scholarly journals Spontaneous Thrombosis of an Orbital Arteriovenous Malformation Revealing Hereditary Haemorrhagic Telangiectasia (Rendu-Osler-Weber Disease)

2011 ◽  
Vol 17 (4) ◽  
pp. 466-471 ◽  
Author(s):  
C. Van Went ◽  
A. Ozanne ◽  
G. Saliou ◽  
G. Dethorey ◽  
I. De Monchy ◽  
...  

Hereditary Haemorrhagic Telangiectasia (HHT) is a genetic disorder responsible for cutaneous or mucosal telangiectasia and arteriovenous malformations (AVMs). The most frequent locations are lung and brain. In contrast, orbital AVMs are very rare. We describe a case of symptomatic orbital arteriovenous malformation due to spontaneous thrombosis. A 65-year-old woman was referred for chronic right eye proptosis associated with dilation of conjunctival vessels with a jellyfish pattern. Right visual acuity was 20/40 and intraocular pressure was 40 mmHg. Personal and familial history of recurrent epistaxis, associated with multiple telangiectasia within lips and palate, led to the diagnosis of HHT. Magnetic resonance imaging (MRI) completed with cerebral angiography found a giant and occluded AVM within the right orbit. Other AVMs were also found in brain and chest, confirming the diagnosis. Antiglaucomatous eyedrops were added to reduce intraocular pressure and a steroid therapy was begun. Two months later, visual acuity decreased in the right eye, due to a central retinal vein thrombosis. In conclusion, Most brain or pulmonary AVM can be treated by embolization. By contrast, this treatment in case of orbital location can lead to central retinal artery and/or central retinal vein occlusion, which may also appear as a spontaneous complication of the orbital AVM. Therapeutic management of orbital AVM is thus not standardized, and the balance between spontaneous and iatrogenic risk of visual loss has to be taken into account.

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110030
Author(s):  
Asfandyar Mufti ◽  
Muskaan Sachdeva ◽  
Khalad Maliyar ◽  
Marissa Joseph

Background: Hereditary haemorrhagic telangiectasia is an autosomal dominant genetic disorder characterized by abnormalities in blood vessel formation. The clinical manifestations of patients affected with hereditary haemorrhagic telangiectasia include mucocutaneous telangiectasias and visceral arteriovenous malformations. Case Summary: We report the case of a 30-year-old female diagnosed with hereditary haemorrhagic telangiectasia presenting with the classic triad of recurrent epistaxis, mucocutaneous telangiectasias and family history of hereditary haemorrhagic telangiectasia with activin receptor-like kinase 1 mutation. Upon skin examination, she was noted to have telangiectasias under left naris, inner lower lip and surface of the tongue, and a vascular malformation on the right forearm. Conclusion: Although the skin involvement and epistaxis may be mild symptoms and signs of hereditary haemorrhagic telangiectasia, timely recognition of these can ensure vigilant monitoring of potential severe complications from cerebral and pulmonary visceral arteriovenous malformations.


2021 ◽  
Vol 19 (6) ◽  
pp. 98-106
Author(s):  
Monawar Muhsin Jabr ◽  
Hussain S. Hasan ◽  
Hind Ahmed Mahdi

Background: Chronic kidney disease (CKD) is a public health problem over all the world. CKD may also be defined by the presence of kidney damage or a reduced glomerular filtration rate (GFR), which is the best overall indicator or index of kidney function. CKD patients are usually treated using kidney dialysis (hemodialysis) that uses a blood filtration mechanism (HD). Several metabolic parameters, such as blood urea, sodium, potassium, and glucose levels, can alter during HD. Osmotic alterations in blood, aqueous and vitreous humor, and other extracellular fluids arise from these fluctuations. That also can affect visual acuity, intraocular pressure (IOP), and retinal thickness. Aim of the Study: To evaluate some of the ocular findings undergoing HD to keep prevent the loss of patient vision such as visual acuity (VA), intraocular pressure (IOP), central corneal thickness (CCT), central Foveal Thickness (CFT), retinal nerve fiber layer (RNFL). Patient& Methods: This is a cohort (prospective) design study. This study including Seventy nine patients divided into two groups the first group from one week to six month (9 femal & 18 males) another group over than six month (36 female & 16 male) the average age between (12 to 70 years). This research performed in the three places department of the eye in Al-Hussein hospital in Samawah city, Al-Haboby hospital, Al-Hussein hospital in Dhi Qar city finally in Al-Shaheed Gazy hospital and Baghdad teaching hospital in Baghdad. Examining Visual Acuity by Snellen chart & auto refractometer, IOP& CCT by (CT.1 Computerized Tonometer TOPCON), RNLF and Central Foveal Thickness by OCT (Carl ZEISS, TOPCON). The inclusion criteria were as follows: all the patients undergoing dialysis from one week to over six months. Exclusion criteria were as follows: the patients have diabetic, any patients have a hereditary disease or glaucoma history or laser therapy, or intraocular injection in the eye before dialysis, the patients have a problem in the eye before dialysis such as cataracts or opacity leads to does surgery, the patients who have a refractive error or wear glass had been also excluded. Result: Includes the results of seventy-nine patients (45 females and 34 males) with chronic kidney disease examined ocular findings before a session of dialysis divided into two groups based on their duration of dialysis. Group one with twenty-seven patients (9 female & 18 male) under dialysis from one week to six months with mean & standard deviation (3.2037, ± 1.89259), group tow with fifty tow patients (36 female & 16 male) under dialysis from the duration over than six months with mean & standard deviation (44.2308, ± 26.24367) respectively. Patients aged (12 to 70 years) had mean age & ± standard deviation (35.1481, ± 12.88918), (44.4038, ± 15.42249) for two groups respectively. Patients in two groups had IOP (Right eye), its mean & standard deviation (15, ± 2.34), (15.69, ± 2.56) for group one & group tow respectively. Also, patients had CCT (Right eye) with mean & standard deviation (5.3467E2, ± 39.00296), (5.2312E2, ± 30.44162) for group one & group tow respectively. Patients had CCT (Left) with mean & standard deviation (5.2878E2, ± 37.55748), (5.2179E2, ± 29.58957) for group one & group tow respectively. Patients in two groups had average thickness RNFL (Right eye) with mean & standard deviation (1.0604E2, ± 25.17551), (95.6154, ± 21.27150) for group one & group tow respectively. Also, patients had average thickness RNFL (left eye) with mean & standard deviation (1.0930E2, ±23.80177), (98.7500, ± 23.77334) for group one & group tow respectively. Conclusions: This study found CCT effective with dialysis tend to be thin (53 patient,18 patient in group one &35 in group two) and that will be had a threefold higher risk of developing glaucoma when compared with thick average because of the IOP value affected by it. Refractive error effective with dialysis & become was more prominent that can be shown in the group two have (40 patient from 52) while (15 patient from27) in the group one although a lot of them corrected to the BCVA. In conclusion high value of the C/D ratio formed about (45.57%, 53.16%) to the right &left eye respectively this value will be form important sign of risk factor to progressive of glaucomatous need to be alert in the future. Also our research reveals CFT effective undergoing dialysis the thick value was (56 in the right eye, 55 in the left eye) high compared with the thin (9 in the right&9 in the left eye) & normal (14 in the right eye, 15 in the left eye). All the two groups of patients will be effected by the duration of dialysis with a time.


2018 ◽  
Vol 28 (2) ◽  
pp. 241-242 ◽  
Author(s):  
Ambreen Sarmad ◽  
Fadi Alfaqawi ◽  
Monali Chakrabarti ◽  
Arijit Mitra ◽  
Bushra Mushtaq

Purpose: Iris vascular tufts (IVT) are rare biomicroscopic capillary outgrowths from the pupillary margins. Patients are usually asymptomatic until presenting with blurred vision due to spontaneous hyphema or with raised intraocular pressure. Case report: A 61-year-old woman presented to eye casualty with left eye (LE) blurred vision and discomfort for 1 day. Her external ocular examination was unremarkable and visual acuity was 6/6 in the right eye (RE) and 6/9 in the LE. Biomicroscopic examination revealed a 2-mm hyphema in her LE and bilateral multiple small IVT and active bleeding from IVT at the pupillary margin of the LE at the 5 o’clock position. Diagnosis of LE active bleeding from IVT was made and she underwent argon laser photocoagulation directed at the source of bleeding. The bleeding stopped immediately after the second burn. She was followed up for 3 months; her visual acuity was 6/5 and 6/6 in the RE and LE, respectively, with no further problems. Conclusions: Iris vascular tufts are benign and recurrent hemorrhages are unlikely. Therefore, definitive argon laser photocoagulation or surgical treatment are reserved to arrest further episodes of hyphema. Our case demonstrates the effective use of argon laser photocoagulation to completely arrest active bleeding from IVT and excellent recovery of hyphema with no further problems for 5 years.


2019 ◽  
Vol 10 (3) ◽  
pp. 304-311
Author(s):  
Andreas F. Borkenstein ◽  
Eva-Maria Borkenstein

We report a case of a patient with progressed retinitis pigmentosa (RP) who underwent bilateral cataract extraction with implantation of a monofocal enlarged optic in the far dominant eye and a high-add AMD intraocular lens (IOL) in the near dominant eye (hybrid monovision XL-MAGS). A 71-year-old woman presented to our clinic complaining of reduced visual acuity additionally to her diagnosis of RP. The high-add IOL LENTIS® MAX LS-313 MF80 (Oculentis, Germany) was implanted in the right eye and the 7.0 mm optic ASPIRA-aXA IOL (HumanOptics, Germany) in the left eye. Six months postoperatively, the uncorrected distance visual acuity improved from hand motion to 0.5 logMAR in the right eye and to 0.3 logMAR in the left eye. Similarly, best corrected near visual acuity significantly improved to 0.4 and 0.7 logMAR, respectively. The patient’s subjective quality of life and autonomy improved significantly. RP is a severe retinal disease which leads to loss of vision and typical “tunnel vision” with visual field defects. As this genetic disorder is incurable, many ophthalmologists are not willing to perform cataract surgery. However, this case report shows that creating hybrid monovision with a high-add lens and a 7.0 mm optic IOL led to improvement of visual function and, more importantly, enhanced quality of life and self-autonomy of the patient.


2003 ◽  
Vol 131 (9-10) ◽  
pp. 396-399 ◽  
Author(s):  
Milos Jovanovic

OBJECTIVE The object of the study was to present successfully performed decompression of the orbit in orbital hematoma. MATERIAL AND METHODS Male 51 years of age sustained the contusion injury of the right eye during his fall on the bath tap. It caused the development of a large orbital hematoma with protrusion, restricted movement of the eyeball, pain, diplopia folding of the retina due to hemtoma compression to bulbus oculi and retinal hemorrhages, higher intraocular pressure and impaired visual acuity to 2/60. He was treated by medicaments for a month, but the results were not favorable. After one month from the injury, anterior-inferior transcutaneous orbitotomy was performed. During the surgical intervention hematoma and a part of orbital fat tissue were removed. RESULTS The globe of the eye was retracted and the exophthalmos disappeared immediately after the surgery, while the mobility of the eyeball, disappearance of retinal folds with gradual receding of hemorrhage, normal intraocular pressure abatement of pain and diplopia as well as the restoration of visual acuity to normal ensued in a few subsequent days. CONCLUSION This study illustrated that decompression of the orbit in orbital hematoma might be successful even in the late phase, e.g., one month following the injury. It is assumed that surgical intervention would be performed by experienced orbital surgeon.


2021 ◽  
Vol 14 (7) ◽  
pp. e242557
Author(s):  
Anusha Sachan ◽  
Deeksha Rani ◽  
Suman Lata ◽  
Rohan Chawla

An 18-year-old man presented with decreased vision in the right eye (OD) noticed for 1 month. On examination, OD best-corrected visual acuity was 3/60 and the left eye (OS) was 6/6 with intraocular pressure of 12 mm Hg in both the eyes (OU). OD fundus revealed an inferior optic-disc-pit with macular-retinoschisis and an inferior choroidal coloboma. OS fundus was normal. On swept-source optical coherence tomography (SSOCT) radial scans, peripapillary-retinoschisis was seen not only in the macular region but in all the four quadrants. To the best of our knowledge, no such case has been reported of optic disc pit with multiquadrant peripapillary retinoschisis and choroidal coloboma coexisting in the same eye. SSOCT radial scans can help detect subclinical retinoschisis as in this case.


2021 ◽  
Vol 10 (23) ◽  
pp. 5619
Author(s):  
Mineo Kondo ◽  
Hidetaka Noma ◽  
Masahiko Shimura ◽  
Masahiko Sugimoto ◽  
Yoshitsugu Matsui ◽  
...  

Purpose: To determine the baseline characteristics of patients with central retinal vein occlusion (CRVO) that were significantly associated with the best-corrected visual acuity (BCVA) at the initial examination. Methods: This was a retrospective multicenter study using the medical records registered in 17 ophthalmological institutions in Japan. Patients with untreated CRVO (≥20-years-of-age) who were initially examined between January 2013 and December 2017 were studied. The patients’ baseline factors that were significantly associated with the BCVA at the initial examination were determined by univariate and multivariate linear regression analyses. Results: Data from 517 eyes of 517 patients were analyzed. Univariate analyses showed that an older age (r = 0.194, p < 0.001) and the right eye (r = −0.103, p < 0.019) were significantly associated with poorer BCVA at the initial visit. Multivariate analyses also showed that an older age (β = 0.191, p < 0.001) and the right eye (β = −0.089, p = 0.041) were significantly associated with poorer BCVA at the initial visit. Conclusions: The results indicate that an older age, a known strong factor, and the right eye were significantly associated with poorer BCVA at the initial visit to the hospital. These results suggest that functional and/or anatomical differences between the right and left eyes may be involved in these results.


2021 ◽  
Author(s):  
Mineo Kondo ◽  
Hidetaka Noma ◽  
Masahiko Shimura ◽  
Masahiko Sugimoto ◽  
Yoshitsugu Matsui ◽  
...  

Abstract This retrospective multicenter study was designed to determine the baseline characteristics of patients in Japan with central retinal vein occlusion (CRVO) that were significantly associated with best-corrected visual acuity (BCVA) at the initial examination. The patients’ baseline factors that were significantly associated with the BCVA at the initial examination were determined by univariate, multivariate, and logistic regression analyses. Data from 517 eyes of 517 patients (mean age, 69.9 ± 12.2 years; men, 57.3%) were analyzed. Univariate analyses showed that an older age (r=0.194, P<0.001) and the right eye (r=-0.103, P<0.019) were significantly associated with poorer BCVA at the initial visit. Multivariate analyses showed that an older age (β=0.191, P<0.001) and the right eye (β=-0.089, P=0.041) were also significantly associated with poorer BCVA at the initial visit. In addition, logistic regression analyses showed that these two factors were also significantly related to the BCVAs poorer than 1.0 logMAR units (0.1 decimal BCVA) at the initial visit. Results indicate that an older age, a known strong factor, and the right eye were significantly associated with poorer BCVA at the initial visit. These results suggest that functional and/or anatomical differences between the right and left eyes may be involved in these results.


2018 ◽  
pp. bcr-2018-226067
Author(s):  
Sakolwat Montrivade ◽  
Patinya Maneesow ◽  
Thamonwan Osotthanakorn ◽  
Pairoj Chattranukulchai

We report a case of 46-year-old Asian woman with a history of recurrent epistaxis who presented with dyspnoea on exertion. Physical examination revealed mucocutaneous telangiectasias and signs of heart failure. Further evaluation showed huge hepatic arteriovenous malformation and severe pulmonary hypertension. This case demonstrates an uncommon manifestation of hereditary haemorrhagic telangiectasia presented with severe pulmonary hypertension.


2011 ◽  
Vol 22 (1) ◽  
pp. 104-110 ◽  
Author(s):  
Eugen Gramer ◽  
Constantin Reiter ◽  
Gwendolyn Gramer

Purpose To evaluate the following in patients with aniridia: age at first examination at the University Eye Hospital and age at diagnosis of glaucoma; visual acuity; frequency of family history of aniridia; and frequency of ocular and general diseases associated with aniridia. Methods This was a consecutive examination of 30 unrelated patients with aniridia and retrospective evaluation of ophthalmologic, pediatric, and internal findings. The relative frequency of age at glaucoma diagnosis within decades was evaluated for the 20 patients with aniridia and glaucoma. Statistical analysis was performed using the Mann-Whitney test. Results Relative frequency of the age of patients with aniridia at time of glaucoma diagnosis within the following decades was as follows: from birth to 9 years: 15%, 10-19: 15%, 20-29: 15%, 30-39: 15%, 40-49: 35%, and 50-59: 5%. Visual acuity in the better eye of 20/100 or less was found in 60%. Family history of aniridia was found in 33.3% of patients, with 1-4 relatives with aniridia. A total of 76.7% of patients had congenital cataract, and 66.7% had glaucoma. Mean maximum intraocular pressure of the 20 patients with glaucoma was 35.9 mmHg in the right and 32.6 mmHg in the left eye. A total of 53.3% had nystagmus, 26.6% corneal opacifications, 16.7% bilateral lens dislocation upwards, 6.7% optic nerve hypoplasia, 3.3% poor foveal development, and 3.3% Wilms tumor. Conclusions Up to the age of 40 years, 15% of patients were diagnosed with glaucoma per age decade. Frequent bilateral glaucoma and similar bilateral height of intraocular pressure suggest a genetic glaucoma disposition with malformation at Schlemm canal, besides possible sequential anatomic changes in the chamber angle. Associated ocular abnormalities limit visual prognosis.


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