• Maxillofacial Plastic and Reconstructive Surgery
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• v.32 no.1
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• pp.81-85
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• 2010

Multiple jaw cysts are one of the most constant features of the basal cell nevus syndrome. Basal cell nevus syndrome is inherited as an autosomal dominant trait with variable expressiveness. 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 with multiple jaw cysts. The odontogenic keratocysts in patients with this syndrome are often associated with the crowns of unerupted teeth and huge size; on radiographs they may mimic dentigerous cysts. The most important feature of the cyst is its extraordinary recurrence rate. Since recurrence may be long delayed in this lesion, follow-up of any case of odontogenic keratocyst with roentgenograms and clinical examination of basal cell carcinoma are essential for at least five years after surgery. We report the result of 7-year follow up after cyst enucleation associated with basal cell nevus syndrome with the literature of review.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.263-268
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• 2005

Epidermal nevus syndrome is a rare disease consisting of epidermal nevus and multisystem pathologic conditions associated with anomalies in the central nervous system, bone, eye, heart, vasculature and genito-urinary system. Hypophosphatemic rickets has been rarely observed in association with this syndrome. We report a case of hypophosphatemic rickets as sociated with epidermal nevus syndrome with review of the literature. (J Korean Soc Pediatr Nephrol 2005;9:263-268)

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.2
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• pp.198-203
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• 2008

We report a 10-year-old girl with the blue rubber bleb nevus syndrome (BRBNS) who had chronic severe anemia caused by chronic occult bleeding in the gastrointestinal (GI) tract. The patient was admitted to the hospital frequently for recurrent pallor and fatigue since the age of 7 years. Gastroduodenoscopy and capsule endoscopy revealed multiple venous malformations with blood oozing in the stomach, small bowel and colon. The patient was treated by aggressive surgical resection of the 23 vascular malformations in the GI tract. The patient is well without anemia 15 months post surgery.

• Archives of Craniofacial Surgery
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• v.25 no.2
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• pp.85-89
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• 2024

Postoperative atlantoaxial rotatory subluxation (AARS) is a rare complication that develops almost exclusively in children following oropharyngeal and otologic surgeries, proposing that oropharyngeal inflammatory responses and excessive head rotation are responsible factors. However, there have been no reports of AARS after excision of a nevus on the head and neck. Here, we present two cases of AARS following limited head rotation during simple nevus excision. Patient 1, a 9-year-old girl, complained of neck pain and limited range of motion after excision of the nevus on the neck. After 2 months, computed tomography and magnetic resonance imaging finally revealed AARS with a ruptured transverse atlantal ligament. A month of halo traction was required for the treatment. Patient 2, an 11-year-old girl, presented with immediate pain and limited neck extension after tissue expander insertion under the upper chest and excision of the nevus on her left cheek. The diagnosis was promptly made using cervical spine radiography. A cervical collar was applied for 1 month. Both patients recovered without any complications after treatment. This report highlights the importance of suspicion for AARS after surgery regardless of surgical duration or amount of head rotation.

• Clinical and Experimental Pediatrics
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• v.49 no.1
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• pp.1-5
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• 2006

Several physiological skin changes such as vernix caseosa, cutis marmorata, physiologic desquamation, and sebaceous hyperplasia have been described in the neonatal period. There are also clinical characteristics of skin peculiar to neonate and infancy. Skin disorders observed during neonatal and infancy period can be divided into transient skin lesions, birth marks, and other diseases. Transient skin lesions include milia, sebaceous gland hyperplasia, erythema neonatorum, transient neonatal pustular melanosis, and acne neonatorum. Nevocellular nevus, mongolian spot, vascular malformation, hemangioma, epidermal nevus, and sebaceous nevus belong to birth marks. There are several common skin diseases such as miliaria, diaper dermatitis, atopic dermatitis, seborrheic dermatitis, and cutaneous candidiasis.

• Imaging Science in Dentistry
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• v.34 no.4
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• pp.203-207
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• 2004

Basal cell nevus syndrome (BCNS) is principally characterized by cutaneous basal cell carcinomas, multiple odontogenic keratocysts and skeletal abnormalities. Our patient represented several characteristics of BCNS, such as, multiple odontogenic keratocysts, facial nevus, calcification of falx cerebri, parietal bossing and mental retardation. The cyst on posterior mandible showed recurrent and newly developing tendency.

• Archives of Craniofacial Surgery
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• v.22 no.2
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• pp.122-125
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• 2021

Basal cell nevus syndrome (BCNS), also known as basal cell carcinoma nevus syndrome, Gorlin syndrome, Gorlin-Goltz syndrome, and nevoid basal cell carcinoma, is a rare autosomal dominant disorder with a prevalence of approximately 1/60,000. A lower prevalence rate of 1/13,939,393 has also been reported in Korea. We report the case of a 40-year-old male patient with multiple black pigmented macules on the face that first appeared when he was a teenager. His clinical features of jaw cysts, bifid ribs, and calcification of the falx cerebri were fitting within the criteria for the diagnosis of BCNS. We excised all suspected macules and sent permanent biopsy. Most of the histological examinations of the biopsy samples taken during surgical excision of the face masses showed basal cell carcinomas. Ten months after the surgery, the patient has remained free from symptoms and is undergoing follow-up observation.

• Archives of Craniofacial Surgery
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• v.23 no.2
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• pp.83-88
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• 2022

Nevus sebaceous of Jadassohn is a congenital cutaneous hamartoma with epidermal, sebaceous, follicular, and apocrine structures that usually appears at birth or in early childhood. It has the potential to generate a variety of secondary neoplasms of different lineages, and the risk increases with patient age. Although multiple neoplasms may occasionally arise within the same lesion, the coexistence of more than five secondary tumors is extremely rare. Here we report a case of seven secondary tumors including syringocystadenoma papilliferum, desmoplastic trichilemmoma, sebaceoma, trichoblastoma, pigmented trichoblastoma, sebaceous adenoma, and tumor of follicular infundibulum arising within a nevus sebaceous. The complete diagnosis relies on the histopathological analysis of multipoint biopsies and delicate pathological sections.

• Archives of Plastic Surgery
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• v.38 no.6
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• pp.883-885
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• 2011

Purpose: Nevus sebaceus is a congenital hamartomatous lesion, typically involving head and neck. Various benign and malignant neoplasms can develop in association with nevus sebaceus. We report a case of simultaneous occurrence of squamous cell carcinoma and basal cell carcinoma in nevus sebaceus. Methods: A 73-year-old man presented with erythematous to black verrucous nodules on the right subauricular area. The upper part was accompanied with inflammation and ulceration, and no specific findings suspicious for malignant degeneration were found in the lower part preoperatively. The mass was totally excised and the defect was directly closed. Lymph nodes were not involved on concomitant neck dissection. Results: Histopathologic examination confirmed the presence of squamous cell carcinoma in the upper part and basal cell carcinoma in the lower part of a nevus sebaceus. Negative margins were achieved on resection. No clinical problems were found during the 3-month follow-up period. Conclusion: We experienced a rare case of simultaneous occurrence of squamous cell carcinoma and basal cell carcinoma within the same nevus sebaceus. Because patients with nevus sebaceus have risk of malignant changes, surgical excision and work-up for recurrence and metastasis should be considered in suspicious cases. And even in a totally asymptomatic case, the possibility of occult secondary cancer should be informed before surgery in aged patients.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.30 no.2
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• pp.172-177
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• 2008

Basal cell nevus syndrome, also known as Gorlin-Goltz syndrome, is a hereditary condition transmitted as an autosomal dominant trait that exhibits high penetrance and variable expressivity. It is an ecto-mesodermal polydysplasia with numerous manifestations that affect multiple organs. Odontogenic keratocysts, palmar and plantar pits, and hypertelorism are the most frequently observed anomalies. 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 two cases of multiple odontogenic keratocysts associated with basal cell nevus syndrome.