Ординатура / Офтальмология / Английские материалы / Pediatric Ophthalmology Current Thought and A Practical Guide_Wilson, Saunders, Trivedi_2008

flammatory agents, the preferred treatment is surgical. This almost invariably involves lengthening of the superior oblique tendon between the trochlea and the scleral insertion. The most useful surgical procedures are tenotomy, spacer (silicone band or suture), and for some borderline cases, posterior tenectomy

[71]. Alternative procedures, designed to shorten the path of the superior oblique muscle and tendon by avulsing the trochlea from the orbital wall, have also been described; however, these involve anterior orbitotomy and are not commonly performed [45].

Prior to surgery, forced ductions need to be performed carefully to confirm the clinical diagnosis.

The restriction to elevation is not subtle. The tight superior oblique tendon is easily appreciated like a “knife edge” as the globe is passively moved from adduction into elevation, and then abduction. Once the superior oblique tendon is transected, forced ductions are immediately improved or normalized, which is a useful method of confirming successful tenotomy; however, repeated manipulation of the globe, forcefully moving the tendon through the trochlea, may temporarily improve ductions until after the patient is awakened from anesthesia and the Brown syndrome reappears, largely unchanged.

Most long-term successes occur in patients with some residual duction deficit post-operatively. This tends to improve with time, as does the residual “underaction” of the inferior oblique muscle. The presence of any post-operative hypertropia in the operated eye is a bad omen. It usually predicts the development of symptomatic superior oblique muscle palsy with inferior oblique muscle over action, positive Bielschowsky head tilt test, and compensatory torticollis. In most cases, ipsilateral inferior oblique muscle recession will resolve the problem but obviously requires a second surgery.

15.2.2 Monocular Elevation Deficiency

Monocular elevation deficiency is an uncommon strabismus disorder, which is almost always congenital, but may have a number of pathophysiologically distinct etiologies [39]. It has been described in the past as “double elevator palsy,” implying that the severe elevation deficiency was necessarily caused by weakness in both the superior rectus and inferior

oblique muscles. This name is actually a misnomer, since the inferior oblique muscle accounts for little of the elevation observed in normal patients, even in adduction [51]. Furthermore, inferior rectus muscle restriction, either primary or secondary, can appear clinically similar to superior rectus muscle weakness, making a more nosologically neutral term preferable.

Monocular elevation deficiency should therefore be considered a clinical presentation, rather than a unique diagnosis.

15.2.2.1Clinical Findings

Monocular elevation deficiency is typically noted during infancy, when the child is noted to adopt a chin-up head posture. In forced primary gaze, a hypotropia is present, with variable degrees of elevation deficit in attempted upgaze (Fig. 15.7). Unlike Brown syndrome, the elevation deficit is usually worse in abduction. Nonetheless, the distinction can occasionally be difficult in young children (Table 15.7).

Most, but not all, cases of monocular elevation deficiency can be explained by unilateral superior rectus muscle weakness, which can be an isolated finding or combined with ipsilateral ptosis (50%). Such cases may result from congenital abnormalities in the third cranial nerve nucleus [55]. Inferior rectus muscle restriction is usually present (70%) [61] and may suggest the alternate diagnosis of primary congenital fibrosis (see Sect. 15.2.3). While ptosis is common in both, there is not usually diagnostic confusion, since the latter diagnosis is typically bilateral, familial (autosomaldominant),andmayinvolvemultipleextraocular muscles. In addition, forced ductions tend to be less restricted in monocular elevation deficiency, and a modest elevation limitation is the rule in secondary inferior rectus muscle contracture. Occasionally, monocular elevation deficiency will be supranuclear in etiology [28], in which case a Bell’s reflex may be present and clearly establishes that diagnosis.

15.2.2.2Management of Monocular Elevation Deficiency

Treatment is often undertaken during early childhood to relieve a chin-up head posture. This almost always involves recession of the inferior rec-

tus muscle. Even in children with evidence of slow upgaze saccades, it is usually preferable to perform inferior rectus muscle recession (typically 5–7 mm) first and reevaluate primary-gaze alignment and head posture post-operatively. Larger recessions, or even free tenotomy, will be necessary in cases of primary congenital fibrosis but may cripple downgaze or pro-

duce severe lower eyelid retraction. Vertical transposition of the medial and lateral rectus muscles can be considered as secondary procedures, if required

(Fig. 15.8) [32]. When upgaze is only moderately limited, a large recession of the contralateral superior rectus muscle can cure a residual hypotropia and help symmetrize upgaze.

15.2.3 Congenital Fibrosis Syndrome

Considered to be a primary dysinnervation syndrome, congenital fibrosis of the extraocular muscles (CFEOM) is characterized by fibrous replacement of extraocular muscle and Tenon’s capsule resulting in moderate-to-severe limitation of ductions. There is almost always associated strabismus. The clinical presentation depends on the specific muscle involvement. The underlying disorder in CFEOM is believed to be primary dysinnervation of the oculomotor and/ or trochlear nerves [16]. The histopathological finding of fibrous replacement of the extraocular muscles is thought to be a secondary effect. It has been suggested that the different phenotypes are manifestations of different congenital anomalies of innervation.

15.2.3.1Clinical Findings

The syndrome has been previously described as the congenital presentation of limitation in elevation or depression, limitation in horizontal movement, eyes fixed in a depressed position, and associated ptosis and chin elevation (Fig. 15.9) [36]. The CFEOM may present sporadically, but three phenotypes have been described [15, 67]. The CFEOM 1 is the most common and presents with bilateral ptosis, eyes fixed in a depressed position, and variable horizontal duction deficits. This phenotype follows an autosomal-dom-

inant inheritance pattern. The CFEOM 2 is inherited in an autosomal-recessive pattern and presents with bilateral ptosis and a large-angle exotropia, with associated vertical duction deficits. The CFEOM 3 has a more variable presentation and can present with unilateral disease. It is inherited in an autosomaldominant pattern.

15.2.3.2Management

of Congenital Fibrosis

The primary goal in these patients is to correct chinup head posture, manage any associated amblyopia, and keep the visual axes clear. There is little hope of curing the strabismus. Extraocular muscle surgery is technically difficult and can challenge the abilities of even a seasoned strabismus surgeon. Very large recessions or even free tenotomy of the inferior rectus muscles can be required [78]; however, realigning the eyes behind ptotic eyelids may actually worsen the patient’s disability. Ptosis repair is usually required, but is potentially hazardous due to corneal exposure, since ocular motility is limited and there is no Bell’s reflex. For this reason, the upper eyelid should be elevated only to the upper pupillary border. Overall, these patients are quite difficult to manage, and careful discussion with the parents of the inherent limitations of surgical treatment is essential.

First described by Johnson [29], the diagnosis of ocular adherence syndrome encompasses a range of restrictive strabismus entities related to abnormal connections among and between the extraocular muscles and the surrounding orbital tissue.

The classic fat adherence syndrome, described by Parks as a complication of inferior oblique muscle myectomy, is an acquired restriction following violation of posterior Tenon’s capsule [47]. Prolapsed orbital fat induces inflammation and fibrosis, and causes adhesions to the sclera and extraocular muscles. This results in a restrictive hypotropia, typically worse in adduction, and positive forced duction testing (Fig. 15.10); however, manipulation of the

globe does not reveal the unyielding superior oblique tendon found in Brown syndrome, nor does one see proptosis in attempted elevation.

Adherence syndrome has been observed following strabismus surgery on other extraocular muscles, as well as following retinal surgery and periorbital trauma.Kushnerhasdescribedspecificcharacteristics of the inferior oblique muscle adherence syndrome, which typically presents with a restrictive hypotropia similar to fat adherence syndrome; however, the abnormalities of ocular motility can be traced to incarceration or scarring of the inferior oblique muscle to, or near, the inferior rectus muscle following inferior rectus surgery or scleral buckling [35].

15.2.4.2Management of Ocular Adherence Syndrome

The best management of adherence syndrome is prevention. Careful surgical technique to limit the posterior dissection of the muscle sleeve and avoid penetration into Tenon’s capsule will significantly reduce the incidence of adherence syndrome. When it does occur, surgical management primarily involves meticulous removal of the fat adherence and, if possible, closure and reinforcement of the defect in Tenon’s capsule. In addition to release of adherent tissues, successful treatment of the strabismus almost always requires surgery on additional extraocular muscles (Fig. 15.11).

Take Home Pearls

for Horizontal Strabismus Syndromes

•Congenital strabismus in which limitations of adduction or abduction are substantial, but the primary gaze deviation small, suggests the diagnosis of DRS.

•Globe retraction in adduction may or may not be observable in infancy.

•In type-I DRS abduction is usually better when the eye is elevated or depressed.

•Surgical treatment is most often indicated to correct primary-gaze strabismus, which causes the abnormal head posture in DRS.

•Most DRS patients can successfully be treated with recession of one or two horizontal rectus muscles.

•Transposition surgery can yield improved ductions and field of single binocular vision in DRS, but increases the complexity, morbidity, and surgical risk.

•Bilateral type-I DRS should be managed with reduced surgical dosage.

•Moebius syndrome is easily recognized by the associated facial palsy.

Take Home Pearls

for Vertical Strabismus Syndromes

•Brown syndrome is almost always caused by abnormal movement of the superior oblique tendon through the trochlea.

•Clinical examination for Brown syndrome is usually diagnostic, with an elevation deficiency that worsens from abduction to

adduction. Because the condition is restrictive, upgaze saccades will be normal when

tested where the globe can move freely.

•Positional globe proptosis is a common associated finding in Brown syndrome and does not occur in superior rectus muscle palsy or inferior rectus muscle restriction.

•Many patients with acquired Brown syndrome and a few with congenital Brown syndrome will spontaneously improve.

•Monocular elevation deficiency can often be managed initially with inferior rectus muscle recession.

•Congenital fibrosis syndrome is difficult to treat surgically. Because of limited globe motility, ptosis repair creates

a risk of exposure keratopathy.

•The surgical management of ocular adherence syndrome usually requires release of the adherence and intervention on non-involved muscles.

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