Osteogenesis imperfecta als seltene Ursache eines Megacolons

Beduhn, D; Daum, Robert S.

doi:10.1055/s-0029-1228881

Fortschr Röntgenstr

1970

DOI: 10.1055/s-0029-1228881

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Osteogenesis imperfecta als seltene Ursache eines Megacolons

D Beduhn¹,

Robert S. Daum²

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1983

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Cited by 5 publications

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“…Megacolon, an abnormally dilated large bowel usually transverse, has been described in association with many and varied conditions from the classic ulcerative colitis to bilateral pheochromocytoma and even osteogenesis imperfecta [1,2]. Differentiation of nonsurgical from surgical causes is paramount especially for the radiologist, who is frequently given little clinical information.…”

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confidence: 99%

Vincristine-induced megacolon

Rosenberg

Caridi

1983

Gastrointest Radiol

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mentioning

confidence: 99%

Vincristine-induced megacolon

Rosenberg

Caridi

1983

Gastrointest Radiol

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Untitled

Violas¹,

Fassier²,

Hamdy³

et al. 2002

J Pediatr Orthop

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Acetabular Protrusion in Osteogenesis Imperfecta

Violas

Fassier

Hamdy

et al. 2002

Journal of Pediatric Orthopaedics

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The charts and radiographs of 79 patients with osteogenesis imperfecta (OI) actively followed at the authors' institution were reviewed to determine the incidence of acetabular protrusion (AP) in OI and to evaluate the clinical consequences of this pelvic deformity. All 79 patients had iliac crest bone biopsy and pelvic radiographs. They were subdivided into OI type 1, 3, and 4, according to the Sillence classification, and type 5, according to Glorieux. AP was measured on pelvic radiographs, using the center-edge angle of Wiberg and the acetabular ilioischial line. Twenty-six patients (33%) had radiologic evidence of AP, with the highest incidence in OI type 3 (69%) and OI type 5 (54%). Only 3 of these 24 patients (13%) had medical problems that could be related to AP: severe constipation and hip ankylosis. The authors conclude that in this series of 79 patients with OI, AP is not uncommon and in rare cases may lead to severe medical complications. Key Words: Acetabular protrusion-Osteogenesis imperfecta.Osteogenesis imperfecta (OI) is a genetically and clinically heterogeneous group of disorders of bone and connective tissue characterized by osteoporosis, fragile bones, multiple fractures, bony deformities, hyperextensible joints, dentinogenesis imperfecta, blue sclerae, and adult-onset hearing loss. In 1979, Sillence et al. (9) proposed a classification that included four types: type 1, a mild form; type 2, the most severe form, causing perinatal death; type 3, a form with a high frequency of fractures, causing severe deformity and short stature; and type 4, in which deformities and short stature are present but to a lesser degree than in type 3. More recently, Glorieux et al.(3) added a type 5 to this classification, characterized by hypertrophic bone callus and ossification of the interosseous membranes in the legs and forearms.Although the radiologic findings of acetabular protrusion (AP) in OI have been well described, the incidence of AP in each type of OI has not been previously reported in the French or English literature to the best of our knowledge. The purpose of this study was to evaluate AP in our own series of OI patients, specifically looking at the incidence of this deformity in each type and at the clinical consequences of this pelvic deformity in OI. METHODSThe charts and radiologic films of all consecutive patients with OI actively followed at our institution were retrospectively reviewed. There were a total of 240 patients, of whom 161 were excluded either because they were not typed yet with iliac crest bone biopsy or because no pelvic radiographs were available. This left 79 patients, which constituted the subjects of this study. These were subdivided into four groups (type 1, 3, and 4 according to the classification of Sillence, plus type 5 according to the Glorieux classification). The lethal form (type 2) was excluded. The OI types, mean age, and sex are shown in Table 1.The AP in all 79 patients was measured (Fig. 1) using the Kohler line, as reported by McCollum and Nunley (8), and t...

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Osteogenesis imperfecta als seltene Ursache eines Megacolons

Cited by 5 publications

References 0 publications

Vincristine-induced megacolon

Vincristine-induced megacolon

Untitled

Acetabular Protrusion in Osteogenesis Imperfecta

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