Nora's Lesion: Bizarre Parosteal Osteochondromatous Proliferation Causing Splay Foot Deformity: A Case Report.

Nora's lesion, or bizarre parosteal osteochondromatous proliferation (BPOP), is a rare benign lesion that is made up of varying degrees of cartilage, bone, and spindle cells. Most notably, calcification of the cartilage or "blue bone," is a feature of the disorder. The condition principally affects long tubular bones of the hands and feet, and is generally seen in patients in their second and third decades of life. We present a case of BPOP occurring in the second interspace with symptoms that would be consistent with a more common diagnosis of predislocation syndrome, or a second interspace neuroma. This case study may help the clinician in considering a more subtle cause of a splay deformity in the second interspace, and walk through the diagnostic and treatment course for BPOP.

Osteochondromatosis and osteochondroma involving bilateral patella and patellar tendon: a case report.

We present a 22-year-old man with osteochondromatosis and osteochondroma involving bilateral patella and patellar tendon with restriction of joint movement and severe arthritis.

Nora's lemsion of the anterior Maxilla: a rare case report and literature review

Bizarre parosteal osteochondromatous proliferation (BPOP), or eponymically Nora's lesion, is a rare growing, to a certain extent, exophytic lesion usually emanating from the periosteum or its adjacent layers. BPOP is idiopathically painful. Given its cartilage-producing nature and pain, BPOP is viewed with great suspicion. Regular post-surgical radiographs and clinical follow-up are mandatory. In the gnathic bones, the lesion is extremely rare. To the author's best knowledge, this paper reports the sophomoric appearance of BPOP in the anterior maxilla, in an adult female (AU)

La proliferación paraosteal osteocondromatosa bizarra (PPOB), o lesión de Nora según su epónimo, es un crecimiento inusual, exofítico, que procede del periostio o sus capas adyacentes. La PPOB es idiopáticamente dolorosa. Dada su naturaleza cartilaginosa y el dolor que la acompaña, la PPOB resulta una lesión sospechosa. Son obligatorios el estudio radiológico y seguimiento clínico postquirúrgicos. En los huesos maxilares, esta lesión es extremadamente infrecuente. En lo que conoce el autor, este artículo es la primera descripción de la PPOB de maxilar, en una mujer adulta (AU)

Gammagrafía ósea de cuerpo completo con 99mTc-MDP de 3 hermanos afectos de osteocondromatosis múltiple familiar / 99mTc-MDP bone scintigraphy of three brothers affected by multiple osteochondromas

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Cavernous sinus syndrome due to osteochondromatosis in a cat.

A 1-year-old sexually intact male Korat cat was referred for ophthalmological consultation due to anisocoria. Mydriasis with external ophthalmoplegia and absence of pupillary light responses in the right eye and nasofacial hypalgesia were seen. Cavernous sinus syndrome (CSS) was suspected. Bilateral deformities of the jaw and phalangeal bones, severe spinal pain and abnormal conformation of the lumbar spine were also present. Radiographic examination revealed several mineralised masses in the appendicular and axial skeleton, indicative of multiple cartilaginous exostoses. For further investigation of the CSS-related neurological deficits, the cat underwent computed tomography (CT) examination of the skull. CT images revealed a non-vascularised, calcified, amorphous mass originating from the right lateral skull base and superimposing on the sella turcica. Based on the severity of diffuse lesions and owing to the clinical signs of extreme pain, the cat was euthanased. A diffuse skeletal and intracranial osteochondromatosis was diagnosed histologically.

Osteocondromatosis múltiple familiar: a propósito de un caso / Multiple familial osteochondromatosis: presentation of a case

Los encondromas son tumores benignos de cartílago que crecen de forma lenta en las metáfisis de los huesos. Pueden ser lesiones solitarias o múltiples. Las encondromatosis comprenden un grupo heterogéneo de síndromes, difíciles de diferenciar, caracterizados por la presencia de múltiples encondromas que pueden llegar a producir malformaciones musculoesqueléticas (secundarias al acortamiento de extremidades), escoliosis, fracturas patológicas o seudoartrosis. La complicación más temida, el osteocondrosarcoma, puede acontecer hasta en el 25% de los pacientes. Exponemos el caso de un varón de 67 años, sin diagnósticos previos conocidos, que consulta por la aparición en cadera izquierda de una tumoración dolorosa y rápidamente deformante en el último año. Los antecedentes familiares y los datos clínico-radiológicos confirmaron el diagnóstico de osteocondromatosis múltiple familiar. Aun cuando la evolución clínica y los estudios de imagen hicieron sospechar una degeneración maligna (osteocondrosarcoma), esta no se confirmó en el estudio histopatológico de la pieza quirúrgica (AU)

Enchondromas are benign cartilage tumours that grow slowly in the bone metaphysis. They may involve solitary or multiple lesions. Enchondromatoses include a heterogeneous group of hardly distinguishable syndromes characterised by the presence of multiple enchondromas that may cause musculoskeletal malformations (secondary to limb shortening), scoliosis, pathological fractures, or pseudoarthrosis. The most dreaded complication, osteochondrosarcoma, occurs in up to 25% of patients. We present the case of a 67-year-old male with no previous diagnosis, requiring attention due to the appearance of a painful tumour in his left hip which degenerated rapidly over the past year. Family history and clinical-radiological data confirmed the diagnosis of Multiple Familial Osteochondromatosis. Although clinical evolution and imaging led to suspect a malignant degeneration (osteochondrosarcoma), this was not confirmed by the histopathological study of the surgical sample (AU)

The role of hip arthroscopy in the treatment of synovial disorders and loose bodies.

Arthroscopic surgery of the hip is an evolving procedure that can facilitate the diagnosis and treatment of synovial-based disease. Radiographic studies have not reproducibly identified many of the synovial conditions that can affect the hip, and arthroscopy has provided a means to improved diagnostic accuracy and the ability to treat certain conditions. Arthroscopic intervention has been reported in synovial chondromatosis and osteochondromatosis, pigmented villonodular synovitis, inflammatory arthropathies including rheumatoid arthritis, and acute septic arthritis. Patients with other conditions that result in acute and chronic synovitis within the hip such as hemosiderotic synovitis secondary to hemophilia and chondrocalcinosis also may benefit from arthroscopic intervention. These synovial and intraarticular abnormalities have been associated with accelerated joint degeneration, and although the pathologic mechanisms have not been delineated clearly, the clinical description has been advanced using hip arthroscopy. Increased awareness of the association between synovial abnormalities and degeneration has resulted from arthroscopy applied to the hip in early stages of disease and early in the course of symptomatic dysfunction. The indications and treatment capabilities will continue to expand with additional understanding of early hip disease, improvements in equipment, and as outcome studies reveal that the patient benefits from the procedure.

Linear osteochondromatosis in a cat.

A domestic shorthair cat was presented with quadriparesis and lumbar hyperesthesia that progressed over 4 months. There were linear and amorphous radiopaque masses throughout the soft tissue surrounding the long bones, vertebral bodies, ribs, pelvis, and scapula. The diagnosis of osteochondromatosis was confirmed by histopathology. Unlike previously reported patients with osteochondromatosis, most of the calcified masses in this cat were not connected to the periosteum; some were linear and were arranged parallel to the long bones involved.

Neuropsychological findings in two children diagnosed with hamartoses: evidence of a NLD phenotypic profile.

Hamartoses (HM) are defined as disorders involving nonneoplastic tissue overgrowth. Studies have examined the neuropsychological profiles of children with common HM, such as neurofibromatosis type 1. Limited information is known regarding neuropsychological profiles of rare HM such as Osteochondromatosis Syndrome (OS) and Klippel-Trenaunay Syndrome (KTS). The current investigation is, to our knowledge, the first attempt to define the cognitive phenotypes in two boys with OS and KTS. Results revealed significantly greater impairments involving sensorimotor and visuospatial skills, while verbal memory and language skills appeared relatively preserved. Significant neurobehavioral problems and marked social difficulties were evident. These findings suggest that these syndromes are on a Nonverbal Learning Disorder (NLD) continuum, with varying degrees of severity.

Uncommon clinical course of multiple osteochondromatosis in a patient with a long-term history of Cushing's disease.

Cushing's disease (CD), the chronic endogenous hypercortisolism derived from an ACTH-secreting pituitary adenoma, and multiple osteochondromatosis (MO), a congenital mesoderm dyschondroplasia, represent two distinct rare neoplastic diseases. Clinical appearance of MO usually occurs during the first-second decade of life. In fact, the growth of osteochondromas parallels the patient's growth, then becoming quiescent after the closure of the epiphyses and the achievement of final stature. Here we describe an uncommon case of a patient with a long-term history of childhood-onset CD, who surprisingly developed MO during the third decade of life, after the remission of CD. Indeed, a female patient had been followed for CD from the age of 12 to the age of 24 years, when CD definitively remitted. At the age of 26 the patient complained progressively worsening backache and pain at level of hips and feet. Standard radiography of skeleton showed multiple bone dysmorphisms at level of the four limbs, spine and pelvis consistent with multiple osteochondromas and exostoses. A diagnosis of MO was performed. Total body bone scintigraphy with 99mTc-MDP revealed an increased uptake of the radioligand, suggesting an increased metabolic turnover in correspondence of the majority of the osteochondromas. However, the negativity of the majority of the lesions at 99mTc-DMSA scintigraphy and the histological diagnosis of benign osteochondroma of the only positive lesion at 99mTc-DMSA evidenced that the high metabolic activity of the osteochondromas was not due to malignant transformation. However, the activity of the lesions was highly surprising considering that they usually become quiescent after the achievement of the final stature. In last analysis, the uncommon characteristics of MO and, particularly, its occurrence after stable remission of hypercortisolism, suggests a possible role of glucocorticoids in influencing the clinical course of the skeletal disease. The inhibitory effect of hypercortisolism on bone growth and maturation could explain the block in the proliferation of skeletal lesions during the developmental age, where CD was in the active phase, and the opposite effect of stimulation of the ostochondromas growth during stable normalization of cortisol secretion, after CD remission.