Congenital Anomalies of the Hand—Principles of Management

Physicians who specialize in pediatric orthopedics and hand surgery frequently encounter congenital hand abnormalities, despite their relative rarity. The treating physician should be aware of the associated syndromes and malformations that may, in some cases, be fatal if not recognized and treated appropriately. Although these congenital disorders have a wide variability, their treatment principles are similar in that the physician should promote functional use and cosmesis for the hand. This article discusses syndactyly, preaxial polydactyly and post-axial polydactyly, and the hypoplastic thumb.

Key points

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Although congenital hand anomalies are relatively rare, pediatric orthopedic surgeons and hand surgeons will frequently see them during the course of clinical practice.
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The clinician should be aware of the associated malformations and conditions that may, in some cases, be fatal if not recognized and treated appropriately.
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The goals of surgery are to improve hand function and cosmesis while limiting complications that could impair function long term.
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The surgeon must balance functional, cosmetic, and cultural goals and align those goals with the proper surgical techniques in order to maximize patient and parent satisfaction following surgical intervention.
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This article discusses syndactyly, preaxial polydactyly and postaxial polydactyly, and the hypoplastic thumb.

Introduction

Congenital anomalies of the upper extremity, although less common than congenital heart disease, are noted in approximately 2 per 1000 live births. This incidence varies by country due to higher incidence of certain malformations in patients of certain ethnic backgrounds, such as polydactyly in those of African descent or amniotic bands in Japanese. Although many of these malformations lead to minor functional deficits, they can pose a concern for the parents and lead to psychological distress in children. In addition, the 1-year mortality of patients with hand malformations is 14% to 16% due to associated malformations, often involving the heart, kidneys, or tracheoesophageal complex. Boys are affected more commonly than girls by a 3:2 ratio, and mothers older than 40 years of age are twice as likely to have children with congenital hand differences as those only 10 years younger.

Malformations of the hand and forearm were classified by Swanson in 1964 and adopted by the International Federation of Societies for Surgery of the Hand ( Table 1 ). Although this classification has its use, it is generally hard to use in clinical instances, because patients may be classified into several categories at once, and it does not guide treatment or prognosis. Oberg and colleagues proposed a modified classification based on a more recent understanding of the embryology of congenital hand malformations. Using this classification, malformations are divided into malformations, deformations, and dysplasias, and then further subdivided ( Table 2 ).

Table 1

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