Frontometaphyseal dysplasia is an uncommon genetic syndrome affecting bone and connective tissue. This condition is characterized by the prominence of supraorbital ridges, hyperostosis of the skull, mandibular hypoplasia and malocclusion with antegonial notching, underdeveloped paranasal flaring of the long bone, and a widened iliac wing. Frontometaphyseal dysplasia has an X-linked dominant trait. Clinical manifestations are more severe in males and have extreme variability in females. We present an 11-year-old boy who had frontometaphyseal dysplasia with disfigured face due to bilaterally urinary tract malformation and chronic urinary tract infection. To improve facial appearance, his prominent supraorbital ridges were contoured by ostectomy and burring through bicoronal incision. Urologic operation was performed simultaneously. Histologically, resected bony specimen revealed bony tissue with normal trabecular pattern suggesting exostotic bone. The patient healed without any postoperative complications and he and his parents were satisfied with his final facial morphology.
Child ; Connective Tissue ; Female ; Humans ; Hyperostosis ; Male ; Malocclusion ; Parents ; Postoperative Complications ; Skull ; Urinary Tract ; Urinary Tract Infections

No abstract available.
Tricuspid Valve Insufficiency*

Restoration of anteroposterior projection of the zygomatic body and facial width are key elements in treatment of complex zygomatic fractures. Traditionally, it has been understood that for most fractures of the zygoma, three-point fixation of the zygomaticofrontal suture, inferior orbital rim, and zygomaticomaxillary buttress generally produces exact structural restoration. Recently, the importance of accurate reduction of the zygomatic arch through coronal incision has been favored in complex zygomatic fractures. Though coronal incision has the advantages of accurate reduction and fixation as a result of extensive exposure of the fractured area, this procedure also has many disadvantages including injury to the facial nerve, paresthesia, alopecia, scar formation, longer operating time, protracted hospitalization, etc. As well, there have been no objective data to prove the fact that four-point fixation through coronal incision is superior to traditional three-point fixation. From May, 1994 to December, 1998, the authors treated 45 patients by traditional three-point (n=20) and coronal four-point fixation (n=25) with random sampling. To assess the difference between the two methods the authors measured the axial angle of the zygoma, the axial angle of the zygomatic arch, and the degree of zygomatic arch inclination on submentovertex X-ray, and then analyzed the measurements by the paired T-test(p < 0.05). As a result, the axial angle of the zygoma and zygomatic arch assessed by anteroposterior projection, as well as the facial width showed no statistical difference between the two groups, respectively(p = 0.26, p = 0.18). Mean while, the degree of zygomatic arch inclination representing the local contour of the fractured zygomatic arch was statistically significant between the two groups(p < 0.05). Thus, the traditional three-point fixation method may be widely acceptable in treatment of complex zygomatic fractures with anterior three-point or zygomatic arch comminution.
Alopecia ; Cicatrix ; Facial Nerve ; Hospitalization ; Humans ; Orbit ; Paresthesia ; Sutures ; Zygoma ; Zygomatic Fractures

No abstract available.
Esophageal Perforation*

No abstract available.
Esophageal Perforation*

Prurigo pigmentosa is a chror ic pruritic inflammatory dermatosis characterized by erythematous papules in a reticulated pattern that resolve leaving a reticulated, mottled hyperpigmentation and rapidly response to dapsone therapy. Most cases that have been reported are from Japan. We report two cases of prurigo pigmentosa in a 21 year-old female and a 23-year-old female. Histopathologic findings of erythematous papules showed exocytosis, spongiosis, liquefaction degeneration of basal cell layer and perivascular lymphohistiocytic infiltration on the upper dermis. We treated them with dapsone, 100mg daily, which resulted in a remarkable regression of the ery thematous papules and improvenien of pruritus.
Dapsone ; Dermis ; Exocytosis ; Female ; Humans ; Hyperpigmentation ; Japan ; Prurigo* ; Pruritus ; Skin Diseases ; Young Adult

Cirsoid aneurysm of the calp is a congenital arteriovenous malformation which is the result of abnormal connections betwe:.n the external carotid artery and scalp veins with a subsequent dilatation of vascular channels nd formation of aneurysm. We report a case of cirsoid aneurysm of the scalp on the left parieto-occipital area of 14-year-old female who had had 8 x 5 x 0.8 cm sized pulsating soft mass for 2 years. 4 vessel angiog raphy revealed a arteriovenous malformation that emerge mainly from a superficial temporal artery and occipital artery of the left external carotid artery and partially from the right superficial temporal artery and drined into the left scalp vein. The mass was removed by excision with ligation of the feeding arter es and followed up for 2 years without recurrence.
Adolescent ; Aneurysm* ; Arteries ; Arteriovenous Malformations ; Carotid Artery, External ; Dilatation ; Female ; Humans ; Ligation ; Recurrence ; Scalp* ; Temporal Arteries ; Veins

No abstract available.
Glomus Tumor*

Cutaneous manifestations of sarcoidosis are highly variable and can be devided into the specific lesions which reveal noncaseating granuloma and the nonspecific lesio is which are referred to as cutaneous changes. Among speciific lesions, annular lesions may be foimied from coalescing papular lesions that may clear centrally and elevate peripherally. We report a case of sarcoidosis with multiple annular lesions on the face in a 33-year-old female who had systemic involvements in the intrathoracic organs and eyes. The main histopathologir. findings were well-demarcated islands of epitheloid cells with a few giant cells in the dermis. In addition, a slight admixfure of lymphoid cells was present at the margins of the epitheloid cell granulomas. Complete regression of t!he skin lesions was obtained with systemic cortiocosteroid therapy and no recurrence has been found for 2 1/2years.
Adult ; Dermis ; Female ; Giant Cells ; Granuloma ; Humans ; Islands ; Lymphocytes ; Recurrence ; Sarcoidosis* ; Skin

Kaposi's sarcoma is a neoplasm of multifocal origin which manifest primarily as vascular nodules in the skin and other organs. We report a case of Kaposis sarcoma of classic type in a 17-year-old boy, who had a 4 x 5cm sized, dusky erythematous plaque with multiple spots of acupuncture on the left foot for 1 year. Histopathologic examination showed dilated, bizarre-shaped vessels, ndothelial cell proliferation, and spindle cell formation with vascular slits throughout the dermis. He was treated with 3000 rads of radiation(300 rad per day for l0 successive days). Six months after radiotherapy, gross findings were much improved and histopathologic findings showed only fibrosismc hemosiderin deposits in the dermis.
Acupuncture ; Adolescent ; Cell Proliferation ; Dermis ; Foot ; Hemosiderin ; Humans ; Male ; Radiotherapy* ; Sarcoma, Kaposi* ; Skin