The first clinicoradiological description of infantile cortical hyperostosis (ICH) was made in 1945 by Caffey and Silverman,1 with the first ophthalmologic description given by Illif and Ossovsky in 1962.2 The characteristic radiologic features of Caffe/s disease are soft tissue sweUing, periosteal new bone formation, and cortical hyperostosis. We present a 40-day-old infant who developed this syndrome and was diagnosed and treated in our department.
A 40-day-old girl was hospitalized in the pediatric department with suspected osteomyelitis of the left humerus (Fig 1). On admission, the child was restless, with a painful sweUing of the left humerus. Her body temperature was 38°C and laboratory tests showed a hemoglobin of 10.4 g/100 mL; white blood ceU count of 15,000; and a thrombocytosis of 584X 103/g.
Within a few days, a tender right-sided periorbital sweUing and a hyperemic sweUing of the right mandible developed. The presumptive diagnosis of periorbital cellulitis was made but, despite antibiotic therapy, a similar display occurred on the left side of the face (Fig 2). Computed tomography (CT) of the orbits, mandible, and humerus showed a thick, periosteal reaction and cortical sclerosis of the right mandible and lateral wall of the right orbit (Fig 3). These findings and the thrombocytosis confirmed the diagnosis of Caffey*s disease.3"4 Antibiotic treatment was ceased, and symptomatic treatment continued. The patient's body temperature returned to normal, and the periorbital sweUing graduaUy resolved over the foUowing weeks without further treatment. One month later, the patient was «gain hospitalized in the pediatric department, with hyperostosis of the right clavicle.
Fig 1: CT of left humerus showing thick periosteal reaction and \ swelling of the soft tissue.
Caffey-SUverman syndrome results from an ????^?t??tory process thought to be caused by obliteration of the small arteries in the region of the bone lesions. The obUteration occurs as a result of proliferation of the intima of arterioles, which results in hypoxia, hyperostosis, and swelling above the afflicted bones.5 As a rule, the disease appears during the first year of Ufe, more frequently within the first 6 months. The syndrome tends to recur (usually until age 3). The children become symptomatic for no obvious reason. Periorbital swelling or proptosis caused by mandibular or orbital bone lesions develops in about one third of the patients, and is often diagnosed clinicaUy as either periorbital or orbital celluUtis, angioneurotic edema, or the beginning of a maUgnancy.6 Mandibular involvement is one of the most striking features of the disease.2·6
Fig 2; Bilateral swelling of periorbital tissue and right mandíbula. Macroscopic full facial view.
Although the cause of the disease remains unknown,6 some reports in the Uterature lean toward the hereditary (autosomal/dominant) nature of the disease.
Some researchers have attempted to separate the disease into two types: hereditary and sporadic. The existing point of view is that, in familial cases, the tibiae, humerus, and ulnae are most often affected, whereas in the sporadic form the mandible, clavicles, and ribs are most often affected.3
This is not supported by our case. The association between prostaglandin E administration and CaffeySilverman syndrome has also been investigated.5 The direct cause of the thrombocytosis remains unknown, although some investigators claim an infection is the cause.
The Caffey-Silverman syndrome is known to be a selflimiting condition with spontaneous resolution within several weeks or months of the swelling of soft tissues and absorption of the hyperostosic bones involved. Correct and timely diagnosis alleviates unnecessary treatment and invasive manipulations.
Fig 3: CT of the head. Top: Hyperostosis of the lateral wall of the orbit. Bottom: Thick periosteal reaction and cortical sclerosis of the right mandíbula.
1. Caffey J, Silverman, WA. Infantile cortical hyperostosis. American Journal of Roentgenology. 1945;54:1-16.
2. Uliff CE. Infantile cortical hyperostosis. Am J Ophthalmol. 1962;53:976-981.
3. Lamont AC, Young DD, Shannon CS. Diagnosis of unexpected injuries in children. British Medical Journal. 1991;382:1083.
4. Tabardel Y, Sghaye MC, Senterre J. Neonatal CalleySilverman disease with thrombocytosis, increase in C-reactive proteins and immunoglobulins. Arch Pediatr. 1988;45:263265.
5. Jakubova J, Macek H, Halasova E. Swelling over the face: infantile cortical hyperostosis. Int J Pediatr Otorhinolaryngol. 1994;29:147-150.
6. Jacobec J, Jones, SI. Mesenchymal and fibro-osseous tumors. In: Duane S, ed. Clinical Ophthalmology. Philadelphia, Pa: Harper & Row; 1990.