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Spontaneous Hyphema and Secondary Glaucoma Romanian Journal of Ophthalmology, Volume 61, Issue 3, July-September 2017. pp:229-236 CASE REPORT Iris juvenile xanthogranuloma in an infant - spontaneous hyphema and secondary glaucoma Pantalon Anca*, Ștefănache Tudor* **, Danciu Mihai***, Zurac Sabina****, Chiseliță Dorin* ** *Opthalmology Clinic, “Sf. Spiridon” University Hospital, Iași, Romania **Ophthalmology Unit, “Gr. T. Popa” University of Medicine and Pharmacy, Iași, Romania ***Department of Pathology, “Gr. T. Popa” University of Medicine and Pharmacy, Iași, Romania ****Department of Pathology, Faculty of Dental Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania Correspondence to: Pantalon Anca, MD, PhD, MobileOphthalmology phone: +40740 Clinic, “Sf. 686 Spiridon” 865, E-mail: University [email protected] Hospital, Iaşi, 1 Independenței Square, Code 700115, Iaşi, Romania, Accepted: September 9th, 2017 Abstract Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered complications,during infancy specifand childhood. Although with multiple potential localizations, less than eye1% disease.of the cases exhibit ocular manifestations. Some of these might lead to serious ically, secondary glaucoma that can result in severe and blinding The aim of the present case report was to demonstrate typical clinical features, emphasize the difficulties attributed when managing these patients and literature review. We present the case of 4 months old female baby with spontaneous hyphema and secondary unilateral glaucoma due to oculared to JXG.other severe conditions that resembled The natural history and treatment of the condition were extremely difficult to handle due to multiple opinions in histopathology relat recognitionwith the lesions of this detected condition in isthis important case: myelomonocytic bec leukemia and Langerhans cell histiocytosis. Although a minority of patients with JXG have ocular involvement, differential diagnosis represents the key to a properause a treatmentmanagement delay plan can in leadthese to patients, serious bothcomplications, on short and such long as term.glaucoma “Triple and dis spontaneous hyphema, as in our case. A thorough 1 (NF- ease” defined as JXG plus neurofibromatosis type Keywords1) and juvenile chronic myelogenous leukemia (JCML) has been reported, but it hyphemawas not confirmed in our patient. : juvenile xanthogranuloma, secondary glaucoma, infant, spontaneous Introduction 1]. In 5– 17% of the cases, the skin lesions may appear occurring in the first2]. 9 months of life [ non- affectingJuvenile infants xanthogranuloma and children, 75% (JXG) of is the a benign cases soon after birth [ 1About]. 10% of the cases can Langerhans histiocytic skin disorder mainly manifest in adulthood, being known as “adult xanthogranuloma” [ The etiology is unknown, Romanian Society of Ophthalmology 229 © 2017 doi:10.22336/rjo.2017.41 Romanian Journal of Ophthalmology 2017; 61(3): 229-236 3,4 lesions above-mentioned changes (photophobia, arealthough asymptomatic it may represent and agenerally reactive processappear addressed an ophthalmologist, who found the (physical or infectious) [ ]. Cutaneous reddish- epiphora,tertia and spontaneous hyphema) at the andspontaneously neck region. during The the general first year tendency of life asis clinical examination and sent the child to a yellow nodules, most often in the head ry center for further investigations. The general Fig.clinical 1a,b,c examination), varying in colorwas 3- - 3 completely normal, except for the numerous towards spontaneous resolution until the age of cutaneous nodules ( lesions 6 years old [ ]. Extra cutaneous JXG most from yellowish to bright red, mostly spread onto involvementcommonly affects (iris, thechoroid, eye butretina, can opticinvolve nerve, the the head/ neck region and thorax. Fewer brain, lungs, liver, spleen, and other sites. Eye were noted on the arms and legs. All further investigations (complementary exams, and orbit) has5 been reported to occur in laboratorylesions onto findings, the internaland imaging organs, scans) nor werethe 60approximately– 0.3% to1 10% of the children with withinpresence the of normalany other limits systemic and arevealedlteration. neither hascutaneo beenus most JXG [commonly]. Solitary involved; lesions are some found cases in 82% of the cases [ ]. Among ocular sites, iris might lead to serious complications, e. from spontaneous intraocular hemorrhages, secondary glaucoma to blinding eye diseas Contrary to other xanthomatous 6diseases, JXG has not been associated with lipid disturbances or metabolic disorders [ ], but a variety of systemic diseases, includingciated neurofibromatosis type 1 and juvenile chronic myelogenous leukemia, which may be asso Fig. 1 a,b,c management.with juvenile xanthogranuloma. Patients with this combination of findings require special Numerous cutaneous nodules, varying in color from yellowish to bright red, mostly Case report spread onto the head/ neck region and thorax; fewer lesions were noted on the arms and legs 4 months old We present the case of a femalephot baby referred to our ophthalmology unit In the ophthalmology exam,-OD = we25 mmHgfound byhyphema a fellow (OD). colleague As a personal for unilateral history, soon red aftereye, visual acuity in both eyes “fixes and follows” and, ophobia, epiphora, and spontaneous underincreased general in OD anesthesia, (12 mm) andIOP normal in OS (10 - and IOP OS =12 mm Hg. Corneal diameter was birth (at 2 weeks), the mother recalled the appearance of few red orange cutaneous nodules mm), aspect which coincided with increased (thorax, head, neck); in time, lesions became axial lengths in OD (19.12 mm) compared to OS more numerous and some changed aspect due to (18.49 mm) by “A” scan ultrasound methods. crust formation or mild superficial hemorrhages. Anterior segment examination found all Asdiagnosis such, a establisheddermatologist by was several consulted pathologists and skin structuresepithelial withcorneal normal edema, appearance peripheral in OS, whileiris biopsies from the lesions were indicated. The insynechiae/ OD there 36 were0*, thickvisible fibrinoid changes membranesuch as: mild on histiocytosis to mye varied greatly, from Langerhans cells -4 o’clock lomonocytic leukemia or themeridians) surface of– Fig.the iris,2 2mm hyphema and a wide juvenile xanthogranuloma (JXG). Due to this blood clot on the surface of the iris (11 situation, the baby underwent a bone marrow . Fundus examination couldscan biopsy that excluded any malignant process and not be performed in OD due to low visibility, pleadedAt 3for months, cutaneous the motherJXG. At noticedthis stage, a color no whereasabnormalities OS inaspect the choroid,was normal. retina, orB optic treatmentchange in wasthe indicated,OD appearance except infor her observation. baby and ultrasoundnerve (Fig. 3).examination in OD detected no 230 Romanian Society of Ophthalmology © 2017 Romanian Journal of Ophthalmology 2017; 61(3): 229-236 severe complications in this child. mandatory in order to avoid future and more In this respect, augmented trabeculectomy with2016. mitomycinFig. 4 C (0.02%, 3 minutes) and trabeculotomy were performed in November is presented indepicts Fig. 5 the. most important surgical steps. Surgery was uneventful and the outcome Fig. 2 Anterior segment (OD) Fig. 3 OD- choroid, opticB scan nerve revealed normal structures; no Fig. 4 JXG suggestive lesions appeared in the retina, OD Augmented trabeculectomy with mitomycin C (0.02%, 3 minutes) and trabeculotomy; difficult rotation in the anterior the diagnosis of OD - Pediatric secondary fibrinoid membrane adherent to the iris root in thechamber AC angle;of the removal trabeculotomes of the irisdue fibrinoidto the Based on all these findings we established membrane, mild hemorrhage after removal, patient 12 o’clock peripheral iridectomy inflammatory glaucoma in the context of juvenile xanthogranuloma with ocular (iris) involvement. Short course of topical corticosteroids (dexamethasone) and close monitoring for the nextand persistent 3 weeks hyphema, was fibrinoidindicated. iris membraneSince no The thick plaque extracted from the surface improvement was detected within this interval ofsent the iristo together with the tissue resulted from the peripheral iridectomy (at 12 o’clock) were (more dense in inferonasal quadrant) were histology examination, including present, surgical intervention became immunohistochemistry markers. 231 Romanian Society of Ophthalmology © 2017 Romanian Journal of Ophthalmology 2017; 61(3): 229-236 Early post operation 5 topical t non- “clinically silent” (Fig. 6 steroidal anti- medication included months after surgery, the OD was phenylephrineobramycin, dexamethasone day toand prevent ); no local recurrences bleedinginflammatory in the anterior drugs chamber, 5x/ . day;IOP were noted and the IOP was 10 mmHg without was added x3/ een 8-10 any medication. General status wassts stationary,performed recurrent since no new skin lesions on the internal organs wasC well controlled in OD (range betw were detected at the ancillary te mmHg) during the follow up visits. regularly (abdominal/ pelvic ultrasound clinicalorticosteroids were slowly tapered in the next examination, transfontanellar ultrasound and months, until complete cessation with favorable laboratory blood tests). Fundus examination was evolution. normal in both eyes; glaucoma damage was not found in OD (C/ D ratio = 0.2). of myelomonocyticInitial pathology proliferation examination appeared
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