Ollier's disease and maffucci's syndrome: distinct entities or a continuum

1988 ◽  
Vol 235 (6) ◽  
pp. 376-378 ◽  
Author(s):  
C. D. Mellon ◽  
J. E. Carter ◽  
D. B. Owen
2004 ◽  
Vol 43 (8) ◽  
pp. 705-710 ◽  
Author(s):  
Georges Noël ◽  
Loïc Feuvret ◽  
Valentin Calugaru ◽  
Khalid Hadadi ◽  
François Baillet ◽  
...  

Children ◽  
2021 ◽  
Vol 8 (6) ◽  
pp. 502
Author(s):  
Aaron J. Huser ◽  
Jason Shih Hoellwarth ◽  
Valentino Coppa ◽  
David S. Feldman ◽  
Dror Paley

There are multiple forms of enchondromatosis with Ollier’s and Maffucci’s being the most prevalent types. Limb length discrepancy is a common problem in patients with Ollier’s and Maffucci’s enchondromatosis. There are multiple reports about lengthening bones in patients with enchondromatosis using external fixators. However, there are no case series regarding the use of implantable lengthening technology. The purpose of this paper is to describe our experience with implantable nail lengthening in patients with enchondromatosis. A retrospective chart and radiographic review of patients with enchondromatosis who underwent implantable nail limb lengthening was performed. Seven patients with 14 bony segments were reviewed. A total of 11/14 lengthenings were completed without difficulty. There were no issues in terms of fixation location in patients with Ollier’s disease. One patient with Maffucci’s syndrome experienced migration of the nail during two lengthenings due to a combination of intralesional fixation and preconsolidation. One patient with Ollier’s disease developed a knee extension contracture requiring manipulation under anesthesia. No other complications were recorded. The use of implantable nail lengthening to resolve limb length discrepancies in patients with Ollier’s disease appears to be safe and effective.


Neurosurgery ◽  
1990 ◽  
Vol 27 (3) ◽  
pp. 487-491 ◽  
Author(s):  
Stephan Patt ◽  
Klaus Weigel ◽  
Heinz Michael Mayer

Abstract We present a 24-year-old patient with multiple chondromas of both hands, the pelvis, the left leg, and an associated brain stem glioma. There was no evidence of hemangioma or dyschromia. and the condition was diagnosed as Ollier's disease, a special type of dyschondroplasia like Maffucci's syndrome and Kast's disease. An increased overall risk for development of malignant skeletal and nonskeletal tumors is associated with Maffucci's syndrome. The risk of malignant degeneration is lower in Ollier's disease. A glioma in the pons and the right lobe of the cerebellum was found in this patient. The literature describes an association with gliomas in only 12 cases of dyschondroplasia and an infratentorial localization in just one case. Signs of malignancy were histologically confirmed in 7 cases without significant preponderance of any one type. Our patient had a low-grade brain stem astrocytoma with fibrillar and gemistocytic components. A stereotactic serial biopsy made it possible to rule out malignant degeneration. Stereotactic brain tumor biopsy as a routine neurosurgical procedure is particularly valuable for deep space-occupying processes and forms the basis for therapy. In the present case, irradiation was not recommended.


Neurosurgery ◽  
1990 ◽  
Vol 27 (4) ◽  
pp. 625-628 ◽  
Author(s):  
Karl-August Bushe ◽  
Markus Naumann ◽  
Monika Warmuth-Metz ◽  
Jürgen Meixensberger ◽  
Justus Müller

Abstract Maffucci's syndrome is characterized by the combination of multiple enchondromas (Ollier's disease) and hemangiomatosis. These hemangiomas develop in the subcutaneous tissue and form red-blue tumors dispersed over the whole body. Intracranial involvement is rare, making a preoperative radiological diagnosis and differentiation from other tumors rather difficult. The radiological characteristics and successful removal of the intracranial part of a chondrosarcoma of the cerebellopontine angle in a case of Maffucci's syndrome are reported in this paper.


1999 ◽  
Vol 113 (9) ◽  
pp. 861-864 ◽  
Author(s):  
S. K. Ahmed ◽  
W. C. Lee ◽  
R. M. Irving ◽  
A. R. Walsh

AbstractOllier's disease and Maffucci's syndrome are similar multiple enchondromatous conditions. Other co-existing pathologies, particularly other primary malignancies, have been described sporadically in both conditions but more so in Maffucci's syndrome. Maffucci's syndrome is distinguished from Ollier's disease by the presence of haemangiomas in the former. In this report, a patient believed to have Ollier's disease for 44 years was subsequently found to have large splenic haemangiomas on magnetic resonance imaging (MRI). In the light of this, we recommend that any patient diagnosed with Ollier's disease to have total body MRI to search for haemangiomas that will alter the diagnosis to Maffucci's syndrome and hence the prognosis. MRI also screens for the presence of other associated malignancies.


2007 ◽  
Vol 65 (3b) ◽  
pp. 816-821 ◽  
Author(s):  
Leandro Infantini Dini ◽  
Gustavo Rassier Isolan ◽  
Glauco Andre Saraiva ◽  
Simone Afonso Dini ◽  
Pasquale Gallo

Maffucci's syndrome is a rare congenital condition, sometimes misdiagnosed as Ollier's disease, characterized by multiple enchondromas combined with hemangiomas and phlebectasia. Coexisting primary malignancies have been described sporadically. We report two cases of Maffucci's syndrome associated with cranial base chondrosarcoma, emphasizing pathophysiological features and the challenging management of intracranial chondrosarcomas. To the best of our knowledge, only twelve similar cases have been reported in the literature.


2017 ◽  
Vol 17 (1) ◽  
Author(s):  
Jamshid Sadiqi ◽  
Najibullah Rasouly ◽  
Hidayatullah Hamidi ◽  
Salahuddin Siraj

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