Pediatric Ptosis Repair: Blepharophimosis Syndrome
Michael J. Richard, MD
Blepharophimosis syndrome is a rare disorder either inherited as an autosomal dominant trait or occurring sporadically. The major findings of the syndrome include severe congenital ptosis, horizontal narrowing of the palpebral fissures, and epicanthal folds extending superiorly from the lower eyelid (epicanthus inversus). Other findings may include telecanthus, absence of a lid crease, low nasal bridge, and euryblepharon (Fig. 41.1). Female blepharophimosis patients can have primary ovarian insufficiency, which may affect menstruation and fertility.
EXAMINATION AND SURGICAL PLANNING
Assess for degree of ptosis including levator function by measuring MRD1 and levator function. Ptosis is typically severe (MRD1 < 1 mm), and levator function is extremely poor (<4 mm), and correction necessitates a frontalis sling procedure.
Note presence and degree of anomalous head position (chin up) and any effect this position is having on development (sitting in an infant, walking in toddler or older child).
Measure visual acuity with attention toward amblyopia.
FIGURE 41.1. Patient with blepharophimosis syndrome findings of congenital ptosis, epicanthus inversus, and telecanthus.
Perform cycloplegic refraction as ptosis can cause significant astigmatism (usually with-the-rule).
Assess degree of telecanthus and epicanthus inversus. Severe telecanthus may require transnasal wiring, but more moderate degrees can be addressed by epicanthoplasty and medial canthal tendon plication.
Some authors advocate staging the procedures. Alternatively, treatment of the ptosis, epicanthal deformities, and telecanthus can be performed concurrently during a single surgery, usually around the age of 1 year. Earlier surgery may be necessary if ptosis is causing deprivational amblyopia or an anomalous head position that is affecting the child’s development.
In an infant, the maximum safe doses of local anesthetic are relatively small, and if performing the ptosis and epicanthus surgery simultaneously and bilaterally, the injection volumes desired for hemostasis in multiple wounds can easily exceed the maximum doses. It is important to calculate the total maximum volume of anesthetic based on weight and not exceed that amount (see Chapters 1 and 42). For example, the maximum dose of lidocaine with epinephrine is 7 mg/kg. In a 6-kg baby, the maximum volume of 2% epinephrine with lidocaine would be 2.1 cc. One way to reduce the amount of anesthetic and still achieve hemostasis is to reduce the percentage of lidocaine from the typical 2% to 1% (which will double the volume that can be safely administered). Another approach would be to inject the needed anesthetic for the first part of the procedure (eg, bilateral epicanthus repair), perform that procedure, and later (after time for drug metabolism) inject for the second procedure (eg, frontalis slings). The anesthesia team can be very helpful in calculating weight-based maximum doses and volumes.
PTOSIS
Materials-BD Visitec Frontalis Suspension set (Seiff) consisting of a 0.9 mm by 6.3-cm silicone rod with swaged needles (20G × 2½ inches) on each end and a 10-mm long silicone sleeve with which the silicone rods can be secured.
The most common frontalis sling suspensory materials include autogenous fascia lata, preserved fascia lata, and 1-mm silicone rods (see chapter 38). Silicone rods produce less lagophthalmos because of the elastic nature of the material and are also adjustable.
Frontalis Sling Surgical Procedure
Many configurations have been described based on the location and number of stab incisions—including triangular, rhomboid, pentagonal, double triangular, and double rhomboid (see chapter 38). The pentagonal configuration (described here) utilizes five stab incisions with a single strand of silicone tubing as the suspensory material.
Mark the planned incisions prior to injecting local anesthetic with epinephrine (Fig. 41.2).
Place a corneal protector in the operative eye.
FIGURE 41.2. Preoperative markings for repair of blepharophimosis by frontalis sling procedure and Y-to-V advancement.
Make a central brow incision using a Bard-Parker no. 15 or no. 64 blade at the superior border of the brow cilia in line with the pupil. Enlarge the incision slightly so that it is just larger than the width of the blade. The stab should be made down to the level of the frontalis muscle, which is adherent to the periosteum at this level. Once the incision is made, use a Stevens scissor to bluntly dissect a pocket in this tissue plane to allow room for the silicone sling.
Make two paramedian brow incisions at the superior border of the brow and just outside the corresponding limbus. Each of these is only the width of the blade. Again, use a Stevens scissor to bluntly dissect a small pocket beneath the skin and subcutaneous tissues.
Make two horizontal eyelid incisions ˜2-6 mm above the lash line (corresponding to the upper half of the tarsal plate) and just inside the corresponding limbus. Sharply dissect down to the epitarsus using Westcott scissors. Alternatively, use a no. 64 blade to make a stab incision down to epitarsus.
Placing these incisions closer together will result in a more peaked lid contour, while placing the incisions further apart will result in a flatter lid contour. Placing these incisions closer to the lid margin may give more lift but may also be more likely to result in entropion.
Use a Webster needle driver and bend the needle to approximate a 3/8 curve suture needle, which gives some mechanical advantage in passing through the tissue.
Introduce one of the needles from the suspension set through the medial eyelid incision and gain purchase of the epitarsus.
Pass the needle along the length of the tarsus until the lateral incision and pull the sling until equal segments are visible from each eyelid incision.
Pass the lateral needle back into the lateral eyelid incision aiming vertically for the lateral paramedian brow incision. Attempt to pass the needle in a postseptal plane and then gain purchase of the arcus marginalis at the superior orbital rim before passing the needle out of the lateral brow incision. This directs the vectors of lift in the most natural and advantageous directions and also minimizes the possibility of seeing the sling through the skin. It is helpful to introduce a partially opened Adson forceps into the brow incision with downward pressure against the frontal bone to receive the needle as you pass it out the incision. This stabilizes the tissues and
prevents you from grasping more superficial tissues, which may lead to dimpling of the skin and soft tissues overlying the sling.
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