Surgical Treatment of Congenital Syndactyly of the Hand

BackgroundSyndactyly of the foot is the second most common congenital foot anomaly. In East Asia, however, no large case study has been reported regarding the clinical features of isolated foot syndactyly. In this study, we report a review of 118 patients during the last 25 years.MethodsWe conducted a chart review of patients who underwent surgical correction for foot syndactyly between January 1990 and December 2014. Operations were performed with a dorsal triangular flap and a full-thickness skin graft. The demographics of included patients and their clinical features were evaluated. Surgical outcomes and complications were analyzed.ResultsAmong 118 patients with 194 webs (155 feet), 111 patients showed nonsyndromic cases and 7 patients showed syndromic cases. In 80 unilateral cases (72.1%), the second web was the most frequently involved (37.5%), followed by the fourth (30%), the first (15%), the third (15%), the first and second in combination (1.3%), and the second and third in combination (1.3%). Among 31 bilateral cases, 2 cases were asymmetric. Among the remaining 29 symmetric bilateral cases, the second web was the most frequently involved (45.2%), followed by the first (22.6%), and the fourth (6.5%). No specific postoperative complications were observed, except in the case of 1 patient (0.51%) who required a secondary operation to correct web creep.ConclusionsThis retrospective clinical study of 118 patients with both unilateral and bilateral foot syndactyly revealed that the second web was the most frequently involved. In addition, complete division and tension-free wound closure with a full-thickness skin graft of sufficient size showed good postoperative results.

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“…The revision rate of our study was very low compared with that of previous studies, which reported revision rates up to 42% [1113]. …”

Section: Discussioncontrasting

confidence: 77%

Foot Syndactyly: A Clinical and Demographic Analysis

Kim

Kwon

2016

Arch Plast Surg

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“…2 The webbing of fingers (unilateral or bilateral variably affecting digits 2 through 5) and toes (bilateral between toes 2 and 3), known as syndactyly, is an additional clinical hallmark. 1,3 Syndactyly is one of the most commonly observed congenital malformations, affecting an estimated 1 in 2000 to 2500 live births (0.04%-0.05%), with regional occurrence rates ranging between 0.06% to 0.12%. [3][4][5][6] The genetic phenomenon of mosaicism, whereby 2 genetically distinct populations of cells coexist within an individual' s somatic cells or gametes, has been observed in TS1 individuals/ families on at least 3 separate occasions.…”

mentioning

confidence: 99%

Maternal Mosaicism Confounds the Neonatal Diagnosis of Type 1 Timothy Syndrome

Dufendach¹,

Giudicessi

Boczek

et al. 2013

Pediatrics

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The presence of 2 distinct populations of somatic or germline cells within a single individual harboring different genotypes is termed mosaicism. Recent reports suggest that parental mosaicism is involved in the heritability of type 1 Timothy syndrome (TS1), an extremely rare and life-threatening multisystem disorder characterized by severe QT interval prolongation, syndactyly, and several other complications. Although full TS1 is caused by a single missense mutation in the CACNA1C-encoded cardiac calcium channel, mosaic TS1 parents can display isolated syndactyly without additional phenotypic manifestations. A newborn boy presented with syndactyly at birth. The presence of syndactyly in his mother led to a diagnosis of benign familial syndactyly. However, at 9 months of age, during his first syndactyly-corrective surgery, intraoperative electrocardiograms revealed extreme QT prolongation and 2:1 atrioventricular block. A comprehensive cardiac evaluation was performed, and both mother and child were tested genetically, confirming a clinical suspicion of TS1. Only the patient tested positive for the TS1 mutation; however, more extensive molecular testing revealed a limited presence of the mutation in maternally-derived DNA. This case illustrates the potential of parental mosaicism to confound the diagnosis of potentially life-threatening genetic diseases, such as TS1. Here, a mother with a partial TS1 phenotype and genetically confirmed mosaicism transmitted the TS1-causative mutation to her son, resulting in fully expressive TS1. Thus, a shared partial phenotype should not be dismissed as a benign or insignificant finding, but should be evaluated further to rule out the possibility of parental mosaicism concealing a potentially fatal heritable disease.

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“…The median age at operation was 8 year (range, [3][4][5][6][7][8][9][10][11][12][13][14]. The mean postoperative follow-up time was approximately 48 months and 37 months in the groups 1 and 2, respectively.…”

Section: Resultsmentioning

confidence: 99%

“…2 Once web creep occurs, it requires the original procedure again as it may cause deformity in growing bones and joints if not reoperated. 6 In our previous surgeries for syndactyly, especially in complex cases with deformities and fused bones in web space, because we were unable to keep the digits in abducted position, it was hard to achieve proper postoperative wound care. 7,8 Therefore, we failed to prevent postoperative wound complications leading to web creep in considerable amount of webs.…”

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confidence: 99%

Prevention of Web Creep Using a New Frame After Synpolydactyly Surgery

Kuru¹,

Demir²,

Maralcan³

et al. 2011

Annals of Plastic Surgery

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The main goal in syndactyly surgery is to create a normal web space; however, it is difficult to achieve the best clinical results in older and complex cases with bony fusions and joint deformities requiring osteotomies. In these complex cases, proper postoperative wound care is difficult to achieve due to the web contractures and deformities. In our report, we introduce a new technique using a frame that we called the "Hittite Sun," which helps in proper wound management to reduce wound healing problems that lead to web creep. Between 2002 and 2006, this frame was used in operations for 46 webs, and the results were compared with those of 30 operated webs in which conventional wound management technique was used. It was found that significantly lower complication rate and lesser web creep formation was seen in these patients, which were managed using the frame, compared with the cases with conventional wound care. The frame was found to be effective for prevention of severe web creep especially in complex synpolydactyly surgery.

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Surgical Treatment of Congenital Syndactyly of the Hand

Cited by 72 publications

References 27 publications

Foot Syndactyly: A Clinical and Demographic Analysis

Foot Syndactyly: A Clinical and Demographic Analysis

Maternal Mosaicism Confounds the Neonatal Diagnosis of Type 1 Timothy Syndrome

Prevention of Web Creep Using a New Frame After Synpolydactyly Surgery

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