scholarly journals Intraocular Lens Subluxation in Marfan Syndrome

2014 ◽  
Vol 8 (1) ◽  
pp. 48-50 ◽  
Author(s):  
Bolaños-Jiménez Rodrigo ◽  
López-Lizárraga E Paulina ◽  
March de R Francesc ◽  
Telich-Tarriba J Eduardo ◽  
Navas Alejandro

Purpose : Ectopia lentis (EL) is a major criteria for the diagnosis of Marfan syndrome, it may vary from an asymptomatic mild displacement to a significant subluxation that places the equator of the lens in the pupillary axis. The purpose of this work is to present the case of a patient with Marfan syndrome who received treatment for subluxation at our institution. Case Report : A 51-year-old female diagnosed with Marfan syndrome presented to the emergency department with bilateral eye redness, foreign body sensation and crusting around the eyes on awakening. She had the following history of cardiac and ophthalmologic complications, including: 1. Lens subluxation 2. High myopia 3. Aortic root dilation, 4. Mitral valve prolapse and 5. Tricuspid insufficiency. Conclusion : The ophthalmological management of Marfan patients is challenging and periodical follow-up is needed. Surgical versus conservative management is controversial, each case needs to be evaluated individually to analyze the risks and benefits of the procedures.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Tianhui Chen ◽  
Michael Deng ◽  
Min Zhang ◽  
Jiahui Chen ◽  
Zexu Chen ◽  
...  

AbstractMarfan syndrome (MFS) is a hereditary disease with an incidence of 0.3% in the general population. Approximately 60% of MFS patients with FBN1 gene mutation will suffer ectopia lentis (EL) from the age of 3. With the development of EL, severe loss of vision will accrue because of lens tilt and glaucoma. Cionni modified capsular tension rings (MCTR) has been applied in the surgery for EL in MFS patients. To evaluate visual acuity and safety of using MCTR during lens subluxation surgery in MFS patients, 66 MFS patients (110 eyes) were included in our study, with the mean duration of follow-up of 4.7 months (SD 1.76 months). The capsular bags were preserved in 101 eyes (91.81%) with MCTR implantation. There was an overall significant improvement in BCVA at 1-month follow-up which was maintained at 3 months. Multivariable linear regression revealed that older age at first visit was associated with greater postoperative BCVA at the 1-month follow-up (P = 0.007). A significant difference was found between different degrees of lens subluxation and the length of surgical time and complications. At follow-up, only two eyes (1.98%) were identified to have developed retinal detachments. In conclusion, better visual outcomes can be achieved when patients received an early operation with MCTR implantation.


2017 ◽  
Vol 8 (1) ◽  
pp. 61-66 ◽  
Author(s):  
María Carmen Guixeres Esteve ◽  
Augusto Octavio Pardo Saiz ◽  
Lucía Martínez-Costa ◽  
Samuel González-Ocampo Dorta ◽  
Pedro Sanz Solana

The early development of lens opacities and lens subluxation are the most common causes of vision loss in patients with anterior megalophthalmos (AM). Cataract surgery in such patients is challenging, however, because of anatomical abnormalities. Intraocular lens dislocation is the most common postoperative complication. Patients with AM also seem to be affected by a type of vitreoretinopathy that predisposes them to retinal detachment. We here present the case of a 36-year-old man with bilateral AM misdiagnosed as simple megalocornea. He had a history of amaurosis in the right eye due to retinal detachment. He presented with vision loss in the left eye due to lens subluxation. Following the removal of the subluxated lens, it was deemed necessary to perform a vitrectomy in order to prevent retinal detachment. Seven months after surgery, an Artisan® Aphakia iris-claw lens was implanted in the anterior chamber. Fifteen months of follow-up data are provided.


2020 ◽  
Vol 28 (1) ◽  
pp. 75-79
Author(s):  
Mark Savage ◽  
Ross Kung ◽  
Cameron Green ◽  
Brandon Thia ◽  
Dinushka Perera ◽  
...  

Objective: To describe the characteristics of patients presenting to an Emergency Department (ED) following overdoses; to identify risk factors for intensive care unit (ICU) admission among these patients; and to identify the rate of mortality and repeat overdose presentations over four years. Methods: Adult patients presenting to ED following drug overdose during 2014 were included. Data were collected from medical notes and hospital databases. Results: During the study period, 654 patients presented to ED 800 times following overdose. Seventy-eight (9.8%) resulted in ICU admission, and 59 (7.4%) required intubation; 57.2% had no history of overdose presentations, and 72.9% involved patients with known psychiatric illness. Overdose of atypical antipsychotics (AAP), age and history of prior overdose independently predicted ICU admission. A third of patients ( n = 196, 30%) had subsequent presentations to ED following overdose, in the four years from their index presentation, with an all-cause four-year mortality of 3.4% ( n = 22). Conclusion: A history of overdose, use of AAP and older age were risk factors for ICU admission following ED presentations. Over a third of patients had repeat overdose presentation in the four-year follow-up with a mortality of 3.4%.


2020 ◽  
Vol 11 (2) ◽  
pp. 237-240
Author(s):  
Anupam Singh ◽  
M Vathulya ◽  
Ajai Agrawal ◽  
Rupal Verma ◽  
S. K. Mittal ◽  
...  

Background: Evisceration and nucleation are commonly performed ophthalmic surgeries for painful blind eye, disfiguring blind eye, endophthalmitis etc. After both these surgeries it is important to replace the lost volume in the orbit with implant. Implant is associated with many complications such as major discharge, exposure with discharge and implant exposure. The main surgical management of implant exposures basically primary revision or patch grafting with or without removal of the implant. Case: A 60 years old man presented to ophthalmic OPD with complaint of foreign body sensation and irritation in left eye. There was history of evisceration with silicon ball implant in left eye done one month back for painful blind eye at another hospital. On ophthalmic examination, there was a 3 × 4 mm of implant exposure most probably dueto tight closure. As per records the size of implant was 22mm. The patient was planned for extra-ocular myoplasty with buccal mucosal graft under general anesthesia. Observation: After sterile prepping and draping, 360˚ degrees peritomy was performed and care was taken to dissect between tenons and orbital implant. Medialand lateral recti were isolated and dissected upto 10-12mm from insertion site. Both the recti were secured with 6-0 vicryl suture and were detached from their respective insertions, advanced and approximated over the site of implant exposure. Thus the exposed implant was covered with a vascularized base which was reinforced with amucosal graft harvested from the buccal mucosa and secured with absorbable sutures. After 1 year of follow up patient was asymptomatic. Conclusion: Extraocular myoplasty with buccal mucosal graft is a good surgical remedy for orbital implant exposure implant.


2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Ana Cristina Silva ◽  
Pedro Soares Moreira ◽  
Vitor Costa Simões ◽  
Mónica Sampaio ◽  
Marisa Domingues Santos

Abstract Abdominal pain in a pregnant woman with a history of laparoscopic Roux-en-Y gastric bypass (LRYGB) in the emergency department is challenging. Intussusception is a rare cause of small bowel obstruction after LRYGB and can lead to intestinal necrosis, perforation, sepsis and death. The authors report a case of a 34-week pregnant patient, previously submitted to LRYGB, presenting to the emergency department with abdominal pain and vomiting. A computed tomography scan suggested the presence of ileoileal intussusception. So, an emergent laparotomy was performed with invagination reduction. The postoperative period was uneventful, as well as pregnancy and caesarian performed 4 weeks after surgery. At the 45-month follow-up, there was no recurrence of intussusception.


2020 ◽  
pp. 112067212093209 ◽  
Author(s):  
Marco H Ji ◽  
Sayena Jabbehdari ◽  
Natalia F Callaway ◽  
Darius M Moshfeghi

Introduction: Focal choroidal excavation (FCE) is a concavity of the choroid detected on optical coherence tomography (OCT). It is usually idiopathic and the affected eyes are otherwise healthy with near-normal overlying retinal architecture and good visual acuity. Purpose: To report a case of bilateral conforming FCE in the setting of Stargardt disease and inactive ocular toxoplasmosis. Case report: A 20-year-old man with known history of Stargardt disease, healed toxoplasmosis, and high myopia presented to our ophthalmology department for follow-up examination and was found with bilateral FCEs not present in any of his previous examinations. Conclusion: FCEs have been reported in the literature in otherwise healthy eyes as well as a broad spectrum of ocular diseases. The case herein reported Stargardt disease, toxoplasmosis, and high myopia all could potentially contribute to the pathogenesis of these findings. Further studies are needed to define etiologies as well as clinical significance and course of FCEs.


2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Abdullah M. Al Alawi

A 23-year-old male presented to the emergency department with one-day history of right-sided pleuritic chest pain, haemoptysis, and fever. In the emergency department, the blood pressure was 140/60 mmHg, heart rate 89/min, body temperature 40°C, respiratory rates 20 breaths/min, and oxygen saturation 98% in room air. Physical examination revealed rales and bronchial breathing in the right infrascapular region. Laboratory analysis showed raised white blood cell counts and elevated inflammation markers. Chest X-ray showed right lower lobe consolidation. Intravenous(IV) ceftriaxone and doxycycline were started for the management of community-acquired pneumonia as per the local guideline. Later, on admission, blood culture was positive for Neisseria meningitidis (N. meningitidis). Ceftriaxone was continued for 4 days, and the patient was discharged while being on oral amoxicillin (1 gm TDS) for another 3 days. He remained well during the outpatient follow-up.


2019 ◽  
Vol 3 (3) ◽  
pp. 226-228
Author(s):  
Oliver Morris ◽  
Josephin Mathai ◽  
Karl Weller

We report a case of polymethylmethacrylate cement pulmonary embolism (PE) that occurred two days following a minimally invasive kyphoplasty procedure. Our patient developed non-specific rib pain postoperatively followed by dyspnea, prompting presentation to the emergency department. The polymethylmetacrylate cement was visualized on initial chest radiograph and further characterized using computed tomography. The patient was admitted and anticoagulation started, later having an uncomplicated hospital course. The polymethylmethacrylate cement has a well-documented history of leakage and other postoperative complications. Cement PE, while rare, can present similarly to a thrombotic PE and requires adequate long-term anticoagulation with close follow-up.


2020 ◽  
Vol 30 (12) ◽  
pp. 1976-1977
Author(s):  
Abraham Groner ◽  
Asad Qadir

AbstractPatients with a known genetic cause of aortic root dilation usually have a single underlying aetiology, either a single gene defect as in Marfan syndrome or chromosomal anomaly as in Turner syndrome. However, it is possible, although unlikely, for a patient to inherit multiple independent risk factors for aortic root dilation. We describe such a patient, who inherited Marfan syndrome and a very unusual form of mosaic Turner syndrome. Long-term follow-up of this patient may provide insight into the natural history of this unique genetic combination.


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