Agreement between Five Experts and the Laguna ONhE Automatic Colourimetric Application Interpreting the Glaucomatous Aspect of the Optic Nerve.

BACKGROUND: Optic nerve head (ONH) interpretation is a glaucoma screening method which may be influenced by criteria variability. Laguna ONhE software is a low-cost and non-invasive method of ONH analysis. METHODS: We analysed the results of the Laguna ONhE application, interpreting 552 ONH images from the ACRIMA database, publicly available on the Internet, and compared them with the opinion of five experts. Diagnostic agreement was investigated using Cohen's kappa (κ) with 95% confidence. RESULTS: The kappa concordance index obtained with Laguna ONhE and the majority of the experts' criterion (0.77) was significantly higher compared to that obtained with ACRIMA and the majority of the experts' criterion (0.61). In 44.7% of the cases there was absolute agreement among the 5 experts and the Laguna ONhE program. Removing borderline cases from the analysis yielded increased diagnostic agreement (0.81). The area under the receiver operating characteristic (AUROC) of the Laguna ONhE program (0.953, p < 0.001) was not significantly different than AUROC of the majority of the experts' criterion (0.925, p < 0.001), p = 0.052. Individually obtained expert's AUROCs were significantly lower (0.636 to 0.913; p < 0.01). CONCLUSIONS: Laguna ONhE's agreement with the experts is high, particularly where the diagnosis may be more obvious by the appearance of the ONH.

Vitreociliary Block in a Patient With Uveitis and Previous Laser Posterior Capsulotomy.

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.

Orbital-Conjunctival-Eyelid Hemorrhage-Hematoma due to Inappropriate use of FFP2/NK95 Facial Mask in COVID-19.

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.

Eyelid Eccrine Poroma: A Case Report and Review of Literature.

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.

Occult Orbital Injury with Dagger Fragment with Resulting Pneumocephalus.

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.

Nasal Cavity or Alveolar Paranasal Sinus Rhabdomyosarcoma with Orbital Extension in Adults: 2 Cases.

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.

Obstructive sleep apnea-hypopnea syndrome (OSAHS) and glaucomatous optic neuropathy.

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.

Bimatoprost/timolol fixed combination versus latanoprost in treatment-naïve glaucoma patients at high risk of progression: a pilot study.

OBJECTIVE: To compare a fixed combination of 0.03% bimatoprost and 0.5% timolol (BTFC) with latanoprost monotherapy (LM) in treatment-naïve patients with open-angle glaucoma (OAG) and risk factors for glaucomatous progression. METHODS: Patients were enrolled at 15 sites in Spain and Portugal, and were randomized 1:1 to BTFC or LM. Patients instilled one drop of medication once per day at 8 pm for 12 weeks. The primary outcome was change in intraocular pressure (IOP) at 12 weeks. RESULTS: Of 81 patients enrolled, 43 were randomized to BTFC and 38 to LM. Mean (SD) change in IOP from baseline to 12 weeks was significantly greater for BTFC than for LM: -13.5 mmHg (4.48) versus -11.4 mmHg (3.19), respectively (P=0.003). Similarly, at 12 weeks, significantly more BTFC patients than LM patients had IOP reductions of ≥40% (74.4% versus 47.4%, P=0.015) or ≥50% (46.5% versus 15.8%, P=0.003). Adverse events were more frequent with BTFC than with LM (33 versus 13 events), but most were mild in severity. The only serious adverse event (colon cancer) was adjudged unrelated to the study medication. CONCLUSION: BTFC was effective and well tolerated in treatment-naïve patients with OAG at high risk of progression.

Isolated tarso-conjunctival superior eyelid traumatic laceration.

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.

Evaluation of the visual function in obstructive sleep apnea syndrome patients and normal-tension glaucoma by means of the multifocal visual evoked potentials.

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). METHODS: This observational, cross-sectional study assessed 20 patients with recently diagnosed OSAS with or without NTG. Diagnosis of sleep apnea was based on overnight polysomnography (ApnoeScreen). All participants underwent a complete physical and ophthalmologic examination. MfVEP recordings obtained using VERIS software (Electro-Diagnostic Imaging, San Mateo, USA), SITA-standard 30-2 automated perimetry (Humphrey Visual Field Analyzer II) and optic coherence tomography (Topcon 3D OCT-1000) exams were performed to evaluate the changes. RESULTS: Abnormal mfVEP amplitudes defects (interocular and monocular probability analysis) were found in 40% of the eyes in the non-glaucoma group and in 90% of eyes of the NTG patients. As well, delayed mfVEP latencies (interocular and monocular probability analysis) were seen in 30 and 60% of the eyes of the non-glaucoma and NTG groups, respectively. The average RNFL (retinal nerve fiber layer) thickness was significantly reduced in the NTG eyes compared to the control database and the non-glaucoma eyes. On the Humphrey Visual Field total deviation analysis, all the NTG eyes showed significant clusters of abnormal points but none was detected in the non-glaucoma group eyes. However, the mfVEP amplitude and latency did not show any significant correlation with the standard perimetry and OCT variables, because the mfVEP technique was able to detect far more early visual defects in these patients. Systolic blood pressure, sleep efficiency, arousal index, mean and minimum arterial oxygen saturation (SaO(2)), time SaO(2) < 90%, oxyhemoglobin desaturation index, number of central and mixed apneas and apnea-hipopneas index were shown to be significantly correlated with mfVEP amplitude and latency. CONCLUSIONS: A significant incidence of subclinical optic nerve involvement, not detected with other structural and psychophysics diagnostic techniques was seen by means of the mfVEP. In this sense, the mfVEP may be a useful diagnostic tool in the clinic for early diagnosis and monitoring of optic nerve function abnormalities in patients with OSAS.

Classical familial homocystinuria in an adult presenting as an isolated lens subluxation.

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.

Bilateral anterior ischemic optic neuropathy after bilateral neck dissection.

PURPOSE: 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.

Management of congenital glaucoma in neurofibromatosis type 1: a report of two cases.

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.

Clinicopathological study of 21 cases of eyelid actinic keratosis.

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.

Bilateral idiopathic orbital inflammation 3 years before systemic Wegener's granulomatosis in a 7-year-old girl.

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.

Schwannoma of the eyelid: report of two cases.

Schwannomas are rare benign neurogenic tumors that show differentiation of Schwann cells that form the neural sheath. Only five reports of eyelid schwannomas in adults have been found in the English literature. We report the unusual cases of two females, aged 41 and 70 years, who developed eyelid schwannomas. Neither tumor was diagnosed clinically; both were erroneously considered as epidermal inclusion cysts. The masses were surgically removed by excisional biopsy. The histopathological examination showed encapsulated tumors composed of interlacing bundles of spindle cells with slightly wavy nuclei. Immunocytochemistry for S-100 protein was strongly positive. The diagnosis of schwannoma was made. After 12 and 2 years of follow-up, no recurrences have been observed. This entity should be included in the differential diagnosis of eyelid tumors.

Cutaneous presentation on the eyelid of primary, systemic, CD30+, anaplastic lymphoma kinase (ALK)-negative, anaplastic large-cell lymphoma (ALCL).

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.

Orbital subperiosteal hemorrhage while scuba diving.

PURPOSE: 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. MATERIAL AND METHODS: Observational case report. RESULTS: 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. CONCLUSION: 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.