BRIEF REPORTS

Madelung Deformity—the deformity with mesomelic dwarfism of Hallmark of Dyschondrosteosis both upper and lower limbs. It is. the commonest variety of mesomelic dwarf- ism(3). Madelung deformity may also be M. Bhat seen associated with diverse disorders such as mucopolysaccharidosis, Turner's R. Chetan syndrome, achondroplasia, dysch- K.A. Shivashankar ondroplasia (Ollier's disease), multiple M. Jayaram exostosis and multiple epiphyseal dys- plasia. Madelung's original description of the lesion befits its restriction to Dyschondrosteosis (2). Case Report Madelung deformity is an abnormal- ity of the distal radial epiphysis wherein An 11-year-old Muslim girl, second a progressive ulnar and volar tilt of its of five siblings of non-consanguienous articular surface occurs in association birth to normal parents presented with with a dorsal subluxation of the distal short stature and progressive bony de- ulna. It is a rare inherited disorder formities. She was 98 cm tall with US: LS accounting for 1.7% of all congenital ratio of 0.9. She had mesomelic shorten- hand anomalies. The inheritance pattern ing of all limbs with a short neck, pectus is autosomal dominant with variable carinatum, kyphoscoliosis and lumbar penetrance (l). lordosis (Fig. 1). The limbs showed wid- ening of wrist, elbow and ankle joints, Madelung-like deformity has been bilateral cubitus valgus, coxa valga and reported following trauma to the distal genu varum. There was limitation in radial epiphysis as also infections and movement at hip and knee joints and neoplasia. However, the term ‘Madelung gait was of waddling type. X-ray of deformity’ should preferably be hand and wrist were diagnostic of confined to the congenital disorder Madelung deformity (Fig. 2). which is also known as Dys- chondrosteosis.(2). Dyschondrosteosis is Discussion an inherited dysplasia of the bone Dyschondrosteosis (Syn. Made- characterized by bilateral Madelung lung's disease(3), Leri-Weill's syndrome) is the commonest type of mesomelic From the Departments of Pediatrics Sri Devaraj dwarfism and is associated with bilateral Urs Medical College, Tamaka, Kolar 563 Madelung deformity. It is often seen in 101, Karnataka girls and becomes clinically apparent by Reprint requests: Dr. Ravi Chetan, Associate late childhood or early adolescene(4). Professor of Pediatrics, 613, 11th cross 2nd Madelung deformity was first described Main, J.P. Nagar-Phase III, Bangalore 560 078 by Malgaigne in 1885 and later by Madelung in 1878 as "Spontaneous for- Received for publication: August 1,1994; ward subluxation of the hand."(2). Accepted: November 28,1994

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Danneberg et al.(5) reviewed 172 cases distal radius is altered to face in ulnar and outlined 12 radiological criteria for and palmar direction; (e) Premature fu- the diagnosis of Madelung deformity: sion of the ulnar half of the lower radial (a) Lateral and dorsal bowing of the epiphysis, accentuating the deformity in radial diaphysis; (b) Widening of the early adolescence; (f) Dislocation/ interosseous space due to (a) above; subluxation of the inferior radio-ulnar (c) Shortening of the radius in compari- joint shifting the distal ulna dorsally; son to other bones and to normal stan- (g) Increased bone density proximal to dards for age; (d) Articular surfaces of the abnormal radio-ulnar fusion line; (h) Exostosis along the inferior ulnar border of the radius: (i) Triangularization of the normally quadrangular shaped outline of the distal radial epiphysis with the apex pointing medially; (j) Hypercon- densation of trabeculations of the ulnar head; (k) Wedging of the carpal bones between the abnormal distal radioulnar joint giving them a triangular configura- tion with the lunate at its apex; and (l) An arched curvature of the carpal bones in continuation with the arch of the dor- sally bowed radial diaphysis. Criterias (c), (i) and (k) are essential for diagnosis whereas (g), (h) and (j) are secondary phenomena(5). The present case had all criteria except (h) and (j). Management is usually conservative. Persistent pain and/or severe deformity call for orthopedic surgery involving ra- dial osteotomy. In addition, ulnar short- ening in skeletally immature patients or excision of distal ulnar head in the skele- tally mature is done. Surgical prophy- laxis by resection of the abnormal part of distal radial epiphysis and its replace- ment by autologous fat (also known as physiolysis) has recently been shown to restore growth and minimize deformi- ty(6). Acknowlegement We thank the Distric Surgeon, S.N.R. Hospital, Kolar for allowing us to pub- lish this case report.

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REFERENCES sias. In: Nelson Text Book of Pediat- rics, 14th edn. Eds. Behrman RE. 1. Jobe MT, Wright PE. Congenital Kliegman RM. Philadelphia, WB anomalies of hand. In: Compbell's Op- Saunders Co, 1992, pp 738. erative Orthopedics, 8th Edn. Ed. Crenshaw AH. New York, Mosby Year 5. Dannenberg M, Anton JI, Spiegel MB. Book, 1992, pp 3419-3421. Madelung's deformity: Consideration 2. Lamb D. Madelung deformity. J Hand of its roentgenological diagnostic crite- ria. Am J Roentgenol Rad Therapy Surg 1988,13-1:3-4. 1939,42: 671-676. 3. Langer LO. Dyschondrosteosis, a heri- table bone dysplasia with characteris- 6. Vickers D, Neilsen G. Madelung de- tic roentgenographic features. Am J formity: Surgical prophylaxis Roentgenol Radio Therapy 1965, 95: (physiolysis) during late growth peri- 178-188. od by resection of dyschondrosteosis lesion. J Hand Surg (British) 1992,17B: 4. Sillence DO. Genetic skeletal dyspla- 401-407.

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