Home Print this page Email this page Small font sizeDefault font sizeIncrease font size
Users Online: 735
 
About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Advertise Login 
     


 
 Table of Contents  
IMAGES IN CLINICAL NEONATOLOGY
Year : 2013  |  Volume : 2  |  Issue : 3  |  Page : 151-152

A case of ectrodactyly in a neonate


Department of Pediatrics, PDU Medical College, Rajkot, Gujarat, India

Date of Web Publication18-Oct-2013

Correspondence Address:
Mitul B Kalathia
Department of Pediatrics, PDU Medical College, Rajkot, Gujarat
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2249-4847.120013

Rights and Permissions
  Abstract 

Ectrodactyly also known as Split hand/foot malformation is a rare limb malformation with autosomal dominant in heritance with variable penetrance, commonly known as "lobster claw hand". Usually it involves midline clefts of the hands and feet with syndactyly. We report a neonate with ectrodactyly and brief review of literature of condition.

Keywords: Ectrodactyly, split hand/foot malformation, syndactyly


How to cite this article:
Kalathia MB, Seta AA, Parmar PN. A case of ectrodactyly in a neonate. J Clin Neonatol 2013;2:151-2

How to cite this URL:
Kalathia MB, Seta AA, Parmar PN. A case of ectrodactyly in a neonate. J Clin Neonatol [serial online] 2013 [cited 2020 Jul 3];2:151-2. Available from: http://www.jcnonweb.com/text.asp?2013/2/3/151/120013


  Introduction Top


Ectrodactyly or split hand-foot malformation (SHFM) is a rare malformation involving the central rays of the hands and feet. Usually, it affects one to all four limbs randomly. It is associated with syndactyly with oligodactyly and rarely polydactyly leading to "lobster claw" appearance. Its prevalence has been reported to be about 1-9/100000. [1] The deformity is present since birth and maybe associated with other dysmorphic features and anomalies in some syndromes. Non-syndromic patients usually follow autosomal dominant inheritance with variablepenetrance. Syndromic patients have autosomal recessive or x-linked recessive inheritance. Sensorineural hearing loss is common in patient with ectrodactyly with syndromic association. It has also been observed with Aniridia and Alport syndrome. [1] Five different genetic mutations are known to be associated with SHFM. Type I, the most frequent variety, is due to a mutation on chromosome 7. [1] We report a neonate showing ectrodactyly [Figure 1] and syndactyly and polydactyly [Figure 2] of right lower limb.
Figure 1: Ectrodactyly of right lower limb

Click here to view
Figure 2: Syndactyly and polydactyly of right lower limb

Click here to view


Ectrodactyly can be treated surgically in order to improve function and appearance. Prosthetics may also be used. [2] Parents should be counseled regarding the possibility of recurrence of the disease in the future siblings and antenatal diagnosis by ultrasonography should be offered. [3]

 
  References Top

1.Jindal G, Parmar VR, Gupta VK. Ectrodactyly/split hand feet malformation. Indian J Hum Genet 2009;15:140-2.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.Pinette M, Garcia L, Wax JR, Cartin A, Blackstone J. Familial ectrodactyly. J Ultrasound Med 2006;25:1465-7.  Back to cited text no. 2
    
3.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.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2]


This article has been cited by
1 Ectrodactyly in a Chinese patient born to a mother with neuromyelitis optica spectrum disorder
Chang Yanyu,Shu Yaqing,Sun Xiaobo,Xu Chengfang,He Dan,Fang Ling,Chen chen,Hu Xueqiang,Allan Kermode,Qiu Wei
Multiple Sclerosis and Related Disorders. 2017;
[Pubmed] | [DOI]
2 Bilateral cleft foot: Radiographic and prenatal ultrasound features of two siblings with a review of literature
Mehmet Sedat Durmaz,Hakan Demirtas,Salih Hattapoglu,Taylan Kara,Cemil Göya,Mehmet Emin Adin
Medicina. 2016;
[Pubmed] | [DOI]
3 A Clinical Picture of Ectrodactyly Hand
Andrea Emilio Salvi,Angelo Chessa,Massimo Pezzoni
JCR Journal of Clinical Rheumatology. 2015; 21(3): 165
[Pubmed] | [DOI]



 

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
References
Article Figures

 Article Access Statistics
    Viewed2421    
    Printed39    
    Emailed0    
    PDF Downloaded243    
    Comments [Add]    
    Cited by others 3    

Recommend this journal