Sturge-Weber syndrome (SWS) is a sporadic disorder and is frequent among the neurocutaneous syndromes specifically with vascular predominance. This syndrome consists of constellation of clinical features like facial nevus, seizures, hemiparesis, intracranial calcifications, and mental retardation. It is characterized by focal port-wine stain, ocular abnormalities (glaucoma), and choroidal hemangioma and leptomeningeal angioma most often involving occipital and parietal lobes. The present paper reports three cases of SWS with oral manifestations and periodontal management, which included thorough scaling and root planing followed by gingivectomy with scalpel and laser in cases 1 and 3 consecutively to treat the gingival enlargement. However, the treatment in case 2 was deferred as the patient was not a candidate for periodontal surgery.
A 75-year-old male patient was planned for dental treatment due to pain of suspected pulpo-periodontal origin in relation to right maxillary first molar. Careful evaluation revealed the pain to be non-odontogenic in nature and led to the diagnosis of temporal arteritis with rheumatoid arthritis along with interstitial lung disease (ILD). Characteristic findings of temporal arteritis include headache, jaw claudication, visual loss, and constitutional symptoms (malaise, fever, weight loss, loss of appetite). Temporal artery biopsy (TAB) remains the gold standard for diagnosis. Additional diagnostic tests include blood tests (ESR, CRP). This article reports and discusses how the orofacial manifestations can lead to misdiagnosis of temporal arteritis. Hence, temporal arteritis should be included in the differential diagnosis of orofacial pain in the elderly especially to prevent complications like vision loss.
ABSTRACT.A 48-year-old woman presented with cough and chest pain. A chest radiograph and CT scans showed bilateral lung masses containing massive venous varices. A core biopsy specimen revealed benign metastasising leiomyoma with strong expression of progesterone receptors. A review of her medical history revealed a hysterectomy 11 years earlier. The lung masses showed significant reduction in size after induction of artificial menopause, although the pulmonary varices persisted. A pulmonary varix is a localised enlargement of a segment of a pulmonary vein [1]. Lung varices have been associated with mitral valve disease, tuberculosis, emphysema, systemic hypertension and arteriovenous fistulae. Benign metastasising leiomyoma (BML) is a rare condition characterised by the presence of histologically benign smooth muscle tumours at extra-uterine sites in the presence of uterine leiomyomas [2][3][4]. Pelvic varices are a common finding in patients with uterine leiomyomas (fibroids); however, varices do not normally occur within the leiomyomas. We report CT findings observed in a 48-year-old woman who developed large varices inside BML lung masses. To our knowledge, this finding has not been previously described in the literature. Case reportA 48-year-old woman was referred for CT evaluation of pulmonary abnormalities that had appeared on a chest radiograph. She initially presented with a cough and pleuritic chest pain on the right side. Her medical history included a hysterectomy for fibroids 11 years earlier. On examination, she was clearly unwell and had a fever. A chest radiograph showed bilateral volume loss with bilateral indistinct hemidiaphragm and a possible mass in the left lower zone (Figure 1). She was hypoxic in room air. Routine haematological tests were unremarkable except for a C-reactive protein level that increased from 31 to 244 mg L 21. An electrocardiogram was unremarkable and D-dimer and troponin T levels were normal.A multislice CT scan of the chest with injection of intravenous contrast medium was performed. The resulting images demonstrated bilateral lung masses up to 9 cm in diameter with obvious venous varices. Vein diameters were as large as 1.2 cm in the lung cortex (Figure 2). Images of the lung varices were demonstrated by CT angiography three-dimensional reconstructions using maximum intensity projection (MIP) and volumerendering techniques (Figure 3).A CT-guided biopsy of the left lung lesion was performed, targeting the less vascularised area. Histological sections showed bland, predominantly fusiform, cells displaying no mitotic activity and arranged in a whorled pattern, typical of leiomyoma. These results were confirmed by immunolabelling for smooth muscle actin (SMA). Cell nuclei strongly expressed the receptor protein for progesterone, but not the oestrogen receptor, indicating that the cells might be responsive to hormonal manipulation. These features are diagnostic of BML. The patient was treated by inducing artificial menopause, which resulted in partial regression of the lun...
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