Fibrous dysplasia is a nonneoplastic developmental disease of osseous tissue. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. In this series of 16 patients with fibrous dysplasia of the craniomaxillofacial bones, the disease was generally monostotic and most commonly maxillary in location. Two patients demonstrated typical symptoms of the McCune Albright syndrome. Marked deformity or functional disturbances were the major indications for treatment. Total excision of the involved bone was the most successful form of treatment but produced the greatest functional and cosmetic deficits and long-term postoperative complications. A conservative therapeutic approach with a modest reduction in the bulk of these lesions may be sufficient to relieve signs and symptoms effectively. Periodic follow-up is indicated to detect recurrences or malignant changes in the early stages.
Epidermoid carcinoma in nonhealing scar tissue, known as Marjolin's ulcer, is not uncommon and is thought to behave in a more aggressive fashion than those from other causes. Between 1982 and 1997, 56 patients with Marjolin's ulcer were treated at our center, Ege University Medical School, Izmir, Turkey. All lesions were secondary to various kinds of burns. Forty of these patients could be followed up 5 years or more. These 40 patients' medical records were reviewed retrospectively.
In this study, 500 frontal and supraorbital transcranial passages were studied in 50 cadavers and 200 crania of 500 samples. One hundred six specimens had a frontal foramen (notch), and all the samples had a supraorbital foramen (notch). The frontal passage was a foramen in 7 samples and a notch in 99 samples. As for the supraorbital passage, it was found as a foramen in 133 of specimens and as a notch in 358 of specimens. In 9 of the specimens, there were double notches or foramina. The distances from the foramina (notches) to angulus oculi medialis were measured in the cadavers. The average distance from the angulus oculi medialis to the frontal foramen (notch) was 4.50 mm, and the average distance to the supraorbital foramen (notch) was 9.87 mm. The distances from the foramina (notches) to the midline were measured in the crania. The average distances from the midline to the frontal foramen (notch) and the supraorbital foramen (notch) were 20.24 mm and 25.23 mm, respectively. The average distance between the frontal foramen (notch) and supraorbital foramen (notch) was 5.37 mm in cadavers and 4.99 mm in crania. In 200 crania, the distances of the frontal and supraorbital transcranial passages to the midline were measured. Types of these passages were also evaluated, and frequencies were calculated. Measurements were made using a digital compass, and the student t test was used in the statistical evaluation of results.
All suspected congenital abnormalities of the nose require further evaluation. The nasal dermoid sinus cyst (NDSC) is one of the many midline nasal masses that often pose diagnostic and treatment dilemmas for the plastic and reconstructive surgeon. NDSCs are distinct from other facial dermoids in their potential for involving deeper contiguous structures, and intracranial extension. Accurate diagnosis and effective treatment are essential to avoid craniofacial skeletal deformation, cyst rupture, and infection that could cause cutaneous, ocular, or intracranial complications. A comprehensive discussion of the embryogenesis, pathogenesis, diagnosis, and surgical management of the NDSC is presented to delineate the role of open rhinoplasty in optimizing the management of this congenital nasal deformity.
Gardner syndrome, a variant of familial adenomatous polyposis, is an autosomal dominant disease characterized by gastrointestinal polyps that develop in the colon as well as in the stomach and upper intestine (duodenum), multiple osteomas, and skin and soft tissue tumors. Cutaneous findings include epidermoid cysts, desmoid tumors, and other benign tumors. Polyps have a 100% risk of undergoing malignant transformation; consequently, early identification and therapy of the disease are critical. Osteoma is a benign neoplasm of bone tissue that is characterized by slow continuous growth and is the most common accompanying bone lesion seen in Gardner syndrome. The authors report a case of Gardner syndrome that was operated on because of the mandibular osteoma.
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