Fig. 18.1
This patient had an initial ptosis repair at 5 months of age with a frontalis suspension procedure on the left upper eyelid. (a) Preoperative and (b) postoperative appearance of the eyelid. The skin was left intact at the time of her initial procedure, but when she returned at 5 years of age (c), it was elected to replace her silicone cable to re-elevate the eyelid, and at that time, the overlapping tissue in the lid fold was removed and lid crease reformation was performed to increase the symmetry between the two eyelids (d)
At the time of the procedure, the ptosis repair is performed first, and then with the eyelid in the new position, the upper skin is draped over the newly positioned eyelid, marking the overlap of the tissue along the eyelid crease with a skin marking pen. This redundant tissue is considered for possible resection. When the excess tissue has been removed, lid crease reformation is also performed to reestablish the connections between the levator muscle and the newly created lid crease. Even in cases of poor levator function, the scar from the lid crease reformation sutures helps to create the illusion of an eyelid crease.
Efforts to match the eyelid crease are also important aesthetic considerations in pediatric ptosis repair. The eyelid crease is formed by anterior attachments of the levator aponeurosis to the underside of the skin. In poor function ptosis or complete aponeurotic disinsertion, the eyelid crease may be indistinct or abnormally high on the involved lids. If one side has a more normal eyelid crease, this height may be used as the benchmark, and the lid incision is matched and marked on the contralateral eyelid. If both lid creases are indistinct, then preoperative measurements are used to set the height of the crease and the location of the incisions. Calipers are useful for locating and marking the planned incisions. If the crease is indistinct or requires modification, then techniques for eyelid crease reformation are used prior to and during closure. The hallmark of these techniques is creation of a purposeful scar attachment that will mimic the natural attachments between the skin and the levator aponeurosis to create the eyelid crease. Placing polyglactin 910 braided suture between the levator complex and the underside of the skin with a buried suture (buried interrupted 6.0 Vicryl {Ethicon, Cincinnati, OH} on a P-1 needle) is one effective lid crease reformation technique. The lid crease may also be magnified during skin closure by placing sutures from the skin to the levator aponeurosis and then to the skin on the other side of the incision. This also creates an adhesion between the skin and the levator complex. Either technique may be used individually, or both techniques may be used on the same eyelid when a very distinct lid crease is desired.
Asymmetric ptosis with poor levator function seen in concert with a contralateral relatively well functioning levator muscle represents one of the more convoluted surgical decision-making processes in congenital ptosis. The conservative choice will be to operate to raise only on the abnormal eyelid and accept compromise, recognizing that there is no mechanical way to get the two eyelids to move similarly. A more controversial but potentially better cosmetic choice would be to recommend that the frontalis suspension procedure be done on the poorly functioning lid and also be performed on the relatively normal eyelid along with extirpation of the levator muscle on the less ptotic side [4]. Crowell Beard described this technique in 1965. In patients with poor levator function and especially when there is amblyopia on the ptotic side, activation of frontalis drive on the more ptotic side may be difficult to achieve when the relatively normal eye and eyelid are working well without effort. In essence, using the bilateral technique, the surgeon is removing the better functioning levator muscle in a quest for improved symmetry and to require the use of the frontalis muscle to lift either eyelid. This requires discussion and consideration by the parents and the surgeon. A variation of this procedure termed the “Chicken Beard” was described by Alston Callahan [5] where the frontalis suspension is performed bilaterally but the levator muscle on the less ptotic eyelid remains intact.
Recognition that true unilateral congenital ptosis is a rare condition and that more commonly there is some alteration of function in the eyelid that appears to be more normal is also a useful concept in managing congenital ptosis. Sometimes better symmetry may be achieved by performing bilateral surgery, combining frontalis suspension on the more ptotic lid and conjunctival Mueller’s muscle resection ptosis repair on the lid that appears to be the less ptotic lid. This could be nicknamed the “Chicken Katowitz.”
In some adolescents or even adults, mild ptosis that never mandated repair for visual distortion may present for relatively cosmetic repair or a repair focused mainly on appearance rather than preventing visual distortion. Recognition that this is still congenital ptosis with all the inherent differences in lid elasticity and muscle function (despite the patient’s age and mild malposition) is important because the techniques and premises for repair remain those of congenital ptosis. Relatively decreased eyelid excursion and increased palpebral fissure in downgaze are clues to the congenital nature of the problem in the previously undiagnosed adolescent or young adult. Failure to recognize these differences may result in undercorrection of the ptosis if the repair is performed as if this were an acquired ptosis. Another consideration in initial ptosis repair in the older or adolescent child is toleration of lagophthalmos and nighttime exposure. Increased age at the time of initial congenital ptosis repair may also be a risk factor for postoperative problems with exposure. The older children and adolescents need a longer adaptation period with increased lubrication when adjusting to postoperative lagophthalmos. Eyelid crease symmetry remains an important consideration when operating on the otherwise mature patient with congenital ptosis. The same indistinct lid crease is seen and lid crease formation is indicated (Fig. 18.2).
Fig. 18.2
Features of congenital ptosis seen in an adult requesting surgery for ptosis of the left upper eyelid. (a) The patient’s photo as a child. (b) The patient presents as an adult requesting surgery for the droopy left upper eyelid. Note the lid crease and fold are also less distinct on the left. Lid crease reformation was performed in association with the external levator resection to improve the postoperative contour of the eyelid. Seven years later, her lid position and crease are stable (c). Note the decreased lid excursion of the ptotic lid in downgaze typical of congenital ptosis (d)
In Marcus Gunn jaw wink ptosis, in addition to the ptosis, there is also a synkinetic abnormal elevation of the upper eyelid that occurs with activation of other facial musculature, typically the muscles of mastication. This abnormal eyelid movement and sometimes frank retraction of the eyelid with oral movement can be an aesthetically detracting feature of this variant of congenital ptosis. Correction of the ptosis and tightening of the levator muscle alone will not correct the overshoot of the eyelid that occurs with eating and speaking. The magnitude of the “wink” is helpful in deciding the technique of ptosis repair. The greater the magnitude of the overshoot, the more important it is to decrease the impact of the levator muscle on eyelid position. For example, in a very mild overshoot, ptosis repair alone may be considered. More commonly, a frontalis suspension procedure is used to elevate the eyelid, and the levator aponeurosis is disinserted from the anterior surface of the tarsus to diminish the synkinetic movement. If the overshoot is more profound, the levator complex can be released and then partially removed to help prevent reattachment and recurrence of the noticeable overshoot.
Cosmetic Considerations in Infantile Hemangiomas
Infantile hemangiomas present a significant cosmetic concern when they develop on the visible skin of the face or eyelid. Ophthalmic intervention is performed for mechanical ptosis, anisometropic amblyopia, or significant disruption of globe position. The full discussion of management of infantile hemangiomas is covered in Chap. 23. Beyond the medical aspects of vision protection, the color change or physical distortion in shape from the hemangioma is also cosmetically disturbing. When interventions have a relatively low incidence or severity of side effects, treating the lesion for cosmetic purposes may be reasonable. Early intervention may also diminish some of the long-term skin changes. The physician weighs the potential side effects associated with treatment against the social and aesthetic gains of a more normal appearance. In this disorder, the physician and family also consider that as the infant matures, the infantile hemangioma is likely to show spontaneous involution.
Topical or systemic beta-blockers (propranolol and timolol) have been used to successfully diminish the color and the bulk of the infantile hemangiomas. When successful, and in the absence of systemic side effects, propranolol is an excellent first-line treatment for infantile hemangioma. Other treatment modalities are also considered when there is limited response, side effects occur, or if systemic conditions such as cardiac disorders or asthma preclude the use of propranolol. Surface laser is successful in diminishing what may be the most striking feature of the hemangioma, the color change in the skin [6, 7]. Laser (pulsed dye laser) can be used to superficially close the abnormal vascular lesions resulting in a much more normal appearance and also potentially diminishing some of the long-term impact of the lesion on the developing skin (Fig. 18.3). This technique is very helpful in decreasing the superficial component of the hemangioma, but the deeper mass remains unaffected. In beta-blocker unresponsive hemangiomas or in hemangiomas treated prior to propranolol therapy, systemic and intralesional steroids are and were employed. From an aesthetic perspective, skin changes from the lesion and also as side effects of the intralesional steroids may result in areas where the skin becomes atrophic or shows a loss of elasticity after regression (cigarette paper skin). Resection of these abnormal areas may improve the aesthetic outcome (Fig. 18.4). After involution, areas of irregularity or fibrosis may also persist in the location of the previous hemangioma. Appearance-based resection of the altered skin or residual mass is usually undertaken when the lesion is felt to have involuted.
Fig. 18.3
In this figure, we see a patient whose infantile hemangioma was only moderately responsive to oral propranolol. Frontalis suspension was performed to elevate the eyelid margin and laser was performed to decrease the impact of the hemangioma. (a) The patient shortly after presentation. Propranolol and laser treatment are initiated as well as patching for amblyopia (b). A frontalis suspension procedure for the mechanical ptosis is performed at the time of additional laser treatment. Note the purpuric effects of the laser in (c). (d) The patient at age 5 after the treatments described above
Fig. 18.4
In the pre-beta-blocker era, vision-threatening hemangiomas were treated with systemic and intralesional steroids. Systemic side effects and also local effects could occur. This patient had an occlusive infantile hemangioma (a). Systemic steroids diminished the capillary hemangioma and the eyelid opened but also resulted in Cushingoid changes (b). Intralesional steroid-induced atrophy and involutional changes were apparent in the skin of the eyelid, brow, and forehead (c). Partial resection of the atrophic areas improves the appearance (d)
Cosmetic Considerations in Congenital Microphthalmos and Surgically Induced Anophthalmos in Children
The ongoing treatment of the child with microphthalmos has long-term goals of successful retention of a symmetrically sized ocular prosthesis and induction/maintenance of the normal eyelid and facial growth and development. Many factors influence the success of intervention: the initial degree of maldevelopment, the age of onset of treatment, the enthusiasm and endurance of the patient’s family, and the availability of a skilled ocularist. In surgically induced loss of the eye during childhood, the starting point is usually one of more normal proportions, but removal of an eye in childhood also has similar concerns with respect to orbital volume replacement to try to create growth and symmetry of the affected side.
The treatment options and interventions for microphthalmos are covered in Chap. 40. These techniques are employed to try to compensate for the retarded growth of the soft tissues on the affected eyelids and replace the soft tissues that are missing in the orbit. Bone growth is often diminished as well. Successful treatment must start with increasing the horizontal and vertical aperture of the eyelids. In mild cases of microphthalmos, the skilled ocularist is sometimes able to improve the cosmetic appearance with an ocular prosthesis alone without requiring any surgical intervention. When the eye is small and the palpebral aperture is tiny, expansive materials such as hydrogel may be employed to increase the opening of the lids, and then increasing size rigid conformers fabricated by the ocularist help to expand the surface area of the socket and create mechanical pressure on the enlarging eyelids. Eyelid growth must be induced rather than surgically fabricated. As the eyelid aperture and socket surface area and the concavity of the space behind the eyelids are increased, the size of the conformer that can fit through the eyelids and fill the space may no longer create pressure on the eyelids. At this point, adding orbital volume to the soft tissue behind the conformer may create resistance to allow the prosthesis to push forward and stretch the eyelids. The earlier in development these techniques are utilized, the easier it is to take advantage of the plasticity of growth. Tissue expanders of various types have been used to successfully increase orbital volume and are sometimes ultimately replaced with living tissue such as dermis fat grafts. Dermis fat grafts in the younger child may act as the ultimate tissue expander by continuing to grow after implantation [8].
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