Parotid Gland Aplasia Research Articles

Goldenhar syndrome: rare case reported from secondary health care facility in Himachal Pradesh

Dr. Maurice Goldenhar, a renowned Swiss ophthalmologist classified the clinical features and named the malformation complex as Goldenhar Syndrome and described it as a congenital defect characterized by constellation of malformations classically involving the face, eyes and ears. Principal deformities of the Goldenhar syndrome are often combined with various malformations, like Cleft lip and/or palate, tongue cleft, unilateral tongue hypoplasia, and parotid gland aplasia, Rib anomalies, Congenital heart disease (ventricular septal defects). A 12-year-old female patient diagnosed with Goldenhar syndrome at secondary care Regional Hospital Bilaspur, Himachal Pradesh. She was diagnosed with a case of Goldenhar syndrome on clinic-radiological investigations. The patient reported to ophthalmology OPD of the hospital with the chief complaint of soft tissue structure in left eye with decreased vision. On examination limbal dermoid was observed. Patient was having pre-auricular tissue tag on both sides, hemifacial micrognathia and microstomia. Correlating the history and physical findings a provisional diagnosis of Goldenhar Syndrome was made with differential diagnosis of Franschetti syndrome or Treacher – Collins syndrome, Nager syndrome or acro-facial dysostosis and Townes-Brocks syndrome. Based on the clinical and radiographic findings a diagnosis of Goldenhar syndrome was made. Severe cases of GS can affect the routine and social life of the patient. Early detection can help avoid complications at a later stage of life. Such patients will have an increased risk for psychosocial difficulties. The study of this condition is still controversial because the symptoms and the physical features may vary greatly in range and severity from case to case.

Partial duplication of the mandible, parotid aplasia and facial cleft: a rare developmental disorder

Duplication deformity of the mandible is exceedingly rare. Its occurrence with congenital facial cleft and parotid gland aplasia has been rarely reported as 1 entity. We report such a case with detailed computed tomography (CT) description and provide a review of the literature on mandible duplication.

Recurrent inflammation of accessory parotid tissue associated with unilateral parotid gland aplasia: diagnostic and therapeutic implications

Aplasia of the major salivary glands is a rare condition due to an alteration in the development of the ectodermal tissue of the oral cavity often related to other craniofacial abnormalities or alteration of structures deriving from the first or second archial branch, in particular the lacrimal glands; it can be total or partial and determine clinical states ranging from an asymptomatic condition to a severe xerostomia. The accessory parotid tissue is similar to normal parotid tissue, completely independent from the main gland and susceptible to the same pathological disorders. We describe a very unusual case of an inflammatory disorder of accessory parotid tissue in a 44-year-old male patient with concomitant, and previously unknown, aplasia of the main ipsilateral parotid gland. We also discuss the role of imaging and conservative therapeutic modalities such as botulinum toxin therapy and, in the future, minimally invasive endoscopic-assisted resection in the management of such salivary disorder.

An unusual case of rudimentary parotid gland with distended Stensen duct

Congenital absence or rudiment major salivary glands, especially of the parotid glands, are a rare entity. Aplasia of parotid glands has been described alone or in association with abnormalities of other salivary glands, first branchial arch developmental disturbances and other congenital anomalies such as lacrimoauriculodentodigital syndrome, mandibulofacial dysostosis and ectodermal dysplasia. Absence is most commonly unilateral, and may be associated with accessory or rudimentary glandular tissue. There are several reported cases of isolated patulous Stensen’s duct causing air insufflations in the glands and recurrent parotid gland enlargement. However, in the literature there is no reported case of association of rudimentary parotid gland with a patulous Stensen’s duct. This paper aims at reporting an interesting and unusual presentation of unilateral imperfectly developed parotid gland with a distended Stensen’s duct in a five year old mentally challenged girl who presented with an unintentional habit of blowing out cheeks several times in a day resulting in air insufflations and recurrent parotid swelling.

Aplasia of the parotid glands with accessory parotid tissue

Congenital absence of the parotid gland is a rare entity. Absence is most commonly unilateral, and is not associated with accessory glandular tissue. In the majority of reported cases, parotid gland aplasia is seen with craniofacial abnormalities or hypoplasia of other ectodermal structures, particularly the lacrimal glands. We present a 14-year-old male with bilateral parotid gland aplasia detected incidentally on MRI of the brain and then confirmed on neck CT. The studies also revealed accessory parotid tissue superficial to the left masseter muscle. There were no associated craniofacial abnormalities. The lacrimal glands and submandibular glands were normal.

Bilateral aplasia of parotid glands correlated with accessory parotid tissue

Congenital absence of major salivary glands, especially the parotid gland, is a rare entity. It is usually monolateral and is not correlated with accessory salivary gland tissue. Aplasia of parotid gland may occur alone or in association with abnormalities of other salivary glands, first branchial arch developmental disturbances or other congenital anomalies.We report an interesting case of bilateral aplasia of the parotid glands together with bilateral accessory parotid tissue, without other congenital or developmental anomalies, and we describe the clinical and radiological findings.

Congenital aplasia of the parotid gland with omolateral cheek angioma: case report and review of the literature

Congenital agenesis of the parotid gland is a rare entity whose etiopathogenesis is still unclear. It is usually bilateral and is sometimes associated with other developmental anomalies of the head and neck region. A case is presented in which aplasia of the right parotid gland was associated with an omolateral angioma of the cheek. The radiological diagnostic approach and a review of the literature on agenesis of the salivary glands are presented.

Bilateral parotid gland aplasia

Bilateral parotid gland aplasia is a cause of xerostomia. A case is presented in which the clinical diagnosis was confirmed with the use of 99 m TcO 4 − salivary gland scintiscanning and computerised tomography. The literature of this rare condition is reviewed and significance to the patient discussed.