Sturge–Weber Syndrome with Bilateral Port-Wine Stain
Sturge–Weber syndrome is a rare congenital neurocutaneous disorder characterized by dermatological, ophthalmological, and neurological manifestations. It occurs due to abnormal persistence of embryonic vascular plexus. Here, we describe a case of four years seven months female with seizures, develop...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2022-01-01
|
| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2022/2191465 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832565872010461184 |
|---|---|
| author | Bishnu Deep Pathak Shriya Sharma Aakriti Adhikari Nabin Simkhada Bhuwan Ghimire Nirjala Aryal |
| author_facet | Bishnu Deep Pathak Shriya Sharma Aakriti Adhikari Nabin Simkhada Bhuwan Ghimire Nirjala Aryal |
| author_sort | Bishnu Deep Pathak |
| collection | DOAJ |
| description | Sturge–Weber syndrome is a rare congenital neurocutaneous disorder characterized by dermatological, ophthalmological, and neurological manifestations. It occurs due to abnormal persistence of embryonic vascular plexus. Here, we describe a case of four years seven months female with seizures, developmental delay, intellectual disability, and bilateral port-wine stain diagnosed as type I (classical) Sturge–Weber syndrome. The ophthalmological evaluation was unremarkable. Electroencephalogram showed abnormalities suggestive of a structural lesion in the right cerebral hemisphere. CT scan of the head revealed volume loss of right brain parenchyma with linear, cortical, as well as subcortical calcifications more evident in the right hemisphere. The child should be followed up regularly until adulthood for ophthalmological evaluation, recurrence of seizures, and other manifestations of this disorder. |
| format | Article |
| id | doaj-art-9502c4a45160460d9572aa8aa73a0d5f |
| institution | Kabale University |
| issn | 2090-6811 |
| language | English |
| publishDate | 2022-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-9502c4a45160460d9572aa8aa73a0d5f2025-02-03T01:06:33ZengWileyCase Reports in Pediatrics2090-68112022-01-01202210.1155/2022/2191465Sturge–Weber Syndrome with Bilateral Port-Wine StainBishnu Deep Pathak0Shriya Sharma1Aakriti Adhikari2Nabin Simkhada3Bhuwan Ghimire4Nirjala Aryal5Nepalese Army Institute of Health SciencesNepalese Army Institute of Health SciencesNepalese Army Institute of Health SciencesDepartment of Internal MedicineNepalese Army Institute of Health SciencesDepartment of PediatricsSturge–Weber syndrome is a rare congenital neurocutaneous disorder characterized by dermatological, ophthalmological, and neurological manifestations. It occurs due to abnormal persistence of embryonic vascular plexus. Here, we describe a case of four years seven months female with seizures, developmental delay, intellectual disability, and bilateral port-wine stain diagnosed as type I (classical) Sturge–Weber syndrome. The ophthalmological evaluation was unremarkable. Electroencephalogram showed abnormalities suggestive of a structural lesion in the right cerebral hemisphere. CT scan of the head revealed volume loss of right brain parenchyma with linear, cortical, as well as subcortical calcifications more evident in the right hemisphere. The child should be followed up regularly until adulthood for ophthalmological evaluation, recurrence of seizures, and other manifestations of this disorder.http://dx.doi.org/10.1155/2022/2191465 |
| spellingShingle | Bishnu Deep Pathak Shriya Sharma Aakriti Adhikari Nabin Simkhada Bhuwan Ghimire Nirjala Aryal Sturge–Weber Syndrome with Bilateral Port-Wine Stain Case Reports in Pediatrics |
| title | Sturge–Weber Syndrome with Bilateral Port-Wine Stain |
| title_full | Sturge–Weber Syndrome with Bilateral Port-Wine Stain |
| title_fullStr | Sturge–Weber Syndrome with Bilateral Port-Wine Stain |
| title_full_unstemmed | Sturge–Weber Syndrome with Bilateral Port-Wine Stain |
| title_short | Sturge–Weber Syndrome with Bilateral Port-Wine Stain |
| title_sort | sturge weber syndrome with bilateral port wine stain |
| url | http://dx.doi.org/10.1155/2022/2191465 |
| work_keys_str_mv | AT bishnudeeppathak sturgewebersyndromewithbilateralportwinestain AT shriyasharma sturgewebersyndromewithbilateralportwinestain AT aakritiadhikari sturgewebersyndromewithbilateralportwinestain AT nabinsimkhada sturgewebersyndromewithbilateralportwinestain AT bhuwanghimire sturgewebersyndromewithbilateralportwinestain AT nirjalaaryal sturgewebersyndromewithbilateralportwinestain |