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Professional awareness is needed to distinguish between child physical abuse from other disorders that can mimic signs of abuse (Skull base sclerosis in infant manifesting features of infantile cortical hyperostosis): a case report and review of the literature.

Al Kaissi A, Petje G, De Brauwer V, Grill F, Klaushofer K - Cases J (2009)

Bottom Line: Infantile cortical hyperostosis is characterised by hyperirritability, acute inflammation of soft tissue, and profound alterations of the shape and structure of the underlying bones, particularly the long bones, mandible, clavicles, or ribs.Narrowing of skull foramina due to sclerosis of skull base is likely to result in cranial nerves deficits.In this baby, the pathology has been judged to be the result of child abuse and it is not, in this case considerable harm to his parents, and the doctor-parent relationship was the outcome.

View Article: PubMed Central - HTML - PubMed

Affiliation: Ludwig Boltzmann Institute of Osteology, at the Hanusch Hospital of WGKK and, AUVA, Trauma Centre Meidling, 4th Medical Department, Hanusch Hospital, Vienna, Austria. ali.alkaissi@osteologie.at.

ABSTRACT

Background: Infantile cortical hyperostosis is characterised by hyperirritability, acute inflammation of soft tissue, and profound alterations of the shape and structure of the underlying bones, particularly the long bones, mandible, clavicles, or ribs.

Case presentation: We report on a clinical case of a 3-months-old baby girl of non-consanguineous parents. Multiple long bone swellings were the motive of referral to our department for clinical evaluation. Radiographic documentation was consistent with infantile cortical hyperostosis (Caffey disease). Interestingly, skull base sclerosis associated with excessive thickening was the most unusual malformation. We report a baby with mixed endochondral and intramembraneous ossification defects.

Conclusion: Bone dysplasias, mucopolysaccharidoses, and metabolic diseases are a group of disorders that cause abnormal growth, density, and skull base shape. Skull base sclerosis/thickening is a well-known malformation in connection with other forms of sclerosing bone disorders such as dysosteosclerosis, frontometaphyseal dysplasia, and progressive diaphyseal dysplasia with skull base involvement. It is noteworthy that our present patient had an unusually sclerosed/thickened skull base. Narrowing of skull foramina due to sclerosis of skull base is likely to result in cranial nerves deficits. In this baby, the pathology has been judged to be the result of child abuse and it is not, in this case considerable harm to his parents, and the doctor-parent relationship was the outcome.

No MeSH data available.


Related in: MedlinePlus

Anteroposterior radiograph of the tibia showed a thick and broad ballooning occupies the diaphyses (proximally and distally) there is gaining in the diameter comes from subperiosteal new bone apposition by intramembraneous bone formation.
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Figure 5: Anteroposterior radiograph of the tibia showed a thick and broad ballooning occupies the diaphyses (proximally and distally) there is gaining in the diameter comes from subperiosteal new bone apposition by intramembraneous bone formation.

Mentions: Anteroposterior radiograph of the skull showed massive sclerosis of the skull bone associated with significant cortical hyperostosis and enlargement of the mandible secondary to cortical new bone formation (fig 1). Lateral skull radiograph showed sclerosis of the skull base (arrow) and hyperostosis of the calvaria (fig 2). Coronal MRI imaging showed significant calvarial/facial and mandibular hyperostosis (fig 3). Anteroposterior radiograph of the radius showed cortical new bone formation associated with subperiosteal thickening. Note marked bloating along the diaphysis with sparing of the epi-metaphyseal components associated with expansion of the bone marrow cavity and a persistent-like deformity (fig 4). Anteroposterior radiograph of the tibia showed a thick and broad ballooning occupies the diaphyses (proximally and distally) there is gaining in the diameter comes from subperiosteal new bone apposition by intramembraneous bone formation (fig 5).


Professional awareness is needed to distinguish between child physical abuse from other disorders that can mimic signs of abuse (Skull base sclerosis in infant manifesting features of infantile cortical hyperostosis): a case report and review of the literature.

Al Kaissi A, Petje G, De Brauwer V, Grill F, Klaushofer K - Cases J (2009)

Anteroposterior radiograph of the tibia showed a thick and broad ballooning occupies the diaphyses (proximally and distally) there is gaining in the diameter comes from subperiosteal new bone apposition by intramembraneous bone formation.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2651856&req=5

Figure 5: Anteroposterior radiograph of the tibia showed a thick and broad ballooning occupies the diaphyses (proximally and distally) there is gaining in the diameter comes from subperiosteal new bone apposition by intramembraneous bone formation.
Mentions: Anteroposterior radiograph of the skull showed massive sclerosis of the skull bone associated with significant cortical hyperostosis and enlargement of the mandible secondary to cortical new bone formation (fig 1). Lateral skull radiograph showed sclerosis of the skull base (arrow) and hyperostosis of the calvaria (fig 2). Coronal MRI imaging showed significant calvarial/facial and mandibular hyperostosis (fig 3). Anteroposterior radiograph of the radius showed cortical new bone formation associated with subperiosteal thickening. Note marked bloating along the diaphysis with sparing of the epi-metaphyseal components associated with expansion of the bone marrow cavity and a persistent-like deformity (fig 4). Anteroposterior radiograph of the tibia showed a thick and broad ballooning occupies the diaphyses (proximally and distally) there is gaining in the diameter comes from subperiosteal new bone apposition by intramembraneous bone formation (fig 5).

Bottom Line: Infantile cortical hyperostosis is characterised by hyperirritability, acute inflammation of soft tissue, and profound alterations of the shape and structure of the underlying bones, particularly the long bones, mandible, clavicles, or ribs.Narrowing of skull foramina due to sclerosis of skull base is likely to result in cranial nerves deficits.In this baby, the pathology has been judged to be the result of child abuse and it is not, in this case considerable harm to his parents, and the doctor-parent relationship was the outcome.

View Article: PubMed Central - HTML - PubMed

Affiliation: Ludwig Boltzmann Institute of Osteology, at the Hanusch Hospital of WGKK and, AUVA, Trauma Centre Meidling, 4th Medical Department, Hanusch Hospital, Vienna, Austria. ali.alkaissi@osteologie.at.

ABSTRACT

Background: Infantile cortical hyperostosis is characterised by hyperirritability, acute inflammation of soft tissue, and profound alterations of the shape and structure of the underlying bones, particularly the long bones, mandible, clavicles, or ribs.

Case presentation: We report on a clinical case of a 3-months-old baby girl of non-consanguineous parents. Multiple long bone swellings were the motive of referral to our department for clinical evaluation. Radiographic documentation was consistent with infantile cortical hyperostosis (Caffey disease). Interestingly, skull base sclerosis associated with excessive thickening was the most unusual malformation. We report a baby with mixed endochondral and intramembraneous ossification defects.

Conclusion: Bone dysplasias, mucopolysaccharidoses, and metabolic diseases are a group of disorders that cause abnormal growth, density, and skull base shape. Skull base sclerosis/thickening is a well-known malformation in connection with other forms of sclerosing bone disorders such as dysosteosclerosis, frontometaphyseal dysplasia, and progressive diaphyseal dysplasia with skull base involvement. It is noteworthy that our present patient had an unusually sclerosed/thickened skull base. Narrowing of skull foramina due to sclerosis of skull base is likely to result in cranial nerves deficits. In this baby, the pathology has been judged to be the result of child abuse and it is not, in this case considerable harm to his parents, and the doctor-parent relationship was the outcome.

No MeSH data available.


Related in: MedlinePlus