Arteriovenous Malformation of the Jaws: a Black Hole for the GDP ­ A Case Report.

We report a case of a mandibular arteriovenous malformation in a 3-year-old child, who attended our department, and have carried out a literature review. Clinical relevance: Arteriovenous malformation (AVM) is a rare condition but clinicians need to be aware of it as this lesion can have potentially life-threatening complications due to massive haemorrhage.

Aberrant expression of beta-dystroglycan may be due to processing by matrix metalloproteinases-2 and -9 in oral squamous cell carcinoma.

Dystroglycan (DG), a non-integrin adhesion molecule, is formed by two subunits, alpha- and beta-DG, which bind to extracellular matrix molecules and cytoskeleton. DG expression is frequently reduced in human cancers and has been related to tumor grade and aggressiveness. The exact proteolytic processing of beta-DG remains largely unknown. In this study, we investigated the correlation of beta-DG degradation with invasiveness in oral squamous cell carcinoma (OSCC) and its possible processing by matrix metalloproteinases (MMP). Immunohistochemical staining was used to assess beta-DG expression in 60 cases of OSCC. The effects of the MMP inhibitor 1,10-phenanthroline on tumour cell invasion and beta-DG degradation were investigated using in vitro invasion assays and immunoblot analysis. Co-immunoprecipitation and N-terminal sequencing were performed to determine the possible cleavage site of beta-DG by MMP. The alpha- and beta-DG expression was reduced or lost in OSCC. In four cell lines studied (SCC-4, SCC-9, SCC-15 and SCC-25), Western blot revealed a 30kDa fragment of beta-dystroglycan (beta-DG30) in addition to beta-DG itself. beta-DG degradation was almost abolished using 1,10-phenanthroline and there was a significant decrease in tumor cell invasion. The N-terminal sequence of beta-DG30 was detected as Ile-Asn-Thr-Asn, or Ile-Val-Thr-Gln. We conclude that beta-DG degradation may play a role both in OSCC invasion and metastasis. MMP activity seems to be one mechanism for beta-DG processing into beta-DG30.

Karapandzic flap for reconstruction of lip defects.

PURPOSE: Systematic evaluation of the Karapandzic flap in the reconstruction of lip defects after ablative surgery. PATIENTS AND METHODS: Patients who had a Karapandzic flap to reconstruct lip defects were analyzed with reference to demographic details, histology and location of the tumor, and dimensions of resection. The functional aspects of the reconstruction were assessed in terms of the size of the oral stoma, preservation of oral competence, and facial expression, in addition to speech, diet and ease of cutlery, and denture usage. The esthetic outcome was assessed with a 4-point scale and in addition the symmetry of the commissure at rest and function, preservation of the philtrum, and lip projection also were assessed. The complications were noted. RESULTS: Seven patients underwent Karapandzic flap reconstruction (4 males, 3 females) with an age range of 43 to 98 years. Three tumors were located in the upper lip, 4 in the lower lip, and there were 5 squamous and 2 basal cell carcinomas. The lip defects ranged from 40% to 75% of the lip circumference. The oral stoma was of a reduced circumference in all cases but did not lead to any functional compromise in terms of oral competence, facial expression, speech, diet, denture and cutlery usage, and sensation. There were no wound complications in our series. The esthetic outcome was considered excellent/good in 85% of cases. The commissure was symmetrical in all except 1 patient, the philtrum was preserved in all cases of lower lip reconstruction, and the projection of the lip was reduced in edentulous patients. CONCLUSIONS: The Karapandzic flap is a reliable technique that offers consistently good functional and esthetic outcomes after reconstruction of lip defects.

Lipomatous lesions of the parotid gland.

PURPOSE: Lipomatous lesions of the parotid gland are rare and seldom considered in the initial diagnosis of a parotid mass. We report our experience in the management of patients with lipomatous lesions affecting the parotid gland. PATIENTS AND METHODS: Retrospective analysis of all parotidectomies performed for neoplastic lesions in the maxillofacial unit between 1975 and 2003 and patients with lipomatous lesions involving the parotid gland were identified; 638 parotidectomies were performed in this period on 629 patients in which 660 neoplasms were identified. Eight patients were found to have lipomatous lesions and form the basis of this study. RESULTS: Lipomatous lesions accounted for only 1.3% of parotid tumors and occurred more frequently in males, at a ratio of 3 to 1. The most common presentation was that of a slowly enlarging, painless mass. Computed tomography scan was the most frequent imaging modality undertaken, and in 3 patients a diagnosis of a lipoma was made preoperatively. Seven patients underwent a superficial parotidectomy and 1 patient had a total conservative parotidectomy because of the deep lobe location of the mass. Five patients were found to have a focal lipoma and 3 patients had diffuse lipomatosis. There were no recurrences. Temporary facial nerve palsy and Frey's syndrome were the most frequent complications. CONCLUSION: Lipomatous lesions accounted for only 1.3% of all parotid tumors. There were no specific distinguishing clinical features in our patients, and an accurate preoperative diagnosis was made in 3 patients based on imaging investigations. A well-circumscribed lipoma was more common than diffuse lipomatosis. Superficial parotidectomy was the treatment of choice and there were no recurrences in our series.

Quality of dying in head and neck cancer patients: a retrospective analysis of potential indicators of care.

OBJECTIVE: The objective of this study was to evaluate the quality of the dying experience in a cohort of head and neck cancer patients by a retrospective analysis of potential indicators of care. STUDY DESIGN: The study design included retrospective case note analysis of previously evaluated factors considered important in the care of terminally ill patients and validated indicators of care obtainable from administrative data. The documented factors were analyzed to indicate specific elements of the quality of death and dying experience. RESULTS: Pain was a common symptom (84%) and was managed successfully in all patients, with 93% receiving opioids. Management of other symptoms, except neuropsychological problems, were satisfactory. Sixty-three percent of patients died in hospital and only 22% had a relative present at the time of death. Resuscitation status was documented in only 65% of the notes, though none were admitted to the intensive care unit or underwent resuscitation. Fifty-three percent of patients were admitted as an emergency in the last month of life and bleeding was the most common cause of admission. CONCLUSION: Valuable information can be obtained from a focused retrospective analysis. The indicators evaluated suggest that biomedical aspects of care are being recognized and addressed, although there is room for improvement. Psychosocial and spiritual aspects of care were not documented and must be taken into account to assess the "complete quality of dying" experience.

Melorheostosis involving the craniofacial skeleton.

Melorheostosis is a rare bone disorder, usually affecting the long bones and adjacent soft tissue. It was originally described by Leri and Joanny in 1922, after its classic x-ray features of flowing hyperostosis resembling dripping candle wax. There have been fewer than 10 reported cases of craniofacial involvement, and in most instances these have also involved the appendicular skeleton. The authors report a case of melorheostosis with isolated craniofacial involvement, describe the clinical course and radiologic and histologic features, and review the pertinent literature.