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| CASE REPORT |
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| Year : 2016 | Volume
: 12
| Issue : 2 | Page : 62-65 |
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Cleft hands and monodactylous feet: A rare variant of ectrodactyly
Emmanuel J.K. Adu, Peter Konadu
Department of Surgery, School of Medical Sciences, College of Health Sciences, Kwame Nkrumah University of Science and Technology, Kumasi, Ghana
| Date of Web Publication | 17-Mar-2017 |
Correspondence Address:
Emmanuel J.K. Adu
FWACS, FGCS, Department of Surgery, Komfo Anokye Teaching Hospital, P.O. Box 1934, Kumasi
Ghana
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0794-9316.202439
A 2-year-old male child presented with bilateral asymmetric cleft hands, a right monodactylous foot (diastatic type), posteriorly dislocated at the ankle joint, and a left monodactylous foot (Type VI, Blauth and Borisch) posteriorly dislocated at the ankle joint. Surgical management has enabled weight bearing and walking. Psychological problems, especially with the hands, are anticipated. Further multidisciplinary management by surgeons, physical therapists, clinical psychologists, child psychologists, and occupational therapists is advocated. Keywords: Cleft feet, cleft hands, ectrodactyly, monodactylous foot
How to cite this article:
Adu EJ, Konadu P. Cleft hands and monodactylous feet: A rare variant of ectrodactyly. Nigerian J Plast Surg 2016;12:62-5 |
| Introduction | |  |
Ectrodactyly is a congenital anomaly of the extremities that presents clinically as a central cleft of the hands or feet or both.[1] It results from the absence or reduction of one or more of the central digits. A typical cleft hand has a V-shaped central cleft, is often associated with syndactyly or other deformities of the digits adjacent to the cleft, and may be unilateral or bilateral.[2] The clinical problems associated with isolated cleft hand and foot are usually cosmetic and psychological rather than functional. However, in the less common variant of monodactylous ectrodactyly, or when a pre-axial anomaly exists, or when central clefts are wide and deep, function may be severely compromised.[3]
We present a 2-year-old male child with bilateral cleft hands and monodactylous feet, dislocated at the ankle joints, in whom weight bearing on the lower limbs and walking was almost impossible.
| Case presentation | | |
A 2-year-old male child presented at the reconstructive plastic surgery and burns unit at Komfo Anokye Teaching Hospital in Kumasi on account of multiple congenital anomalies of the hands, feet, and ankles and an inability to stand or walk. He was the first and only child of the mother who was a teenager. The father was a 26-year-old farmer. There was no history of congenital anomalies in the maternal or paternal relatives. The mother did not recall taking any drugs that might be teratogenic; she denied ever attempting to terminate the pregnancy by any means, and did not suffer any major febrile illness during the pregnancy. Pregnancy was hence uneventful, progressed to term, and delivery was spontaneous at a maternity clinic. At birth, the child was found to have the following:
The right hand comprised two digits: a thumb and little finger, separated by a V-shaped cleft. The three central rays were absent [Figure 1a].
The left hand comprised three digits with a V-shaped cleft between the thumb and the ring finger. The little finger was present as the most medial digit. The second and third rays were completely absent [Figure 1b].
The right foot had only one toe; the foot was dislocated posteriorly at the ankle joint with the sole orientated superiorly. The fibula was separated from the tibia at the distal end of the leg, and did not articulate with the foot at the ankle joint [Figure 2a].
The left foot had only one toe. The foot was dislocated posteriorly at the ankle joint with the sole orientated supero-medially [Figure 2b]. The radiographs of the hands, ankles, and feet are shown in [Figure 3]. | Figure 3: X-rays of the extremities showing the anomalies in the hands, feet and ankles
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The rest of the systems were normal and the patient had no other obvious congenital anomalies. Despite the poor cosmetic appearance of the hands, function was very satisfactory and the patient did not require further reconstructive surgery. Reconstruction of the ankle joints was, however, necessary to enable the patient to bear weight on the lower limbs. The right ankle joint was opened through a medial longitudinal incision. The distal tibia and fibula were freed of fibrous tissue, aligned anatomically, and fixed with Kirschner wires; the Achilles tendon was lengthened; the dislocation was then reduced, and the ankle joint arthrodesed.
The left ankle joint was similarly exposed through a medial longitudinal incision, the Achilles tendon was lengthened, the dislocation was reduced, and the left ankle joint arthrodesed [Figure 4]. | Figure 4: Post-operative pictures of the lower limbs after open reduction, internal fixation and repair
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Weight bearing on the lower limbs was commenced 4 weeks after surgery, with some difficulty initially [Figure 5]; this improved gradually, and by 8 months after surgery, the patient could walk without support.
| Discussion | | |
Development of the limbs occurs during the period of organogenesis, extending from the fourth to the eighth week of embryonic life. During this period, the developing embryo is most susceptible to the effects of teratogens.[4] A malformation syndrome refers to congenital anomalies involving multiple structural defects that cannot be explained by a single initiating cause and its consequences.[5] Known causes include chromosomal abnormalities, mutant genetic disorders, and teratogens.[6] Cleft hands may occur in isolation, but most patients present with cleft hands and cleft feet, a combination that has been designated as split hand and split foot (SH/SF) complex.[7] An abnormality in the chromosome 3q.27 region is important in the development of the central portion of the apical ectodermal ridge, and the patients with these deficiencies present with SH/SF syndromes.[8]
Blauth and Borisch[9] studied 173 cleft feet with regard to their roentgenographic morphology. They classified them into six groups based on the number of metatarsal bones: Type I had five normal metatarsals; Type II had five metatarsals, which were partially hypoplastic; Type III had four metatarsals; Type IV had three metatarsals; and Type V had two metatarsals. Type VI represents the monodactylous cleft foot. Two additional groups were found: cleft feet with central polydactyly (polydactylous type) and monodactylous feet with lower-leg diastasis or tibial aplasia or both (the diastatic type).[9] It is apparent that our patient had the diastatic type on the right and Type VI on the left. It is also apparent from this classification that the higher the group is on this scale, the more severe the functional impairment, culminating in the crippling of our patient. Milder functional limitations include difficulty in obtaining adequately fitting shoes. This problem can be solved by excision of anomalous bones, osteotomies for correction of major deviation, and partial cleft closure to release symptomatic pressure points and facilitate shoe wear.[10]
From the distribution of the syndactylies, synostoses, and aplasia, Blauth and Borisch[9] concluded that cleft formation begins at the second or third ray and proceeds longitudinally from distal to proximal, as well as in a transverse direction from tibial to fibular while the first ray remains intact. The first ray is absent only in the monodactylous cleft foot, which contains only the fifth ray [Figure 3].[9] Psychological problems are more common with cleft hands than with cleft feet.[11] These begin in mid-childhood as reluctance to use the hands. The patient may even attempt to hide the hands from public view. Nonverbal communication may be affected, and common important gestures such as hand shaking are often avoided.[12] It is clear that surgery and physical therapy alone would be insufficient in addressing these problems. The patient would require informed supportive counseling, which can be better provided by clinical psychologists, child psychologists, and occupational therapists as the child grows.
| Conclusion | | |
The management of this 2-year-old male child with a severe form of ectrodactyly would require a multidisciplinary approach to achieve optimal functional, cosmetic, and psychological result.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Warkany J. Syndromes. Am J Dis Child 1971;121:365.  |
| 2. | Sandzen SJ. Classification and functional management of congenital central defect of the hand. Hand Clin 1985;1:483–98. |
| 3. | Beaty JH, Terry Canale S. Congenital anomalies of the lower extremity. In: Canale ST, editor. Campbell’s Operative Orthopedics. 9th ed. St Louis: Mosby-Year Book Inc.; 1998. p. 928–30. |
| 4. | O’Rahilly R, Gardner E. The timing and sequence of events in the development of the limbs in the human embryo. Anat Embryol (Berl) 1975;148:1–23. |
| 5. | Jones K. Smith’s Recognizable Patterns of Human Malformation. Philadelphia: WB Saunders; 1988. p. 673. |
| 6. | Smith D. Recognizable Patterns of Human Malformation. Philadelphia: WB Saunders; 1982. p. 1–9. |
| 7. | Zguricas J, Bakker NF, Heus H, Lindhout D, Heutink P, Hovius SE. Genetics of limb development and congenital hand malformations. Plast Reconstr Surg 1998;101:1126–35. |
| 8. | Ianakiev P, Kilpatrick MW, Toudjarska I, Basel D, Beighton P, Tsipouras P. Split-hand/split-foot malformation is caused by mutations in the p63 gene on 3q27. Am J Hum Genet 2000;67:59–66. |
| 9. | Blauth W, Borisch NC. Cleft feet. Proposals for a new classification based on roentgenographic morphology. Curr Orthop Relat Res 1990;258:41–8. |
| 10. | Upton III J. Management of transverse and longitudinal deficiencies (failure of formation). In: Mathes SJ, Hentz VR, editors. Plastic Surgery VIII, The Hand and Upper Limb, Part 2. Philadelphia: Saunders Elsevier; 2006. p. 51–137. |
| 11. | Choudry Q, Kumar R, Turner PG. Congenital cleft foot deformity. Foot Ankle Surg 2010;16:e85–7. |
| 12. | Buss PW. Cleft hand/foot: Clinical and developmental aspects. J Med Genet 1994;31:726–30. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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