with concomitant hydramnios. The mother had spontaneous rupture of membranes with preterm labor at 29 weeks' gestation. A 1860-g male neonate was delivered by classical cesarean delivery. A tracheostomy was performed in the delivery room for ventilation after the upper airway could not be accessed by bronchoscopy. Histologic examination after surgical excision confirmed a congenital teratoma with immature neuroectodermal tissue and alpha-fetoprotein-bearing endodermal sinus tumor components with exclusively polyvesicular vitelline characteristics. The origin of the pharyngeal mass was the base of the tongue. CONCLUSION: The prenatal diagnosis of a pharyngeal teratoma should prompt a careful delivery plan to optimize perinatal outcome.

Extracerebral intracranial glioneural hamartoma with extension into the parapharyngeal space.

A newborn had an extracerebral, intracranial mass extending from the right middle cranial fossa through the base of the skull to the parapharyngeal space. The mass was predominantly composed of immature brain tissue. It was enclosed by its own leptomeninges and dura and was classified as a glioneural hamartoma.

Advanced practice case study: congenital hemangiomas.

Congenital hemangioma is the most common tumor of infancy. Usually it occurs singly in the skin. Occasionally multiple tumors are present and can involve other body organs. Congenital subglottic hemangioma is considered rare, but when it occurs can be life threatening. This article discusses the different options available for treatment of subglottic hemangioma and the course of treatment chosen for Baby S. taking into consideration her diagnosis, family situation, and the availability of an experimental drug therapy.

Radiographic manifestations of congenital anomalies affecting the airway.

Congenital anomalies of the airway are generally uncommon, but a vast array of possibilities exists. Some present life-threatening emergencies at birth, and others go undiagnosed for years. Clinical symptoms are often nonspecific, and radiographic evaluation is frequently requested to localize and characterize the lesion before endoscopy, surgery, or medical management. The most common intrinsic congenital anomalies causing airway compromise in infants include choanal atresia, mandibular hypoplasia, laryngomalacia, vocal cord paralysis, and congenital subglottic stenosis. The radiologist must be on the alert for unsuspected additional anomalies involving the airway, lungs, and esophagus, which occur with relative frequency. Numerous extrinsic congenital masses of the head, neck, and mediastinum may compromise the airway. Hemangiomas, lymphangiomas, and teratomas are more likely to be noted at birth, whereas branchial cleft cysts, thyroglossal duct anomalies, and dermoid cysts frequently present later. Mass location and radiographic characteristics usually allow accurate preoperative diagnosis. Intracranial involvement by nasal or nasopharyngeal masses, intrathoracic involvement by lower neck mass, and intraspinal involvement by posterior neck mass must always be sought for radiographically. Persistence of respiratory symptoms after removal of such masses is not uncommon because tracheal deformity and laxity may take months or years to resolve. Recent advances in cine CT and MR imaging promise to improve imaging of the airway in general and in the pediatric population in particular. Significant limitations in imaging of the pediatric larynx remain. As a result, endoscopy continues to be the primary diagnostic tool for airway anomalies of this region.

A pharyngeal hairy polyp causing neonatal airway obstruction: a case study.

A case of neonatal respiratory distress due to an oropharyngeal hairy polyp is reported with its MRI assessment. The place of hairy polyps in the differential diagnosis of upper respiratory tract obstruction is discussed. Early and rapid diagnosis and treatment may be lifesaving for the newborn.

Per-oral excision of a branchial cyst.

The treatment of branchial cysts is by surgical excision. We describe a case where the cyst was medial to the pharyngeal constrictors, making a per-oral approach the safest, and easiest method of removal. The position of the cyst supports the theory that branchial cysts are congenital in origin.

Retropharyngeal neuroblastoma causing airway obstruction in a newborn--survival with surgical treatment alone.

Neuroblastoma is known to occur occasionally in newborns, but very few are of cervical origin. We have encountered a newborn with retropharyngeal neuroblastoma causing airway obstruction, and successfully treated it by surgical removal alone.

Retropharyngeal masses in infants and young children.

Six patients are described who illustrate different causes of retropharyngeal mass lesions in infants and young children. Differential diagnosis, clinical history, physical examination, and adequate roentgenographic evaluation of the neck are important.