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Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy

AIM: This is to report a case of Klippel Trenauay Weber syndrome in a fifteen year old Nigerian boy. This is a rare syndrome and it is the first case to be reported in UCTH Calabar. CASE PRESENTATION: Product of a full term uneventful pregnancy, delivered to non-consanguineous apparently healthy par...

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Autores principales: Ikpeme, Anthonia Asanye, Usang, Usang Edet, Inyang, Akan Wilson, Ani, Nchiewe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Institute of Immunobiology and Human Genetics 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4877876/
https://www.ncbi.nlm.nih.gov/pubmed/27275244
http://dx.doi.org/10.3889/oamjms.2015.036
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author Ikpeme, Anthonia Asanye
Usang, Usang Edet
Inyang, Akan Wilson
Ani, Nchiewe
author_facet Ikpeme, Anthonia Asanye
Usang, Usang Edet
Inyang, Akan Wilson
Ani, Nchiewe
author_sort Ikpeme, Anthonia Asanye
collection PubMed
description AIM: This is to report a case of Klippel Trenauay Weber syndrome in a fifteen year old Nigerian boy. This is a rare syndrome and it is the first case to be reported in UCTH Calabar. CASE PRESENTATION: Product of a full term uneventful pregnancy, delivered to non-consanguineous apparently healthy parents. At birth was noted to gradually develop swelling on the right leg, worse at the right foot. There was crossed hemi-hypertrophy with right leg bigger than the left. As child grew symptoms worsened, parents separated and eventually he was abandoned to the streets. He presented at University of Calabar Teaching Hospital for medical care at the age of fifteen years with lymphatic obstruction, persistent foul smelling drainage, lipodermatosclerosis of right foot as well psycho-social and financial constraints. The diagnosis was made with x-rays and Doppler studies of the lower limb vessels. He is currently being managed conservatively with compression dressings on the affected limbs, Antibiotics for the infection and analgesics. De-bulking surgery is being anticipated at this time. CONCLUSION: This is a case of KTWS presenting in adolescence and due to its rarity in Nigeria, this report is to increase awareness.
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spelling pubmed-48778762016-06-06 Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy Ikpeme, Anthonia Asanye Usang, Usang Edet Inyang, Akan Wilson Ani, Nchiewe Open Access Maced J Med Sci Case Report AIM: This is to report a case of Klippel Trenauay Weber syndrome in a fifteen year old Nigerian boy. This is a rare syndrome and it is the first case to be reported in UCTH Calabar. CASE PRESENTATION: Product of a full term uneventful pregnancy, delivered to non-consanguineous apparently healthy parents. At birth was noted to gradually develop swelling on the right leg, worse at the right foot. There was crossed hemi-hypertrophy with right leg bigger than the left. As child grew symptoms worsened, parents separated and eventually he was abandoned to the streets. He presented at University of Calabar Teaching Hospital for medical care at the age of fifteen years with lymphatic obstruction, persistent foul smelling drainage, lipodermatosclerosis of right foot as well psycho-social and financial constraints. The diagnosis was made with x-rays and Doppler studies of the lower limb vessels. He is currently being managed conservatively with compression dressings on the affected limbs, Antibiotics for the infection and analgesics. De-bulking surgery is being anticipated at this time. CONCLUSION: This is a case of KTWS presenting in adolescence and due to its rarity in Nigeria, this report is to increase awareness. Institute of Immunobiology and Human Genetics 2015-06-15 2015-03-28 /pmc/articles/PMC4877876/ /pubmed/27275244 http://dx.doi.org/10.3889/oamjms.2015.036 Text en Copyright: © 2015 Anthonia Asanye Ikpeme, Usang Edet Usang, Akan Wilson Inyang, Nchiewe Ani. http://creativecommons.org/licenses/by/2.5/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Case Report
Ikpeme, Anthonia Asanye
Usang, Usang Edet
Inyang, Akan Wilson
Ani, Nchiewe
Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy
title Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy
title_full Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy
title_fullStr Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy
title_full_unstemmed Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy
title_short Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy
title_sort klippel trenaunay syndrome: a case report in an adolescent nigerian boy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4877876/
https://www.ncbi.nlm.nih.gov/pubmed/27275244
http://dx.doi.org/10.3889/oamjms.2015.036
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