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PubMed1984

Osteopoikilosis: a radiological and pathological study.

Anatomico-pathological and radiological studies of osteopoikilosis were performed in two cases, one involving a femoral head, excised after a fracture of a femoral neck, in an elderly man and the other following biopsy of an iliac crest in a young woman. In both patients wide-spread radiological evidence of the disorder was present as an incidental finding. The radiological appearance of rounded and linear densities corresponded to old and inactive remodelling of spongy trabeculae in epiphyseal and metaphyseal locations. The distribution and appearance of these osteopoikilotic densities suggested them to have been related intimately to mechanical strain on spongy bone trabeculae. The diffuse nature of the lesions, their hereditary character, and their possible association with abnormalities of the skin suggest the existence of a particular terrain in which general metabolic conditions of connective tissue may interact with mechanical stresses in bone. Careful analysis of the findings in osteopoikilosis is desirable in order to provide data concerning the physiopathology of the skeleton and to permit more definitive interpretation of localised areas of bone condensation, including those observed not only in solitary bone islands and such conditions as osteopathia striata, but also those associated with infections and tumours.

Adult↗
PubMed1991

Mixed sclerosing bone dystrophy with features resembling osteopoikilosis and osteopathia striata.

A 64-year old man, presenting pain in his back and left sciatalgia, was found to have a mixed sclerosing bone dystrophy with features resembling osteopoikilosis and osteopathia striata. Oval and round densities were found in the humeral heads, elbows, wrists, hands, pelvis, knees, feet. Striata densities were in the diaphyses of metacarpal and metatarsal bones. Bone scan was negative. Standard biochemical examinations of the blood and urine were negative. According to our investigations no evidence of osteopoikilosis other sclerosing bone dystrophies were found in the family of our patient. These data were discussed.

Aged↗
PubMed2005

Coexistence of osteopoikilosis with reactive arthritis: a case report.

Osteopoikilosis, a form of osteosclerotic disorders, is a rare bone dysplasia with typical radiologic findings. The pathology, which sometimes requires attention in differential diagnosis due to its radiologic appearance, has been reported previously with other diseases. In this report, a case diagnosed with both reactive arthritis and osteopoikilosis will be presented and current arguments about its differential diagnosis and pathophysiology and concomitant disorders shall be discussed.

Adult↗
PubMed2005

Osteopoikilosis in an ancient skeleton: more than a medical curiosity.

We describe the palaeopathologic and radiographic findings of the human skeletal remains that belonged to a female who lived in Mexico's viceroyship period (seventeenth and eighteenth centuries A.D.). Radiographic studies showed numerous, radiodense, ovoid, small and well-defined foci in the long tubular bones, sacrum, scapulae and iliac bones. Computed tomography (CT) examination revealed multiple hyperdense foci located in the central marrow portion of the bones. Measurements of attenuation coefficient revealed +1548 HU. The findings are consistent with osteopoikilosis, an uncommon, benign sclerosing bone dysplasia transmitted in an autosomal dominant fashion, which in the clinical setting is important to set apart from different bone pathologies to avoid unnecessary interventions and treatments. To the best of our knowledge, this is the first report of osteopoikilosis in ancient human remains.

Bone and Bones↗
PubMed1989

Connective tissue nevus and osteopoikilosis in the hand: the Buschke-Ollendorff syndrome.

The Buschke-Ollendorff syndrome is a rare disorder of uncertain etiology characterized by osteopoikilosis and connective tissue nevi. An unusual presentation of this syndrome is described that involves both the cutaneous and skeletal manifestations affecting the hand. The bones of the hand and carpus are a common site for osteopoikilosis. Connective tissue nevi of the hand may require excision for diagnosis or mechanical impingement. Special elastin stains must be done on these cutaneous lesions to confirm the diagnosis. Routine hematoxylin-eosin stains may fail to show any pathologic change in mild cases.

Child↗
PubMedDate not supplied

[A case of osteopoikilosis combined with dermal changes and compression syndromes of peripheral nerves].

In the case of combination of osteopoikilosis with dermal alterations we wanted to know if the hereby discussed general mesenchymal lesions are the cause of the additional entrapment syndromes of peripheral nerves present in our case. For this purpose we recorded the pressure at the distal median and ulnar nerves within and out of the entrapment location. The results of the pressure recording of not point to a primary nerve lesion by a elevated pressure susceptibility or a pressure elevation at the peripheral nerve out of a defined entrapment location e.g. by an increase of connective tissue. Because of a hypertrophic scar formation in this case it should be paid attention to the wound healing of all patients with osteopoikilosis. The histologically verified nevoid mesenchymal alterations of the connective tissue found in this case, are to be delineated from the disseminated lenticular dermatofibrosis of the Buschke-Ollendorff syndrome.

Bone and Bones↗
PubMed1993

Dacryocystitis associated with osteopoikilosis.

We report five members of a family with dacryocystitis associated with osteopoikilosis. The inheritance is autosomal dominant. Review of the literature revealed no other report of this kind of association. Osteopoikilosis must not be considered as a coincidental radiographic finding but as part of a systemic disorder.

Adolescent↗
PubMed1992

Epidemiological, clinical and radiological aspects of osteopoikilosis.

Osteopoikilosis is a rare condition showing characteristic sclerotic lesions on radiographic examination, which are diagnostic for the trait. We report four patients presenting with various complaints and 49 members of their families who later were found to have osteopoikilosis. The mean age of all 53 was 27.5 years and the male:female ratio was 33:20. Most had lesions in the small tubular bones. We studied the epidemiological, clinical and radiological features of these patients and from the pedigrees conclude that the disease is inherited as an autosomal dominant.

Adolescent↗
PubMed2000

[Osteopoikilosis: report of 3 cases and review of the literature].

Osteopoikilosis, osteopathia condensans disseminata, is a rare hereditary autosomal dominant sclerosing bone dysplasia, more common in males. The diagnosis is usually made incidentally from radiographs which show multiple, small, well-defined, variably shaped and widely distributed (over the skeleton) sclerotic areas. The involvement is symmetrical, and the predilected locations are the phalanges of the hand, carpal bones, metacarpals, foot phalanges, metatarsals, tarsal bones, ilium, femur, radio and sacrum. It must be distinguished from melorheostosis, osteopathia striata and fundamentally from osteoblastic bone metastases, on the basis of the clinical, radiological (roentgenographs, computed tomography and magnetic resonance) and radionuclide scanning characteristics. Histologically, there are focal condensations of compact lamellar bone within the spongiosa. We report three cases of osteopoikilosis and review the literature. Two cases didn't have affectation in phalanges of the hand, which had not been previously reported, to our knowledge.

Adult↗
PubMed2002

[Femoral neck fracture in osteopoikilosis].

Osteopoikilosis was discovered in a 53-year-old patient who suffered a fracture of the right femoral neck. Plain X-rays demonstrated symmetrical bilateral stippling of variable size. No images were seen in the skull and spine. The fracture of the femoral neck was treated by total hip replacement. At surgery, the joint cartilage of the femoral head and the acetabulum had a normal aspect. Pathology examination of the femoral head demonstrated regular thick dense lamellae. Functional outcome at ten months was good (Postel-Merle-d'Aubigné score). Osteopoikilosis is an uncommon clinically latent condensing bone disease which can be revealed by bone fracture.

Arthroplasty, Replacement, Hip↗
PubMed2005

[Osteopoikilosis--rare, hereditary determined, benign bone dysplasia. case report].

Osteopoikilosis is a rare, hereditary disorder of bone tissue. Most frequently this abnormality is asymptomatic and diagnosed incidentally on the basis of X-ray images, taken on other occasions. The characteristic radiographic appearance consists of oval or round, well-defined osteosclerotic foci in various sites of skeleton (pelvis, hands and feet, epiphyses of long bones). In the present paper a case of inborn osteopoikilosis in 23-year-old female patient is described. Typical bone abnormalities founding in her mother implicate an inherited character of this disorder.

Adult↗
PubMedDate not supplied

Reasoned approach to multiple osteosclerotic lesions. Combined diagnostic imaging in a case of osteopoikilosis.

The case of a female patient who had undergone mastectomy for infiltrating ductal carcinoma and come to the Orthopedics Division for lumbosacral pain unresponsive to conventional treatment is discussed. Pelvic X-ray, CT and scintigraphy were performed. The presence of multiple areas of selerosis of the pelvis and proximal femurs were documented. The differential range could be limited to osteoblastic metastasis and osteopoikilosis. The diagnosis of osteopoikilosis was established based on scintigraphy which proved the absence of uptake of the radiotracer.

Adult↗
PubMedDate not supplied

[Osteopoikilosis: 2 clinical cases].

Osteopoikilosis is a rare, benign and autosomal dominant bone disease. It is usually asymptomatic and diagnosed as a radiological finding. Plain X- Ray films show multiple sclerotic lesions on periarticular areas, epiphyses and metaphyses of long tubular bones. The authors describe two cases of osteopoikilosis in subjects belonging to the same family (brothers).

Adult↗
PubMed1990

[A case of osteopoikilosis].

On the basis of literature and own observation a very rare form is discussed of skeletal system anomaly--osteopoikilosis+ which is considered to be a genetically determined, benign skeletal dysplasia. It manifests itself with numerous spotty densities of bony structure situated usually near the joints of long bones. They may be erroneously regarded as malignant metastases. The reported case of osteopoikilosis+ conformis the hereditary character of the disease.

Adult↗
PubMed1990

[Melorheostosis, osteopoikilosis and ostiopathia striata. Their clinical significance and the value of scintigraphy in the differential diagnosis].

Melorheostosis, Osteopoikilosis and Osteopathia striata Clinical Relevance and Bone Scintigraphy Melorheostosis, Osteopoikilosis and Osteopathia striata are rare conditions. Although there is no casual therapy for any of the three diseases, differential diagnosis is important because concomitant or resulting impairments deserve orthopaedic or medical treatment. The analysis of typical cases with their clinical, roentgenological and radionuclide presentation shows the need of an accurate diagnosis to prevent senseless treatment or lost therapeutic opportunities.

Adult↗
PubMed1989

[Changes in phosphorus-calcium metabolism in osteopoikilosis (Buschke-Ollendorf syndrome)].

Osteopoikilosis has always been described as a radiological finding of no clinico-biological significance with no deforming consequences for the skeleton. The authors present three cases with a past family history (mother suffered from osteopoikilosis and two sons with Buschke-Ollendorf syndrome) in whom we found alterations of phosphorus and calcium metabolism, this not having been previously reported in the literature.

Adult↗
PubMed1989

[Osteopoikilosis--fracture healing].

Between 1956 and 1988 five cases of osteopoikilosis have been observed. All cases were found by chance. In two cases there were fractures: one of the femur shaft, which has been stabilized by intramedullary nailing, one of the fifth metacarpal bone which has been treated conservatively. In both cases no delay of bony union could be observed. Osteopoikilosis- as a mesenchymal lesion--seems to have no negative influence on fracture healing.

Bone and Bones↗