Clinical features and orbital anomalies in Fraser syndrome and a review of management options
Purpose: Fraser syndrome is a rare congenital disorder comprising cryptophthalmos, syndactyly, and many times, urogenital anomalies. Herein, the authors aimed to study and report the clinical features and orbital anomalies in cases diagnosed with Fraser syndrome. Methods: The authors retrospectively...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2022-01-01
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| Series: | Indian Journal of Ophthalmology |
| Subjects: | |
| Online Access: | http://www.ijo.in/article.asp?issn=0301-4738;year=2022;volume=70;issue=7;spage=2559;epage=2563;aulast=Das |
| _version_ | 1818474415950659584 |
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| author | Deepsekhar Das Sujeeth Modaboyina Sandton Raj Sahil Agrawal Mandeep Singh Bajaj |
| author_facet | Deepsekhar Das Sujeeth Modaboyina Sandton Raj Sahil Agrawal Mandeep Singh Bajaj |
| author_sort | Deepsekhar Das |
| collection | DOAJ |
| description | Purpose: Fraser syndrome is a rare congenital disorder comprising cryptophthalmos, syndactyly, and many times, urogenital anomalies. Herein, the authors aimed to study and report the clinical features and orbital anomalies in cases diagnosed with Fraser syndrome. Methods: The authors retrospectively evaluated the records of patients with Fraser syndrome who had presented to a tertiary eye care hospital in northern India in the last 2 years (from January 2019 to December 2020). The clinical features were studied, entered in MS Excel, and the data was evaluated. Results: Data of 15 patients with Fraser syndrome were found. Majority of the patients were males and presented in the pediatric age group. Bilateral involvement was more common, and the most common variant of cryptophthalmos was abortive. Complete and medial madarosis of the eyebrows was the most common periocular finding. Complete cryptophthalmos was associated with cystic globes, whereas abortive forms had superior symblepharon. Common systemic features included syndactyly, bifid nose, and urogenital anomaly. Conclusion: Fraser syndrome is an extremely rare developmental disorder; it encompasses a wide range of ocular, periocular, and orbital anomalies, along with multiple pre-existing systemic anomalies. The treating ophthalmologist should always be careful in examining these patients. |
| first_indexed | 2024-04-14T04:36:34Z |
| format | Article |
| id | doaj.art-e4c6a50aa5a649e8abac4903dfffed1a |
| institution | Directory Open Access Journal |
| issn | 0301-4738 1998-3689 |
| language | English |
| last_indexed | 2024-04-14T04:36:34Z |
| publishDate | 2022-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Ophthalmology |
| spelling | doaj.art-e4c6a50aa5a649e8abac4903dfffed1a2022-12-22T02:11:51ZengWolters Kluwer Medknow PublicationsIndian Journal of Ophthalmology0301-47381998-36892022-01-017072559256310.4103/ijo.IJO_2627_21Clinical features and orbital anomalies in Fraser syndrome and a review of management optionsDeepsekhar DasSujeeth ModaboyinaSandton RajSahil AgrawalMandeep Singh BajajPurpose: Fraser syndrome is a rare congenital disorder comprising cryptophthalmos, syndactyly, and many times, urogenital anomalies. Herein, the authors aimed to study and report the clinical features and orbital anomalies in cases diagnosed with Fraser syndrome. Methods: The authors retrospectively evaluated the records of patients with Fraser syndrome who had presented to a tertiary eye care hospital in northern India in the last 2 years (from January 2019 to December 2020). The clinical features were studied, entered in MS Excel, and the data was evaluated. Results: Data of 15 patients with Fraser syndrome were found. Majority of the patients were males and presented in the pediatric age group. Bilateral involvement was more common, and the most common variant of cryptophthalmos was abortive. Complete and medial madarosis of the eyebrows was the most common periocular finding. Complete cryptophthalmos was associated with cystic globes, whereas abortive forms had superior symblepharon. Common systemic features included syndactyly, bifid nose, and urogenital anomaly. Conclusion: Fraser syndrome is an extremely rare developmental disorder; it encompasses a wide range of ocular, periocular, and orbital anomalies, along with multiple pre-existing systemic anomalies. The treating ophthalmologist should always be careful in examining these patients.http://www.ijo.in/article.asp?issn=0301-4738;year=2022;volume=70;issue=7;spage=2559;epage=2563;aulast=Dasabortive cryptophthalmoscomplete cryptophthalmoseyelid developmental disorderfraser syndromesyndactyly |
| spellingShingle | Deepsekhar Das Sujeeth Modaboyina Sandton Raj Sahil Agrawal Mandeep Singh Bajaj Clinical features and orbital anomalies in Fraser syndrome and a review of management options Indian Journal of Ophthalmology abortive cryptophthalmos complete cryptophthalmos eyelid developmental disorder fraser syndrome syndactyly |
| title | Clinical features and orbital anomalies in Fraser syndrome and a review of management options |
| title_full | Clinical features and orbital anomalies in Fraser syndrome and a review of management options |
| title_fullStr | Clinical features and orbital anomalies in Fraser syndrome and a review of management options |
| title_full_unstemmed | Clinical features and orbital anomalies in Fraser syndrome and a review of management options |
| title_short | Clinical features and orbital anomalies in Fraser syndrome and a review of management options |
| title_sort | clinical features and orbital anomalies in fraser syndrome and a review of management options |
| topic | abortive cryptophthalmos complete cryptophthalmos eyelid developmental disorder fraser syndrome syndactyly |
| url | http://www.ijo.in/article.asp?issn=0301-4738;year=2022;volume=70;issue=7;spage=2559;epage=2563;aulast=Das |
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