Congenital syndactyly: case by case analysis of 47 patients

Mandarano-Filho, Luiz Garcia; Bezuti, Márcio Takey; Akita, Rubens; Mazzer, Nilton; Barbieri, Cláudio Henrique

doi:10.1590/S1413-78522013000600007

Abstracts

OBJECTIVE: To assess and report clinical data from patients with syndactyly. METHODS: A retrospective review of 47 patients treated between April 2002 and April 2012. RESULTS: Among the 47 analyzed patients, 33 (70%) were male and 14 (30%) female. The total number of syndactylies was 116. The right hand was affected in 19 patients (40%), the left hand in 12 (24%) and 31 (36%) were bilaterally compromised. Sixteen patients (34%) also presented genetic syndromes. Among the 31 (66%) patients without syndromes, 12 (39%) had isolated syndactyly and 19 (61%) presented association with other hand anomalies. The third web space was affected 45 (39%) times; the fourth, 35 (30%) times; the second, 22 (19%) times and the first web space 14 (22%) times. Simple syndactyly was found 68 (59%) times, complete syndactyly in 44 (65%) and incomplete in 24 (55%). Complex syndactyly was found 48 (41%) times. CONCLUSION: The results in this study are similar to the literature. Epidemiological Study.

Hand deformities, congenital; Syndactyly; Child

OBJETIVO: Analisar e comparar as características dos pacientes com sindactilia congênita com os dados da literatura. MÉTODOS: Estudo retrospectivo baseado na análise do prontuário médico de 47 pacientes tratados em um período de 10 anos. RESULTADOS: Dos pacientes, 33 (70%) eram do sexo masculino e 14 (30%) do feminino. O número total de sindactilias foi de 116. A mão direita foi acometida em 19 casos (40%), a mão esquerda em 12 (24%) e em 17 (36%) o acometimento foi bilateral. Dezesseis (34%) pacientes possuíam síndromes genéticas. Entre os 31 (66%) não-sindrômicos, 12 (39%) apresentavam sindactilias isoladas e 19 (61%) associação com outras malformações da mão. O terceiro espaço foi acometido 45 (39%) vezes, seguido do quarto espaço com 35 (30%), do segundo com 22 (19%) e do primeiro com 14 (12%). As sindactilias simples foram encontradas 68 (59%) vezes, sendo completas em 44 (65%) situações e parciais em 24 (55%). As complexas foram identificadas 48 (41%) vezes. CONCLUSÃO: Os resultados obtidos na presente série são muito semelhantes aos dados encontrados na literatura. Estudo Epidemiológico.

Deformidades congênitas da mão; Sindactilia; Criança

ORIGINAL ARTICLE

Hospital das Clínicas, Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto, SP, Brazil

Correspondence

ABSTRACT

OBJECTIVE: To assess and report clinical data from patients with syndactyly.

METHODS: A retrospective review of 47 patients treated between April 2002 and April 2012.

RESULTS: Among the 47 analyzed patients, 33 (70%) were male and 14 (30%) female. The total number of syndactylies was 116. The right hand was affected in 19 patients (40%), the left hand in 12 (24%) and 31 (36%) were bilaterally compromise. Sixteen patients (34%) also presented genetic syndromes. Among the 31 (66%) patients without syndromes, 12 (39%) had isolated syndactyly and 19 (61%) presented association with other hand anomalies. The third web space was affected 45 (39%) times; the fourth, 35 (30%) times; the second, 22 (19%) times and the first web space 14 (22%) times. Simple syndactyly was found 68 (59%) times, complete syndactyly in 44 (65%) and incomplete in 24 (55%). Complex syndactyly was found 48 (41%) times.

CONCLUSION: The results in this study are similar to the literature. Epidemiological Study.

Keywords: Hand deformities, congenital. Syndactyly. Child.

INTRODUCTION

Syndactyly is a defect in the connection between two or more fingers. It is one of the most common congenital anomalies of the upper limb and occurs in approximately one in 2500 births.¹ It can occur isolated or associated with other malformations. Its cause is a failure in the differentiation of mesenchymal structures in single digits, where the longitudinal interdigital necrosis does not occur between the sixth and eighth week of intrauterine life. It is usually bilateral and symmetrical, mainly affecting males and is uncommon in blacks. It is most common between the third and fourth fingers, followed by the fourth and fifth and the second and third. Syndactyly between the first and second fingers is rare because the thumb of the hand is separated before the rest of fingers.²

The deformity is usually a result of sporadic mutations, but there are reports of autosomal dominant inheritance with variable expressivity in up to 40% of cases.³ It is classified as simple when the fusion occurs only by the skin, being subdivided into complete or incomplete depending on the extent of interconnection, and as complex as the fusion occurs also through the bone structure of the fingers, with the possibility in such cases of abnormal tendineae, vasculonervous² and ungal.⁴

Several surgical techniques have been described to correct this deformity, most of them using skin grafts, since the surface area of the fingers apart is greater than the area of the fingers united.^5-9 There is also available the classic surgical technique associated with the patchwork degreasing with subsequent healing by secondary intention of bloody areas, in order to simplify the procedure and avoid possible complications due to grafts use.

MATERIALS AND METHODS

A retrospective descriptive study based on analysis of data from medical records of patients with syndactyly treated by Discipline of Hand and Microsurgery, Hospital das Clínicas de Ribeirão Preto, Universidade de São Paulo, Brazil over a 10 years period (between April 2002 and April 2012). Secondary cases due to trauma and burns were excluded, totalizing 47 patients with congenital syndactyly. Of these, we investigated the presence of genetic syndromes, other malformations, the total number of syndactylies, gender, affected side, the affected space, symmetry, whether simple or complex and whether partial or complete. Data were tabulated and expressed in absolute and relative frequencies. This study was submitted to and approved by the Institutional Ethics Committee Research.

RESULTS

Of the 47 patients with congenital syndactyly, 33 (70%) were male and 14 (30%) female. Sixteen (34%) patients had some genetic syndrome (six cases of Apert syndrome, two cases of Down's, one of Poland's and seven different syndromes), 31 (66%) patients had no associated syndromes. In 17 (36%) patients the disease was bilateral in 12 (70%) of these cases being symmetric. The right side was affected in 19 (40%) patients and the left side in 12 (24%). (Table 1) The total number of syndactylies was 116, and the third web space was affected 45 times (39%), followed by the fourth space 35 times (30%), the second space 22 times (19%) and the first space with 14 times (12%). Simple syndactylies accounted for 59% (68) of the total, 35% of them (24) were partial and 65% (44) complete. Complex syndactylies were 41% (48) of the cases. (Table 2, Figures 1 and 2)

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Of the 31 (66%) patients without any associated syndrome, 12 (39%) had isolated syndactyly and 19 (61%) associated with other malformations. The most common malformation was acrosyndactyly in five cases; chipped hand in four patients; polissindactily, brachydactyly and several agenesis in three different situations each. Only one patient had associated congenital constriction band.

DISCUSSION

The importance of this type of case series is to compare the data in the literature with those found in practice at our service. The number of cases of congenital syndactyly in this study outperforms most publications related to this topic. Ekerot published in 1996 his experience with 11 patients and 17 syndactylies over a period of three years.⁶ Withey et al.¹⁰ presented their results on 19 syndactylies in 12 patients in 2001. Deunk et al.⁴ reported in 2003 a case series of 27 patients over 21 years. Lumenta et al.¹¹ in 2010 published their experience on 26 syndactylies in 19 patients over a period of 42 years. Greuse et al.¹² in 2001 reported the evaluation of 16 patients presenting 24 syndactylies in two years. Works that include the highest number of patients are those of Bandoh et al.⁸ in 1997 with 58 patients in nine years; D'Arcangelo et al.⁹ in 1996 with 50 cases and 122 syndactylies in nine years, and the impressive study with 681 patients in 20 years in the study of Muzaffar et al.¹³ published in 2004. In our midst Barboza et al.¹⁴ in 2006 reported experience with 13 patients in two years, and Cortez et al.¹⁵ with 72 patients in five years in 2010. (Table 3)

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Regarding gender distribution results show a predominance of cases in boys, frequent presence of bilateral and symmetric compromise, the third space being the most affected, which is in agreement with literature data. More than one third of the patients had some genetic syndrome, and in cases with no associated syndromes 61% of the cases syndactyly was accompanied by other malformations of the hand. These data show that, almost always, the treatment involves a multidisciplinary approach and with various surgical steps. Among all cases there is a slight predominance of simple syndactyly, mostly cases of easier surgical approach and better results.^16,17 The set of data collected allows further studies to assess the correlation between age at surgery, technique used and malformations and syndromes associated with the aesthetic and functional quality of the final result.

CONCLUSION

The results obtained in this case series are very similar to those found in the literature, which validates the study and leads us to a better understanding of this condition in our midst.

REFERENCES

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