Iain Whitaker

Cronfa at Swansea University

149ONLINE CASE REPORTAnn R Coll Surg Engl 2012; 94: e149–e151doi 10.1308/003588412X13171221589577The oldest reported mirror hand? The curse of the coal-house frog!NJ Marsden, IS Whitaker, DE BoyceAbertawe Bro Morgannwg University Health Board, UKAbSTRACTDespite being one of the rarest congenital upper limb abnormalities, a wide spectrum of the typical mirror hand has been de-scribed in the literature. We report a very interesting case of a new variant of mirror hand presenting in a 78-year-old man. The unique features of the case include the age at diagnosis, the anatomical features present and the acquired function despite no form of reconstructive surgery.KEywORdSLaurin-Sandrow Syndrome – Congenital Abnormalities – Polydactyly Accepted 18 November 2011; published online 30 April 2012CORRESPONdENCE TONick Marsden, Hand and Peripheral Nerve Surgery Unit, Welsh Centre for Burns and Plastic Surgery, Morriston Hospital, Heol Maes Eglwys, Morriston, Swansea SA6 6NL, UKE: nick_marsden@hotmail.comAnn R Coll Surg Engl 2012; 94: e149–e151Mirror hand is one of the rarest congenital abnormalities affecting the upper limb, with around 70 cases reported in the literature. It is characterised by multiple digits, typical-ly seven in total, with symmetry about the midline, absent thumb, excess carpal bones, absence of radius and dupli-cation of the ulna (ulna dimelia).1 We describe an unusual case of delayed diagnosis of mirror hand that presented to a hand unit when the patient was aged 78 years.Case historyA 78-year-old man was referred with a suspicious skin le-sion on his left shoulder and at the time an abnormal left upper limb was noted. He was on dialysis for chronic renal failure, secondary to polycystic kidney disease. He was born via normal delivery to non-consanguineous parents with no family history of congenital defects. He had two fingers re-moved from his left hand at 6 months of age but no further investigation or reconstructive surgery had taken place. He was told by his mother that he was born with a hand like a frog as a consequence of a frog having jumped on to her abdomen during pregnancy while collecting coal from a coal-house!On examination, the patient had absence of the thumb with five fingers on his left hand. The most radial digit tended to partial opposition to the other four fingers, simi-lar to the appearance after a pollicisation procedure (Fig 1). He had a range of movement of 15–70º at the wrist and 10–60º at the elbow. The forearm was held in pronation and the left arm was slightly shorter than the right (Fig 2). An ar-teriovenous fistula was present to facilitate haemodialysis.The patient had good function with both hands. He worked previously as a draftsman for a toy company and in his spare time he was keen on DIY. He had no need to hold a pen in his left hand as he was right hand dominant but holding a knife and fork or other household objects caused no problem. He drove a non-adapted manual car.Radiological evaluation revealed five triphalangeal digits with normal metacarpals and an absent thumb. The radius was absent and two ulna bones were present. Figure 1  Left forearm and hand: The posture of the most radial digit tends towards opposition.1706 Marsden.indd   149 16/04/2012   11:27:34150 Ann R Coll Surg Engl 2012; 94: e149–e151MArSDEN WHiTAKEr BoyCE ThE OLdEST REPORTEd MIRROR hANd? ThE CuRSE OF ThE COAL-hOuSE FROg!discussionA wide spectrum of variants ranging from ulna dimelia to the multiple hand have been described in the literature. Al-Qattan produced a classification of the mirror hand-multi-ple hand spectrum (Table 1).2The exact genetic basis is unknown. However, the most commonly proposed event is impairment of the mechanism responsible for the organisation and differentiation of the limb bud,3 not that of primary duplication, which was ini-tially thought to be the case. Several authors have demon-strated cases where the distal end of the preaxial ulna has broadened to take on a shape similar to that of the radial head, to aid mechanical function of the hand, which tends Table 1  The classification of the mirror hand-multiple hand spectrum described by Al-Qattan2Type Features Syndrome1A Ulna dimelia (classic mirror hand)1B Ulna dimelia – preaxial ulna hypoplastic2 2 ulnas (one vestigial) + radius3A Multiple fingers + 1 ulna + 1 normal radius3B Multiple fingers + 1 ulna + 1 hypoplastic radius4A Bilateral multiple fingers with complex syndactyly +feet/facial defects + ulna dimeliaSandrow4B Bilateral multiple fingers with complex syndactyly +feet/facial defects + ulna + radiusMartin5 Multiple hand (complete duplication) + normal ulna/radiusFigure 3  oblique and lateral plain x-rays of left wrist and forearm, showing duplicate ulna and additional carpal bones. Each ulna has an olecranon.Figure 4  Vestigial humerus extending proximally from the lateral epicondyle to mid-humerus levelInterestingly, both ulnas had a broadened distal end, which mimicked that of distal radii (Fig 3). The humerus appeared abnormal with a thin vestigial bone extending proximally from the lateral epicondyle to the mid-humerus level (Fig 4). The scapula and humeral head were hypoplastic in comparison to the right. More detailed radiology could not be arranged as the patient passed away soon after present-ing to our department.Figure 2  Photograph demonstrating the shorter left forearm and flexion deformity of the wrist. The arteriovenous fistula is evident.1706 Marsden.indd   150 16/04/2012   11:27:34151Ann R Coll Surg Engl 2012; 94: e149–e151MArSDEN WHiTAKEr BoyCE ThE OLdEST REPORTEd MIRROR hANd? ThE CuRSE OF ThE COAL-hOuSE FROg!to favour failure of differentiation as the primary event.2–4 In our case, both ulnas had a broadened distal end, similar to that of a distal radius. To our knowledge, this is the first description of such a case in the literature, possibly due to the age of the patient at diagnosis, resulting in a protracted period for bony remodelling. This may possibly be an adap-tation to the abnormal mechanical load at the wrist between the two ulnas and the abnormal carpus.It is unknown whether the patient’s renal disease was associated with the congenital hand. However, there is one reported case in the literature of a child with mirror hand who also had a multicystic kidney, raising the possibility of a link.5Treatment options vary between the spectrum of defects. The main principle of surgical correction is amputation of the accessory digits (usually radially), either at the level of the carpometacarpal joints or at the metacarpal base, and pollicisation of the most radial retained digit. Problems with flexion deformities of the wrist can be treated with splinting, arthrodesis of the joint or tendon transfer to aid wrist ex-tension. Our case highlights that it is possible for the upper limb to adapt to provide a well functioning hand, even in the absence of reconstructive surgery.ConclusionsWe describe a case of mirror hand in a 78-year-old man, who despite no reconstructive surgery had a well function-ing hand. Not only is this case unusual due to the age of the patient at diagnosis but also because of the features present. We believe our patient may be a new variant on the typical 1A mirror hand, with duplicate ulnas, multiple metacarpals and fingers, associated with an abnormal distal humerus, which we believe has not been described previously in the literature.References1. Wood VE. Ulnar Dimelia (the Mirror Hand). in: Green DP, ed. Operative Hand Surgery. 2nd edn. New york: Churchill Livingstone; 1988. pp485–491.2. Al-Qattan MM, Al-Thunayan A, De Cordier M et al. Classification of the mirror hand-multiple hand spectrum. J Hand Surg Br 1998; 23: 534–536.3. Gorriz G. Ulnar dimelia – a limb without anteroposterior differentiation. J Hand Surg Am 1982; 7: 466–469.4. Bhaskaranand K, Bhaskaranand N, Bhat AK. A variant of mirror hand: a case report. J Hand Surg Am 2003; 28: 678–680.5. El Hage S, Ghanem i, Megarbané A et al. Mirror hand deformity: a new phenotype with literature review. Rev Chir Orthop Reparatrice Appar Mot 2008; 94: 174–178.1706 Marsden.indd   151 16/04/2012   11:27:34

The oldest reported mirror hand? The curse of the coal-house frog!

Abstract

Similar works

Full text

Available Versions

Cronfa at Swansea University