Outcome
of Y to V Medial Canthoplasty With Medial Canthal Tendon Plication for the
Correction
of
Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome
Molham
A Elbakary
Molham A Elbakary, Oculoplasty Unit, Department of
Ophthalmology, Faculty of Medicine, Tanta University. Egypt
Correspondence to: Molham A Elbakary, MD, Oculoplasty Unit,
Department of Ophthalmology, Faculty of Medicine, Tanta University. Egypt.
Email: melbakary75@yahoo.com
Telephone: +201148282205
Fax: +20403407734
Received: March 22,
2015
Revised: May 12, 2015
Accepted: May 16, 2015
Published online: June 1, 2015
ABSTRACT
AIM: Evaluation of the results of Y to V
medial canthoplasty with medial canthal tendon (MCT) plication for the
correction of Blepharophimosis-Ptosis-Epicanthus inversus Syndrome (BPES).
MATERIALS AND
METHODS: Prospective
interventional case series which included 15 cases of BPES. Two stages
management was used in all cases. The first stage was Y to V medial
canthoplasty with MCT plication. 3 months later, ptosis was corrected by
bilateral frontalis suspension or maximum levator resection in the more ptotic eyelid
in patients with asymmetric ptosis. Data regarding margin reflex distance 1
(MRD1), horizontal palpebral fissure length (HPFL), inner intercanthal distance
(IICD), and the ratio of IICD to HPFL were recorded before and after surgery
and were statistically analyzed. Post operative IICD/HPFL ratio of 1.5 or less
was considered successful outcome.
RESULTS: The mean age of patients was 3.7¡À0.8
years. According to the postoperative IICD/HPFL, 12 patients (80%) had
successful outcome. The preoperative IICD had a mean of 37.6¡À1.5 mm, which was
reduced post operative to a mean of 33.0¡À2.3 mm. The HPFL improved from
preoperative mean 21¡À1.2 mm to postoperative mean 24.2¡À1.4 mm. The mean
preoperative IICD/HPFL ratio was 1.8¡À0.2 which was reduced post operative to a
mean of 1.37¡À0.2. The MRD1 improved from preoperative mean of 1¡À0.4 mm to
3.6¡À0.3 mm post operative. The main complication recorded was undercorrection
which was observed in 3 patients (20%).
CONCLUSION: The two stages management using
simple procedures as Y to V medial canthoplasty with MCT plication followed by
frontalis suspension or maximum levator resection yielded satisfactory results
in the correction of complicated deformity as BPES.
© 2015 ACT. All
rights reserved.
Key words: Blepharophimosis; Medial canthoplasty; MCT plication
Elbakary MA. Outcome of Y to
V Medial Canthoplasty With Medial Canthal Tendon Plication for the Correction
of Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome. International
Journal of Ophthalmic Research 2015; 1(1): 28-31 Available from: URL:
http://www.ghrnet.org/index.php/ijor/article/view/1148
INTRODUCTION
Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is an
uncommon congenital disorder characterized by a narrowed horizontal palpebral
aperture, ptosis, epicanthus inversus, and telecanthus. It typically has
bilateral features, although not always symmetrical[1,2]. This
syndrome is inherited as an autosomal disease, but also can be manifested as a
result of new genetic mutations and sporadic cases without family history of
the disease can occur. Several studies confirmed the presence of mutations such
as deletion or translocation of the FOXL2 gene, which maps to chromosome
3q21-24[3,4]. Surgical treatment for BPES is one of the most complex
in the field of eyelid surgery due to the presence of multiple deformities.
Many surgical techniques have been described to address the epicanthus inversus
and accompanying telecanthus including Y to V flaps, the Mustarde technique,
the 5-flap technique, medial canthal tendon shortening or plication, and
transnasal wiring, and none of them is free from criticism[5-9].
Some authors recommended that medial canthoplasty should be performed first
followed by ptosis correction, while others recommended one stage. Therefore,
there is no unanimous consensus on BPES correction with respect to either
staging or choice of surgical techniques[9-13].
This study was
conducted to evaluate the outcome of 2 stages management of BPES using Y to V
medial canthoplasty with medial canthal tendon plication followed by frontalis
suspension or maximum levator resection.
methods
This prospective interventional case series included 15 patients with
BPES who underwent corrective surgeries at Oculoplasty unit, Tanta University
Eye Hospital. The study adhered to the principles of the declaration of
Helsinki, and was approved by the institutional ethical committee. All patients
underwent a full ophthalmological evaluation that included visual acuity,
ocular motility, slit lamp examination when possible and fundus examination.
Margin reflex distance 1 (MRD1) was measured as the distance between the
corneal light reflex and upper lid margin centrally in the primary position,
horizontal palpebral fissure length (HPFL) was measured from the precise point
of contact of the upper with the lower lid medially to the lateral canthus,
inner intercanthal distance (IICD) was measured from the precise point of
contact of the upper with the lower lid on both sides, and the ratio of IICD to
HPFL was calculated. All these measurements were recorded before and after
surgery and were statistically analyzed. The first stage was to perform medial
canthoplasty using the Y to V technique with medial canthal tendon placation.
All procedures were performed under general anesthesia. A Y was marked on the
medial canthal skin with the base of the stem in the desired new position of
the medial canthus, which was a point half the distance from the pupil center
to the midpoint of the nasal bridge. The other point of the stem was marked
just nasal to the medial canthus. The incisions were made with a no 15
Bard-Parker blade, and the flaps were undermined with scissors to expose the
medial canthal tendon (MCT) (Figure 1). Any dense fibrous tissue or excessive
orbicularis muscle between skin and the MCT were removed. The MCT was shortened
via plication using 5-0 prolene sutures. The sutures were fixed to the
periosteum posterior to the original insertion of the tendon on the frontal
process of the maxilla. The subcutaneous tissue of the medial canthus was fixed
to the MCT with a 6-0 vicryl suture. The incision was closed with 6-0 prolene
sutures, which were removed 7-10 days post operative.
The correction
of ptosis was done 3 months later. Fox pentagon frontalis suspension using
athebond 3-0 sutures was used in the majority of cases. Maximum levator
resection was used in patients with asymmetric ptosis requiring unilateral
surgery.
To compare the
preoperative and post operative measurements of HPFL, IICD, IICD/HPFL ratio and
MRD1, t-test for paired variables was used. A P less than 0.05 level of
probability was considered statistically significant. The ratio between IICD
and HPFL normally ranges between 1.0 and 1.2, so post operative result less
than 1.3 was considered good. Post operative IICD/HPFL ratio of 1.3-1.5 was
considered acceptable, while ratio more than 1.5 was considered poor
correction. 3 months after the medial canthoplasty, the parents were asked to
evaluate the medial canthal scar on a 3 grades scale, where grade 1 referred to
minimally visible scar, grade 2 moderately visible but not annoying scar, and
grade 3 which referred to markedly visible disfiguring scar.
RESULTS
The study included 15 patients. Nine were females (60%) and 6 were
(40%) males. The age of the patients ranged between 3 and 5 years with a mean
3.7¡À0.8 years. All the procedures were done under general anesthesia. The first
stage was Y to V medial canthoplasty with medial canthal tendon plication. Two
patients (13.3%) had moderate ptosis and didn't prefer to undergo 2nd stage
surgery for ptosis correction. Three (20%) patients had asymmetric ptosis and
were seeking for unilateral ptosis correction in the side with more severe
ptosis, which was done by maximum levator resection. In the remaining 10
patients, bilateral frontalis suspension was used for ptosis correction.
All eyelid
measurements showed post operative significant improvement. The preoperative
IICD ranged between 36 and 40 mm with a mean of 37.6¡À1.5 mm, which was reduced
post operative to range from 30 to 35 mm with a mean of 33.0¡À2.3 mm (P= 0.012).
The HPFL improved from preoperative mean 21¡À1.2 mm (range 19.0 to 22.0 mm) to
post operative mean 24.2¡À1.4 mm (range 22.5 to 26.0 mm) (P= 0.003). The
range of the preoperative IICD/HPFL ratio was 1.6 to 2.0 with a mean of
1.8¡À0.2. This ratio improved post operative to a mean of 1.37¡À0.2 (range 1.15
to 1.6) (P= 0.021). According to the postoperative IICD/HPFL, 12
patients (80%) had successful outcome. Five patients (33.3%) had good results
with postoperative IICD/HPFL less than 1.3, while 7 patients (46.7%) had
acceptable results with postoperative ratio 1.3-1.5 (fig 2). Lastly, the MRD1
in ptosis operated patients improved from pre operative mean of 1¡À0.4 mm (range
0.5 to 1.5 mm) to 3.6¡À0.3 mm post operative (range 3.0 to 4.0 mm).
The main
complication recorded was undercorrection which was observed in 3 patients
(20%) who had post operative IICD/HPFL ratio greater than 1.5. Although this
ratio showed post operative improvement in these patients, it was still away
from the normal range. Two patients (13.3%) showed marked visible scarring in
the medial canthal region. Other complications as infection, granuloma
formation, or lacrimal passages injuries were not recorded.
Discussion
BPES is an uncommon condition hence it is difficult to study the
surgical outcome on large numbers of patients. Patients with BPES undergo
multiple periocular operations mostly during childhood. Occasionally these are
performed urgently to preserve visual functions, but most are an endeavour to
improve patient's facial features[10]. Correction of BPES is
difficult and controversial due to the complexity of the defects, the large
number of different techniques employed, and the possibility of using a one
stage or a multiple stage procedure[9]. Many surgeons believe that
the epicanthus inversus and telecanthus should be addressed before ptosis
surgery because the tension created by the respective surgeries opposes each
other. When ptosis correction surgery and medial canthoplasty are performed at
the same time, the vertical and horizontal lengths pull against each other. The
patients are at high risk for insufficiency or loosening of the medial
canthoplasty and poor elevation of the upper eyelid. Therefore, it was
advocated that the first stage is to correct epicanthus and telecanthus, then
ptosis can be corrected 3-6 months later[5,8,10,14]. This regimen of
two stages surgery was used in this study.
Other surgeons
advocated one stage surgical repair of BPES and reported satisfactory results.
This approach would decrease the number of total procedures and reduce the time
needed for surgical rehabilitation, reducing the health costs and
patient/parental discomfort[9,12,15]. To avoid the tendency for
loosening of the medial canthoplasty associated with one stage surgery, Wu et
al recommended 4 mm overcorrection of telecanthus using a transnasal wiring
procedure, and they also concluded that patients with severe ptosis are not
good candidates for one stage procedure[16].
On the other
hand, Beckingsale et al recommended to start with ptosis surgery to
avoid amblyopia in patients with severe ptosis. They recorded a rate of 39% of
amblyopia among patients of their study. But 46% of amblyopia were attributed
to strabismus, 9% to astigmatism and 18% to mixed causes[17]. The risk
of developing amblyopia in unilateral ptosis is higher than in bilateral
symmetric ptosis as is found in the majorityof BPES patients because in this
condition the patient adopts a characteristic elevation of the chin and
extension of the neck that seems to diminish the risk of amblyopia development,
and consequently the need for early ptosis correction[9].
The first
stage surgery (medial canthoplasty) is performed to eliminate the epicanthal
fold and shorten the IICD. It is traditionally recommended to perform it
between 3 and 5 years of age. Before 3 years of age the face is not
sufficiently developed, and late surgery may result in more prominent scarring[5,8,18].
The age of the patients in this study ranged between 3 and 5 years with a mean
3.7¡À0.8 years. Various techniques for the epicanthus correction have been
described. One of the most widely used methods is that which was described by
Mustarde[6,19]. Meticulous measurements and mapping out of lines and
angles are necessary for the this procedure. Some believe that the geometric
basis of these flaps is too complicated, and the design is confusing. The
transposition of various flaps may be challenging and a significant amount of
flap trimming may be required before suturing[5,7,20]. Sebastia et
al reported the use of Z-epicanthoplasty which is simpler to perform and
yielded results similar to Mustarde flaps[9]. Sa et al used
the skin redraping method[8]. This method was originally described
to correct epicanthic folds for cosmetic concerns. It was used to correct
epicanthic folds in Asian patients with satisfactory results[21]. Li
et al reported better results with the Y to V procedure especially in
patients older than 10 years[5]. The Y to V medial canthoplasty was
used in this study and yielded good satisfactory results.
There is also
controversy about the correction of telecanthus. Some authors prefer the use of
transnasal wiring, the technique which was originally described by Callahan. It
provides a more effective traction of the ligaments and better reduction of the
intercanthal distance. It's especially beneficial in one stage procedure to
avoid the tendency of recurrence. It underwent many modifications including the
use of Y shaped titanium miniplates or the use of titanium medial canthal
screws. However, it is well known that it's the treatment with more morbidity
and is difficult to implement. It has the risk of infection, nasal bleeding,
and lacrimal injuries[9,16,22-24].
A plication of
the medial canthal tendon with fixation of the subcutaneous tissue to the
canthal tendon seems to be effective in the surgical correction of telecanthus
in patients with BPES. The tendon must be sutured as far posteriorly as
possible to create a posteriorly directed tension vector. This creates the most
natural anatomical reconstruction possible. The use of this procedure by a
broader surgical practitioner base with less experience may not be the most
appropriate[5,8,10,12,25].
As regards
ptosis correction, frontalis suspension is preferred in patients with no or
minimal levator function, which is the case in patients with BPES. Autogenous
fascia lata is the most appropriate material for the use in frontalis
suspension, but there are insufficient amounts of this material in children
younger than 3 years of age. Donor fascia lata provides a reasonable
alternative that decreases the attendant morbidity and reduces the complexity
of the procedure[16,26,27]. When fascia lata is not available,
whether autogenous or donor, synthetic materials can be used.
Other authors
consider levator resection as a more physiologic procedure, with a better
eyelid crease and involves no brow scars. Blomgren and Holmstrom suggested that
Levator resection can be used for all types of congenital ptosis. Mauriello et al
reported that maximal levator resection is indicated for severe congenital
ptosis and poor to absent levator function, especially if the ptosis is
unilateral[28,29]. In this series, frontalis suspension was used in
10 patients, and maximum levator resection was used in 3 patients who had
asymmetric ptosis requiring unilateral surgery. MRD1 significantly improved
from 1¡À0.4 mm preoperative 3.6¡À0.3 mm post operative.
In conclusion, there are various
strategies and complicated techniques that have been suggested for the
management of BPES. Two stages surgeries using the least complex procedures as
Y to V medial epicanthoplasty, medial canthal tendon plication, frontalis
suspension or maximum levator resection can yield acceptable results in the
correction of this complicated deformity.
CONFLICT OF INTERESTS
The author has no conflicts of interest to declare.
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Peer reviewer: Michael K. Yoon, MD,
Ophthalmic Plastic Surgery
Massachusetts Eye and Ear Infirmary, 243 Charles Street,
Boston, MA 02114, USA.
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