A 4 months old male child was brought to the hospital with a history of fever, irritability and excessive crying. Mild swelling and tenderness was noted over the lower ends of both forearms and both sides of root of the neck. Investigations were done. X ray features were diagnostic.
A 4 months old male child was brought to the paediatric department by his parents with a history of fever, irritability and excessive crying for two weeks. There was no history of trauma. There were no further relevant details in the past medical history. On clinical examination, there was a palpable swelling on lower end of both forearms which were tender. Palpable hardness with tenderness also elicited over clavicular regions. However, overlying skin appeared relatively normal. No palpable regional lymphadenopathy detected. Serologic investigations for viral and bacterial infections were negative. Sedimentation rate and alkaline phosphatase were high. The clinical impression was of an inflammatory condition due to systemic cause. The patient was subjected to X ray of chest (Fig ) including both hands which revealed cortical hyperostosis involving diametaphyseal regions of both radiuses (Fig 2,3) and diaphyses of both clavicles (Fig 4,5). Careful examination of chest X ray revealed similar changes of lower ribs (Fig 6) and mandibles (Fig 7,8) though to a lower extent. The X ray findings were suggestive of infantile cortical hyperostosis or Caffey’s disease. Palliative treatment was given and the symptoms subsided after 6 weeks.
Infantile cortical hyperostosis or Caffey’s disease is a rare disorder affecting the skeleton and some of its contiguous fasciae and muscles, mainly involving children below 1 year of age group. The cause is unknown and pathogenesis is obscure. Caffey and Silverman first reported this disease as a distinct entity in 1945. Cases are seen in all manner of circumstances – in cities and rural communities, in poverty and luxury, in all climates and in all races with no sex preference. There is a striking age limitation with majority cases appear before 5th-7th month of life. Average age at onset is 9 weeks. The disease is self limiting and symptoms lasting from 2-3 weeks to 2-3 months.
There are 3 main manifestations common to all patients: hyperirritability, swelling of the soft tissues and cortical thickenings of the underlying bones. The soft tissue swelling appears suddenly at the onset and present as painful wooden hardness during the active phase of the disease. They are almost always deeply situated and do not extend up to the subcutaneous tissue or are not overly warm or discoloured. Swelling subsided long before hyperostosis resolves.
The radiographic features are the sine qua non for diagnosis. Hyperostosis develops in contact with external cortical surface, expands and then remodel by resorption externally or expansion from internal aspects of bone. Cortical hyperostosis has been demonstrated in all the tubular bones of the skeleton except the phalanges. Vertebral bodies are spared. Mandible, clavicles and ulna are most commonly affected bones.
Complications depend upon the site of involvement. Pseudoparalysis, pleural effusion and dysphagia are reported. Most common laboratory findings are raised erythrocyte sedimentation rate and alkaline phosphatase. Thrombocytosis is more common than thrombocytopenia. Anemia is common. Differential diagnosis includes injury, vitamin A toxicity, syphilis, scurvy, osteomyelitis, parotitis and malignancy. The majority of these conditions can be ruled out by age group, demonstrating mandibular involvement, and observing triad of irritability, swelling and osseous lesions etc.
As it is a self limiting condition, palliative treatment is the one which is generally given. Long lasting morbidity or mortality is rare in this condition.
Infantile Cortical Hyperostosis or Caffey's Disease.
Based on the provided X-ray images, the following key features are observed:
Regarding soft tissues, there is local swelling but no obvious subcutaneous air, exudate, or abscess formation. Overall, there are no signs of bone marrow cavity invasion or bone destruction. The radiological findings correlate with the regions of swelling observed in both forearms and the base of the neck in the patient.
Considering the patient's clinical presentation (fever, irritability, local swelling, and pain) along with the radiological features (bone cortical thickening, soft tissue swelling), the following differential diagnoses should be considered:
After comprehensive analysis: The patient, approximately 4 months old, falls within the typical age range for Caffey Disease (Infantile Cortical Hyperostosis). Clinical symptoms include fever, irritability, swelling of the forearms and base of the neck, and no significant purulent signs. Radiological findings demonstrate symmetrical cortical thickening and new bone formation in both the radius and ulna, as well as in the clavicle, consistent with hallmark presentations of Caffey Disease. Given the disease’s self-limiting nature and corresponding laboratory findings (elevated serum alkaline phosphatase, elevated erythrocyte sedimentation rate, etc.), the most likely final diagnosis is:
Infantile Cortical Hyperostosis (Caffey Disease).
As Caffey Disease is self-limiting, most pediatric patients recover within weeks to months. Treatment is primarily conservative and symptom-relieving, with vigilant monitoring of disease progression. For pain and fever, recommended approaches include:
In terms of rehabilitation, although this condition typically has minimal long-term impact on bone morphology, it is important to reduce strain on affected areas during the acute phase to avoid aggravating pain or periosteal irritation.
Exercise or rehabilitation plans should follow a gradual, stepwise approach, taking the child’s response and developmental level into full consideration. If the child experiences marked pain, pronounced irritability, or a resurgence of fever, a medical evaluation is advised, and related exercises should be suspended if necessary.
Disclaimer:
This report, based on the provided clinical and imaging information, is intended for reference only and does not replace an in-person consultation or professional medical advice. If you have any questions or notice any change in condition, please seek guidance from a qualified healthcare professional or specialist promptly.
Infantile Cortical Hyperostosis or Caffey's Disease.
