Unusual anterior and posterior segment features of coats disease.

PURPOSE: To report the clinical course and management of unusual anterior and posterior segment features of Coats disease and their relation to the age of the patients to increase the awareness towards these rare clinical features rarely described in the current literature. METHODS: A retrospective descriptive review of 45 eyes of 45 patients affected by Coats disease was conducted at the Retinoblastoma Referral Center and Ophthalmology Unit of the University of Siena in Italy analyzing data from 2000 to 2022. Medical records and images were revised to find some cases presenting unusual anterior and posterior segment features in patients affected by Coats disease.We identified therefore 4 unusual clinical conditions: retinal macrocysts, anterior chamber cholesterolosis, fovea-sparing Coats disease and secondary vasoproliferative tumor. RESULTS: Two patients presented with retinal macrocyst (2/45 = 4.4%), one with anterior chamber cholesterolosis (1/45 = 2.2%), two with fovea sparing Coats disease (2/45 = 4.4%) and one with vasoproliferative tumor associated (1/45 = 2.2%) for a total of six (6/45 = 13.3%) patients manifesting unusual anterior or posterior segment features in Coats disease. CONCLUSION: Unusual anterior and posterior segment features of Coats disease such as retinal macrocyst and anterior chamber cholesterolosis have been more frequently reported in younger children while fovea-sparing and vasoproliferative tumors have been more commonly described in older patients. Age is then a strong prognostic marker which allows to distinguish two different phenotypes of Coats disease: patients younger and older than 3 years old with more aggressive and milder phenotype respectively.

Medulloepithelioma in newborn: Advanced clinical status.

We report three cases of congenital medulloepithelioma, which is an extremely rare clinical pattern of a tumor rare by definition. The aim of this study is to underline the clinical features of advanced medulloepithelioma in newborns.

Fovea-sparing coats disease: A rare clinical entity.

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.

Thyroiditis process as a predictive factor of sternotomy in the treatment of cervico-mediastinal goiter.

BACKGROUND: About 10% of cervico-mediastinal goiter need to associate cervicotomy with a total or partial sternotomy to allow a safe removal of the goiter. Aim of this study is to identify preoperative predictors of sternotomy for mediastinal goiter. METHODS: Between January 2008 and December 2015, 586 patients were submitted to total thyroidectomy at Surgical Clinic of Brescia, Italy. Among these, patients with cervico-mediastinal goiter have been divided in two groups based on the necessity of an associated sternotomy in the operating field: Group 1 (n = 40 patients) did not need sternotomy and Group 2 (n = 4 patients) underwent cervicotomy associated with sternotomy. Clinical and pathological characteristics of patients were retrospectivelly recorded. RESULTS: Among study group, 44 patients had cervico-mediastinal goiter. Thoracic CT was performed in all patients: an extension above aortic arch was found in 41 patients (93.18%) while an extention below aortic arch was found in 3 patients (6.82%). The extension of the goiter below the aortic arch resulted as a predictive value in the choice of surgical treatment (p = 0.0001). The thyroiditis process was found to be a significant predictive of the extention to a sternotomic approach (p = 0.029). The years of goiter's presence were on average 8.40 years in Group 1 and 14.75 years in Group 2. These parameters proved to be predictive when choosing a cervicotomy with sternotomy. CONCLUSIONS: Our study, despite limitations posed by small sample and its retrospective analisys, highlights the role of goiter's extention (below the aortic arch), disease length (for more than 14.75 years) and flogistic process (positivity of Tg Ab and anti-TPO-Ab) in the choice of combined (cervicotomic and sternotomic) approach to goiter's removal.