Journal of Pediatric Surgery 49 (2014) 474–476

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Ulnar polydactyly — An analysis of appearance and postoperative outcome☆,☆☆ Georg Singer ⁎, Stephanie Thein, Tanja Kraus, Thomas Petnehazy, Robert Eberl, Barbara Schmidt Department of Pediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria

a r t i c l e

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Article history: Received 18 May 2013 Accepted 27 June 2013 Key words: Malformation hand Outcome Ulnar polydactyly

a b s t r a c t Background/Purpose: Ulnar polydactylies present with a wide variation in the size and appearance of the affected finger, ranging from small cutaneous appendages to fully formed additional fingers. Since ulnar polydactyly is rarely associated with functional disabilities, psychological and cosmetic reasons are the main rationale for surgical treatment. The aim of this study was to present the appearance, treatment regimen, and outcome of a series of children treated with ulnar polydactylies. Methods: Data of all pediatric patients treated between 2000 and 2010 were included. For assessing long-term outcome, patients were contacted via mail and asked to complete a questionnaire concerning location and appearance of the scar as well as functional and cosmetic satisfaction (VAS 0–100). Results: In that period, 32 patients (20 male, 12 female) with 53 ulnar polydactylies were treated. In 20 patients the polydactyly occurred bilaterally (63%), in 10 patients on the left side (31%), and 2 patients presented with right-sided ulnar polydactyly (6%). The vast majority of ulnar polydactylies were floating cutaneous appendages. The mean age of the patients at operation was 8.6 months (range 0 to 10 years). 20 patients (63%) responded to the postal questionnaire. Follow-up time was 4.9 years (range 2.1 to 10 years). The majority of patients reported a flat scar (n = 18, 90%). Mean overall satisfaction with the postoperative result using a VAS 0–100 score was 89. Conclusion: We were able to report a good postoperative outcome of a series of patients suffering from ulnar polydactylies. © 2014 Elsevier Inc. All rights reserved.

Digital duplications rank amongst the most common congenital hand anomalies [1] and may exist either on the radial (preaxial polydactyly) or ulnar (postaxial polydactyly) side. The rarely encountered duplications of the second to fourth digit are referred to as central polydactylies [2]. While in African–Americans polydactyly is more common on the ulnar side of the hand [3], radial polydactyly is the more common form of polydactyly in Caucasians [4]. The incidence varies from 1:1500 to 1:3300 and from 1:100 to 1:300 live-births in Caucasians and African–Americans, respectively [5]. Ulnar polydactyly most often is an isolated finding occurring sporadically in an autosomal dominant trait. Nevertheless, associations with other defects have been reported. These defects can be confined to other limb malformations or can be part of different syndromes. Associated syndromes include – amongst others – Ellis– van Crefeld, McKusik–Kaufman and Bardet–Biedl syndrome [6]. There is a wide variation in the size and appearance of the affected finger ranging from small cutaneous appendages to fully formed

☆ Conflict of Interest: none. ☆☆ Funding: none. ⁎ Corresponding author. Department of Pediatric and Adolescent Surgery, Medical University of Graz, Auenbruggerplatz 34, 8036 Graz, Austria. Tel.: +43 316 385 13762; fax: +43 316 385 13775. E-mail address: [email protected] (G. Singer). 0022-3468/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.06.029

additional fingers. Temtamy and McKusick termed a fully formed supernumerary digit type A polydactyly and a non-functioning and incompletely formed finger type B polydactyly [7]. Stelling and Turek classified ulnar polydactyly into three types based on the degree of duplication: type 1, duplication of soft parts only; type 2, partial duplication of the digit, including osseous structures and type 3, complete duplication of the ray including the metacarpal [8]. Since ulnar polydactyly rarely is associated with functional disabilities, psychological and cosmetic reasons are the main rationale for surgical treatment. Depending on the grade of polydactyly, surgical procedures range from simple suture ligation performed in the newborn nursery to more complex reconstructive interventions. However, there are still uncertainty and inequality in treatment decisions for all but the most complex forms of ulnar polydactyly [9]. Suture ligation is described to lead to painful neuroma and/or an unaesthetic amputation stump [10]. Therefore, a more specific surgical approach has been recommended by some authors [9]. The aim of the present study was to present the appearance, treatment regimen and outcome of a series of children treated with ulnar polydactylies. 1. Patients and methods Following IRB approval (EK 24/380 ex 11/12), all children treated with ulnar polydactylies between 2000 and 2010 were included. Data

G. Singer et al. / Journal of Pediatric Surgery 49 (2014) 474–476

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Fig. 1. Example of a 6 month old girl with Stelling and Turek Type I ulnar polydactyly on the left side and Stelling and Turek Type II ulnar polydactyly on the right side. The patient was treated with surgical excision of both additional fingers. At follow-up up of 7.4 years the patient reported a VAS of 95, a flat scar, no pain and normal function.

were collected retrospectively and analyzed with regard to gender, type of ulnar polydactyly, association with other hand/foot deficiencies, age at surgery, type of treatment and complications during treatment. The patients’ radiographs were reviewed and the type of ulnar polydactyly was classified according to Stelling and Turek (Fig. 1). Additionally, bilateral radiographs of the feet were performed in cases with clinical signs of polydactyly of the feet. 1.1. Treatment strategies In cases of Type 1 ulnar polydactylies according to Stelling and Turek (floating appendages) oval excision was performed under general anesthesia. Care was taken to avoid an unaesthetic amputation stump and to carry out a deep resection of the nerve in order to prevent the development of a painful neuroma. Stelling and Turek Type II and III ulnar polydactylies were treated with more complex ablation of the digit including corrective osteotomy, ligament and meticulous soft tissue reconstruction and articular contouring. Attention was paid to preserve the tendinous and neurovascular structures in order to facilitate optimal function of the remaining digit. 1.2. Follow up In order to assess the long-term outcome, patients were contacted via mail and asked to complete a postal questionnaire. The questionnaire included questions concerning location and appearance of the scar as well as functional and cosmetic satisfaction (VAS 0–100). Additionally, a family history of hand and/or foot deficiencies was surveyed. Data were entered in a Microsoft Excel spreadsheet for descriptive analysis. 2. Results Between 2000 and 2010, 32 patients (20 male, 12 female) with 53 ulnar polydactylies were treated. Of these, the malformation occurred in three pairs of siblings. In 20 patients the polydactyly occurred

bilaterally (63%), in 10 patients on the left side (31%) and 2 patients presented with right-sided ulnar polydactyly (6%). The vast majority of ulnar polydactylies were floating cutaneous appendages. The exact distribution of the 53 polydactylies according to Stelling and Turek is shown in Table 1. While 22 patients (69%) presented with ulnar polydactylies without other anomalies, additional polydactylies of the hands or feet were recorded in 10 patients (31%). The distribution of these additional polydactylies is presented in Table 2. Six patients (20%) presented with a syndrome (two with Ellis–van Creveld Syndrome, two with Jeune Syndrome and two with an unclassified dysmorphic syndrome) and one girl suffered from a duodenal atresia. Of the 32 patients in our series, 13 children (40%) had a positive family history for other types of polydactylies. The mean age of the patients at operation was 8.6 months (range 0 to 10 years). Minor complications occurred in a total of 4 patients (12%), of which three patients developed a hypertrophic scar and in one patient a superficial wound infection occurred. Another patient developed a painful neuroma necessitating a second surgical intervention three years following the initial resection of a type I ulnar polydactyly. The questionnaire was sent to all 32 patients. 20 patients (63%) responded, 3 patients (9%) could not be reached due to unknown address and 9 patients (28%) did not respond. The follow-up time for the patients was 4.9 years (range 2.1 to 10 years) after operation. The 20 patients who responded in total had 30 ulnar polydactylies (n = 27 Stelling and Turek Type I, n = 3 Type II). The majority of patients reported a flat scar (n = 18, 90%), while 2 patients stated to suffer from a residual nubbin (10%). None of patients reported functional differences between the two hands. One patient (5%) reported occasional pain of the scar. Mean overall

Table 2 Characterization concerning the occurrence of other polydactylies of 32 patients presenting with 53 ulnar polydactylies. Patients

left ulnar

Table 1 Classification of 53 ulnar polydactylies seen in 32 patients according to Stelling and Turek. Type

Appearance

Number of polydactylies

Percentage

1 2 3

floating cutaneous appendage partial duplication complete duplication

41 8 4

77 15 8

14 7 4 2 1 1 1 1 1

right radial

X X X X X X X

foot

ulnar

radial

foot

X X X X X X X

X X X X X

X X X

X

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G. Singer et al. / Journal of Pediatric Surgery 49 (2014) 474–476

Table 3 Detailed findings of the long-term outcome of 20 patients treated for ulnar polydactylies. Occurrence

bilateral left left bilateral bilateral bilateral right left bilateral left bilateral bilateral left right bilateral left bilateral bilateral left left

Stelling and Turek right

left

2

1 1 1 1 1 1

1 1 1 1 1 1 2 1 1 1 1

1 1 1 1 1 1 1 1 1 1 2 2

time of f/u [a]

VAS

scar

function

pain

3.13 4.93 5.94 2.23 6.59 3.22 2.16 2.55 4.39 7.78 3.02 7.39 5.65 4.27 4.88 7.13 3.22 10.09 6.40 4.25

100 90 90 100 100 100 100 10 90 85 100 95 90 90 100 75 100 100 85 85

flat flat flat flat flat flat flat nubbin flat flat flat flat flat flat flat nubbin flat flat flat flat

normal normal normal normal normal normal normal normal normal normal normal normal normal normal normal normal normal normal normal normal

no no no no no no no no no occasional no no no no no no no no no no

f/u…follow-up, VAS…visual analogue scale (rated by the patients and the parents).

satisfaction with the postoperative result using a VAS 0–100 score was 89 (ranging from 10 to 100). The detailed findings of the long-term outcome are presented in Table 3. 3. Discussion The present study aims to assess the postoperative outcome of a series of children treated with ulnar polydactylies. Generally, ulnar polydactyly represents a common malformation of the hand. Two clinical forms of ulnar polydactyly are described in the literature, the African and the Caucasian form [11]. The African form is reported with an incidence of 1:150 to 1:300, more often occurs bilaterally without association with syndromes and mostly is familial (90%). In contrary, the Caucasian form is reported with an incidence between 1:1500 and 1:3000, most cases are sporadic, the majority of the cases are unilateral (80%) and in 10% the ulnar polydactyly is part of a syndrome [11]. Due to demographic reasons, the series of patients reported in the present study more closely resembles the Caucasian form. While we cannot provide information about the incidence, the occurrence of other malformations of the hand or foot in a third of our patients and the association with a syndrome in a fifth of the children in this series confirm these previous findings. However, compared to other reports about the Caucasian form, we found a familial occurrence in 40% and a bilateral occurrence in more than 60% of our patients. A recent publication by Pritsch and coworkers describes a positive family history of polydactyly of the foot or hand in 47% of 49 mostly Caucasian patients [8]. More than 40 syndromes are described to be associated with ulnar polydactyly including Trisomy 13, McKusic–Kauffman and Pallister– Hall syndrome [2]. The syndromal cases in the present series include Ellis–van Creveld, Jeune syndrome and two patients with an unclassified dysmorphic syndrome. The high number of syndromal cases underlines the necessity of an accurately performed examination when dealing with children seen with ulnar polydactyly. It is widely accepted that the more complex forms of ulnar polydactylies require elaborate reconstructive procedures performed in specialized centers. Nevertheless, the majority of ulnar polydactylies are Stelling type I resembling floating cutaneous appendages [2]. Dodd and colleagues have demonstrated that there are uncertainty and inequality in treatment decision of these forms [9]. Suture ligation

at the base of the duplicated digit done in the nursery represents the most commonly performed procedure [12]. The aim of this approach is to produce necrosis and subsequent autoamputation of the additional digit. Advantages of suture ligation include its cost-effectiveness and simplicity. In recent years, however, the practice of suture ligation has been questioned by various authors due to the possible development of painful neuroma or incomplete amputation leaving behind an unaesthetic scar [13]. In 2011, Patillo and Ryan reported two complications of suture ligation ablation for ulnar polydactyly. One case consisted of bilateral retained gangrene and a second case consisted of a residual sensitive skin tag [10]. When performing suture ligation the nerve of the rudimentary digit is divided at the level of the skin and it cannot retract into the soft tissue. Therefore, the development of painful neuroma is favoured. Various authors have reported patients with sensitive amputation neuromas necessitating surgical revision [14,15]. Based on these results we prefer to perform excision of Stelling and Turek type 1 ulnar polydactylies under general anesthesia with meticulous resection of the nerve. Watson and Hennrikus re-examined 15 of 21 children treated with ligation of an ulnar polydactyly at an average age of 20 months. No finger had a perfectly normal cosmetic appearance and the authors noticed a residual bump in 43% of the patients [16]. In contrary, only 2 of the 20 patients (10%) included in our study developed a bump at the excision site at a mean follow-up time of 4.9 years (see Table 3). The subjective satisfaction with the postoperative results seemed to correlate with the presence of a postoperative bump. While the two patients with the elevated scars reported a subjective satisfaction of VAS 10 and 75, the remaining patients reported a satisfaction of mean 94. These results demonstrate the superiority of the formal surgical excision of floating cutaneous ulnar digits related to postoperative patient satisfaction. In conclusion, we were able to report the good postoperative outcome of a series of patients suffering from ulnar polydactylies. Moreover, we suggest the formal excision with meticulous resection of the additional nerve in Stelling and Turek type 1 ulnar polydactylies. References [1] Castilla E, Paz J, Mutchinick O, et al. Polydactyly: a genetic study in South America. Am J Hum Genet 1973;4:405–12. [2] Rayan GM, Frey B. Ulnar polydactyly. Plast Reconstr Surg 2001;6:1449–54 [discussion 1455–1447]. [3] Kanter WR, Upton J. “Pacifier polydactyly”: a transitional form between pedunculated polydactyly and rudimentary polydactyly. Plast Reconstr Surg 1989;1:136–9. [4] Chong AK. Common congenital hand conditions. Singapore Med J 2010;12: 965–71. [5] Galjaard RJ, Smits AP, Tuerlings JH, et al. A new locus for postaxial polydactyly type A/B on chromosome 7q21-q34. Eur J Hum Genet 2003;5:409–15. [6] Wollina U, Verma SB. Sporadic familial ulnar hexadactyly of all four limbs. J Dermatol Case Rep 2010;1:6–10. [7] Temtamy SA, McKusick VA: The genetics of hand malformations. Birth Defects Orig Artic Ser 1978;3:i-xviii, 1–619. [8] Pritsch T, Ezaki M, Mills J, et al. Type A ulnar polydactyly of the hand: a classification system and clinical series. J Hand Surg Am 2013;3:453–8. [9] Dodd JK, Jones PM, Chinn DJ, et al. Neonatal accessory digits: a survey of practice amongst paediatricians and hand surgeons in the United Kingdom. Acta Paediatr 2004;2:200–4. [10] Patillo D, Rayan GM. Complications of suture ligation ablation for ulnar polydactyly: a report of two cases. Hand (NY) 2011;1:102–5. [11] Al-Qattan MM, Al-Shanawani B, Al-Thunayan A, et al. The clinical features of ulnar polydactyly in a Middle Eastern population. J Hand Surg Eur Vol 2008;1:47–52. [12] Woolf CM, Myrianthopoulos NC. Polydactyly in American negroes and whites. Am J Hum Genet 1973;4:397–404. [13] Mullick S, Borschel GH. A selective approach to treatment of ulnar polydactyly: preventing painful neuroma and incomplete excision. Pediatr Dermatol 2010;1: 39–42. [14] Hartzell TL, Taylor H. Traumatic amputation of a supernumerary digit: a 16-yearold boy's perspective of suture ligation. Pediatr Dermatol 2009;1:100–2. [15] Leber GE, Gosain AK. Surgical excision of pedunculated supernumerary digits prevents traumatic amputation neuromas. Pediatr Dermatol 2003;2:108–12. [16] Watson BT, Hennrikus WL. Postaxial type-B polydactyly. Prevalence and treatment. J Bone Joint Surg Am 1997;1:65–8.

Ulnar polydactyly - an analysis of appearance and postoperative outcome.

Ulnar polydactylies present with a wide variation in the size and appearance of the affected finger, ranging from small cutaneous appendages to fully ...
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