Background: Ollier's disease is a non-hereditary, benign bone tumor which is usually characterized by presence of multiple radiolucent lesions (enchondromas) in the metaphysis of long bones with unilateral predominance. The disease is a rare clinical entity with 1/100000 occurrence in early childhood. Patients mostly present with multiple hard swellings and deformity of the tubular bones specially hands and feet with leg discrepancy and pathologic fractures.
Case Presentation: We present two cases of Ollier's disease in a 13 years old female and 8 years old boy which had no specific symptoms. The girl had multiple hard swellings and deformity in the fingers of both hands and left toes with left leg deformity and discrepancy. Her plain radiographs demonstrated multiple expansile enchondromas in the phalanges of hands, left toes and metaphyses of upper humeri as well as left leg bones. The enchondromas were also noted in the left iliac bone and anterior end of ribs. The boy had bowing deformity and shortage of left leg with multiple enchondromas in the metaphyses of left femur, left tibia and fibula as well as left iliac bone in his radiographic images.
Conclusion: Ollier's disease is usually diagnosed by clinical signs and typical location of enchondromas across skeleton in conventional radiography. It usually does not need specific treatment. Well understanding of the clinical manifestation and radiographic features can prevent unnecessary application of other imaging modalities; while other diagnostic imaging modalities like MRI, ultrasound and scintigraphy can be used in complicated and painful conditions.
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http://dx.doi.org/10.1186/s12880-017-0230-8 | DOI Listing |
BMC Musculoskelet Disord
January 2025
Department of Pediatric Orthopaedics, Children's Hospital of Fudan University, National Children's Medical Center, 399 Wanyuan Rd, Minhang District, Shanghai, 201102, China.
Purpose: Ollier's disease (multiple enchondromatosis) can cause severe lower limb length discrepancy and deformity in children. Osteotomy and limb lengthening with external fixation can correct the lower extremity deformity. There may be lesions in the osteotomy part (OP), and the internal fixation part of the external fixation(FP).
View Article and Find Full Text PDFJ Orthop Case Rep
December 2024
Department of Orthopaedics, ACS Medical College and Hospital, Dr MGR Educational and Research Institute, Chennai, Tamil Nadu, India.
Introduction: Enchondroma protuberans (EP), a rare form of enchondroma with exophytic growth, differs radiographically from classical enchondromas and can mimic osteochondroma, periosteal chondroma, or chondrosarcoma. Proper differentiation is crucial to avoid unnecessary radical resection, as EP requires intralesional curettage rather than the surgical removal typical for osteochondromas.
Case Report: A 14-year-old male presented with a progressively enlarging, painless mass on the lateral aspect of his left hand, initially noticed 4 years ago.
Orphanet J Rare Dis
November 2024
Sorbonne Université, Inserm, CNRS, UMR S 1127, Institut du Cerveau, ICM, AP-HP, Hôpitaux Universitaires La Pitié Salpêtrière - Charles Foix, Service de Neuro-oncologie, Paris, France.
Sci Rep
October 2024
Department of Orthopedic Surgery, Duke University School of Medicine, Durham, NC, USA.
Enchondromas are a common tumor in bone that can occur as multiple lesions in enchondromatosis, which is associated with deformity of the affected bone. These lesions harbor somatic mutations in IDH and driving expression of a mutant Idh1 in Col2 expressing cells in mice causes an enchondromatosis phenotype. Here we compared growth plates from E18.
View Article and Find Full Text PDFSICOT J
October 2024
Service de Chirurgie Pédiatrique, CHU Rennes, Hôpital Sud, 16 Boulevard de Bulgarie, 35200 Rennes, France.
Introduction: Leg length discrepancy (LLD) and malalignment of long bones are frequent orthopedic problems encountered in Maffucci syndrome and Ollier disease (OD). Orthopedic surgeons used historically external fixators to address the deformities. In this multicentric case series, we propose the use of motorized intramedullary nails.
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