Caffey disease or Infantile cortical hyperostosis, is a rare disorder of unknown etiology, characterized by cortical hyperostosis with inflammation of contiguous fascia and muscle [1]. It is self-limiting and usually affects young infants. The diagnosis may be delayed as it mimics a wide range of conditions like osteomyelitis, hypervitaminosis A, bone tumor and child abuse. We present an infant with pseudoparalysis, who was initially considered to have osteomyelitis of scapula, but was later diagnosed to have Caffey disease.

Fig. 1 MRI showing altered signal intensity in the body of left scapula with periosteal reaction.

A 75-day-old boy with uneventful antenatal and neonatal period, presented to us with complaints of paucity of movement of left upper limb for one week. There was no history of fever, intramuscular injection or trauma, lethargy or seizures. He was not on any vitamin supplementation. On examination, he was irritable. Neurological examination revealed paucity of movement of left upper limb. Deep tendon reflexes were preserved. Detailed general examination showed mild soft tissue swelling over the body of left scapula. Left shoulder movement was painful. There was no other bony swelling or tenderness. After consulting Orthopedician, chest X-ray and magnetic resonance imaging (MRI) of left shoulder with upper limb was done. MRI (Fig. 1) showed altered signal intensity in the body of left scapula with periosteal reaction and adjacent soft tissue edema, features favoring osteomyelitis. Chest X-ray (Fig. 2) showed slightly thickened and sclerotic left scapula suggestive of Caffey disease. Laboratory tests showed leukocytosis, anemia (Hb 8.4 g/dL), thrombocytosis, positive C-reactive protein (25.1 mg/L), and high ESR (20 mm/hr). Blood culture was sterile. Infant was treated with Ibuprofen. Left upper limb movement improved and infant is doing well on follow-up.

Fig.2 X-ray showing showing thickened and sclerotic left scapula.

Though MRI was reported as osteomyelitis of scapula in our infant, the absence of underlying risk factors for bone infection made us suspect an alternative diagnosis. The presence of irritability, soft tissue swelling and hyperostosis on X-ray helped us clinch the diagnosis. Thrombocytosis has also been reported in caffey disease [2]. Though Mandible is the most commonly involved bone, scapular involvement presenting as Erb’s palsy has been earlier reported [3]. A high index of clinical suspicion is required to diagnose Caffey disease and avoid unnecessary investigations and intervention.