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COMPLETE SIMPLE SYNDACTYLY OF ALL DIGITS WITH POLYDACTYLY : A Rare Case Report

Harish S Hosalkar*, AD Kulkarni**, MG Yagnik***,Hetal Shah****, Priti Gujar*****
*Senior Registrar; **Intern; ***Consultant Hon. HOD Paediatric Orthopedics; ****Lecturer, Department of Anaesthesia; *****Lecturer, Department of Radiology. Bai Jerbai Wadia Hospital for Children, Parel, Mumbai.

Syndactylism is the most common congenital anomaly of the hand with an incidence of 1 in every 2250 births. [1] Complete syndactyly, involving both hands and both feet is rare. We herewith report one such case of complete syndactyly with polydactyly of hand and review the literature.

INTRODUCTION

Syndactyly is webbing or fusion of two digits due to failure of separation during embryological development, usually between sixth and eighth weeks of intrauterine life. Involvement is bilateral and symmetrical in about 50 per cent of cases. It is two times more common in males as compared to females. Although syndactyly of adjacent fingers or toes is extremely common, complete syndactyly of all fingers and toes associated with polydactyly is a rare anomaly.

CASE REPORT

3 year old male child presented to our clinic with webbing of both hands and feet and an extra-digit in the right upper hand (Fig. 1). He was first born child of normal parents born out of non-consanguineous marriage. The perinatal history and family history were not contributory.

On examination there was complete simple syndactyly of all fingers and toes with webbing. Active movements were present in all digits. There was postaxial polydactyly in the right upper limb with a floating digit and active movements. Rest of the sensory system, motor system were normal. All other developmental milestones were normal and there were no congenital anomalies.

X-Ray findings revealed no evidence of bony anomalies in the webbed digits. There was absence of bone in the post-axial polydactyly.

DISCUSSION

Syndactyly is usually described as complete or incomplete and as simple or complex. [2,3] Complete means interconnection between digits extends as far as tips of involved digits. Incomplete involves more than normal interconnection. Simple syndactyly refers to abnormal interconnections formed by skin and fibrous tissue or ligaments. Complex syndactyly has abnormal digit interconnection by bone. Bilateral and symmetrical involvement is seen in 50 per cent of cases. Most common is syndactyly between long and ring fingers (57 per cent) next is between little and ring fingers (27 per cent), between long and index fingers (14 per cent) and least frequently between thumb and index finger (3 per cent). [4]

Fig 1
Fig 1

As regards inheritance, 10 to 40 per cent [3] of cases have a family history. However, current literature suggests that approximately 80 per cent of cases are sporadic. [5]

The association of syndactyly with other anomalies is exceedingly common. Common syndrome that includes syndactyly are Apertís syndrome and Polandís syndrome. It is also associated with other skeletal problems, visceral problems such as heart anomalies, skin dysplasias such as ectodermal dysplasia and focal dermal hypoplasia, oculodigital anomalies, orodigital anomalies, haemangioma, gigantism.

Management Principles include:

(a) Surgical excision of polydactyly.

(b) In case of syndactyly with multiple digit involvement.

i) Thumb - index finger syndactyly should be separated by 6 months of age.
ii) Ring - little finger syndactyly before one year of age.
iii) Broader digits are released early followed by subsequent release after a 6 month waiting period.
iv) Simultaneous release of radial and ulnar sides of a finger are contraindicated and may jeopardize viability of finger.

In conclusion, we highlight the rarity of this clinical presentation and propose that extreme variability in penetrance and gene expression being responsible for this malformation.

ACKNOWLEDGEMENT

Dr. AR Bacha Consultant Paediatric Orthopedics. Dr. AN Johari Consultant Paediatric Orthopedics. Dean Dr. RH Merchant for having permitted to carry out this analysis and presentation.

REFERENCES

    1. Temtamy SA Carpenterís Syndrome : Acrocephalopolysyndactyly : an autosomal recessive syndrome. J Pediatr 1966; 69 : 111.
    2. Buck - Gramcko D. Congenital malformations of the hand. Indications, operative treatment and results. Scand J Plast Reconstr Surg 1975; 9 : 190-98.
    3. Flatt AE. The care of congenital hand anomalies CV Mosby St Louis. 1977.
    4. Wolf CM, Wolf RM. A genetic study of syndactyly in. Utach Soc Biol 1973; 20 : 335.
    5. Kelikian H. Congenital Deformities of Hand and forearm WB Saunders Philadelphia. 1974.

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