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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.

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.

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.

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.

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.

Purpose: To share our experience on deep lateral wall rim-sparing orbital decompression for the prevention of further spontaneous globe subluxation, in patients with shallow orbits and eyelid laxit...

Purpose To report a case of vitreociliary block (VCB) six months after a laser posterior capsulotomy (LPC). Case report A 25-year-old man with uveitis, retinal vasoproliferative tumor, cataract, and acute angle-closure glaucoma due to pupillary seclusion, which required laser iridotomies, implantation of an Ahmed valve, phacoemulsification, and LPC. Six months after capsulotomy, he presented a generalized flattening of the anterior chamber (AC) and ocular hypertension, with patent iridotomies. Hyperechoic anterior hyaloid and hypoechoic spaces in the vitreous were seen in ultrasound imaging. The VCB did not respond to pharmacological treatment and was solved immediately after laser hyaloidotomy. Conclusion There are three cases of VCB after LPC described in the literature. Our patient presented a chronic inflammatory process that generated an inflammatory membrane at the level of the anterior hyaloid with adhesion to the ciliary processes, causing posterior misdirection of the aqueous humor, decreased permeability of the anterior hyaloid, and finally, VCB.

Wild birds have been identified as a relevant reservoir of Campylobacter spp., therefore, a potential source of infection in humans and domestic animals. The objective of this study was to determine the occurrence of Campylobacter spp. on birds of prey in Spain. In addition, antibiotic resistance profiles of the isolates were evaluated. A total of 689 specimens of 28 raptor species were analyzed, with a resulting individual prevalence of 7.5%. C. jejuni was the most frequently isolated species (88.5%), followed by C. coli and C. lari (3.8% each). The occurrence of Campylobacter was significantly higher (p 0.05) in nocturnal birds of prey (15.3%), in spring season (12.2%) and in carnivorous species (9.4%). Isolates displayed a remarkable resistance to nalidixic acid (69.9%), ciprofloxacin (69.9%), and tetracycline (55.6%), and a low resistance to streptomycin (6.7%). Our findings highlight the importance of birds of prey as reservoirs of Campylobacter strains and their significant role as carriers of antimicrobial resistance.

Purpose: Orbital tumor surgery can be challenging when the tumor is located in a very narrow surgical field and close to important structures, such as nerves or extraocular muscles that can be damaged during surgery. Advances in technology and special surgical techniques help to avoid such damage. We describe our experience using SONOPET® ultrasonic surgical aspirator to remove 12 different orbital tumors that were difficult to treat due to their poorly defined borders, adhesions, or location.Methods: This is a retrospective case series that describes 12 operations that occurred between March 2016 to December 2018 using an ultrasonic device to debulk or remove orbital tumors. Different approaches and handpieces were used for each case depending on the location and consistency of the tumor.Results: All patients experienced an improvement in preoperative signs and symptoms, pain, proptosis, diplopia, or lagophthalmos. Visual acuity, which had been reduced due to the tumor, was also recovered. There were no intraoperative or postoperative complications due to the use of the device.Conclusions: The ultrasonic aspirator is a safe, useful device that can successfully remove or debulk infiltrating orbital masses through any orbital access, regardless of their consistency. It is helpful in cases of difficult anatomical access or difficult extraction due to size or adhesions to the surrounding tissues.

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.

A non-traumatic or spontaneous orbital hemorrhage (NTOH) is usually caused by the presence of an orbital mass, an inflammation, an infection, a bleeding disorder or those called idiopathic. This entity usually affects elderly adults and some risk factors can be identified. The NTOH normally acts like a benign and self-limited process, but attending to its anatomical pattern, may need specific management. A 64-year-old male referred to us with sudden binocular double vision, without loss of visual acuity (VA) or pain immediately after fitting the FFP2/NK95 facial mask for air protection during the pandemic COVID-19. He presented sudden orbital-subconjunctival-eyelid cutaneous hemorrhage-hematoma with conjunctival protrusion from the palpebral fissure without proptosis. There was also limitation in adduction and a cutaneous hematoma in the inner third of the lower eyelid. After 48 hours, the diplopia had resolved and ocular motility was completely re-established with persistence of a massive hyposphagma. No radiological image test was performed due to the COVID-19 epidemiological situation, as the patient was in good systemic situation and it was not a vital emergency. The evolution was favorable with conservative treatment, cold application, and moisturizing eye drops. After 10 days, the bruising was almost completely gone. During the ongoing novel coronavirus disease (COVID-19) pandemic caused by the novel enveloped RNA virus named severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), face mask use has drastically increased among the healthcare professionals and the general population. The importance of this case lies in the new adverse effects caused by the misuse of mandatory face masks in the general population.

Poroma is a rare benign tumor of the epidermal sweat duct unit with predilection for the head and neck. Only six cases with eyelid location have been described in the literature (PubMed). A 34-year-old male presented with a single tumor on the left upper eyelid. It was skin-colored, nodular, solid, tender with some telangiectatic vessels, and showed no ulcerated lesion. Clinical diagnosis was basal cell carcinoma. This type of lesion can mimic a malignancy. Complete excisional biopsy revealed features consistent with eccrine poroma. After three year of follow up, no recurrence was observed. The authors reviewed all the cases reported in the literature and made a summary comparing them.

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.

Introduction Anti-thrombotic drugs (antiplatelets and anticoagulants) are widely used for different clinical reasons. This means that there are an increasing number of patients undergoing elective ophthalmic surgery that are being treated with these drugs. A better knowledge of their implications and their peri-operative use may help to prevent surgical and secondary adverse events. There is often no firm recommendation on how to manage certain drugs in certain surgeries. Objective To review the recommendations in the scientific literature as regards managing anti-thrombotic agents during the peri-operative period of ophthalmic surgery. Material and methods A review was made of the relevant guidelines and studies using an antiplatelet and anticoagulant drugs approach for cataract, vitreo-retinal, glaucoma, oculoplastic, and strabismus surgeries. Results Recommendations about whether to continue or discontinue anti-thrombotic drugs in the peri-operative period of different ophthalmic surgical fields are presented. Conclusions There are only firm recommendations of maintaining anti-thrombotic drugs as regards cataract surgery using phacoemulsification with topical anaesthesia. In other surgical fields, ophthalmologists should balance the risk of thromboembolic events and risks of haemorrhagic complications in order to carry out a proper management. A multi-disciplinary approach is recommended for complex cases. Additional studies should be performed to better characterise the peri-operative use of anti-thrombotic agents in order to prepare clinical guidelines for ophthalmic surgery.

Purpose. To examine the magnitude, orientation, and age-related changes of corneal astigmatism of the eyes before cataract surgery. Setting. Hebei Eye Hospital, Hebei, China. Design. A clinical-based retrospective study. Results. The study consisted of 5662 eyes of 5662 consecutive cataract surgery patients with a mean age of 68.26 ± 10.39 (mean ± standard deviation (SD)) years (range 40 to 97 years), and 59.86% of the patients were women. Mean corneal astigmatism was 0.98 ± 0.76 diopter (D) (range 0.00–9.61 D). Corneal astigmatism of 0.50–0.99 D was the most common range of values (30.08%), followed by 1.00–1.49 D (22.15%), ≤0.50 D (21.21%), and 1.50–1.99 D (10.28%). There was a strong U-shaped relation between corneal astigmatism and age ( p for nonlinearity p = .001 ; r = 0.10, p = .010 , respectively). However, in the old patients with age above and equal to 65 years, all types of astigmatism were positively correlated with age. Conclusion. This study may provide valuable and practical information to surgeons when selecting the appropriate surgical method and toric intraocular lens (IOLs).

Specific genetic deficiencies are a rare cause that should be included in the diagnostic algorithm of disseminated herpetic lesions. The aim of this article is to describe the ocular herpetic manifestations in a rare genetic disorder called GATA2 deficiency. We present the clinical case of a 26-year-old male with dendritic ulcers in his cornea, marrow aplasia and idiopathic chronic lymphedema. He was diagnosed with GATA2 deficiency. GATA2 gene is critical for the genesis and function of hematopoietic stem cells. Its deficiency can cause myelodysplastic syndromes, congenital lymphedema and severe viral infections. Our patient presented these three manifestations, added to a deletion in 20q12 that confirmed the suspicion of GATA2 mutation. A bone marrow transplant was suggested as definitive treatment. The corneal herpetic epithelial lesion was analogous to a regular hepetic keratitis with none stromal keratitis. Keywords: GATA2 deficiency, Herpetic keratitis, HIV, Myelodysplastic syndrome, 20q12 deletion, Foscarnet, Herpetic disseminated infection

Antimicrobial resistance (AMR) is one of the most important threats of the 21 st century. Wild birds have been described as reservoirs of AMR in different bacterial species, such as Salmonella spp. Privation of food, climate change and overpopulation have forced many wild species to modify their feeding habits, attending urban areas. In this context, the aim of this study was to study Salmonella presence, as well as related AMR in urban birds that inhabit the city and its surroundings. A total of 300 urban birds were sampled for Salmonella detection according to the ISO 6579-1:2017 (Annex D) recommendations, and serotyping was carried out according to the White-Kauffman-Le Minor scheme. Antimicrobial susceptibility was tested following 2013/652/EU Decision guides. Wild birds analysed were positive for Salmonella in 12.3 % of cases, with white storks fed in landfills as the most Salmonella prevalent species (p 0.05). The most common serovars isolated were zoonotic (S. Enteritidis, S. Typhimurium and S. Typhimurium monophasic variant). From Salmonella isolated strains, 40.5 % were resistant to the most prevalent AMRs found in urban birds were ciprofloxacin (36.4 %), nalidixic acid (36.4 %) and colistin (27.3 %). The scientific community, public administration and population in general should work together to control antimicrobial administration and drug waste management in order to decrease the development and spread of AMR.

ResumenIntroducciónLa histiocitosis de células de Langerhans es una causa infrecuente de tumor orbitario. El diagnóstico definitivo es anatomopatológico.Presentación del casoVarón 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.DiscusiónEn 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.ConclusionesEl 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.AbstractIntroductionLangerhans cell histiocytosis is a rare cause of orbital tumor. The final diagnosis is histopathologic.Case reportSixteen year-old male with right superior eyelid swelling and ptosis. Imaging tests showed an orbital mass that produced bone erosion. Systemic examination was negative. Surgical excision of the tumor was performed. The histopathologic examination resulted in Langerhans cell histiocytosis. After 17 years of follow-up, there have not been signs of recurrence.DiscussionIf lesions suggestive of Langerhans cell histiocytosis are found, staging of the disease is important to plan the treatment, which ranges from surgery to chemotherapy.ConclusionsThe course of the disease is unpredictable. Because of that, it is very important to determinate the disease's stage: unisystem (unifocal or multifocal) or multisystem. Since late recurrences may occur, patient's follow-up is necessary.

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.

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.

Objectives To evaluate the analgesic effect as well as patient cooperation and satisfaction with sub-Tenon's anesthesia in glaucoma surgery. Materials and methods Prospective uncontrolled study. Fifty-three patients who underwent glaucoma surgery, with or without cataract extraction, with sub-Tenon's anesthesia have been studied. Satisfaction and cooperation were evaluated on a qualitative scale. The pain level was quantified using a numerical scale from 1 (no pain) to 10 (unsupportable pain). Results The respective mean pain score at the different periods was: 2.13 (SD 1.47) on administering anesthesia, 1.74 (SD 1.27) during the surgery, 1.63 SD (1.23) immediately after the procedure, 1.38 (SD 0.38) 30 min later, and 1.38 (SD 0.63) when leaving the recovery unit. The postoperative satisfaction was “fairly or very satisfied” in 92.6% of the cases for both the surgeons and the patients. Twenty-four patients complained during the surgery, 15 of them at the conjunctival suture; in these cases additional anesthesia was administered, and, in 4 cases, intravenous paracetamol was given. Pain scores were significatively higher during the surgery ( p = 0.033), and immediately after the procedure ( p = 0.027) in trabeculectomy than in deep sclerectomy patients. Conclusions The good analgesic level reached led to a high level of patient and surgeon satisfaction, even in longer procedures such as deep sclerectomy combined with cataract extraction, or implantation of drainage devices. Pain scores were significatively higher in trabeculectomy than in deep sclerectomy patients.

Background This study was designed to objectively evaluate visual function and the causal relationship between sleep apnea and optic nerve dysfunction in patients with obstructive sleep apnea syndrome (OSAS) with or without diagnosis of normal-tension glaucoma (NTG) using multifocal visual evoked potentials (mfVEP).

To report a case of late diagnosis of a classical familial homocystinuria based on an ophthalmologic examination. A 35-year-old male with Marfan-like phenotype complained of a progressive increase of myopia during the previous 2 years. Ophthalmologic exploration showed a bilateral subluxation of the lens with inferior and nasal displacement. Biochemical study detected a profile of increased amino acid levels (homocysteinemia) consistent with suspected homocystinuria. Vascular and skeletal studies ruled out Marfan syndrome. Response to treatment demonstrated B(6)-non-responsive homocystinuria. Molecular study showed compound heterozygous T353 N and D444 N mutations of the cystathionine beta-synthase (CBS) gene, and also a C667T homozygous mutation of the methylenetetrahydrofolate-reductase (MTHFR) gene. Family study showed classical homocystinuria in his father and sister, although they did not present any systemic or ocular features of the disease. Homocystinuria is a metabolic disease usually presenting at an early age as vascular, skeletal and neuropsychiatric abnormalities, as well as ectopia lentis. Our case is atypical because of the absence of thromboembolism and the mild phenotype, in spite of being B(6)-non-responsive, and the association of a rare compound heterozygous mutation of the CBS gene and also an homozygous mutation of the MTHFR gene. It is necessary to rule out homocystinuria in patients with ectopia lentis, even in the absence of systemic symptoms.

Case report A 27-year-old female presented to us with a complaint of proptosis of her right eye. Imaging tests showed an extraconal mass in the medial orbital wall, which affected the medial rectus. The tumor was removed and adjuvant radiotherapy was required. The histological examination showed an undifferentiated high-grade pleomorphic sarcoma of the orbit. There has not been recurrence after 6 years of follow-up. Discussion Undifferentiated high-grade pleomorphic sarcoma is an uncommon cause of malignant orbital mass. It grows fast and can metastasize, so extension studies are required. Surgical excision is the treatment of choice.

The most frequent ophthalmic lesions associated to neurofibromatosis type 1 include iris Lisch nodules, optic nerve gliomas, and neurofibromas located on the eyelid, conjunctiva or orbit. Glaucoma is much less frequent, and it may be difficult to diagnose and treat. We present two patients with neurofibromatosis type 1 and associated congenital glaucoma. In case 1, the glaucoma was present at birth, and was the first symptom of the disease. It was surgically treated by means of an Ahmed glaucoma valve implantation, with good intraocular pressure (IOP) control after 13 months of follow-up. In case 2, the glaucoma appeared at 5 months old, and a trabeculectomy was initially performed; it failed 2 years later and an Ahmed glaucoma valve was then implanted, with adequate IOP control after 7 years of follow-up. Both children were males, and in both cases there were orbital neurofibromas and a dysplasia of the greater wing of the sphenoid of the same side. In case 2, orbital enlargement surgery was also performed at 4 years old. Congenital glaucoma management in the context of neurofibromatosis is very complex due to the frequent association of orbital and eyelid tumors and bone dysplasia, and its prognosis is usually poor. We present two cases in which good control of IOP has been achieved with Ahmed glaucoma valve implantation after 13 months and 7 years of follow-up.

Background Actinic keratosis (AK) is an intraepidermal malignancy precursor form of cutaneous squamous cell carcinoma (SCC), which generally occurs in fair-skinned individuals with long-term sun exposure. We present a case series of AKs on the eyelid. Methods This was a retrospective study. Data of patients seen from 1994 to 2005 were used in the analyses. Results There were 21 cases of eyelid AKs in 18 patients, 11 men, and seven women, with a mean age of 70 (range 43–80) years. Most tumors were located on the upper eyelid. The mean size was 0.8 ± 0.4 cm. The most frequent AK type was type I (76.2%). Men had bigger lesions and more frequent free-margin eyelid involvement. Conclusions In our series, tumors were more frequent and had worse clinical and histopathological features in men. AKs are closely related to SCC and need to be removed before they turn into SCC. They can be safely and effectively removed, and therapy is therefore warranted.

Aim To report an unusual case of cutaneous presentation on the eyelid of systemic (or nodal), CD30+, anaplastic large-cell lymphoma (ALCL). Methods A 39-year-old man presented with a rapidly growing exophytic mass on the left upper eyelid, with a protuberant, ulcerated aspect and with discharge. The patient showed lymph node involvement 3 months after the appearance of the lesion on the eyelid (the lesion itself appeared 1 week before examination). Results The histopathologic and immunohistochemical diagnosis was ALCL, T-cell phenotype, strongly positive for CD43 and CD30, and negative for CD3, anaplastic lymphoma kinase (ALK), and B-cell antigens. Treatment was by radiotherapy and, later, chemotherapy (cyclophosphamide, adriamycin, vincristine, and prednisolone, CHOP) for skin recurrences and lymphadenopathies over 5 years. There has been no recurrence for more than 6 years. Conclusions Primary, systemic, CD30+, ALK-negative, ALCL presentations generally have a poor prognosis and tend to occur in older individuals, although the clinical outcome is highly variable and difficult to predict in individual cases. Only three cases of ALCL have been described in the ocular adnexae and none was ALK-negative.

Background: Basal cell carcinoma (BCC) is a common malignant skin neoplasm. The surface of the caruncle contains sebaceous glands, hair follicles, and lacrimal and sweat gland elements. Consequently, the caruncle may spawn any neoplasm that occurs in the conjunctiva, skin, or lacrimal gland. We report a patient with a primary BCC located on the lacrimal caruncle. Methods: An 80-year-old man with a 5-month history of a gradually enlarging left caruncular neoplasm was seen. The lesion was nodular and irregularly brown-black colored, with no involvement of adjacent conjunctiva or skin. He had a history of sun exposure, but no personal or family history of other malignant neoplasms. Results: The mass was excised completely. Histopathologic examination revealed a BCC originating in the basal layer of the conjunctival epithelium. No tumor recurrence has been noted after 7 years of follow-up. Primary BCC of the caruncle is unusual, and only four cases have been described in the PubMed. BCC should be considered in the differential diagnosis of caruncle and adjacent skin lesions.

Case report A 15-year-old male, with no previous traumatic o surgical ocular injury, presented with a right eye proptosis and inferior displacement which began 4 months earlier. Ultrasound and CT showed a cystic lesion of 3.5 cm × 2 cm in the superior orbit. Surgical resection was performed by transconjunctival orbitotomy. The histopathology examination showed a cyst with nonkeratinized epithelium, and without goblet cells. After 10 years of follow-up, there have been no new lesions. Discussion Conjunctival primary orbital cysts are very uncommon. They are usually of small-moderate size; giant cysts are exceptional. Diagnosis by imaging is essential to establish the differential diagnosis and surgical treatment.

Background: Cutaneous horn (cornu cutaneum) is a morphological designation for a protuberant mass of keratin that resembles the horn of an animal. It results from unusual cohesiveness of keratinized material from the superficial layers of the skin or implanted deeply in the cutis. This lesion may be associated with a benign, premalignant, or malignant lesion at the base, masking numerous conditions. Methods: A retrospective analysis of 48 cases of cutaneous horns of the eyelid treated between 1992 and 2002 has been performed. Results: Twenty-four men and 19 women, with a mean age of 62 years (range 16–90), were treated by surgery. Histologically, 77.1% were associated with benign specimens at the base pathology, 14.6% were premalignant, and finally, 8.3% were caused by malignant skin tumors. The most common lesion was seborrheic keratosis among the benign lesions, actinic keratosis among the premalignant ones, and basal cell carcinoma and squamous cell carcinoma among the malignant ones. Conclusion: Cutaneous horns usually appear on exposed skin areas in elderly men. The important issue in this condition is not the horn itself, which is just dead keratin, but rather the nature of the underlying disease, although the horns are usually benign.

Purpose To report a case of congenital phakomatous choristoma (PC) of the eyelid, a rare tumor of lenticular anlage in the subcutaneous tissue and dermis. Case A boy had a mass in the right lower eyelid near the inner canthus at birth. At age 10 months, the tumor was excised. Results The tumor cells showed intense immunoreactivity positive for S-100 protein, vimentin, and periodic acid-Schiff. Keratin markers, epithelial membrane antigen, glial fibrillary acidic protein, muscle specific actin, and epithelial membrane antigen were negative. The histopathologic features were consistent with those of a PC. Conclusions This histopathologic and immunoreactivity study supports the proposal that PC is a rare entity of lenticular anlage origin. Only 18 such cases have been described previously.

The authors introduce a 42-year-old woman with a choroidal osteoma. After 10 years the patient presented a second choroidal osteoma in the same eye; this osteoma has been growing in the last years. New tests that were unavailable during the first diagnosis were performed such as Fundus Autofluorescence or Enhanced Depth Imaging-Optical Coherence Tomography (EDI-OCT). These tests show characteristics of the tumors and allow us to realize a visual prognosis for the patient.

The incidence of ocular lesions in midfacial fractures ranges from 4 to 67%, depending on the criteria used in the previous examination. We report a retrospective study of 219 patients (233 fractures) who were admitted to our hospital between January 1990 and June 1994. The fractures are classified according to Henderson's classification (X-ray and Computed Tomography). We present the results of the ophthalmologic evaluation, after excluding palpebral lesions, and discuss the correlation with the etiology and the kind of fracture. The ocular lesions are divided into extraocular and intraocular ones. The extraocular lesions that we found are the following: damage to the lacrimal system, cranial nerve damage, displacement of the eye, corneal erosion and corneal foreign body and conjunctival lesions (subconjunctival hemorrhages, lacerations and chemosis). The intraocular lesions studied are divided into lesions of the anterior structures (hyphema, traumatic cataract, lens dislocation and hernia of the iris) and lesions of the posterior structures (vitreous and macular hemorrhage, eyeball perforation, optic nerve damage and Berlin's edema). Our conclusion is that comminuted fractures, fractures of the orbital rim only and those of the orbital floor are the most frequently associated with ocular damage, being mainly caused by automobile accidents and assaults.

Obstructive sleep apnea–hypopnea syndrome (OSAHS) is becoming widely accepted as a risk factor for glaucoma. We discuss the proposed mechanism involved in the pathogenesis of glaucoma in OSAHS, and review the published data on the association between these two conditions, as well as papers regarding functional and structural tests related with glaucomatous damage. There is increasing evidence that the prevalence of glaucoma is higher in OSAHS patients, especially in those with severe disease with apnea-hypopnea index (AHI) >30, and also that sleep disorders may be more frequent in patients with glaucoma, especially in those with normal tension glaucoma (NTG). Several ophthalmic signs and symptoms have been associated with this condition. Raised intraocular pressure (IOP), possibly related to increased body mass index, thinning of retinal nerve fiber layer (RNFL), and alteration of visual field (VF) indices has been demonstrated in many studies, in patients with no history of glaucoma or evidence of glaucomatous changes in the ophthalmic examination. A correlation of AHI with RNFL and VF indices has been described in some studies. Finally, corneal thinning, suspicious glaucomatous disc changes and anomalies in electrophysiological tests such as multifocal visual evoked potential have been described in patients with OSAHS, even in patients with normal findings in the optic nerve and VF, suggesting subclinical optic nerve involvement not detectable in conventional ophthalmic examinations. The pathogenesis of optic nerve involvement has been related to vascular and mechanical factors. Vascular factors include recurrent hypoxia with increased vascular resistance, autonomic deregulation, oxidative stress and inflammation linked to hypoxia and subsequent reperfusion, decreased cerebral perfusion pressure and direct hypoxic damage to the optic nerve. Proposed mechanical factors include increased IOP at night related to supine position and obesity, raised intracranial pressure and elastic fiber depletion in the lamina cribosa and/or trabeculum. In conclusion, ophthalmic evaluation should be recommended in patients with severe OSAHS, and the presence of sleep disorders should be investigated in patients with glaucoma, especially in NTG patients and in those with progressive damage despite controlled IOP, as treatment with continuous positive airway pressure may contribute to stabilizing the progression of glaucomatous damage.

Orbital involvement in primary amyloidosis is uncommon. A 71-year old man with primary amyloidosis presented with painless proptosis, a palpable orbital mass, and restricted extraocular motility. Computed tomography, magnetic resonance imaging, and histopathologic analysis documented the mass. The patient had no clinical evidence of intraocular amyloid deposits, family history of amyloidosis, or systemic involvement. However, 7 years later intracranial involvement and secondary glaucoma became apparent.

Caso clínico: Varón de 62 años de edad con trasplante renal y tratamiento inmunosupresor que acude por disminución de la visión (20/100) en ojo izquierdo, edema corneal y presión intraocular de 46mmHg. Un mes después aparece queratitis dendrítica marginal inferior. El raspado corneal y la proteína C reactiva demuestran la presencia de un virus del herpes simple (HVS). Discusión: La epiteliopatía corneal autoinmunitaria o endotelitis idiopática corneal se caracteriza por edema corneal y precipitados queráticos. Se cree que el HVS podría ser secretado desde el trabeculum, inervado por el nervio trigémino, apoyado clínicamente por la progresión del edema estromal desde la periferia. Case report: A 62-year-old man with previous renal transplant and immunosuppressive treatment presented with decreased visual acuity (20/100) in his left eye, corneal oedema and intraocular pressure of 46mmHg. One month later an inferior marginal dendritic keratitis appeared. Corneal scraping and real-time polymerase chain reaction showed herpes simplex virus (HSV). Discussion: The autoimmune corneal endotheliopathy or acute idiopathic corneal endotheliitis is characterised by corneal stromal oedema and keratic precipitates. HSV might be secreted from the trabeculum, innervated by the trigeminal nerve. This hypothesis is supported by the clinical observation that the corneal stromal oedema usually starts from the periphery.

To report a unusual case of an isolated traumatic stellated tarso-conjunctival laceration located in the upper left eyelid without eyelid margin involvement and with normal ocular examination. A 19 year-old male wounded by a bokken (wooden katana) while practicing a sham duel had an isolated eyelid tarso-conjunctival laceration, without any other eyelid layer damage, neither skin nor muscle. Treatment was conservative without suture, by means of compressive occlusion fixing the pieces of broken tarsus in the correct position. The aim of the treatment was to avoid any wrong eyelid position secondary to healing. The eyelid maintained normal structure and movement in the follow-up at 8 months. Tarsal plate rupture is usually combined with other eyelid layer damage, which usually requires suture by layers. If eyelid skin and muscle are intact, we may choose conservative management.

Propósito: Evaluar los resultados y complicaciones a largo plazo de los dispositivos de drenaje para glaucoma (DDG) en pacientes pediátricos (0-15 años). Material y métodos: Estudio retrospectivo de cohortes de 17 DDG implantadas desde julio de 1994 hasta abril del año 2007 en 14 pacientes (17 ojos). En dos pacientes (3 ojos) se implantó un DDG de Molteno (DDGM) y en 12 pacientes (14 ojos) un DDG de Ahmed (DDGA). Se han recogido datos demográficos y relacionados con la afección glaucomatosa de cada paciente. Se ha estudiado el porcentaje de éxito de la cirugía, así como el tiempo durante el cual se ha mantenido controlada la tensión desde el implante del dispositivo y las complicaciones derivadas de este. Resultados: De los 14 pacientes, nueve (64,28%) presentaban glaucoma congénito, y cinco (35,71%) glaucoma afáquico secundario a cirugía de catarata. La media de la presión intraocular (PIO) preoperatoria fue de 29,82mmHg (DS: 6,98). La media de la PIO posquirúrgica fue de 14,05mmHg (DS: 7,57). La media del seguimiento ha sido de 3,14 años (3 meses-8,3 años). Se ha considerado como éxito mantener, en las dos últimas revisiones, la PIO igual o por debajo de 21mmHg sin signos de progresión de glaucoma con o sin uso de fármacos hipotensores tópicos, sin necesidad de cirugías de glaucoma adicionales y sin la aparición de complicaciones devastadoras para la visión. La probabilidad acumulada de éxito según el análisis de Kaplan Meier ha sido de 76, 63 y 55% a los 6 meses, 1-3 años y 4-8 años respectivamente. El 41,17% de los DDG fracasaron (7/17). Conclusiones: Los DDG son una buena opción quirúrgica para los glaucomas refractarios pediátricos cuando otros procedimientos quirúrgicos han fracasado o tienen un mal pronóstico. Purpose: To evaluate the results and long-term complications of glaucoma drainage devices (GDD) in paediatric patients (0-15 years). Methods: Retrospective cohort study was conducted on 17 implanted glaucoma drainage devices from July 1994 to April 2007 in 14 patients (17 eyes). In two patients (3 eyes) a Molteno GDD (MGDD) was implanted, and in 12 patients (14 eyes) an Ahmed GDD (AGDD) was used. We studied the demographic and glaucoma related patient data, as well as the probability of surgical success. The time which intraocular pressure (IOP) was controlled and the postoperative complications were also studied. Results: Of the fourteen patients, 9 (64.28%) showed congenital glaucoma, and 5 (35.71%) aphakic glaucoma. The pre-aqueous drainage device median IOP was 29.82mmHg (SD: 6.98), and 14.05mmHg (SD: 7.57) postoperative. The median follow-up was 3.14 years (3 months-8.3 years). Success of aqueous drainage device was defined as an IOP less than 21mmHg with or without medication on the last two follow-up visits, and without severe complications or further glaucoma surgery. Using a Kaplan Meier analysis there was success in 76%, 63% and 55% at the six months, 1-3 years and 4-8 years respectively. The GDD was a failure in 41.17%. Conclusions: GDDs are a good surgery option for refractory paediatric glaucoma when other surgery procedures have failed or have bad prognosti.

Purpose To report a case of Propionibacterium acnes endophthalmitis in a patient with an Ahmed glaucoma valve. Case report A nine-year-old boy with bilateral congenital glaucoma, with an Ahmed glaucoma valve implanted in the left eye, had recurrent conjunctival dehiscence and endophthalmitis. Results Vitreous cultures demonstrated the presence of Propionibacterium acnes. Conclusions This is the first reported case of Propionibacterium acnes endophthalmitis in an Ahmed glaucoma valve and the second one in a glaucoma drainage device. We strongly recommend using a patch graft to prevent and treat tube exposure. Conjunctival grafts may be useful to close the conjunctiva when there is marked scarring to prevent patch exposure and melting or extrusion.

PURPOSE: To report a case of primary cutaneous adenoid cystic carcinoma involving the eyelid. METHODS: We examined a 70-year-old woman with an 18-month history of a gradually enlarging, blue-colored mass in the medial third of the right upper eyelid. Excisional biopsy and additional resection of the medially infiltrated margin resulted in tumor-free edges. Superior cervical and orbital evaluations were performed. RESULTS: Histopathologic evaluation revealed a primary cutaneous adenoid cystic carcinoma. No gland pathology was documented. No tumor recurrence has been noted. CONCLUSION: Primary cutaneous adenoid cystic carcinoma, an uncommon tumor of the skin, should be added to the differential diagnosis of eyelid tumors.

We report two new cases of chondroid syringoma (CS) of the eyelid. Until 1961, this entity was known as pleomorphic adenoma or benign mixed tumour of the skin of salivary glands type. This tumour occurs most commonly in the head and neck regions.We describe two cases of CS with rapid growth, in the upper right eyelid, with no relation with the palpebral lobe of the lacrimal gland. RESULTS. Treatment consists of wide local surgical excision with its capsule, in its entirety.These tumours of the ocular adnexa are exceedingly rare. Malignant transformation is possible.

Objetivo: Evaluar los resultados en cuanto a control de presión intraocular (PIO) y agudeza visual (AV) tras cirugía de implante de drenaje para glaucoma (DDG) combinada con vitrectomía pars plana (VPP) simultánea. Material y métodos: Revisión retrospectiva de 8 ojos (7 pacientes). El diagnóstico era glaucoma neovascular (GNV) secundario a retinopatía diabética proliferativa en 4 ojos, en los que se colocó un implante de Molteno de doble plato, y glaucoma secundario (GS) a cirugía de catarata complicada en 3 ojos y a traumatismo penetrante en otro, en los que se usó una válvula de Ahmed. Resultados: La PIO media pasó de 35,77 mmHg (20-50) preoperatoria a 11,5 mmHg (2-20) postoperatoria, y el número de fármacos hipotensores medio de 2,33 (0-3) preoperatorio a 0,62 (0-3) postoperatorio. En los GNV, la AV preoperatoria era percepción de luz o contar dedos, y se perdió en 3 ojos. En los GS era inferior a 0,1, aumentando hasta una AV media de 0,42 (0,1-0,8) a los 3 meses. El seguimiento medio fue de 10,12 meses (5-27). Las complicaciones fueron obstrucción del tubo por fibrina en 2 casos, uno de los cuales (GNV) presentó una hemorragia supracoroidea tras la desobstrucción y evolución a ptisis bulbi, extrusión del reservorio en un caso, y descompensación corneal tardía en un caso. Conclusiones: En los GS los resultados han sido buenos, con mejoría de la AV y PIO controlada. En los GNV se consiguió controlar la PIO y evitar dolor, pero los resultados visuales fueron malos por la enfermedad de base. Purpose: To evaluate intraocular pressure (IOP) and visual acuity (VA) results after glaucoma drainage device (GDD) implantation combined with simultaneous pars plana vitrectomy (PPV). Material and methods: Retrospective review of 8 eyes (7 patients). The diagnosis was neovascular glaucoma (NVG) secondary to proliferative diabetic retinopathy in 4 eyes, in a which a double plate Molteno implant was placed, and glaucoma secondary (GS) to complicated cataract surgery in 3 eyes and penetrating trauma in one eye, in which a glaucoma Ahmed valve was implanted. Results: Mean preoperative IOP was 35.77 mmHg (20-50) and 11.5 mmHg (2-20) postoperatively, and mean number hypotensive drugs was decreased from 2.33 (0-3) before surgery to 0.62 (0-3) after it. Preoperative VA in NVG was light perception or counting fingers, and it was lost in three eyes. In GS, VA before surgery was below 0.1 in all eyes, and three months after surgery mean VA was 0.42 (0.1-0.8). Mean follow-up was 10.12 months (5-27). The complications were tube obstruction in two cases, one of these presenting also a suprachoroidal hemorrage and ptisis bulbi, plate exposure in one case, and bullous keratopathy as a late complication in another eye. Conclusions: The VA and IOP were well controlled with this procedure in GS. In NVG, IOP was controlled, but visual results were poor due to complications and the underlying pathology.

espanolObjetivos: Analizar los resultados a medio plazo de los dispositivos de drenaje para glaucoma (DDG) en cuanto a control de la presion intraocular (PIO), del glaucoma y del mantenimiento de la vision preoperatoria. Metodos: Estudio de cohortes retrospectivo de 86 ojos de 77 pacientes en los que se ha implantado un DDG. Se ha empleado estadistica descriptiva y analisis de supervivencia. Resultados: Se han considerado como exito 53 ojos (61,6%), completo (sin tratamiento) en 34 ojos (39,5%); y relativo (con tratamiento) en 19 ojos (22,1%). Las causas de fracaso han sido: mal control de la PIO en 13 ojos - (15,1%), y complicaciones en 20 ojos (23,2%) con reduccion severa o perdida de vision (extrusion del reservorio, hemorragia supracoroidea, desprendimiento de retina). Se ha obtenido control de la PIO en 66 ojos (76,7%), en 47 de ellos sin tratamiento (54,6%), aunque 13 de estos han sido fracasos por complicaciones. A pesar del control de la PIO, en siete ojos (8,1%) se ha observado progresion del glaucoma. En cuanto a la vision, en 46 ojos (53,5%) se ha mantenido estable y en 40 ha disminuido 3 o mas lineas (46,5%), siendo la causa mas frecuente la descompensacion corneal. En 21 ojos (24,4%) se ha perdido la percepcion de la luz, y cuatro ojos (4,6%) han sido eviscerados. Discusion: Los DDG son una opcion quirurgica eficaz para el control de la PIO en ojos con mal pronostico para la cirugia convencional de glaucoma, pero presentan complicaciones graves y deterioro visual en un porcentaje elevado de casos. EnglishObjective: To study the intermediate-term results of glaucoma drainage devices (DDG) with respect to control of intraocular pressure (IOP), control of glaucoma, and maintenance of pre-operative visual acuity. Methods: This was a retrospective cohort study of 86 eyes in 77 patients in whom a DDG was implanted, using descriptive statistics and survival analysis. Results: Success was achieved in 53 eyes (61.6%), complete (without treatment) in 34 eyes (39.5%) and qualified (needing treatment) in 19 eyes (22.1%). In the 33 eyes where the DDG treatment was unsuccessful, poor IOP control occurred in 13 eyes - (15.1%), and complications occurred in 20 eyes (23.2%) resulting in a severe reduction or loss of visual acuity (plate exposure, suprachoroidal hemorrhage, retinal detachment). IOP control was obtained in 66 eyes (76.7%), 47 of them without treatment (54.6%), although on 13 occasions the overall treatment failed due to complications occurring. Despite IOP control, glaucoma progression occurred in 7 eyes (8.1%). Preoperative vision was maintained in 46 eyes (53.5%), but decreased by 3 or more lines in 20 eyes (46.5%); bullous kerathopathy was the most frequent cause of the worsening. Loss of light perception occurred in 21 eyes (24.4%) and 4 eyes (4.6%) were eviscerated. Conclusions: DDG are an effective surgical option for control of IOP when conventional surgery has a poor prognosis, but they are associated with an increased risk of serious complications and loss of visual acuity in a significant proportion of cases.

Summary: Alveolar rhabdomyosarcoma (RMS) has a predilection for the deep soft tissues of the extremities and mainly occurs in children. Although the tumor may originate in other sites, such as the nasal cavity or paranasal sinus, invasion of the orbit is unusual. We describe the clinicopathological features of 2 cases of alveolar RMS of the nasal cavity or paranasal sinus in adult patients with orbital extension. These cases of alveolar RMS of the nasal cavity or paranasal sinuses are described in 2 men, both in the third decade of life. These patients were evaluated with radiological studies. The histological diagnosis was confirmed by immunohistochemical methods. Treatment consisted in a combination of chemotherapy and radiation therapy following excisional biopsy. Alveolar subtype RMS is an extremely aggressive neoplasm that rarely presents in the orbit or paranasal sinuses of adults but should be considered in the differential diagnosis of tumors with this localization. Myoglobin, Myo D1, and myogenin seem to be the most specific markers for RMS.

To report an uncommon case of unilateral subperiosteal hemorrhage while scuba diving involving the orbit, a condition characterized by proptosis and associated severe ocular motility disturbances with displacement of the eyeball.Observational case report.Unilateral subperiosteal hemorrhage in a 31-year-old woman while scuba diving at a depth of 20 meters. This was documented by clinical and radiographic examination. Computed tomography (CT) scan demonstrated a subperiosteal hemorrhage as a self-limited mass protruding into the left orbit. The process resolved without treatment and without visual or motility sequelae. A CT-scan, nuclear magnetic resonance, and conventional angiography did not show any venous abnormalities in the brain.During scuba diving at a depth of 20 meters, the pressure is three atmospheres, whereas within the diving mask the pressure is one atmosphere if it is not equilibrated; thus, a negative pressure is created within the mask. Small vessels can be broken in the conjunctiva or subperiosteal space by this force. It is important to exclude vascular abnormalities, especially if there is a positive family history.

espanolCaso clinico: Paciente varon de 36 anos de edad con historia de abuso de alcohol y con perdida brusca de vision. La exploracion oftalmologica mostro una retinopatia tipo Purstcher y permitio, tras los estudios de laboratorio y de imagen complementarios, el diagnostico de una pancretitis aguda incipiente. Discusion: La presentacion de una pancreatitis aguda con perdida brusca de vision y retinopatia tipo Purstcher como sintoma de presentacion, previos al cuadro abdominal, es excepcional. Sin embargo, se debe tener en cuenta esta posibilidad, a fin de efectuar un diagnostico y tratamiento precoces. EnglishCase report: A 36-year-old man with a history of alcohol abuse presented with sudden blindness. The ophthalmologic examination showed Purtscher-like retinopathy. The presumed diagnosis was acute pancreatitis, which was confirmed by complementary laboratory studies. Discussion: Sudden acute visual loss with Purstcher-like retinopathy may be present in acute pancreatitis, although it is a very rare as a presenting symptom. Early diagnosis based on ophthalmic symptoms may help in the recognition and treatment of the disease and prevent later complications.

espanolCaso clinico: Presentamos un paciente varon de 56 anos de edad con un exoftalmos maligno en el contexto de una enfermedad de Graves, que sufrio un claro empeoramiento coincidiendo con su entrada en crisis hipotiroidea, con una evolucion torpida, que requirio de distintas medidas terapeuticas para la reduccion de la proptosis. Discusion: En el manejo de este tipo de pacientes hay que tener en cuenta si cumplen o no criterios de gravedad y/o actividad para aplicar las distintas opciones terapeuticas en el momento preciso. EnglishCase report: We present the case of a 56-year-old male with a malignant exophthalmos in the context of Graves’ disease. He suffered a significant worsening coincident with a hypothyroid crisis, and various therapeutic approaches were required for reduction of the proptosis. Discussion: When managing this condition, both the severity of the exophthalmos and the activity of the thyroid must be considered when choosing which of the different therapeutic approaches should be employed.

espanolObjetivo: Analizar los resultados de la cistitomia de la ampolla de filtracion en ojos operados de cirugia filtrante de glaucoma seis meses antes o mas. Material y metodos: Estudio retrospectivo de 23 ojos de 21 pacientes en los que se ha realizado revision de la ampolla de filtracion con una aguja de 25 G, entre 6 meses y 23 anos tras cirugia filtrante (media 67,3 meses). Se realizaron una media de 1,2 cistitomias por ojo y 3,9 inyecciones de 5-fluorouracilo subconjuntival. Resultados: De una presion intraocular (PIO) media preoperatoria de 26 mmHg y 1,3 farmacos hipotensores hemos pasado a una PIO media postoperatoria de 16,3 mmHg y una media de 0,7 farmacos, con una ampolla de filtracion difusa en 12 casos (54,5%). Los resultados fueron peores en ojos con cirugia previa de glaucoma u otra cistitomia previa fracasada (75% fracaso). Conclusiones: La revision con aguja es una tecnica sencilla y segura que ofrece buenos resultados incluso anos despues de una cirugia filtrante, y debe ser considerada de manera previa a la reinstauracion de tratamiento medico o quirurgico en los pacientes operados de glaucoma con fracaso tardio de una ampolla de filtracion previamente funcionante y esclerectomia abierta en la gonioscopia. EnglishPurpose: To evaluate the results of bleb needling in eyes subjected to filtering surgery six months or more previously. Methods: A retrospective study of 23 eyes in 21 patients subjected to bleb needling performed by means of a 25G needle, 6 months to 23 years after failed filtering surgery (mean 67.3 months). The mean number of bleb needlings was 1.2 and the mean number of 5-fluorouracil subconjunctival injections was 3.9. Results: The mean intraocular pressure (IOP) before needling was 26 mmHg and the mean anti-glaucoma medications was 1.3 per eye. The mean postoperative IOP was 16.3 mmHg and mean anti-glaucoma medications 0.7. A diffuse filtering bleb was evident in 12 eyes (54.5%). The results of needling were worse in eyes subjected to previous surgery for glaucoma or other failed bleb needling procedures (75% failure). Conclusion: Needle revision of the filtering bleb is a simple and safe technique that offers good results even years after a filtering surgery had been performed. It should be considered before other medical or surgical treatments in patients with a previously functioning bleb and a permeable sclerectomy site at gonioscopy.