scholarly journals Coats’ Disease: Very Long-Term Outcome After Early Stage Conventional Treatment

2016 ◽  
Vol 10 (1) ◽  
pp. 22-26 ◽  
Author(s):  
Salvatore Perrone ◽  
Alberto Rossetti ◽  
Patrick Sportiello ◽  
Pierfrancesco Mirabelli ◽  
Pierangela Cimatti ◽  
...  

Purpose: To report on the outcome of conventional therapy in patients with Coats’ disease. Methods: Retrospective analysis of the charts of thirteen patients with Coats’ disease. Results: Mean age of 9 male (70%) and 4 female (30%) patients was 17.7 (range, 5-33) years; one female had bilateral disease. Eleven eyes with retinal telangiectasia and exudation were treated with argon laser photocoagulation alone or photocoagulation associated with cryotherapy; the mean follow up was 32.5 (range,17-41) years. In four eyes without foveal involvement (stage 2a) the mean presenting visual acuity (VA) remained at 0.8 or improved, whereas poor VA in seven stage 2b eyes deteriorated minimally over time. In one and two of the three eyes with total retinal detachment, phthisis or neovascular glaucoma ensued. Conclusion: About three decades after conventional treatment of Coats’ disease stage 2a, treated eyes maintained good VA, and stage 2b eyes did not progress to advanced stages.

2020 ◽  
pp. 112067212097428
Author(s):  
Matteo Maria Girolamo ◽  
Sonia De Francesco ◽  
Federica Salvoldi ◽  
Matteo Barchitta ◽  
Cristina Menicacci ◽  
...  

Purpose: To evaluate the rarity, clinical features and management of Coats disease characterized by fovea-sparing enhancing the importance of pediatric retinal screening and early management to maintain a good visual acuity. Methods: Retrospective analysis of approximately 40 patients affected by Coats disease between 2000 and 2020 at the Retinoblastoma Referral Center and Ophthalmology unit of the University of Siena in Italy. Results: Two patients with fovea sparing Coats disease were included. Both presented an extrafoveal Coats disease (stage 2A by Shields classification) when they were 5 and 6 years old respectively. They had no anterior findings and a presenting visual acuity of 20/20 reflecting the early stage and a milder phenotype of the disease which are indeed more likely to be found in patients older than 3 years at presentation. Both presented telangiectasia and retinal exudation in the affected eye. Standard Argon laser photocoagulation and subsequently Cryotherapy were performed in the telangiectatic retinal periphery of both patients obtaining an excellent control and regression of the disease. Conclusions: Careful pediatric retinal screening and early management are crucial to ensure a good visual prognosis in such an early feature of Coats disease as fovea sparing since this condition unfortunately tends to recall the physician’s attention in more advanced stages. Due to the extremely poor number of articles regarding such a rare feature of Coats disease like fovea sparing, we report our experience.


2021 ◽  
pp. 1-11
Author(s):  
Hamid Reza Niknejad ◽  
Melissa Frederickx ◽  
Emiel Salaets ◽  
Jurgen Lemiere ◽  
Lieven Lagae ◽  
...  

<b><i>Introduction:</i></b> Tectal plate gliomas (TPG) constitute a distinct entity of benign tumors of the brain stem which show an indolent clinical course. Adequate treatment of secondary hydrocephalus is undoubtedly a major factor in the outcome. However, little is known about to what degree the tumor itself determines the long-term outcome of these patients. <b><i>Methods:</i></b> We retrospectively analyzed and compared the clinical and radiological data of 16 pediatric TPG patients with data of 12 pediatric idiopathic aqueductal stenosis (IAS) patients treated in our center from 1988 to 2018. For both groups, we assessed the long-term outcome in terms of hydrocephalus management, and for the TPG group, we assessed tumor growth during follow-up. In a separate prospective part of the study, we performed a neuropsychological evaluation in a subgroup of patients using a standardized testing battery, covering intelligence, learning, memory, executive functions, and an inventory on depression. <b><i>Results:</i></b> In the TPG group, the mean clinical and radiological follow-up was 84 and 70 months, respectively. On average, the maximum diameter of the tumor increased by 11% (<i>p</i> = 0.031) and the estimated tumor volume with 35% (<i>p</i> = 0.026) on radiological follow-up. The fronto-occipital horn ratio (FOHR) decreased by 23% on average after treatment. In the IAS group, the mean clinical and radiological follow-up was 117 and 85 months, respectively. In this group, the FOHR decreased by 21% on average. Neurocognitive testing revealed significant higher scores in the TPG group on global intelligence (TPG = 109, IAS = 85.5, <i>U</i> = 3, <i>p</i> &#x3c; 0.01, <i>z</i> = −2.71), performance (TPG= 100, IAS = 85, <i>U</i> = 7, <i>p</i> = 0.03, <i>z</i> = −2.2), and verbal intelligence (TPG = 122, IAS = 91.5, <i>U</i> = 2, <i>p</i> &#x3c; 0.00, <i>z</i> = −2.87) as well as working memory (TPG = 109.5, IAS = 77, <i>U</i> = 0.5, <i>p</i> = 0.01, <i>z</i> = −2.46). <b><i>Conclusion:</i></b> Our results suggest that the long-term outcome in TPG patients is acceptable and that cognition is substantially better preserved than in patients with IAS. This puts the idea of a significant contribution of the tumoral mass to disease outcome on the long term in question. Adequate and prompt management of hydrocephalus is the most important factor in long-term cognitive outcome.


2018 ◽  
Vol 35 (04) ◽  
pp. 263-269 ◽  
Author(s):  
Amir Bigdeli ◽  
Emre Gazyakan ◽  
Volker Schmidt ◽  
Christoph Bauer ◽  
Günter Germann ◽  
...  

Background Vascular occlusion after free flap surgery has become a rare complication but still poses a major challenge. It necessitates urgent re-exploration, but the logistic challenge to provide sufficient resources for the emergency intervention remains. The aim of this study was to analyze the long-term outcome after successful lower extremity free flap salvage. Methods A single-center retrospective study including long-term follow-up was approved by the local ethics committee. From January 1999 to December 2010, a total of 581 free flaps were performed for lower extremity reconstruction. Eighty-six flaps required emergency re-exploration, of which 65 could be salvaged. Fifteen salvaged flaps were excluded from the study because of secondary amputation. Of 50 patients, 29 (6 females and 23 males) were eligible for follow-up. The mean follow-up time was 54.5 ± 32.9 months. Health-related quality of life (Short Form 36 [SF-36]) and scar quality (Vancouver Scar Scale [VSS]) were analyzed. Results The overall flap survival rate was 94.7% and the total loss rate was 5.3%. The re-exploration rate was 14.8% (86 of 581 flaps). The salvage rate was 75.6% (65 of 86 flaps). Twenty-one free flaps were totally lost (24.4%). Partial flap loss occurred in 12 cases (14.0%); 67.5% of the vascular complications occurred during the first 24 hours, 20.9% between 24 and 72 hours, and 11.6% after more than 72 hours. The mean time from the first signs of impaired flap perfusion to re-exploration was 1.3 ± 0.4 hours, and from free tissue transfer to re-exploration was 16.2 ± 1.9 hours. The overall scar appearance was good with an average VSS score of 4.0 points. The average SF-36 physical component score was 54.4 ± 5.4 and the mental component score was 63.1 ± 10.7. Conclusion Careful monitoring and the opportunity for urgent re-exploration are the key to success for free flaps salvage. Following these principles, an acceptable long-term outcome can be achieved.


2017 ◽  
Vol 26 (9) ◽  
pp. 780-786 ◽  
Author(s):  
Jeffrey R. Peterson ◽  
John W. Anderson ◽  
Lauren S. Blieden ◽  
Alice Z. Chuang ◽  
Robert M. Feldman ◽  
...  

2018 ◽  
Vol 44 (2) ◽  
pp. 146-150 ◽  
Author(s):  
Thomas F. M. Yeoman ◽  
Oliver Stone ◽  
Paul J. Jenkins ◽  
Jane E. McEachan

The aim of this study was to investigate the long-term outcome of simple trapeziectomy by a single surgeon and to compare this with pre-operative function. Two hundred and five patients completed the shortened disabilities of the arm, shoulder and hand questionnaire (QuickDASH) and the EuroQoL five-dimensional questionnaire (EQ-5D) scores at a mean of 8.2 (range 3.5–17) years after simple trapeziectomy. There were no pre-operative scores available, so case controls were selected from our prospective database to compare pre- and post-operative patient-reported outcome measures. The mean QuickDASH score of the post-operative matched group was 37 (SD 17.0) and the mean EQ-5D was 0.56 (SD 0.31). The mean QuickDASH score of the pre-operative group was 54 (SD 17.0). The mean difference in QuickDASH between the pre- and post-operative groups was 17 (95% CI: 8 to 26, p = 0.0003). This study demonstrated a significant and sustained improvement in patient-reported function after simple trapeziectomy. It supports that simple trapeziectomy is a simple, safe and effective treatment for advanced trapeziometacarpal joint arthritis. Level of evidence: IV


2021 ◽  
Vol 12 (5) ◽  
pp. 616-627
Author(s):  
Alqasem Fuad H. Al Mosa ◽  
Sreenath Madathil ◽  
Pierre-Luc Bernier ◽  
Christo Tchervenkov

Background: Late pulmonary valve replacement following repair of tetralogy of Fallot may become necessary in patients with chronic pulmonary insufficiency. There is limited information on the long-term outcome of these prostheses, which is the focus of this study. Methods: We conducted a retrospective study of patients with repaired tetralogy of Fallot who underwent pulmonary valve replacement from 1990 to 2015 in our institution. We investigated imaging and clinical parameters including mortality and late adverse events (reintervention [surgical or transcatheter]), infective endocarditis, or arrhythmias requiring device implantation or ablation. Results: There were 69 patients divided into 3 groups: Carpentier-Edwards (n = 14), Contegra (n = 40), and pulmonary homograft (n = 15). The mean age at the time of pulmonary valve replacement was 21 ± 12 years. The mean follow-up was 8.5 ± 4.7 years. The mean preoperative and postoperative right ventricular end-diastolic volume index was 210 ± 42 and 120 ± 24 mL/m2, respectively. There were no mortalities. Late adverse events were observed in 23 (33%) patients: 15 (22%) reintervention (surgical or transcatheter), 11 (16%) endocarditis, and 11 (16%) arrhythmias. Overall, 1-, 5-, and 10-year freedom from surgical reintervention was 98.5%, 93.6%, and 79.3%, respectively. The Contegra group had significantly higher pulmonary valve gradients, a higher risk of developing late adverse events compared to Carpentier-Edwards ( P = .046) and pulmonary homograft ( P = .055) in multivariate analysis and increased risk for reintervention in the univariate analysis (hazard ratio: 3.4; 95% CI: 0.92-13; P value.066). Conclusion: Pulmonary valve replacement in patients with repaired tetralogy of Fallot has acceptable short- and intermediate-term outcomes. Contegra prosthesis had a higher risk of late adverse events with higher pulmonary valve gradients.


Author(s):  
K. Nielsen ◽  
M.V. Maraldo ◽  
M.C. Aznar ◽  
P.M. Petersen ◽  
I. Vogelius ◽  
...  

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