A Patient With PHACE Syndrome With Marked Ipsilateral Cerebral Atrophy
The association of posterior fossa malformation, facial cavernous hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects and eye abnormalities (PHACE syndrome) represents a rare congenital anomaly with a broad spectrum of clinical manifestations and female predominance. We herein r...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2010-04-01
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| Series: | Pediatrics and Neonatology |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S187595721060023X |
| _version_ | 1819268344278155264 |
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| author | Nan-Koong Wang Kun-Long Hung Hon-Chai Liao Yung-Ming Lin |
| author_facet | Nan-Koong Wang Kun-Long Hung Hon-Chai Liao Yung-Ming Lin |
| author_sort | Nan-Koong Wang |
| collection | DOAJ |
| description | The association of posterior fossa malformation, facial cavernous hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects and eye abnormalities (PHACE syndrome) represents a rare congenital anomaly with a broad spectrum of clinical manifestations and female predominance. We herein report on a girl who manifested the typical clinical features of PHACE syndrome, unusually associated with severe ipsilateral cerebral atrophy and hemiplegia. She received surgical aortoplasty, local steroid injection and laser therapy for the hemangioma, and intense physical therapy soon after diagnosis. The etiology of PHACE syndrome remains unclear, and its clinical spectrum is broad. The current case suggests that the spectrum of PHACE syndrome should be further expanded to include other forms of cerebral disorder. |
| first_indexed | 2024-12-23T21:31:34Z |
| format | Article |
| id | doaj.art-6cbe259dc6b34788aa2b7f1775ee83bb |
| institution | Directory Open Access Journal |
| issn | 1875-9572 |
| language | English |
| last_indexed | 2024-12-23T21:31:34Z |
| publishDate | 2010-04-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Pediatrics and Neonatology |
| spelling | doaj.art-6cbe259dc6b34788aa2b7f1775ee83bb2022-12-21T17:30:26ZengElsevierPediatrics and Neonatology1875-95722010-04-0151213013410.1016/S1875-9572(10)60023-XA Patient With PHACE Syndrome With Marked Ipsilateral Cerebral AtrophyNan-Koong WangKun-Long HungHon-Chai LiaoYung-Ming LinThe association of posterior fossa malformation, facial cavernous hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects and eye abnormalities (PHACE syndrome) represents a rare congenital anomaly with a broad spectrum of clinical manifestations and female predominance. We herein report on a girl who manifested the typical clinical features of PHACE syndrome, unusually associated with severe ipsilateral cerebral atrophy and hemiplegia. She received surgical aortoplasty, local steroid injection and laser therapy for the hemangioma, and intense physical therapy soon after diagnosis. The etiology of PHACE syndrome remains unclear, and its clinical spectrum is broad. The current case suggests that the spectrum of PHACE syndrome should be further expanded to include other forms of cerebral disorder.http://www.sciencedirect.com/science/article/pii/S187595721060023Xcerebral atrophyPHACE syndrome |
| spellingShingle | Nan-Koong Wang Kun-Long Hung Hon-Chai Liao Yung-Ming Lin A Patient With PHACE Syndrome With Marked Ipsilateral Cerebral Atrophy Pediatrics and Neonatology cerebral atrophy PHACE syndrome |
| title | A Patient With PHACE Syndrome With Marked Ipsilateral Cerebral Atrophy |
| title_full | A Patient With PHACE Syndrome With Marked Ipsilateral Cerebral Atrophy |
| title_fullStr | A Patient With PHACE Syndrome With Marked Ipsilateral Cerebral Atrophy |
| title_full_unstemmed | A Patient With PHACE Syndrome With Marked Ipsilateral Cerebral Atrophy |
| title_short | A Patient With PHACE Syndrome With Marked Ipsilateral Cerebral Atrophy |
| title_sort | patient with phace syndrome with marked ipsilateral cerebral atrophy |
| topic | cerebral atrophy PHACE syndrome |
| url | http://www.sciencedirect.com/science/article/pii/S187595721060023X |
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