Madelung Deformity Workup

  • Author: Paul M Lamberti, MD; Chief Editor: Dennis P Grogan, MD   more...
 
Updated: Aug 10, 2010
 

Imaging Studies

Posteroanterior (PA) and lateral plain radiographs of the forearm and wrist

The diagnosis is confirmed radiographically with PA and lateral views of the forearm and wrist. Carter and Ezaki have standardized the way they obtain the PA forearm radiographs in patients with MD.[19] Both right and left forearm images are placed on the same radiographic plate for comparison. The true PA radiograph is obtained with the forearm in neutral rotation, the elbow at 90° of flexion, and the shoulder abducted to 90°. They have found this method of analysis useful in preoperative planning, and it is highly reproducible by radiology staff. A lateral radiograph is obtained by rotating the shoulder to the side of the patient and laying the ulnar border of the forearm on the plate.

Several distinct radiographic features of MD exist. Dannenberg et al described elements of radiographic diagnosis after their review of 172 cases.[20] Criteria include the following:

  • Lateral and dorsal curvature of the radius
  • Widened interosseous space
  • True shortening of the total length of the radius
  • Premature fusion of the ulnar half of the distal radial physis
  • Focal osteopenia in the area of the ulnar portion of the distal radius
  • Exostosis at the distal ulnar border of the radius
  • Triangularization of the distal radial epiphysis
  • Ulnar and palmar facing distal radial articular surface
  • Relative dorsal subluxation of the ulna
  • Increased radiodensity of the ulnar head
  • Carpal wedging with the lunate at the apex of the wedge
  • An arched curvature of the carpal bones in direct continuation of the dorsal bowing of the radius on the lateral radiograph

Carter and Ezaki added the observations that there is an increased radial tilt on the PA radiograph and the radial epiphysis becomes teardrop shaped.[19] On the lateral view, the radius tilts volarward until the ulna appears dislocated from its normal articulation with the radius. The ulna lies dorsal to the proximal carpal row. The distal ulna does not actually sublux dorsally. Rather, the hand and radius are translated palmarward, resulting in incongruence of the DRUJ. The unaffected ulna continues to grow, ultimately becoming longer than the ulnar aspect of the radius.

Vickers and Nielsen use a physeal measurement for preoperative and postoperative assessment of severity and correction, respectively.[21] PA radiographs are used for measurement, as lateral views have too much variation to be of value. The longitudinal and transverse axes of the radius are difficult to determine due to the considerable bowing of the radius. The ulna usually is straight, which provides a reliable longitudinal axis. Therefore, a perpendicular line to the long axis of the ulna can be passed through the most radial extent of the radial physis. A line joining the most radial and ulnar extents of the physis is drawn. The angle subtended by the transverse line and the physeal line is termed the physeal angle. With increasing severity, the lunate follows the ulnar-volar corner and eventually becomes interposed between the radius and ulna. The relative position of the proximal aspect of the lunate can be measured from the transverse line.

CT scan or tomogram

Because the deformity is 3-dimensional, a better understanding may be obtained with tomograms or CT scans. These studies may be helpful in precisely visualizing physeal and articular morphology.

A CT scan–derived 3-dimensional reconstruction may assist in understanding the articular orientation. However, Carter and Ezaki state that after the complexity of this lesion is appreciated, CT scans and 3-dimensional imaging are not necessary for routine treatment.[19]

Proceed to Treatment & Management
 
 
Contributor Information and Disclosures
Author

Paul M Lamberti, MD  Principal Surgeon, Lamberti Orthopedic and Hand Surgery, LLC

Paul M Lamberti, MD is a member of the following medical societies: American Academy of Surgeons Orthopedic Surgery, American Medical Association, and American Society for Surgery of the Hand

Disclosure: Nothing to disclose.

Coauthor(s)

Terry R Light, MD  Dr William M Scholl Professor and Chair, Department of Orthopedic Surgery, Loyola University School of Medicine; Attending Surgeon, Department of Orthopedic Surgery and Rehabilitation, Loyola University Medical Center

Terry R Light, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association for Hand Surgery, American College of Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, Association of Bone and Joint Surgeons, and Illinois Association of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Charles T Mehlman, DO, MPH  Professor of Pediatrics and Pediatric Orthopedic Surgery, Division of Pediatric Orthopedic Surgery, Director, Musculoskeletal Outcomes Research, Cincinnati Children's Hospital Medical Center

Charles T Mehlman, DO, MPH is a member of the following medical societies: American Academy of Pediatrics, American Fracture Association, American Medical Association, American Orthopaedic Foot and Ankle Society, American Osteopathic Association, Arthroscopy Association of North America, North American Spine Society, Ohio State Medical Association, Pediatric Orthopaedic Society of North America, and Scoliosis Research Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

George H Thompson, MD  Director, Pediatric Orthopedics, Rainbow Babies and Children's Hospital

George H Thompson, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Association, Pediatric Orthopaedic Society of North America, and Scoliosis Research Society

Disclosure: OrthoPediatrics None Consulting; Journal of Pediatric Orthopaedics Salary Management position

Dinesh Patel, MD, FACS  Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital

Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Dennis P Grogan, MD  Clinical Professor, Department of Orthopedic Surgery, University of South Florida College of Medicine; Chief of Staff, Department of Orthopedic Surgery, Shriners Hospital for Children of Tampa

Dennis P Grogan, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, American Orthopaedic Association, American Orthopaedic Foot and Ankle Society, Eastern Orthopaedic Association, Irish American Orthopaedic Society, Pediatric Orthopaedic Society of North America, and Scoliosis Research Society

Disclosure: Nothing to disclose.

References

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  2. Nielsen JB. Madelung's deformity. A follow-up study of 26 cases and a review of the literature. Acta Orthop Scand. 1977;48(4):379-84. [Medline].

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  7. Beals RK, Lovrien EW. Dyschondrosteosis and Madelung's deformity. Report of three kindreds and review of the literature. Clin Orthop Relat Res. May 1976;24-8. [Medline].

  8. Berdon WE, Grossman H, Baker DH. Dyschondrosteose (Leri-Weill syndrome): congenital short forearms, Madelung-type wrist deformities, and moderate dwarfism. Radiology. Oct 1965;85(4):677-81. [Medline].

  9. Langer LO. Dyschondrosteosis, a hereditable bone dysplasia with characteristic roentgenographic features. Am J Roentgen Radium Ther Nucl Med. 1965;95(1):178-88.

  10. Blanco ME, Pérez-Cabrera A, Kofman-Alfaro S, Zenteno JC. Clinical and cytogenetic findings in 14 patients with madelung anomaly. Orthopedics. Mar 2005;28(3):315-9. [Medline].

  11. Palka G, Stuppia L, Guanciali Franchi P. Short arm rearrangements of sex chromosomes with haploinsufficiency of the SHOX gene are associated with Leri-Weill dyschondrosteosis. Clin Genet. Jun 2000;57(6):449-53. [Medline].

  12. Felman AH, Kirkpatrick JA Jr. Madelung's deformity: observations in 17 patients. Radiology. Nov 1969;93(5):1037-42. [Medline].

  13. Dawe C, Wynne-Davies R, Fulford GE. Clinical variation in dyschondrosteosis. A report on 13 individuals in 8 families. J Bone Joint Surg [Br]. 1982;64(3):377-81. [Medline].

  14. Plafki C, Luetke A, Willburger RE, Wittenberg RH, Steffen R. Bilateral Madelung's deformity without signs of dyschondrosteosis within five generations in a European family--case report and review of the literature. Arch Orthop Trauma Surg. 2000;120(1-2):114-7. [Medline].

  15. Shears DJ, Vassal HJ, Goodman FR. Mutation and deletion of the pseudoautosomal gene SHOX cause Leri-Weill dyschondrosteosis. Nat Genet. May 1998;19(1):70-3. [Medline].

  16. Schwartz RP, Sumner TE. Madelung's deformity as a presenting sign of Turner's syndrome. J Pediatr. Apr 2000;136(4):563. [Medline].

  17. Benito-Sanz S, del Blanco DG, Aza-Carmona M, Magano LF, Lapunzina P, Argente J. PAR1 deletions downstream of SHOX are the most frequent defect in a Spanish cohort of Léri-Weill dyschondrosteosis (LWD) probands. Hum Mutat. Oct 2006;27(10):1062. [Medline].

  18. Huber C, Rosilio M, Munnich A, Cormier-Daire V,. High incidence of SHOX anomalies in individuals with short stature. J Med Genet. Sep 2006;43(9):735-9. [Medline].

  19. Carter PR, Ezaki M. Madelung's deformity. Surgical correction through the anterior approach. Hand Clin. Nov 2000;16(4):713-21, x-xi. [Medline].

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  21. Vickers D, Nielsen G. Madelung deformity: surgical prophylaxis (physiolysis) during the late growth period by resection of the dyschondrosteosis lesion. J Hand Surg [Br]. Aug 1992;17(4):401-7. [Medline].

  22. Aharoni C, Glard Y, Launay F, Gay A, Legré R. [Madelung deformity: isolated ulnar wedge osteotomy]. Chir Main. Dec 2006;25(6):309-14. [Medline].

  23. de Billy B, Gastaud F, Repetto M. Treatment of Madelung's deformity by lengthening and reaxation of the distal extremity of the radius by Ilizarov's technique. European Journal of Pediatric Surgery. 1994;7:296-298.

  24. dos Reis FB, Katchburian MV, Faloppa F, et al. Osteotomy of the radius and ulna for the Madelung deformity. J Bone Joint Surg Br. Sep 1998;80(5):817-24. [Medline].

  25. Gong HS, Roh YW, Oh JH, Lee YH, Chung MS, Baek GH. Computed tomographic assessment of reduction of the distal radioulnar joint by gradual lengthening of the radius. J Hand Surg Eur Vol. Jun 2009;34(3):391-6. [Medline].

  26. Houshian S, Jorgsholm PB, Friis M, et al. Madelung deformity treated with Ilizarov technique: a report of two cases. J Hand Surg [Br]. Aug 2000;25(4):396-9. [Medline].

  27. Laffosse JM, Abid A, Accadbled F, Knör G, Sales de Gauzy J, Cahuzac JP. Surgical correction of Madelung's deformity by combined corrective radioulnar osteotomy: 14 cases with four-year minimum follow-up. Int Orthop. Dec 2009;33(6):1655-61. [Medline].

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Preoperative wrist posteroanterior radiograph of a 13-year-old girl (patient A) with dyschondrosteosis and Madelung deformity of the wrist.
Preoperative lateral wrist radiograph of patient A.
Lateral radiograph of elbow of patient A, depicting a dysplastic proximal radius. This is characteristic of dyschondrosteosis.
Preoperative anteroposterior radiograph of wrist of patient B. This patient has primary Madelung deformity (no sign of dyschondrosteosis).
Preoperative lateral radiograph of wrist of patient B. The flame-shaped radiolucency in the metaphysis of the radius is occupied by the fibrocartilaginous Vickers ligament.
Postoperative anteroposterior radiograph of wrist of patient B following Vickers physiolysis. Vickers ligament and the ulnar abnormal physis have been excised.
Postoperative lateral radiograph of the wrist of patient B following Vickers physiolysis.
Postoperative anteroposterior radiograph from patient A following biplane osteotomy of distal radius and ulnar shortening procedure.
Postoperative lateral radiograph from patient A following biplane osteotomy of radius and ulnar shortening procedure.
Preoperative photograph of 17-year-old girl (patient C) with idiopathic Madelung deformity.
Preoperative photograph of 17-year-old girl (patient C) with idiopathic Madelung deformity.
Preoperative posteroanterior radiograph of wrist from patient C.
Preoperative lateral radiograph of wrist from patient C.
Intraoperative photo of Vickers ligament, outlined in red.
Intraoperative color photograph of Vickers ligament. The ligament is outlined in the previous image.
Postoperative result in patient C. Compare with preoperative appearance.
Postoperative lateral radiograph. Note dorsal translation of distal radius after Carter-Ezaki dome osteotomy.
Postoperative posteroanterior radiograph with Kirschner-wire fixation in place. Note combination of ulnar translation of distal radius and correction of radial tilt towards normal after Carter-Ezaki dome osteotomy.
Preoperative plan prior to Carter-Ezaki dome osteotomy. Dorsal translation of the distal radius is depicted.
Preoperative plan prior to Carter-Ezaki dome osteotomy. Rotation of the distal radius to accomplish both ulnar translation and normalization of radial tilt is depicted.
 
 
 
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