Ptosis Ptosis is present when the upper eyelid is less than 2 mm from the center of the pupil. The causes of ptosis may be classified as mechanical, neurogenic, myogenic, and neuromuscular junctional (Kersten, 1995; Oosterhuis, 1996). What Distinguishes Ptosis from Pseudoptosis? A number of conditions may cause downward displacement of the eyelid without true ptosis (pseudoptosis). These conditions are listed in Table 18–1. What Is Blepharospasm or Hemifacial Spasm? Blepharospasm or hemifacial spasm may produce a pseudoptosis due to the intermittent (voluntary or involuntary) closure (spasm) of the upper and lower eyelid(s) resulting from contraction of the orbicularis oculi muscles. The lid position during the periods without contraction of the orbicularis muscles is normal. Voluntary lid closure may create pseudoptosis and occur as a conversion reaction. Aberrant regeneration of the facial nerve Anophthalmic socket Apraxia of lid opening Downgaze paralysis and pseudoblepharoptosis Blepharospasm or hemifacial spasm Hypertropia or hyperglobus (Kratky, 1992; Lyon, 1993; Meyer, 1991) Lid retraction in the contralateral eye Microphthalmia or phthisis bulbi Psychogenic pseudoptosis (all show depression of eyebrow on affected side) (Hop, 1997) What Is Apraxia of Eyelid Opening? Apraxia of eyelid opening is a supranuclear inability to open the eyelids voluntarily. Spontaneous and reflex eyelid opening are normal, and patients may manually open the lids or employ a head thrust as a compensatory movement. Aramideh et al correlated the clinical findings of apraxia of eye opening with synchronous levator palpebrae (LP) and orbicularis oculi (Ooc) electromyographic (EMG) recordings (Aramideh, 1995). EMG was characterized by either intermittent LP inhibition (ILPI) or a continuation of Ooc activity (Tozlovanu, 2001) following voluntary closure of the eyes (pretarsal motor persistence [PMP]). This study demonstrated the following: 1. In some patients there may be intermittent involuntary eye closure as a result of ILPI. Persistence of ILPI following eye closure would interfere with eye opening. When there is no ILPI, these patients have no difficulty opening their eyes at will following voluntary closure. 2. In other patients, closure of the eyes due to ILPI may activate Ooc. These patients have PMP in addition to ILPI and are unable to open their eyes at will following voluntary closure. 3. Patients who have PMP alone may be unable to open their eyes at will following voluntary closure. Once open, the eyes do not have the tendency to close by themselves. Unilateral or bilateral hemispheric disease and extrapyramidal disease may produce apraxia of lid opening. The etiologies of apraxia of eye opening are listed in Table 18–2. What Is Hypertropia or Hyperglobus? Hypertropia or hyperglobus may result in an abnormal position of the eye under a normal eyelid. Fixation with the hypertropic eye may eliminate the pseudoptosis. Likewise, contralateral eyelid retraction (e.g., thyroid eye disease) may produce an apparent ptosis in the fellow (normal) eye (Kratky, 1992; Lyon, 1993; Meyer, 1991). Is the Ptosis Acquired or Congenital? Ptosis may occur on a congenital or acquired basis. Long-standing isolated and static congenital ptosis with no other signs of systemic neurogenic (e.g., third nerve palsy), myogenic (e.g., chronic progressive external ophthalmoplegia), or neuromuscular disease (e.g., myasthenia gravis) does not require additional evaluation. Other forms of congenital nonisolated ptosis necessitate the same evaluation as the patients with acquired nonisolated ptosis. Table 18–3 lists the various forms of congenital ptosis. Congenital ptosis may be associated with other ocular and nonocular defects. Although children with congenital ptosis in isolation do not usually require any further evaluation, they may need amblyopia treatment or surgical therapy to avoid amblyopia (Cibis, 1995; Gusek-Schneider, 2000; Hornblass, 1995; McCulloch, 1993; Steel, 1996). Intermittent lid retraction of a ptotic eyelid may occur during chewing, jaw movement, or sucking in a child due to aberrant innervation of the oculomotor (levator) and trigeminal nerves (the Marcus-Gunn jaw winking phenomenon). No evaluation is required in children with the jaw-winking phenomenon (class IV, level C). Patients with acquired forms of ptosis should undergo further diagnostic evaluation (class III–IV, level B). Extrapyramidal disease Parkinson’s disease MPTP-induced parkinsonism Progressive autosomal-dominant parkinsonism and dementia with pallido-ponto-nigral degeneration Huntington’s disease Multiple systems atrophy (e.g., Shy-Drager syndrome) Progressive supranuclear palsy Wilson’s disease Neuroacanthocytosis Cortical-basal ganglionic degeneration Adult-onset Hallervordan-Spatz syndrome Unilateral (especially nondominant hemisphere) or bilateral hemispheric lesions Focal inferior and lateral frontal lobe cortical degeneration Motor neuron disease Post-bilateral stereotactic subthalamotomy Post-implantation of bilateral subthalamic nucleus electrical stimulators for Parkinson’s disease Unilateral putaminal hemorrhage Isolated finding (may be levodopa responsive) Source: Abe, 1995; Adair, 1995; Aramideh, 1995; Averbuch-Heller, 1998; Boghen, 1997; Dewey, 1994; Jancovic, 1995; Klostermann, 1997; Krack, 1994; Limousin, 1999; Nazarian, 1998; Riley, 1990; Verghese, 1999; Wszolek, 1992. MPTP, l-methyl-4-pheny 1-1,2,3,6 tetrahydropyridine. Isolated Nonisolated Associated other ocular defects Congenital cataracts Epicanthus Punctal abnormalities Refractive error/anisometropia Strabismus and amblyopia Associated nonocular defects (e.g., skeletal, central nervous system [CNS]) Blepharophimosis syndrome Synkinetic ptosis (e.g., Marcus-Gunn jaw-winking ptosis) Myogenic (e.g., congenital fibrosis) Neurogenic (e.g., third nerve palsy) Neuromuscular junction (e.g., myasthenia gravis) Combined valproate and hydantoin embryopathy with anomalous septum pellucidum (Gigantelli, 2000) X-linked congenital isolated ptosis (McMullan, 2000, 2001) Is the Ptosis Isolated or Nonisolated? A nonisolated acquired ptosis is defined as ptosis associated with other findings that may suggest a specific etiology as listed in Table 18–4. Is the Ptosis Due to Mechanical Causes? Any mechanical disturbance of the upper eyelid may result in ptosis. Mechanical causes of ptosis are listed in Table 18–5. Patients with an underlying mechanical ptosis that might be due to an underlying mass or infiltrative lesion should undergo imaging of the orbit (e.g., magnetic resonance imaging of the head and orbit with fat suppression and gadolinium contrast) (class IV, level C). Is the Ptosis Due to Myogenic Causes? Chronic progressive external ophthalmoplegia (CPEO) includes a spectrum of disorders that may result in a syndrome of painless, pupil-sparing, slowly progressive, and generally symmetric ophthalmoplegia. One subset of CPEO, the Kearns-Sayre syndrome, is characterized by the clinical triad of early-onset (usually before the age of 20 years) CPEO, pigmentary degeneration of the retina, and cardiac abnormalities (e.g., intraventricular conduction defects, bundle branch block, and complete heart block). Other features of Kearns-Sayre syndrome are elevation of cerebrospinal fluid protein, other neurologic dysfunction (e.g., cerebellar, auditory, and vestibular dysfunction), cognitive dysfunction, short stature, and developmental delay. Muscle biopsy may show “ragged red fibers” or other changes in mitochondria (Gross-Jendroska, 1992; Simonsz, 1992). Pathologic examination of the brain may demonstrate spongiform degeneration. Mechanical (e.g., lid mass, infection, or inflammation) Myogenic (e.g., external ophthalmoplegia) Neurogenic (e.g., ocular motor deficit and/or diplopia, other neurologic findings, Horner’s syndrome) Neuromuscular junction disease (e.g., signs of myasthenia gravis such as fatigue, enhancement, variability, Cogan’s lid twitch sign) Redundant skin or fat on the upper eyelid (e.g., dermatochalasis) Tumors or cysts of the conjunctiva or eyelid (Avisar, 1991) Infection (e.g., preseptal or orbital cellulitis) Cicatricial scarring (e.g., posttraumatic, postsurgical, or postinflammatory) (Singh, 1997) Inflammation and edema Graves’ disease Blepharochalasis Dermatomyositis Infiltration Amyloid Sarcoid Neoplastic (e.g., breast cancer) (Po, 1996) Waldenström’s macroglobulinemia (Klapper, 1998) Primary or metastatic tumors or orbital pseudotumor (Kersten, 1995; Po, 1996) Neurofibroma Hemangioma Dermoid Lymphoma Contact lenses related (Kersten, 1995; Levy, 1992; van der Bosch, 1992) Foreign body reaction
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