Infantile cortical hyperostosis presenting with exophthalmos. [canadianjournalofophthalmology.ca]

The disease may be present at birth or occur shortly thereafter. The cause is unknown. Both familial and sporadic forms occur. It is also known as Caffey disease or Caffey's disease. [en.wikipedia.org]

Two cases are presented of congenital Caffey's disease in premature babies. The first baby was hydropic at birth and had cortical hyperostosis involving the mandible and long bones of right upper limb and both lower limbs. [thieme-connect.com]

Epidemiology: Clinical Findings: The most common presentation is that of an irritable child. Fever, tenderness, and soft tissue swelling overlying the involved bone are occasionally present. [posna.org]

Patients present with major angulation of the long bones, generalized symmetrical involvement of the skeleton, and polyhydramnios. [emedicine.medscape.com]

• Fever

  Farlex Partner Medical Dictionary © Farlex 2012 Caffey disease An autosomal dominant condition MIM 114000 characterised by fever, inflammation, swelling of affected bones (tibia, mandible, ulna and facial bones) and restlessness. [medical-dictionary.thefreedictionary.com]

  One day after interruption of the drug the fever disappeared. [scielo.br]

  The child was not irritable and no fever was observed at this time. Article History Accepted: Apr 1949 Published in print: May 1950 [pubs.rsna.org]

  Hyperostose Maladies osseuses Dysplasies osseuses Ostéochondrodysplasies Hyperostose corticale infantile Visualisez ce concept sur le site Concept TE.183821 définition A disease of young infants characterized by soft tissue swellings over the affected bones, fever [termsciences.fr]

• Angioedema

  Many cases are misdiagnosed and treated as orbital cellulitis, angioedema, or even dacryocystitis., , 10 Bywaters Jr, T.W. Bailey R.W. Carroll C.J. O’Connor G.A. Infantile cortical hyperostosis presenting with exophthalmos. [canadianjournalofophthalmology.ca]

  Many cases are misdiagnosed and treated as orbital cellulitis, angioedema, or even dacryocystitis. [17] Imaging Modalities Radiography Radiography is the most valuable diagnostic study in infantile cortical hyperostosis. [emedicine.medscape.com]

• Unilateral Proptosis

  Ophthalmic manifestations of ICH include periorbital swelling and edema, unilateral proptosis, chemosis, and conjunctival hyperemia. [canadianjournalofophthalmology.ca]

  Infantile cortical hyperostosis manifesting as congenital unilateral proptosis. [pubfacts.com]

  These need to be excluded when diagnosing infantile cortical hyperostosis. [16] Ophthalmic manifestations include periorbital swelling and edema, unilateral proptosis, chemosis, and conjunctival hyperemia. [emedicine.medscape.com]

• Chemosis

  Ophthalmic manifestations of ICH include periorbital swelling and edema, unilateral proptosis, chemosis, and conjunctival hyperemia. [canadianjournalofophthalmology.ca]

  These need to be excluded when diagnosing infantile cortical hyperostosis. [16] Ophthalmic manifestations include periorbital swelling and edema, unilateral proptosis, chemosis, and conjunctival hyperemia. [emedicine.medscape.com]

• Conjunctival Hyperemia

  Ophthalmic manifestations of ICH include periorbital swelling and edema, unilateral proptosis, chemosis, and conjunctival hyperemia. [canadianjournalofophthalmology.ca]

  These need to be excluded when diagnosing infantile cortical hyperostosis. [16] Ophthalmic manifestations include periorbital swelling and edema, unilateral proptosis, chemosis, and conjunctival hyperemia. [emedicine.medscape.com]

• Macrocephaly

  Ectopia Lentis Kozlowski Tsuruta Taki Syndrome Langer Mesomelic Dysplasia Laplane Fontaine Lagardere Syndrome Larsen Syndromes + Leri-Weill dyschondrosteosis Lethal Chondrodysplasia with Long Bone Angulation and Mixed Bone Density Lowry Wood Syndrome Macrocephaly [rgd.mcw.edu]

  Labrets Nasal and earrings Hair and nails Circumcision Tattoos and scarification Purpose(s) Part Four: Congenital anomalies Congenital skeletal anomolies Skull malformations Craniosynostoses Complex craniofacial deformities Anencephaly Microcephaly Macrocephaly [corp.credoreference.com]

Knowing which lesions to leave alone or which ones require workup can prevent misdiagnosis and reduce patient anxiety. [ijri.org]

Being aware of this rare disease allows for early suspicion and a better workup and contributes to orthopedic knowledge as its musculoskeletal alterations are reported. [pesquisa.bvsalud.org]

• Staphylococcus Aureus

  The patient was originally treated with antibiotics for a neonatal Staphylococcus aureus infection complicated with endocarditis and conjunctivitis. [jpeds.com]

Treatment and prognosis Generally, it is a reversible, benign, self-limiting process and often disappears after discontinuation of the prostaglandins but can persist for months 1-3. [radiopaedia.org]

[citation needed] Prognosis[edit] Infantile cortical hyperostosis is a self-limited condition, meaning that the disease resolves on its own without treatment, usually within 6–9 months. [en.wikipedia.org]

This treatment helps to provide adequate time for the infant to mature in preparation of surgical intervention. However, cortical hyperostosis can occur as a complication of long-term treatment (4-6 wk). [emedicine.medscape.com]

Treatment: Most cases do not require active treatment. Fever and pain may be managed expectantly. In a reported case, naproxen given as a prostaglandin inhibitor was successful in treating one case of recurrent infantile cortical hyperostosis. [posna.org]

A newborn girl was affected by a “ductal-dependent” complex congenital heart disease, which necessitated a long period of prostaglandin (prostaglandin E1) infusion; during this treatment, she developed cortical hyperostosis of the long bones. [jpeds.com]

Surgery, King Fahad National Guard Hospital, Riyadh, Saudi Arabia Further Information Publication History Publication Date: 04 March 2008 (online) ABSTRACT Prognosis of congenital infantile cortical hyperostosis (Caffey's disease) is poor particularly [thieme-connect.com]

Abstract Prognosis of congenital infantile cortical hyperostosis (Caffey's disease) is poor particularly in premature babies. Two cases are presented of congenital Caffey's disease in premature babies. [ncbi.nlm.nih.gov]

Four to six months after discharge from hospital, the symptoms disappeared, confirming the good prognosis of this condition. [pubmed.ncbi.nlm.nih.gov]

[citation needed] Prognosis[edit] Infantile cortical hyperostosis is a self-limited condition, meaning that the disease resolves on its own without treatment, usually within 6–9 months. [en.wikipedia.org]

Treatment and prognosis Generally, it is a reversible, benign, self-limiting process and often disappears after discontinuation of the prostaglandins but can persist for months 1-3. [radiopaedia.org]

A variety of malignant neoplastic, benign neoplastic, cystic, and vascular etiologies have been associated with proptosis at birth. [canadianjournalofophthalmology.ca]

(Dorland, 27th ed) Year introduced: 1991 PubMed search builder options Subheadings: analysis anatomy and histology blood chemically induced classification complications diagnosis diagnostic imaging drug therapy enzymology epidemiology etiology genetics [ncbi.nlm.nih.gov]

Etiology: A mutation in the COL1A1 gene has been identified in patients with Caffey disease. This mutation may allow the periosteum to separate from the bone in infancy, leading to the typical clinical findings. [posna.org]

Qualifiers English: BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology [decs.bvs.br]

See: Alkaline Phosphatase - Discussion: - disease of unknown etiology affecting skeleton & adjacent tissues; - characterized by fever, rirritability, swelling of soft tissues, & cortical bone thickening; - thickening of jaw & forearm are the most common [wheelessonline.com]

(Dorland, 27th ed) Year introduced: 1991 PubMed search builder options Subheadings: analysis anatomy and histology blood chemically induced classification complications diagnosis diagnostic imaging drug therapy enzymology epidemiology etiology genetics [ncbi.nlm.nih.gov]

[…] membrane-associated guanylate kinase… Plasticity of the developing visual system has been regarded as the best model for changes of neuronal connections under the… To investigate the relation of ultraviolet radiation and cataract formation, we undertook an epidemiologic [semanticscholar.org]

CONGENITAL 1964-90 Allowable Qualifiers English: BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology [decs.bvs.br]

[citation needed] Epidemiology[edit] The disease has been reported to affect 3 per 1000 infants younger than 6 months in the United States. No predilection by race or sex has been established. Almost all cases occur by the age of 5 months. [en.wikipedia.org]

[citation needed] Genetics[edit] It has been associated with COL1A1.[1] Pathophysiology[edit] In the early stages of infantile cortical hyperostosis, biopsy shows inflammation of the periosteum and adjacent soft tissues. [en.wikipedia.org]

[…] diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PS parasitology PA pathology PP physiopathology PC prevention [decs.bvs.br]

Centers for Disease Control and Prevention Intersex (Medical Encyclopedia) [ Read More ] [icdlist.com]

The bilateral, almost symmetrical changes are typical of this preventable infection. Note the classical Wimberger's sign of destructive metaphysitis involving the medial aspects of the distal femora and proximal tibae. [isradiology.org]

All efforts must be made to attempt interruption of the drug as soon as possible, aiming at preventing the potential complications. REFERENCES 1. Hoch M, Netz H. Heart failure in pediatric patients. Thorac Cardiovasc Surg. 2005;53:S129-S134. 2. [scielo.br]