Ординатура / Офтальмология / Английские материалы / Pediatric Ophthalmology for Primary Care 3rd edition_Wright, Farzavandi_2008
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Figure 16 12.
Axial computed tomography scan in a patient with a right orbital cellulitis and ethmoid sinusitis. Note there is a subperiosteal abscess, thickening of the eyelids, and proptosis on the right side.
the paranasal sinuses and spreads into the orbit. The fungus causes vascu lar occlusion, leading to infarction and necrosis of the tissue. The disease is often fatal, and management includes local and systemic treatment with amphotericin B in addition to surgical excision of necrotic tissue. Hyper baric oxygen therapy may be helpful.
Orbital Pseudotumor (Idiopathic Orbital
Inflammatory Disease)
By definition, orbital pseudotumor is orbital inflammation having no known cause or underlying disease. Orbital inflammation can be associated with Wegener granulomatosis, polyarteritis nodosa, sarcoidosis, and systemic lupus erythematosus. These systemic diseases should be considered in those patients with noninfectious orbital inflammation. Orbital pseudotumor may affect any part of the orbit and in children, 45% may be bilateral. The pseudotumor may be anterior to the orbital septum and involve the eye lids or be posterior and cause proptosis. When anterior, the inflammation
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presents similar to a preseptal cellulitis with erythema and swelling of the involved eyelid (Figure 16 13). In addition to these most common signs, ocular pain and pain with eye movement appear more commonly and are more striking than in infectious cellulitis. Inflammation deep in the orbit will result in proptosis and in some cases, limited ocular motility.
Tolosa Hunt Syndrome
Tolosa Hunt syndrome is a variant of a pseudotumor that affects the supe rior orbital fissure and cavernous sinus. It is described as a painful, external ophthalmoplegia. Patients present with pain behind the eye and limited motility, secondary to impairment of the third, fourth, and sixth cranial nerves. Affected patients also may have hypoesthesia of skin around the eye and decreased vision if the optic nerve is involved. In addition to the eye involvement, children may present with systemic symptoms such as head ache, fever, vomiting, pharyngitis, anorexia, abdominal pain, and lethargy. Pseudotumor tends to be recurrent and may alternate from one eye to the other. A biopsy may be necessary to establish a diagnosis and rule out a neo plasm. Histology shows nonspecific pleomorphic infiltrate of inflammatory cells including lymphocytes, plasma cells, macrophages, and eosinophils. The eosinophils are more commonly found in children. After the diagnosis is made and neoplasms have been ruled out, treatment is started with oral
Figure 16 13.
Two-year-old child with pseudotumor involving the left lower lid. Note the erythema and swelling of the lid.
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corticosteroids. Initial doses are 1 mg/kg per day of oral prednisone. After 10 to 14 days, the dose can be tapered over a 3 week period. In difficult cases that do not respond to the corticosteroids, immunosuppressive agents may be used.
Viral Papilloma
Viral papilloma are caused by human papillomavirus and may appear any where on the conjunctival surface. Papilloma may be difficult to see unless magnification is used for the examination. Papilloma may protrude from the lid margins (Figure 16 14 A and B). “Kissing” lesions may appear where the lids are in apposition. A vascular core serves to differentiate these lesions from nevi with which they may be confused clinically.
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B
Figure 16 14.
Papilloma of the lower eyelid and medial canthal area. A, Photo shows lesion in the medial canthus. B, Photo shows eversion of the eyelid and reveals that the lower eyelid conjunctiva is involved.
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Treatment for papilloma is observation and cryotherapy. Surgical exci sion is likely to be followed by recurrence of the nevi. Cryotherapy should be done with care to avoid freezing the globe, skin, and tarsal plate.
Chapter 17
Eyelid Disorders
The most common pediatric eyelid malformations are ptosis and epiblepha ron (Table 17 1), with less common malformations including entropion, ectropion, colobomas, blepharophimosis syndrome, euryblepharon, and cryptophthalmos (Table 17 2).
Congenital Ptosis
Congenital ptosis is a droopy eyelid at birth (Figure 17 1). This can be uni lateral or bilateral. Congenital ptosis is associated with an abnormal levator muscle, the muscle responsible for lifting the eyelid. The levator muscle is the main elevator of the eyelid, with the Müller muscle contributing less significantly (Figure 17 2). In congenital ptosis the normal levator muscle is replaced by fibrosis resulting in an inelastic, weak muscle. It is thought to be a localized developmental muscle dysgenesis of unknown cause. Some patients may show improvement during the first year or two of life
Table 17-1. Common Eyelid Disorders
• Ptosis—eyelid drooping
• Epiblepharon —eyelashes scratching the eye secondary to a redundant skin fold inducing a vertical orientation of the eyelashes
Table 17-2. Less Common Eyelid Disorders
• Blepharophimosis Syndrome—Autosomal dominant. Most common findings are ptosis, blepharophimosis, epicanthus inversus, and telecanthus.
• Coloboma —An embryologic cleft found in the upper or lower eyelid. They can be unilateral or bilateral.
• Cryptophthalmos —Failure of eyelid formation. This can involve all of the eyelid or just portions.
• Ectropion —Out-turning of the eyelid secondary to insufficient skin. • Entropion —In-turning of the eyelid. The eyelashes scratch the eye.
• Euryblepharon —A combination of horizontal eyelid laxity and vertically shortened skin, giving an appearance similar to ectropion.
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Figure 17 1.
Patient with bilateral upper eyelid ptosis, right greater than left. Note absent lid crease on the right and brow lift as patient attempts to open the eyes.
but generally, the disorder remains static throughout life if not repaired. Children with unilateral or bilateral ptosis will show a compensatory chin elevation to avoid the droopy lid. Most cases are sporadic; however, familial inheritance cases have been reported.
Congenital ptosis also can occur as part of blepharophimosis syndrome (discussed later in this chapter), Marcus Gunn jaw winking syndrome, congenital third nerve palsy (see Figure 4 13), or syndromes involving con genital fibrosis of the extraocular muscles, or secondary to trauma at birth.
Marcus Gunn jaw winking syndrome is a rare syndrome caused by aber rant innervation of the levator muscle of the eyelid from the third branch of
Figure 17 2.
Drawing of superior orbit. Levator muscle is shown in red.
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the trigeminal nerve. This results in ptosis and eyelid movement (winking) synchronized with mouth movement, crying, eating, and sucking.
Children with congenital ptosis should be referred for ophthalmologic evaluation, as even mild ptosis can interfere with visual development and cause amblyopia (poor vision). Ptosis causes amblyopia by physically block ing the visual axis or inducing astigmatism from pressure on the cornea from the droopy lid. If the ptosis is causing amblyopia, appropriate treat ment is most effective when started as early as possible. The first line of treatment is to prescribe glasses for the astigmatism (if present) and use part time patching of the good eye to improve the vision of the amblyopic eye. If the amblyopia is significant and not improved with these measures, early ptosis surgery is indicated, even in the first few months of life. If the vision is not affected, the child needs to be monitored closely until the ptosis is repaired, generally until the child is 3 to 5 years of age. Amblyopia may still develop as the patient grows while the ptosis is still present. Ptosis can also cause abnormal head positions (chin elevation) from the patient trying to see better, indicating that the ptosis is significant.
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B
Figure 17 3
A, Patient with congenital left upper eyelid ptosis. Note poor upper lid crease and brow lift as patient attempts to open the eyes. B, Postoperative photograph taken after levator tightening procedure and revision of lid crease (surgery by Wright).
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There are several different operations that can repair congenital ptosis. The most important factor in determining which operation is most appro priate is the status of the levator muscle function. In patients with good levator function and mild to moderate ptosis (3 to 4 mm), a tightening pro cedure of the levator muscle is indicated (Figure 17 3). If levator function is poor, a frontalis eyelid suspension surgery is indicated. This is an operation whereby autologous or synthetic materials such as fascia lata or silicone sus pend the eyelid to the frontalis muscle above the eyebrows (Figure 17 4). There are some surgeons who may still advocate levator surgery even in the face of poor levator function. Ptosis surgery results in elevation of the eyelid but uniformly limits eyelid closure and the eye often appears open when the child sleeps. Dry eye after ptosis surgery is unusual because the eyes roll up under the lid during sleep or attempted eye blinking. This rolling up of the eyes is called Bell phenomenon. Ptosis surgery is delicate and challenging but can result in significant improvement in lid position. Often, more than one surgery may be necessary, and residual ptosis is not uncommon.
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Figure 17 4
A, Sixteen-month-old with right congenital ptosis causing amblyopia. B, Early surgery was performed consisting of a silicone frontalis sling. Postoperative photograph shows right eyelid now elevated with the visual axis clear (surgery by Wright).
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Epiblepharon and Entropion
Epiblepharon is a condition seen in infants whereby a horizontal redundant fold of skin immediately behind the eyelash margin pushes the lashes in toward the cornea (Figure 17 5). It is most common in the Asian popula tion. If the eyelashes touch the cornea, this is termed trichiasis. Trichiasis causes trauma to the cornea and microabrasions of the corneal epithelium called punctate keratopathy. Punctate keratopathy is best seen by slitlamp examination using fluorescent stain. In most cases epiblepharon will sponta neously resolve by 1 to 2 years of age as the infant grows out of baby fat and the extra fold of skin shrinks. If there is minimal keratopathy and the symp toms are mild, treatment consists of observation and possible use of artificial tears and lubricant ointments. If, however, there is severe keratopathy caus ing tearing and conjunctival injection, surgical correction is probably neces sary. In most cases surgery is reserved for children older than 1 year with persistent epiblepharon and significant keratopathy. If surgery is indicated it consists of removing an ellipse of skin and orbicularis muscle in the area of the epiblepharon. Epiblepharon surgery in general is associated with excel lent results.
Entropion is a congenital true eyelid margin rotation, often causing tri chiasis (Figure 17 6). Congenital entropion can occur in conjunction with congenital epiblepharon. The treatment of congenital entropion is surgical. The entropion does not resolve as the patient gets older; therefore, surgical
Figure 17 5.
Epiblepharon characterized by redundant pretarsal skin pressing the lashes against the cornea. The tarsal plate is in an upright position.