Ulnar dimelia variant: a case report
© The Author(s) 2011
Received: 9 September 2010
Accepted: 17 June 2011
Published: 19 July 2011
We report a case of ulnar dimelia, commonly called mirror hand, in a 2-month-old female child who had restriction of elbow flexion and forearm rotation. There was no facial or other internal organ malformation. Radiographs revealed seven triphalangeal digits with double ulnae (one following the other) and absent radius. To the best of the authors’ knowledge, this is the first report of this mirror hand deformity in which fingers are symmetrical while duplicated ulnae are not.
Mirror hand (ulnar dimelia) is an extremely rare congenital anomaly of the upper limb . Characteristically there is duplication of the ulna, absent radius and multiple fingers which are symmetrical around the midline. Over the years, there have been several case reports in the literature, and some variations of the deformity have been described.
We describe another variant of ulnar dimelia with multiple fingers symmetrical around midline along with duplication of the ulna, which was asymmetrical around midline, i.e. instead of facing each other, they were following each other.
Mirror hand (ulnar dimelia) is an extremely rare congenital anomaly of the upper limb . Characteristically there is duplication of the ulna with both ulnae facing each other [2, 3], absent radius and multiple fingers which are symmetrical around the midline. There are few descriptions of mirror hand deformity with different variants in the literature. Al-Qattan and Al-Thunayan  proposed a classification of mirror hand deformity based on presence or absence of other congenital anomalies and the type of forearm bones present. Type 1 includes ulnar dimelia, type 2 includes two ulnae and one radius, type 3 includes one radius and one ulna, type 4 includes associated anomalies (syndromic) and type 5 includes multiple hands on a single limb. In the literature several variants of mirror hand deformity have been described. Multiple fingers with one ulna and one distinct radius , multiple fingers with two ulnae (one vestigial) and one radius  and multiple fingers with two ulnae (one vestigial) and one radius with duplicated proximal humerus  have been described. In the literature, no case has been reported so far with type 1 mirror hand variant, in which complete duplication of hand with seven fingers and double ulna occur and one ulna follows the other, instead of facing each other as mirror image.
Management of this condition is directed at achieving a functional and aesthetic upper limb and involves multiple, complex operations. Jafari performed pollicisation after excision of three digits in a case of ulnar dimelia with eight digits in a 20-year-old patient . Avadis, in a 7-year-old patient of ulnar dimelia with seven digits, performed single-stage surgery involving pre-axial ulnar excision, excision of two radial digits, pollicisation and release of wrist flexors and achieved acceptable functional and cosmetic result . Afshar also performed pollicisation after excision of one digit in a six-digit child of 4 years of age with ulnar dimelia .
In this patient, because of unusual duplication of ulna, the radial ulna seems to be non-articulating with trochlea, hence excision of proximal end of radial ulna along with repair of collateral ligament and soft tissues to the proximal part of the normally placed ulna by antero-lateral approach would give a reasonable result regarding elbow flexion and forearm rotation.
Informed consent was taken from the parents of the patient prior to inclusion in the study. The study was performed according to the Declaration of Helsinki, and the Institutional Ethical Board approved it.
Conflict of interest
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