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Familial ectrodactyly: a rare report of lobster-claw in a Malay family

We describe a case of ectrodactyly in isolated non-syndromic form claw in three living generation of a Malay family which presented as autosomal dominant pattern of inheritance with sporadic occurrence. To our knowledge, this is the first report of such case in Malaysia. A three-year-old boy wit...

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Main Authors: K.A, Nur Ayuni, I, Imma Isniza, L, Rahul, Faa’iza, Naadira, A, Nur Syahirah
Format: Article
Published: Diyarbakir: Ektodermal Displazi Grubu-Türkiye 2021
Online Access:http://psasir.upm.edu.my/id/eprint/97304/
https://www.jidmr.com/journal/contents-of-jidmr-2021-vol-14-no-3/
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spelling my.upm.eprints.973042024-07-29T02:42:10Z http://psasir.upm.edu.my/id/eprint/97304/ Familial ectrodactyly: a rare report of lobster-claw in a Malay family K.A, Nur Ayuni I, Imma Isniza L, Rahul Faa’iza, Naadira A, Nur Syahirah We describe a case of ectrodactyly in isolated non-syndromic form claw in three living generation of a Malay family which presented as autosomal dominant pattern of inheritance with sporadic occurrence. To our knowledge, this is the first report of such case in Malaysia. A three-year-old boy with claw toes presented to Paediatric Orthopaedic Unit accompanied by his mother and grandfather. He is an active child with normal intellectual. Neither facial dysmorphism nor cleft lip and palate was observed. His right tibia is shorter than the left one and his ankle is totally dislocated posteriorly. His right foot has only one ray and his right ankle joint is in 100’ equinus. For his left lower limb, the leg and ankle are normal. However, the left foot is bifid with two lateral and one medial ray. He can walk on the stump of distal right ankle but with short limb gait. He prefers to walk in kneeling position as he finds it a bit faster and that he can move freely. Other systemic reviews are unremarkable. His mother was born with the similar deformity on the feet and ankle. She underwent below knee amputation at the age of 6 and has been using prosthesis since after corrective surgery failed. The grandfather has central splits in both hands and bifid femur bilaterally. Only one ray present on each foot. The prognosis for most people with ectrodactyly syndrome is very good. Various cosmetic prosthesis, amputation or reconstructive surgery might help, depending on the type of deformity and as per case basis. Multidisciplinary care would contribute significantly to improve the survival and the quality of life of a child with congenital musculoskeletal malformations such as ectrodactyly. Diyarbakir: Ektodermal Displazi Grubu-Türkiye 2021 Article PeerReviewed K.A, Nur Ayuni and I, Imma Isniza and L, Rahul and Faa’iza, Naadira and A, Nur Syahirah (2021) Familial ectrodactyly: a rare report of lobster-claw in a Malay family. Journal of International Dental and Medical Research, 14 (3). pp. 1149-1153. ISSN 1309-100X https://www.jidmr.com/journal/contents-of-jidmr-2021-vol-14-no-3/
institution Universiti Putra Malaysia
building UPM Library
collection Institutional Repository
continent Asia
country Malaysia
content_provider Universiti Putra Malaysia
content_source UPM Institutional Repository
url_provider http://psasir.upm.edu.my/
description We describe a case of ectrodactyly in isolated non-syndromic form claw in three living generation of a Malay family which presented as autosomal dominant pattern of inheritance with sporadic occurrence. To our knowledge, this is the first report of such case in Malaysia. A three-year-old boy with claw toes presented to Paediatric Orthopaedic Unit accompanied by his mother and grandfather. He is an active child with normal intellectual. Neither facial dysmorphism nor cleft lip and palate was observed. His right tibia is shorter than the left one and his ankle is totally dislocated posteriorly. His right foot has only one ray and his right ankle joint is in 100’ equinus. For his left lower limb, the leg and ankle are normal. However, the left foot is bifid with two lateral and one medial ray. He can walk on the stump of distal right ankle but with short limb gait. He prefers to walk in kneeling position as he finds it a bit faster and that he can move freely. Other systemic reviews are unremarkable. His mother was born with the similar deformity on the feet and ankle. She underwent below knee amputation at the age of 6 and has been using prosthesis since after corrective surgery failed. The grandfather has central splits in both hands and bifid femur bilaterally. Only one ray present on each foot. The prognosis for most people with ectrodactyly syndrome is very good. Various cosmetic prosthesis, amputation or reconstructive surgery might help, depending on the type of deformity and as per case basis. Multidisciplinary care would contribute significantly to improve the survival and the quality of life of a child with congenital musculoskeletal malformations such as ectrodactyly.
format Article
author K.A, Nur Ayuni
I, Imma Isniza
L, Rahul
Faa’iza, Naadira
A, Nur Syahirah
spellingShingle K.A, Nur Ayuni
I, Imma Isniza
L, Rahul
Faa’iza, Naadira
A, Nur Syahirah
Familial ectrodactyly: a rare report of lobster-claw in a Malay family
author_facet K.A, Nur Ayuni
I, Imma Isniza
L, Rahul
Faa’iza, Naadira
A, Nur Syahirah
author_sort K.A, Nur Ayuni
title Familial ectrodactyly: a rare report of lobster-claw in a Malay family
title_short Familial ectrodactyly: a rare report of lobster-claw in a Malay family
title_full Familial ectrodactyly: a rare report of lobster-claw in a Malay family
title_fullStr Familial ectrodactyly: a rare report of lobster-claw in a Malay family
title_full_unstemmed Familial ectrodactyly: a rare report of lobster-claw in a Malay family
title_sort familial ectrodactyly: a rare report of lobster-claw in a malay family
publisher Diyarbakir: Ektodermal Displazi Grubu-Türkiye
publishDate 2021
url http://psasir.upm.edu.my/id/eprint/97304/
https://www.jidmr.com/journal/contents-of-jidmr-2021-vol-14-no-3/
_version_ 1805889947666743296
score 13.211869

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