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LETTER TO EDITOR
Year : 2021  |  Volume : 70  |  Issue : 3  |  Page : 186-187

A woman with “lobster-claw” hands – Isolated nonsyndromic ectrodactyly of both hands


1 Department of Internal Medicine, JSS Medical College and Hospital, JSSAHER, Mysore, Karnataka, India
2 Department of Pediatrics, JSS Medical College and Hospital, JSSAHER, Mysore, Karnataka, India

Date of Submission15-Feb-2021
Date of Acceptance02-Jul-2021
Date of Web Publication23-Sep-2021

Correspondence Address:
Dr. H S Kiran
Department of Internal Medicine, JSS Medical College and Hospital, JSSAHER, Mysore, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jasi.jasi_26_21

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How to cite this article:
Kiran H S, Rajani H S, Rashmi N. A woman with “lobster-claw” hands – Isolated nonsyndromic ectrodactyly of both hands. J Anat Soc India 2021;70:186-7

How to cite this URL:
Kiran H S, Rajani H S, Rashmi N. A woman with “lobster-claw” hands – Isolated nonsyndromic ectrodactyly of both hands. J Anat Soc India [serial online] 2021 [cited 2021 Dec 3];70:186-7. Available from: https://www.jasi.org.in/text.asp?2021/70/3/186/326428



A 37-year-old unmarried woman came to the outpatient department for upper respiratory tract infection (URI). She had deformities of both hands only. On further inquiry, she revealed that she had this deformity since birth, but it did not interfere with her daily routine activities or household work. She was born of nonconsanguineous marriage and was the only child of her parents. She had studied till 7th standard, she was staying with her aged parents, and she was unmarried as none of the marriage proposals went through because of the social stigma attached to the deformity. None of her grandparents and relatives (both maternal and paternal) had any similar anomalies. On examination [Figure 1], she was found to have “claw-” shaped hands with a “V-” shaped cleft in the middle of both hands (giving a “lobster-” like appearance), absence of middle finger in both hands, and an appearance suggestive of symbrachydactyly of ring finger–little finger and thumb–index finger in both hands. Both feet were normal, without any similar deformities. Systemic examination revealed no other deformities. She had scribbled circles on her hands using a ball pen while sitting in the waiting area before the consultation. Her mental status and intelligence appeared to be normal without any features of mental retardation. She was prescribed treatment for URI only as she was not willing for further investigations and workup. She was diagnosed to have isolated nonsyndromic ectrodactyly of both hands.
Figure 1: “Claw-” shaped hands with a “V-” shaped cleft in the middle of both hands (giving a “lobster-” like appearance)

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Split-hand/split-foot malformation/ectrodactyly is a congenital limb deformity in which the central rays of the autopod (distal parts of developing limbs, i.e. hands/feet) are affected resulting in syndactyly, median clefts of the hands and feet, and aplasia/or hypoplasia of the phalanges, metacarpals, and metatarsals.[1],[2] It is also called “lobster-claw” deformity as there is median cleft in the hands and feet resulting from the absence of the central digital rays, resembling a lobster.[1],[2] Ectrodactyly may be an isolated deformity or may be one of the features of a syndrome and could be either sporadic or familial.[2] It is a very rare and complex entity with varied clinical manifestations and irregular genetic transmission.[3] Our patient had isolated, sporadic, nonsyndromic ectrodactyly of both hands which is a very rare entity.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  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]  [Full text]  
2.
Duijf PH, van Bokhoven H, Brunner HG. Pathogenesis of split-hand/split-foot malformation. Hum Mol Genet 2003;12 Spec No 1:R51-60.  Back to cited text no. 2
    
3.
Umair M, Hayat A. Nonsyndromic split-hand/foot malformation: Recent classification. Mol Syndromol 2020;10:243-54.  Back to cited text no. 3
    


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