Infantile Cortical Hyperostosis - Signs and Symptoms

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Dr. Reeja Tharu
Medically Reviewed by The Medindia Medical Review Team  on Apr 19, 2013
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Signs and Symptoms of Infantile Cortical Hyperostosis

The most striking feature of Infantile Cortical Hyperostosis is the presence of soft tissue swellings that are deeply placed in relation to the bone involved. Additionally, cortical thickening of the affected bone is also present. These swellings usually start to appear within 90 days of birth although in some cases the condition may present itself as late as two years after birth. The mandible and the clavicle or the collarbones are the ones more frequently affected. The presence of facial swelling is considered pathognomic of the disease but these are not those well-defined in the inherited or familial form of the disease. The ribs, tibia, scapula and the calvarium are the bones affected in the familial form of the disease. The soft tissue swellings are invariably associated with deep muscles and occur most frequently on the neck, face, chest and the lower limbs. Other features associated with Infantile Cortical Hyperostosis are as follows:

  • Fever
  • Hyperirritability
  • Pleurisy
  • Dysphagia
  • Pseudoparalysis

When the mandible is involved there are thick swellings at the angle of the bone in what is called the ramus area. This gives a chubby appearance to the infant and is often mistaken for cherubism. It leads to malocclusion, where the teeth are not properly aligned in relation to each other, resulting in a grotesque appearance in early childhood.

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my baby sarah fought brilliantly against all odds but she could not tolerate the hospitalized infection(septicemia)and died on 8th nov 09.she was admitted for caffeys symptoms but got infected,drs says this that,but i would say one thing before admission drs should know the patient or else why they have admitted my kid,simply to study the case or they dont want to loose study material.end of the day i have lost my darling.
Sayed Aslam
Hubli
india

Its been 4 months ever since my daughter confirmed with Caffeys,she is on naproxen but no signs of improvement her body weight remains the same ever since her birth,her platelet count is more than 9.50 due to which drs are not giving even steroids which may further worsen her condition.she is been through severe pain and fever due to which she hardly sleeps.

MY BABY GIRL IS CONFIRMED WITH CAFFEYS DISEASE,SHOCKING IS NO FAMILY HISTORY DRS SAYS ITS A GENETIC DISEASE AND BABY IS 3 MONTH OLD IS THERE ANY TREATMENT..........

SAYED ASLAM
HUBLI.
INDIA

MY BABY BOY IS HAVING CAFFEY DISEASE, NO FAMILY HISTORY OF CAFFERY, HIS BLOOD TEST SHOWS HIGH HEMOGLOBIN, HIGH PLATELES AND GOANT TROMBOCIATES CELL, HE IS NOW 15 MONTHS, RIGHT RIB , MANDIBLE AND MAXILLE SWELLING, THE SWELLINGIS ALMOST NEARLY ONE YEAR. WHAT TREATMENT DID U GIVE YOUR CHILD

Annie0909

My mother had Caffey disease, I did when I was a child, and now my baby boy also has it. I am very interested to share experiences with other patients...

please let me know the treatment and the time period you all have taken to recover,as my 4 month old baby girl is also suffering from caffeys and her symptoms are very severe.please help me.....

guest

Similar experience-your son is over a year now,how is he getting on?

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