Blepharophimosis syndrome is also known as ptosis and
epicanthus inversus syndrome. BPES is referred as an uncommon developmental
condition which affects the eyelids as well as ovary. Generally, there are four
types of major facial features that are present from birth such as droopy
eyelids, narrow eyes, an upward fold of skin or the inner lower eyelids with
widely set eyes. Thus, Blepharophimosis surgery is the only way to get rid of
this syndrome.
Blepharophimosis syndrome is divided into two
subdivisions such as:
1. BPES
Type1:
In medical term, this is premature ovarian
insufficiency (POI) in females. Through this, the menstrual period's frequency
eventually stops at the age of 40.
2. BPES
Type2:
This does not involve any kind of infertility. Type 2
is characterized by malformation of the eyelids. Blepharophimosis is a disorder
in which the eyelids suffer from malformation. This is categorized into four
distinct features such as:
•
Blepharophimosis
• Ptosis
• Epicanthus
inversus
• Telecanthus
Thus, combining the above features together form a
single term i.e. BPES syndrome. Therefore in this, the nose is noted to be
flat.
Symptoms of Blepharophimosis syndrome:
Some of the symptoms which you can notice in people
suffering from Blepharophimosis are categorized below:
• Broad and
low nasal bridge
• Arched
eyebrows
• Cup-shaped
ears
• Strabismus
•
Abnormalities with lacrimal drainage
• Narrowing
of the eye
• Optic disc
coloboma
• Upward
folding of the inner corners of the eye
•
Farsightedness or hyperopia
•
Nearsightedness or myopia
• Droopy
nature of the eyelids
• Lazy eye or
amblyopia
• Unclear
vision as eyelids does not open fully
Treatment of Blepharophimosis syndrome:
Proper diagnosis is required to implement the best
treatment. Diagnose procedure is based on gene testing for any kind of
abnormalities in FOXL2 gene.
Blepharophimosis Treatment is possible by a specialist
such as an endocrinologist, oculoplastic surgeon, geneticist, pediatric ophthalmologist,
gynecologists. The treatments
for Blepharophimosis syndrome include:
Surgery
In Blepharophimosis surgery the eyelids are modified
with the help of medial canthoplasty. This surgery process is used to rectify
the damages by epicanthus or Blepharophimosis as well as telecanthus. This
procedure is implemented on a child between 3-5 years.
Later, ptosis correction takes place it is a surgical
operation for correction of brow suspension.
Telecanthus is operated by reducing the medial canthal
length. After that, a tendon is combined with a transnasal wire.
For correction of epicanthus, if a fold is not
big, then Y-V canthoplasty is done. And if a fold is big enough, a double Z
canthoplasty is done.
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