248 MARCUS GUNN SYNDROME

Syndrome Gunn Marcus • 248 CHAPTER

Eyelids • 21 SECTION

completely removed from the lid and attached to the superior orbital rim at the arcus marginalis or extirpated.

DIAGNOSIS

●The initial presentation typically occurs soon after birth, when the infant begins to nurse. The ptotic lid is noted to rhythmically jerk upward.

●The retraction is most commonly demonstrated by opening and closing the mouth or by moving the jaw from side to side.

●It is rarely demonstrated by coughing, smiling, the Valsalva maneuver, swallowing, inspiration, or contraction of the platysma muscle.

●Several series have reported significant associations with other ocular problems, including strabismus, anisometropia, amblyopia, and Duane’s syndrome. The frequency of strabismus has been reported to be as high as 58% and includes superior rectus palsies, double elevator palsies, and horizontal deviations. Trigeminal-Abducens synkinesis has been reported.

TREATMENT

If amblyopia is present, it must be treated vigorously, and anisometropia must be corrected if it is significant.

Because the patient with Marcus Gunn syndrome brings to the clinician two separate problems (ptosis and jaw winking), the satisfactory elimination of both often is a challenging endeavor. In the evaluation of a patient, one must decide which component is more cosmetically intolerable-only then can the surgical plan be tailored accordingly and the best results obtained. A discussion with the patient or parents is important to ensure that they have realistic expectations and are aware that a compromise may be necessary.

In patients with very mild ptosis (less than 2 mm) and mild jaw wink (3 to 4 mm), one can perform a Müller’s muscle conjunctival resection as described by Putterman or a levator resection; even no treatment may be acceptable. If the ptosis is moderate and the jaw winking is minimal, a levator resection is the treatment of choice. Undercorrection of the ptosis has been a complication of this approach, and it is recommended that the resection be greater (4 to 5 mm more) than in the usual case of congenital ptosis. More predictable elevation may be achieved with a combined levator resection, Muellerectomy and conjunctivotarsectomy from an anterior approach. Unfortunately, an exacerbation of the jaw wink may occur because this procedure strengthens the levator muscle and the eyelid excursion then begins at a higher level.

When the jaw-winking component is severe, a levator resection exacerbates the wink. In this case, the jaw wink must be obliterated through release of the levator aponeurosis followed by a frontalis suspension. Dryden and coworkers described a reversible technique of suturing the levator aponeurosis to the arcus marginalis of the upper orbital rim to ensure its deactivation. Bilateral levator release with fascia lata suspension of both eyelids is the procedure of choice because the often-marked asymmetry of unilateral surgery is avoided. Bilateral frontalis suspension combined with only unilateral levator excision of the affected eye also is advocated (‘chicken-beard’ operation). If the patient or parents refuse bilateral surgery, the frontalis suspension may be performed on the affected eye with or without prior levator release.

To effect a levator release, a skin crease incision is made, and a small amount of preseptal orbicularis is resected. The underlying septum is identified, and nearly its entire length is incised horizontally. This allows identification of the levator aponeurosis, which is released from the tarsus and attached to the arcus marginalis with multiple 5-0 Dacron sutures. Care should be taken to avoid the supraorbital neurovascular bundle. A frontalis sling of the surgeon’s choice can then be performed. A double rhomboid as described by Beard is an excellent choice in this setting.

In cases in which the wink is not prominent and the levator does not have to be released, a transconjunctival frontalis suspension is an excellent option. Before the age of 3, the use of a synthetic sling is suggested, such as silicone rods or tubing. Banked fascia can be used, but in the authors’ experience, this material has not been satisfactory. After the age of 3, the child’s fascia lata has developed sufficiently to enable harvesting from either the midthigh or the hip area.

A stab incision is made at the upper edge of the brow where elevation of the eyelid by the surgeon’s finger gives the most pleasing cosmetic appearance. This incision need not be carried down to the periosteum. A small amount of skin undermining will ensure good closure over the fascia or silicone knot later. The eyelid is then everted over a Desmarres retractor and held in place with a forceps. A fascia lata strip measuring approximately 3 mm in width and 12 to 15 cm in length is loaded on a half-circle, reverse-cutting needle (Richard Allen no. 2164-4) and passed horizontally through the lid just anterior to the superior tarsal border. This horizontal limb should measure approximately 6 to 10 mm depending on the patient’s size. An attempt is made to incorporate the tarsal plate. The needle is then placed back in the lid, where it has just exited, and driven in a slightly posterior direction to an area immediately behind the superior orbital rim, ‘walked’ anteriorly over the rim, and brought out through the brow incision. Before this vertical fascia limb is pulled through, a small piece of 4-0 silk is placed as a marker suture at the junction of the horizontal and vertical limbs to facilitate removal of the vertical portion should repositioning of the fascia be necessary. The needle is reloaded with the opposite end of the fascia, brought through the entry site of the horizontal limb, and then directed superiorly to the brow incision in the same fashion. The result is a triangle-shaped sling with its base at the anterosuperior tarsal margin. The two vertical limbs can now be elevated to check lid margin height, contour, and symmetry. Any adjustments are made, and then the fascia is secured in the frontalis with a square knot. A 5-0 Dacron is sewn through the knot to ensure it will not slip. At this point, a good lid crease should be present, and the lid margin should be 1 to 2 mm above the desired postoperative level and in apposition with the globe.

Several modifications of the frontalis suspension have been advocated. The author favors the transconjunctival suspension in cases in which the levator has not been released. The advantages of this approach include a lesser chance of infection because it avoids low anterior skin incisions near the lash line, avoidance of visible traction lines below the skin, and facilitation of crease formation because the fascia is placed posteriorly to the orbital septum. In addition, normal attachments of the levator to the tarsus and skin are preserved so that when the frontalis muscle elevates the brow, the lid is indirectly raised and normal lid margin contour and apposition to the globe are maintained. This procedure is also inherently safer because the needle used to pass the fascia lata is never directed toward the eyeball, therefore minimizing the chance of ocular penetration.

In cases of unilateral ptosis in which the levator is released, a promising technique involving the advancement of a flap of frontalis muscle to the superior tarsus has been successful. The long-term results have not been published, but it appears to be far more successful than the corrugator muscle transpositions used in the 1960s. When an anterior approach is performed, the sling material is sutured to the tarsal surface. The Richard Allen needle is used to pass the free ends in a post-septal plane and out the suprabrow incision in the same manner as the transconjunctival approach. Fixation sutures may be used to evert the eyelashes prior to skin closure to avoid lash ptosis that can occur with frontalis suspensions.

COMMENTS

The jaw-winking syndrome is one type of congenital ptosis of a synkinetic nature that classically consists of ptosis of the upper eyelid associated with involuntary retraction of the lid during contraction of the ipsilateral pterygoid muscle. In general, the wink tends to become less noticeable with age, so if it is minimal and the patient has more than 4 mm of levator function, a standard levator resection should be highly successful. In cases of poor levator function, a frontalis suspension of some type should be used; if the wink is severe, the levator should be released from the tarsus and tacked to the arcus marginalis, followed by frontalis suspension.

REFERENCES

Beyer-Machule CK, Johnson CC, Pratt SG, Smith BR: The Marcus Gunn phenomenon. Orbit 4:15, 1985.

Bullock JD: Marcus Gunn jaw-winking ptosis: classification and surgical management. J Pediatric Ophthalmol Strabismus 17:375, 1980.

Dailey RA, Wilson DJ, Wobig JL: Transconjunctival frontalis suspension (TCFS). Ophthalmol Plast Reconstr Surg 7, 1991.

Darcet TW, Crawford JS: The quantification, natural course, and surgical results in 57 eyes with Marcus Gunn (jaw-winking) syndrome. Am J Ophthalmol 92:702–707, 1981.

Dryden RM, Fleuringer JL, Quickert MH: Levator transposition and frontalis sling procedure in severe unilateral ptosis and paradoxically innervated levator. Arch Ophthalmol 100:462, 1982.

Kodsi S: Marcus Gunn jaw winking with trigemino-abducens synkinesis. J AAPOS 4:316–7, 2000.

249 MELANOCYTIC LESIONS OF

THE EYELIDS 172.1

(Ephelis, Lentigo, Nevocellular Nevus,

Dermal Melanocytosis, Malignant

Melanoma)

Steven A. McCormick, MD, FACP

New York, New York

Tatyana Milman, MD, FAAO

New York, New York

ETIOLOGY

Benign melanocytic lesions of the eyelids are a diverse group of congenital and acquired lesions derived from neural crest mela-

nocytes. Three distinct cells of origin-epidermal melanocytes, nevocellular nevus cells, and dermal melanocytes-result in a variety of clinically distinct pigmented lesions of the skin. Classification schemes should distinguish among these three cell types and the location of origin. All of these cells have the capacity to synthesize and excrete melanin, the primary cutaneous pigment.

CLINICAL DIAGNOSIS

Examination should be performed in good light; the wide beam of the slit lamp is ideal. Size, location, and description of the lesion (height, surface and border characteristics, color, and so on) should be carefully recorded. The goal of the examination is to determine whether the lesion is likely to be a benign or malignant melanocytic lesion or another pigmented lesion of nonmelanocytic origin, such as seborrheic keratosis or pigmented basal or squamous cell carcinoma. Photographs of suspicious lesions are helpful in documenting the growth or changes in coloration that may signal malignancy.

The clinicopathologic features of these entities are discussed to assist the examiner in establishing the correct clinical diagnosis and in affecting appropriate management.

BENIGN LESIONS

Benign lesions derived from the epidermal melanocytes include the ephelis (common freckle), lentigo simplex, and solar lentigo (‘age spot’). All commonly affect the periocular skin, as well as skin elsewhere. Ephelides are not truly tumors, but are perhaps the most common of all skin lesions, affecting all whites to some degree, as well as pigmented races. Solar lentigines are also common in whites with excessive sun exposure.

Benign tumors composed of nevocellular nevus cells are referred to as melanocytic nevi (clinically, the simple term nevus is usually applied). Nevi are classified as junctional, intradermal, or compound based on the histologic location of the nevocellular proliferation. Most of the brown to black coloration results from melanin in the superficial skin, whereas tumefaction results from a dermal component. Location in the basal epidermis (junctional component) usually imparts a darker color (black to brown) than when the proliferation is limited to the dermis (intradermal nevus). When nevus cells are present in both locations, the term compound nevus is applied. Common nevi tend to evolve throughout life, beginning usually as pigmented macules or papules, increasing in height and/or pigmentation (especially under the hormonal influences of puberty or pregnancy), and finally regressing with depigmentation, fat infiltration, and fibrosis. Some larger nevi may harbor hair follicles or have a papillomatous surface.

Most nevi are noted at birth or in early childhood, and it is often difficult to determine whether a given lesion is congenital or acquired. Unlike ‘typical’ nevi, large congenital nevi (1 cm or more in diameter and evident at birth) are treated aggressively because of their propensity to give rise to malignant melanoma in approximately 12% of the cases. An example of congenital nevus affecting the eyelids is the ‘kissing’ nevus, which develops in utero before the normal development of the eyelid fissure. The nevus, thus, is located on apposed areas of the upper and lower eyelids. These, like other bulky nevi, are usually removed for cosmetic or mechanical reasons.

Eyelids249the ofCHAPTERLesions Melanocytic •

Eyelids • 21 SECTION

ranging from pink/red to brown/black, whereas nevi tend to be uniformly pigmented. Acquired nevi tend to be less than 5 mm in diameter; malignant lesions are usually larger. However, other pigmented, nonmelanocytic lesions (e.g. seborrheic keratosis and keratoacanthoma) may be large. Change in clinical appearance and symptoms (i.e. evolution) of the lesion are uncommon in nevi, but are frequently observed in cutaneous malignant melanoma. The ‘ABCDE rule’ of clinical signs is a helpful mnemonic: asymmetry, border irregularity, variation in color, diameter of more than 5 mm, and evolution.

Lentigo maligna (Hutchinson’s freckle) may be considered a premalignant lesion that has a natural history unlike the other types of malignant melanoma. It is a relatively common finding on the face in elderly Caucasian individuals, and often involves the lower eyelid and lateral canthal region. Lentigo maligna begins as one or more pigmented patches with irregular borders, and enlarges slowly (over several years). As the lesion expands, the pigmentation may change as some areas regress and others proliferate. Lentigo maligna may, after this protracted in situ phase, progress to lentigo malignant melanoma, probably the most common melanoma type affecting the eyelid skin. The appearance of nodularity within the lesion is often an indicator of progression to the invasive stage. The risk of progression appears to be low (variable lifetime risk figures are quoted: from 5% to 50%).

Malignant melanoma of the periocular skin, arising de novo or within a pre-existing acquired or congenital nevus, is classified as either superficial spreading melanoma or nodular melanoma. Superficial spreading melanoma, the most common melanoma subtype of the head and neck region, begins as a brown to gray or rose-colored, minimally elevated in situ lesion with irregular, notched borders. Unlike with lentigo maligna, the lesion is rarely more than a few centimeters in its radial growth phase and becomes minimally invasive much earlier (typically within 1 year). It is important to recognize and excise the lesion at this stage. Induration, elevation, and mottling are early signs of invasion.

Nodular malignant melanoma begins as an elevated, often darkly pigmented tumor that increases in bulk rapidly and, unlike superficial spreading melanoma, often ulcerates and bleeds. A radial growth phase is not usually recognized; an in situ lesion is not included in the classification. Because the vertical invasion occurs early, nodular melanoma usually presents with a smaller diameter than superficial spreading melanoma (often less than 1 cm). As a result of its early and aggressive invasive growth, this type of cutaneous melanoma carries the worst prognosis.

Staging schemes separate patients with local disease only (stages I and II), with local nodal metastases (stage III), or with distant nodal or visceral metastases (stage IV). Approximately 80–90% of cases are diagnosed in stages I and II, with a 5-year survival rate of around 90%. Prognosis in localized disease is gauged on the depth of invasion as measured microscopically. Breslow found that tumors of less than 0.76 mm in thickness (measured from the granular cell layer of the epidermis to the deepest extent of invasion) did not metastasize (and therefore did not require regional node dissection). In patients with tumors of more than 1.5 mm, survival rates are doubled if regional lymphadenectomy is performed. Sentinel lymph node biopsy is currently recommended for localized lesions with tumor thickness greater than 1 mm; if positive, the biopsy is followed by regional lymph node dissection. Prognosis is poor for stages III and IV; 5-year survival rates are reported as 60% for regional (stage III) disease, and 14% for distal metastatic

disease. In regional disease, the number of involved lymph nodes, tumor burden within the lymph nodes, and ulceration within the primary tumor are important predictors of survival.

Lentigo maligna and lentigo maligna melanomas

●Etiology: may occur as a result of chronic, cumulative sun exposure; atypical melanocytes proliferate in the basal regions of the epidermis in Hutchinson’s freckle; invasion in 5%–50% (lenitgo maligna melanoma), but usually only after many years of evolution.

●Clinical findings: slowly enlarging irregular pigmented macule or macules with irregular borders that evolve over several years; frequently involve the lateral canthal region and lower eyelid; induration, elevation, and change in coloration possible signs of invasion.

●Treatment: excision, especially if nodularity develops within the macule.

Malignant melanoma (superficial spreading or nodular)

●Etiology: may be caused by episodic, intense sun exposure; malignant transformation of basal epidermal melanocytes (or less commonly, malignant degeneration of nevus cells); growth proceeds from an in situ phase (recognized in superficial spreading melanoma) to invasive growth, which proceeds more rapidly in nodular melanoma.

●Clinical findings: the ABCDE rule (asymmetry, border irregularity, variation in color, diameter of more than 5 mm, evolution) is useful; pigmented macules with these features should be considered suspicious for superficial spreading melanoma; nodular melanomas present as enlarging, elevated lesions with variable dark pigmentation; evolution may be noted in up to 80%; induration and changing pigmentation are harbingers of malignancy.

●Treatment: excisional biopsy of suspicious lesions, preferably with up to 1-cm margins of resection; incisional biopsy of large lesions if the diagnosis is in question; sentinel lymph node biopsy for lesions with tumor thickness greater than 1 mm; regional lymph node dissection if positive nodes are identified.

SUMMARY

Pigmented lesions of the eyelid and periocular skin present a challenge to the ophthalmologist, but the importance of recognizing suspicious tumors cannot be overemphasized. Lesions removed early in their course carry an extremely favorable prognosis, and surgical extirpation in the facial region is accomplished with limited cosmetic effects when lesions are small. The task of the clinician is to differentiate among benign, premalignant, and malignant lesions with the use of clinical examination and review of the patient’s historical observations of the lesion. The clinician must also recognize that several nonmelanocytic lesions may present as pigmented tumors; these include pigmented basal cell carcinoma, actinic keratosis, keratoacanthoma, seborrheic keratosis, and dermatofibroma. Suspicious lesions should be biopsied.

REFERENCES

Abbasi NR, Shaw HM, Rigel DS, et al: Early diagnosis of cutaneous melanoma: revisiting the ABCD criteria. JAMA 292(22):2771–2776, 2004.

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Eyelids • 21 SECTION

Balmaceda CM, Fetell MR, O’Brien JL, et al: Nevus of Ota and leptomeningeal melanocytic lesions. Neurology 43(2):381–386, 1993.

Clark WH, Elder DE, Guerry D, et al: A study of tumor progression: the precursor lesions of superficial spreading and nodular melanoma. Hum Pathol 15:1147–1165, 1984.

Cramer SF: The histogenesis of acquired melanocytic nevi. Based on a new concept of melanocytic differentiation. Am J Dermatopathol 6 Suppl: 289–298, 1984.

Cramer SF: The origin of epidermal melanocytes: implications for the histogenesis of nevi and melanomas. Arch Pathol Lab Med 115:115–119, 1991.

Crowson AN, Magro CM, Sanchez-Carpintero I, et al: The precursors of malignant melanoma. Recent Results Cancer Res160:75–84, 2002.

Elder DE, Murphy GF: Atlas of tumor pathology: melanocytic tumors of the skin (third series, fascicle 2). Washington, DC, Armed Forces Institute of Pathology, 1991.

Grossniklaus HE, McLean IW: Cutaneous melanoma of the eyelid: clinicopathologic features. Ophthalmology 98:1867–1873, 1991.

Kienstra MA, Padhya TA: Head and neck melanoma. Cancer Control 12(4):242–247, 2005.

Le AD, Fenske NA, Glass LF, et al: Malignant melanoma: differential diagnosis of pigmented lesions. J Fla Med Assoc 84(3):166–174, 1997.

Lever WF, Schaumberg-Lever G: Histopathology of the skin. Philadelphia, JB Lippincott, 1990, pp. 756–805.

Lopransi S, Mihm MC: Clinical and pathological correlation of malignant melanoma. J Cutan Pathol 6:180–194, 1979.

Rager EL, Bridgeford EP, Ollila DW: Cutaneous melanoma: update on prevention, screening, diagnosis, and treatment. Am Fam Physician 72(2):269–276, 2005.

Spencer WH (ed): Ophthalmic pathology: an atlas and text. Philadelphia, WB Saunders, 1996:2261–2278.

Stevenson O, Ahmed I: Lentigo maligna : prognosis and treatment options. Am J Clin Dermatol 6(3):151–164, 2005.

Vaziri M, Buffam FV, Martinka M, et al: Clinicopathologic features and behavior of cutaneous eyelid melanoma. Ophthalmology 109(5):901– 908, 2002.

250 ORBITAL FAT HERNIATION 374.34

(Eyelid Fat Prolapse, Orbital Fat

Prolapse, Adipose Palpebral Bags,

Baggy Eyelids)

Eric A. Steele, MD

Portland, Oregon

Roger A. Dailey, MD

Portland, Oregon

ETIOLOGY

Fullness of either the upper or lower eyelid can result from the herniation of orbital fat through the orbital septum, fluid retention or accumulation in the periocular tissues, prolapse of the lacrimal gland, or excess skin (dermatochalasis). Orbital fat herniation results from atrophy and dehiscence of the orbital septum allowing for forward prolapse of the orbital fat pads. In the upper eyelid, orbital fat prolapse may contribute to mechanical ptosis, as well as pose cosmetic concerns in conjunction with dermatochalasis. In the lower eyelid, fat prolapse will result in cosmetic deformity based on underlying bony anatomy and the status of other mid facial soft tissues.

●The thin nature of the orbital septum allows hereditary or involutional herniation of orbital fat.

●Thyroid immune-related orbitopathy may increase the volume of fat and lead to prolapse in the upper and lower eyelids.

●Posterior sub-Tenon injection of triamcinolone may cause ptosis and/or orbital fat prolapse.

DIAGNOSIS

The diagnosis is made on the basis of careful general medical and ophthalmologic history and clinical examination. An examination of eyelid fullness is made by palpation and ballottement of the globe with observation of the fat pads.

Differential diagnosis

●Dermatochalasis.

●Prolapsed lacrimal gland.

●Eyelid edema or blepharochalasis.

●Thyroid immune related orbitopathy.

●Festoons or cheek bags.

All of the above may be present in conjunction with orbital fat prolapse.

TREATMENT

Local

●Make-up applied by a skilled aesthetician may slightly improve the cosmetic appearance.

Surgical

Upper eyelid

●Surgical intervention in the upper eyelid will often involve simultaneous treatment of dermatochalasis.

●Surgery is performed most commonly with the patient under local or monitored anesthesia. After infiltration with a local anesthetic agent containing epinephrine, the amount of skin to be excised is marked. The lower border of skin excision coincides with the eyelid crease.

●Incision through skin and muscle is made with a scalpel or CO2 laser. Skin and muscle are excised either in one flap or separately. The orbital septum is identified and incised in a complete horizontal ‘open sky’ fashion to expose the preaponeurotic fat pads.

●After supplemental injection of fat pads with anesthetic agent if necessary, the medial and central fat pads are excised as needed to obtain the desired result. Clamping of the fat pads can be used; meticulous hemostasis is mandatory.

●Care is taken to avoid resection of the lacrimal gland or overzealous resection of fat, especially in the medial central pad, to avoid an A-frame deformity or hollowed appearance after surgery.

●The skin edges are closed with a running nylon suture (6-0 or 7-0); the septum is not sutured. Crease formation stitches through the levator aponeurosis are used as desired.

Lower eyelid: transcutaneous

●The transcutaneous approach can be used where excessive skin (dermatochalasis) accompanies orbital fat herniation. Anesthesia is performed as described for the upper eyelid.

251 CHAPTER Ptosis •

Eyelids • 21 SECTION