Disorders associated with osteopoikilosis

Although osteopoikilosis is generally considered an accidental finding, several developmental dysplasias coexisting with this disorder have been reported. However, all authors have mentioned only one coexisting finding, and most of them are case reports. We report a family in whom various members had osteopoikilosis with 5 different associated lesions. We suggest that osteopoikilosis is a bone manifestation of a generalized fibroproliferative or stenosing disease.     

Fifteen-year-old patient with osteopoikilosis

After accidental sport traumatisms, radiological exploration of the injured zone can show infrequent accidental findings. We present the case of a 15-year-old girl, with no significant medical history, who on investigating a wrist traumatism revealed multiple sclerotic areas in the carpal bones in X-rays with no associated symptoms. This is a rare case of osteopoikilosis.     

Contribution to the knowledge on natural history of giant hepatic angioma

Hepatic haemangiomas are mostly discovered by chance because of their limited dimensions. Their treatment is optional and very often an observing conservative strategy is adopted whilst a danger is foreseeing from different facts. Very different is the case of giant haemangiomas discovered because their bulk and discomfort coming from the compression exerted on near structures. In this cases a surgical treatment, segmentectomy or hemiepatectomy, are the current demanding choices. But if the volume of haemangioma is too bulky and occupies most hepatic parenchyma the necessary resection may be too extended and possibly dangerous. The two observations of the paper refer to two patients followed conservatively for over 20 years. In fact the volume of the haemangiomas in both patients was too large, the symptoms were only related to the weight of the mass and therefore a surgical solution was deferred to a possible worsening of the symptomatology. Such worsening didn't happen in the time for both the patients, demonstrating that the natural history of such lesion can also be very benign over many years.     

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.     

A new case of osteopoikilosis

Osteopoikilosis is an autosomal-dominant bone dysplasia characterized by the presence of multiple hyperostotic areas in different parts of the skeleton. It was first described by Albers-Schönberg in 1915, and it is also named “osteopathia condensans disseminata”. It is an unusual abnormality with a prevalence of less than 0.1 per million [3].     

骨斑点症1例报道

骨斑点症又称骨斑驳症,全身脆性骨硬化,是一种罕见的家族性常染色体显性遗传性骨病。骨斑点症好发于手、足短骨,管状骨的干骺端、骨端和骨盆、肩胛骨等扁骨、不规则骨,很少累及颅骨、肋骨、脊柱、胸骨、锁骨。1临床资料患者,男,52岁,因外伤后X线片检查发现双侧股骨近端及骨盆诸骨可见对称分布的多发小圆形,椭圆形骨硬化斑点阴影,边界清楚,骨硬化斑大小不一,直径2-10 mm,两侧股骨颈病灶呈长条状,并与股骨颈长轴一致。实验室检查未见异常。     

A rare form of osteopoikilosis

The authors report a typical case of osteopoikilosis striata Voorhoeve in a 51-years-old female. In connection with case they make some consideration concerning the diagnostic of this very infrequent sclerotic bone dysplasia.