Heddie O. Sedano

Multiple mucosal neuromas, pheochromocytoma and medullary carcinoma of the thyroid—a syndrome

An analysis of 17 cases of the syndrome of multiple mucosal neuromas, pheochromocytoma and medullary carcinoma of the thyroid is presented and its relationship to similar syndromes discussed. The mucosal neuromas primarily involve the lips, anterior tongue, conjunctiva and nasal and laryngeal mucosa. Medullated corneal nerve fibers traverse the cornea and anastomose in the pupillary area. A Marfanoid build and diverticulosis may be associated findings.

Aneurysmal bone cyst of the jaws. Report of a case associated with fibrous dysplasia and review of the literature.

A case of aneurysmal bone cyst (ABC) of the mandible, recurring for the third time and arising in conjunction with fibrous dysplasia, is reported. A comprehensive review of the literature and analysis of over 50 cases of ABC of the jaws yielded the following results: ABCs of the jaws constitute 1.5 % of all non-odontogenic and non-epithelial cysts of the jaws, and 1.9 % of all ABCs of the skeleton. The mandible was affected in 55 % of the cases, the maxilla in 45 % of the patients. Females represented 53 % of the patients and males the remaining 47 %. Seventy-four percent of the ABCs occurred in patients 20 years old and younger. This lesion is regarded as non-neoplastic and as most likely secondary to a pre-existing bone lesion. Our review established that 21 % of the reported cases in the jaws had developed in association with various primary lesions of bone, including fibro-osseous lesions. The primary bone lesion initiates an osseous, arteriovenous fistula which, through its hemodynamic forces, creates a secondary reactive lesion, that is an aneurysmal bone cyst. We also established a recurrence rate of 26 %, based upon previously reported cases as well as our own. The treatment of choice is surgical curettage with cryosurgery and immediate packing with bone chips.

Clinical orodental abnormalities in Mexican children

A total of 32,022 Mexican children (16,473 boys, 15,549 girls) were examined for several congenital oral and paraoral anomalies. The findings for commissural lip pits (boys 53.1, girls 52.4 per 1000) are less than those reported for adults. This may indicate that pits become accentuated with age. Fordyce granules were seen with a prevalence of 1.2 per 1000. This is in contrast to the reported 85.6% prevalence for the adult population, also possibly reflecting increased manifestation with increased age. Our data for exogenous tooth pigmentation show increased prevalence with age (group I [5 to 10 1/2 years], 9.8%, versus group II [10 1/2 to 14 1/2 years], 12.9%), possibly indicating decrease in attention to oral hygiene. The prevalence of talon cusp was found to be 0.6 per 1000, and for ankyloglossia 8.3 per 1000. Prevalence values for bifid tongue are reported for the first time, indicating one affected per 187 children examined. The prevalence of fissured tongue (15.7%) shows a statistically significant difference between boys (16.8%) and girls (14.5%). The prevalence of geographic tongue (1.9%) shows a marked difference between group I (2.2%) and group II (1.2%).

Multifocal papilloma virus epithelial hyperplasia.

Multifocal papilloma virus epithelial hyperplasia is an infection of the oral mucosa produced by human papilloma virus types 13 and 32, which primarily bilaterally affects lips, lateral borders of tongue, and buccal mucosa. The attached oral mucosa, floor of mouth, soft palate, and oropharynx are sites that appear not to be affected. This study comprises 110 patients with multifocal papilloma virus epithelial hyperplasia identified over a period of 3 years in Guatemala City and neighboring rural areas. All but four patients were younger than 18 years of age. More than one affected patient was observed in several families. All but three cases occurred in patients living in extreme poverty (annual family income less than

Giant osteoid osteoma of the maxilla: Report of a case

1,200 in U.S. dollars). Biopsies of 60 lesions demonstrated acanthosis and nuclear degeneration within the epithelium. We suggest that the eponym Hecks disease be abandoned because this disease was described in the Latin-American literature before the initial description in the American literature. We propose the term multifocal papilloma virus epithelial hyperplasia, which best describes the nature and multifocality of this disease.

Autosomal dominant cemental dysplasia.

Abstract The first reported case of giant osteoid osteoma occurring in the maxilla has been reported. The patient, a 21-year-old mulatto man, had a local recurrence 5 months after the initial surgical treatment. Wider excision is suggested to prevent the local recurrence of this tumor.

Oral Manifestations of Systemic Genetic Disorders: Second of Four Parts

Abstract Autosomal dominant cemental dysplasia is the term chosen to describe what is considered to be a new entity affecting cementum and neighboring bone. The condition was present in ten members of the same family, segregating as an autosomal dominant trait. A review of the literature failed to demonstrate previously reported cases. The name was chosen because microscopic evidence and clinical location of the changes observed point to cementum as the affected tissue.

Systemic Inherited Disorders with Dental Involvement

In many genetic diseases and syndromes the involvement of the structures of the mouth is only a small part of the overall picture of multiple systemic involvement with a wide range of symptoms, congenital defects and deficiencies. The combinations of features in some of the syndromes give affected persons a distinctive, unforgettable appearance.

Ringed enamel hyperplasia

This chapter discusses systemic inherited disorders with dental involvement. It discusses acroosteolysis, amelocerebrohypohidrosis syndrome, cryptodontic brachymetacarpalia, hypodontia and nail dysgenesis, hypophosphatasia, hypophosphatemic vitamin D-resistant rickets, hypoplastic-hypocalcified enamel, onycholysis, functionali hypohidrosis, incontinentia pigmenti, oculodentoosseous dysplasia, otodental syndrome, pseudohypoparathyroidism and pseudo-pseudohypoparathyroidism, and rothmund−thomson syndrome among others. Acroosteolysis, which has an autosomal dominant mode of inheritance, is characterized by short stature, disintegration of the terminal phalanges of fingers and toes, abnormal skull shape, and premature loss of teeth. The Amelocerebrohypohidrosis syndrome is characterized by the following features: (1) severe seizures, (2) progressive mental retardation, (3) muscular spasticity, (4) hypohidrosis, and (5) enamel hypoplasia. There is marked hypoplasia of enamel, resulting in complete absence, except for small zones of thin enamel at cervix of the tooth crowns. The enamel defect is generalized and involves all teeth in both primary and secondary dentitions.

Genetic Counseling and Oral Examination

An apparently new disorder of enamel is briefly described.