Caffey's Disease Sans Mandibular and Clavicular Involvement: A Rare Case Report

Abstract

Caffey’s disease, also known as Infantile Cortical Hyperostosis, is a rare, self-limited, benign, inflammatory gene-related disorder of infants that causes bone changes, soft tissue swelling, and irritability. The mandible (75%), clavicles, and ulnae are the bones most frequently involved, others being long bones, lateral ribs, ilia with skull being the rarest. However, we report a case of a 5-month-old male diagnosed with Infantile cortical hyperostosis but with absent mandibular and clavicular involvement, thus depicting the unusual presentation of this disease.

Caffey-Silverman syndrome – a case report of a two-monthold boy with a positive family history

“…The described ailments and affected bones seen on X-ray proved to be consistent with the literature [4,[6][7][8][9]. This disease can be insidious, in 2017 Sachin Khanduri et al presented for the first time the case of a boy in whom the mandible and collarbone were not affected, but the child was diagnosed with ICH [16].…”

“…After administering Ibuprofen in the form of a syrup, we observed an improvement in his clinical condition. Narayanan Kutty et al [17] and Sachin Khanduri et al [16] also observed a good therapeutic effect of ibuprofen. However, the literature describes the case of one of the dizygotic twins, a two-month-old boy, suffering from ICH, whose treatment with ibuprofen was ineffective and only administration of glucocorticosteroids (prednisolone 1 mg/kg/24 h) brought about positive results [22].…”

Craniofacial disorders and dysplasias: Molecular, clinical, and management perspectives

“…The symptoms usually resolve spontaneously within a few months and bone growth returns to normal. However, the jaw bones especially the mandible as well as the clavicles and scapulae, can also be affected ( Khanduri et al, 2017 ). In some cases, Caffe's disease occurs due to somatic mosaicism, where the mutation is located in a subset of germline cells resulting in sporadic cases of the disease.…”