Simultaneous, congenital, mucocoele of the Blandin-Nühn glands and teratoma of the tongue: early management and follow up at one year.

Mucocoele of the Blandin-Nühn glands and teratomas of the tongue are rare in newborn babies. We present what is to our knowledge the first documented case of both at the same time, and describe management and follow up over 12 months after the birth. Prenatal diagnosis defined the treatment plan during delivery to establish an airway for the baby promptly. A normal delivery is possible if the cyst is anterior, and can be punctured during delivery. Delayed excision reduces the possibility of oedema and obstruction of the airway, ensures complete resection, avoids recurrence, and confirms the diagnosis. Mucocoeles are classified as extravasation or retention types; our observation supports the hypothesis of a "malformative type". A long follow-up is essential to monitor macroglossia, open bite, and the development of the mandible.

Transoral robotic surgery-assisted excision of a congenital cervical salivary duct fistula presenting as a branchial cleft fistula.

BACKGROUND: Congenital cervical salivary duct fistulae are rare entities and can mimic branchial cleft fistulae. Ectopic salivary tissue associated with these pharyngocervical tracts may have malignant potential. METHODS AND RESULTS: We present a case report of a novel surgical approach and review of the literature. A 27-year-old man presented with complaint of drainage from the right side of his neck since early childhood. A tract was found from the posterior tonsillar pillar into the neck and ectopic salivary tissue was found along the tract. A congenital hearing loss was also present. Transoral robotic (TORS)-assisted surgery was used in the management of this patient and allowed excellent visualization of the pharyngeal component of the lesion and a minimally invasive approach. The patient did well with no recurrence. CONCLUSION: TORS was helpful for management of a congenital salivary fistula and may be helpful for branchial cleft fistulae. These lesions may be associated with the branchio-oto-renal (BOR) syndrome.

Congenital agenesis of all major salivary glands and absence of unilateral lacrimal puncta: a case report and review of the literature.

Abstract Congenital agenesis of the salivary glands is an extremely rare congenital condition, which may cause severe xerostomia, progressive dental caries, and oropharyngeal candidiasis in children. To date, there have been few documented cases of aplasia of the major salivary glands. Congenital agenesis of the salivary glands accompanied by absence of the lacrimal puncta is even more rare. We report a case of a 5-year-old boy with xerostomia and extensive dental caries. Salivary gland imaging with sodium pertechnetate 99mTcO(4) showed bilateral aplasia of the parotid, submandibular, and sublingual glands. The patient was found to lack the left lacrimal puncta through physical examination. Here we describe the clinical presentation, diagnostic essentials, and medical and dental management of the patient.

Congenital ranula.

This article presents 3 cases of infants with congenital ranula, a rare salivary gland pathology. Pathophysiology is discussed, differential diagnosis, and different treatment choices are explained.

Congenital absence of salivary and lacrimal glands accompanied by growth and development retardation.

Congenital absence or hypoplasia of some or all major salivary or lacrimal glands, nasolacrimal ducts, and puncta is a rare autosomal dominant disorder. Phenotypical expression may show great variability, and the diagnosis may be difficult. Only a few cases have been reported in the literature. This report describes a patient who has all of the essential features suggestive of aplasia of the lacrimal and salivary glands, as well as retardation of growth and development. Although the characteristic features of aplasia of the lacrimal and salivary glands have been described, an association with development and growth retardation has not been reported in the literature.

[Congenital ranula in a newborn]. / Sublinguale, livide Schwellung am Mundboden eines Neugeborenen: Kongenitale Ranula.

Ranulas are cystic lesions in the floor of the mouth. They are either retention cysts of the excretory duct of the sublingual gland or pseudocysts formed by excretory duct rupture followed by extravasation and accumulation of mucus in the surrounding tissue. We report the case of a premature newborn with a congenital ranula in the floor of mouth. The ranula caused no discomfort or complications, so that immediate intervention was not necessary. The cyst resolved completely by the age of 4 months. Complications in newborns especially include airway obstruction and feeding difficulties. Surgical treatment options are needle aspiration, excision of the ranula, marsupialization, cryosurgery, and--in addition to excision of the cyst--removal of the ipsilateral sublingual gland. Sclerotherapy has shown good results as well. As many congenital cysts resolve or rupture spontaneously, they should be observed for potential resolution for several months in uncomplicated cases.

Non-neoplastic salivary gland diseases.

A wide range of non neoplastic disorders can affect the salivary glands, although the more common are: mumps, acute suppurative sialadenitis, Sjögren's syndrome and drug-induced xerostomia. Salivary dysfunction is not a normal consequence of old age, and can be due to systemic diseases, medications or head and neck radiotherapy. Diagnosis of salivary disorders begins with a careful medical history, followed by a cautious examination. While complaints of xerostomia may be indicative of a salivary gland disorder, salivary diseases can present without symptoms. Therefore, routine examination of salivary function must be part of any head, neck, and oral examination. Health-care professionals can play a vital role in identifying patients at risk for developing salivary dysfunction, and should provide appropriate preventive and interventive techniques that will help to preserving a person's health, function, and quality of life. The present work provides an overview of most of the non neoplastic disorders of the salivary glands, in which the general presentation, pathology, and treatments are discussed.

Lesions of the accessory parotid gland in children.

The accessory parotid gland, a little-known and seldom-mentioned anatomical variant, is a nodule of normal salivary tissue separate from the main parotid gland, located on the masseter muscle and connected to the Stensen duct at that level. It can be the site of both congenital and acquired lesions.

Recurrent congenital bilateral ranula: a case report.

A case of congenital bilateral ranula in a one-week-old male baby is reported. At presentation the cyst measured 3.5 x 2.5 cm but did not interfere with feeding. Initial marsupialisation resulted in recurrence of the cyst. It was subsequently re-excised completely together with the associated sublingual salivary gland. The cyst has not recurred six months after treatment. The usually large size of the cyst and its bilateral presentation makes this an interesting case.

Syndromes with salivary dysfunction predispose to tooth wear: Case reports of congenital dysfunction of major salivary glands, Prader-Willi, congenital rubella, and Sjögren's syndromes.

Four cases-of congenital dysfunction of the major salivary glands as well as of Prader-Willi, congenital rubella, and Sjögren's syndromes-were identified in a series of 500 patients referred for excessive tooth wear. Although there was evidence of consumption of highly acidic drinks, some occlusal parafunction, and unacceptable toothbrushing habits, salivary dysfunction was the salient factor predisposing a patient to tooth wear in these syndromal cases. The 500 subjects have been characterized either as having medical conditions and medications that predispose them to xerostomia or lifestyles in which workplace- and sports-related dehydration lead to reduced salivary flow. Normal salivation, by buffering capacity, clearance by swallowing, pellicle formation, and capacity for remineralization of demineralized enamel, protects the teeth from extrinsic and intrinsic acids that initiate dental erosion. Thus, the syndromes, unrelated in many respects, underline the importance of normal salivation in the protection of teeth against tooth wear by erosion, attrition, and abrasion.

Lingual cyst in infancy: importance of palpation for diagnosis.

Two infants, 5 and 12 weeks of age, with lingual cysts were presented. Histological findings were compatible with a salivary retention cyst in one and a thyroglossal duct cyst in the other. Both infants were admitted to our hospital because of severe stridor that had developed from one to two weeks of age. Their lingual cysts were easily recognizable by simple palpation and were confirmed by non-invasive imaging techniques, such as ultrasound sonography, computed tomography and magnetic resonance imaging. Lingual cyst in infancy may be large enough to cause stridor and dyspnea, and occasionally result in sudden infant death, although once diagnosis is made a radical operation can be easily performed. Therefore, it is important that palpation in the oral cavity should be performed with all infants with persistent stridor as a part of a physical examination.

[The etiology of sialolithiasis]. / K voprosu ob étiologii sliunnokamennoi bolezni.

The authors have revealed dilatation of the excretory ducts of salivary glands involved in sialolithiasis and of other salivary glands in a patient. A hypothesis on the primary etiological role of ductal ectasia in the development of sialolithiasis in case some other additional factors are present is put forward. The authors recommend resorting to computer-aided tomography and functional digital subtraction sialography of the salivary glands for the diagnosis of sialolithiasis and discuss the advantages of these methods in comparison with other ones.

Salivary gland disease in infancy and childhood: non-malignant lesions.

The differential diagnosis for benign lesions of the salivary glands in infancy and childhood can be somewhat overwhelming, especially in light of their infrequent occurrence in a general otolaryngologic practice. The purpose of this review is to discuss these conditions under the headings of inflammatory conditions, immunologic disorders, granulomatous disease, trauma, cysts, systemic disorders and benign neoplasms. Basic techniques of diagnosis and treatment as they apply to children will be outlined.

Parotid duct communicating with a labial pit and ectopic salivary cyst.

Labial pits are usually described as blind epidermal invaginations of lip mucosa with occasional drainage of minor salivary gland secretions. A case of an aberrant parotid duct in communication with both a unilateral, commissural labial pit and an interposed salivary cyst is presented. A 7-year-old boy who had experienced lifelong clear drainage from a congenital pit at his right oral commissure developed fever, pain, and a submucosal mass of the right cheek over a 6-week period. A preoperative sialogram through the pit demonstrated a communication between the oral commissure fistula, an intervening salivary cyst, and a proximal tract from the cyst to the parotid gland. An en bloc resection of the pit, fistula tract, and cyst was performed through a transoral approach. The dissection was medial to the buccinator muscle. There has been no recurrence of the cyst after 1 year follow-up. A description of labial pits and parotid duct anomalies is presented; however, to our knowledge, this combination of anomalies has not been previously described.

Congenital cystic dilatation of the submandibular duct.

A lesion which appeared clinically consistent with a ranula was explored surgically and found to represent a dilated submandibular duct. The differential diagnosis of cystlike swelling in the floor of the mouth should include sialolithiasis, mucous retention phenomenon (mucocele, ranula), dermoid and epidermal inclusion cyst, thyroglossal duct cyst, branchial cleft cyst, hemangioma, lymphangioma, cystic hygroma, lipoma, and occasionally pleomorphic adenoma. The case presented here demonstrates, both clinically and histologically, the cystic appendix-like appearance of a dilated submandibular duct manifest at birth. Reference is made to the limited number of similar cases noted in the relevant literature.

Benign parotid swellings: a review.

The one common factor associated with the innumerable causes of benign parotid enlargement is xerostomia which influences the quantity and quality of parotid secretion and thus paves the way for duct obstruction and pyogenic infection.