Symposium - Pediatric Ophthalmology andOculoplasty Update Current Techniques in Surgical Correction of Congenital

Felicia D. Allard, Vikram D. Durairaj

ABSTRACT

Ptosis refers to vertical narrowing of the palpebral fissure secondary to drooping of the upper to a lower than normal position. Ptosis is considered congenital if present at birth or if it is diagnosed within the first year of life. Correction of congenital ptosis is one of the most difficult challenges ophthalmologists face. Multiple surgical procedures are available including, frontalis sling, levator advancement, Whitnall sling, frontalis muscle flap, and Mullerectomy. Selection of one technique over another depends on the consideration of several factors including the surgeon experience, the degree of ptosis in the patient, as well as the degree of levator muscle function. Current recommendations for the correction of congential ptosis vary based on clinical presentation. Advantages and disadvantages of each of these procedures are presented with recommendations to avoid complications.

Key words: Complications, Congenital Ptosis, Frontalis Sling, Levator Advancement, Mullerectomy, Ptosis Complications

DOI: 10.4103/0974-9233.63073

BACKGROUND as a component of many congenital syndromes including Duane retraction syndrome, ptosis epicanthus tosis, an abbreviation for the term blepharoptosis, refers inversus syndrome, congenital fibrosis of , Pto vertical narrowing of the palpebral fissure secondary Marcus Gunn jaw winking syndrome, and congenital Horner’s to drooping of the upper eyelid to a lower than normal syndrome. Presently, very few studies have been conducted position. Ptosis is considered congenital if present at birth or to track the prevalence of these genes or the prevalence of if it is diagnosed within the first year of life. Congenital ptosis congenital ptosis. The only large-scale epidemiological data is generally unilateral (70%), but may be bilateral, and can available is from the Section of Ophthalmic Genetics in China.10 be isolated or associated with disease of one or more of the Data from this registry reported a prevalence of congenital ptosis extraocular muscles and/or other systemic conditions.1,2 More of 0.18% (1:552) in this population.10 This data was obtained severe forms may involve hypoplasia of the levator palpebrae from a registry containing information from China only and it superioris muscle or tendon with a minimal or absent eyelid is difficult to predict whether the incidence reflective of other crease.3 Children with this condition suffer from obstructed populations. vision in their upper visual quadrants and frequently require surgery to elevate their [Figure 1]. Congenital ptosis is generally considered a nonprogressive condition; however, it is associated with the development of Several genes that lead to isolated congenital ptosis or a syndrome visual disturbances such as , , , involving congenital ptosis have been identified. These include , ocular torticollis, and . These sequelae PTOS1, PTOS2, and ZFH-4 which lead to autosomal-dominant of ptosis provide a compelling reason to pursue early surgical forms of isolated congenital ptosis.4–9 Ptosis can also be seen correction.11,12 This is particularly true in cases of congenital

Department of Ophthalmology, Division of Oculoplastic and Orbital Surgery, Rocky Mountain Lions Eye Institute, University of Colorado, Aurora, CO, USA Corresponding Author: Vikram D. Durairaj, Associate Professor of Ophthalmology and Otolaryngology -Head and Neck Surgery, Director of Oculoplastic and Orbital Surgery, University of Colorado, Mail Stop F731, 1675 Aurora Court, Aurora, CO 80045, USA. E-mail: [email protected]

Middle East African Journal of Ophthalmology, Volume 17, Number 2, April - June 2010 129 Allard and Durairaj: Surgical Correction of Congenital Ptosis ptosis since clearance of visual axis is essential for prevention of SURGICAL TECHNIQUES visual loss related to amblyopia. After careful examination and establishment of the diagnosis of congenital ptosis, the need Traditional frontalis sling for possible surgical options should be carefully discussed with One surgical approach to patients with congenital ptosis and the parents of a child. poor levator function or congenital Marcus Gunn jaw wink phenomenon is the traditional frontalis sling. This procedure Surgical considerations involves creation of a linkage between the frontalis muscle The original surgical technique for the correction of ptosis and the tarsal and epitarsal tissue of the upper eyelid, which utilized resection of upper lid skin to more effectively allow the allows for a better eyelid position in primary gaze [Figure 2]. frontalis muscle to elevate the eyelid. This method was found This allows eyelid elevation to be performed via the use of to be temporarily successful, failing after the skin stretched and the frontalis muscle, thereby bypassing a poorly functioning eyelid returned to its original position. Failure of skin excision levator.13 Disadvantages of this procedure include the risk of methods led to the modern, muscle-based surgical techniques. and eyelid lag in down gaze.14 Other cosmetic Maintenance of correct eyelid position is an important considerations include scarring in young children, unsatisfactory consideration when selecting a technique to correct congenital geometric tenting of the pretarsal and preseptal skin, loss of ptosis. Other considerations which have shaped the evolution the eyelid crease, and a poor tarso-corneal interface seen upon of these surgical techniques include the need for cosmetically brow elevation and down-gaze. These cosmetic defects may acceptable results, preservation of the normal eyelid crease, be related to the choice of sling material and position of the maintenance of the normal tear film, and prevention of exposure material within the eyelid.13 The recurrence rate of ptosis after keratopathy by prevention of over correction. 20 months postoperatively is 26%.13

Currently, several types of materials are available to create the sling between the frontalis muscle and the eyelid tarsus. Some of these include autogenous or banked fascia lata and alloplastic materials that include chromic gut, collagen, polypropylene, silicone, stainless steel, silk, nylon monofilament, polyester, and polytetrafluoroethylene (PTFE).13 Fascia lata is not always a viable option in all patients as they need to be at least 3 years of age in order to have adequate leg length to provide suitable fascia lata.15 Ben Simon et al.13 reported that PTFE slings produced the lowest incidence of ptosis recurrence (15% compared to 30% from other materials), and that nylon slings produced the best cosmetic results.

Two sling placement patterns have been developed for the Figure 1: A patient with left upper eyelid congenital ptosis frontalis sling method: single loop or double pentagon sling

Figure 2: Intraoperative photo of a patient having frontalis sling suspension of eyelid Figure 3: External levator approach to congenital ptosis repair

130 Middle East African Journal of Ophthalmology, Volume 17, Number 2, April - June 2010 Allard and Durairaj: Surgical Correction of Congenital Ptosis techniques. No difference in recurrence, function, or cosmetic takes advantage of this second support structure of the eyelid. result has been reported between these two commonly This procedure involves resecting the levator aponeurosis up to performed techniques.13 Two incisional approaches have also the point of Whitnall’s ligament (maximal levator resection), and been developed: the eyelid crease incision and the supralash stab then suturing both Whitnall’s ligament and the underlying levator incision. A study comparing the function and cosmetic results muscle to the superior portion of the tarsal plate. If the suturing of these two incisional techniques found that the eyelid crease of Whitnall’s ligament to the tarsus does not provide satisfactory approach yields better results in terms of lid contour and lid lid elevation, a superior tarsectomy may be performed in crease symmetry.16 addition to the sling procedure.22 This approach offers many of the same benefits as aponeurosis advancement: preservation Levator resection and advancement of the levator muscle, Müller’s muscle, and Whitnall’s ligament Resection and advancement of the levator aponeurosis is a without altering the structures that produce the three-layer technique often used in correction of ptosis in patients with tear film. One potential complication of the Whitnall sling is greater than 5 mm of levator function. This technique is mistaking the lower-positioned transverse ligament (LPTL) for performed via the exposure of the levator aponeurosis through Whitnall’s ligament. If the LPTL is mistakenly fixed to the tarsus, an anterior approach, traditionally using an incision running the elevation will be insufficient.23 the entire length of the upper eyelid crease, then advancing the levator aponeurosis by folding or excising the muscle, Eyelid eversion may result if Whitnall’s ligament is advanced and reattaching the aponeurosis to the anterior surface of the too far down the tarsal plate. Other reported complications tarsus [Figure 3]. This method results in an elevation in the include corneal exposure (up to 100% of patients in the contour of the upper lid by effectively shortening the levator early postoperative period), overcorrection, undercorrection, muscle itself.17 This approach has the advantages of preserving poor eyelid contour or crease, lagophthalmos and conjunctival normal anatomical planes and structures of the eyelid as well prolapse.22,24 Undercorrection was seen more often over time in as preservation of all elevating structures, including Mueller’s the reported series.22,24 Anderson et al.22 reported a series of 69 muscle and Whitnall’s ligament.18 This procedure may also be ptotic eyelids that were corrected with Whitnall’s sling without carried out using a trans-conjunctival approach or a small skin tarsectomy. Over a one-year follow-up, 20 out of 65 (30.7%) incision (8–13 mm) which requires minimal local anesthetic of the eyelids which were originally considered satisfactory agent, causing less distortion of the tissue arguably leading to (lid height within 2 mm of the contralateral lid) became better cosmetic results and faster recovery.19 Disadvantages of unacceptable and required reoperation.22 This high incidence the small incision approach are related to the limited view of of late undercorrection seen led to the recommendation that the surgical field. the Whitnall’s sling procedure be augmented with tarsectomy in most cases, especially ones with very poor levator function.22,24 Cates and Tyers et al.20 reported their results from a series of 100 patients (108 eyelids) with congential ptosis and levator function FRONTALIS MUSCLE FLAP of greater than 4 mm who underwent correction via anterior levator advancement. From their study, 76% of unilateral The frontalis muscle flap procedure is recommended for use cases had a successful outcome at 6 weeks postoperatively in cases of severe ptosis with levator function less than 4 mm. (as measured by eyelid level being within 1 mm of each other Historically, this procedure evolved from the frontalis sling side-to-side) falling to 74% at 6 months postoperatively.20 The procedure. The flap procedure involves elevating the innervated most common postoperative side effects at 6 months were frontalis muscle flap, passing it over a pulley created near the undercorrections in 19% of cases and overcorrections in 7% insertion of the orbital septum at the superior orbital rim, of cases.20 In this retrospective study, the authors found that which redirects the pull of the frontalis to elevate the lid, and the factor most predictive of outcome was levator function then attaching the frontalis muscle to the tarsal plate.15 The with increasing levator function leading to increasing risk of advantages of this technique include eliminating the need for overcorrection.20 alloplastic or autologous tissue to connect the caudal portion of the frontalis muscle to the eyelid as well as improving the WHITNALL SLING direction of the pull.15 In this case, the pulley lifts the eyelid toward the brow rather than along the surface of the .15 The Whitnall sling procedure is used to correct severe ptosis Additional advantages over the frontalis sling procedure include with levator function of 3–5 mm. The Whitnall’s ligament acts minimal ptosis on upward gaze, less lid lag on downward gaze, as a support structure for the upper eyelid and superior preservation of eyelid contour and reduced tendency for the by functioning as a superior suspensory ligament for the eyelid eyelid to pull away from the eye. This procedure may also and lacrimal gland and a pulley off of which the levator muscle be performed on patients at a younger age than a traditional gains support and direction.21,22 The Whitnall sling procedure frontalis sling because the frontalis muscle is well-developed

Middle East African Journal of Ophthalmology, Volume 17, Number 2, April - June 2010 131 Allard and Durairaj: Surgical Correction of Congenital Ptosis by two years of age whereas fascia lata maturation may take an arguably one of the most difficult challenges for ophthalmologists. additional year.25 Many authors have reported difficulty not only with adequately correcting ptosis, but also with functional and cosmetic results. Complications of frontalis muscle flap include: (1) transient There are currently several surgical options for the treatment postoperative forehead anesthesia with spontaneous recovery, of congenital ptosis. Selection of one technique over another (2) eyebrow asymmetry, (3) reduced eyelid excursion with will depend on consideration of several factors including the extreme upward and downward gaze, (4) lagophthalmos, and experience and comfort level of the surgeon with various (5) overcorrection possibly due to the frontalis muscle being techniques, the degree of ptosis in the patient, as well as the stronger than the levator muscle. In one report that followed degree of levator muscle function. Current recommendations for 42 patients, no was reported; however, the correction of congential ptosis vary by clinical scenario. In eyelid asymmetry was seen in 14% of patients, more frequently children less than 3–4 years of age in which levator function is in unilateral cases than in bilateral cases.15 Similar results were poor, the frontalis sling procedure is recommended. In children reported in smaller studies.25,26 One study did report a single with less than 3 mm of levator function, options include the case of lagophthalmos complicated by corneal erosion.27 frontalis sling, Whitnall sling (for very poor levator function),22 and dynamic frontalis muscle flap.15 In patients with more than MULLERECTOMY 5 mm of levator function, levator resection and advancement may be used.20,22 For patients in which primary correction has Müller’s muscle-conjunctival resection surgery is another failed, Whitnall sling procedure with or without tarsectomy technique that may be used to advance the levator aponeurosis is recommended after failed frontalis sling,2 and frontalis of the upper eyelid to correct ptosis. The success of this procedure sling procedure is recommended for re-correction after failed is dependent upon the combined function of the Muller’s muscle Whitnall sling.22 and levator muscle hence use recommended in patients with fairly good muscle function.28 The Muller’s muscle is an involuntary, REFERENCES sympathetically innervated muscle that originates below the levator aponeurosis just distal to the Whitnall’s ligament. It attaches to 1. Smith B, McCord CD, Baylis H. Surgical treatment of the superior tarsal border by a small tendon and is responsible blepharoptosis. Am J Ophthalmol 1969;68:92-9. 29 2. Sakol PJ, Mannor G, Massaro BM. Congenital and acquired for an estimated 2–3 mm of eyelid elevation. This particular blepharoptosis. Curr Opin Ophthalmol 1999;10:335-9. procedure is recommended for patients who respond well to 3. Guercio JR, Martyn LJ. Congenital malformations of the eye the phenylephrine test, thereby shortening a responsive Muller’s and orbit. 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