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Sirenomelia- A rare congenital anomaly: Case report

Sirenomelia is an extremely uncommon congenital deformity in which neonate has fused lower limbs associated with various visceral malformations including urogenital and gastrointestinal tract, pulmonary hypoplasia, and potter's facies. The prevalence is 0.8 to 1 per 100,000 newborns. About 300...

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Autores principales: Thombare, Dipanjali, Dixit, Prachi, Chavan, Ankit, Najan, Ashwini
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10506765/
https://www.ncbi.nlm.nih.gov/pubmed/37727419
http://dx.doi.org/10.4103/jehp.jehp_160_23
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author Thombare, Dipanjali
Dixit, Prachi
Chavan, Ankit
Najan, Ashwini
author_facet Thombare, Dipanjali
Dixit, Prachi
Chavan, Ankit
Najan, Ashwini
author_sort Thombare, Dipanjali
collection PubMed
description Sirenomelia is an extremely uncommon congenital deformity in which neonate has fused lower limbs associated with various visceral malformations including urogenital and gastrointestinal tract, pulmonary hypoplasia, and potter's facies. The prevalence is 0.8 to 1 per 100,000 newborns. About 300 cases were recorded till date. We describe a case of 31-year G4P1L1A2 with previous lower segment caesarean section with gestational age of 22 weeks 5 days with anomaly scan suggestive of single live intrauterine pregnancy of 21 weeks 5 days with bilateral renal agenesis with placenta previa and no demonstrable amniotic fluid pocket seen. Her pregnancy was uneventful without any medical disorder, drug history, and no congenital anomaly in the family. Termination of pregnancy done by emergency hysterotomy and delivered anomalous foetus with fused lower limb with absent toes, low set ears, absent left ear ostium, imperforate anus, and absent external genitalia. Sirenomelia is fatal congenital anomaly with unclear etiology. Early antenatal diagnosis and termination of pregnancy is the treatment.
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spelling pubmed-105067652023-09-19 Sirenomelia- A rare congenital anomaly: Case report Thombare, Dipanjali Dixit, Prachi Chavan, Ankit Najan, Ashwini J Educ Health Promot Case Report Sirenomelia is an extremely uncommon congenital deformity in which neonate has fused lower limbs associated with various visceral malformations including urogenital and gastrointestinal tract, pulmonary hypoplasia, and potter's facies. The prevalence is 0.8 to 1 per 100,000 newborns. About 300 cases were recorded till date. We describe a case of 31-year G4P1L1A2 with previous lower segment caesarean section with gestational age of 22 weeks 5 days with anomaly scan suggestive of single live intrauterine pregnancy of 21 weeks 5 days with bilateral renal agenesis with placenta previa and no demonstrable amniotic fluid pocket seen. Her pregnancy was uneventful without any medical disorder, drug history, and no congenital anomaly in the family. Termination of pregnancy done by emergency hysterotomy and delivered anomalous foetus with fused lower limb with absent toes, low set ears, absent left ear ostium, imperforate anus, and absent external genitalia. Sirenomelia is fatal congenital anomaly with unclear etiology. Early antenatal diagnosis and termination of pregnancy is the treatment. Wolters Kluwer - Medknow 2023-07-29 /pmc/articles/PMC10506765/ /pubmed/37727419 http://dx.doi.org/10.4103/jehp.jehp_160_23 Text en Copyright: © 2023 Journal of Education and Health Promotion https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Thombare, Dipanjali
Dixit, Prachi
Chavan, Ankit
Najan, Ashwini
Sirenomelia- A rare congenital anomaly: Case report
title Sirenomelia- A rare congenital anomaly: Case report
title_full Sirenomelia- A rare congenital anomaly: Case report
title_fullStr Sirenomelia- A rare congenital anomaly: Case report
title_full_unstemmed Sirenomelia- A rare congenital anomaly: Case report
title_short Sirenomelia- A rare congenital anomaly: Case report
title_sort sirenomelia- a rare congenital anomaly: case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10506765/
https://www.ncbi.nlm.nih.gov/pubmed/37727419
http://dx.doi.org/10.4103/jehp.jehp_160_23
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