Fetal klippel–Trenaunay–Weber syndrome: Antenatal diagnosis and postnatal management
Context: Klippel–Trenaunay–Weber syndrome (KTWS) is a rare disease characterized by a triad of venous malformations, vascular skin nevus and asymmetric hypertrophy of bone and soft tissue. The spectrum of disease in utero varies from asymptomatic nevus flammeus to life threatening complications like...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2023-01-01
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| Series: | Journal of Indian Association of Pediatric Surgeons |
| Subjects: | |
| Online Access: | http://www.jiaps.com/article.asp?issn=0971-9261;year=2023;volume=28;issue=5;spage=387;epage=391;aulast=Singh |
| _version_ | 1827779491817062400 |
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| author | Neeta Singh Ankur Mandelia Neha Nigam Sangeeta Yadav Mandakini Pradhan |
| author_facet | Neeta Singh Ankur Mandelia Neha Nigam Sangeeta Yadav Mandakini Pradhan |
| author_sort | Neeta Singh |
| collection | DOAJ |
| description | Context: Klippel–Trenaunay–Weber syndrome (KTWS) is a rare disease characterized by a triad of venous malformations, vascular skin nevus and asymmetric hypertrophy of bone and soft tissue. The spectrum of disease in utero varies from asymptomatic nevus flammeus to life threatening complications like Kasabach–Merritt phenomena.
Aim: The aim of this study was to review our experience of antenatal diagnosis of KTWS and it's postnatal management.
Settings and Design: This was a retrospective observational study of all pregnant women who were antenatally diagnosed with KTWS and postnatally confirmed at a tertiary care center in north India between 2012 and 2021.
Subjects and Methods: The electronic medical records were reviewed and data were collected regarding demographic information, obstetric history, clinical presentation, sonographic findings, mode of delivery, fetal outcome, and follow-up.
Results: During the study period, four fetuses were diagnosed with KTWS on sonography. Three women were multigravida whereas one was a primigravida. Two women opted for medical termination of pregnancy and one each had liveborn child and an intra-uterine fetal death. KTWS was confirmed in all cases. The liveborn child underwent treatment for the vascular malformation and is alive at 4 years of age.
Conclusions: This study attempts to add onto the available literature regarding the spectrum of prenatal presentations of KTWS. It emphasizes the importance of prenatal diagnosis and follow-up of the fetus/neonate. |
| first_indexed | 2024-03-11T14:50:20Z |
| format | Article |
| id | doaj.art-f5f04e7af91842709a29cad3490e4b8d |
| institution | Directory Open Access Journal |
| issn | 0971-9261 1998-3891 |
| language | English |
| last_indexed | 2024-03-11T14:50:20Z |
| publishDate | 2023-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Journal of Indian Association of Pediatric Surgeons |
| spelling | doaj.art-f5f04e7af91842709a29cad3490e4b8d2023-10-30T10:03:35ZengWolters Kluwer Medknow PublicationsJournal of Indian Association of Pediatric Surgeons0971-92611998-38912023-01-0128538739110.4103/jiaps.jiaps_170_22Fetal klippel–Trenaunay–Weber syndrome: Antenatal diagnosis and postnatal managementNeeta SinghAnkur MandeliaNeha NigamSangeeta YadavMandakini PradhanContext: Klippel–Trenaunay–Weber syndrome (KTWS) is a rare disease characterized by a triad of venous malformations, vascular skin nevus and asymmetric hypertrophy of bone and soft tissue. The spectrum of disease in utero varies from asymptomatic nevus flammeus to life threatening complications like Kasabach–Merritt phenomena. Aim: The aim of this study was to review our experience of antenatal diagnosis of KTWS and it's postnatal management. Settings and Design: This was a retrospective observational study of all pregnant women who were antenatally diagnosed with KTWS and postnatally confirmed at a tertiary care center in north India between 2012 and 2021. Subjects and Methods: The electronic medical records were reviewed and data were collected regarding demographic information, obstetric history, clinical presentation, sonographic findings, mode of delivery, fetal outcome, and follow-up. Results: During the study period, four fetuses were diagnosed with KTWS on sonography. Three women were multigravida whereas one was a primigravida. Two women opted for medical termination of pregnancy and one each had liveborn child and an intra-uterine fetal death. KTWS was confirmed in all cases. The liveborn child underwent treatment for the vascular malformation and is alive at 4 years of age. Conclusions: This study attempts to add onto the available literature regarding the spectrum of prenatal presentations of KTWS. It emphasizes the importance of prenatal diagnosis and follow-up of the fetus/neonate.http://www.jiaps.com/article.asp?issn=0971-9261;year=2023;volume=28;issue=5;spage=387;epage=391;aulast=Singhantenatal diagnosisklippel–trenaunay–weber syndromepostnatal outcomesonography |
| spellingShingle | Neeta Singh Ankur Mandelia Neha Nigam Sangeeta Yadav Mandakini Pradhan Fetal klippel–Trenaunay–Weber syndrome: Antenatal diagnosis and postnatal management Journal of Indian Association of Pediatric Surgeons antenatal diagnosis klippel–trenaunay–weber syndrome postnatal outcome sonography |
| title | Fetal klippel–Trenaunay–Weber syndrome: Antenatal diagnosis and postnatal management |
| title_full | Fetal klippel–Trenaunay–Weber syndrome: Antenatal diagnosis and postnatal management |
| title_fullStr | Fetal klippel–Trenaunay–Weber syndrome: Antenatal diagnosis and postnatal management |
| title_full_unstemmed | Fetal klippel–Trenaunay–Weber syndrome: Antenatal diagnosis and postnatal management |
| title_short | Fetal klippel–Trenaunay–Weber syndrome: Antenatal diagnosis and postnatal management |
| title_sort | fetal klippel trenaunay weber syndrome antenatal diagnosis and postnatal management |
| topic | antenatal diagnosis klippel–trenaunay–weber syndrome postnatal outcome sonography |
| url | http://www.jiaps.com/article.asp?issn=0971-9261;year=2023;volume=28;issue=5;spage=387;epage=391;aulast=Singh |
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