• Archives of Plastic Surgery
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• v.40 no.5
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• pp.570-574
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• 2013

Background The elliptical excision is the standard method of removing benign skin lesions, such as congenital melanocytic nevi. This technique allows for primary closure, with little to no dog-ear deformity, but may sacrifice normal tissue adjacent to the lesion, resulting in scars which are unnecessarily long. This study was designed to compare the predicted results of elliptical excision with those resulting from our excision technique. Methods Eighty-two patients with congenital melanocytic nevus on the face were prospectively studied. Each lesion was examined and an optimal ellipse was designed and marked on the skin. After an incision on one side of the nevus margin, subcutaneous undermining was performed in the appropriate direction. The skin flap was pulled up and approximated along several vectors to minimize the occurrence of dog-ear deformity. Results Overall, the final wound length was 21.1% shorter than that achieved by elliptical excision. Only 8.5% of the patients required dog-ear repair. There was no significant distortion of critical facial structures. All of the scars were deemed aesthetically acceptable based on their Patient and Observer Scar Assessment Scale scores. Conclusions When compared to elliptical excision, our technique appears to minimize dogear deformity and decrease the final wound length. This technique should be considered an alternative method for excision of facial nevi.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.28 no.2
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• pp.136-140
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• 2002

Basal cell nevus syndrome is inherited as an autosomal dominant trait with variable expressivity. This syndrome comprises a number of abnormalities such as multiple nevoid basal cell carcinomas of the skin, skeletal abnormalities as bifid rib and fusion of vertebrae, central nervous system abnormalities as mental retardation, eye abnormalities, and multiple odontogenic kerato cysts. In 1960, Gorlin and Goltz first described the features of this disease as constituting a true syndrome; since then, it has been realized that it is much more complex and encompassing than initially thought. This patient has many symtoms of basal cell nevus syndrome. - we has known multiple jaw cysts through panorama and facial computed tomography. He has hyperchromatism on basal cell through skin biopsy. In ophthalmologic consult, he has blindness on right. On his past medical history, amputation was done on his toes for polyductalism. - So we report with literature reviews

• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.789-793
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• 2007

Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder characterized by variable manifestations, including multiple basal cell carcinomas, odontogenic keratocysts of the jaw, skeletal anomalies including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We report a case of a 9-year-old boy with the clinical features of basal cell nevus syndrome, in which a PTCH gene mutation was confirmed by DNA testing.

• Archives of Plastic Surgery
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• v.37 no.6
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• pp.819-822
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• 2010

Purpose: Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder. It is characterized by complex neoplastic syndrome with multisystemic manifestations, involving six major features. This article presents a rare genetic disorder and usage of the author's methods for odontogenic keratocyst, developed in the maxillary sinus. Methods: A 67-year-old man was presented with large calcified maxillary mass and multisystemic manifestations and findings that matched with basal cell nevus syndrome. The calcified maxillary mass was removed via the versatile maxillary window and maxillary bone segment was repositioned. Results: Histopathologic findings revealed that maxillary and mandibular lesions were odontogenic keratocysts and the skin lesions were basal cell carcinoma. Conclusion: Basal cell nevus syndrome is a rare genetic disease that requires surveillance and care for basal cell carcinoma and multisystemic problems. The author's method was satisfactory for maxillary odontogenic keratocyst in the aspect of the approach and reconstruction.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.26 no.3
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• pp.305-309
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• 2000

The basal cell nevus syndrome is a well recognized entity, the major symptoms of which are basal cell nevi, multiple jaw cysts, skeletal anomalies, and ectopic calcification. The syndrome follows a hereditary pattern, which is characterized by a highly penetrant, autosomal dominant gene with multiple and variable effects. The patient often has a characteristic face, with frontal and temporoparietal bossing, which results in an increased cranial circumference. The eyes may appear widely separated, and 40 percent of patients have true ocular hypertelorism. Jaw cysts are one of the most constant features of the syndrome and are present in at least 75 percent of the patients. The cysts are odontogenic keratocysts and frequently multiple. Radiographically, the cysts in patients with basal cell nevus syndrome do not differ significantly from isolated keratocysts. The cysts in patients with this syndrome are often associated with the crowns of unerupted teeth; on radiographs they may mimic dentigerous cysts. We report a case of multiple odontogenic keratocysts associated with basal cell nevus syndrome with the literature of review.

• Archives of Craniofacial Surgery
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• v.24 no.2
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• pp.78-82
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• 2023

Blue nevi, which are characterized by collections of pigment-producing melanocytes in the dermis, have a variety of clinicopathological characteristics. Plaque-type blue nevus (PTBN) is a variant of blue nevi. PTBN presents at birth or arises in early childhood, and it shows a combination of the features found in common blue nevus and cellular blue nevus. It is typically found on the dorsal surface of the hands and feet or on the head and neck, and it is usually benign and stable over time. However, reports have occasionally described malignant melanomas developing in or associated with a PTBN. Malignant blue nevi are most commonly found on the scalp. We report the case of an 88-year-old woman with a malignant melanoma associated with a PTBN of the cheek.

• Imaging Science in Dentistry
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• v.38 no.2
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• pp.109-115
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• 2008

The basal cell nevus syndrome (BCNS) is an autosomal dominant disorder, characterized by basal cell carcinomas, odontogenic keratocysts and skeletal abnormalities. We experienced two cases that represented several characteristics of BCNS. Case 1: a thirty three year-old man visited CSU hospital. His radiographs showed four cystic lesions at both maxillary sinus and both mandibular angle, with bifid rib and ectopic calcification of falx cerebri. After marsupialization and enucleation, recurrent and newly developing tendency were found on his follow-up radiographs. Case 2: a seventeen year-old man had four large cystic lesions which were diagnosed as odontogenic keratocysts. He had craniofacial anomalies which included ectopic calcification and frontal bossing.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.18 no.1
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• pp.5-12
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• 1988

Several cases of jaw cyst-basal cell nevus-bifid rib syndrome are presented. This syndrome consists principally of multiple jaw cysts, basal cell nevi, and bifid ribs but no one component is present in all patients. The purpose of this paper is to review the multiple characteristics of this syndrome and present three cases in a family and additional 4 cases. The many malformations associated with the syndrome have variable expressivity. In the cases, multiple jaw cysts, palmar and plantar pittings, bridging of sella, temporoparietal bossing, hypertelorism, cleft palate, and dystopia canthorum have been observed.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.31 no.5
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• pp.429-434
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• 2009

Basal cell nevus syndrome is a rare inherited disorder characterized by mulitple cutaneous basal cell carcinoma, pits of the palms and soles, cysts of the jaws, skeletal abnormalities and ectopic calcifications. Currently there are new lines of investigation based on biomolecular studies, which aim at identifying the molecules responsible for these cysts and thus early allowing an early diagnosis of these patients. We report a case of a 9-year-old boy with the various manifestation of basal cell nevus syndrome, which are multiple odontogenic keratocysts, pits of the soles, bifid ribs, ectopic calcification, macrocephaly, and hypertelorism, etc. Total five odontogenic keratocysts were found. For the reduction of the size of the odon-togenic keratocysts, following preoperative marsupialization, there were surgically enucleated. And the impacted upper right lateral incisor and canine are tracted orthodontically.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.34 no.4
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• pp.485-489
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• 2008

Basal cell nevus syndrome is a hereditary disease of an autosomal dominant trait with variable conditions such as basal cell carcinomas of the skin, deformity of rib, fusion of vertebrae, mental retardation, hypertelorism, and multiple odontogenic keratocysts. A 32 years old man with pus discharge from fistula on the vestibule of left upper 1st molar visited to Chosun University Dental Hospital. Radiographic evaluation revealed multiple maxillary and mandibular cysts that had multilocular radiolucency on left mandibular body area, thining of inferior border of left border of ramus and well defined unilocular radiolucency above right upper 1st and 2nd molar and from left upper 1st premolar to 2nd molar. In chest PA view, he had a forked rib in the left 4th rib and in skull PA view the calcification of falx cerebri was observed. There was not any skin lesion. After the preliminary evaluation, the patient was diagnosed with basal cell nevus syndrome and he underwent marsupialization for decreasing the size of cystic lesion and came to hospital for dressing 3days a week. As time goes by, the size of lesion decreased. So, one and half year after marsupialization, he underwent cyst enucleation and iliac bone graft for the mandibular lesion and buccal fat pad grafts for the maxillary lesions. After the surgery, the patient experienced normal healing without any complications and he is on long-term follow-up.