Ординатура / Офтальмология / Учебные материалы / Color Atlas of Ophthalmology The Quick-Reference Manual for Diagnosis and Treatment
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12 Strabismus 351
Fig . 12.11 Sixth-nerve palsy.
Differential Diagnosis
Duan e syn drom e, fibrosis of th e ext raocular m u scles, Möbiu s syn drom e, in flam - m ator y disorders, m yasth en ia gravis, in fan t ile esot ropia, h orizon tal gaze palsy, divergen ce palsy, an d previou s ext raocu lar m uscle surger y
Management
Obser ve for th e first 6 m on th s. Com plete resolu t ion is seen in up to 90% of cases w ith in th e first 6 m on th s after on set . Cases secon dar y to m igrain e an d vascular lesion h ave th e ten den cy to recu r. Pediat ric pat ien t s w ith a persisten t p alsy beyon d 3 m on th s an d adult cases in w h om th e angle of deviat ion in creases an d th ose w h o develop n eu rological deficits requ ire reevalu at ion . Ru le ou t gian t cell ar th rit is in pat ien t s older th an 50 years.
Non su rgical t reat m en t in clu des occlusion , t reat m en t of am blyopia, prism , an d bot ulin um toxin
Surgical approach depen ds on th e follow ing variables: residual fun ct ion of th e lateral rect u s m uscle, ocu lar rot at ion s, saccades, forced duct ion an d forced gen era- t ion test , deviat ion in p rim ar y posit ion , an om alous h ead post ure. Lateral rect us m u scle resect ion in p at ien ts w ith residual lateral rect u s m u scle fu n ct ion an d ver- t ical rect u s m uscle t ran sposit ion in pat ien ts w ith com plete lateral rect us m u scle palsy are th e procedu res of ch oice in th ese pat ien ts. Many cases require ipsilateral m edial rect us m u scle recession .
Duane Syndrome
Duan e syn drom e involves m isin n er vat ion of th e lateral rect us m u scle associated w ith absen ce or hypoplasia of th e sixth cran ial n er ve. Most frequen tly fibers of th e th ird cran ial n er ve going to th e m edial rect us m u scle are redirected to th e lateral rect us m u scle. Th e lack of n orm al in n er vat ion from th e sixth n er ve to th e lateral
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rect us m uscle resu lts in lim itat ion of abduct ion . Th e m isin n er vat ion of th e lateral rect us m u scle w ith fibers from th e th ird cran ial n er ve result s in abn orm al con t rac- t ion of th e lateral rect u s m uscle in at tem pted adduct ion (Fig. 12.12).
Presentation
Duan e syn drom e occu rs m ore frequen tly in fem ales th an in m ales, an d it m ost com m on ly involves th e left eye. Th e m ajorit y of pat ien t s w ith Duan e syn drom e h ave st raigh t eyes. Bilateral involvem en t is seen in 20%of th e cases. Th e m ost ch aracterist ic clin ical presen tat ion s are u n ilateral lim itat ion of abdu ct ion associated w ith globe ret ract ion an d n arrow ing of th e palpebral fissure in at tem pted adduc-
A
B
C
D
Fig . 12.12 (A) Duane t ype 1. (B) Duane t ype 2. (C) Duane t ype 3. (D) Bilateral Duane.
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t ion . Oth er m an ifest at ion s in clude an om alou s h ead post ure, lim it at ion to addu c- t ion , st rabism u s in th e prim ar y posit ion , an d an om alous vert ical m ovem en t s.
Classically th ree t ypes of Duan e syn drom e h ave been described . Type 1 is ch aracterized by esot ropia in th e prim ar y posit ion w ith lim itat ion to abdu ct ion , globe ret ract ion , an d palpebral fissu re n arrow ing in addu ct ion . Type II is ch aracterized by exot ropia in th e prim ar y posit ion an d lim it at ion to adduct ion . Type III is ch aracterized by exot ropia or esot ropia w ith lim it at ion to abdu ct ion an d addu ct ion .
Duan e syn drom e m ay be associated w ith system ic m an ifest at ion s, in clu ding h earing loss, Klippel-Feil syn drom e, Golden h ar syn drom e, HoltOram syn drom e, at rial sept al defect , h an d an om alies, crocodile tears, an d cleft palate.
Differential Diagnosis
Sixth -n er ve palsy, in fan t ile esot ropia, fibrosis of th e ext raocu lar m u scles, congen i- tal m isin n er vat ion syn drom es
Management
In dicat ion s for su rger y in clude st rabism u s in th e prim ar y posit ion , diplopia, an d an om alous h ead post ure. Th e su rgical approach is based on th e align m en t in th e prim ar y posit ion , lim itat ion to ocular rotat ion s, severit y of ret ract ion , an d th e presen ce of an om alou s vert ical m ovem en ts. In gen eral, m edial rect us recession an d ver t ical rect us m u scle t ran sposit ion s are th e procedu res of ch oice in pat ien ts w ith esot rop ia in th e prim ar y p osit ion . Lateral rect u s m uscle recession is th e procedu re of ch oice in pat ien t s w ith exot ropia in th e prim ar y p osit ion . Y split t ing of th e lateral rect u s m u scle m ay be added in pat ien ts w ith u p/dow n sh oot .
Brow n Syndrome
Brow n syn drom e is a congen ital or acquired lim itat ion to elevat ion in adduct ion .
Presentation
Th e p at ien t’s in abilit y to elevate th e eye in addu ct ion is w orse th an in th e m idlin e or abdu ct ion . Th ere is a V pat tern , m in im al superior oblique overact ion , w iden ing of th e palpebral fissure in addu ct ion , overdepression in addu ct ion (“dow n sh oot” of th e affected eye in adduct ion ), an om alous h ead post u re, an d hypot ropia of th e affected eye. Th e in abilit y to elevate th e eye in addu ct ion on forced du ct ion test ing is con firm ator y of Brow n syn drom e. Pat ien ts w ith acquired Brow n syn drom e m ay presen t w ith a t roch lear ten dern ess an d click (Fig. 12.13).
Fig . 12.13 Brown syndrome.
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Differential Diagnosis
Mon ocular elevat ion deficien cy, fibrosis of th e ext raocular m u scles, in ferior obliqu e m u scle palsy, orbital floor fract ure, thyroid op h th alm opathy, an d h eterotopic or dyn am ic displacem en t of th e rect u s m uscles
Management
Acquired Brow n syn drom e is frequen tly in flam m ator y. Spon tan eous resolut ion is com m on . Medical t reat m en t in clu des local an d system ic cor t icosteroids. In dica- t ion s for su rgical correct ion in clu de disrupt ion of th e bin ocu lar vision in a con - gen it al Brow n syn drom e, an om alou s h ead post ure, hypot ropia in prim ar y posi- t ion , diplopia, an d dow n sh oot in adduct ion . Ipsilateral superior obliqu e m u scle w eaken ing is th e procedure of ch oice in Brow n syn drom e.
Monocular Elevation Deficiency
Mon ocular elevat ion deficien cy is a su pran u clear or in fran u clear lim it at ion to elevat ion frequ en tly associated w ith ptosis or pseu doptosis (Fig. 12.14).
A
B
Fig. 12.14 (A) Monocular elevation deficiency. (B) Supranuclear palsy.
12 Strabismus 355
Presentation
Lim itat ion to elevat ion in th e prim ar y posit ion an d lateral gaze is greater in u p - gaze. Ptosis or pseudoptosis is p resen t in ~50%of th e cases. Pu pillar y abn orm ali- t ies are u su ally seen in acqu ired cases. An an om alou s ch in elevat ion h ead post u re is frequ en tly seen . Congen it al cau ses in clude supran uclear abn orm alit ies, su perior rect us m u scle w eakn ess, an d in ferior rect u s m uscle rest rict ion . Acquired m on ocu - lar elevat ion deficien cy m ay result from cen t ral n er vou s system t um or, in flam m a- t ion , in fect ion , or st roke.
Differential Diagnosis
Brow n |
syn drom e, Du an e syn drom e, fibrosis of th e ext raocular m uscles, th ird - |
n er ve |
palsy, Graves disease, m yasth en ia gravis, orbit al floor fract ure, m yosit is, |
m yotoxicit y, cerebellar t u m ors
Management
Man agem en t con sist s of t reat m en t of am blyopia. Th e goal of surger y is align m en t in th e prim ar y posit ion an d im provem en t of elevat ion . Th e surgical approach is based on th e presen ce of rest rict ion , w eakn ess, or a com bin at ion of both factors an d in clu des in ferior rect u s recession , superior rect u s m u scle resect ion , or h orizon tal rect us m u scles t ran sposit ion .
Graves Ophthalmopathy
Proptosis, eyelid retraction, and extraocular m uscle m otility disorders associated w ith hyperthyroidism or inflam m atory disease of the thyroid gland are characteristic of Graves ophthalm opathy. Many patients are euthyroid at presentation (Fig. 12.15).
Presentation
Pat ien ts p resen t w ith proptosis, conjun ct ival inject ion , ch em osis an d lid edem a, eyelid ret ract ion , an d lid lag on dow ngaze. Graves op h th alm opathy occurs m ore frequ en tly in w om en (3:1), w ith t w o peaks, on e at age 40 an d on e at age 60. Rest rict ion to ocu lar m ovem en ts m ore com m on ly affects th e in ferior rect us m u scle,
Fig . 12.15 Graves disease.
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follow ed by th e m edial rect us m u scle, superior rect u s m uscle, lateral rect u s m uscle, an d th e obliqu e m uscles. Orbit al im aging dem on st rates th e t ypical ext raocu lar m u scle en largem en t sparing th e ten don s at th e m u scle in ser t ion .
Differential Diagnosis
Orbit al in flam m ator y disease, m yosit is, lym p h om a, sarcoidosis, am yloidosis, orbital t u m ors, vascu lar lesion s, h igh m yopia
Management
Treat m en t of th e hyper thyroidism . System ic steroids, im m un osuppressan ts, an d radiat ion th erapy are u sed during th e acute ph ase of th e disease. St rabism us surger y sh ou ld be delayed u n t il th e in flam m ator y ph ase is resolved an d th e st rabis- m u s is stable. Th e prim ar y su rgical approach in clu des recession of th e ext raocular m u scle involved .
Myasthenia Gravis
Th is is an au toim m un e disorder, m ore com m on in w om en , ch aracterized by w eak- n ess an d fat igabilit y of st riated m uscles (Fig. 12.16).
A
B
Fig . 12.16 (A) Myasthenia gravis. (B) Myasthenia gravis post ice -pack test.
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Presentation
Myasth en ia gravis can be congen it al, juven ile, or adu lt on set . Ocu lar m yasth en ia is localized to th e ext raocular m uscles. System ic m yasth en ia involves oth er skelet al an d bulbar m uscu lat u re. Th e m ost com m on ocu lar m an ifest at ion is ptosis follow ed by u n deract ion of th e ext raocu lar m uscles. Ph arm acological test ing w ith an t ich olin esterases is th e stan dard diagn ost ic test for m yasth en ia.
Differential Diagnosis
Ch ron ic extern al oph th alm oplegia, m edicat ion s in terfering w ith n eu rom uscu lar t ran sm ission , cen t ral n er vous system t um ors
Management
Man agem en t in cludes thym ectom y, oral an t ich olin esterases, steroids, prism , botulin um toxin , an d st rabism us su rger y.
Congenital Fibrosis of the Extraocular Muscles
Ptosis an d rest ricted lim it at ion of ocu lar rot at ion s secon dar y to m yopath ic an d n eurogen ic abn orm alit ies lead to fibrosis of th e ext raocu lar m uscles. Th e con di- t ion m ay in clu de on e or m u lt iple ext raocular m u scles. Gen e m ut at ion s h ave been iden t ified for CFEOM t ype 1 (KIF21A gen e), CFEOM t ype 2 (PHOX2A), an d CFEOM t yp e 3 (FEOM3) (Fig. 12.17).
Presentation
Presen t at ion in clu des un ilateral or bilateral bleph aroptosis, fibrosis of th e ext raocu lar m u scles, ch in -u p post ure, lim it at ion to elevat ion an d h orizon tal ocular rotat ion s, an d absen ce of Bell ph en om en on . Th e con dit ion is som et im es associated w ith opt ic n er ve an d ret in al abn orm alit ies.
Fig . 12.17 Fibrosis syndrome.
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Differential Diagnosis
Mon ocular elevat ion deficien cy, th ird -n er ve p alsy, Brow n syn drom e, ch ron ic progressive extern al oph th alm oplegia, Möbiu s syn drom e, m yasth en ia gravis, orbital fract u re, at ypical Duan e syn drom e
Management
Man agem en t con sists of t reat m en t of am blyopia an d lu brican t s to avoid corn eal dr yn ess du e to poor Bell’s ph en om en on an d eyelid closu re. St rabism us surger y con sists of large recession of involved m u scle.
13 Neuro-Ophthalmology
Soosan Jacob and Am ar Agarw al
Cranial Nerve Palsies
Isolated Oculomotor (Third) Nerve Palsy
Th e com m on causes of oculom otor n er ve palsy in clude n eoplasm s, t raum a, an eu - r ysm s, isch em ic lesion s, an d oph th alm oplegic m igrain e. Ocu lom otor n er ve palsy cou ld be congen it al du e to a h eredit ar y cause. Th e m ode of t ran sm ission cou ld be eith er autosom al dom in an t or recessive.
Presentation
Total th ird -n er ve paresis m ay be cen t ral, sparing th e pupil, or periph eral w ith pu - pillar y involvem en t . If th e pup il is spared, th e m ost likely cause is a vascular lesion . If th e pu pil is involved, it is m ost likely du e to an an eu r ysm . Th e pat ien t h as a large exot ropia w ith hypot ropia. A fixed, dilated pu pil is seen . On at tem pted addu ct ion , th e eye in tor ts as th e su perior obliqu e w ou ld be n orm al. On e can also get p ar t ial paresis as th e th ird n er ve divides in to superior an d in ferior division . If th e su perior division of th e th ird n er ve is involved, gen erally oth er cran ial n er ves are also involved . On e can get an isolated involvem en t of th e in ferior division of th e th ird cran ial n er ve (Figs. 13.1A,B,C).
Nuclea r Third-Nerve Pa resis
Th is is ext rem ely rare. Follow ing are th e im por t an t poin ts about th is lesion :
Each superior rect u s is in n er vated by th e con t ralateral th ird -n er ve n u cleu s; if th ere w as n u clear th ird -n er ve palsy on on e side th en th ere w ould be a paresis of th e con t ralateral su perior rect u s.
Both levator palpebrae su perioris are in n er vated by on e subn uclear st ruct u re, th e cen t ral caudal n u cleu s. Th erefore, n u clear th ird -n er ve palsy leads to bilateral ptosis.
Third-Nerve Fascicle Syndromes
In th ese cases th e th ird n er ve h as already left th e n u cleu s, so th e lesion s affect on ly on e side. Th ere are various syn drom es th at can occur, depen ding on th e site of lesion . Th ey are due gen erally to an isch em ic, in filt rat ive (t u m or) or rarely to an in flam m ator y lesion .
Nothnagel Syndrome
In th is case th e lesion is in th e area of th e su perior cerebellar pedun cle. As th e lesion involves th e superior cerebellar pedu n cle th e pat ien t h as an ipsilateral th ird - n er ve paresis w ith cerebellar at axia.
Benedikt Syndrome
In Ben edikt syn drom e th e lesion is in th e area of th e red n ucleus. Th is leads to con t ralateral h em it rem or w ith ipsilateral th ird -n er ve paresis.
Cla ude Syndrome
Th is syn drom e h as feat u res of both Noth n agel an d Ben edikt syn drom es.
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A
B