Corrección quirúrgica en una adolescente con hiperplasia gingival hereditaria / Surgical correction in an adolescent with hereditary gingival hyperplasia

Se describe el caso clínico de una adolescente que fue atendida en la consulta de Periodoncia del Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, por presentar agrandamiento de las encías desde hacía más de un año, lo que le dificultaba la masticación de los alimentos y, por tanto, le producía trastornos digestivos transitorios, además de afectar su estética. Luego de realizados los exámenes físico y complementarios, se estableció el diagnóstico presuntivo de hiperplasia gingival hereditaria o familiar y se indicó el tratamiento, que incluyó 3 fases: la inicial, la correctiva quirúrgica y la de soporte periodontal. En la fase quirúrgica se tomó una muestra hística cuyo análisis anatomopatológico confirmó el diagnóstico presuntivo inicial

The case report of an adolescent that was assisted in the Periodontics Service of Dr Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba is described. He presented enlargement of the gums for more than a year, which made mastication of foods difficult and therefore, transitory digestive dysfunctions took place, besides affecting her aesthetics. After the physical and complementary tests, the presumptive diagnosis of hereditary or family gingival hyperplasia was established and the treatment was indicated into 3 phases: initial phase, surgical corrective phase and that of periodontal support. In the surgical phase a tissue sample was taken which pathologic analysis confirmed the initial presumptive diagnosis

Síndrome de Proteus: relato de casos / Proteus Syndrome: case reports

INTRODUÇÃO: A síndrome de Proteus é uma doença complexa e rara, classificada nos grupo das hamartoses. Foi primeiramente descrita em dois pacientes, em 1979, por Cohen e Hayden. Existe dificuldade no diagnóstico, sendo comum a confusão com síndromes de Klippel-Trenaunay-Weber, neurofibromatose ou Stuge-Weber. Apresentamos dois casos tratados no Serviço de Cirurgia Plástica e Reparadora da Universidade Federal do Paraná. MÉTODO: Paciente masculino (caso 1), que chegou ao serviço aos 6 anos de idade, tendo como principais apresentações lipomatoses e assimetrias. A segunda paciente (caso 2) deu entrada no serviço com 20 anos de idade e diagnóstico de síndrome de Klippel-Trenaunay-Weber, que posteriormente mostrou se tratar de síndrome de Proteus. CONCLUSÃO: A hipótese etiológica mais aceita para a doença é genética. Acredita-se que exista mosaicismo somático e que a doença seja letal no estado não mosaico. Morte prematura é bastante frequente. Entretanto, a sequela mais comum é a ocorrência de tumores incomuns. O cuidado dos pacientes portadores da síndrome é um desafio devido às suas consequências médicas e psicossociais.

INTRODUCTION: Proteus syndrome is a complex and rare disorder classified as a hamartomatous disease. It was first described in two patients in 1979, by Cohen and Hayden. Proteus syndrome is difficult to diagnose, and is often confused with Klippel-Trenaunay-Weber syndrome, neurofibromatosis, or Sturge-Weber syndrome. In this study we describe two patients who were treated at the Plastic and Reconstructive Surgery Service of the Federal University of Paraná. METHOD: A 6-year-old male patient (case 1) presented to the Service with lipomatosis and asymmetry, as the primary findings. A 20-year-old (case 2) was admitted to the Service with a diagnosis of Klippel-Trenaunay-Weber syndrome, which later was shown to be Proteus syndrome. CONCLUSION: The etiological hypothesis that is most accepted for this disease is genetic. It is believed that somatic mosaicism may occur during pathogenesis, which can be lethal in the mosaic state. Premature death is common. However, the most common sequelae are the occurrence of unusual tumors. The care of patients with this syndrome is a challenge due to medical and psychosocial consequences.