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Fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report

BACKGROUND: Fused lower limbs combined with severe urogenital malformation, also known as sirenomelia, is a rare congenital clinical syndrome. The etiology is unknown, and the outcome for the affected fetus is rather uncertain. CASE REPORT: Mrs RB, a Kuwaiti woman primigravida, married to a non-cons...

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Autores principales: Al Hadhoud, Fatemah, Kamal, Abeer H, Al Anjari, Abdulmohsen, Diejomaoh, Michael FE
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove Medical Press 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5614763/
https://www.ncbi.nlm.nih.gov/pubmed/29033614
http://dx.doi.org/10.2147/IMCRJ.S139067
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author Al Hadhoud, Fatemah
Kamal, Abeer H
Al Anjari, Abdulmohsen
Diejomaoh, Michael FE
author_facet Al Hadhoud, Fatemah
Kamal, Abeer H
Al Anjari, Abdulmohsen
Diejomaoh, Michael FE
author_sort Al Hadhoud, Fatemah
collection PubMed
description BACKGROUND: Fused lower limbs combined with severe urogenital malformation, also known as sirenomelia, is a rare congenital clinical syndrome. The etiology is unknown, and the outcome for the affected fetus is rather uncertain. CASE REPORT: Mrs RB, a Kuwaiti woman primigravida, married to a non-consanguineous husband, had uneventful antenatal care in a private health service, until she was admitted to the Maternity Hospital, Kuwait, at 31 weeks of gestation with a 3-hour history of ruptured membranes. She had a negative family history for diabetes mellitus, and her past surgical/medical/gynecological history was noncontributory. General physical examination revealed a healthy parturient with normal vital signs, clear lungs and normal heart sounds. Obstetric examination revealed a fundal height compatible with the gestational age; there was a single living fetus in breech presentation; she was not in labor. The mother was managed conservatively with antibiotics and dexamethasone injections. Labor ensued later, progressing rapidly to the second stage. Assisted breech delivery was performed, and a live baby, birth weight 1570 g and Apgar score 3/9, was delivered. The neonate had multiple congenital abnormalities, including fusion of both lower limbs, ambiguous genitalia, cloacal anomaly and tracheoesophageal fistula. The neonate was admitted to the neonatal intensive care unit, fully investigated, managed medically and surgically and eventually discharged home after a hospital stay of 123 days for further management. CONCLUSION: A case of peculiar fetal anomaly called sirenomelia, the first case in Kuwait, has been presented. Survival of such babies requires costly management with average results.
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spelling pubmed-56147632017-10-13 Fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report Al Hadhoud, Fatemah Kamal, Abeer H Al Anjari, Abdulmohsen Diejomaoh, Michael FE Int Med Case Rep J Case Report BACKGROUND: Fused lower limbs combined with severe urogenital malformation, also known as sirenomelia, is a rare congenital clinical syndrome. The etiology is unknown, and the outcome for the affected fetus is rather uncertain. CASE REPORT: Mrs RB, a Kuwaiti woman primigravida, married to a non-consanguineous husband, had uneventful antenatal care in a private health service, until she was admitted to the Maternity Hospital, Kuwait, at 31 weeks of gestation with a 3-hour history of ruptured membranes. She had a negative family history for diabetes mellitus, and her past surgical/medical/gynecological history was noncontributory. General physical examination revealed a healthy parturient with normal vital signs, clear lungs and normal heart sounds. Obstetric examination revealed a fundal height compatible with the gestational age; there was a single living fetus in breech presentation; she was not in labor. The mother was managed conservatively with antibiotics and dexamethasone injections. Labor ensued later, progressing rapidly to the second stage. Assisted breech delivery was performed, and a live baby, birth weight 1570 g and Apgar score 3/9, was delivered. The neonate had multiple congenital abnormalities, including fusion of both lower limbs, ambiguous genitalia, cloacal anomaly and tracheoesophageal fistula. The neonate was admitted to the neonatal intensive care unit, fully investigated, managed medically and surgically and eventually discharged home after a hospital stay of 123 days for further management. CONCLUSION: A case of peculiar fetal anomaly called sirenomelia, the first case in Kuwait, has been presented. Survival of such babies requires costly management with average results. Dove Medical Press 2017-09-21 /pmc/articles/PMC5614763/ /pubmed/29033614 http://dx.doi.org/10.2147/IMCRJ.S139067 Text en © 2017 Al Hadhoud et al. This work is published and licensed by Dove Medical Press Limited The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.
spellingShingle Case Report
Al Hadhoud, Fatemah
Kamal, Abeer H
Al Anjari, Abdulmohsen
Diejomaoh, Michael FE
Fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report
title Fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report
title_full Fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report
title_fullStr Fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report
title_full_unstemmed Fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report
title_short Fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report
title_sort fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5614763/
https://www.ncbi.nlm.nih.gov/pubmed/29033614
http://dx.doi.org/10.2147/IMCRJ.S139067
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