[Orbital tumor--hemangiopericytoma--in a neonate]. / Guz oczodolu--haemangiopericytoma--u noworodka.

PURPOSE: To present a case of Haemangiopericytoma (HPC), a rare neopalsm which originates from the vascular pericytes. HPC occurs most commonly in adults. Only 5-10% of cases occur in children. Congenital orbital HPC is generally unknown in the field of ophthalmology. MATERIAL AND METHODS: A case of congenital, large exophthalmus is reported in a 1 day old male neonate. Imaging studies demonstrated a vascular, orbital mass involving skull base and cranial fossa. RESULTS: The diagnosis of HPC was established after histological exmination. Lesion did not qualify to surgical resection. The child was treated with chemotherapy for 10 months and a great regression of tumor was noted. There was no tumor recurrence during 4 years of a follow up. CONCLUSIONS: Chemotherapy may have a significant role in the treatment of infants with nonoperative malignant hemangiopericytoma.

Orbital tumor as an initial manifestation of Wegener's granulomatosis in children: a series of four cases.

BACKGROUND: Wegener's granulomatosis (WG) is a rare idiopathic disease in which small and medium-sized arteries are affected by necrotizing granulomatous inflammation. It is associated with a triad of pulmonary (cavitating granulomatous lesions with hemoptysis, cough, and dyspnea), renal (glomerulonephritis with hematuria, proteinuria), and head (otitis media, recurrent sinusitis, eye or orbital involvement) manifestations. CASE REPORT: Four children aged 7-11 years diagnosed with WG between 1995-2008 initially presented with unilateral proptosis and ptosis due to orbital tumor. CT or MRI, orbital lesion biopsy, and laboratory tests (ERS, CRP, ANCA) were part of the diagnostic workup. The diagnoses were based on correlation between clinical presentation and diagnostic findings. All four patients had orbital lesions on contrast-enhanced CT and MRI. Two had lesions of the temporal pyramid. Orbital tumor biopsies showed granulomatous lesions in two patients, necrotizing vasculitis with leukocytoclasia in three, and an orbital pseudotumor in one. ESR and CRP were positive in all. ANCA positivity was variable (c-ANCA did not allow WG diagnosis or there were atypical ANCAs). All had blood and protein in the urine, but only one had advanced renal involvement. All were treated with oral steroid and immunosuppression; remission was successful. CONCLUSIONS: WG is often more difficult to diagnose in children than in adults due to frequent absence of its signature features. The absence of the classic triad and atypical laboratory or biopsy findings do not exclude a diagnosis of WG. Orbital demonstration helps achieve early diagnosis and treatment of this potentially fatal rheumatologic disease.

[Intraocular lens implantation in developmental lens disorders in children]. / Implantacja sztucznej soczewki wewnatrzgalkowej w anomaliach rozwojowych soczewki u dzieci.

The pediatric cataract surgery in eyes with developmental disorders, stay with still considerable challenge. At children, the lasting vision development extorts necessity quick settlement of refraction defect formed after operation. The intraocular lens old boy with cataract in microspherophakia and 12 years old boy with cataract in lens with coloboma. One-piece flexible and rigid PMMA intraocular lens was placed with success at posterior chamber without scleral fixations and without using capsular tension ring in this cases. After 3 years of observation there were no decentration or dislocation of intraocular lens in both children. Authors concluded that in some cases posterior chamber intraocular lens implantation despite defective zonular or capsular support, can make up the effective method of surgical treatment without risk of early dislocation.

[Ocular changes in course of Mycohacterium tuberculosis infection--own observations]. / Zmiany w narzadzie wzroku w przebiegu zakazenia pratkiem gruzlicy--obserwacje wlasne.

Ocular tuberculosis occurs rarely and causes many diagnostic difficulties. The aim of this paper is to provide an analysis of diagnostic problems of ocular changes as a consequence of Mycobacterium tuberculosis infection in children. Between 2006 and 2007 there were 7 children at the age 8 to 16 years old, treated in Ophthalmic Department. The infection or contact with Mycobacterium tuberculosis was diagnosed through the range of examination. Diagnosis of tuberculosis included chest radiography, computed tomography of lungs, tuberculin skin test and bronchoscopy. Gastric and bronchial aspirates were used for bacterial and genetic investigations. In eyes with ocular tuberculosis there were various intraocular changes. The most common clinical presentation occurred by intermediate uveitis, posterior uveitis and optic neuritis. 5 children were treated with antituberculous drugs, 2 children were observed without treatment. Examination and treatment of ocular tuberculosis required cooperation with specialists of pulmonary diseases.

[Spontaneous bilateral posterior capsule rupture in child--case report]. / Samoistne obustronne uszkodzenie tylnej torebki soczewki u dziecka--opis przypadku.

Isolated posterior capsule rupture without penetrating injury has rarely been reported. Posterior capsule tears can be preexisting, spontaneous or intrasurgical. Authors report a case of progressing bilateral cataract in 3 months old boy, caused by posterior capsule rupture. Rapidly developing cataract with intumescent lens material in vitreous cavity required surgical procedure: pars plana lensectomy and vitrectomy in both eyes.

[Evaluation of the results of treatment orbital rhabdomyosarcoma in children]. / Ocena wyników leczenia miesaka miesni prazkowanych oczodolu u dzieci.

PURPOSE: To estimate the state of the vision organ in the children treated for orbital rhabdomyosarcoma. Rhabdomyosarcoma (RMS) is the most common primary malignant orbital tumor in children. RMS usually manifest clinically as rapidly progressive exophthalmus and displacement of the globe. The diagnosis is based on biopsy, CT and MR images. The treatment includes radiation, chemotherapy, and surgery. MATERIAL AND METHODS: The retrospective review of data of 14 children between 0 and 11 years old with rhabdomyosarcmoa of orbit. After a biopsy, with precedent CT or MRI, all patients were treated with chemotherapy including or not including radiotherapy. RESULTS: 3 children died, orbital exentaration was necessary because of tumor recurrence in 3 cases, 8 children remained healthy (without recurrent disease). CONCLUSIONS: Fast diagnosis using CT, MRI and the result of biopsy, have a positive influence on the effect of neoplastic treatment and prognosis.

[Lens subluxation as a sign of medulloepithelioma in 13 years boy--case report]. / Podwichniecie soczewki jako objaw nabloniaka rdzeniowego (medulloepithelioma) u 13-letniego chlopca--opis przypadku.

The paper presents the case of the 13 years old boy with the deterioration of vision acuity of the right eye, caused by lens subluxation. The condition was caused by tumour of ciliary body. Additional tests: USG, UBM, CT did not resolve diagnostic doubts. After the biopsy medulloephitelioma was diagnosed and the decision of the eye enucleation was made. Histopathological examination confirmed the diagnosis.

[Childhood glaucoma associated with Sturge-Weber syndrome --the efficacy of cyclofotocoagulation and other therapeutic methods]. / Jaskra w zespole Sturge-Webera u dzieci --skutecznosc cyklofotokoagulacji oraz tradycyjnych metod terapeutycznych.

PURPOSE: Sturge-Weber syndrome is a rare congenital neurooculocutaneous disorder. Ocular involvement can include glaucoma and vascular malformations of the conjunctiva, episclera, choroid and retina. MATERIAL AND METHODS: 16 children (16 eyes) with Sturge-Weber syndrome associated with glaucoma (mean age--34 month, mean follow up 8.87 years) treated in our institution, were reviewed. In retrospective analysis were assessed: IOP and postoperative complications after diode laser cyklophotocoagulation (16 eyes), after trabeculectomy (6 eyes) and trabeculectomy with MMC (3 eyes). RESULTS: In ten eyes (62.5%) good result (IOP 6-22 mmHg) post cyclophotokoagulation--(3x) was recorded, as well as in 3 eyes (50%) post trabeculectomy and in 3 eyes (100%) post trabeculectomy with MMC 0.2/4 min. No postoperative complications occured in cyclophotocoagulation group. Complications after trabeculectomy were the following: 3 cases of hypotony, 4 cases with shallow anterior chamber and 5 with choroidal effusions. CONCLUSIONS: Diode laser cyclophotocoagulation combined with topical medication is an effective and safe treatment of glaucoma in children with Sturge-Weber syndrome.

[Influence of intraoperative complication on intraocular rigid lens fixation in pediatric cataract surgery]. / Wplyw powiklan sródoperacyjnych na fiksacje sztucznej sztywnej soczewki wewnqtrzgalkowej w operacjach zacmy u dzieci.

PURPOSE: This paper presents the intraoperative complications in pediatric cataract surgery with IOL implantation and their influence on fixation place. MATERIAL AND METHODS: 384 eyes of 276 children undergone operative procedure for cataract. Anterior capsulorhexis, lens cortical aspiration, primary posterior capsulorhexis with anterior vitrectomy and IOL implantation were done in all eyes. The place of IOL implantation was capsular sac or ciliary sulcus. RESULTS: There were no serious intraoperative complications but in cases with large anterior (5.2%) and posterior (14.6%) radial capsule tears, vitreous loss (12.3%), and hemorrhage (5.5%) to anterior and posterior chamber the IOL was fixated at ciliary sulcus (in 37.5%). CONCLUSIONS: The surgical procedure is useful and safe in the management of pediatric cataract. Location of an IOL in the ciliary sulcus in a child, is acceptable. To avoid decentration in this cases, we recommend rigid PMMA IOLs.

[Bilateral optic nerve sheath meningioma (ONSM) --diagnostic and therapeutic problems]. / Obustronny oponiak oslonek nerwu wzrokowego (ONSM)--problemy diagnostyczne i lecznicze.

The authors describe the case of bilateral optic nerve sheath meningioma /ONSM/ in 14 years old girl. When being hospitalized at the Neurology Clinic with paresis n.VII, the patient was examined by ophthalmologist. The diagnosis was made on the basis of clinical and characteristic changes revealed by USG, CT and MRI. The very first ophthalmologic examination confirmed a visual acuity 5/5 OD and hand movements OS, associated with papilloedema OD and edematous optic atrophy OS. The use of USG, CT and MRI revealed bilateral tubular thickening of the optic nerves (8mm), with calcification. After the period of one year, clinical progression was observed. The intracranial tumor extended. It was shown in diagnostic imaging. The patient was operated on by neurosurgeons with histopathology verification. In the later stage she underwent radiotherapy. The case has been described because of its diagnostic and therapeutic difficulties.

[Evaluation of results of enucleations with orbital implant in children and adolescents]. / Ocena wyników operacji usuniecia galki ocznej z wszczepem oczodolowym u dzieci i mlodziezy.

PURPOSE: The aim of the research is the assessment of results of enucleation with orbital implant in children. MATERIAL AND METHODS: Examinations included the group of 99 children between 6 months and 19 years of age. The examined group consisted of 39 boys (39.4%) and 60 girls (60.6%). 96 eyes were removed because of non-regressive retinoblastoma (despite applied treatment), two eyes with buphtalmus (with no light perception), as a result of glaucoma, and one case of medulloepithelioma. The applied implants were: Castroviejo--in 28 patients, Medpor--in 24 patients, Hydroxyapatite--46 patients. In one patient was applied Baush and Lomb orbital implant. RESULTS: In 91 patients (91.9%) no significant complications occurred. In 2 patients (2%), the hemorrhage occurred during the surgery. Postoperative complications included: in 6 patients (6.1%) exposition of orbital implant was observed; in one patient the exposition was enlarging which led to removal of the implant; in 2 patients with implant exposition (2%) inflammatory granuloma occurred on the edge of the exposition. In one patient (1%) cyst of conjunctiva in the postoperative scar area occurred 3 months after operation. CONCLUSIONS: Enucleation with orbital implant enables normal development of the orbit and improves cosmetic effect. The frequency of complications depends on type of applied orbital implant.

[Surgical treatment of lens dislocation in children--analysis of complications]. / Leczenie operacyjne przemieszczonej soczewki u dzieci--analiza powiklan.

PURPOSE: To present the surgical complications in eyes with ectopia lentis in children. MATERIAL AND METHODS: 116 eyes of 65 children were operatively treated for lens dislocation. Pars plana lensectomy, vitrectomy and IOL implantation with scleral fixation were done in all eyes. Time of observation ranged between 1-6 years (mean 3.4). RESULTS: There were no serious intraoperative complication (mainly hemorrhage to anterior and posterior chamber). Postoperative complications included retinal detachement in 3 (2.6%) eyes and endophthalmitis in 1 (0.8%) eye, were the major complications observed in all patients. Complications related to scleral fixation included exposure of the scleral fixation suture in 8 (6.9%) eyes, lens decentration in 9 (7.8%) eyes, pupil capture in 3 (2.6%). CONCLUSIONS: The surgical procedure is useful and safe in the management of subluxated lenses in children.

[Scleritis posterior in child--case report]. / Zapalenie tylnej czesci twardówki u dziecka--opis przypadku.

We present the case of 13-years old girl with recurrent unilateral painful proptosis and associated with other ocular features: loss of vision, disc edema, macular edema, folds at the posterior pole. Ultrasound examination with CT scan were necessary to make the differential diagnosis of orbital tumors. The patognomonic sign "T" on B scan was extremely essential in the clinical diagnosis of posterior scleritis.

[Surgical management of pathologic filtering blebs in pediatric glaucoma]. / Chirurgiczne leczenie nieprawidlowych pecherzyków filtracyjnych u dzieci i mlodziezy.

The aim of this paper is to present our own experience in the treatment of cystic blebs in children and young adults. This complication, which is the most popular after trabeculectomy with antimetabolic agents, can be the reason of late endophthalmitis. Surgical treatment is one of the method to prevent it. In the study 6 patients with cystic blebs after trabeculectomy with mitomycin C was presented. 4 of them was treated surgically. We described the method of surgery and the results.