Images in Clinical Practice

Indian Pediatrics 2000;37: 1021-1022

Diaphyseal Aclasia (Multiple Exostoses)

A 9-year-old Hindu female child of non-consanguineous marriage persented with multiple bony projections above and below the knees, around the ankles, elbows, wrists and proximal phalanx of right middle finger. There were swelling on the ribs and at the inner margin of scapulae. The bony swellings were noted since 2 years of age. One of her four brothers has similar swelling of the legs, ribs and scapulae. Their father also has bony projections on legs, hands and on acromian process of right scapula (Fig. 1).

Skeletal survey showed multiple osteo-cartilagenous tumors at the metaphysis of the distal femur, proximal and distal tibia and fibula; (Fig. 2) distal radii and ulna, proximal phalanx and of right middle finger with widening of metaphysis. These changes are consistent with sessile and pedunculated multiple diaphyseal aclasia.

Multiple exostoses is an autosomal domi-nant abormality commoner in males. Cartilage capped osteomas occur at the metaphysis, particularly at the ends of the long bones. These osteochondromes grow slowly with the child, cease to grow at maturity and may cause local problems by interfering with adjacent structures. A policy of selective excision should be adopted. Malignant changes may occur in adult life, particularly with exostoses affecting flat bones. Any growth in an exostoses after skeletal maturity should be considered suspicious of malignancy.

Sutapa Ganguly,
Debasis Adhya,
Department of Pediatrics,
IPGMER and SSKM Hospital,
Calcutta 700 020, India.

Fig. 2. X-ray of the girl showing multiple osteo-cartilagenous tumors at the metaphysis of distal femur and promixal and distal tibia and fibula.