Novi način liječenja bilateralnog varus deformiteta kuka korištenjem „pločice osmice“ kod djece s kongenitalnom spondiloepifizealnom displazijom

Spondyloepiphysal dysplasia congenita (SEDc) is a rare autosomal dominant genetic disorder. Femoral head ossification delay and the proximal femur varus deformity i.e. coxa vara (CV) are the major features of SEDc. The accepted treatment is a valgus femoral osteotomy. The data on hip surgery in SEDc are scarce. In our database from 2006 to 2020, there were 6 SEDc patients. Four patients had surgery on 8 hips. Surgical treatment was indicated due to progressive CV deformity i.e. a decreasing neck-shaft angle (NSA), pain, limited hip abduction, and gait disturbances. In three patients, a novel surgical treatment was applied – a greater trochanter apo-physiodesis using “Eight-plate”. The patients were evaluated clinically and radiologically. The median age at first surgery was 6.3 years (range, 3.2 to 9.5 y) and the median follow-up period was 7 years (range, 5.6 to 14 y). The postoperative NSA was significantly improved with a mean increase of 13 degrees (P<0.001). Additional surgeries were needed in two patients. Overall, our results showed improved clinical and radiological parameters. The purpose of this study was to determine whether a method using an “Eight- plate” applied early to greater trochanter apophysis in SEDc patients with bilateral CV could reduce the need for more aggressive surgery in near future.Spondiloepifizna displazija kongenita (SEDc) je rijedak autosomno dominantan genetski poremećaj. Glavna značajka SEDc je odgoda okoštavanja glave bedrene kosti i varusna deformacija proksimalnog femura, tj. coxa vara (CV). Prihvaćeno liječenje je valgus osteotomija proksimalnog femura. Podaci o operaciji kuka u SEDc pacijenata su oskudni. U našoj bazi od 2006. do 2020. bilo je 6 SEDc pacijenata. Četiri pacijenta imala su 8 operacija kukova. Indikacija za kirurško liječenje bila je progresivna CV deformacija tj. smanjenje vratno-dijafizičnog kuta (NSA), bol, ograničena abdukcija kuka i poremećaji hoda. Kod tri pacijenta primijenjeno je novo kirurško liječenje - apofiziodeza velikog trohantera pomoću “pločice osmice”. Bolesnici su praćeni klinički i radiološki. Medijan dobi kod prve operacije bio je 6,3 godine (raspon od 3,2 do 9,5 godina), a medijan praćenja 7 godina (raspon od 5,6 do 14 godina). Postoperativni NSA je značajno poboljšan s prosječnim povećanjem od 13 stupnjeva (P<0,001). Dva pacijenta su trebala dodatne operacije. Sve u svemu, naši su rezultati pokazali poboljšane kliničke i radiološke parametre. Svrha ove studije bila je utvrditi može li metoda koja koristi “pločicu osmicu”, rano primijenjena na apofizu velikog trohantera kod SEDc pacijenata s obostranom CV-om, smanjiti potrebu za ekstenzivnijom operacijom u bliskoj budućnosti

Meningeomi orbite - kliničko zapažanje

The aim of the study was to assess the characteristics and clinical features of orbital meningiomas during a 5-year period (2001-2005). Due to their typical posterior location, slowly progressive and painless visual loss, orbital meningiomas are intriguing and challenging lesions to diagnose and manage. This prospective study identified five cases (four female, mean age 46.5±14.5 years and one male aged 68 years) of orbital meningiomas, treated at University Department of Ophthalmology, Split University Hospital in Split from January 2001 until December 2005. In each case, the diagnosis was confirmed by history review. In our study, the incidence of orbital meningiomas was 0.2 per 100,000 inhabitants, however, some cases may have been recognized and treated at other hospitals in Croatia. In this study, orbital meningiomas showed a female predominance (F/M, 4:1). The mean visual acuity was 0.7 (range 0.4-0.9). Discrete proptosis was found in two cases with a slightly blurred optic disc margin and discrete optic disc edema. Optic nerve discoloration was observed in three cases. In one case, meningioma showed intracranial extension. Mild optic disc discoloration and blurred optic disc margin with slow and painless visual loss, particularly in females, may be the first signs of orbital meningioma.Meningeomi orbite zbog svoje stražnje lokalizacije i polaganog bezbolnog gubitka vida često predstavljaju dijagnostički i terapijski izazov. Cilj rada bio je ispitati incidenciju meningeoma orbite tijekom 5 godina (siječanj 2001. - prosinac 2005.) na Klinici za očne bolesti Kliničke bolnice Split. Retrospektivnom analizom su ispitane povijesti bolesti petoro bolesnika s kliničkom dijagnozom meningeoma orbita, liječenih na našoj Klinici. Analiza je obuhvatila četiri žene srednje dobi 46,5±14,5 godina i jednog muškarca starog 68 godina. Incidencija meningeoma orbite u našem ispitivanju bila je 0,2 na 100.000 stanovnika, iako je moguće da su neki bolesnici dijagnosticirani i liječeni na drugim klinikama u Hrvatskoj. Meningeomi orbita su bili češći kod žena nego kod muškaraca (4:1). Srednja vidna oštrina je bila 0,7 (raspon 0,4-0,9). Diskretna proptoza s nejasnim granicama i blagim edemom glave vidnog živca nađena je u dva slučaja, a diskoloracija vidnog živca u druga tri slučaja. Kod jednog bolesnika meningeom orbite je očitovao intrakranijsku ekstenziju. Blagi i bezbolni pad oštrine vida jednoga oka, diskoloracija i nejasne granice vidnog živca na očnoj pozadini, naročito kod žena, mogu biti prvi znaci meningeoma orbite

Optički neuritis i multipla skleroza: naša iskustva

Idiopathic optic neuritis is idiopathic inflammation of the optic nerve. Multiple sclerosis is a chronic inflammatory demyelinating process of the central nervous system that affects mostly women aged 20-40. Modern diagnostic methods (MRI, VEP, and computerized perimetry) can confirm or exclude demyelinating etiology of the process. The study included 31 patients with optic neuritis hospitalized at University Department of Ophthalmology, Split University Hospital in Split, Croatia, between January 1, 2004 and December 31, 2005. The incidence of idiopathic optic neuropathy at Department was 3.2/ 100,000 in 2004 and 3.4/100,000 in 2005. The majority of patients were in the 20-40 age groups. In 22 (84.62%) patients, MRI showed brain demyelinating lesions. Most patients had prolonged VEP latencies. The incidence of idiopathic optic neuropathy has shown a significant increase in the last two years. Brain MRI was the key diagnostic method, along with significant symptoms and signs of idiopathic optic neuropathy. High dose corticosteroid pulse therapy, as described before, was demonstrated to have a beneficial effect on quick recovery of visual acuity and lengthening of relapse-free period.Idiopatski optički neuritis je upala vidnoga živca nepoznatog uzroka. Multipla skleroza je kronični upalni demijelinizirajući proces središnjega živčanog sustava, koji se najčešće javlja kod žena u dobi od 20 do 40 godina. Moderne dijagnostičke metode (magnetska rezonanca, vidni evocirani potencijali i kompjutorizirana perimetrija) omogućavaju potvrđivanje ili isključivanje demijelinizacijske etiologije procesa. U ovu studiju bio je uključen 31 bolesnik hospitaliziran između 1. siječnja 2004. i 31. prosinca 2005. godine u Klinici za očne bolesti Kliničke bolnice Split u Splitu, Hrvatska. Incidencija optičkog neuritisa u Klinici bila je 3,2/100.000 stanovnika u 2004. te 3,4/100.000 stanovnika u 2005. godini. Većina bolesnika je bila u dobi od 20 do 40 godina. Magnetska rezonanca je pokazala demijelinizacijske promjene u 22 (84,62%) bolesnika. Većina bolesnika je imala produžene VEP latencije. Incidencija idiopatske optičke neuropatije je pokazala značajan porast u posljednje dvije godine. Magnetska rezonanca mozga je bila ključna dijagnostička metoda uza značajne simptome i znakove idiopatske optičke neuropatije. Pulsna terapija kortikosteroidima u visokim dozama, kako je to prethodno objašnjeno, pokazala se je korisnom za brz oporavak vidne oštrine te za produljenje vremena do recidiva

Sljepoća i poremećaji vida kod dijabetičara u Hrvatskoj

New estimates show reduction in the number of people who are blind or suffer from vision impairment as a sequel of infectious diseases, while there is an increase in the number of people who are blind due to conditions related to longer life span and diabetes. Diabetic retinopathy is the leading cause of blindness in active population in developed countries. Epidemiological studies evaluated the incidence and describe many facets of this disease the incidence of which is being followed in most countries. The aim of this paper is to present some epidemiological data on diabetic retinopathy caused by type 1 diabetes in Croatia, to compare them with other countries, and to evaluate its importance in causing severe visual impairments and blindness. These data are considered highly relevant because they are changing constantly and require continuous monitoring in spite of all advances in understanding the distribution, causes and severity of diabetic retinopathy. It is of great importance because the increasing burden of diabetes will pose ever greater burden on the population at large and on healthcare systems providing care for these patents.Najnovije procjene ukazuju na smanjenje broja slijepih osoba i osoba sa slabijim vidom uzrokovanih zaraznim bolestima, no sve je veći broj ljudi slijepih zbog bolesti vezanih za produženje životnog vijeka ljudi i dijabetes. Dijabetična retinopatija je vodeći uzrok sljepoće u radnoj populaciji u razvijenim zemljama. Mnoge epidemiološke studije su procijenile incidenciju i sagledale ovu bolest iz svih njezinih aspekata. Cilj ovoga rada je prikazati epidemiološke podatke o dijabetičnoj retinopatiji uzrokovanoj dijabetesom tip 1 u Hrvatskoj, te ih usporediti s podatcima iz drugih zemalja i ocijeniti koliko ona utječe na broj slijepih osoba i osoba sa slabijim vidom. Smatramo ove podatke bitnima unatoč napretku u saznanjima o mehanizmima, raspodjeli i uzrocima bolesti. Treba također naglasiti da se ovi podatci neprestance mijenjaju i zbog toga ih valja stalno pratiti. To je osobito važno zbog toga što dijabetes uzrokuje veliko opterećenje za zajednicu i zdravstveni sustav koji se skrbi o ovim bolesnicima

Effect of Botulinum Toxin-A Injection on Intraocular Pressure and Proptosis in Thyroid Associated Orbitopathy

The aim of this study was to evaluate the effects of extraocular muscle injection of botulinum toxin-A (BTX-A) on intraocular pressure (IOP) and proptosis in patients with ocular motility disturbances in thyroid associated orbitopathy (TAO). In 17 patients (20 eyes) with restrictive strabismus and diplopia, BTX -A injections were applied. Intraocular pressure was measured with a Goldmann applanation tonometer in primary gaze position before and 2–4 weeks after BTX-A injection. Extraorbital prominention of the eyeball was measured before and after BTX -A injection using a Hertel instrument. Before the injection, the mean IOP in primary position of gaze was 18.6± 2.8 mmHg and 2–4 weeks after BTX-A injection was 16.9±3.3 mmH; (p=0.001). There was no statistically significant difference in Hertel egzophthalmometer readings before and after BTX-A injection, 21.5±2.7 mm vs 22.0± 2.6 mm; (p=0.678). In conclusion, BTX-A injection has a secondary lowering effect on IOP in TAO due to relaxation of extraocular muscles, but with no influence on proptosis

Retinal Branch Vein Occlusion by Arterial Wall Plaque in Hereditary Amyloidosis

Opisuje se očni nalaz kao prvi znak nasljedne amiloidoze. Opisuje se slučaj 34-godišnjeg muškarca s iznenadnim gubitkom vidne oštrine, pozitivnom obiteljskom anamnezom i sistemskim manifestacijama amiloidoze uključujući perifenu neuropatiju, restriktivnu kardiomiopatiju i nakupine amiloida u uzorku dobivenom biopsijom kolona. Glavne mjere ishoda bile su vidna oštrina, fotografija fundusa i fluoresceinska angiografija (FA). U ranom stadiju nasljedne amiloidoze fotografija fundusa desnoga oka pokazala je subretinalno makularno krvarenje, retinalna krvarenja i bojenje u stijenci donjeg temporalnog ogranka središnje retinalne arterije. FA je pokazala vaskularnu okluziju i žarišno bojenje arterije, a u kasnijem stadiju istjecanje fluoresceina u inframakularnom venskom ogranku. Mjesto "istjecanja" liječeno je laserskom fotokoagulacijom. Fotografija fundusa provedena nakon 3 tjedna pokazala je da su se subretinalna i retinalna krvarenja uglavnom iznova apsorbirala, ali je žarišno bojenje donje temporalne arterije jo. uvijek bilo prisutno, dok se na gornjoj temporalnoj arteriji pojavilo novo žarišno bojenje. FA je pokazala dvije vaskularne okluzije uz žarišno bojenje arterijskih stijenka, dok su u kasnijem stadiju mjesta hiperfluorescencije ukazala na daljnje napredovanje ovih vaskularnih promjena. Žarišne nakupine amiloida u retinalnoj arteriji mogle bi biti rijedak očni nalaz kod nasljedne amiloidoze, koji uzrokuje klinički nalaz okluzije venskih ogranaka.We report on ocular finding that was the first sign of hereditary amyloidosis. This case report describes a 34-year-old man with a sudden loss of visual acuity, positive family history, and systemic manifestations of amyloidosis that included peripheral neuropathy, restrictive cardiomyopathy and amyloid deposits detected in colon biopsy specimen. The main outcome measures were visual acuity, fundus photography and fluorescein angiography (FA). In the early stage of hereditary amyloidosis, fundus photography of the right eye demonstrated subretinal macular hemorrhage, retinal hemorrhages and staining in the lower temporal branch wall of the central retinal artery. FA showed vascular occlusion and focal staining of the artery, and in the late-stage fluorescein leakage of the inframacular branch vein. The site of "leakage" was treated with laser photocoagulation. After 3 weeks fundus photography showed that subretinal and retinal hemorrhages were mainly reabsorbed, but focal staining of the lower temporal artery wall was still present, and new focal staining of the upper temporal artery wall appeared. FA showed two vascular occlusions, with focal staining of arterial walls, and in late stage the sites of hyperfluorescence revealed that these vascular changes continued to progress. Focal retinal arterial amyloid deposits may be a rare ocular finding of hereditary amyloidosis, causing clinical finding of vein branch occlusion