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| CASE REPORT |
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| Year : 2014 | Volume
: 10
| Issue : 2 | Page : 18-20 |
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Split-hand/Split-foot malformation
Namgwa J Kortor1, Williams T Yongu2, Itodo C Elachi2, Daniel D Mue2
1 Department of Accident and Emergency, Benue State University Teaching Hospital (BSUTH), Makurdi, Benue State, Nigeria
2 Department of Surgery, Benue State University Teaching Hospital (BSUTH), Makurdi, Benue State, Nigeria
| Date of Web Publication | 15-Apr-2015 |
Correspondence Address:
Namgwa J Kortor
Department of Accident and Emergency, Benue State University Teaching Hospital, Makurdi, Benue
Nigeria
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0794-9316.155181
Split-hand/split-foot malformation (SHFM), also known as ectrodactyly or lobster claw hand deformity, is a rare congenital limb malformation characterized by a median cleft of hands and/or feet due to the absence of the central rays. SHFM has a reported incidence of 1 in 90,000 live births. It may occur either as an isolated anomaly or as part of a syndrome. We present a rare case of a 9-year-old boy with SHFM who underwent surgical correction of the cleft hand, with improved cosmetic and functional outcome. Keywords: Congenital, malformation, median cleft, split hand
How to cite this article:
Kortor NJ, Yongu WT, Elachi IC, Mue DD. Split-hand/Split-foot malformation. Nigerian J Plast Surg 2014;10:18-20 |
| Introduction | |  |
Split-hand/split-foot malformation (SHFM) is a rare congenital limb malformation involving the central rays of the hand and/or foot. [1] It may present with syndactyly, median clefts of the hands and feet, and aplasia and/or hypoplasia of the phalanges, metacarpals, and metatarsals. SHFM was first described by Birch-Jensen and it occurs in 1 in 90,000 live births. [2],[3] Clinical variants of SHFM exist between individuals as well as between limbs in a single individual, and these include missing digits and varying degrees of syndactyly. [4] Two modes of expression exist for SHFM: Isolated nonsyndromic expression that is limited to just the limbs, and syndromic expression involving other anomalies. [5]
The syndromic type occurs in association with other anomalies such as cleft lip and palate, tibial aplasia, or genitourinary defects. [6],[7],[8] The isolated type is inherited as an autosomal dominant trait and usually occurs with 100% penetrance. [9],[10],[11]
We report a case of a 9-year-old boy with SHFM involving the right hand and both feet, who underwent surgical correction of the right cleft hand, with good cosmetic and functional outcome.
| Case Report | | |
The patient is a 9-year-old boy who presented at our hospital with deformity of the right hand and both feet since birth. The right hand had a cleft between the index finger and the middle finger, and syndactyly of the middle finger with the ring finger; the thumb and the little finger were normal [Figure 1]. The right foot had a deep median cleft extending proximally, involving the distal half of the foot. The second, third, and fourth toes were missing [Figure 2]. The left foot had a cleft between the second toe and the third toe, and syndactyly of the big toe with the second toe and the third toe with the fourth toe [Figure 3]. The left hand was normal. There was no other associated congenital anomaly. | Figure 3: Photograph of left foot showing combination of syndactyly and cleft foot deformity
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Systemic examination was normal and the boy exhibited normal development for his age. He is the fifth child out of five siblings. There was no history of similar deformity in the family. There was no history of consanguinity, drug intake, or exposure to radiation.
X-ray of the right hand showed cleft between the second and the third digits, with a transverse bridging bone at the level of the metacarpal heads [Figure 4]. X-ray of the right foot showed only four metatarsals and phalanges of the first and fifth digits [Figure 5]. | Figure 4: X-ray both hands showing transverse bone between second and third digits of the right hand
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The boy underwent surgical release of syndactyly of the second and third digits of the left hand and excision of the transverse bone. He also underwent syndactyly release of the left foot. However, he did not give consent for the reconstruction of the right foot as it was functionally acceptable.
There was good functionality and cosmetic appearance of the right hand, as is shown in [Figure 6].
| Discussion | | |
SHFM is a rare congenital limb anomaly. Some of the cases of SHFM are sporadic; however, familial forms exist and commonly show autosomal dominant inheritance. Our case illustrates the sporadic type of SHFM with no history of familial inheritance.
Barsky [5] described two types of cleft hand: Typical and atypical. The typical variety has a V-shaped central defect and the atypical form, usually sporadic, has a U-shaped cleft. In the typical form, the deformity is usually bilateral. It is inherited as an autosomal dominant trait with incomplete penetrance. An uncommon autosomal recessive transmission has also been reported. [3] In the less common atypical form, the deformity is unilateral (as seen in our case) and there is no evidence of familial inheritance.
Based on a study of the radiographic characteristics of 173 cleft feet, Blauth and Borisch [12] classified the deformities into the following six types, based on the number of metatarsal bones present. Type I: It is a minor deficiency, with five metatarsals that are normal; type II: There are five metatarsals but they are hypoplastic; type III: There are four metatarsals; type IV: There are three metatarsals; type V: There are two metatarsals; and type VI: There is only one metatarsal. Our own case is a type III deformity.
Treatment of SHFM is surgical and is aimed at reconstructing "basic" hands and feet, functionally and cosmetically. Counseling of parents is important with respect to the management of a child with deformities and the possibility of recurrence of the disease in future siblings, and antenatal diagnosis by ultrasonography should be offered.
| References | | |
| 1. | Ram KT, Goffman D, Ilagan J, Dar P. First-trimester diagnosis of familial split-hand/split-foot malformation. J Ultrasound Med 2009;28:1397-400.  |
| 2. | Arbués J, Galindo A, Puente JM, Vega MG, Hernández M, de la Fuente P. Typical isolated ectrodactyly of hands and feet: Early antenatal diagnosis. J Matern Fetal Neonatal Med 2005;17:299-301. |
| 3. | Manske PR, Halikis MN. Surgical classification of central deficiency according to the thumb web. J Hand Surg Am 1995;20:687-97. |
| 4. | Sharma S, Chhetri A, Singh A. Congenital cleft foot and hand. Indian Pediatr 1999;36:935-8. |
| 5. | Ockeloen CW, Cobben JM, Marcellis CL, Koolen DA. A rare complex malformation of the hand in split hand foot malformation type 3 (SHFM3). Clin Dysmorphol 2013;22:106-8. |
| 6. | Singh D, Daniel R, Verma M, Akhter Z, Beri RS. Split hand/split foot syndrome with atresia of nasolacrimal ducts and buphthalmos. Indian Pediatr 1989;26:1053-5. [ PUBMED] |
| 7. | Sharma AK, Sharin YK. Congenital cleft foot (lobster claw): An unusual association with ano-rectal malformation. Indian Pediatr 1992;29:1295-7. |
| 8. | Barboni C, Cereda A, Mariani M, Gervasini C, Ajmone P, Biondi A, et al. A new report of Cornelia de Lange syndrome associated with split hand and feet. Am J Med Genet A 2012;158A: 2953-5. |
| 9. | Duijf P, van Bokhoven H, Brunner HG. Pathogenesis of split-hand/split-foot malformation. Hum Mol Genet 2003;12:R51-60. |
| 10. | Pinette M, Garcia L, Wax JR, Cartin A, Blackstone J. Familial ectrodactyly. J Ultrasound Med 2006;25:1465-7. |
| 11. | Kang YS, Cheong HM, Moon Y, Lee IB, Kim SM, Kim HS, et al. Molecular genetic characterization of a Korean split hand/split foot malformation. Mol Cells 2004;17:397-403. |
| 12. | Blauth W, Borisch NC. Cleft feet. Proposals for a new classification based on roentgenographic morphology. Clin Orthop Relat Res 1990;41-8. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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