Isolated Cleft Foot: A Case Report and Review of Literature

Abstract

Cleft foot is a rare congenital anomaly (syndromic/isolated). Although there have been published reports about this anomaly, none has so far described standardized treatment guidelines. In this case report, we describe the details of operative management of an isolated cleft foot in an 8-year-old girl with a brief review of literature, keeping in mind the aims of the treatment, governed by the local culture and preferred footwear design in the Indian scenario.

Keywords

cleft foot - ectrodactyly - split foot - congenital malformation - case report

Introduction

Cleft foot is a rare congenital abnormality, manifested as the deficiency of central rays of the foot. The deficiency may range from shortening to complete absence of different rays (digits) of the foot. Split foot and ectrodactyly are the synonyms of this clinical condition and often associated with cleft hands. It was first reported in 1770 from South Africa.[1] It may be isolated or syndromic. It can be unilateral, but the bilateral variety is more common.[2] Usually, the disorder is inherited as an autosomal dominant variety, reduced penetrance, but can be autosomal recessive, sporadic, and X-linked.[3] [4] Because the condition is rare, no standard surgical technique, so far, has been described in the literature. Authors have treated this condition with the principles and techniques used to treat similar pathologies in the hand.

Case Report

An 8-year-old girl, the first child of her parents, born out of a nonconsanguineous marriage presented with a congenital cleft right foot. She faced no obvious difficulty in walking and had been using the same size of shoes on both feet. There was no history of any other surgical or medical illness at the time of presentation. Developmental milestones were normal and immunization was complete as per age. None of her family members had a similar deformity, her mother was not exposed to any teratogen during the antenatal period.

Clinical examination revealed normal left foot and hands. Her right foot had a cleft with the absence of the second and third toes with medial deviation of the fourth toe ([Fig. 1a, b]). The skiagram of the 0right foot showed the absence of the second and third toes with a hypoplastic wedge of the third metatarsal bone fused with the base of the fourth metatarsal ([Fig. 1c]).

Fig. 1 (A) Isolated cleft foot (right side) showing the absence of the second and third toes. (B) Plantar view. (C) Skiagram showing a hypoplastic wedge of the third metatarsal bone fused with the base of the fourth metatarsal.

Surgery was planned for cleft closure and recreation of near normal first web space. A zigzag skin incision was marked along the cleft margin on the medial aspect of the fourth ray and the lateral aspect of the first ray both meeting at the proximal end of the cleft ([Fig. 2a, b]). After elevation of the skin flaps, distally based periosteal flaps were harvested for the reconstruction of the intermetatarsal ligament and dissection done, till the tarsometatarsal joint ([Fig. 2c]). The flattened lateral cuneiform bone was found to restrict the medialization of the fourth ray, which was excised to allow the desired extent of medialization ([Fig. 2b]). Periosteal flaps, which were elevated from the first and fourth metatarsal were sutured by double breasting, using polypropylene 4–0 in an attempt to reconstruct the intermetatarsal ligament ([Fig. 2d]). The fifth tarsometatarsal joint capsulotomy was also done to achieve a more stable medialization ([Fig. 2e]). The metatarsals were approximated and maintained in position with two Kirschner's wires passed transversely ([Fig. 2f, g]). Wedge of excess skin was excised and closure was completed in layers ([Fig. 2f, g]). After 3 months of surgery, the appearance of the foot ([Fig. 3a, b]) was acceptable and aesthetic improvement was quite satisfactory for the family and the treating team. Minor medial deviation of the fourth and fifth toes was noticed and planned to be corrected later on. Fifteen months after the surgery, the outcome of treatment was found to be stable with minimal hypertrophy of dorsal scar ([Fig. 3d]). The deviation of the lateral digits was minimal and not at all disturbing to the child as far as walking, running, and wearing regular footwears with straps at the first web space was concerned ([Fig. 3d, e] and [Video 1]). The follow-up skiagram ([Fig. 3c]) showed the skeletal framework of the foot was well maintained. She was advised the common scar management protocol in the form of wearing compression garment and massage with silicone gel and emollients.

Fig. 2 Intraoperative steps. (A) Marking of incision along the cleft margins. (B) Wedge osteotomy to correct lateral deviation. (C) Periosteal flaps raised for reconstruction of intermetatarsal ligaments. (D) Intermetatarsal ligament reconstruction and cleft closure. (E) The fifth tarsometatarsal joint capsulotomy to correct lateral deviation of the fifth ray. (F) Wound closure and transverse intermetatarsal K-wires. (G) Plantar view after closure.

Fig. 3 (A) Dorsal and (B) plantar views of the foot at 3 months postoperatively. (C) Anteroposterior and oblique views of the skiagram of the foot at 15 months postoperatively. (D) Dorsal view of the foot at 15 months postoperatively. (E) Bilateral feet in footwear with a strap at 15 months postoperatively.

Video 1 Demonstration that the patient has adapted to walking using footwear with a strap at 15 months postoperatively.

Discussion

Cleft foot, also known as split foot or ectrodactyly, is a rare congenital anomaly with an incidence of 1 in 1,000,000 live births.[5] The embryonic component apical ectodermal ridge (AER) develops into the future limb. Any abnormality in the AER can result in malformations in the limbs. The extent and severity of the AER defect can determine the type of malformation that occurs.[6] This is the first of its type the senior author came across during 20 years of his practice in tertiary care level hospitals. It can present in an isolated form or as a part of a syndrome. Although it can be unilateral, the bilateral variety is more common.[2] There is no definitive surgical treatment described in the literature as standard. A variety of techniques have been used by different authors.[5] [7]

These conditions need corrective surgery in order to improve the function of the foot as well as the aesthetic appearance, even if the foot is well supported and fit to normal shoes. Some recommend early intervention, preferably by the age of 1 year is often advised for many other congenital anomalies, in order to avoid the psychological trauma that the child might experience. The condition is characterized by various bony as well as soft-tissue deficiencies in the forefoot. In milder varieties, the second or third rays may be hypoplastic, whereas in severe cases, the first to the fourth ray may be completely absent. However, the fifth ray is always present. Blauth and Borish have elaborately explained this disorder.[8] They reviewed the radiographic findings of 173 feet and classified the defects into six major types, with subtypes in most groups.

The goal of treatment includes closing the cleft and medializing the toes to the central axis as well as respecting the plantar arch. In extreme cases, amputation may be necessary. Surgical correction is primarily demanded for aesthetic improvement although the function of the foot improves invariably after a corrective surgery. Giorgini et al[9] and Coleman and Aronovitz[10] have suggested a staged approach to treat the cleft foot. The first stage involves reducing the foot width and the second stage involves correcting any digital abnormalities. The techniques described by various authors include the following: incorporation of different designs of skin flaps to reduce scar complications, excision of skeletal remnants, and intermetatarsal sutures to maintain the interdigital space. In our case, we planned a skin incision in a zigzag manner to prevent scar contracture. We excised the abnormal lateral cuneiform bone and reconstructed the intermetatarsal ligament to achieve all the possible goals of treatment of this anomaly.

Our case belongs to Blauth and Borisch type III for which there is a paucity of literature providing guidance on the most appropriate surgical treatment. In comparison to cleft hand, correction of cleft foot does not necessitate release and deepening of the syndactyly of the first web space. Rather we need to create a first web space of reasonable depth by closing the cleft, thereby enabling the child to put on the footwear commonly used in India with a strap passing through this space. At the same time, we must attempt to avoid scar complications and correct the abnormal deviation of the toes, in order to make it cosmetically more acceptable. We could achieve an aesthetically and functionally acceptable outcome ([Fig. 3d, e] and [Video 1]) by using this technique, with minimal deviation of the lateral toes (may be addressed later).

Conclusion

Cleft foot is such a rare congenital anomaly that so far there are no standard surgical techniques and guidelines for its treatment. The treatment aims to improve both the aesthetic appearance of the foot and its function, which is also governed to some extent by the local culture and footwear design preferred in that geographic region. So, the surgeon should be guided by wisdom and vigilance to achieve the goals set by him or her and as desired by the patient or family.

Conflict of Interest

None declared.

Authors' Contributions

S.K.R. contributed to the concept, designing, and manuscript editing. He was also a treating surgeon. M.M. contributed to the concept, design, illustrations and photographs, writing the manuscript, and final review of the manuscript. P.P. and A.R. contributed to writing the manuscript, and illustrations and photographs.

• References

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Publication History

Article published online:
27 September 2024