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The increasing urbanization of ecosystems has had a significant impact on wildlife over the last few years. Species that find an unlimited supply of food and shelter in urban areas have thrived under human presence. Wild birds have been identified as amplifying hosts and reservoirs of Campylobacter worldwide, but the information about its transmission and epidemiology is still limited. This study assessed the prevalence of Campylobacter in 137 urban birds admitted at a wildlife rescue center, with 18.8% of individuals showing positive. C. jejuni was the most frequent species (82.6%), followed by C. coli and C. lari (4.3% each). The order Passeriformes (33.3%) showed significant higher presence of Campylobacter when compared to orders Columbiformes (0%) and Ciconiiformes (17.6%), as well as in samples collected during the summer season (31.9%), from omnivorous species (36.8%) and young individuals (26.8%). Globally, Campylobacter displayed a remarkable resistance to ciprofloxacin (70.6%), tetracycline (64.7%), and nalidixic acid (52.9%). In contrast, resistance to streptomycin was low (5.8%), and all the isolates showed susceptibility to erythromycin and gentamycin. The results underline the importance of urban birds as reservoirs of thermophilic antimicrobial-resistant Campylobacter and contribute to enhancing the knowledge of its distribution in urban and peri-urban ecosystems.

Purpose. Basal cell carcinoma (BCC) is the most frequent malignant periocular tumor. It is associated with exposure to ultraviolet radiation, and its incidence is gradually increasing. It may occasionally display more aggressive behavior and result in orbital or intracranial invasion. Mortality from periocular BBC with orbital invasion is very low, but the associated morbidity can be significant, from disfigurement to blindness. Traditionally, these cases have been treated with orbital exenteration or with radiotherapy (RT), but in recent years, hedgehog pathway inhibitors (HPIs) have emerged, are effective in more serious cases, and are used primarily or combined with surgery, changing our perspective on the management of these patients. Methods. We studied 24 cases of periocular BCC with orbital invasion, some primary and others recurrent, which were treated between 2011 and 2021 in the same hospital. All patients had clinical or radiological evidence of orbital invasion. Orbital exenteration was performed on 9/24 of the patients (1 received vismodegib after surgery), and 12/24 were treated, surgically preserving the eyeball, with 3 of them receiving adjuvant vismodegib. Three of the twenty-four patients were treated exclusively with vismodegib (Erivedge®, Genentech). Results. One patient died due to poor tumor evolution, but the rest evolved favorably and they have had no recurrences. Vismodegib was generally well tolerated, except for in one patient who discontinued treatment due to the side effects. Conclusions. In advanced BBC with orbital invasion, mutilating surgical treatments such as exenteration or potentially vision-threatening treatments such as RT remain as options. In recent years, however, very promising new medical therapies have emerged, such as HPI, which can be used effectively instead of surgery or in combination with it, preserving the eye and vision, which implies a new approach to treatment.

Injuries and bone fractures are the most frequent causes of admission at wildlife rescue centers. Wild birds are more susceptible to open fractures due to their anatomical structure, which can lead to osteomyelitis and necrosis. Antibiotic therapy in these cases is indispensable, but the increase of antimicrobial-resistant isolates in wildlife has become a significant concern in recent years. In this context, the likelihood of antibiotic failure and death of animals with infectious issues is high. This study aimed to isolate, identify, and assess the antimicrobial resistance pattern of bacteria in wounds and open fractures in wild birds. To this end, injured birds admitted to a wildlife rescue center were sampled, and bacterial isolation and identification were performed. Then, antimicrobial susceptibility testing was assessed according to the disk diffusion method. In total, 36 isolates were obtained from 26 different birds. The genera detected were Staphylococcus spp. (63.8%), Escherichia (13.9%), Bacillus (11.1%), Streptococcus (8.3%), and Micrococcus (2.8%). Among Staphylococcus isolates, S. lentus and S. aureus were the most frequent species. Antimicrobial resistance was detected in 82.6% of the isolates, among which clindamycin resistance stood out, and 31.6% of resistant isolates were considered multidrug-resistant. Results from this study highlight the escalating scope of antimicrobial resistance in wildlife. This level of resistance poses a dual concern for wildlife: firstly, the risk of therapeutic failure in species of significant environmental value, and, secondly, the circulation of resistant bacteria in ecosystems.

Background: Glaucoma stands as a prominent contributor to irreversible vision impairment on a global scale. For decades, the Baerveldt Glaucoma Implant (BGI) has been used to treat refractory glaucoma. Yet, the cost‐effective Aurolab Aqueous Drainage Implant (AADI) has gained clinical attention as a viable alternative for managing glaucoma. Objective: The purpose of this study was to evaluate and compare the efficacy and safety of AADI and BGI in the treatment of refractory glaucoma. Methods: Following PRISMA guidelines, we conducted a systematic search of multiple databases, identifying relevant comparative studies assessing AADI versus BGI in patients with refractory glaucoma. Key outcomes included postoperative IOP, surgical success rates, antiglaucoma medication reduction (AGMR), and complication rates. Quality assessment was performed using the Newcastle–Ottawa Scale (NOS). Results: Three studies comprised a total of 176 individuals with refractory glaucoma, with 107 patients receiving the AADI and 69 patients receiving the BGI. The meta‐analysis revealed a statistically borderline significant reduction in postoperative IOP favoring the AADI at 3 months (mean difference [MD] = −2.74, p = 0.05). There was no significant difference in the MD of AGMR between the AADI and BGI groups. The rates of total complications and surgical success did not differ significantly between the AADI and BGI groups. Conclusion: AADI demonstrates promising results in reducing IOP at 3 months compared to BGI, with comparable surgical outcomes and complication rates over the long term. Further studies with larger samples are warranted to validate these findings and assess cost‐effectiveness, particularly in developing countries. [ABSTRACT FROM AUTHOR]

Background: No consensus has been reached on the adequate dose of mitomycin C (MMC) in XEN45 gel stent implantation. Lower doses have the potential to reduce MMC‐linked side effects. This study aimed to evaluate treatment efficacy of ab interno XEN45 gel stent in primary open‐angle glaucoma (POAG) with three different MMC doses. Methods: This retrospective single‐centre nonrandomised trail included 54 patients (56 eyes) who underwent XEN45 gel stent implantation for POAG with above‐target intraocular pressure (IOP) under medical therapy. Eyes were grouped according to the received MMC dose: Group 1 (20 μg; n = 21), Group 2 (10 μg; n = 14) and Group 3 (5 μg; n = 21). The primary endpoint was the mean IOP change in the three MMC dose groups after 6, 12 and 24 months. Secondary endpoints included the success rate defined as lowering of baseline IOP ≥ 20% and below a cut‐off IOP set at three different levels: ≤ 18, ≤ 16 and ≤ 14 mmHg (Criteriums 1, 2 and 3), the mean number of ocular hypotensive medications and the frequency of needling procedures. Results: After 24 months, the overall mean (standard error) IOP was significantly reduced from 24.7 (0.9) mmHg to 15.2 (0.7) mmHg (p < 0.0001). The average IOP change (standard error) in MMC dose groups 1, 2 and 3 was −8.6 (2) mmHg, −10.1 (2.1) mmHg and −10.4 (2.8) mmHg. Complete success (Criterium 1) was achieved in 50%, 62% and 43% of the eyes in groups 1, 2 and 3. No statistically significant difference was found within the first 24 months between the three MMC dose groups for IOP change, success rate, number of ocular hypotensive medications and the frequency of needling procedures. Conclusions: XE45 was effective in all three dose groups. As the success rate did not significantly differ between the MMC doses, these results may support the use of the lowest dose. Trial Registration: ClinicalTrials.gov identifier: 559/2016BO2 [ABSTRACT FROM AUTHOR]

The superior orbital fissure contains cranial nerves III, IV, VI, and V1 with their three branches: frontal, lacrimal, and nasociliary. Superior orbital fissure syndrome (SOFS) is rare and can occur as a result of compression of these nerves due to trauma, bleeding, or inflammation in the retrobulbar space, but no cases of SOFS after deep lateral orbital wall decompression (DLOWD) have been reported. The aim of this paper is to describe this pathology, its possible causes, management, and outcome. Retrospective study of 575 DLOWD in patients with disfiguring exophthalmos due to Graves' ophthalmopathy performed in our hospital between 2010 and 2023. Three cases of postoperative SOFS were identified based on clinical presentation, history, physical examination, and radiological study. All patients were observed for a minimum of 12 months. SOFS was diagnosed with the presence of ophthalmoplegia, ptosis, fixed and dilated pupils, hypo/anesthesia of the upper eyelid and forehead, loss of corneal reflex, and no loss of vision after DLOWD. Fractures, edema, and hemorrhages were excluded. They were treated with high-dose intravenous steroids and the patients recovered completely. DLOWD challenges orbital surgeons because it requires removing bones near the globe or neurovascular structures. SOFS may occur due to the proximity and increased pressure on these structures. [ABSTRACT FROM AUTHOR]

Most hemoparasites hosted by wild birds appear to be harmless, but most of the blood parasite studies in avian wildlife are mainly focused on passerines or migratory species. This study aimed to assess the occurrence of blood parasites in nocturnal raptors (Strigiformes order) and their effect on hematological parameters. A total of 134 blood samples were collected during a four-year period for hematological analysis and hemoparasite detection and quantification by microscopical examination of the samples. Overall, the occurrence of hemoparasites was 35.1%, with Leucocytozoon being the most frequently detected (32.1%), followed by Haemoproteus (11.2%), Trypanosoma and Plasmodium (2.2% each). Among the different bird species, the Eurasian eagle-owl (Bubo bubo) showed the highest blood parasite positivity (94.7%). In barn owls, the positive birds displayed a lower hematocrit measurement and body condition score than the non-parasitized ones (p = 0.007 and p = 0.005, respectively), especially those parasitized by Leucocytozoon. Moreover, the analysis of the magnitude of this association revealed that the presence of hemoparasites is five times more frequent in barn owls with a 2/5 body condition score. Despite the host–parasite coevolution in Strigiformes, our results show a correlation between the presence of hemoparasites and some health parameters, including blood parameters.

Salmonella is mostly noted as a food-borne pathogen, but contact with chelonians has also been reported as a source of infection. Moreover, high levels of antimicrobial resistance (AMR) have been reported in Salmonella isolated from wild and captive reptiles. The aim of this study was to assess the occurrence of Salmonella AMR carriage by chelonians admitted to two zoological institutions in Spain, characterizing the isolates to assess the Salmonella AMR epidemiology in wildlife. To this end, 152 chelonians from nine species were sampled upon their arrival at the zoological nuclei. Salmonella identification was based on ISO 6579-1:2017 (Annex D), isolates were serotyped and their AMR analysed according to the EU Decision 2013/652. Moreover, the genetic relationship of the isolates was assessed by pulsed-field gel electrophoresis (PFGE). Results showed 19% (29/152) of the chelonians positive to Salmonella, all of them tortoises. For all isolates, 69% (20/29) were resistant and 34% (10/29) multidrug-resistant (MDR) strains. PFGE clustered isolates according to the serovar, confirming a low genetic diversity. In conclusion, this study shows a high presence of MDR Salmonella strains in tortoises at their entry into zoological nuclei. This condition highlights the need to establish Salmonella detection protocols for the entry of animals into these centres.

Giant cell reparative granuloma (GCRG) is a rare fibroosseous lesion uncommonly seen in the orbital area. Although benign, it is known to be recurrent and locally destructive. We report two cases of GCRG of the orbit. In both cases, computed tomography revealed a heterogeneously growing well-defined mass, arising from the roof of the orbit, affecting the cortex, and invading the orbit. In the first case, the mass extended into the anterior cranial fossa. Magnetic resonance imaging with gadolinium showed, in both cases, a cystic character of the lesion with fluid levels. The surgical treatment was performed via an upper crease incision. An ultrasonic aspirator system was used to remove the tumor tissue and its extension into cranial fossa. Careful histopathologic analysis established the diagnosis of GCRG. Symptoms resolved completely with no evidence of recurrence after a follow-up of 18 and 14 months, respectively. We present the clinicopathological and radiological findings, and we describe the surgical approach. As a rare entity, GCRG of the orbit should be considered in differential diagnosis of fibroosseous orbital masses. Complete surgical excision carries a low risk of recurrence.

Conjunctival neoplasia is one of the most frequent tumors in the eye. Actinic keratosis (AK) or solar keratosis is a precancerous lesion that is included with other epithelial tumors. This alteration does not break the basal membrane. There is enough evidence of successful outcomes to consider interferon alfa-2b (IFN alfa-2b) as the first choice of treatment for this type of tumors. In addition, side effects are mild and uncommon. We report a case in an 83-year-old woman who was referred to evaluate a leukoplakia in the tarsal conjunctiva of the lower left eyelid that measured 1 cm in diameter. Pathological study revealed AK. After the INF alfa-2b treatment, we observed conjunctival hyperemia, noninfiltrated upper nasal de-epithelization, and inferior nasal bulla. AK with presentation in conjunctiva is rarely described and in tarsal conjunctiva is exceptional. It is the first case published with only tarsal conjunctiva affectation.

Localized or isolated neurofibromas are peripheral nerve sheath tumors. They are rare in the orbit and occur without a systemic neurofibromatosis. There are few cases of bilateral tumors reported but none affecting both supraorbital and infraorbital nerves. We report a 45-year-old female who presented an extraconal mass in the right orbit as an incidental finding in a head computer tomography, without ocular symptoms. Magnetic resonance image showed a well-defined oval mass in the right supraorbital and infraorbital nerves, of similar characteristics, as well as smaller masses in the left supraorbital and infraorbital nerves. A progressive increase in size of the left supraorbital and infraorbital tumor motivated their surgical excision. The histological result was compatible with a neurofibroma. These uncommon orbital tumors are slow growing and affect the sensory nerves of the trigeminal nerve. Neurofibromas usually present progressive symptoms due to the orbital mass, proptosis, or visual changes although not in this case. Surgical removal is the only definitive treatment.

Purpose. To retrospectively describe the clinical characteristics, management, and outcomes of four cases of orbital solitary fibrous tumor (SFT). In one patient, we present an ultrasonic aspirator system for tumor removal. Methods. Four patients with orbital SFT were selected: one patient with orbital SFT, another patient with frontal and ethmoidal SFT and orbital affectation with high rates of recurrence, the third patient with frontal lobe SFT and orbital invasion with multiple recurrences, and the fourth case with a history of craniopharyngioma surgery and SFT located on the orbital apex. Results. All cases showed proptosis, eye movement restriction, and, in three cases, visual acuity alteration. Different treatments were applied: in three cases, excision was performed, one of them with an ultrasonic aspirator system, and in the remaining case, an exenteration was done (in two cases, radiosurgery treatment was also applied). The immunohistochemical study revealed SFT, similar to hemangiopericytomas (HPCs). No recurrence has been observed after surgical treatment. Conclusion. The SFT is a spectrum of different tumors with similar histopathological characteristics. The use of immunohistochemical markers is very helpful in the diagnosis. The main problem of orbital involvement is the risk of damaging important structures adjacent to the tumor during the surgical removal. The ultrasonic aspirator system allows elimination of the tumor without damaging other orbital structures.

Vascular iris lesions are rare and can sometimes be associated to systemic vascular lesions. They usually cause spontaneous recurrent hyphema. The differential diagnosis should be considered primarily with iris rubeosis and with highly vascular tumors as iris melanoma. Generally, vascular lesions of hemangioma type are located in the iris without extension to iridocorneal angle. We present a case of a 77-year-old male with an iris vascular lesion suggestive of racemose hemangioma, who is asymptomatic, and with 360-degree iridocorneal angle affectation showing no lesions at any other location.

Purpose. To analyze patients’ anxiety levels using the Visual Analog Scale for Anxiety (VASA), in regard to intravitreal injection treatment and to determine possible associated risk factors related to the disease and treatment characteristics. Methods. Cross-sectional observational study with consecutive sampling of patients who were going to receive an intravitreal injection. Subjects completed the VASA prior to the procedure, and afterwards, their data were collected from the electronic medical history. Analysis was performed through a linear regression model. Results. Fifty-five men and forty-seven women were enrolled. The mean age was 73.9 ± 12.4 years (mean ± standard deviation (SD)), and the mean ± SD of previous injections was 12.8 ± 12. The most frequent pathologies found were age-related macular degeneration with 46.1% and diabetic macular edema with 36.3%. The median of anxiety levels measured in millimeters (mm) was 16 (interquartile range: 0–48). In univariate models, women presented a mean of 10.8 mm of anxiety more than men (p=0.03). The adjusted multivariate analysis demonstrated that younger patients declared higher anxiety levels (p=0.036). No significant association was found between the best corrected visual acuity (BCVA) on the day of the injection, the change in BCVA since the beginning of the treatment or the number of injections received, and the registered anxiety levels. Conclusions. Sex and age may have an influence on anxiety levels. BCVA and the number ofinjections received did not seem to have an influence on our patients anxiety levels.

Introducción: La histiocitosis de células de Langerhans es una causa infrecuente de tumor orbitario. El diagnóstico definitivo es anatomopatológico. Presentación del caso: Varón de 16 años con edema palpebral y ptosis derecha. Las pruebas de imagen mostraron una masa orbitaria que producía erosión ósea. El estudio sistémico fue negativo. Se realizó escisión quirúrgica del tumor, siendo el resultado del análisis histopatológico histiocitosis de células de Langerhans. Tras 17 años de seguimiento, no se han encontrado signos de recidiva. Discusión: En pacientes con lesiones sugestivas de histiocitosis de células de Langerhans es importante la estadificación de la enfermedad para planificar el tratamiento, que puede ir desde la cirugía hasta la quimioterapia. Conclusiones: El curso de la enfermedad es impredecible, por ello es muy importante la estadificación de la enfermedad: monosistémica (monofocal o multifocal) o multisistémica. Dado que pueden presentarse recidivas tardías, es necesario el seguimiento del paciente a largo plazo.

Purpose. We describe a modification of Cutler-Beard’s technique, using a contralateral tarsoconjunctival graft, in patients who underwent excision of large malignant tumors of the upper eyelid. Methods. This is an interventional retrospective review (clinical study) of ten cases, with age range from 64 to 86 years (mean: 71.2 years ± 6.49) with malignant upper eyelid tumors, operated by the same surgeon (AB-G), between 2011 and 2016. The resulting defects were reconstructed using a modification of Cutler-Beard’s technique. A tarsoconjunctival graft was harvested from the contralateral upper eyelid, with an extra 3 mm of conjunctiva from the superior edge of tarsus and was attached to the upper eyelid defect, different from that previously described. Follow-up ranged between 24 and 60 months (mean: 41.6 ± 9.87). Functional, cosmetic outcomes and postoperative complications were evaluated. Results. No upper eyelid retraction, eyelid margin entropion, or graft retraction was observed in any of the cases. All of the patients were satisfied with the aesthetic result. Conclusion. This technique allows us to safely inset a suitable graft on the ocular surface, with sufficient blood supply, resulting in a stable margin and good contour.

Purpose. To describe the results of thyroid-related orbitopathy (TRO) treated by ultrasonic deep lateral wall bony decompression with partial rim sparing (DLW-PRS). Methods. A review was carried out, from January 2015 to September 2017, of all patients treated with ultrasonic DLW-PRS decompression using a SONOPET® (Stryker, Kalamazoo, MI, USA) ultrasonic aspirator, using a lateral, small triangle flap incision for access. The primary outcome was the change in proptosis (measured by the difference in Hertel exophthalmometry measurements). Other secondary outcomes were changes in visual acuity (VA) (using Snellen scale, decimal fraction), presence of lagophthalmos, eyelid retraction (measured by upper eyelid margin distance to the corneal reflex (MRD1) and lower eyelid margin distance to the corneal reflex (MRD2), and presence of exposure keratopathy). Results. A total of 58 orbital decompressions in 35 patients were reviewed, with 23 patients (65.7%) having bilateral decompressions. There was a female preponderance with 26 patients (74.2%), and the mean age ± standard deviation was 52.6 ± 13.9 years. Mean proptosis was 24.51 ± 1.76 mm preoperatively, reduced to 19.61 ± 1.27 mm in final follow-up. The mean reduction was 4.9 ± 1.54 mm. VA improved from 0.8 ± 0.14 to 0.9 ± 0.12, p=0.039. 5 of 13 patients (38.4%) with preoperative diplopia reported improvement or complete resolution after surgery. MRD1 was reduced from 5.25 ± 0.88 mm to 4.49 ± 0.7 mm. MRD2 was also reduced from 6.3 ± 0.88 mm to 5.0 ± 0.17 mm. Presence of lagophthalmos was reduced from 35 eyes (60.3%) to five (8.6%); the presence of epiphora was also reduced from 20 patients (57.1%) to 3 (8.5%) following decompression. Complications of the surgery included zygomatic hypoaesthesia in 14 (40%) patients in the early postoperative period and chewing alterations in 10 (28.5%) of the patients. All of these complications were resolved at the 6-month follow-up visit. We noted no surgical complications such as ocular or soft tissue damage, infection, inflammation, or visual loss. Conclusions. The SONOPET® ultrasonic bone curette can be used safely and effectively for DLW orbital decompression surgery. The main benefits were good visualization and handling of tissues and speed and ease of use of the equipment. This trial is registered with ClinicalTrials.gov identifier: NCT04025034.

Purpose: Mucormycosis is an infection caused by fungi to the class Zygomycetes that usually appears in immunosuppressed patients. Diagnostic confirmation is often delayed, with fatal prognosis in cases in which treatment is not rapidly established. Case report: We present two clinical cases of rhino-orbito-cerebral mucormycosis with an atypical presentation form, consisting of a unilateral complete sudden vision loss. Intravenous treatment with liposomal amphotericin B was started and total orbital exenteration surgery was performed. The removed surgical area was filled with gauze impregnated with liposomal amphotericin B and was left open for cures every 12 hours. Due to the good clinical evolution, a reconstruction of the orbital exenteration defect was performed in Case 1 with a temporal muscle flap and a skin island pedicled flap. In Case 2, reconstruction was not performed due to the poor evolution of the patient. Discussion: As it is a very aggressive surgery, the aesthetic and functional sequelae are very important. When the survival of the patient is achieved, we should offer reconstructive solutions that improve their quality of life. The reconstruction carried out using a flap of the temporal muscle can be made in a single act without requiring microvascular surgery.

Penetrating injuries of the cranium are relatively uncommon, only 0.4% of all head injuries. In patients with disturbed conscious level, an extensive examination should be performed in the emergency unit to rule out transorbital penetrating brain injury. A 25-year-old male was attacked with a dagger. He presented with ethylic intoxication and the physical examination demonstrated a small skin injury on the lateral canthus of the left eye with a large periocular hematoma which prevented eyelid opening. Cranial CT scan showed a metallic intraorbital foreign body consisting of a fragment of a dagger which perforated the eyeball, and penetrated through the superomedial wall of the orbit into the anterior cranial fossa. Reconstruction of the eyeball was performed and the fragment was removed. Orbital injuries with a knife in situ are very unusual. Early identification and removal of retained foreign bodies are essential.

María J Suárez-Fernández, Antonio Clariana-Martín, Enrique Mencía-Gutiérrez, Esperanza Gutiérrez-Díaz, Teresa Gracia-García-MiguelOphthalmology Department, 12 de Octubre Hospital, Complutense University, Madrid, SpainPurpose: The risk of visual loss after nonocular surgeries is very low, between 0.2% and 4.5%. According to the American Society of Anesthesiologists, ischemic optic neuropathy has been reported mostly after spinal surgery (54.2%), followed by cardiac surgery and radical neck dissection (13.3%). It may occur in association with some conditions that include systemic hypotension, acute blood loss and hypovolemia.Case report: A 46-year-old woman, whose diagnosis was laryngeal squamous cell carcinoma, complained of visual loss in her right eye two days after surgery (laryngectomy with bilateral radical neck dissection and left jugular ligature) and one day later in her left eye. The diagnosis was nonarteritic anterior ischemic optic neuropathy.Conclusion: Anterior ischemic optic neuropathy related to nonocular surgery is usually bilateral and its prognosis is very poor, resulting in blindness or severe visual loss. Although rare, patients should be warned about this complication, which has a profound impact on quality of life, since no therapeutic measure, including correction of hypotension and anemia, seems to improve the prognosis of this complication.Keywords: ischemic optic neuropathy, visual loss, radical neck dissection, blood loss

Juan D Martínez-Gutiérrez1, Enrique Mencía-Gutiérrez1, Esperanza Gutiérrez-Díaz1, José L Rodríguez-Peralto2Departments of 1Ophthalmology and 2Pathology, 12 de Octubre Hospital, Complutense University, Madrid, SpainAbstract: Wegener’s granulomatosis (WG) is a necrotizing granulomatous vasculitis characterized by the involvement of the upper or lower airways, lungs, and kidneys, but it can affect almost any organ including the orbit. WG is rare in childhood. This case report describes a 7-year-old girl who presented bilateral idiophatic orbital inflammation and antineutrophil cytoplasmic antibodies-negative titres. Computed tomography scan and magnetic resonance imaging showed enlargement of both lacrimal glands with infiltration. Treatment with corticosteroids achieved remission of the disease. Three years later, she developed a systemic affectation with tracheal stenosis, pulmonary affectation, renal failure, and respiratory tract mucosa inflammation. Lacrimal gland biopsy showed perivascular nonspecific granulomas and ANCA titres remain negative. Treatment with corticosteroids and cyclophosphamide was done. A relapse occurred 2 years later, with complete remission with antitumor necrosis factor-alpha. No other symptoms have appeared after 9 years of follow-up. Early diagnosis and treatment is crucial to increase the survival rate in these patients.Keywords: ANCA, children, idiopathic orbital inflammation, exophthalmos, vasculitis, Wegener’s granulomatosis