The origin of the levator muscle is just above that of the superior rectus muscle at the orbital apex. The levator muscle becomes a flattened, thinned layer of noncontractile tissue as it approaches its insertion into the tarsal plate and into the skin of the upper lid. This nonmuscular portion of the levator is termed the levator aponeurosis. Transition from muscle to aponeurosis is abrupt and can be visually identified easily as the tissues are exposed surgically.

The levator muscle is a relatively weak muscle the sole function of which is elevation of the eyelid. An observer easily perceives any asymmetry in lid elevation, contour, or width of the palpebral fissures.

Blepharoptosis may result from localized primary or secondary dystrophy of the levator muscle, neurologic dysfunction, myoneural junction dysfunction, or, for complex and poorly understood reasons, as a developmental defect associated with other craniofacial and genetic syndromes. Clinical experience with childhood ptosis suggests that most levator muscles of ptosis patients are dystrophic. Levator muscle and aponeurosis tissues appear to be infiltrated or replaced by fat and fibrous tissue. However, levator muscle fiber size and distribution are not abnormal in other patients and blepharoptosis is not related to other forms of muscular dystrophy.¹

Astigmatism and amblyopia occur as a result of blepharoptosis.^2–5 Surgical repair of blepharoptosis does not mitigate the need to treat amblyopia. Indeed, amblyopia treatment in young patients may become more necessary after surgical treatment of ptosis.² Any noted increased astigmatism after surgical repair of ptosis may be transient in other patients.⁶ Severe ptosis has been shown to produce axial myopia in experimental animals.⁷ Transient ptosis early in the neonatal period may not produce amblyopia.⁸

Blepharoptosis has other physical, functional, and psychological consequences. Repair of blepharoptosis in childhood usually has functional implications. Many methods of blepharoptosis repair have been described in detail.¹ Procedure choice depends on treatment goals, the underlying diagnosis, degree of levator function, and the skill and experience of the surgeon. Surgical treatment results can be pleasing to the patient and to the family (Fig. 1).

Visual acuity and refractive error should be recorded. A thorough eye examination, particularly for strabismus, corneal sensitivity, and retinal dysfunction, should be done. Tear function should be measured if any doubt as to the adequacy of tear production exists.

Occasionally, the examiner must determine whether apparent ptosis is in fact pseudoptosis or whether lid retraction of the contralateral upper lid exists. To detect pseudoptosis, lid height should be observed and measured with each eye fixing on a distant target. Lid position in downgaze should be noted. If ptosis is congenital, the ptotic lid appears short (higher) in downgaze. A ptotic lid does not appear short in downgaze when ptosis is acquired. If lid retraction is present, the higher lid remains high in downgaze (Fig. 2). An exophthalmometer is frequently used to assess relative proptosis or enophthalmos of each eye. The patient should be asked to close eyes forcefully as the examiner holds the upper and lower lids apart. If the globe elevates during forced lid closure, a normal Bell phenomenon is present. External photographs of the eyes and face should be included in the patient's records.

Lid height, contour, and crease, lash position, and any special facial characteristics should be noted and described in writing. Levator function should be measured. Various measurement schemes have been described. The most basic and most universal is to measure the greatest width of the palpebral fissure with the patient's eyes in level gaze. The patient then is asked to look downward, as a ruler is positioned with a mark adjacent to the upper lid margin. With the examiner's hand eliminating brow action by the patient, the patient is asked to look upward as far as possible without changing head position. Lid elevation is measured directly from the ruler and is recorded in millimeters of levator function.

The amount of surgical correction desired can then be determined and procedure choice can be made. Refinements in numbers of millimeters of levator resected often are made during the surgical procedure but the preoperative plan remains as the foundation for the overall procedure. If a patient has an anomaly of the globe, orbit, or lower lid, corneal light reflex-lid margin measurements can be helpful in positioning the ptotic lid (Fig. 3).

Surgical correction of blepharoptosis can be undertaken at virtually any age. Intervention may be required by the discovery of significant amblyopia, anisometropia, or strabismus. Correction of ptosis will not alleviate any of these conditions, which, once developed, must be treated directly. Severe bilateral ptosis in an infant may delay walking because of the balance problems produced by an extreme chin-up head position. Although surgical correction of ptosis may be undertaken at any age, frequently surgery is delayed until a child is 3 or 4 years old if no complications of the ptosis dictate earlier intervention. At age 3 or 4 years, accurate measurements can be obtained preoperatively, and postoperative cooperation of the child can be expected. Earlier intervention, however, can lead to equally acceptable results and is recommended by many surgeons. Parents often are anxious for early surgical intervention but can be reassured that it is not mandatory in most cases.

The amount of levator-aponeurosis complex resected significantly differs in children with congenital blepharoptosis compared with adults with acquired blepharoptosis. In general, treatment of adult acquired blepharoptosis with the same numbers of millimeters of levator resection recommended for children would result in significant overcorrection.

INDICATIONS

Adequate levator function must be present to offer the ptosis patient a reasonable chance for correction with a levator resection. Many authors are in general agreement as to how much levator function is required to justify this procedure. Most suggest that a measurable lid excursion from downgaze to upgaze of 4 mm or less (measured with the ipsilateral brow inactivated with thumb or finger pressure) is too poor to permit lasting results from levator resection. If levator function is more than 4 mm but less than 6 mm, a “large” levator resection is recommended. If levator function is between 6 and 8 mm, a “moderate” levator resection is indicated. If levator function exceeds 8 mm, a smaller resection of the levator is indicated. A large levator resection is defined as 22 mm or more, moderate as 16 to 18 mm or more, and small as 13 to 15 mm.

Others clinicians prefer to measure the height of the lid margin relative to the corneal light reflex or corneoscleral limbus or the width of the palpebral fissures and to use these measurements as guides to the amount of surgery to be performed.¹⁴ Lid height relative to the cornea can be measured toward completion of the surgical procedure using either Berke's recommendations or the lid margin to corneal reflex distances, thus allowing further refinement in the amount of levator aponeurosis to be resected.

CONTRAINDICATIONS

The external levator resection surgical technique is applicable to a patient of any age, but it is best used when levator function can be assessed accurately.

As a practical guide, correction of congenital blepharoptosis with this approach is often done in the preschool age group, when the patient is 3 or 4 years old. At these ages, accurate visual acuities have been obtained, amblyopia has been identified and treated, and reliable measurements of levator function have been recorded. However, amblyopia or parental concerns often dictate earlier intervention.

An external levator resection is not indicated when levator function is less than 4 mm. In such cases, immediate postoperative results might be acceptable after a large levator resection but experience has shown that the lid height will not be maintained over time.

Relative contraindications to an external approach include cases in which a skin incision is undesirable, such as in the Asian eyelid. Although creation of an undesirable lid crease with an external approach can be avoided, a skin incision scar can be visible and therefore unacceptable to the patient. An alternative procedure is generally preferable. The Asian eyelid differs in several important features from that in caucasian.¹⁵

Any ptosis procedure performed in a patient with a poor Bell phenomenon, limited elevation of the eye, reduced corneal sensitivity, or poor tear production risks development of postoperative corneal complications.

SURGICAL TECHNIQUE FOR EXTERNAL LEVATOR RESECTION

Preoperative photographs are helpful, in that such photographs can become part of the patient's permanent record and can be referenced during the surgical procedure as desired. Used properly, these photographs can help prevent inadvertent surgery on the wrong lid and can assist the surgeon with the artistic portions of the procedure, such as lid contour and lash position.

General anesthesia is required for children, but either local or general anesthesia is satisfactory for adults. Lid position may be adjusted precisely at the time of surgery if the patient is awake and only local anesthesia is used.

When ptosis is bilateral, bilateral symmetric or asymmetric levator resection usually is done in one surgical procedure. If an increased risk of corneal complications exists in that patient, the procedure should be done unilaterally. If ptosis is severe but still amenable to levator resection, the surgeon may prefer to operate first on the more severely involved side. After healing has occurred and final lid position is established, surgery can then be performed on the second lid. It is generally easier to match the less ptotic lid to the more severely affected, but already corrected, lid.

Existing lid creases are outlined on both lids with a fine-tipped surgical marking pencil (Fig. 4A). If the existing lid crease does not match the desirable lid crease in the fellow eyelid, a new position is marked. The marked areas are compared visually and are measured with a ruler or calipers for accuracy. In children without ptosis, the height of the center of the lid crease averages 6.5 to 7 mm from the lid margin. The nasal and temporal ends of the lid crease measure about 4.5 to 5 mm above the lid margin in children. The height of the lid crease increases with age and is about 10 mm centrally in adults.

After the proposed incision site has been marked and verified, the lid to be incised is further isolated from the fellow eye with appropriate drapes. A corneal protector is inserted between the eyelids. After the anesthesiologist is informed, a solution of 1% lidocaine containing epinephrine in a concentration of 1:200,000 is injected into skin and orbicularis muscle beneath the eyelid mark. A 27 gauge needle held parallel to the lid is used for the injection. Only one or two skin punctures are required to traverse the length of the incision with a short needle. No more than 0.5 ml of solution is required, because larger volumes of fluid only distort the anatomy of the lid. If the needle tip is just beneath the skin, it is in the orbicularis layer because no subcutaneous fat exists in the lids. The lidocaine-epinephrine solution facilitates dissection and reduces bleeding. Some surgeons omit this step if the patient is under general anesthesia, whereas others use it in virtually all cases. Epinephrine is omitted from the injection if any risk factors regarding its use exist. If the patient is awake, topical tetracaine can be added as dissection approaches the conjunctival layer.

An incision is made with a curved scalpel blade from one end of the eyelid mark to the other without lifting the blade from the lid. The incision is made more precisely if it is made from a nasal to temporal direction; in this instance, the surgeon places slight tension on the temporal side of the lid as the blade is drawn from nasal to temporal. This prevents the loose skin of the upper lid from gathering beneath the knife and forcing the blade away from the mark. The depth of the incision should extend through the skin and may extend through the orbicularis layer as well (see Fig. 4B). More experienced surgeons make the initial incision deeper relative to the desired depth beneath orbicularis. If the incision is only skin deep, further dissection through orbicularis is completed with scissors that have a rounded tip. Skin hooks help the assistant to provide adequate exposure. The transverse muscle bundles of the orbicularis layer can be identified and separated parallel to the incision with minimal bleeding. Separation with blunt dissection is preferable to cutting across the muscle bundles. After dissection extends beneath the orbicularis layer, the aponeurosis-orbital septum is the next layer encountered. The surgeon must be aware that the orbital septum may extend further than usual toward the lid margin, particularly in Asian patients and in patients who have blepharophimosis.¹⁵

When the depth of the dissection extends beneath the orbicularis, the orbicularis is undermined superiorly until the orbital septum is identified (see Fig. 4C). The septum is then elevated at its inferior margin from the anterior surface of the levator aponeurosis, usually without disruption of retroseptal fat (see Fig. 4D). Orbicularis is undermined inferiorly to near the lid margin. As undermining continues, lash follicles may become visible inferiorly, which indicates that dissection is adequate. Lash follicles should not be incised or cauterized.

With the orbicularis exposed from its posterior surface, the remaining attachments of the aponeurosis to the anterior surface of the tarsal plate can be elevated. Holding blunt-tipped scissors vertically with the tips parallel to the skin incision and making a series of small cuts across the length of the tarsus best accomplishes aponeurosis elevation. If this incision is started at about the vertical center of the tarsus, little aponeurosis tissue is encountered with the first series of snips. Aponeurosis becomes quite thin as it terminates on the anterior tarsal surface. The tarsal plate should not be incised. Incised aponeurosis tissue is gently elevated and undermined, further separating it from the tarsus. Meibomian glands become visible within the tarsal plate as the dissection proceeds. With each millimeter of vertical progress, aponeurosis tissues become thicker.

As the superior border of the tarsal plate is reached, the surgeon can see that the depth of the dissection increases slightly. Bleeding is encountered at the superior tarsal border as Müller's muscle is exposed (see Fig. 4E). Bleeding is minimal if dissection has been made in the correct tissue plane. Müller's muscle is undermined and elevated from the only remaining deeper layer, palpebral conjunctiva. At this point, dissection usually can be accomplished with a moistened cotton-tipped applicator. Blunt dissection is facilitated by clamping the distal free end of the dissected levator aponeurosis with a clamp of the type devised by Berke for this purpose. An Ehrhardt's clamp placed on the lid further facilitates this part of the dissection (the corneal protector can be removed while this clamp remains in place; see Fig. 4F).

Vertical parallel incisions are made at the temporal and nasal borders of the levator-aponeurosis tongue of tissue held in the ptosis clamp. These incisions are made under direct view and control approximately at the nasal and temporal limits of the horizontal skin incision. Attention is paid to the location of the superior oblique tendon nasally and the lacrimal gland temporally. Additional dissection is carried out beneath the levator, and the vertical incisions are extended until the desired length of levator muscle is free from surrounding attachments. It is important to free only the amount of levator desired because excessive dissection in the area of Whitnall's ligament (superior suspensory ligament) may diminish the results. Moreover, if the superior conjunctival fornix is totally freed from surrounding attachments, the probability of postoperative conjunctival prolapse increases.

After dissection of levator aponeurosis and muscle, the Ehrhardt's clamp is exchanged for the corneal protector. Three or four (usually three) double-armed (spatula needles) 5-0 synthetic absorbable sutures are passed across the upper one third of the tarsus parallel to the lid margin. About 3 mm of tarsus is included in each bite. The corneal surface of the tarsus is inspected to ensure that the sutures were placed within but not through the tarsus. Sutures placed too deep will be exposed on the ocular surface of tarsal conjunctiva where they may produce corneal erosion.

Needles on the sutures are brought through the tongue of the levator-aponeurosis complex at a measured height, emerging on the external levator surface (Fig. 5A and B). A single overhand knot is tied, apposing the aponeurosis and the tarsal plate. Clamps and corneal protector are removed, and the lid is inspected for height and contour. The single-throw knot may be untied and the sutures repositioned in the levator if modifications in height or contour are required. It is better to reposition the sutures in the levator-aponeurosis complex and not in the tarsus because the tarsal plate will not accept repeated passes of a needle without becoming friable and unreliable in retaining a suture. When the surgeon is satisfied with the lid position, a second throw is added to the knot, forming a square knot on the external levator-aponeurosis surface (see Fig. 5C). Redundant levator muscle and aponeurosis distal to the sutures can then be excised (see Fig. 5D).

If the skin incision and knot position are in alignment, needles on the existing sutures may be passed through the skin edges and retied on the skin surface (see Fig. 5E). Skin edges pulled to the levator surface form a lid crease. The tarsal-levator sutures, however, may not match the location of the skin incision. If skin crease and levator sutures do not match, the sutures through the levator may be cut distal to the square knots on the levator surface. A second row of sutures is then placed above the first row. This row of sutures engages only the levator and is placed directly beneath the proposed skin crease. After each double-armed suture is tied in place, the needles are brought externally through the skin edges and retied, thus forming a lid crease in the desired position. In either case, the needles engage only the skin edges, not the orbicularis layer. This technique produces the deepest and most acceptable lid crease.

An antibiotic ointment are lubricants are used in the eye and on the incision. No bandages are required. The patient is advised to avoid strenuous activity, swimming, or being anywhere dusty or dirty for about 2 weeks.

ALTERNATIVE TECHNIQUES

Levator aponeurosis need not be dissected from the tarsus. Rather, the aponeurosis may be engaged with nonabsorbable sutures at a measured position above the lid margin. These sutures can be used to attach this area to the aponeurosis at the superior tarsal border.

The area of Whitnall's ligament may be used for tarsal attachment when levator function is poor and maximum effect is required. Use of nonabsorbable sutures is desirable.

The entire operation of levator resection may be performed from the conjunctival surface (Iliff's procedure). This approach is more difficult for the inexperienced surgeon but it offers the important advantage that no skin incision is necessary. This procedure is identical to the external approach in concept, but the lid is everted on a Desmarres's retractor, a conjunctival incision is made at the superior tarsal border, and the conjunctival-levatoraponeurosis complex is engaged with a Berke type ptosis clamp. Conjunctiva then is incised across the superior tarsal border, separated from the levator aponeurosis, and, after levator shortening, is reattached to the superior tarsus.

RESULTS

Results from levator resection surgery are excellent if the chosen procedure is appropriate for the patient's problem and if the procedure is performed skillfully and artistically (see Fig. 1). The better the preoperative levator function, the better the surgical prognosis will be. In the ideal case, symmetry is achieved, no evidence of the surgery is apparent on casual inspection once healing occurs, and results are stable over time. Less than ideal results occur if final lid contour is asymmetric, lid height is asymmetric, the lid crease is poorly developed or positioned, or lashes are lost or misdirected.

When required, reoperation is best done 1 to 3 days after the original procedure, when tissues can be separated without additional sharp dissection. If the original procedure was performed with minimal manipulation of tissues, postoperative swelling does not preclude making such an early decision. If a decision cannot be made in the first few days after the original procedure, reoperation is best delayed for several months unless corneal conditions dictate otherwise.

COMPLICATIONS

Complications occur when a new problem results directly from the surgical procedure, such as infection or conjunctival prolapse, or indirectly, as in corneal exposure with secondary corneal complications.

Anesthetic complications are not discussed in detail in this chapter, except to note that patients with ptosis may have a higher risk of malignant hyperthermia and cardiac arrhythmia.

If the lid is positioned too high and is fixed in position to a degree that lid closure cannot occur, the cornea may suffer from exposure, drying, and ulceration. Corneal perforation and permanent visual impairment can result. This type of complication is particularly likely to occur when upward movement of the eye is impaired or when corneal sensitivity is reduced.

If surgical dissection superiorly has been aggressive, conjunctiva may prolapse into the palpebral fissure some time after the surgical procedure (Fig. 6A). Once exposed and with normal lubrication by the tear film impaired, further edema, drying, and ulceration may occur (see Fig. 6B). In some cases, the prolapsed conjunctiva recedes if it is kept moist and if the eye is patched judiciously. Surgical replacement of prolapsed tissue may be necessary and conjunctiva may be sacrificed in the process. If replacement can be done, sutures are required to hold the prolapsed tissue in position. This complication can be avoided by placing a single absorbable suture in the superior fornix during the original procedure. This fornix suture is placed in a double-armed fashion through the conjunctival surface, exiting onto the external surface of the levator, where it is tied before skin closure. Another technique involves grasping the superior rectus through the conjunctiva in the superior fornix, passing a suture through the conjunctiva and superior rectus, and tying the knot in the superior fornix.

INDICATIONS

The procedure is indicated when levator function is less than 4 mm such as is found in patients with severe congenital ptosis, blepharophimosis syndrome, partial CN-3 palsy, or agenesis of the nerve supply to the levator muscle. Frequently, levator function is poor in one eye, but good or normal in the fellow eye. Frontalis suspension procedures produce lagophthalmos that is particularly noticeable in downgaze. Lagophthalmos effect is more apparent if a unilateral suspension procedure is done. Most surgeons prefer to perform a bilateral suspension procedure for unilateral severe ptosis. Disinsertion of the normal levator has been advocated when severe unilateral ptosis is present but is seldom done in practice. Unilateral procedures are performed when the family will not permit a bilateral procedure to be done in such circumstances.

CONTRAINDICATIONS

This procedure is relatively contraindicated in patients with limited elevation of the eye, as is seen with complete CN-3 palsy, double elevator palsy, and inferior restriction syndromes. Anesthetic corneas are also a relative contraindication. Mild to moderate degrees of ptosis are better treated with alternative procedures. An advantage of the frontalis suspension procedure is that it can be “undone” relatively easily if corneal exposure becomes a problem. In patients with the above conditions, frontalis suspension procedure may be done more safely if final lid height is positioned lower than ideal to minimize the potential corneal risks.

SURGICAL TECHNIQUE

Several materials are available for securing the lids to the frontalis muscles. Consensus suggests that the best material in terms of strength, longevity, and absence of complications is autogenous fascia lata. Fascia lata can be obtained from the leg of a child who is 3 years old or older. For younger patients, alternative materials are often necessary. Alternatives to autogenous fascia lata include preserved (tissue bank) fascia, nonabsorbable suture material (such as 2-0 Prolene), silicone bands, suture-reinforced sclera, and Gore-tex. Other autogenous materials used less frequently include palmaris longus tendon and temporalis fascia.¹⁶

Autogenous fascia is obtained from the lateral thigh after identifying the anterior superior iliac spine and the head of the fibula. Just posterior to a line connecting these two points is the area of the thigh where fascia lata is best obtained (Fig. 7A). An incision is made far enough above the knee to avoid the joint capsule. The incision must be 3.5 to 4 cm long (see Fig. 7B). Dissection proceeds through skin and subcutaneous fat to the glistening surface of the fascia. In an older child, the vertical direction of the fascia fibers is easily discerned (see Fig. 7C). In a younger child, the fiber orientation is less well defined. Along the line described above, the fibers of fascia are incised with two parallel incisions made about 1 to 1.5 cm apart. The underlying muscle bulges outward. With long-handled, blunt-tipped scissors, fascia is separated from overlying fat and underlying muscle (see Fig. 7D). A Crawford's fascia stripper is used to obtain a strip of fascia that is 12 to 15 cm long (see Fig. 7E-G).

Bleeding is minimal and may be controlled with compression. No attempt is made to close the fascia wound in the leg. Skin and subcutaneous tissues are closed with interrupted absorbable sutures. A nonencircling pressure dressing is applied. The excised fascia strip is placed on a sterile surface, such as a tongue depressor, separated from any attached fat or muscle, and split into two strips with a single incision made with a scalpel. The fascia strips are kept moist with saline and are covered until they are required for placement.

A surgical marking pencil is used to outline proposed skin incision sites on the lids and brow. Several geometric configurations have been described for the placement of the suspension material. In smaller children with limited space, fewer incisions and simpler configurations are desirable. A horizontal lid mark is made 1.5 mm above the lid margin and in line with the nasal limbus of the cornea. A second mark is made at the temporal lid margin in line with the temporal limbus. When viewed together, these marks appear to be skewed slightly toward the nasal side of the lid. A third mark is made just above the brow and centered between the marks on the lid margins. A fourth and then a fifth mark are made in the brow nasal and temporal to the lid marks, forming a pentagonal configuration as the marks are viewed together. Similar marks are made on the opposite lid and brow. The marks are inspected for symmetry and are repositioned as required.

Corneal protectors are placed in both eyes, and the skin is incised where marked (Fig. 8A). Incisions are short, almost like puncture wounds, and are made only through the skin. The central incision is made slightly larger and is undermined superiorly (see Fig. 8B). A Wright's needle or its equivalent is used to thread the suspension material through the wounds, beginning at the lid incisions and eventually exiting with both ends of the suspension material through the central incision (Fig. 8C and D). After the suspension material has been placed in the lid and brow incisions but before placement into the central incision, the surgeon can elevate the lid and check for contour and height (see Fig. 8E). Depending on the material used, the suspension material is tied either to itself and sutured or simply sutured one arm to the other. If autogenous fascia is used, a single-throw knot is tied in the fascia (see Fig. 8F). This knot is secured with an absorbable synthetic suture (see Fig. 8G). If heterogeneous fascia is used, no knot is tied in the fascia, but the ends of the fascia strip are sutured to each other with nonabsorbable sutures. The knot of material and sutures is positioned deep in the central incision. The incision is closed with one or two mattress sutures. All suspension material must be located below the skin edges or erosion through the skin with granuloma formation will occur. A single suture through the skin edges of the incisions in the brow prevents any skin dimpling that otherwise might occur. The incisions at the lid margins do not require sutures if autogenous material is used. All incisions are better closed with a single suture if synthetic materials are used for the suspension.

ALTERNATIVE TECHNIQUES

An incision in the midthigh area over fascia lata directly dissecting with blunt-tipped scissors a suitable strip of the underlying fascia lata is an alternative. The skin incision may be made in the iliac area for the same purpose.

A double-rhomboid placement of incisions may be made instead of pentagonal placement. Some surgeons believe that this technique provides greater control over lid contour. This technique appears to be more useful in older patients who have a larger lid-brow area.

A separate lid crease incision may be made to permit direct attachment of fascia or synthetic material to tarsal plate. Advocates of this technique believe that a better lid crease is assured. Variations in this technique have been described.¹⁷

EXPECTED RESULTS

Results of frontalis suspension procedures can be quite satisfactory. Lid opening with brow elevation is greatly facilitated, and the patient's appearance usually is satisfactory to the family. The immediate postoperative appearance of lids that do not close with sleep and of brow tissue that is swollen and distorted is distressing to parents (Fig. 9). Families must be warned before the operation that this postoperative distortion in appearance will occur. Eyelids that are open during sleep are especially troublesome to observing parents. The problem of open lids during sleep diminishes with time but may persist to some degree indefinitely. Lagophthalmos, prominent initially, also diminishes with time as the patient learns compensatory head maneuvers (Figs. 10 to 12). Autogenous materials remain stable over time (Fig. 13).

COMPLICATIONS

If suspension materials are not placed well beneath the skin, granuloma formation may occur, particularly if donor fascia is used (Fig. 14). Granulomas should be treated conservatively because they resolve eventually. Dissolution of heterogeneous fascia can occur, however. Topical antibioticcorticosteroid ointments may be helpful in management. Closure of skin wounds with attention to the placement of the suspension materials helps to obviate this complication.

Lid-brow incisions should be marked and placed symmetrically. Postoperative lid position may be too high or too low. Overcorrection rarely is a problem in children if ocular rotations are normal. When CN-3 palsy or other conditions limit elevation of the eye, a suspension procedure may have to be adjusted downward or removed altogether if corneal problems develop.

Suspension materials may dissolve or break. Suture material that is too small may tear through through soft tissue, like a wire cutter through soft cheese, thus reducing the effectiveness of the procedure. The most enduring and least complicated suspension materials are autogenous fascia or (presumably) autogenous tendon. However, patients who have had fascia lata harvested may complain of leg pain or appearance of the scar.¹⁸ F.D.E. has observed a small herniation of muscle through the fascia lata harvest site.

INDICATIONS

Limited indications for this procedure exist in children with ptosis except for those with Horner's syndrome, which is not uncommon in children, but, when it occurs, any associated ptosis can be corrected with an external levator resection or with a tarsal Müller muscle-shortening procedure. We have not seen a single case of non-Horner's congenital ptosis that was treated adequately with a procedure of the tarsal- Müller muscle type although others have reported excellent results.¹⁹ Occasionally, the procedure is useful as an office procedure in an older child or adult with a small residual ptosis when no modification of the lid crease is desired.

CONTRAINDICATIONS

These procedures are not indicated in the management of non-Horner-type congenital blepharoptosis. If reposition or creation of a lid fold is a consideration, these procedures are not indicated. Conjunctival resection should not be done if conjunctival disease such as cicatricial pemphigoid or Stevens-Johnson syndrome is present.

SURGICAL TECHNIQUE

The lid is everted to expose the superior tarsal border. Two small curved hemostats are placed across the horizontal length of the tarsus at the height that was determined previously. The tissue incorporated in the clamps is excised. If a suture closure is used, a collagen suture brought into the temporal side of the tarsal plate from the external lid surface is placed superior to the hemostats before the block of tissue is excised. This suture is woven across the tarsus in a mattress fashion to the nasal border, then back to the entrance wound after the block of tissue is excised. A small scratch mark is made in the skin before the lid is everted so that the tied suture can be buried externally. To avoid any risk of corneal irritation from the sutures, some surgeons do not close the wound with suture. A soft absorbable suture seems to minimize this risk.

ALTERNATIVE TECHNIQUES

The procedure may spare the tarsal plate if special clamps are used or the technique is varied to include only tissues located above the tarsal plate (Muller's muscle and aponeurosis).²⁰ Conjunctiva may be spared if desired, but it is rarely dissected from the other tissues because doing so complicates and prolongs the procedure.

RESULTS

If the selected procedure is appropriate, the desired results are produced.

COMPLICATIONS

Corneal irritation from sutures exposed on the conjunctival surface may occur. Suture removal, patching, and appropriate treatment of any secondary infection can manage these problems in most cases. The most likely complication from this type procedure is that it is used for the wrong type of ptosis and therefore proves inadequate. The superior palpebral conjunctiva, with the goblet cells and accessory lacrimal glands, is sacrificed to some degree with this type of procedure unless special techniques are used.

DISCUSSION

Blepharoptosis in children should be repaired. The primary reason for repair is functional; improvement in appearance is a secondary but important objective of repair. A high incidence of amblyopia occurs with unilateral ptosis. This amblyopia is more likely due to anisometropia than to occlusion of the pupil. The mechanical effect of an atonic lid resting on a soft, pliable infant cornea is presumed to be the underlying cause of the anisometropia, although some experimental evidence suggests that partial occlusion of the visual axis may play a role.

Repair of ptosis can produce excellent functional and cosmetic results. The surgeon should endeavor to produce symmetry not only of lid height but also of contour, lash position, lid crease, and skin folds.

ISOLATED BLEPHAROPTOSIS

Blepharoptosis is a common isolated finding in young children. Often family members report that a child's ptotic lid was swollen at birth and did not elevate for several days thereafter. Frequently, the family suspects forceps injury or other birth injury. However, it seems unlikely that mechanical trauma at birth is responsible for a significant percentage of ptosis cases. Ptosis may occur through autosomal dominant inheritance.^21,22 Common familial occurrences suggest that genetic or chromosomal defects are likely, even in the isolated case.

The position and presence of a lid crease suggests a mechanism other than dehiscence. When viewed microscopically, excised levator muscle and aponeurosis generally appear dystrophic. In severe cases, little or no striated muscle can be identified at the time of operation. This suggests that ptosis is secondary to local developmental defects in muscle structure. The role of local innervation is not clear except when ptosis is associated with other innervation defects.

COMPLICATED BLEPHAROPTOSIS

Craniofacial and Genetic Syndromes

Many developmental disorders are accompanied by ptosis. In fact, the finding of ptosis is so frequent with these disorders that its presence is rarely helpful with the differential diagnosis of a specific disorder. Therefore, no attempt will be made to classify all of the associated conditions wherein ptosis is also seen. Only a few of the more classic or important entities are discussed.

BLEPHAROPHIMOSIS SYNDROME. This condition is known by several names, including no-name syndrome, tetrad syndrome, and BPE syndrome. Short palpebral fissures, congenital ptosis, epicanthus inversus, and telecanthus characterize the condition. Brow elevation is a prominent feature. Levator function is poor. Other neurologic and systemic abnormalities may be present.^23–27 Children with this syndrome should receive thorough genetic and pediatric evaluations because of potential associated problems.

Preferred method of ptosis repair is frontalis suspension. If levator resection is done, the anatomy of the lid is seen to vary from the usual in that the orbital septum extends inferiorly onto the tarsus, often inserting on the anterior surface of the tarsal plate along with the levator aponeurosis.¹⁵ In part because of the associated lid fissure phimosis, the degree to which the lids can be opened with any procedure will be less than desirable. Nevertheless, function and appearance can be improved significantly.

CN-3 PALSY. Congenital CN-3 palsy is not unusual in a pediatric ophthalmology practice. A structural lesion rarely is demonstrable in congenital palsies, but occasionally, central defects involving the CN-3 and other central structures may be seen.^28,29 Signs of aberrant regeneration usually are present. The pupil may be paradoxically small and not reactive. Any combination of CN-3-supplied extraocular muscle dysfunction may be demonstrable. Extraocular muscle surgery may be required. Extraocular muscle surgery should be performed before or in conjunction with lid surgery. Depending on the degree of levator function present, either a levator resection or a frontalis suspension procedure may be indicated. A frontalis suspension is required if levator function is poor. Special consideration must be given to the cornea in cases of CN-3 palsy. If the globe does not elevate, corneal drying will occur if the upper lid is significantly elevated surgically. Lubricants should be applied liberally in the postoperative period. Often, only partial elevation of the upper lid is indicated. If progressive corneal complications do occur, the suspended lid should be lowered. The outcome of lid surgery and extraocular muscle surgery for cases of CN-3 palsy depends to a degree on the severity of the palsy and the specific muscles involved. More than one procedure frequently is required. Amblyopia is frequent but can be treated successfully.^30,31

DOUBLE ELEVATOR PALSY. This condition is also known as monocular elevation deficiency. It is usually congenital, rarely acquired, but, in any case, can be associated with other conditions.^32,33 Congenital blepharoptosis may be accompanied by dysfunction of the ipsilateral superior rectus muscle without other signs of CN-3 palsy. These findings suggest that the abnormality is limited to the superior division of the CN-3. Bell's phenomenon generally is demonstrable in these patients unless an inferior restriction is present. Upward saccades may be rapid or slow. Several variations in presentation have been studied in detail.³⁴ Superior rectus weakness alone appears to account for the clinical findings in some cases. Magnetic resonance imaging has demonstrated decreased volume of the superior rectus muscle of the affected eye.³⁵ An implication of inferior oblique muscle weakness as a part of the syndrome is difficult to explain on an innervation basis and is not necessary to explain the clinical findings.³⁶ Inferior rectus muscle restriction accounts for the clinical findings in some cases. Thus, viewed in an office setting, monocular elevation deficiency may be considered to be secondary to deficient supranuclear innervation, superior rectus weakness, or inferior rectus restriction. Paradoxically, hypertropia of the ipsilateral eye in upgaze has been reported in other cases of congenital ptosis.³⁷ Refinement of the diagnosis of monocular elevation deficiency and proper treatment of the underlying condition is necessary before ptosis repair.³⁸ Ptosis in such cases may, in fact, be pseudoptosis. Pseudoptosis disappears when the patient fixes with the involved eye. In other cases of monocular elevation deficiency, ptosis improves after globe elevation is improved by extraocular muscle surgery, particularly if an inferior rectus muscle restriction has been found at the time of surgery, but also when a full tendon transfer has been done. For these reasons, it is better to wait until the globe position is corrected before correcting the ptosis. Choice of a specific procedure depends on the globe position and on the precise nature of the elevation deficiency.

HORNER'S SYNDROME. Congenital Horner's syndrome is characterized by ipsilateral findings of mild degrees of ptosis, miosis, and, frequently, anhidrosis or flushing of the ipsilateral face. The ipsilateral lower eyelid may be elevated (ptotic), thus further narrowing the palpebral fissure and producing the appearance of enophthalmos. The condition may be a manifestation of neuroblastoma, but it is more likely to be associated with other congenital anomalies or with cardiopulmonary or neck surgery.^39,40 Etiology of many congenital cases remains obscure. Horner's syndrome must be included in the differential diagnosis of anisocoria and all cases of mild ptosis (Fig. 15). Signs of sympathetic stimulation, the most prominent being that of mydriasis, may precede development of classic Horner's syndrome.

The syndrome results from sympathetic denervation of Müller's muscle and other structures that receive sympathetic innervation. Deficient melanocyte development in the involved iris leads to less pigmentation in the eye if the condition is congenital or if it is acquired neonatally (see Fig. 15).

Cocaine will not dilate a Horner's syndrome pupil. Paredrine (Pharmics, Salt Lake City) will dilate the pupil if the third-order neuron is intact.

Horner's syndrome may be treated successfully with a Fasanella-Servat or tarsal Müller's muscle resection. A small levator resection produces equally satisfactory results.

CONGENITAL

The classic ptosis synkinesis syndrome is that of Marcus Gunn jaw-winking phenomenon. In this condition, the motor nerve to the external pterygoid muscle is misdirected to the ipsilateral levator muscle. Lid elevation occurs with mastication or with movement of the jaw to the opposite side (contraction of the ipsilateral external pterygoid muscle; Fig. 16). The condition may occur as an isolated finding or in association with other conditions, such as fibrosis of the extraocular muscles.⁴¹

Treatment is directed toward elimination of lid elevation with mastication, yet providing adequate lid height. The abnormally innervated levator muscle must be detached from the lid to reduce winking. A frontalis suspension procedure then becomes necessary. In cases in which winking is minimal, an external levator resection can be performed, although winking persists in many cases.^42,43 Any residual wink may be accentuated by the higher lid position.

CN-3 palsy may be associated with aberrant regeneration, producing ipsilateral lid elevation on attempted adduction or infraduction of the globe. Constriction of the pupil may occur under the same circumstances. Treatment must be directed toward positioning the lid in the primary position after the globe is repositioned.

Duane's syndrome is produced when the CN-6 fails to adequately innervate a lateral rectus muscle that then acquires a twig of the motor nerve to the medial rectus muscle. Although the synkinesis produced does not involve lid innervation, enophthalmos with apparent ptosis may be produced and may be evident with the eyes in primary position. The upper lid descends further and the lower lid apparently elevates when the eye is adducted because of cocontraction of the horizontal rectus muscles. Treatment is directed toward achieving proper globe position before the problem with lid position is addressed. Levator function usually is excellent in these patients and lid surgery rarely is indicated.

ACQUIRED BLEPHAROPTOSIS

Crushing injuries around the skull and face that result in CN palsies may result in abnormal neurologic connections if reinnervation occurs. Aberrant regeneration of the CN-3 also may occur as a result of brain tumors, aneurysms, and neurosurgical procedures. Vascular complications from diabetes mellitus never produce aberrant innervation.

KEARNS-SAYRE SYNDROME AND CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA

Kearns-Sayre syndrome is a mitochondrial deletion disorder characterized by progressive external ophthalmoplegia, heart block, retinitis pigmentosa and, occasionally, with endocrine and central nervous system manifestations.^44–46 These disorders begin in childhood and patients often present to the ophthalmologist with complaints of blepharoptosis. The conditions are most likely to become symptomatic in the first or second decade. Bilateral ptosis is a prominent feature of the syndromes, and levator function is poor. Systemic associated problems are of primary importance. Frontalis suspension procedures are required for correction of ptosis but limited ocular rotations make corneal exposure risks significant. However, ptosis surgery has functional implications in most cases. Upper lid blepharoplasty has been recommended as a safer alternative to suspension when corneal exposure is expected to be a postoperative problem.⁴⁷

Chronic progressive external ophthalmoplegia may be considered a limited form of Kearns-Sayre syndrome that shares the ocular manifestations but has fewer systemic manifestations. However, this distinction is not helpful in the individual case because affected patients also may have night blindness, cardiac conduction defects, and other systemic symptoms. Progressive external ophthalmoplegia usually begins in the second or third decade and ptosis is a common presenting sign. Ophthalmoplegia develops fairly early and may be accompanied by diplopia in the occasional case in which extraocular involvement is asymmetric. Many systemic associations have been described. Extraocular muscle surgery can be helpful in certain patients.⁴⁸

Myotonic Dystrophy

Myotonic dystrophy shares many of the manifestations of Kearns-Sayre and chronic progressive external ophthalmoplegia syndromes, but, in addition, patients have polychromatic cataracts, gonadal atrophy, premature thinning or loss of the hair, and other systemic problems. Delayed (myotonic) relaxation of skeletal muscles may prolong a hand-shake.

The disorder is believed to be the most common inherited neuromuscular disease in adults. It is an autosomal dominant disorder that is characterized clinically by myotonia and progressive muscular weakness. Myotonic dystrophy may occur congenitally. The disorder has been localized to chromosome 19. Ptosis is a prominent early feature. Myasthenia gravis has been reported to be associated but is considered to be a separate and distinct entity.^49,50

Blepharochalasis

Infiltrative processes that thicken the lids and produce blepharoptosis characterize blepharochalasis. Angioedema frequently involves the upper lids through recurrent episodes of eyelid edema. Edema can be of startling proportions. The condition may be unilateral or bilateral, active or quiescent, with hypertrophic and atrophic forms. Thinning of the levator aponeurosis occurs with repeated attacks until ptosis results. The lacrimal glands may prolapse. Orbital fat also is affected and progressively atrophies. As a result of fat atrophy and ptosis, patients have an emaciated appearance about the eyes. C₁ esterase inhibitor levels may be deficient, particularly if the condition is familial.⁵¹ Because of poor levator function in severe cases, the surgeon may perform a frontalis suspension but the suspension material will be visible beneath the very thin eyelid skin. A surgical procedure on the eyelids may be more hazardous because of the extreme thinning of the lids. The best procedure is an aponeurosis repair in which nonabsorbable sutures are used to reattach aponeurosis to tarsus. Aponeurosis dehiscence may not be as readily visible to the surgeon as in the adult with acquired ptosis. The aponeurosis usually can be found detached from the tarsus and elevated behind the orbital septum which must be unrolled to expose the aponeurosis. Redundant eyelid skin will be evident after the levator is reattached and the lid is repositioned. Excess skin should be excised.

Other causes of blepharochalasis should be treated by treating the underlying disease process or by blepharoplasty procedures.

Myasthenia Gravis

A defect at the myoneural junction that produces relative unresponsiveness to released acetylcholine characterizes myasthenia gravis. This condition may be transient, congenital, and familial. Presenting symptoms are frequently ocular and ptosis is a prominent feature of the disorder.

Several simple office tests have been useful in the office diagnosis of myasthenia.^52–58 The office diagnosis of myasthenia gravis usually is verified with a Tensilon (ICN Pharmaceuticals, Costa Mesa, CA) test, although false-positive, false-negative, and paradoxical responses occur. Preinjection and postinjection measurements of lid position are made. Elevation of the lids in response to the injection indicates the likelihood of myasthenia gravis. Diagnosis also may be made with sophisticated eye movement recording devices or with single fiber electromyography. Additional confirmation of the diagnosis by measurement of serum acetylcholine receptor antibodies can be done but, in our office, this test has been positive in fewer than 50% of our patients with proven ocular myasthenia. Response to treatment is useful diagnostically.

Myasthenia gravis with significant eyelid and eye muscle involvement seldom responds to Mestinon (Roche, Nutley, NJ) alone; often, corticosteroids are required for control of the myasthenia. Even then, restoration of lid position to acceptable levels is not always achieved. Surgical procedures can be helpful in the management of myasthenic blepharoptosis. However, benefits may be temporary, and operative procedures may have to be repeated. Depending on levator function, an external levator resection or a frontalis suspension procedure may be required although the general rule is to be conservative both in the recommendation for and in the performance of surgery.⁵⁹

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