RESUMO
OBJECTIVE: To investigate the clinical, imaging and pathological features of congenital infiltrating lipomatosis of the face (CILF) and to discuss whether it is a subtype of hemifacial hyperplasia (HH). METHODS: Sixteen patients diagnosed with CILF were included in this study. All patients had undergone panoramic radiography and spiral CT examinations. Thirteen patients received biopsy, surgery treatment and pathological examination. The clinical documentation and imaging data were retrospectively reviewed. RESULTS: The cheeks (14/16), parotid glands (12/16), tongues (9/16), masticatory muscles (8/16) and the lips (7/16) were the most frequently affected soft tissue organs. The maxilla (14/16), zygoma (13/16), mandible (13/16) were involved among the maxillofacial bones. Dental malformations included macrodontia (8/16), poor formation of the roots (7/16), accelerated tooth germ development or premature eruption of permanent teeth (7/16) and missing of the permanent teeth (4/16). All malformations were restricted to one side of the face and did not trespass the middle line. Pathologically, CILF was featured by the diffuse infiltration of redundant mature adipose tissue into the tissue of the affected organ. CONCLUSION: CILF is a congenital developmental facial malformation characterized by infiltration of nonencapsulated, mature adipose tissue, resulting in facial soft and hard tissue hypertrophy and dental malformations in hemifacial structures. CILF could be considered as a subtype of HH.
Assuntos
Face/anormalidades , Face/cirurgia , Assimetria Facial/congênito , Hiperplasia/etiologia , Lipomatose/congênito , Lipomatose/diagnóstico por imagem , Adolescente , Adulto , Criança , Pré-Escolar , Face/diagnóstico por imagem , Assimetria Facial/diagnóstico por imagem , Assimetria Facial/etiologia , Assimetria Facial/cirurgia , Feminino , Humanos , Hiperplasia/diagnóstico por imagem , Hiperplasia/cirurgia , Lipomatose/cirurgia , Masculino , Radiografia Panorâmica , Estudos Retrospectivos , Tomografia Computadorizada Espiral , Anormalidades Dentárias/diagnóstico por imagem , Anormalidades Dentárias/etiologia , Adulto JovemRESUMO
La lipomatosis congénita infiltrante de la cara (CLIF) es una enfermedad de origen congénito de baja prevalencia. Sus características clínicas son bien definidas. Éstas generan asimetrías faciales evidentes en los individuos que las padecen, las que pueden observarse clínicamente así como también en radiografías y estudios histopatológicos. El diagnóstico de lipomatosis se hace al nacimiento o a edades muy tempranas y su tratamiento también comienza cuando los pacientes son muy pequeños. Es de gran importancia el trabajo en equipo con cirujanos plásticos, cirujanos maxilofaciales, ortodoncistas, cirujanos dentistas y sicólogos. Este trabajo presenta el caso clínico de una niña chilena con diagnóstico de lipomatosis congénita infiltrante de la cara y el tratamiento que se le ha realizado hasta la fecha.
Congenital infiltrating lipomatosis of the face (CLIF) is a present at birth disease of low prevalence. Its clinical characteristics are well defined. They generate evident facial asymmetries in individuals who suffer from them which can be seen clinically as well as in X-rays or histopathological studies. Diagnosis of lipomatosis is made at birth or at very early stages and its treatment also starts when the patients are very young. It is of great importance the teamwork with plastic surgeons, maxillofacial surgeons, orthodontists, dental surgeons and psychologists. This paper shows the case of a Chilean girl with diagnosis of congenital infiltrating lipomatosis of the face and the treatment that she has received up to date.
Assuntos
Humanos , Criança , Face , Assimetria Facial , Lipomatose/congênito , Lipomatose/terapiaRESUMO
The congenital infiltrating lipomatosis (LCI) represents a distinct, clinical and pathological aspect of fat soft-tissue neoplasia; very few cases of which have been described in literature. In 1983, Slavin and Cols described the main characteristics of LCI as:(1) non-encapsulated tumours containing mature adipocites, (2) fat infiltration in muscles and adjacent soft parts, (3) absence of malignant characteristics, (4) absence of lipoblasts, (5) presence of fibrous elements, increased number of vessels and nerves and (6) adjacent bone hypertrophy. In this article, we describe a congenital infiltrating lipomatosis of the face in a child and discuss the diagnoses and the treatment of this disease.