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An unusual presentation of pulmonary alveolar microlithiasis and diaphyseal aclasia

D. A. Ritchie, MB, ChB, FRCR ^,* S. A. O'Connor, MB, BS, FRACP and D. McGivern, DM, MRCP

^* Department of Radiology, Hull Royal Infirmary, Anlaby Road, Hull HU3 2JZ, UK Department of Respiratory Medicine, Hull Royal Infirmary, Anlaby Road, Hull HU3 2JZ, UK Department of Cardiology, Royal Canberra Hospital, Acton, Canberra, ACT 2601, Australia

This excerpt was created in the absence of an abstract.

Pulmonary alveolar microlithiasis is a rare disease characterized by deposition of calcified bodies in the alveoli, which produce dense sand-like micronodules throughout the lungs. The radiological, pathological and clinical features have been previously well described (Sosman et al, 1957; Thurairajasingam et al, 1975; Prakash et al, 1983; Fraser et al, 1991).

Diaphyseal aclasia (Solomon, 1963; Murray et al, 1990) is one of the more common anomalies of skeletal development with an estimated prevalence of nine per million (Voutsinas & Wynne-Davis, 1983). Inherited as an autosomal dominant trait, most cases present during childhood development owing to deformity and compli cations; however, milder cases may only be detected later in life by chance.

Both pulmonary alveolar microlithiasis and diaphyseal aclasia demonstrate a familial tendency and have specific radiological features, but they are not known to be related. We present a case of pulmonary alveolar microlithiasis found incidentally while under investigation of skeletal deformity.

In 1967, a 5-year-old boy was referred to an orthopaedic clinic with right upper limb deformity. His developmental milestones were slightly retarded, but he was otherwise well.

A radiograph of his right humerus revealed an osteochondroma in the upper shaft and an abnormal adjacent lung field. A chest radiograph (Fig. 1) showed a diffuse dense interstitial process with a predominantly nodular pattern. There was blurring of the mediastinal borders and a faint negative pleural shadow. Further osteochondromas were demonstrated in other parts of the skeleton in keeping with diaphyseal aclasia.

Key Words: Pulmonary alveolar microlithiasis • Diaphyseal aclasia

Present address: Department of Radiology, Royal Liverpool University Hospital, Prescot St, Liverpool L7 8XP, UK.

Received for publication April 3, 1991. Revision received July 8, 1991. Accepted for publication September 20, 1991.

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