Concomitant Congenital Intraoral Dermoid Cyst and Heterotopic Gastrointestinal Cyst.

Sublingual dermoid cysts and oral heterotopic gastrointestinal cysts are well-documented causes of sublingual swellings in infants. However, the simultaneous occurrence of both cysts is an extremely rare phenomenon, with only two previous cases reported in literature. We present a case of 1-month-old neonate with a cystic ventral tongue mass that on histologic examination showed a dermoid cyst with co-existing heterotopic gastric-type epithelium in the wall. Additional histochemical stains revealed focal areas of colonic metaplasia within the heterotopic gastric epithelium. CDX2 immunohistochemical staining confirmed derivation from colonic epithelium.

Congenital Oral Masses: An Anatomic Approach to Diagnosis.

Although congenital oral masses are rare, they are readily detectable during fetal US screening. Most congenital oral masses are benign, but some may cause mechanical airway obstruction, resulting in poor outcomes at delivery. The radiologist's ability to describe these abnormalities and their physiologic sequelae accurately can have a substantial effect on perinatal treatment. Furthermore, despite being rare, congenital oral lesions encountered at screening and at follow up fetal MRI provide the opportunity to make a specific diagnosis by following a simple anatomic approach. This article describes an anatomic algorithm as the framework for accurate diagnosis of congenital oral lesions. The imaging appearance of the most common congenital oral cavity neoplasms is outlined, including vascular anomalies, epulides, choristomas, congenital lingual thyroid anomalies, lingual hamartomas, and epignathi, and other conditions that mimic these at US. Also reviewed are perinatal management of masses that affect the fetal airway and the imaging features key to optimizing delivery outcomes. Online supplemental material is available for this article. ©RSNA, 2019.

From newborn to toddler: report of two cases of congenital granular cell tumor.

A congenital granular cell tumor (CGCT) is an uncommon benign soft tissue lesion that usually arises from the alveolar ridge in newborns. It can severely interfere with the respiratory and feeding systems, if left untreated. We present the cases of 2 newborn infants with protruded intraoral tumors that severely compromised breastfeeding. The tumors were removed by water laser without the need for suturing or pain medication. Histopathologic assessment was performed for definitive diagnosis, and the children were followed until full primary dentition was observed without any sign of CGCT recurrence.

Surgical management of immature teratoma involving the oral cavity and orbit in a neonate.

Teratomas are congenital germ cell tumors composed of elements from 1 or more of the embryonic germ layers and contain tissues usually foreign to the anatomic site of origin. In the head and neck region, these lesions are rare, and 90% of head and neck teratomas present during the neonatal and infantile periods. Besides, in neonates, it carries serious risk for respiratory distress as well as feeding problems due to oral cavity and airway obstruction.Here, an unusual case of intraoral teratoma involving the orbit in a newborn who underwent immediate surgical excision successfully is described.

Head and neck teratoma: from diagnosis to treatment.

INTRODUCTION: Head and neck teratoma is a rare entity. Its prognosis mostly depends on the risk of neonatal respiratory distress, its extension and potential malignancy. Surgical management must be as complete as possible to avoid recurrences and malignant transformation. The authors present a retrospective analysis of 6 cervicofacial teratomas and a review of the literature. The aim of the study was to analyse prenatal, neonatal and postnatal management of teratoma. MATERIALS AND METHODS: Charts of children presenting with a head and neck teratoma, managed by our maxillofacial and plastic surgery unit, were analysed and antenatal, clinical, biological, radiological and pathological characteristics were collected. Surgical treatment, recurrences and surgical outcomes were analysed. RESULTS: Six patients were included: 2 with a cervical teratoma, 2 with a facial teratoma and 2 with intraoral teratomas. In 2 cases, the lesions were diagnosed antenatally and both patients required neonatal resuscitation. All the patients underwent early surgery, and 3 with complete excisions. All patients with an initial incomplete excision eventually presented a recurrence and therefore second look surgery. No malignant transformation was noted. CONCLUSION: Early prenatal diagnosis is crucial to neonatal care. Early surgery and meticulous follow-up are critical in the long-term favourable outcome.

Congenital tri-cavernous hemangiomas of the right buccal region, right accessory parotid gland, and masseter muscle region.

We report a rare case of congenital tri-cavernous hemangiomas of the right buccal region, right accessory parotid gland, and masseter muscle region in an adult. The patient, a 25-year-old woman, complained of 3 masses in her right midcheek. Ultrasonographic and computed tomographic findings showed an irregular-shaped mass (multiple calcifications) with a well-defined margin in the masseter muscle region, an ellipse-shaped mass (multiple calcifications) with a well-defined margin in the right buccal region, and a comma-shaped mass (no calcifications) with a well-defined margin separate from the parotid gland in the right accessory parotid gland region. These iconographic findings suggested that the masses were all hemangiomas separately originating from the parotid gland, accessory parotid gland, and masseter muscle. The masses were completely removed through a standard parotid incision without postoperative facial palsy, skin deformity, and difficulty in secreting saliva. Findings from histologic examination of the tumor revealed multiple, thin-walled, and dilated blood vessels, confirming the diagnosis of cavernous hemangiomas. Ultrasonographic and computed tomographic findings were extremely useful in diagnosing the mass/masses as hemangioma before surgery, clarifying relationships between the mass and adjacent structures, and determining the surgical approach to the mass/masses.

Emergency management of a congenital teratoma of the oral cavity at birth and three-year follow-up.

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.

Prenatal assessment of a fast-growing giant epignathus.

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.

Retrospective analysis of ten cases of congenital sublingual teratoid cyst.

AIM: The aims of this study were to perform clinicopathological analyses of teratoid cysts of the floor of the mouth and to assess the possibility of surgical treatment. MATERIALS AND METHODS: The incidence, manifestations, histogenesis, treatment and prognosis of 10 patients admitted to our department between April 2000 and May 2008 were reviewed. RESULTS: Teratoid cysts occurred almost exclusively in neonates and children, and appeared as mobile, cystic solid and well-defined lesions. Because of the special location, consequences such as difficulty in feeding, breathing and even impairment in growth and development frequently occur. Results of histological examination indicated that squamous, respiratory and gastrointestinal epithelium composed the basic structure of teratoid cyst. Transonogram, CT and MRI examination were conducted, and complete surgical excision was the only treatment of choice. Usually, immediate treatment is not necessary unless the cyst obstructs the upper airway. There has been no recurrence of the lesion after surgery. CONCLUSION: Although teratoid cyst of the floor of the mouth is a cystic disease, it affects patients to a great extent because of its special location. Therefore, early and complete excision is mandatory for good prognosis.

Functional reconstruction of epignathus with cleft palate using part of a mature teratoma.

Epignathus is an extremely rare, benign, congenital teratoma that arises from the oral cavity. When treating epignathus with cleft palate, it is particularly important to consider velopharyngeal function and maxillary growth after surgical repair. The case of an infant with a complete cleft palate and a large mass, histologically diagnosed as a mature teratoma, that protruded from the right soft palate is described. At 8 months of age, a double-opposing Z-plasty was performed using a part of the tumor over the right soft palate that had been left at the time of primary excision at 3 months of age for an epignathus protruding from the mouth. Though all that could be done was suture tumor tissue on the right side and the left hypoplastic levator veli palatine muscle using the double-opposing Z-plasty, velopharyngeal function and maxillary growth were good at 10 years of age.

Multiple intraoral teratoma in a newborn infant: epignathus.

Teratomas originating from the oral cavity are named as epignathus. It is a rare type of teratoma. An 11-day old male newborn was diagnosed with cleft palate and intraoral masses. The mass on the right side was protruding from the mouth. Another one on the left side was extending from the nasopharynx to the oropharynx. The diagnosis of mature teratoma was made based on the histopathological study of surgically excised masses. We desired to report on this case since multiple localized epignatus in a newborn with cleft palate has not been described yet in the literature.

Novel application of percutaneous cryotherapy for the treatment of recurrent oral bleeding from a noninvoluting congenital hemangioma involving the right buccal space and maxillary tuberosity.

Cryotherapy is the application of varying extremes of cold temperatures to destroy abnormal tissue. The intent of this article is to describe a novel technique using percutaneous cryotherapy for treating a noninvoluting congenital craniofacial hemangioma (NICH). An 18-year-old woman with type 1 von Willebrand's disease, as well as a qualitative platelet aggregation disorder, presented with multiple recurrent episodes of oral bleeding from a NICH involving the right buccal space and maxillary tuberosity. The patient was initially treated with a combination of endovascular particulate embolization, percutaneous sclerotherapy, tissue cauterization, and laser therapy between the ages of 4 and 8 years of age. At 18 years of age, the patient presented with recurrent episodes of oral bleeding related to the NICH. Endovascular embolization was performed using particulate and a liquid embolic agent with limited success. Due to the refractory nature of this bleeding, the patient underwent successful lesion ablation using percutaneous cryotherapy. At 9-month follow-up, the patient is asymptomatic with no episodes of recurrent bleeding.

A unique presentation of epignathus.

Palatal clefts in conjunction with space-occupying lesions of the oral or nasal cavities are of interest because they may represent a developmental etiology of palatal clefts. Epignathus is a rare space-occupying tumor of the nasopharynx that can arise from the upper jaw, palate, and sphenoid. It can protrude through the mouth, causing respiratory embarrassment and death. The pathogenesis of epignathus is unknown, but several theories have been proposed. Management depends on the size of the tumor and requires a multidisciplinary approach.

Massive immature teratoma in a neonate.

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.