Ex-utero Intrapartum Treatment (EXIT) Procedure for Giant Fetal Epignathus.

BACKGROUND: Large fetal oropharyngeal tumors are rare, and have the potential to cause airway obstruction during birth. CASE CHARACTERISTICS: A 35-year-old woman with antenatally diagnosed large heterogenous mass in fetal neck displacing trachea and filling up the orophanygeal space. INTERVENTION: The infant was delivered at 31 weeks of gestation by ex-utero intrapartum therapy procedure to secure the airway. OUTCOME: Tumor was resected successfully on day 8 of life. Histopathology revealed mixed teratoma. MESSAGE: Ex-utero intrapartum therapy for fetuses with severe upper airway compromise may prove life-saving.

Perinatal management of congenital oropharyngeal tumors: the ex utero intrapartum treatment (EXIT) approach.

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 185 min (range: 166-281) and median maternal operative time was 99 min (range: 85-153). Median maternal blood loss was 550 ml (range: 350-2000); one mother required a blood transfusion. Mean maternal hospital stay was 4 days. Median hysterotomy-to-cord clamp time was 24 min (range: 18-66). Mean fetal birth weight was 2.7 kg (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.

Oropharyngeal true teratoma.

Oropharyngeal teratoma in newborn is very rare. Here we report a case of oropharyngeal true teratoma where a 17-day-old female baby presented with a protruding mass from oropharynx with episodic respiratory distress and feeding difficulty complicated by aspiration pneumonia, and treated successfully with coordinated team approach.

Two unusual cases of pharyngeal hairy polyp causing intermittent neonatal airway obstruction.

Hairy polyp of the pharynx is an uncommon developmental malformation that is most frequently seen as a penduculated tumour in the neonate. The clinical presentation is characterized by the presence of a polypoid mass protruding through the mouth as 'a second tongue' causing respiratory distress. Two patients are presented with this condition.

Oropharyngeal tumor in the newborn: a case report.

BACKGROUND: An oropharyngeal tumor presenting as a protruding mass from the mouth of the newborn infant is very rare. This mass has the potential to obstruct the airway and requires urgent medical management, diagnosis and appropriate surgical management. OBJECTIVES: To report a case of a newborn with an oropharyngeal mass presenting at birth and focus on key clinical issues for the physician faced with the care of such an infant. METHODS AND RESULTS: We describe a newborn infant who was born with a large mass protruding from the oral cavity at birth, without respiratory distress. The mass was surgically removed with no complications. The histology of the mass revealed it to be a pharyngeal dermoid polyp, also called 'hairy polyp'. The infant did well after tumor removal and was discharged home within a couple of days. CONCLUSIONS: We report a case of a newborn with a pharyngeal dermoid polyp presenting as an oropharyngeal mass.

[Two cases of congenital airway obstruction managed with ex utero intrapartum treatment procedures: anesthetic implications]. / Dos casos de obstrucción congénita de la vía aérea sometidos a E.X.I.T. ("ex-utero intrapartum treatment"): implicaciones anestésicas.

An ex utero intrapartum treatment (EXIT) procedure provides sufficient time to gain control of the potentially obstructed fetal upper airway while uterine placental circulation is maintained during cesarean section. We report 2 cases in which fetal congenital upper airway obstruction was managed without complications during EXIT procedures. We also discuss general considerations concerning the obstetric patient and the performance of intramuscular fetal anesthesia. Before the hysterotomy, sevoflurane at 1.5 minimum alveolar concentration was administered to assure sufficient uterine relaxation during EXIT. The 2 parturients remained hemodynamically stable during the procedure and uterine and placental perfusion was adequate. Nasotracheal intubation was possible in 1 fetus after a cervical mass was dissected. In the other, a tracheostomy was created. After the umbilical cord was clamped, the concentration of sevoflurane anesthetic gas was reduced and oxytocin and methylergometrine were administered to induce adequate uterine contractions within a few minutes. Both neonates survived the EXIT procedure with no complications.

Dos casos de obstrucción congénita de la vía aérea sometidos a E.X.I.T. ("ex-utero intrapartum treatment"): implicaciones anestésicas / Two cases of congenital airway obstruction managed with ex utero intrapartum treatment procedures: anesthetic implications

El procedimiento E.X.I.T. (“Ex-Utero Intrapartum Treatment”) proporciona un tiempo adecuado para conseguir el control de una vía aérea fetal potencialmente obstruida mientras se mantiene la circulación útero-placentaria durante la cesárea. Describimos dos casos clínicos de fetos con obstrucción congénita de la vía aérea alta en los que la estrategia E.X.I.T. permitió permeabilizar con éxito y sin complicaciones en la vía aérea. Además de tomarse las consideraciones generales de la paciente obstétrica y de realizarse anestesia fetal intramuscular, previamente a la histerotomía se administró sevofluorano a 1,5 CAM para conseguir una adecuada relajación uterina durante el procedimiento. Las dos gestantes permanecieron hemodinámicamente estables durante el procedimiento con una adecuada perfusión útero-placentaria. En un feto la intubación nasotraqueal fue posible tras disecar la masa cervical mientras que en el otro se realizó una traqueostomía. Tras el clampaje del cordón umbilical se redujo la concentración de halogenado y se administraron oxitocina y metilergometrina para conseguir una contracción uterina adecuada en pocos minutos. Ambos neonatos sobrevivieron al procedimiento sin complicaciones

An ex utero intrapartum treatment (EXIT) procedure provides sufficient time to gain control of the potentially obstructed fetal upper airway while uterine placental circulation is maintained during cesarean section. We report 2 cases in which fetal congenital upper airway obstruction was managed without complications during EXIT procedures. We also discuss general considerations concerning the obstetric patient and the performance of intramuscular fetal anesthesia. Before the hysterectomy, sevoflurane at 1.5 minimum alveolar concentration was administered to assure sufficient uterine relaxation during EXIT. The 2 parturients remained hemodynamically stable during the procedure and uterine and placental perfusion was adequate. Nasotracheal intubation was possible in 1 fetus after a cervical mass was dissected. In the other, a tracheostomy was created. After the umbilical cord was clamped, the concentration of sevoflurane anesthetic gas was reduced and oxytocin and methylergometrine were administered to induce adequate uterine contractions within a few minutes. Both neonates survived the EXIT procedure with no complications

Successful ex utero intrapartum treatment (EXIT) procedure for congenital high airway obstruction syndrome (CHAOS) owing to a large oropharyngeal teratoma.

Large fetal neck masses can present a major challenge to securing an airway at birth, with associated risks of hypoxia, brain injury and death. The authors report a case of a giant oropharyngeal teratoma diagnosed in a fetus of 19 weeks gestation. The fetus was delivered by the ex utero intrapartum treatment procedure allowing sufficient time on placental support for bronchoscopy and tracheostomy to secure the airway. A multidisciplinary team approach combined with an accurate prenatal diagnosis obtained through fetal ultrasound was the key to a successful outcome. Unfortunately due to the large size of tumour and intracranial extension, the lesion was unresectable and the baby died 6 days after birth.

Autoamputation of a congenital oropharyngeal hairy polyp.

Hairy polyps or dermoids are rare, benign congenital tumours of the oronasopharynx containing elements of both ectodermal and mesodermal origin. They occur most commonly in young infants and can cause respiratory distress or failure to thrive due to feeding difficulties. Computed tomography, magnetic resonance imaging, and barium video fluoroscopy can be useful in delineating the origin and extent of these lesions. Treatment usually consists of surgical removal. We describe a case of spontaneous resolution of symptoms in an 11-week-old baby due to autoamputation of the lesion.

Epignathus: report of a case with successful outcome.

Epignathus is an extremely rare form of teratoma that arises from the palate or pharynx in the region of the basisphenoid (Rathke's pouch). This condition is associated with a high mortality rate caused by severe airway obstruction in the neonatal period, thus requiring prenatal planning and prompt surgical treatment after birth. The authors describe a case of a giant epignathus that was successfully resected followed by an uneventful recovery.

Hairy polyp of the oropharynx: case report and literature review.

Hairy polyp of the oronasopharynx is an uncommon developmental malformation that is most frequently seen as a pedunculated tumor in the neonate. Derived from the ectoderm and mesoderm, this benign tumor generally has been classified as dermoid. The clinical presentation is dependent on the polyp's size and location. A full-term girl was evaluated for an oral mass that was first noted at the time of birth. Evaluation showed a 5- x 2.5-cm soft, nontender, skin-covered mass that protruded from the oral cavity. During surgery, it was noted that the stalk was attached to the superior pole of the left tonsil. The histology of the mass was consistent with a hairy polyp. Knowledge of this type of malformation facilitates early intervention and avoids significant morbidity.

Hairy polyp of the oropharynx: an unusual cause of intermittent neonatal airway obstruction.

Oropharyngeal hairy polyps are rare benign congenital tumours that usually present at birth or shortly afterwards with acute upper airway obstruction or feeding difficulties. We describe a case of intermittent airway obstruction in a neonate caused by a mobile hairy polyp of the tonsil. This rare benign lesion proved difficult to diagnose because of its tendency to prolapse into the nasopharynx.

Oropharyngeal hairy polyp with meningothelial elements.

The so-called hairy or teratoid polyp is a rare lesion of bigerminal origin that comprises elements derived from both ectodermal and mesodermal cell lines. In this article we report the presence of meningothelial elements in a hairy polyp, a previously undescribed component of this entity. The lesion was characterized by a pedunculated outgrowth from the hard palate. The surface of the outgrowth was covered by squamous epithelium and a central core of fibroadipose tissue, pilosebaceous glands, cleftlike pseudovascular spaces, and groups of epithelioid cells. These reticulated and cellular foci had the immunohistochemical and ultrastructural features of meningothelial tissue.

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.

Oropharyngeal teratoma in a child: a case report.

Teratoid tumours of the oropharynx are notorious for obstructing the airways and interfering with alimentation. We report here a healthy 20-day old neonate who presented with a painless growth in the floor of the mouth from birth. In this neonate, the tumour interfered with breast-feeding but not with bottle-feeding which the child tolerated very well. At operation, the tumour was easily enucleated, weighed 6G and was histologically consistent with a teratoma. The commonest site for this tumour is the nasopharynx and only 26 oropharyngeal teratomas have been reported in the English literature. The differential diagnosis of congenital oral growths should include a teratoma.

The perioperative management of neonates with congenital oropharyngeal teratomas.

Oropharyngeal teratomas are rare congenital neoplasms that distort orofacial anatomy and often cause respiratory embarrassment at birth. Immediate management of such lesions should include establishment of a secure upper airway, radiographic exclusion of midline central nervous system anomalies, and early surgical excision to prevent asphyxia or permanent disfigurement. Perioperative assessment and surgical management are reported for three oropharyngeal teratomas.

Hairy polyp of the oropharynx. A case report with speculation on nosology.

The hairy polyp of the oronasopharynx is a rare congenital malformation that has been classified as a dermoid, teratoid, teratoma, or hamartoma in the past. A case of oropharyngeal hairy polyp is presented that occurred in a male neonate with severe intermittent respiratory obstruction. The precise nosology of this unusual malformation is discussed, with the speculation that it is classified as a choristoma.