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Unique case of 11-fingered polydactyly with cleft hand
Journal of Plastic, Reconstructive & Aesthetic Surgery (2011) 64, 685e687
CASE REPORT
Unique case of 11-fingered polydactyly with cleft hand Jian Zhu, Jiansong Chen*, Guoqiang Zhao, Liping Yang Children’s Hospital, School of Medicine, Zhejiang University, Hangzhou 310003, PR China Received 14 March 2010; accepted 27 July 2010
KEYWORDS Polydactyly; Cleft hand; Hand anomaly; 11 fingers
Summary Polydactyly is a common congenital extremity deformity in live births. We describe a 3-month-old boy with 11-fingered polydactyly with cleft hand, an extremely rare hand anomaly. The patient was treated with excision of the supernumerary rays and metacarpals, closure of the cleft and removal of excess adipose tissue. Follow-up, lasting 3 years, showed satisfactory functional and aesthetic results. ª 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
Polydactyly of the hand is a common congenital deformity characterised by the presence of one or more supernumerary digits and, occasionally, extra metacarpals. When non-syndromic, it is thought to occur as an autosomaldominant trait with varied gene penetrance.1 Cleft hand is defined as a failure of formation or central ray deficiency. A typical cleft hand has a V-shaped or funnel-shaped central cleft and lacks a central digit. The typical cleft hand is an autosomal-dominant condition with variable expressivity.2 A certain percentage of cases also involves new mutations, but the exact chromosomal defect in isolated cleft hand has not yet been identified. We present an extremely rare case of 11-fingered polydactyly with cleft hand. We describe the components of this anomaly and the details of our surgical approach. * Corresponding author. Zhugan Xiang 57, Hangzhou 310003, PR China. Tel.: þ (86) 571 87061007 60917. E-mail address: [email protected] (J. Chen).
Case report A 3-month-old boy was evaluated for a right upperextremity abnormality. He was the full-term product of a normal spontaneous vaginal delivery. There were no perinatal medical problems. The family history was negative for musculoskeletal deformities or any congenital anomalies. At birth, he was found to have a 12-digit right hand (the 12th digit developed necrosis and was resected before our involvement), but a complete examination revealed no other musculoskeletal, craniofacial or visceral anomalies. No obvious genetic or chromosomal anomaly was found, and he developed normally, achieving all of his developmental milestones. Examination of the right hand revealed 11 digits (Figure 1). Only the first, second and third digits could be actively flexed and extended. A cleft divided the second and third digits. The hand was broad, with an accentuated web space between the fourth and fifth digits. The
1748-6815/$ - see front matter ª 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2010.07.029
686
Figure 1 fingers.
J. Zhu et al.
A view of right hand at 3 months, showing 11
forearm had normal contours and was equal in length to the contralateral forearm. Full forearm rotation as well as full range of elbow motion was observed. The upper arm and shoulder had a normal appearance and excellent range of motion. Radiography of the hand showed that all the digits, except the first digit, had the usual triphalangeal appearance (Figure 2). There were nine metacarpals without obvious symmetry. The ulna and radius were normal in appearance and length. No accessory forearm bones were detected. The elbow joint and humerus were unremarkable.
Figure 2
Preoperative X-ray show 9 metacarpals.
Surgical correction was performed in stages. The first stage consisted of ray amputation of the 4th, 8th, 9th, 10th and 11th digits (Figure 3(A)). The digital nerves to these supernumerary fingers were transected proximally and allowed to retract into the palm. The digital arteries and veins were ligated. The second stage consisted of amputation of the seventh finger and excision of the extra metacarpals (Figure 3(B)). In the cleft-closure stage, we used a simple, random transposition flap of dorsal skin and repositioned the intrinsic muscles and skeletal parts (Figure 3(C)). After these operations, the boy’s parents
Figure 3 A: a view of hand after first stage surgery. B: X-ray of hand after second stage surgery. C: a view of hand after closure of the cleft.
Unique case of 11-fingered polydactyly with cleft hand
Figure 4
A view of hand at 3 years postoperative.
complained that the index finger and middle finger seemed too thick; hence, removal of excess adipose tissue of these two fingers was executed in the fourth stage. The boy’s recovery was uneventful in our 3 years of follow-up. The surgical scar was faint, without contraction. The boy’s parents were satisfied with the improved appearance and function of the hand (Figures 4 and 5).
Discussion Various forms of polydactyly have been reported and different classification methods described. The disorder is generally classified into three major groups: preaxial (thumb), postaxial (little finger) and central (index, middle and ring fingers). Our case of 11-fingered polydactyly with cleft hand did not fit into any of the classic descriptions and was the only such case we have ever encountered. The classic ‘mirror hand’ deformity, also known as ulnar dimelia, is characterised by the absence of the thumb with multiple fingers and two ulnas. Recently, however, several investigators have clearly shown that duplication of the ulna is not always present in this deformity and have described cases with multiple fingers and a distinct radius and ulna in the forearm.3 An essential feature of classic and non-classic mirror hands is the presence of symmetry (although not necessarily perfect) around the midline. The hand deformity, in our case, lacked this essential feature. The complexity of our hand deformity ranges from a simple soft-tissue problem to a completely developed
687
Figure 5
The function of hand at 3 years postoperative.
accessory ray. Careful clinical and radiographic evaluation is necessary before treatment to achieve good functional and cosmetic results. In this case, only three fingers had flexor and extensor tendons. Therefore, we preserved these three fingers as the thumb, index finger and middle finger in our operation. It has been recommended that surgical intervention take place between 6 and 12 months of age. This allows a good definition of the bones and soft tissues and reduces the risk of anaesthetic complications. Because of the parents’ strong desire, we performed the first operation, in this case, when the boy was only 3 months old, with subsequent operations at 3month intervals. Despite the early surgery, we did not encounter any problems either intra-operatively or postoperatively.
Conflict of interest statement None.
References 1. McCarthy G, Lindaman L, Stefan M. Pedal polydactyly: an overview with case report. J Foot Ankle Surg 1995;34:577e82. 2. Axel AF. Analysis of anatomic variations in cleft hands. J Hand Surg 2004;29:994e1001. 3. Al-Qattan MM, Al-Thunayan A, De Cordier M, et al. Classification of the mirror hand-multiple hand spectrum. J Hand Surg 1998; 23B:534e6.
CASE REPORT
Unique case of 11-fingered polydactyly with cleft hand Jian Zhu, Jiansong Chen*, Guoqiang Zhao, Liping Yang Children’s Hospital, School of Medicine, Zhejiang University, Hangzhou 310003, PR China Received 14 March 2010; accepted 27 July 2010
KEYWORDS Polydactyly; Cleft hand; Hand anomaly; 11 fingers
Summary Polydactyly is a common congenital extremity deformity in live births. We describe a 3-month-old boy with 11-fingered polydactyly with cleft hand, an extremely rare hand anomaly. The patient was treated with excision of the supernumerary rays and metacarpals, closure of the cleft and removal of excess adipose tissue. Follow-up, lasting 3 years, showed satisfactory functional and aesthetic results. ª 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
Polydactyly of the hand is a common congenital deformity characterised by the presence of one or more supernumerary digits and, occasionally, extra metacarpals. When non-syndromic, it is thought to occur as an autosomaldominant trait with varied gene penetrance.1 Cleft hand is defined as a failure of formation or central ray deficiency. A typical cleft hand has a V-shaped or funnel-shaped central cleft and lacks a central digit. The typical cleft hand is an autosomal-dominant condition with variable expressivity.2 A certain percentage of cases also involves new mutations, but the exact chromosomal defect in isolated cleft hand has not yet been identified. We present an extremely rare case of 11-fingered polydactyly with cleft hand. We describe the components of this anomaly and the details of our surgical approach. * Corresponding author. Zhugan Xiang 57, Hangzhou 310003, PR China. Tel.: þ (86) 571 87061007 60917. E-mail address: [email protected] (J. Chen).
Case report A 3-month-old boy was evaluated for a right upperextremity abnormality. He was the full-term product of a normal spontaneous vaginal delivery. There were no perinatal medical problems. The family history was negative for musculoskeletal deformities or any congenital anomalies. At birth, he was found to have a 12-digit right hand (the 12th digit developed necrosis and was resected before our involvement), but a complete examination revealed no other musculoskeletal, craniofacial or visceral anomalies. No obvious genetic or chromosomal anomaly was found, and he developed normally, achieving all of his developmental milestones. Examination of the right hand revealed 11 digits (Figure 1). Only the first, second and third digits could be actively flexed and extended. A cleft divided the second and third digits. The hand was broad, with an accentuated web space between the fourth and fifth digits. The
1748-6815/$ - see front matter ª 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2010.07.029
686
Figure 1 fingers.
J. Zhu et al.
A view of right hand at 3 months, showing 11
forearm had normal contours and was equal in length to the contralateral forearm. Full forearm rotation as well as full range of elbow motion was observed. The upper arm and shoulder had a normal appearance and excellent range of motion. Radiography of the hand showed that all the digits, except the first digit, had the usual triphalangeal appearance (Figure 2). There were nine metacarpals without obvious symmetry. The ulna and radius were normal in appearance and length. No accessory forearm bones were detected. The elbow joint and humerus were unremarkable.
Figure 2
Preoperative X-ray show 9 metacarpals.
Surgical correction was performed in stages. The first stage consisted of ray amputation of the 4th, 8th, 9th, 10th and 11th digits (Figure 3(A)). The digital nerves to these supernumerary fingers were transected proximally and allowed to retract into the palm. The digital arteries and veins were ligated. The second stage consisted of amputation of the seventh finger and excision of the extra metacarpals (Figure 3(B)). In the cleft-closure stage, we used a simple, random transposition flap of dorsal skin and repositioned the intrinsic muscles and skeletal parts (Figure 3(C)). After these operations, the boy’s parents
Figure 3 A: a view of hand after first stage surgery. B: X-ray of hand after second stage surgery. C: a view of hand after closure of the cleft.
Unique case of 11-fingered polydactyly with cleft hand
Figure 4
A view of hand at 3 years postoperative.
complained that the index finger and middle finger seemed too thick; hence, removal of excess adipose tissue of these two fingers was executed in the fourth stage. The boy’s recovery was uneventful in our 3 years of follow-up. The surgical scar was faint, without contraction. The boy’s parents were satisfied with the improved appearance and function of the hand (Figures 4 and 5).
Discussion Various forms of polydactyly have been reported and different classification methods described. The disorder is generally classified into three major groups: preaxial (thumb), postaxial (little finger) and central (index, middle and ring fingers). Our case of 11-fingered polydactyly with cleft hand did not fit into any of the classic descriptions and was the only such case we have ever encountered. The classic ‘mirror hand’ deformity, also known as ulnar dimelia, is characterised by the absence of the thumb with multiple fingers and two ulnas. Recently, however, several investigators have clearly shown that duplication of the ulna is not always present in this deformity and have described cases with multiple fingers and a distinct radius and ulna in the forearm.3 An essential feature of classic and non-classic mirror hands is the presence of symmetry (although not necessarily perfect) around the midline. The hand deformity, in our case, lacked this essential feature. The complexity of our hand deformity ranges from a simple soft-tissue problem to a completely developed
687
Figure 5
The function of hand at 3 years postoperative.
accessory ray. Careful clinical and radiographic evaluation is necessary before treatment to achieve good functional and cosmetic results. In this case, only three fingers had flexor and extensor tendons. Therefore, we preserved these three fingers as the thumb, index finger and middle finger in our operation. It has been recommended that surgical intervention take place between 6 and 12 months of age. This allows a good definition of the bones and soft tissues and reduces the risk of anaesthetic complications. Because of the parents’ strong desire, we performed the first operation, in this case, when the boy was only 3 months old, with subsequent operations at 3month intervals. Despite the early surgery, we did not encounter any problems either intra-operatively or postoperatively.
Conflict of interest statement None.
References 1. McCarthy G, Lindaman L, Stefan M. Pedal polydactyly: an overview with case report. J Foot Ankle Surg 1995;34:577e82. 2. Axel AF. Analysis of anatomic variations in cleft hands. J Hand Surg 2004;29:994e1001. 3. Al-Qattan MM, Al-Thunayan A, De Cordier M, et al. Classification of the mirror hand-multiple hand spectrum. J Hand Surg 1998; 23B:534e6.