Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case report
Abstract Background Kaposiform Hemangioendothelioma is a rare vascular neoplasm that typically presents in neonates and infancy. Most of the cases of Kaposiform Hemangioendothelioma are reported in neonates (60%) and infancy (93%), and very few cases have been reported prenatally. We describe here a...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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SpringerOpen
2023-05-01
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| Series: | The Egyptian Journal of Radiology and Nuclear Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s43055-023-01028-7 |
| _version_ | 1827952836466442240 |
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| author | Vinay Maurya Shweta Sharma Samarjeet Singh Pankaj Sharma Amul Gupta |
| author_facet | Vinay Maurya Shweta Sharma Samarjeet Singh Pankaj Sharma Amul Gupta |
| author_sort | Vinay Maurya |
| collection | DOAJ |
| description | Abstract Background Kaposiform Hemangioendothelioma is a rare vascular neoplasm that typically presents in neonates and infancy. Most of the cases of Kaposiform Hemangioendothelioma are reported in neonates (60%) and infancy (93%), and very few cases have been reported prenatally. We describe here a case which was diagnosed prenatally in the 3rd trimester with rapid growth of upper limb and appearance of non-immune hydrops. Case presentation A 25-year-old primigravida, an antenatally booked case, presented for routine third trimester level III scan at 34-week gestation. Level I scan for nasal bone and nuchal translucency at 12 weeks and level II anomaly scan at 20 weeks were normal. Level III ultrasound scan revealed increased echogenicity and hypertrophy of soft tissues of right upper limb and trunk involving both the subcutaneous and muscular compartments. Fetal middle cerebral artery Doppler, echocardiography and magnetic resonance imaging revealed fetal anemia, cardiac failure, and non-immune hydrops. These findings with hemodynamic changes and rapid evolution were suggestive of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon. Conclusion The diagnosis of Kaposiform Hemangioendothelioma should be suspected in prenatal period if there is unilateral hypertrophy of limb which has evolved rapidly over a short interval of time and shows findings suggestive of Kasabach–Merritt Phenomenon. Prenatal presentation is life threatening if it is associated with poor fetal hemodynamics. Prenatal counseling in such cases should include advice on termination of pregnancy with emphasis on neonatal management. |
| first_indexed | 2024-04-09T14:03:52Z |
| format | Article |
| id | doaj.art-eb0f8b9c7ff4493a8ed00f57e1ebbc90 |
| institution | Directory Open Access Journal |
| issn | 2090-4762 |
| language | English |
| last_indexed | 2024-04-09T14:03:52Z |
| publishDate | 2023-05-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | The Egyptian Journal of Radiology and Nuclear Medicine |
| spelling | doaj.art-eb0f8b9c7ff4493a8ed00f57e1ebbc902023-05-07T11:09:21ZengSpringerOpenThe Egyptian Journal of Radiology and Nuclear Medicine2090-47622023-05-015411510.1186/s43055-023-01028-7Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case reportVinay Maurya0Shweta Sharma1Samarjeet Singh2Pankaj Sharma3Amul Gupta4Department of Radiology Base Hospital Delhi CanttDepartment of Radiology Base Hospital Delhi CanttDepartment of Radiology Base Hospital Delhi CanttDepartment of Radiology Base Hospital Delhi CanttDepartment of Radiology Base Hospital Delhi CanttAbstract Background Kaposiform Hemangioendothelioma is a rare vascular neoplasm that typically presents in neonates and infancy. Most of the cases of Kaposiform Hemangioendothelioma are reported in neonates (60%) and infancy (93%), and very few cases have been reported prenatally. We describe here a case which was diagnosed prenatally in the 3rd trimester with rapid growth of upper limb and appearance of non-immune hydrops. Case presentation A 25-year-old primigravida, an antenatally booked case, presented for routine third trimester level III scan at 34-week gestation. Level I scan for nasal bone and nuchal translucency at 12 weeks and level II anomaly scan at 20 weeks were normal. Level III ultrasound scan revealed increased echogenicity and hypertrophy of soft tissues of right upper limb and trunk involving both the subcutaneous and muscular compartments. Fetal middle cerebral artery Doppler, echocardiography and magnetic resonance imaging revealed fetal anemia, cardiac failure, and non-immune hydrops. These findings with hemodynamic changes and rapid evolution were suggestive of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon. Conclusion The diagnosis of Kaposiform Hemangioendothelioma should be suspected in prenatal period if there is unilateral hypertrophy of limb which has evolved rapidly over a short interval of time and shows findings suggestive of Kasabach–Merritt Phenomenon. Prenatal presentation is life threatening if it is associated with poor fetal hemodynamics. Prenatal counseling in such cases should include advice on termination of pregnancy with emphasis on neonatal management.https://doi.org/10.1186/s43055-023-01028-7Kaposiform HemangioendotheliomaKasabach–Merritt phenomenonPrenatalFetalCase report |
| spellingShingle | Vinay Maurya Shweta Sharma Samarjeet Singh Pankaj Sharma Amul Gupta Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case report The Egyptian Journal of Radiology and Nuclear Medicine Kaposiform Hemangioendothelioma Kasabach–Merritt phenomenon Prenatal Fetal Case report |
| title | Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case report |
| title_full | Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case report |
| title_fullStr | Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case report |
| title_full_unstemmed | Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case report |
| title_short | Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case report |
| title_sort | prenatal detection of kaposiform hemangioendothelioma with kasabach merritt phenomenon a case report |
| topic | Kaposiform Hemangioendothelioma Kasabach–Merritt phenomenon Prenatal Fetal Case report |
| url | https://doi.org/10.1186/s43055-023-01028-7 |
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