Natural history
- Ptosis can improve or resolve with time in certain circumstances.
- Mechanical ptosis due to infections or excisable mass.
- Traumatic ptosis including birth trauma can resolve
- A small percentage of pediatric ocular myasthenia will spontaneously resolve.
- Adequate follow-up to monitor vision is important to prevent deprivation amblyopia.
- Consider prophylactic contralateral eyelid patching or cycloplegia during observation period.
- Ptosis that occludes the central visual axis or with documented asymmetric vision is an indication for patching or cycloplegia.
- Consider amblyopia therapy even if surgery is pending for cases of severe ptosis (MRD1 ≤ 1mm).
- The incidence of amblyopia in congenital ptosis is approximately 18% (reports have ranged from 11% to 25%) (J Pediatr 2014; 165:820).
- Correct refractive error — ptosis can induce astigmatism with mechanical corneal modeling.
- Table 1 outlines threshold for refractive errors that should be treated with glasses.
Table 1. Guideline for refractive correction for infants and young children.
Medical therapy options
- No medical treatment for congenital myogenic ptosis
Radiation therapy options
Surgery options
- Indications
- The presence or risk of amblyopia due to covering or encroachment of the central visual axis.
- Psychosocial: Any eyelid asymmetry should be seen as a potential cause for body dysmorphism. It is important to take into account both the patient's and the parent's desires to achieve eyelid symmetry.
- Timing
- If good vision is documented there is no urgency for ptosis repair
- After 6 months of age elective surgery is considered to be of a lower risk for general anesthesia.
- Severe ptosis that covers the central visual axis such as with congenital 3rd nerve palsy might require earlier repair.
- Mechanical ptosis (eg, large infantile hemangioma) that has not responded well to medical management might require removal of the mass
- If child has a planned general anesthesia for another problem ptosis repair can be arranged at same anesthesia.
- Goal of surgery
- Functional success
- Eyelid height is elevated to clear the central visual axis (Usually an MRD1 of ≥2mm).
- Chin up position is resolved or improved due to better eyelid height.
- Excessive brow use can be resolved in younger children, but not always.
- Cosmetic success
- A functional success with good eyelid symmetry and contour as compared to the fellow eye. The eyelid height should have an MRD1 of ≥ 3mm in most patients (Figure 1).
Figure 1. The margin reflex distance 1 (MRD1) as measured in millimeters from the center of the cornea (right column of numbers). This is compared to an estimated amount of ptosis as measured from the superior corneal limbus (left column of numbers). It is best when describing ptosis to make MRD1 measurements.
- Choice of procedure
- Müllerectomy (Putterman's technique) with or without a tarsectomy (modified Fasanella-Servat procedure or tarsal-conjunctival-Müllerectomy).
- Good response to 2.5% Phenylephrine is a good predictor of surgical success.
- Indication might be patients at high risk of exposure keratopathy with poor or missing Bell's phenomenon
- Anterior levator resection
- Fair to excellent levator function (≥ 5 mm)
- Frontalis sling with synthetic or banked sling material
- Poor levator function (≤4mm)
- Age below 4 years (insufficient leg length for autogenous fascia lata graft)
- Potential need for removal (eg, 3rd nerve palsy with risk of cornea exposure)
- Frontalis sling with autogenous fascia lata
- Poor levator function (≤ 4mm)
- Age above 4 years (adequate leg length for autogenous fascia lata harvesting — ideal graft is ≥12cm)
- Table 2 depicts Beard's selection of ptosis repair based on levator function.
Table 2. Selection of ptosis repair based on levator function.
- Types of Müller's muscle surgery
- Modified Fasanella Servat
- Ideal procedure for mild ptosis with a good response to 2.5% phenylephrine
- Surgical outcome usually will be close to the result of a good phenylephrine response.
- Two matching hemostats are used (or a Putterman clamp) over an everted eyelid to crush the tarsus, conjunctival and Müller's muscle at about 4–5mm from the eyelid margin.
- This is then excised and anastomosed with a gut or nonabsorbable suture.
- If a Nylon or Prolene suture is used it should be removed in 5–7 days.
- Putterman procedure — Müller's muscle conjunctival resection (MMCR)
- Also ideal procedure for mild ptosis with a good phenylephrine response.
- The conjunctival and Müller's muscle are measured with calipers for a desired amount of resection and held on traction with a suture or skin hook.
- A Putterman clamp is used to isolate this complex for ease of resection and suturing.
- Variants of Fasanella Servat
- Can remove 9mm of Müller's muscle and 1mm of tarsus for every undercorrected 1mm on phenylephrine testing (OPRS 2002; 18:426).
- Can remove 2 mm of tarsus for each mm of desired eyelid elevation (OPRS 2013; 29:30).
- Can remove 2mm of Müller's muscle for each 1mm of undercorrected ptosis on phenylephrine testing.
- For patients at higher risk of exposure keratopathy, for developmental or behavioral reasons, ptosis procedures such as frontalis slings and levator resection should be avoided to minimize postoperative lagophthalmos.
- Modified FS can be used in these cases emphasizing functional outcome while sacrificing cosmetic outcome for the benefit of the patient.
- Anterior levator resection (Figures 2 and 3)
Figure 2. Levator resection. Intraoperative photo of a levator muscle dissection with three 5‑0 Vicryl sutures passed from the mid-tarsus to the levator aponeurosis (anterior to Whitnal ligament).
Figure 3. A. Preoperative photo of a patient with right-sided ptosis with good levator function. B. Postoperative photo 1 year after a right anterior levator resection.
- Ideal for a patients with a moderate to excellent levator function (6mm and above) and poor response to 2.5% phenylephrine.
- Nomogram 1 (Table 3) created by Berke in 1961 targets intraoperative eyelid height based on preoperative levator function.
Table 3. Amount of levator surgery for congenital, myogenic ptosis based on levator function and intraoperative eyelid level (under general anesthesia).
- Nomogram 2 (Table 4) created by Beard in in 1976 correlates amount of levator resection with amount of ptosis.
Table 4. Estimation of levator resection (Beard 1976).
- An eyelid skin crease incision is made and the levator muscle is advanced to the tarsus with an absorbable suture (5‑ or 6‑0 Vicryl).
- Excess aponeurosis is excised when the desired intraoperative height is achieved.
- Frontalis Suspension (Sling):
- Indications
- Congenital ptosis with poor levator function (usually less than 4 mm of excursion)
- Neurogenic ptosis with poor levator function
- Traumatic ptosis with poor levator function
- Mechanical ptosis where the mass effect on the eyelid is still amblyogenic.
- Contraindications
- If good levator function is present and alternative should be considered first.
- Significant keratopathy
- Missing corneal sensation.
- Complete facial nerve palsy with absent frontalis function
- Caution if poor motility or absent Bells phenomenon.
- Sling materials
- Synthetic: Silicone, expanded polytetrafluoroethylene (ePTFE (Gortex)), Nylon monofilament (Supramid), or Ptose-up.
- Banked fascia (Tutoplast)
- Autologous fascia lata
- Sling configurations
- Triangle — 1 brow incision
- Rhomboid — 2 brow incisions
- Pentagon — 3 brow incisions
- Double Triangle — 2 or 3 brow incisions (Figure 4)
- Double Rhomboid — 3 brow incisions
Figure 4. Frontalis suspension: double-triangle technique. In this illustration, the material is locked (crossed) in the center of the eyelid (optional).
- Choice of sling material
- All nonautologous materials carry a higher risk for rejection and infection.
- Silicone slings are very well tolerated.
- Figure 5 depicts a silicone sling (Seiff set, FCI Ophthalmics) placed in a rhomboid configuration with 2 brow incisions.
Figure 5A. Intraoperative photo of frontalis suspension with silicone sling placed in a rhomboid fashion. The silicone material is being tightened through a sleeve until the desired eyelid height is reached.
Figure 5B. Top: Preoperative showing severe left ptosis with poor levator function and a missing eyelid crease. Bottom: Postoperative 1 year after a left sided silicone frontalis suspension.
- Autologous fascia lata is very well tolerated and can be harvested when a patient is tall enough (Ideally over the age of 4–5 years).
Describe other management considerations
- Levator extirpation
- Can be considered to remove a Marcus Gunn jaw wink. To be performed when a frontalis sling is placed.
- Whitnall's sling
- This is an alternative to frontalis sling surgery where only the aponeurosis is excised and Whitnall's ligament and the underlying levator muscle is sutured directly to the tarsus (Arch Ophthalmol 1990; 108:1628).
- Temporary suture tarsorrhaphy
- In the setting of a maximal levator resection or frontalis sling surgery a temporary lateral suture tarsorrhaphy can be placed.
- This can decrease the initial risk of postoperative corneal exposure.
- Can be removed 2–7 days after surgery when orbicularis compliance (relaxation) is improved and lagophthalmos is improved.
- Unilateral versus bilateral surgery
- Asymmetric preoperative ptosis is always a challenge
- Pediatric patients are unable to participate to assess intraoperative eyelid height.
- In the setting of unilateral ptosis, contralateral ptosis might become uncovered due to Herring's law (Figure 6).
Figure 6. Right-sided ptosis with mild left upper eyelid retraction.
B. Demonstration of Herring's law. Postoperative photo 1 year after a right autogenous fascia lata frontalis suspension sling. The left upper eyelid is now lower in height.
- Bilateral symmetric ptosis repair where the same procedure is performed on both eyelids may allow for better symmetry, but may be unnecessary. This is in contrast to a per-eyelid approach where the choice of procedure is made independent of each eyelid (eg, right Fasanella for mild ptosis with a left sling for severe ptosis).