Posteriorna kortikalna atrofija (PCA) je neurodegenerativno kliničko-radiološko stanje s atrofijom posteriornih dijelova moždanog korteksa uključujući vidni korteks i dijelove parijetalnog i ...temporalnog korteksa. Nespecifični vidni poremećaji često se pogrešno interpretiraju kao oftalmološki, što vodi prema zakašnjeloj pravoj dijagnozi. Razlikovanje PCA od drugih bolesti može biti zahtjevno i može oduzeti mnogo vremena. Prikazuje se bolesnica s PCA u dobi od 57 godina koja godinama pokazuje vidne perceptualne poremećaje koji se inicijalno dijagnosticiraju kao oftalmološke bolesti te potom kao neobjašnjeno oštećenje vida. Dvadeset pet godina ranije obostrano je učinjena radijarna keratotomija, a šest godina prije prijema u oba se oka implantira fakična intraokularna Verisyse leća. Unazad četiri godine liječi glaukomsku bolest. Tijekom neurooftalmološke opservacije nije potvrđena sumnja na optičku neuropatiju niti na makulopatiju. Pažljivom anamnezom saznaje se da obitelj već godinama ranije zapaža tegobe bolesnice kod prepoznavanja lica, pri čitanju i pisanju. Navode se tegobe procjene dubine i prostorne orijentacije, problemi sa stepenicama, osobito pri silasku. Nađena je velika diskrepancija između iskazanih funkcionalnih vizualnih oštećenja (vidna oštrina, vidno polje, Ishihara kolorni test) s jedne strane i s druge strane urednoga oftalmoskopskog nalaza na mrežnicama i urednog morfološkog nalaza OCT dijagnostike. Učinjena MR pretraga također otvara sumnju na kortikalnu problematiku te je bolesnica usmjerena na daljnju neurološku i neuro-radiološku opservaciju uz PET-FDG, što je dovelo do dijagnoze PCA. PCA je važan dijagnostički entitet jer se ti bolesnici obično najprije javljaju oftalmolozima i nerijetko se svrstavaju u skupinu onih s neobjašnjenim oštećenjem vida. Kod diskrepancije vizualnih oštećenja registriranih funkcionalnom dijagnostikom u odnosu na srazmjerno uredan oftalmoskopski nalaz i nalaz OCT dijagnostike, važno je ponovno pažljivo provjeriti anamnezu i heteroanamnezu i pomisliti na mogućnost kortikalnog uzroka iskazanih oštećenja s ciljem ranog otkrivanja uzroka neobjašnjenog oštećenja vida. Tu je važna dobra suradnja s kognitivnim neurolozima specijaliziranim za neurodegenerativne bolesti. Cilj je rada podignuti svijest liječnika o postojanju ovog entiteta kao mogućeg uzroka neobjašnjenog gubitka vida i olakšati rano prepoznavanje uz primjenu odgovarajućih dijagnostičkih metoda.
Objective: To present a patient with a sudden onset ocular tilt reaction (OTR) and review recent knowledge and evolving insights of the underlying pathophysiological mechanisms of skew deviation and ...OTR. Methods: A middle-aged hypertensive man who had previously suffered stroke with good recovery presented with sudden-onset double vision, slurred speech, ataxia, and a head tilt. Romberg test was positive. The patient denied having disturbances of visual acuity, eye pain, or recent trauma. The right eyeball was pushed upward. The patient complained of double vision in any gaze direction. Movements of the extraocular muscles (EOMs) in the horizontal plane were normal, whereas vertical version and convergence were not possible. We administered a Hess-Lancaster test, cover test, fundoscopic examination, Parks-Bielschowsky three-step test, upright-supine test, brain magnetic resonance imaging (MRI), transcranial doppler (TCD) ultrasonography, electrocardiogram (ECG), Holter monitor (24 h), and echocardiography. Results: The Hess-Lancaster test showed superior rectus muscle and inferior obliquus muscle palsy to the left and rectus inferior muscle and superior obliquus muscle palsy to the right. The right eyeball fell behind when looking downward and the left eyeball when looking upward. Cover alternating test was positive from vertical, R/L. Examination of the ocular fundus showed incyclotorsion of elevated right eye and excyclotorsion of depressed eye. The Parks-Bielschowsky three-step test was negative. A brain MRI with gadolinium revealed a small zone of diffusion restriction in the medial portion of the right cerebral peduncle and right thalamus. There was a gradual improvement in the patient's neurological status following treatment. Conclusion: Skew deviation, a not uncommon clinical condition, should be promptly recognized when binocular vertical diplopia cannot be interpreted by trochlearis and oculomotor nerve lesion, myasthenia gravis, or orbital pathology. Maddox rod, cover test, Parks-Bielschowsky three-step, and other tests should help to establish the diagnosis. The prognosis depends on etiology, but it is commonly favorable; the majority of patients recover spontaneously after less than a year. More invasive management options should be discussed thereafter.
Cilj: Cilj rada je prikazati klinički tijek, dijagnostiku i liječenje šestgodišnje djevojčice s giratnom atrofijom i obostranim cistoidnim edemom makule. Prikaz slučaja: U četverogodišnje djevojčice ...tijekom sistematskog pregleda nađeni su slabija vidna oštrina i konvergentni strabizam. Nakon dvogodišnjeg liječenja slabovidnosti primjećuje se obostrani makularni edem te se dijete upućuje na daljnju obradu na Kliniku za oftalmologiju. Djevojčica je tada imala vidnu oštrinu 0.15 na oba oka, obostrani edem makule i ovalne zone korioretinalne atrofije na periferiji mrežnice. Uvedena je antiedematozna terapija. Učinjena je cjelovita klinička i laboratorijska obrada i u plazmi nađena visoka koncentracija ornitina, te je postavljena dijagnoza giratne atrofije (GA) korioretine. U terapiju je uveden piridoksin (vitamin B6). Po terapiji je došlo do neznatnog smanjenja koncentracije ornitina u plazmi te je uvedena i prehrana s ograničenim unosom proteina kojom se također nije postigao željeni učinak. Naposljetku je uz navedeno započeto liječenje aminokiselinom L-lizinom. Kombinacija navedenih terapijskih mjera dovela je do značajnog smanjenja koncentracije ornitina u plazmi, koja je smanjena na oko trećinu u odnosu na koncentracije prije početka liječenja. Uz laboratorijske pokazatelje bolje kontrole bolesti zamijećeno je prolazno kliničko poboljšanje, smanjenje makularnog edema, ali se progresija perifernih atrofičnih lezija nije zaustavila. Zaključak: Cistoidni edem makule u pacijenata s giratnom atrofijom praćen je oslabljenom vidnom oštrinom. Unatoč intenzivnom liječenju edem makule perzistira te je vidljiva daljnja progresija atrofičnih žarišta na periferiji fundusa. Smanjenje koncentracija ornitina u plazmi bitan je preduvjet za usporenje progresije bolesti i odgađanje trajnog gubitka vidne funkcije.
Aim: The aim is to present the clinical course, diagnostics and treatment of six-year-old girl with gyrate atrophy and bilateral cystoid macular edema. Case report: Four-year old girl was found with low vision and convergent strabismus in a systematic medical examination. After two years of the amblyopia treatment bilateral macular edema was detected and the child was refered to The Department of Ophthalmology. She presented with the both eyes best corrected visual acuity 0.15, bilateral cystoid macular edema and peripheral, oval zones of chorioretinal atrophy. Antiedematous therapy was administered. Clinical work-up revealed extremely high plasma concentrations of ornithine and the diagnosis of gyrate atrophy (GA) of the chorioretina due to the lack of mitochondrial enzyme ornithine aminotransferase was established. The therapy with pyridoxine (vitamin B6) was started. This treatment led to slight reduction of serum ornithin concentrations; hence, low-protein diet was introduced, but decrease of ornithine concentrations was still insufficient. Therefore, the treatment with amino acid L-lysine was introduced in the therapy. Plasma ornithine concentrations successfully decreased to almost one third of the initial concentration. There was a transient decrease of cystoid macular edema, but atrophic lesions of the peripheral fundus progressed. Conclusion: Cystoid macular edema in patients with gyrate atrophy was followed by impaired visual acuity. Despite the intensive treatment macular edema persisted and further progression of atrophic lesion on the fundus periphery was detected. The maintenance of low plasma ornithine levels is an essential prerequisite for slowing the development of the disease and postponing the permanent loss of visual function.
JIA is the most common rheumatic disease of childhood and JIA-U is its most frequent and most devastating extraarticular manifestation. This form of uveitis is usually asymptomatic, chronic anterior ...uveitis, often accompained with complications. JIA-U is the main cause of vision loss and even blidness in childhood. Thus, screening for JIA-U in all JIA patients and early treatment is of prime importance. Over the last 15-20 years, ever since IMT has been used, studies generally show trends toward decrease of JIA-U onset, complications frequency, improvement of prognosis and remission achievement. Despite evident improvements, over 20% JIA-U patients still develop complications in long-term follow-up. Moreover, about 50% JIA-U patients continue to have active uveitis in adulthood. Therefore, JIA-U is still associated with high risk of late sequelae and visual acuity loss, functionally and structurally eye damage and quality of life impairment.
Changes in the eye axial diameter were studied to assess the eye globe impact of conventional operation for retinal detachment. The study included 69 eyes in 69 patients operated on for ...rhegmatogenous retinal detachment. There were 46.4% of men and 53.6% of women, mean age 52.7 (+/- 15.21) years. Results of preoperative and postoperative ultrasonographic measurement of axial diameter are presented. The mean preoperative and postoperative eye axial diameter was 23.69 (+/- 1.84) mm and 24.43 (+/- 1.91) mm, respectively. Postoperative results showed the axial eye length to increase by a mean of 0.74 (+/- 0.44) mm, yielding a statistically significant difference from the preoperative measurement (p < 0.001). The mean myopia induced by this eyeball elongation was 1.77 D. The encircling band with and without segmental buckling used in surgical repair of retinal detachment creates circular and segmental indentation of the eyeball, thus increasing its axial length. The myopia induced by elongation of the eyeball results in considerable myopia, which requires appropriate correction in the early postoperative period to achieve favorable vision rehabilitation.
Cilj: Cilj rada je prikazati klinički tijek, dijagnostiku i liječenje šestgodišnje djevojčice s giratnom atrofijom i obostranim cistoidnim edemom makule. Prikaz slučaja: U četverogodišnje djevojčice ...tijekom sistematskog pregleda nađeni su slabija vidna oštrina i konvergentni strabizam. Nakon dvogodišnjeg liječenja slabovidnosti primjećuje se obostrani makularni edem te se dijete upućuje na daljnju obradu na Kliniku za oftalmologiju. Djevojčica je tada imala vidnu oštrinu 0.15 na oba oka, obostrani edem makule i ovalne zone korioretinalne atrofije na periferiji mrežnice. Uvedena je antiedematozna terapija. Učinjena je cjelovita klinička i laboratorijska obrada i u plazmi nađena visoka koncentracija ornitina, te je postavljena dijagnoza giratne atrofije (GA) korioretine. U terapiju je uveden piridoksin (vitamin B6). Po terapiji je došlo do neznatnog smanjenja koncentracije ornitina u plazmi te je uvedena i prehrana s ograničenim unosom proteina kojom se također nije postigao željeni učinak. Naposljetku je uz navedeno započeto liječenje aminokiselinom L-lizinom. Kombinacija navedenih terapijskih mjera dovela je do značajnog smanjenja koncentracije ornitina u plazmi, koja je smanjena na oko trećinu u odnosu na koncentracije prije početka liječenja. Uz laboratorijske pokazatelje bolje kontrole bolesti zamijećeno je prolazno kliničko poboljšanje, smanjenje makularnog edema, ali se progresija perifernih atrofičnih lezija nije zaustavila. Zaključak: Cistoidni edem makule u pacijenata s giratnom atrofijom praćen je oslabljenom vidnom oštrinom. Unatoč intenzivnom liječenju edem makule perzistira te je vidljiva daljnja progresija atrofičnih žarišta na periferiji fundusa. Smanjenje koncentracija ornitina u plazmi bitan je preduvjet za usporenje progresije bolesti i odgađanje trajnog gubitka vidne funkcije.
Changes in the eye axial diameter were studied to assess the eye globe impact of conventional operation for retinal
detachment. The study included 69 eyes in 69 patients operated on for ...rhegmatogenous retinal detachment. There were
46.4% of men and 53.6% of women, mean age 52.7 (15.21) years. Results of preoperative and postoperative ultrasonographic
measurement of axial diameter are presented. The mean preoperative and postoperative eye axial diamter
was 23.69 (±1.84) mm and 24.43 (±1.91) mm, respectively. Postoperative results showed the axial eye length to increase
by a mean of 0.74 (0.44) mm, yielding a statistically significant difference from the preoperative measurement (p
0.001). The mean myopia induced by this eyeball elongation was 1.77 D. The encircling band with and without segmental
buckling used in surgical repair of retinal detachment creates circular and segmental indentation of the eyeball,
thus increasing its axial length. The myopia induced by elongation of the eyeball results in considerable myopia,
which requires appropriate correction in the early postoperative period to achieve favorable vision rehabilitation.
PDF
