Bilateral cleft foot with cleft hand: a case report
Vishal Mago1 (vkk64 at yahoo dot com) #, Neetu Kochhar2
1 Department of Plastic Surgery, BPS Govt Medical College For Women, Khanpur kalan, Sonepat, Haryana, India. 2 Department of Obstetrics and gynecology, BPS Govt Medical College For Women, Khanpur kalan, Sonepat, Haryana, India
# : corresponding author
DOI
//dx.doi.org/10.13070/rs.en.1.1078
Date
2014-10-15
Cite as
Research 2014;1:1078
License
Abstract

Cleft foot and hand (split or lobster foot and hand) or partial adactyly is a rare inherited defect where there is absence of 2 or 3 central digital rays of the hand/foot. It usually occurs in association with clawing of the hand. Cleft foot deformity is an extremely rare deformity (1/90,000). This is an interesting case report of bilateral cleft feet and cleft right hand with triphalangeal left thumb. Two members of the same family are affected.

Introduction

Cleft foot (lobster foot) characterized by the absence of 2 or 3 central digital rays of the foot. One or more toes and parts of their metatarsals are absent, and often the tarsals are abnormal. Human and mouse mutations for lobster foot will reveal pical ectodermal ridge (AER) functions and the pathogenesis of this entity. Amongst the congenital anomalies, central deficiency or cleft hand is relatively rare. The association of cleft foot with cleft hand is an even rarer occurrence.

Surgery for cleft foot should improve function and appearance. Procedure of surgical correction is performed by raising dorsal and plantar flaps from the skin of the apposing surfaces, which are then sutured together. If a metatarsal has no corresponding toe, it is resected, and the cleft is closed. Any bony or joint deformity of the first or fifth ray should be corrected at the time of surgery.

Wood, Peppers, and Shook described a simplified cleft closure using rectangular flaps. Their technique was easier and produced better cosmetic results. They recommended correction of the cleft foot at 6 months old because of fewer anesthesia risks, minimal growth deformities, and malleability of the soft tissues. This type of defects runs in families [7].

Management of cleft foot depends upon the deformity. Abraham’s classification is widely used for managing cleft feet. Type I having a central ray or deficiency (usually 2nd or 3rd ray) extending upto mid-metatarsal level. This deformity is treated with soft tissue syndactylism and partial hallux valgus correction, if needed. Type II has a deep cleft up to the tarsal bones with forefoot splaying, for which soft tissue syndactylism with first-ray osteotomy is needed. Type III is a complete absence of the first through third or fourth rays, for which no surgery is required [8].

Blauth and Borisch classified the deformities into six types based on the presence of number of metatarsal bones. Type I and type II are cleft feet with minor deficiencies, both having five metatarsals. The metatarsals are normal in type I and partially hypoplastic in type II. The number of identifiable metatarsals decreases progressively: type III, four metatarsals; type IV, three metatarsals; type V, two metatarsals; and type VI, one metatarsal.

The management of cleft hand must start at an early age to achieve good functional results. The obstetricians and the pediatricians must be aware of this condition and early referral to orthopaedicians may help in advising proper use of footwear or prosthesis to achieve normal gait.

Bilateral cleft foot with cleft hand: a case report figure 1
Figure 1. Clinical photograph showing bilateral cleft foot.
Case report

A 5 year old male child was brought with a history of malformed hands and feet since birth. He was first in birth order and was the second generation to be affected in succession. There was no history of consanguinity or drug intake or exposure to radiation. There was no history of cleft lip or palate in the family.

Examination revealed syndactyly of thumb and index finger of right hand (Figure 1). The right hand had deep cleft separating thumb and index finger syndactyly with normal middle and ring fingers with a normal little finger. There was duplication of left thumb at the distal interphalangeal joint. The left thumb showed two separate nails. Feet revealed deep cleft extending proximally, involving distal one third of feet with normal great toes and normal fifth toes on the right side with similar picture on the left side (Figure 2). This patient was classified as type III (3 metatarsals are present) according to Blauth and type II based on Abraham’s classification.

Bilateral cleft foot with cleft hand: a case report figure 2
Figure 2. Clinical photograph showing syndactyly right hand.

The patient was born full-term by normal vaginal delivery. The antenatal and the peri-natal history did not reveal any significant problem. The general and systemic examination of the child was normal, except for the abnormalities in the hand and the foot. The child had not received any sort of treatment for this condition.

The radiograph of the right hand revealed absence of all phalanges of the middle finger. The gap between the third and fourth metacarpals was increased with the latter being smaller in size. A transverse bone was seen lying in the base of the cleft. The proximal phalanx of the fourth metacarpal was short and thick and formed a pseudo-joint with the cross-over bone. Radiographs of the right foot showed absence of the second and 3rd metatarsal and all phalanges of the second, third and fourth toes. Surgery with release of syndactyly has been performed to release thumb and index finger and resultant defects covered with split skin grafts. Postoperative recovery was uneventful. Conservative management was planned for foot deformity. The patient was lost to follow up so movements could not be assessed postoperatively.

Discussion

Split-hand/split-foot malformation (SHFM) is a limb malformation involving the central rays of foot or hand and presents with syndactyly, median clefts of the hands and feet, and aplasia and/or hypoplasia of the phalanges, metacarpals and metatarsals [1].

In a study of the roentgenographic characteristics of 173 cleft feet (128 from the literature), the deformities were classified into six types based on the number of metatarsals present [2]. Our case had type III deformity on the left side and type IV on the right side (based on Abrahahm and Blauth classification). The inital embryological defect is a bifid thumb; that is, two digits arisng from a common metacarpal. Resorption of one of these is a reversion to normal [4]. Adams oliver syndrome is a combination of limb malformations which include syndactyly, brachydactyly, polydactyly, oligodactyly and hypoplastic finger/toenails [5].

Cleft hand has many variations, but the absence of the central portion of the hand is the main feature of the condition. Deficiency varies from absent long phalanges to absent individual finger, to monodactyly or even the absence of all digits. The depth of the palmar cleft depends upon the remaining metacarpal bones. The principles of cleft hand surgery are good pinch and grasp, followed by good cosmesis. Surgical reconstruction involves cleft closure, syndactyly release, thumb adduction correction and removal of transverse or deforming bones. Syndactyly needs to be released first, with border digits by 6 months and central digits by 18 months. Barsky has described two types of cleft hand. One is typical cleft hand with a deep palmar cleft which separates the two central metacarpals. It is often bilateral and may involve the feet. Our case belonged to this type. Usually the middle or index ray is deficient in a V-shaped deformity and atypical is a form of sym brachydactyly [6]. Abraham’s classification helps in guiding treatment and followup of these patients [8].

Onizuka and Sumiya reported the reconstruction of five toes and found 16 cleft foot anomalies. Follow up was done from 3 years to 7 years. They advocated that this operation should be done before the age of one year due to deleterious effects of weight bearing. Salati SA reported a similar case in a 15 month old female child with bilateral deformities in hands and feet [14]. Autosomal-recessive and X-linked forms occur more rarely and other cases of Split Hand Foot Malformation are caused by chromosomal deletions and duplications [15]. Snow and Litttler described a technique of simultaneous correction. Miura and Komada technique is a technically simple method of correction of both cleft and thumb adduction.

Treatment is surgical and is directed at attainment of the features of a basic hand and feet, functionally and cosmetically . Counseling of parents is important with respect to management of deformed offspring and the possibility of recurrence of the disease in the future siblings.

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