Infantile cortical hyperostosis

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Classification according to ICD-10
M89.8 Other specified bone diseases

Including: Infantile cortical hyperostosis Post-traumatic subperiosteal ossification

ICD-10 online (WHO version 2019)

The Infantile cortical hyperostosis , even after Erstbeschrieb by the American pediatrician and founder of the Pediatric Radiology Dr. John Caffey named Caffey disease in 1946 , is a rare self-limiting disease in early childhood characterized by soft tissue swelling, cortical thickening and circumscribed hypersensitivity of the skin.

There are both familial and sporadic forms. An autosomal recessive type I can be distinguished from an autosomal dominant type II.

The frequency is given as 3 in 1000 children under 6 months.

Pathologically, there is a change in the periosteum , often affecting the upper or lower jaw or the ulna , less often the tibia or collarbone .

clinic

The disease can be present before birth or occurs in the first months of life with a sudden onset and rough thickening and hypersensitivity of the skin without reddening. It is an inflammatory process of unknown origin. The changes are mostly asymmetrical and can occur in several places.

diagnosis

The x-ray of the skull shows massive sclerosis of the bone with significant cortical hyperostosis and enlargement of the mandible due to secondary cortical new bone formation.

The clinical finding on physical examination is considered characteristic.

The x-ray shows a massive cortical thickening in the affected region due to periosteal apposition.

Differential diagnosis

In the differential diagnosis, osteomyelitis , hypervitaminosis A, Ewing's sarcoma , metastasis from neuroblastoma , hyperphosphataemia , trauma or child abuse are to be distinguished .

therapy

The disease is considered self-limiting; the changes cease within 6 to 12 months. However, recurrences have been reported.

Corticosteroids can be used to treat severe cases .

literature

  • JA Herring (Ed.): Infantile cortical hyperostosis. In: Tachdjian's Pediatric Orthopedics. 3. Edition. Saunders, Philadelphia 2002, ISBN 0-7216-5682-X , pp. 1561-1565.
  • H. Nistala, O. Mäkitie, H. Jüppner: Caffey disease: new perspectives on old questions. In: Bone. 2014, No. 60, pp. 246-251. doi: 10.1016 / j.bone.2013.12.030 , Epub 2013 Dec 31.
  • A. de Blas, M. Martín-Frías, M. Toledano, M. Gomez, J. Corbatón, R. Yturriaga: Hiperostosis cortical neonatal (enfermedad de Caffey) Neonatal cortical hyperostosis (Caffey disease). In: Anales de pediatría (Barcelona). Volume 79, No. 3, September 2013, pp. 189-190, ISSN  1695-9531 . doi: 10.1016 / j.anpedi.2012.08.007 . PMID 23085318 .

Individual evidence

  1. ^ J. Caffey: Infantile cortical hyperostoses. In: Journal of Pediatrics. No. 29, 1946, pp. 541-559, doi : 10.1016 / S0022-3476 (46) 80122-7
  2. whonamedit
  3. F. Hefti: Pediatric Orthopedics in Practice . Springer 1998, ISBN 3-540-61480-X , p. 674.
  4. A. Kamoun-Goldrat, J. Martinovic, J. Saada et al: Prenatal cortical hyperostosis with COL1A1 gene mutation. In: American Journal of Medical Genetics. A 146A, 2008, pp. 1820-1824, doi: 10.1002 / ajmg.a.32351 , PMID 18553566 .
  5. P. Navarre, I. Pehlivanov, B. Morin: Recurrence of infantile cortical hyperostosis: a case report and review of the literature. In: Journal of Pediatric Orthopedics. 2013, No. 33, pp. E10 – e17. doi: 10.1097 / BPO.0b013e318277d3a2

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