Biomedical subjects
Publications and source records attributed to K Kozlowski.
A 6-year-old Algerian boy with "caudal regression syndrome" and spondylo-epiphyseal dysplasia is reported. We believe this represents a new malformation syndrome or a random association of two non-related entities.
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16 cases of primary sacral bone tumours in children are reported. These include 13 patients with Ewing's sarcoma and 3 with very rare primary sacral bone tumours in childhood--chordoma, haemangiopericytoma and osteoblastoma. All sacral bone tumours, with the exception of Ewing's sarcoma are very rare in childhood. The possibility of a sacral tumour should be considered in a child with radiculopathy. CT and MR make the diagnosis of primary sacral bone tumours much easier with the added possibility of recognition of the true nature of the lesion in many instances. Reports of primary sacral bone tumours in children are scarce. Most of the patients are incorporated in adult series which do not specify the age of the child and the site of the tumour. The purpose of this paper is to describe 16 children with primary sacral bone tumours.
We present six cases of chondrodysplasia punctata with radiographic findings that are either very rare or previously unreported in this entity.
Four boys with congenital hypoplastic/dysplastic carpal changes are reported. In 3 patients the right carpus and in 1 patient the left carpus was involved. Two other males, not examined, in two previous generations were also probably affected. No females in the family are known to have the disease. Unilateral involvement, absence of progress of the changes with age and type of inheritance allows us to designate the abnormality as a new, X-linked syndrome.
Twelve cases of Mucolipidosis II (I-cell disease) with a wide range of severity of skeletal involvement were studied. Pathological findings in two cases provided helpful information in understanding the radiographic features of dysostosis multiplex. Inhibition of the growth plate cartilage calcification and rickets-like lesions were observed in the metaphyses. Enhanced subperiosteal remodelling and paratrabecular fibrosis were also evident in the diaphyses. High levels of parathormone were found in one case. This finding supports the hypothesis that bone lesions may be secondary, at least in part, to damage in such viscera as the kidney and/or the liver and that they are mediated by vitamin D and parathormone.
A new, dominantly inherited form of peripheral dysplasia in three members of an Algerian family is reported. Two further individuals, not investigated, are also probably affected. The disease is characterised by distinctive clinico-radiological findings. A case of acromesomelic dysplasia of Maroteaux is also reported and the differential diagnosis of peripheral dysplasias is discussed.
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Congenital cystic adenomatoid malformation (CCAM) of the lung is one of the rarest causes of neonatal distress. The principal radiological sign of CCAM is an intrapulmonary mass of soft tissue density, containing cystic areas of different sizes and shapes. The mass usually compresses the rest of the affected lung and displaces the mediastinum and heart to the opposite side, compressing the lung which is often therefore hypoplastic. If CCAM is diagnosed in utero by ultrasound, the treatment of choice is surgery as soon as possible after birth, with good survival rates. Sixteen cases of CCAM are presented, one with bilateral disease, diagnosed at different times, and one with an associated prune belly syndrome, to be added to the 405 already reported in the literature, and their clinical, radiological and pathological features are described.
Six boys with traumatic ischial apophysis are reported. Two cases were diagnosed as stress apophysis and four as apophyseolysis. Two of our patients were referred to the hospital as malignant bone tumours--Ewing sarcoma and osteosarcoma.
27 cases of primary malignant and benign rib tumours are reported. The most common malignant rib tumour in childhood is Ewing sarcoma (20 cases). Some other rare bone tumours and tumorous conditions (reticulosarcoma, aneurysmal bone cyst, monostotic rib eosinophilic granuloma, osteoid osteoma and lymphangioma) are also described. In the authors' opinion Ewing sarcoma presents with characteristic clinico-radiographic findings in most of the cases. Other monostotic, primary rib tumours and tumorous conditions in childhood--with the exception of exchondroma and enchondroma--rarely show diagnostic radiographic features.
30 children with Ewing's sarcoma, the most common malignant pelvic tumour in childhood, were analysed. The diagnosis of Ewing's sarcoma is relatively easy and can be established in most of the cases on plain radiography. The diagnostic radiographic features of the tumour are discussed. The two most important conditions in differential diagnosis are eosinophilic granuloma and the rare primary bone lymphoma. Osteomyelitis should rarely cause confusion unless the clinico-radiographic findings are not properly evaluated.
14 cases of rare, primary iliac, pubic and ischial bone tumours or tumorous conditions are reported. These include aneurysmal bone cyst, eosinophilic granuloma, cavernous haemangioma, osteoid osteoma, fibrous dysplasia, fibrous dysplasia with sarcomatous degeneration, chondrosarcoma, lymphoma and atypical malignant histiocytosis. The possibilities to be considered in the accurate radiographic recognition of primary tumours of iliac, pelvic and ischial bones are discussed.
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Explore the source record for details and available documents.