Cranial Sclerosis with Striated Bone Disease (Osteopathia Striata)

Piechowiak, H; Goebel, F. D.; Hirche, U.; Tyrell, Richard

doi:10.1055/s-2008-1033900

Cited by 16 publications

(9 citation statements)

References 4 publications

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“…In our case 3 the clavicles were straight and long with broad medial and lateral ends, causing marked protuberances on the shoulder. Similar clavicular changes were mentioned by others [Gay et al, 1994;Piechowiak et al, 1986;Winter et al, 19801. Involvement of the skull is manifested by increased thickness and density mostly of the cranial base, but also of the other parts.…”

Section: Discussionsupporting

confidence: 86%

“…*Bass et al, 1980;Bloor, 1954;de Boer and van Gool, 1974;Clement et al, 1982;Clementi et al, 1993;Cortina et al, 1981;Currarino and Friedmann, 1986;Culver and Thumasathit, 1972;Franklyn and Wilkinson, 1978;Gay et al, 1994;Goodmann and Robertson, 1993;Hoeffel and Merle, 1990;Horan and Beighton, 1978;Hurt, 1953;Jones and Mulcahy, 1968;de Keyser et al, 1983;Kornreich et al, 1988;Nakamura et al, 1985;Odrezin and Krasikov, 1993;Paling et al, 1981;Piechowiak et al, 1986;Robinow and Unger, 1984;Sevaux and Galmiche, 1970;Schnyder, 1980;Taybi and Nurock, 1969;Walker, 1969;Winter et al, 1980; own cases 11-2, 111-1,111-2, IV-1, and IV-2.…”

Section: Discussionmentioning

confidence: 99%

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Osteopathia striata with cranial sclerosis: Variable expressivity in a four generation pedigree

König¹,

Dukiet²,

Dörries³

et al. 1996

Am. J. Med. Genet.

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Osteopathia striata is a manifestation of several bone dysplasias. In association with cranial sclerosis it represents a separate entity, which is not limited to the bones but may affect other structures, leading to abnormal face, cleft palate, deafness, heart defects, and vertebral anomalies. Neurological findings range from normal development to marked retardation with hydrocephalus, cranial nerve deficiencies and deafness. Ten families, including our own, clearly demonstrate autosomal dominant inheritance with female preponderance and great inter‐ and intrafamilial variability. © 1996 Wiley‐Liss, Inc.

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Osteopathia striata with cranial sclerosis: Variable expressivity in a four generation pedigree

König¹,

Dukiet²,

Dörries³

et al. 1996

Am. J. Med. Genet.

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“…Osteopathia striata with cranial sclerosis was described by Hurt [1953] and was established as an entity with autosomal dominant inheritance by Horan and Beighton [1978]. However, no association of bone fragility and/or short stature was described previously [De Keyser et al, 1983;Piechowiak et al, 1986;Kornreich et al, 1988;Clementi et al, 1993;König et al, 1996].…”

Section: Discussionmentioning

confidence: 99%

Unclassified sclerosing bone dysplasia with osteopathia striata, cranial sclerosis, metaphyseal undermodeling, and bone fragility

Nakamura

Nakada²,

Nakada³

1998

Am. J. Med. Genet.

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Sclerosing bone dysplasias are diagnosed on the basis of a characteristic pattern of osteosclerosis and clinical manifestations; in many of them, cause and pathogenesis are still unknown. A 33-year-old man had five fractures of the humerus, tibiae, and femur as a result of mild traumatic incidents that occurred between the ages of 18 and 33 years as well as a remnant of rib fractures without apparent trauma on radiographs. His height was 158 cm (-2.2 SD). Radiographic evaluation showed cranial sclerosis, longitudinal striations in the metaphyses of the femur and tibia, fan-like striation in the ilium, metaphyseal widening in the femur and tibia, and sclerosis of the ribs. The blood chemistry findings, including serum calcium, phosphorus, and alkaline phosphatase, were normal. Biopsy from the ilium showed thick trabeculae composed of woven bone. The coexistence of osteopathia striata, cranial sclerosis, metaphyseal undermodeling, and bone fragility has not been recognized previously. Our case appears to represent a new form of sclerosing bone dysplasia.

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“…Prevalence is less than 0.1 in 1 million, with predominantly female phenotype[ 4 ]. In fact, OS-CS is 2.5 times more common in female with more severe complications than male patients[ 13 ]. The linear striations of the long bones typically first appear between five months and six years of age and are usually of little clinical significance[ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Osteomyelitis in an Osteopathia Striata with Cranial Sclerosis Patient

Park

Kim

et al. 2014

Maxillofacial Plastic and Reconstructive Surgery

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Osteopathia striata with cranial sclerosis (OS-CS) is characterized by linear bone dysplasia at the long bone radiographically and sclerotic change at the cranium. The purpose of this case report is to study the symptoms and treatments of osteomyelitis in a patient with OS-CS. A 41-year-old patient had pus discharge from a fistula at the mental region and increase in radiolucencies with sequestra in panoramic radiograph images. Computed tomography (CT) as well as radiograph images for the whole skeleton were taken. The patient was diagnosed with OS-CS. Sequestrectomy and fistulectomy were performed. The patient recovered and no relapse occurred within six months after surgery. For diagnosis of OS-CS, CT and additional radiograph images for the whole skeleton are required. Because of the increased bone density, this patient is prone to relapse after sequestrectomy. Therefore, the surgeon must minimize trauma with the least incision and exfoliation, and preoperative antibiotics.

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Cranial Sclerosis with Striated Bone Disease (Osteopathia Striata)

Cited by 16 publications

References 4 publications

Osteopathia striata with cranial sclerosis: Variable expressivity in a four generation pedigree

Osteopathia striata with cranial sclerosis: Variable expressivity in a four generation pedigree

Unclassified sclerosing bone dysplasia with osteopathia striata, cranial sclerosis, metaphyseal undermodeling, and bone fragility

Osteomyelitis in an Osteopathia Striata with Cranial Sclerosis Patient

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