Maffucci’s Syndrome: report of a case with oral manifestation / Síndrome de Maffucci: relato de caso com manifestação oral

Introduction: Maffucci’s Syndrome is a rare nonhereditary mesodermal dysplasia consisting of multiple haemangioma of the soft tissue and enchondromas, mostly affecting phalanges and long bones. The syndrome can also be associated with a variety of other benign and malignant tumors. Case report: Here we report a case of Maffucci’s Syndrome and haemangioma of lip and palate which is rare in this syndrome. This case report describes an 18 year old boy with multiple nodular soft tissue swellings involving the anterior hard palate and lower labial mucosa and bony abnormalities (enchondromas) involving the lower limb.

Introdução: A Síndrome de Maffucci é uma displasia mesodermal não hereditária rara. Consiste de múltiploshemangiomas de tecido mole e encondromas, a maioria afetando as falanges e os ossos longos. A síndromepode também estar associada a uma variedade de tumores benignos e malignos. Relato de caso: O presente trabalho apresenta um caso de Síndrome de Maffucci e hemangioma de lábio e palato, o que é raro nessa síndrome, descrevendo o caso de um garoto de 18 anos de idade com inchaços nodulares múltiplos de tecido mole envolvendo o palato duro anterior e a mucosa labial inferior, além de anomalias ósseas (encondromas)acometendo um membro inferior.

Enfermedad de Ollier: a propósito de un caso / Ollier's disease: a purpose of a acase

Enfermedad de Ollier o encondromatosis múltiple, 1 transtorno infrecuente caracterizado por masas cartilaginosas itraoseas asimétricas, 2 aparecen en la infancia no hereditaria, 3 tiene predominio unilateral principalmente manos y pies, 4 radiológicamente se observan estrías, máximas en metafisis. Existe riesgo de generación sarcomatosa a condrosarcomas (30 por ciento). Se presenta caso; femenina de 6 años con afectación de los cuatro miembros, deformidad, disfuncionalidad y dolor en las manos, limitación de actividades físicas. Se manejo con resección de encondromas en humero proximal, radio distal, F1 anular, F2 de do medio y fèmur distal del lado derecho, igualmente tibia proximal izquierda; evolucionando sin residivas y autolimitaciones de las lesiones.

Enfermedad de Ollier: reporte de un caso / Ollier's Disease: a case report

La enfermedad de Ollier es una encondromatosis múltiple querepresenta el 3 % de los tumores óseos benignos. Las encodromatosisson tumores cartilaginosos que pueden ensanchar el hueso yproducir la clásica característica de la calcifi cación moteada dentrode las lesiones. Se presenta el caso de un escolar masculino de6 años de edad con presencia de múltiples encondromas en lasextremidades torácicas y pélvicas derechas (húmero- radio y cubito,falanges de la mano, fémur, tibia, peroné, y falanges del pie), quehan producido deformidades angulares, rotacionales y asimetría deextremidades pélvicas, que han sido tratadas con osteotomías yfi jación externa tipo Ilizarow a nivel del defecto tumoral en la Unidadde Ortopedia pediátrica del Hospital del Niño de La Paz-Bolivia.

Ollier’s disease is a multiple enchondromatose which constitutes3% of benign bone tumors. Enchondromatoses can thicken thebone and produce the classical characteristics of the cartilaginoustumors: spotted calcifi cations within the lesion. I present the caseof a six year-old school boy who had multiple enchondromes in theright thoracic and pelvic extremities (humero-radius and cubitus,phalanges of the hand, femur, tibia, fi bula, and phalanges of the foot).that had produced angular, rotational deformities and asymmetry ofthe pelvic extremities which had been treated with osteotomy andexternal fi xation according to IIizarow at the level of the tumorousdefect, in the Unit of Pediatric Orthopedics of the Children’s Hospitalin La Paz, Bolivia.

Multiple enchondromatosis [Ollier's disease] with two primary brain tumors

A rare case of multiple enchondromatosis associated with 2 primary brain tumors is reported. A 21-year-old female who has diagnosed as Ollier's disease at the age of 9-years was admitted to the hospital with complaint of diplopia and progressive right upper and lower limb weakness. Magnetic resonance imaging of the brain depicted 2 intra-axial mass lesions. Stereotactic biopsy showed low grade fibrillary astrocytoma in both lesions. This report emphasizes that patients with Ollier's disease are at a higher risk for primary brain tumors than has been previously recognized

Endoprótese näo convencional de quadril "bloqueada" em um caso de condrossarcoma secundário à doençä de Ollier / Unconventional \"blocked\" hip endoprosthesis in a case of chondrossarcoma due to Ollier disease

Os autores apresentam o emprego de cuma endoprótese convencional de metade proxímal do fêmur com o componente acetabular bloqueado em um paciente com condrossarcoma secundário à doença de Ollier; discutem sua indicaçäo, resultado funcional imediato e a evoluçäo do caso