ABSTRACT
Congenital syngnathia (CS), first reported by Burket in 1936, is a rare condition, with <50 reported cases and is associated with other conditions and syndromes. CS restricts mouth opening, causing difficulty in feeding, swallowing, and respiration. This report puts forth the clinical findings and management of this challenging condition in association with tracheoesophageal fistula in a neonate.
ABSTRACT
BACKGROUND: Assess clinicopathological features of patients with keratocystic odontogenic tumor (KCOT) associated with Gorlin-Goltz syndrome in our institution from 2004 to 2015. METHOD: After histopathological analyses of KCOT related to Gorlin-Goltz syndrome, 7 patients were assessed. These patients presented a total of 15 primary and 2 recurrent KCOT. RESULTS: All patients presented a multiple KCOT, and 13 lesions were located in mandible (77%) and 4 (23%) in maxilla. Most of the tumors presented a unilocular pattern (71%) and had tooth association (88%). Four patients (57%) were in the age group of 10-19 years and three patients (43%) were in the age group of 20-29 years. There were four male and three female patients. CONCLUSION: KCOT is a frequent manifestation of Gorlin-Goltz syndrome and can be its first sign, mainly in young patients. The four patients presented with two lesions (57%) and three lesions in three patients (43%).
ABSTRACT
An accessory submandibular salivary gland is a rare anatomical variant, and a tumour within one is even rarer. We describe a 54-year-old man who presented with a slowly-enlarging mass in the right submandibular region which on magnetic resonance imaging (MRI) seemed to be close to, but not arising from, the right submandibular salivary gland. This was found to be a benign pleomorphic adenoma arising from an accessory submandibular salivary gland. To our knowledge it is only the second report of a pleomorphic adenoma that developed within an accessory submandibular salivary gland.
Subject(s)
Adenoma, Pleomorphic/diagnosis , Neoplasms/diagnosis , Salivary Glands/diagnostic imaging , Submandibular Gland Neoplasms/diagnosis , Submandibular Gland/diagnostic imaging , Adenoma, Pleomorphic/pathology , Adenoma, Pleomorphic/surgery , Biopsy, Fine-Needle , Humans , Magnetic Resonance Imaging , Male , Margins of Excision , Neoplasms/pathology , Neoplasms/surgery , Submandibular Gland Neoplasms/pathology , Submandibular Gland Neoplasms/surgeryABSTRACT
Neural tumors located centrally in jaw bones are relatively rare compared with soft tissue neurofibromas. Less than 50 cases have been reported in the literature with a predilection for mandible. This article aims to elucidate a unique case of intraosseous neurofibroma of mandible in a 62-year-old edentulous female patient associated with facial asymmetry due to the swelling extending from the right body of mandible to left body of mandible. The uniqueness of this case is related to the age and extensiveness of this lesion. A review of clinical, radiographic, histological, and immunohistochemical features, and the surgical management pertaining to this case are discussed along with a review of the literature.
