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Ulnar Dimelia
Zaid Sharif, Dhia Saeed  Saleh,

Medical City Teaching Hospital, Baghdad, Iraq

Address for Correspondence
Zaid Sharif,
Medical City Teaching Hospital, Baghdad, Iraq


We describe a case of Ulnar Dimelia commonly called Mirror hand. This is an exceedingly rare congenital anomaly of the upper limb. In this case we found a complete dislocation of the shoulder with a limited range of movement. No other facial or internal organ malformation, the patient has complete absence of the Radius including  the capitalum. 

J.Orthopaedics 2006;3(4)e15

Case Report

A two years old female patient presented with left upper limb deformity since birth involving hand, wrist, elbow and shoulder impeding the normal function of the limb. She is the second child of the family. There was no similar condition among the family or relatives.

On examination, the patient was well built, with good mentality. Localy, the left hand has seven fingers, six of them are well formed but the seventh is small and rudimentary. All fingers lie in the same plane with slight apposition between the two halves. There is no thumb. The fingers are somewhat flexed, and the hand as a whole is usually radialy deviated at the wrist and dropped and cannot be extended

The elbow is stiff in extension and the arm is short. Pronation and supination were limited. As to the shoulder, there is loss of the normal contour, with prominence of the tip of the acromian, all shoulder movements were grossly restricted especially abduction , with the humeral head can be felt in the axilla lying against the ribs.




A picture showing the absence of the thumb. The fingers are all in one line  X- Ray showed The ulna and the ulnar carpal bones are completely duplicated, the scaphoid and trapezium are replaced, and distal ulnar epiphysis is broadened.
At the elbow, each of the duplicated ulnae articulates with the distal humerus separately and they tend to face each other .There is no capitalum on the distal humerus. At the shoulder, it is dislocated with the glenoid.hypoplasia of the Scapula.

(Fig.3) an Ap view of the forearm 


Discussion :

Ulnar Dimelia occurs as radial and ulnar clusters of fingers in the same hand that are near images of each other. It is considered  aduplication phenomenon of substitution of the radial components as well,

The understanding of the embryology of mirror image deformities has increased dramatically since the early work of Saunders and Gasseling, who first produced mirror image digit duplications in chicks by grafting a small piece of posterior border mesoderm into an anterior position(9).

It is now understood that this zone of polarizing activity expresses the gene sonic hedgehog, which regulates limb patterning on an anteroposterior axis(5,6). Ectopic expression of the zone of polarizing activity cells or the sonic hedgehog gene can therefore produce mirror image deformities(1).

More recently, secondary signaling molecules such as bone morphogenic proteins and certain Hox genes have been implicated in the embryogenesis of mirror image deformities(6). Hox genes encode positional information during embryogenesis. Hox b-8 is thought to be important in the specification of the zone of polarizing activity cell positioning, ectopic expression of which has experimentally resulted in mirror image duplication(3,6) Retinoids seem to regulate the expression of Hox genes(3).

Others may argue that Ulnar Dimelia is not as easily classified as pure duplication (11, 8). This indicates that the Radius was absent and the ulna noticed in place of the radius. Her the archipterygeal theory could be taken into consideration. It is stated that due to reduplication the main stem resulted in the development of the double ulna(13).

Approximately 70cases have been reported,  4 of which with shoulder dislocation including our case(7) . The largest reported series is that of Harrison, Pearson and Roaf in which they described the deformity in three patients (10), its occurrence is usually sporadic (10).

Ulnar dimelia is usually associated with some degree of hypoplasia of the arm and scapula which is present in this case, and sometimes with fibular dimelia and absent tibia which is not in our case. However: a multimodality imaging approach, exploring the various aspects of the malformation is mandatory to help the surgeon in order to obtain a functional and aesthetic upper limb after complex surgical procedures taking into account the various aspects of the malformation(7).The reconstructive procedures are described toward restoring elbow flexion, forearm supination (by excision of the proximal part of one of the Ulnae ), wrist extension and policization to allow apposition (4).

Reference :

  1. Viljoen, D. L., and Kidson, S. H. Mirror polydactyly: Pathogenesis based on a morphogen gradient theory. Am. J. Med. Genet. 35:229, 1990.

  2. Sinha, D.N. Possibility of Cell Death Induced Skeletal Malformations Of The Upper Limb J Anat. Soc. India 49(2) 158-160 (2000) Vol. 49, No. 2, December, 2000.

  3. Hatchwell, E., and Dennis, N. Mirror hands and feet: A further case of Laurin-Sandrow syndrome. J. Med. Genet. 33:426, 1996.

  4. Lister G: Wrist and Hand : Congenital Anomalies And  Pediatric Reconstruction .In: Orthopedic Knowledge Update 4, Upper Extremity ,Chapter 30. p 178- 180.

  5. Hersh, J. H., Dela Cruz, T. V., Pietrantoni, M., et al. Mirror image duplication of the hands and feet: Report of a sporadic case with multiple congenital anomalies. Am. J. Med. Genet. 59:341, 1995

  6. Al-Qattan, M. M., Al-Thunayan, A., De Cordier, M., Nandagopal, N., and Pitkanen, J. Classification of the mirror hand: Multiple hand spectrum. J. Hand Surg. (Br.) 23:534, 1998.

  7. Radiol. J  Ulnar dimelia: imaging modalities and surgical implications 2000 Mar.;81(3):219-22.

  8. Chinegwundoh JO, Gupta M, Scott WA Ulnar dimelia. Is it a true duplication of the ulna? J Hand  Surg. [Br]. 1997 Feb;22(1):77

  9. Saunders, J. W., and Gasseling, M. T. Ectodermal–Mesenchymal Interactions in the Origin of Limb Symmetry. In R. Fleishnajer and R. E. Billingham (Eds.), Epithelial–Mesenchymal Interactions. Baltimore: Williams & Wilkins, 1968. Pp. 78–97.

  10. Harrison RG,Pearson MA, Roaf 1960 J.B.J.s , 42-B: 549 .

  11. Mark T. Jobe and Phillip E. Wright II . Congenital Anomalies of Hand. In: S .

  12. Terry Canale, editor.  Campells Operative Orthopaedics,9th. Edition, ,1998. Vol.4 p.3792.


This is a peer reviewed paper 

Please cite as :Zaid Sharif : Ulnar Dimela

J.Orthopaedics 2006;3(4)e15







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