|Year : 2021 | Volume
| Issue : 4 | Page : 417
Blepharophimosis-ptosis-epicanthus inversus syndrome (type 1)
Manpreet Singh1, Arshiya Saini1, Manpreet Kaur1, Bhavna Singla2
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 Submission||28-Apr-2020|
|Date of Decision||20-Jul-2020|
|Date of Acceptance||03-Mar-2021|
|Date of Web Publication||21-Dec-2021|
Dr. Manpreet Singh
Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh
Source of Support: None, Conflict of Interest: None
|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
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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