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CASE REPORT Table of Contents   
Year : 2003  |  Volume : 37  |  Issue : 3  |  Page : 17
Unusual cases of preaxial polydactyly of hand- Report of three cases


General Hospital, Godhra, India

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How to cite this article:
Jain S. Unusual cases of preaxial polydactyly of hand- Report of three cases. Indian J Orthop 2003;37:17

How to cite this URL:
Jain S. Unusual cases of preaxial polydactyly of hand- Report of three cases. Indian J Orthop [serial online] 2003 [cited 2019 Dec 10];37:17. Available from: http://www.ijoonline.com/text.asp?2003/37/3/17/48559

   Introduction Top


Duplication of the thumb is the most common duplication pattern [1] as well as the most common congenital anomaly of the hand [2] occurring in 1:3000 births. It usually is unilateral and cause is unknown. Most occur sporadically and are unassociated with other malformation syndromes. [3] Three unusual cases of thumb duplication are presented.


   Case Reports Top


Case 1: A 22 year male presented with duplication of thumb on left hand. On examination, he was found to have Z shaped preaxial polysyndactyly with fully developed nail with an angle of 70° to proximal phalanges. Both distal phalanx were parallel to each other. The thumb was not shortened though abnormally broad and exhibited a radial deviation of the terminal phalange. The thumb bore two-separated nail with well-formed lunula. The radial duplicate was mild hypoplastic with divergence. There was no movement at interphalangeal joint. The thumb impression was a single loop in spite of two thumbs [Figure 1].

X-rays revealed picture of duplication at the level of proximal phalange without formation of a joint. The length of duplicated distal phalanges was one and half times of the parent distal phalanges [Figure 2].

Case 2: A 24 years male was found to have radially deviated supernumerary thumb. It was hypoplastic with well-developed nail with an angle of 45° to metacarpophalangeal joint [Figure 3]. There was no movement at polydactylous thumb. Sensation was intact with good nail bed circulation.

X-rays revealed relative long, thin monophalangeal polydactyly near the metacarpophalangeal joint, which made a difference in Wassel's type IV thumb duplication. There was no articular surface for thumb. Other carpals, metacarpals, phalanges were normal [Figure 4].

Case 3: A 26 years male was found to have floating thumb on right hand and an accessory-floating thumb in 1st web space in left hand with a parent swan-neck thumb.

Floating thumb on right hand and floating accessory thumb on left-hand take origin somewhat more distally than usual, but the site of origin of both was same. Fingernail was hypoplastic and there was neither extrinsic nor intrinsic muscle function. In left hand, depth of first web space was somewhat narrow and extends distally, giving picture of a triphalangeal thumb. Thumb was stiff with no active movement. Fixed hyperextension at metacarpophalangeal joint and fixed flexion by 80° at interphalangeal joint of thumb was seen. The thumb gave an appearance of "swan-neck deformity" [Figure 5].

In right hand, floating thumb consisted of three phalanges, proximal one was the small and broad giving different look. Between atypical proximal phalange and middle phalange, joint space was maintained. All carpal bone gave somehow atypical appearance. Right thumb showed maintained joint metacarpophalangeal and 80 degrees flexion at interphalangeal joint. Floating thumb was three phalanged with end-to-end fusion (trisymphalangism). Proximal half of phalange was broad and osteolytic and distal half of phalange was thin and osteosclerotic [Figure 6].

No other congenital anomaly was found in all cases and also no particular congenital problems were documented in the family history. The patients were well adjusted to their deformity.


   Discussion Top


Duplication of the thumb is the most common anomaly of the hand. [2] The incidence of thumb duplication has varied, ranging from 1/713 birth 4 to 1/12500 births. [5] Wassel described a group of 70 patients (79 hands) with bifid thumbs and suggested a now widely used classification of seven types of deformity. [7] Martinot- Duquennoy et al [8] studied 32 duplicated thumbs in which Wassel's type II comprised 25% of cases and most of them were not operated on due to refusal by the parents.

Tosti et al [9] described bifid thumbs, which were variation of Wassel's type I (partial duplication of the distal phalange and a common epiphysis). The thumb bore two nails that were separated by a longitudinal incision and each nail had a well-formed lunula. The presence of double nail of the thumb permitted a diagnosis of thumb polydactyly of Wassel's type 2, but the roentgenogram of the hand showed a bifid distal phalange with a common epiphysis (Wassel's type 1). Thus bifid nail was an additional clinical sign of the bone abnormalities of the distal phalanges. In case no. 1, findings were same but both morphology and roentenography were of Wassel's type 2.

Hung et al [10] had classified Wassel's type IV thumb duplication into 4 types: A. hypoplastic, B. ulnar deviated; C. divergent and D. convergent or complex. Type B ulnar deviated was the commonest (64%) and type D convergent (9%) was the rare. Case 2 was type C divergent.

Tuch et al [11] classified 36 duplicate thumbs according to Wassel's scheme, in which 5 thumbs did not fit any of the 7 types. Their 3 thumbs were variation of Wassel's type VII and 2 thumbs were variation of Wassel's type IV. There 2 thumbs variation of Wassel's type IV were ulnar deviated type of thumb polydactyly i.e. Hung's type B ulnar deviated. Author reported case no.2 was variation of Wassel's type IV with radial deviated type of thumb polydactyly i.e. Hung's type C divergent. Their 3 thumbs variation of Wassel's type VII were; triphalangism both thumb, incomplete and complete duplication with triphalangism variation one each of that. Duplication of floating thumb was near metacarpophalangeal jointed but it was of index finger in reported case 3. In this case, polydactyly was not fitting into Wassel's type as the supernumerary floating trisymphalangism thumb took origin near the head of second metacarpal at the same site of contra-lateral floating thumb. This type of variation created a lot of confusion. The site of both floating thumbs was same.

Ezaki [12] classified radial polydactyly of the hand into four type: Type I - thumb polydactyly (Wassel's type I-VI); Type II - polydactyly of a triphalangeal thumb; Type III - index finger polydactyly or five fingered hand and Type IV - thumb polydactyly with syndactyly. In case 3, it was near to type III but not type III as polydactylous part was representing most feature of floating thumb bilaterally.

Dobyns et al [13] reviewed 49 patients (54 thumbs) with radial polydactyly into 3 groups; Group 1 - severe ypoplastic duplication; Group 2 - partial / attempted duplication, which include Wassel's type and Group 3 - complete duplication or pseudoduplication. They noted only 2 cases of group1. Author noted case no.3 also was group 1. Kozin et al have done index finger pollicization for congenital aplasia of thumbs (10 hands) and hypoplasia of thumb (4 hands). [14] Associated anomalies were numerous and commonest was radial club hand in 4 cases. They did not noted bilateral floating thumb with an accessory thumb. No remote anomalies were detected in case 3. Ogino et al analyzed a total of 122 hands in 110 patients with thumb polydactyly in Japan and noted 8 patients with floating type of thumb polydactyly. [15] No one was radially deviated type of floating thumb polydactyly and all 8 cases were unilateral.

Congenital swan-neck deformity of the thumb is a rare congenital anomaly. The deformity is usually unilateral and is probably sporadic rather than inherited. However, several associated diseases do appear with this deformity: Trisomy D or Trisomy [13],[14],[15] , partial Trisomy D, Rothmund - Thompson syndrome etc. [16] Author reported case was associated with bilateral floating thumb apart from swan-neck deformity on left thumb.

 
   References Top

1.Flatt AE. The care of congenital hand anomalies. St Louis; CV Mosby-Year Book, Inc. 1977: 99-117.   Back to cited text no. 1    
2.Woolf CM, Myrianthopoulos NC. Polydactyly in Ameri-can negroes and whites. J Hum Genet 1973; 25: 397- 404.   Back to cited text no. 2    
3.Mark TJ, Phillip EW. Congenital anomalies of hand. In: Campbell's operative orthopaedics (Ed. Crenshaw AH). 5th ed. St Louis; Mosby-Year Book, Inc. 1992 : 3353-3425.  Back to cited text no. 3    
4.Sesgin MZ, Stark RB. The incidence of congenital de-fects. Plast Reconstr Surg 1961; 27: 261.  Back to cited text no. 4    
5.Temtamy SA, McKusick VA. The genetics of hand mal­formations. Births Defects, Original Article Series V, New York; Alan Liss. 1978; 14(3): 364.  Back to cited text no. 5    
6.Swanson AB, Swanson G, Tada K. A classification for congenital limb -malformation. J Hand Surg 1983; 8: 639- 702.   Back to cited text no. 6    
7.Wassel HD. The results of surgery for polydactyly of the thumb: a review. Clin Orthop 1969; 64: 175.   Back to cited text no. 7  [PUBMED]  
8.Martinot - Duquennoy V, Lahouel K, Herbaux B, Debeugny P. The treatment of duplication thumbs in children in the series of 32 patients. Eur J Pediatr Surg 1993; 3 (1): 37 - 40.  Back to cited text no. 8    
9.Tosti A, Paoluzzi P, Baron R. Doubled nail of the thumb a rare form of polydactyly. Dermatology 1992; 184 (3): 216-8.  Back to cited text no. 9    
10.Hung L, Cheng JC, Bundoc R, Leung P. Thumb dupli­cation at the metacarpophalangeal joint: Management and a new classification. Clin Orthop 1996; 323: 31-41.   Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.Tuch BA, Lipp EB, Larson IJ, Gordon LH. A review of supernumerary thumb and its surgical management. Clin Orthop 1977; 125: 159-67.  Back to cited text no. 11    
12.Ezaki M. Radial polydactyly. Hand Clin 1990; 6(4): 577-88.   Back to cited text no. 12    
13.Dobyns JH, Lipscomb PR, Cooney WP. Management of thumb duplication. Clin Orthop 1985; 195: 26-44.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]
14.Kozin SH, Weiss AA, Webber JB. Index finger pollicization for congenital aplasia or hypoplasia of the thumb. J Hand Surg 1992; 17-A: 880-84.  Back to cited text no. 14    
15.Ogino T, Ishii S, Minami M. Radially deviated type of thumb polydactyly. J Hand Surg 1988; 13(3): 315-9.   Back to cited text no. 15    
16.Wood VE. Congenital thumb deformities. Clin Orthop 1985; 195: 7-25.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]

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Correspondence Address:
Sanjay Jain
454-C, Sneh Nagar, Jabalpur-482002
India
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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