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         Enchondromatosis:     more detail

al. reported humeral lengthening in 6 extermities; the diagnosis was arthrogryposis1, enchondromatosis 1, posttraumatic hypoplasia 2, and congenital humerus
MAGDY ABDEL-MOTA'AL, M.D. , Orthopaedic Research Fellow . WILLIAM MACKENZIE, M.D. , Pediatric Orthopaedic Surgery Attending . March 12, 1996 CLINICAL CASE PRESENTATION ORTHOPAEDIC DEPARTMENT THE ALFRED I. DUPONT INSTITUTE WILMINGTON, DELAWARE INTRODUCTION Upper extremity limb length discrepancy is a rare occurrence. Although pediatric leg length equalization is well understood, surgical treatment of upper limb length discrepancy has rarely been described. This case report illustrates the use of Ilizarov's method for the treatment of humeral shortening secondary to septic neonatal growth arrest . CASE REPORT A three year one month old boy presented to AIDI with shortening of the right humerus. Clinical examination revealed 4 cm shortening of right arm and painless limitation of ROM of right shoulder; abduction was 150 degrees with 80 degrees at the glenohumeral joint and internal rotation was significantly limited. There was also 1 cm shortening of the right leg and a 10 degree valgus deformity of the same knee. Radiological examination showed absence of proximal epiphysis of the right humerus with marked deformity of the metaphyseal region. There was mild shortening of the right tibia in association with destruction of the lateral part of the proximal metaphysis and epiphysis. The diagnosis was neonatal sepsis with multifocal osteomyelitis involving the right humerus

62. Ollier Disease
enchondromatosis, or multiple enchondromas, refers to a group of disordersof which Ollier disease is the best known. This is nonhereditary

Up Neurofibromatosis Pyknodysostosis ... Multiple hereditary exostoses [ Ollier disease ] Achondroplasia Diastrophic dwarfism Chondrodysplasia punctata Thanatophoric dwarfism ... Dyschondrosteoses Enchondromatosis, or multiple enchondromas, refers to a group of disorders of which Ollier disease is the best known. This is nonhereditary disorder which usually presents in childhood. Nominally, the disease consists of multiple enchondromas. However, on radiographs, streaks of low density are seen projecting through the diaphyses into the epiphyses of the long bones, due to ectopic cartilage deposits. With age, the cartilage may calcify in the typical "snowflake" pattern. The affected extremity is shortened (asymmetric dwarfism) and sometimes bowed due to epiphyseal fusion anomalies. Patients are at risk for development of sarcomas, specifically osteosarcomas and chondrosarcomas. These occur in approximately 25 % of patients. Mafucci syndrome represents enchondromatosis with oft tissue hemangiomas, usually in the hands and feet. As with Ollier disease, there is typically a shortening of the long bones. These patients are at higher risk for sarcomatous transformation of both the vascular and cartilaginous portions of the disease. REFERENCES Flemming DJ, Murphey MD. Enchondroma and chondrosarcoma. Semin Musculoskelet Radiol. 2000;4(1):59-71.

63. Children's Mercy Hospitals And Clinics
These protuberances undergo ossification. Maffucci Syndrome A syndrome ofenchondromatosis, bone deformities, hemangiomas, and phlebolithiasis.

64. Oncology Diagnosis Information
Multiple enchondromatosis (Ollier's and Maffucci's syndromes) is less commonthan the enchondroma and does occur with increased frequency in families.
Orthopaedic Oncology
Determining Diagnosis
Tumors: Benign vs. Malignant Staging and Staging Studies
Benign Bone Tumors ...
Malignant Schwannoma (Neurofibrosarcoma)
DETERMINING DIAGNOSIS Your first office visit may be overwhelming because we attempt to confirm a diagnosis for you. You meet a lot of staff, hear a lot of information and may have a lot of tests performed. Your first visit maybe tiring, somewhat confusing and stressful depending on your situation.
If you brought blood results, MRI, bone or CT scans, we review all of them. If you have had a biopsy, we consult with our pathologists to review your pathology slides. If you have not had any of these studies done, we attempt to schedule all of them during your first visit.
Plain x-rays, CT scans of the tumor area and of your chest, MRI, PET and bone scans are called staging studies. These are the common studies performed to 'workup' a patient with a questionable tumor of the soft tissues or bone. They assist us in determining whether your tumor is benign or malignant, and whether the malignant ones (sarcomas) have traveled to other sites of your body. When a sarcoma has traveled, this is called a metastasis. If sarcomas metastasize, the most common site is the lung.
We will also schedule a biopsy that day if deemed necessary. A biopsy is a needle aspirate or surgical removal of a small piece of tissue for microscopic examination. There are four types of biopsies which are done to make a diagnosis:

65. Ollier Disease And Maffucci Syndrome - Tutorial
Characteristics of Ollier disease include multiple enchondromatosis, usuallyunilateral benign cartilage tumors of bone. Maffucci syndrome.
Ollier disease and Maffucci syndrome
Tutorial Frank J. Frassica, MD, Franklin H. Sim, MD

Tutorial Other benign cartilage tumors besides those mentioned in this section are Ollier disease and Maffucci syndrome. Characteristics of Ollier disease include multiple enchondromatosis, usually unilateral benign cartilage tumors of bone.
Maffucci syndrome
Maffucci syndrome - enchondromatosis that is associated with multiple cutaneous or viceral hemangiomas. Some of the hemangiomas have calcified walls which can be observed as seen on x-ray examination (they appear as small round calcifications phleboliths).
Ollier disease
To make the diagnosis of Ollier disease, the bone should be dysplastic. Patients may have multiple enchondromas with normal-appearing bones and, hence, they do not have Ollier disease. Ollier disease is a rare syndrome. Historically, we are taught that approximately 30% of patients with Ollier disease will develop a chondrosarcoma. These are usually very low-grade lesions. If the patient has associated soft tissue angiomas, then the patient has Maffucci syndrome. The risk of chondrosarcoma in Maffucci syndrome may be as high as 100%. Again, these are low-grade chondrosarcomas. The patient is more typically to die from a high-grade visceral carcinoma, such as an astrocytoma, pancreatic tumor, liver tumor, or breast cancer.

66. October 1998 Discussion
Diagnosis enchondromatosis (Ollier's disease). When enchondromatosis is associatedwith multiple hemangioma it is called Maffucci's syndrome.
Case of the Month ( October 1998 )
  • 5 year old girl. Similar lesions seen in multiple bones
Diagnosis: Enchondromatosis (Ollier's disease). Findings: Both knee joints AP view and Left knee magnified view A bone lesion with multiple linear channel like elongated radiolucencies are seen in the left lower femoral meta-diaphyseal region. Note the shortening and lateral bowing of the left femur. Another lesion of smaller size is also seen in the upper left tibia. Similar lesions were also seen in other bones. Discussion: Ollier's disease is characterized by multiple enchondromas distributed throughout the tubular and flat bones of the body. They involve one side of body either exclusively or predominantly. Affected bones are shortened and deformed. In childhood these lesions are subject to pathologic fractures and in adults (over 50 years of age) they are at risk of malignant transformation. Typically, patients present in the first decade with palpable bone masses, asymmetric shortening of extremities and osseous deformities related to fractures. On radiographs

67. Juvenile Granulosa Cell Tumor
Rare after 30 years. DISEASE ASSOCIATIONS, CHARACTERIZATION. Ollier's disease(enchondromatosis), Mafucci's syndrome (enchondromatosis and hemangiomatosis),
Background This tumor, as the name implies, usually presents in children before puberty. In spite of its name, it bears little histologic resemblance to the adult granulosa cell tumor. About 80% of these tumors presenting in children result in isosexual precocity. Tumors that present after puberty may have abdominal swelling and pain associated with menstrual irregularities or amenorrhea. OUTLINE Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing ... Internet Links
5% of all granulosa cell tumors AGE RANGE-MEDIAN 97% occur within first three decades
50% prepubertal Rare after 30 years DISEASE ASSOCIATIONS CHARACTERIZATION Ollier's disease (Enchondromatosis) Mafucci's syndrome (Enchondromatosis and hemangiomatosis) GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION General Bilateral in 2%
Ruptured at initial discovery in 10%
Ascites in 10% Size Average 12.5 cm
Range 3-32 cm Appearance Solid and cystic neoplasm with cysts containing hemorrhagic fluid Solid is yellow-gray with extensive necrosis or hemorrhage HISTOLOGICAL TYPES CHARACTERIZATION Classic Solid cellular neoplasm with focal follicle formation Solid areas with diffuse cells that may be divided into nodules by fibrous septa Granulosa and theca cells may be admixed Follicles vary in size and shape with eosinophilic or basophilic secretions staining positive for mucicarmine in 2/3 of cases

68. Foot Problems > Deformities > Enchondroma
Ollier's Disease, also known as enchondromatosis, frequently occurs in the smallbones in the hands and toes (phalanges) and the long bones behind the
3800 Highland Ave, Suite #103, Downers Grove, IL 60515
Foot Problems Deformities Enchondroma Amniotic Band Syndrome Bunions ... Tarsal Coalition Deformities - Enchondroma
Small tumors called enchondromas can sometimes form in the bone beneath the toenail.
An enchondroma is a fairly common benign tumor, which is the most common bone tumor of the hands and feet. The tumor can involve large portions of the bones, causing thinning of the cortex. This can weaken the bone and cause it to break spontaneously. When enchondromas occur in the small bone in the end of the toe, they can cause pain that may mimic the pain of ingrown toenails. Ollier's Disease , also known as enchondromatosis, frequently occurs in the small bones in the hands and toes (phalanges) and the long bones behind the phalanges called metatarsals. Maffucci's Syndrome is a very rare form of enchondromatosis associated with multiple soft tissue hemangiomas. This tumor frequently occurs in the hands and feet, and has a greater tendency toward malignant transformation than Ollier's Disease
The height of arches and the shape of the toes vary from person to person. It is the deviation from normal arches and toe position that lead to foot problems.

69. - Alles Over Het Menselijk Steun- En Bewegingsapparaat
Vaak komt de afwijking op zich zelf voor, maar soms in veelvoud (enchondromatosis).De kans op kwaadaardige ontaarding is bij op
beginpagina nieuws vragen lotgenoten ... professie Beentumoren Wilt u de nieuwsbrief ontvangen en meepraten?
Meld u dan aan! Kenmerken van een aantal goedaardige primaire beentumoren Chondroom Dit is een goedaardige gezwel, dat rijp kraakbeen vormt. De afwijking kan centraal in een skeletdeel liggen (enchondroom), of buiten het centrum tegen de wand van het bot (excentrisch chondroom). Vaak komt de afwijking op zich zelf voor, maar soms in veelvoud (enchondromatosis). De kans op kwaadaardige ontaarding is bij op zich zelf voorkomende chondromen 1%, echter bij enchondromatosis 20- 30%. Dan ontstaat een chondrosarcoom (zie Kwaadaardig primair Soorten ). Goede controle van chondromen is vaak om deze reden gewenst. De meeste chondromen komen aan de vingers voor, maar ook elders in het skelet. Niet alle chondromen behoeven behandeling; vaak kan men volstaan met controle. Exostose Dit is een benige uitwas van het bot, bedekt met een kraakbeenkap. De afwijking komt vooral bij kinderen voor; de afwijking kan tijdens de groei van het kind groter worden. Dat is normaal. Na de groei van het kind is groter worden van de Exostose vreemd; men moet dan rekening houden met kwaadaardige ontaarding. De kans op kwaadaardige ontaarding is 1% bij enkel voorkomende exostosen en minstens 10% bij leden van families, die deze afwijkingen vrijwel allemaal in veelvoud hebben. Zorgvuldige controle is bij hen gewenst. Exostosen komen in alle beenderen voor. Chondroblastoom Reusceltumor Dit is een goedaardige agressieve tumor, waarin talrijke reuscellen voorkomen. De tumor wordt in 4 stadia ingedeeld; 1 t/m 3 zijn goedaardig; 4 is kwaadaardig. Kwaadaardigheid is zeer zeldzaam. 90% van de patiënten is ouder dan 20 jaar. De gemiddelde leeftijd ligt bij 33 jaar. Ook deze tumor ligt vlak naast of in het gebied van de oorspronkelijke groeischijf, vooral in bovenarm, bovenbeen of onderbeen. Ook in de goedaardige variant komen er uitzaaiingen van de tumor in de longen voor. De prognose van de goedaardige variant is meestal goed; die van de kwaadaardige variant vaak slecht.

70. Multiple Enchondromatosis º Doc Nido º Multiple
Name MAFFUCCI SYNDROME Synonyms, multiple enchondromatosis º doc nidoº multiple enchondromatosis (codes). Definition The cardinal

71. A Csontrendszer Vizsgálata és Diagnosztikája Képalkotó Vizsgáló Módszere
a. multiple cartilaginous exostoses b. enchondromatosis c. polyostotic fibrousdysplasia d. neurofibromatosis. enchondromatosis több faja ismeretes.
A csontrendszer vizsgálata és diagnosztikája képalkotó vizsgáló módszerekkel
vezérfonal a radiológusok szintentartó továbbképzéséhez)
Írta: Dr. Weisenbach János
POTE, Gyermekklinika, Röntgen Részleg, Pécs I. Bevezetés
A csontok szervezetünknek egyik legcsodálatosabb rendszerét képezik, amely quantitative és qualitative tükrözi az egész szervezetünk müködõképességét, egészséges vagy pathológiás állapotát. Ha jól meggondoljuk, alig akad olyan fejlõdési rendellenesség, amelyhez nem csatlakozik valamilyen csontanomália. Alig van olyan vérképzõ szervi vagy hormonális megbetegedés, amelybõl teljesen kimaradna a csontstructura változása. Ha a gastrointestinalis tractusban vagy a húgyúti rendszerben keletkezik jelentõs, s hosszabb ideig fennálló zavar, a csontrendszerben is megjelenik valamilyen következményes változás, például a gyermek fejlõdésében való elmaradás, stb. Természetesen a csontrendszerben priméren jelentkezõ elváltozások kihatással vannak az egész szervezetre is. Jól kell tehát ismernünk a csontrendszeri elváltozásokat, hogy egy egységes egész képben jelenjenek meg képzeletünkben a változatos pathológiás állapotok, kórképek. A Gyermekradiológus Társaság egyik munkacsoportja a képzés és továbbképzés számára összefoglalót jeletetett meg a Pediatric Radiology (1995, 25, 401-407) oldalain, amely elsõsorban a residens képzést kívánja szolgálni a gyermekradiológiában. Ebben a curriculumban szerepel a csontrendszer pathológiájának felosztása is, s ezt a felosztást kívánom közreadni e vázlatos ismertetõmben, amely a hazai gyermekradiológiai továbbképzés keretén belül is hasznos lehet.

72. Skeletal Dysplasias
Syndactyly. enchondromatosis (Ollier); Fibrous dysplasia usual form(JaffeLichtenstein); enchondromatosis. Most enchondromas are solitary.
UW Radiology Home Approaches To DDx In Musculoskeletal Imaging Contents Preface ... Osteonecrosis Skeletal
Dysplasias Search this site

Approaches To Differential Diagnosis In Musculoskeletal Imaging
Michael L. Richardson, M.D.
Skeletal Dysplasias
In her classic children's book, The Tale of the Flopsy Bunnies , Beatrix Potter began the story with the following sentence: "It is said that the effect of eating too much lettuce is soporific". She might well have been talking about skeletal dysplasias. Surely there are few topics in radiology more soporific than that of skeletal dysplasias. The usual LD-50 for a chapter on dysplasia is about 1 - 2 minutes. Even so, some skeletal dysplasias occur with enough frequency that even general practice radiologists will see them from time to time and should know something about them. This chapter gives an extremely simplified meatball approach to the diagnosis of these disorders. You won't find any bird-headed dwarves on the differentials listed herein. (Note to the reader: the following paragraph contains a sappy but heartwarming story about dysplasias, aimed at convincing the reader of the importance of learning something about dysplasias. The information content of this chapter will resume in the next paragraph if you want to skip this one. You have been warned.)

73. Ovary And Peritoneum
Androgenic manifestations can occasionally occur; Interesting clinical associationwith Ollier's disease (enchondromatosis) and Maffucci's syndrome
OVARY normal gross normal micro
  • Presence of endometrial tissue outside the uterus
  • Ovary is common site
  • Majority of patients are of reproductive age
  • True prevalence unknown since many patients asymptomatic
  • Symptoms include pain, dysmenorrhea, dyspareunia,irregular vaginal bleeding, and infertility GROSS: MICRO:
    • Endometrial glands and stroma (both can be scant) with variable amount of old and/or new hemorrhage
    • Clinically can resemble neoplasia
    • Tumor-like enlargement of one or, uncommonly, both ovaries
    • Young patients (children to 4th decade)
    • Menstrual irregularity, abdominal pain and distension
    • Androgenic manifestations in 20%, associated with presence of lutein cells
    • Can be huge (up to 35cm in diameter)
    • Marked stromal edema often spares superficial cortex
    BENIGN SEROUS TUMORS (Cystadenomas, cystadenofibromas)
    • Common neoplasms (1/4 of all ovarian tumors) 50 -70% of all serous tumors
    • Peak age of incidence: 30's and 40's
    • Bilateral in 1/6 of the cases
    • Three patterns: cystic, papillary, adenofibromatous

74. E Index
ENCEPHALOMYELOPATHY. ENCEPHALOTRIGEMINAL ANGIOMATOSIS. enchondromatosis.Please click on the box below to view the following topics. E Part II.
A B C D ... Z Please click on the "box below" to view the following topics. E Part I EAR ANOMALIES/DISORDERS EAR INFECTIONS, ETC. ... ENCHONDROMATOSIS Please click on the "box below" to view the following topics. E Part II ENDOCRINE DISEASES ENTERAL NUTRITION ...

75. POHYBOVÉ ÚSTROJÍ, 3, 1996, è
Marík I, Kuklík M. Metachondromatosis combined with multiple cartilaginous exostosesand enchondromatosis a review and two severe case reports. Summary.
SOUBORNÉ REFERÁTY Kuklík, M.: SPARC - ubikviterní protein pojivových tkání organismu.................... 3 PÙVODNÍ PRÁCE Hajniš, K., Blažek, V., Brùžek, J.: Zmìny rùstu a proporcionality konèetinových segmentù v dìtství. Srovnání s nìkterými øády savcù ........................................ 10 Sobotka, Z.: Biomechanické funkce dolních konèetin a chodidel .......................... 28 Maøík, I., Èerný, P., Sobotka, Z., Korbeláø, P., Kuklík, M., Zubina, P.: Konzer va tivní terapie deformit páteøe dynamickou trupovoou ortézou ............................... 38 ZPRÁVY Životní jubileum Doc. Ing. Zdeòka Sobotky, DrSc. .............. 42 SMÌRNICE AUTORÙM LOCOMOTOR SYSTEM, Vol. 3, 1996, No. 1 CONTENTS REVIEWS Kuklík, M.: SPARC - ubiquituous protein of organism connective tissue ................. 3 ORIGINAL PAPERS Hajniš, K.,Blažek, V., Brùžek, J.: Changes of growth and proportionality of extremity segments in childhood. (Comparison w ith some orders of mammals) .......................10 Sobotka, Z.: Biomechanical functions of lower limbs and soles ........................ 28

76. Congenital, Developmental, Genetic
Diastrophic dwarfism. Multiple epiphyseal dysplasia. Multiple cartilaginous exostoses.enchondromatosis (Ollier andMaffucci). Infantile (juvenile) fibromatosis., Developmental, Gene

Packet No. 7 70433 IMAGE enchondromatosis (ollier disease) this radiograph showstypical features of enchondromatosis in the right femur of a 13year-old boy.

Packet No. 1 14971 SYNONYMS Congenital enchondromatosis Diaphyseal aclasis Dyschondroplasiaenchondromatosis Hereditary deforming chondrodysplasia Ollier

79. Eponyms - Full List
Mafucci's syndrome, Multiple enchondromatosis (Ollier's disease)+ multiple soft tissue haemangiomas may be cause of clubbing.
Addison's disease Primary adrenocortical insufficiency Adie syndrome Tonic pupil syndrome - associated with diminished or absent tendon jerks - common in young women. Agnell's rule In Wilm's tumor - after resection if no recurrence of tumor for a period of (child's age at presentation) + 9 months - child considered cured. Allen Brown's criteria For diagnosis of Raynaud's phenomenon Anderson Hynes operation Dysjunction pyeloplasty for repair of idiopathic P-U junction obstruction. Aphorism of Mayo, Charles "A Meckel's diverticulum is frequently suspected, often sought, and rarely found." Aphorism of Moynihan "A gallstone is a tombstone to the memory of the organism within it." Aphorism of Moynihan - 2 "The stomach is the mother of the GIT. With any disease of the tract, like a mother weeps, so is there vomiting." Arnold Chiari malformation Herniation of Clar tonsils thro foramen magnum - lower CN palsies+Clar limb signs+UMN signs in legs Atkins-Wolff operation Microdochectomy - done for persistent nipple discharge from 1 or few ducts. Bakamgian flap Deltopectoral flap - for repair of cheek defects like in Ca cheek - operated, or in cancrum oris.

80. Wheeless' Textbook Of Orthopaedics
Effusion, Hip Joint EhlersDanlos Syndrome Elastic Strain Electromyography ElbowJoint Enchondral Ossification Enchondroma enchondromatosis Endoscopic Carpal
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