Table of Contents  
ORIGINAL ARTICLE
Year : 2020  |  Volume : 16  |  Issue : 2  |  Page : 61-64

Ectrodactyly or lobster claw feet: our surgical experience


Sapthagiri Institute of Medical Sciences, Bangalore, India

Date of Submission02-Oct-2019
Date of Acceptance06-Oct-2020
Date of Web Publication18-Dec-2020

Correspondence Address:
Dr. Chetan Satish
67, 14th Cross,1st Block, R.T. Nagar, Bangalore-560032
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njps.njps_9_19

Rights and Permissions
  Abstract 


Introduction: Ectrodactyly, often the center of attraction due to the mystifying appearance, is a challenging surgical entity as more often the affected children seek cosmetic improvement and also want their feet to fit into normal footwear. Aims and objectives: Not much has been written in the literature of the surgical treatment of this rare entity. This article has been written to address the surgical options and treatment of this rare deformity. Results: We report a case series of five affected children treated in our institute over a period of 4 years. The cases were followed up for a period of 1 year, and there were no recurrence. Conclusion: Surgical descriptions for correction of this rare entity are few and this article aims to address this lacuna, helping in proper correction of this deformity.

Keywords: ectrodactyly, lobster claw feet, split foot


How to cite this article:
Satish C. Ectrodactyly or lobster claw feet: our surgical experience. Nigerian J Plast Surg 2020;16:61-4

How to cite this URL:
Satish C. Ectrodactyly or lobster claw feet: our surgical experience. Nigerian J Plast Surg [serial online] 2020 [cited 2021 Jun 23];16:61-4. Available from: https://www.njps.org/text.asp?2020/16/2/61/303839




  Introduction Top


Ectrodactyly or lobster claw feet is a rare but challenging entity. Due to the abnormal appearance, the affected people are often viewed with interest by others. Although the alien-like appearance of feet often can be a hindrance for walking, most of affected people do not usually have much of gait abnormalities. Their problem is more of the cosmesis of feet and that they cannot fit into normal footwear.

Ectrodactyly is characterized by a deep median cleft of the hand and/or foot with an absence of the central rays. It is associated with syndactyly of the digits, median clefts of the feet, or aplasia/hypoplasia of the phalanges or metatarsals.

The lobster claw is a rare congenital autosomal dominant defect, also known as split hand foot malformation (SHFM) or ectrodactyly. It has an incidence of one in 90,000 live births.[1] Genetic mutations are in homeobox genes DLX5 and DLX6 most commonly (DLX: distal-less-like-genes; chromosome 7q).

Dermatologically, patients with ectrodactyly-ectodermal dysplasia may present with fine hair, dermatitis or folliculitis, thickening and yellowing of all 10 fingernails and toenails, and/or hyperkeratosis of the palms and soles.[2]

Two expressions of SHFM occur, the nonsyndromic with isolated involvement of the limbs, and the other the syndromic form, with associated anomalies such as tibial aplasia, mental retardation, orofacial clefting, and deafness.


  Material and methods Top


Aims and objectives

The study is done to enlighten surgical treatment of ectrodactyly. We report a case series of five affected children treated in our institute over a period of 4 years from March 2013 to March 2017. The cases were followed up for a period of 1 year, and there were no recurrence. Two cases had bilateral foot deformities, which were corrected. The age group varied from 2 to 10 years. Three patients were males and two females in our study.

Surgical technique

All cases were done under general anesthesia after proper workup for associated congenital anomalies. All cases were done under tourniquet control.

The surgical incision is planned on the medial sides of the claw feet with z-plasty included to prevent any contracture. Any obstructing bony elements to narrow the cleft are excised.

Sometimes wedge osteotomies may be required when the metatarsals are fused.

Under c-arm guidance, Kirschner wires were applied longitudinally, which maintained stability in case wedge osteotomies were done. We also applied Kirschner wire obliquely from the digit to adjacent metatarsals, which helped in reducing the cleft space [Figure 1].
Figure 1 Intra-operative picture of kirschner wire applied obliquely

Click here to view


Soft tissue closure was then done incorporating z-plasties. All dead space was closed with deep sutures.

Plaster of paris (POP) immobilization was given to protect the soft tissue closure.

The kirschner wires were removed at 6 weeks post operation and all children were given splintage with help of an occupational therapist to maintain the narrowing of the cleft space.

Pre- and postoperative results in a 2 year old are shown in [Figure 2] and [Figure 3].
Figure 2 Pre-operative photograph in 2 year old

Click here to view
Figure 3 Post-operative photograph in 2 year old

Click here to view


Pre- and postoperative results in a 10 year old are shown in [Figure 4] and [Figure 5].
Figure 4 Pre-operative photograph in 10 year old

Click here to view
Figure 5 Post-operative photograph in 10 year old

Click here to view



  Results Top


All the five cases had good narrowing of the clefting of feet and no recurrence was seen in any of our cases. No other complications were noted in our study. We attribute these good results to the following:
  1. Removal of all obstructing elements to narrow the claw feet with wedge osteotomies wherever necessary.
  2. Proper soft tissue closure with incorporation of z-plasties.
  3. Use of Kirschner wires under c-arm guidance, both longitudinally and obliquely.
  4. Postoperative splintage to maintain narrowing for 6 months.



  Discussion Top


The aim of treatment is to achieve a balanced plantigrade feet and feet narrow enough to fit in normal footwear. This will improve both appearance and function.

Past surgical treatments have included removal of obstructing elements, osteotomies of metatarsal bones, and soft tissue closure of clefts. However, these measures often will not be able to narrow the wide webbing and often will lead to recurrence.[3]

We propose to use Kirschner wires both longitudinally and obliquely, which will help reduce the webbing and width of the feet. Also, postoperative splintage to keep the feet narrow helps to prevent recurrence.

Some authors have proposed a two-staged repair, first stage to reduce clefting with skin plasties, osteotomies, and ostectomies and second stage to correct any digit abnormalities like syndactyly and other deformities.[4]

None of the cases in our series required a second stage. However second stage surgery will help to correct difficult associated deformities of digits and associated syndactyly.[5]


  Conclusion Top


Ectrodactyly of feet, being a rare entity, often is a surgical challenge given that the deformity cannot be corrected entirely. However, narrowing the clefting helps the patient to get better function and fit into normal footwear with better cosmesis. This helps these patients a long way in leading a normal life like other children. Surgical descriptions for correction of this rare entity are few and this article aims to address this lacunae, helping in proper correction of this deformity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Ethical approval

Yes

Informed consent

Yes



 
  References Top

1.
Leon-Mateos A, Monteagudo B, Rodriguez L, Suarez I, Zulaica A. Patient with “lobster claw” hands and feet: ectrodactyly-ectodermal dysplasia-clefting syndrome. Actas Dermosifiliogr 2008;99:822-3.  Back to cited text no. 1
    
2.
Cyriac MJ, Lashpa E. Lobster-claw hand: a manifestation of EEC syndrome. Indian J Dermatol Venereol Leprol 2006;72:54-6.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Dwyer FC, Maisels DD. Lobster-claw deformities of the feet. Proc R Soc Med 1976;69:178-81.  Back to cited text no. 3
    
4.
Giorgini RJ, Capa CJ, Potter GK. Two stage surgical correction of cleftfoot. J Am Podiatr Med Assoc 1985;75:481-7.  Back to cited text no. 4
    
5.
Sumiya N, Onizuka T. Seven years’ survey of our new cleft foot repair. Plast Reconstr Surg 1980;65:447-59.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Material and methods
Results
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed1162    
    Printed42    
    Emailed0    
    PDF Downloaded57    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]