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OPHTHALMIC IMAGES
Year : 2021  |  Volume : 59  |  Issue : 4  |  Page : 417

Blepharophimosis-ptosis-epicanthus inversus syndrome (type 1)


1 Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Canton Health Centre, Canton, MI, USA

Date of Submission28-Apr-2020
Date of Decision20-Jul-2020
Date of Acceptance03-Mar-2021
Date of Web Publication21-Dec-2021

Correspondence Address:
Dr. Manpreet Singh
Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_45_20

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How to cite this article:
Singh M, Saini A, Kaur M, Singla B. Blepharophimosis-ptosis-epicanthus inversus syndrome (type 1). TNOA J Ophthalmic Sci Res 2021;59:417

How to cite this URL:
Singh M, Saini A, Kaur M, Singla B. Blepharophimosis-ptosis-epicanthus inversus syndrome (type 1). TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2022 Jan 25];59:417. Available from: https://www.tnoajosr.com/text.asp?2021/59/4/417/333170



A 27-year-old unmarried female had complaints of small appearance of both eyes since childhood. She had a history of menstrual irregularities. She maintained a chin up posture with prominent forehead wrinkles and eyebrow lift on the left side. Local examination showed bilateral severe upper blepharoptosis, absent eyelid crease, and poor levator excursion [Figure 1]a. Lower eyelids showed bilateral epicanthus inversus. The horizontal palpebral fissure length was 22 mm in both eyes while the intercanthal distance was 38 mm. Rest of the ophthalmic examination was unremarkable. She was diagnosed as type-1 blepharophimosis-ptosis-epicanthus inversus syndrome, secondary to associated premature ovarian failure. A two-step surgical correction was performed. Bilateral Verwey's Y-V medial canthoplasty was followed by bilateral frontalis sling surgery (silicon rod) at 6-week interval. At 4-month follow-up, the palpebral fissure length increased by 4 mm each while the intercanthal distance improved 35 mm [Figure 1]b. Genetic testing and gynecological management were advised.
Figure 1: (a) Bilateral severe upper blepharoptosis, epicanthus inversus, blepharophimosis (reduced horizontal palpebral fissure length), and mild lateral ectropion. (b) Postbilateral Verwey's Y-V medial canthoplasty and bilateral frontalis sling surgery, satisfactory correction of blepharoptosis, epicanthus, and blepharophimosis

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.




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