• Journal of Periodontal and Implant Science
• /
• v.24 no.2
• /
• pp.255-260
• /
• 1994

We experienced a case of Sturge-Weber Syndrome in a 32 years old female. The diagnosis was established by clinical features of Sturge-Weber Syndrome including unilateral facial portwine nevus, vascular hyperplasia of oral mucosa(espectially 1st & 2nd division of trigeminal nerve). But, plain radiographys of the skull revealed no evidence of calcification. A brief review of related literatures was made. Common Clinical findings in Sturge-Weber Syndrome and specific signs & symptoms manifested by this patient were discussed.

• Journal of Chest Surgery
• /
• v.25 no.8
• /
• pp.791-794
• /
• 1992

Authors experienced a case of malignant melanoma of the mediastinum, At the time of first visit to the hospital, the female patient, aged 48, and had chief complaints of right shoulder pain that was radiated to its forearm and right Horner`s syndrome. In chest X-ray and CT, extrapulmonary circumscribed mass in the right apicomedial aspect of mediastinum was found. She had no nevus and no melanoma on entire skin area. We excised the tumor and confirmed it as malignant melanoma by histopathology.

• Archives of Craniofacial Surgery
• /
• v.17 no.2
• /
• pp.93-95
• /
• 2016

• Clinical and Experimental Pediatrics
• /
• v.46 no.9
• /
• pp.909-912
• /
• 2003

Klippel-Trenaunay syndrome is a rare mesodermal phakomatosis characterized by cutaneous haemangiomata( usually unilateral and involving an extremity), venous varicosities and osseous and soft tissue hypertrophy, of the affected limb. Sturge-Weber syndrome, also a mesodermal pharkomatosis, is characterized by a port-wine nevus, which is present from birth and covers the face and cranium in the territory of the first division of the trigeminal nerve. Homolateral to the skin lesion, there is atrophy and calcification of the cerebral hemisphere. We experienced an unusual 26-months-old female, who had features of both Klippel-Trenaunay syndrome and Sturge-Weber syndrome. She had an extensive nevus flammeus which extended primarily over both sides of her face and the right side of the trunk and extremities, hypertrophy of the right extremity without evidence of arterovenous fistula, right glaucoma, choroidal hemangioma and leptomeningeal hemangioma, which combined Klippel-Trenaunay syndrome and Sturge-Weber syndrome. We reported this rare case with a brief review of some related literatures.

• Archives of Plastic Surgery
• /
• v.47 no.6
• /
• pp.551-558
• /
• 2020

Background Giant congenital melanocytic nevus (GCMN) is a rare disease, for which complete surgical resection is recommended. However, the size of the lesions presents problems for the management of the condition. The most popular approach is to use a tissue expander; however, single-stage expansion in reconstructive surgery for GCMN cannot always address the entire defect. Few reports have compared tissue expansion techniques. The present study compared single and serial expansion to analyze the risk factors for complications and the surgical outcomes of the two techniques. Methods We retrospectively reviewed the medical charts of patients who underwent tissue expander reconstruction between March 2011 and July 2019. Serial expansion was indicated in cases of anatomically obvious defects after the first expansion, limited skin expansion with two more expander insertions, or capsular contracture after removal of the first expander. Results Fifty-five patients (88 cases) were analyzed, of whom 31 underwent serial expansion. The number of expanders inserted was higher in the serial-expansion group (P<0.001). The back and lower extremities were the most common locations for single and serial expansion, respectively (P =0.043). Multivariate analysis showed that sex (odds ratio [OR], 0.257; P=0.015), expander size (OR, 1.016; P=0.015), and inflation volume (OR, 0.987; P=0.015) were risk factors for complications. Conclusions Serial expansion is a good option for GCMN management. We demonstrated that large-sized expanders and large inflation volumes can lead to complications, and therefore require risk-reducing strategies. Nonetheless, serial expansion with proper management is appropriate for certain patients and can provide aesthetically satisfactory outcomes.

• Asian Pacific Journal of Cancer Prevention
• /
• v.17 no.1
• /
• pp.425-429
• /
• 2016

Background: Dermatoscopy can be applied to diagnose pigmented skin lesions. The aim of the present study was to compare dermatoscopic and histopathologic results in basal cell carcinoma (BCC) and melanocytic nevus of theface. Materials and Methods: In an analytical-descriptive study, 61 patients suspected of BCC or melanocytic nevi of face were randomly selected. The skin lesions of patients were evaluated with dermatoscopic method from February 2012 to February 2014 and results were compared with pathological features of samples. Results: In this study, mean age of patients was $49.5{\pm}18.9$. Some 25 (41%) were men and 36 (59%) were women. In 27 cases (44.3%) there was diagnosis of melanocytic nevus, in 28 cases (45.9%) diagnosis of BCC, and in 3 cases (4.9%) there was mixed diagnosis. The relationship between patients' gender and dermatoscopic diagnosis of the patients was statistically significant (P=0.001). For BCC the sensitivity and specificity of dermatoscopic method were 100% and 97% respectively and for melanocytic nevi 96.4% and 97%. Conclusions: Dermatoscopic study not only can be helpful in improving clinical diagnosis while guiding missed malignant lesions to pathologic evaluations, but also could be useful in evaluating further suspicious or recurrent cases.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.21 no.1
• /
• pp.74-80
• /
• 1999

A family is presented in which multiple odontogenic keratocysts have been expressed. 3 members of the two generations of family with features of multiple keratocysts, suspected basal cell nevus syndrome have been investigated. The proband, 19-year-old woman and her mother, and her brother suffered from these cysts and two female patients' disease have been recurred multiply though careful treatments. Close attention to the family and routine follow up will alert the clinician to its recurrences and genetic counseling and serial screening for the development of other symptoms, that is malignant skin carcinoma etc.

• Journal of Korean Dental Science
• /
• v.4 no.1
• /
• pp.33-37
• /
• 2011

Gorlin-Goltz syndrome is an autosomal dominant disorder with a high degree of penetrance. It is characterized by basal cell carcinomas, odontogenic keratocysts, palmar and plantar pits and ectopic calcifications of falx cerebri. The presence of two major and one minor criteria or one major and three minor criteria are necessary to establish a diagnosis. Early diagnosis and treatment of Gorlin-Goltz syndrome, as well as family screening and genetic counseling are essential as it may be associated in 10% of patients with aggressive basal cell carcinoma and malignant neoplasias. We report here a patient with Gorlin-Goltz syndrome.

• Clinical and Experimental Pediatrics
• /
• v.57 no.5
• /
• pp.245-249
• /
• 2014

Gastrointestinal (GI) hemangiomas are relatively rare benign vascular tumors. The choice of an appropriate diagnostic method depends on patient age, anatomic location, and presenting symptoms. However, GI hemangiomas are not a common suspected cause of GI bleeding in children because of their rarity. Based on medical history, laboratory results, and imaging study findings, the patient could be treated with either medication or surgery. Herein, we report 3 cases of GI hemangioma found in the small bowel, rectum, and GI tract (multiple hemangiomas). Better knowledge and understanding of GI hemangioma could help reduce the delayed diagnosis rate and prevent inappropriate management. Although rare, GI hemangiomas should be considered in the differential diagnosis of GI bleeding.

• Imaging Science in Dentistry
• /
• v.42 no.1
• /
• pp.55-60
• /
• 2012

Gorlin-Goltz syndrome is an autosomal dominant disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocystic odontogenic tumors, and skeletal anomalies. This syndrome may be diagnosed early by dentist because keratocystic odontogenic tumors are usually one of the first manifestations of the syndrome. Early diagnosis and treatment are of utmost importance in reducing the severity of long term sequelae of this syndrome. This report presents a rare event of Gorlin-Goltz syndrome occurring in a 39-year-old male and his 8-year-old daughter. The clinical and investigative features of this familial disorder has been described in detail.