Darina Pospíšilová, Iveta Němcová, Jiří Pašta, Kateřina Hladíková, Eva Vyplašilová, Jan Havlik, Jan Tesař, Martin Šín
{"title":"阿尔波特综合征患者的屈光手术。病例报告。","authors":"Darina Pospíšilová, Iveta Němcová, Jiří Pašta, Kateřina Hladíková, Eva Vyplašilová, Jan Havlik, Jan Tesař, Martin Šín","doi":"10.31348/2024/28","DOIUrl":null,"url":null,"abstract":"<p><p>The authors present a case of a thirty-eight-year-old patient with Alport syndrome. The patient had several ocular symptoms of the disease and has been treated for systemic problems in connection with Alport syndrome since he was fifteen years old. At that age the patient also underwent a kidney transplant in order to deal with renal insufficiency. To date, he still uses immunosuppressants and antihypertensives. Furthermore, the patient suffers from perceptive deafness. The patient visited our clinic in 2021 with a request to solve his high refractive error, in which the diopters were so high that it was not possible to place them in spectacles. The patient's best corrected visual acuity was 0.6 with -8.0sph/-4.0cyl/ax15 in the right eye and 0.7partim with -8.0sph/-4.0cyl/ax155 in the left eye. The autorefractometer values were -6.25sph/-6.75cyl/ax17 in the right eye and -6.75sph/-6.5cyl/ax155 in the left eye. During the eye examination we found a number of ocular manifestations that are typical of Alport syndrome. On the cornea there were opacities as a residue of corneal erosions, and at one of the following check-ups we also found a newly developed corneal erosion. Subsequently, we found an anterior lenticonus and incipient cataract. Upon performing OCT, a typical temporal macular atrophy was evident. Fundus examination in artificial mydriasis showed just a minimal manifestation of fleck retinopathy. Due to the clinical manifestation we decided to perform cataract surgery and implant a monofocal toric intraocular lens in both eyes. There were no complications during the operations, however the surgeon registered a non-standard structure of the lens capsule. The capsule was more fragile, and performing capsulorhexis was much more complicated. A week after the surgery, higher cylinder diopters were still present. A decrease of the higher diopters was noticeable one month after surgery. The time interval between the first operation and the second operation was one month. The patient was highly satisfied with result, and uncorrected visual acuity improved by over four lines. After surgery the patient needed low diopters for near as well as far distance. In the case of this patient, the ocular manifestations were detected and treated in adulthood. Nevertheless, early detection of ocular symptoms of Alport syndrome in young patients before renal failure could lead to timely start of the treatment and delay a possible renal transplant. In case of any suspicion of Alport syndrome it is advised to send the patient to a pediatrician, and at an older age to an internal medicine specialist, for further examination.</p>","PeriodicalId":39839,"journal":{"name":"Ceska a Slovenska Oftalmologie","volume":"80 Ahead of print","pages":"332-337"},"PeriodicalIF":0.0000,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Refractive Surgery in a Patient with Alport Syndrome. A Case Report.\",\"authors\":\"Darina Pospíšilová, Iveta Němcová, Jiří Pašta, Kateřina Hladíková, Eva Vyplašilová, Jan Havlik, Jan Tesař, Martin Šín\",\"doi\":\"10.31348/2024/28\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>The authors present a case of a thirty-eight-year-old patient with Alport syndrome. The patient had several ocular symptoms of the disease and has been treated for systemic problems in connection with Alport syndrome since he was fifteen years old. At that age the patient also underwent a kidney transplant in order to deal with renal insufficiency. To date, he still uses immunosuppressants and antihypertensives. Furthermore, the patient suffers from perceptive deafness. The patient visited our clinic in 2021 with a request to solve his high refractive error, in which the diopters were so high that it was not possible to place them in spectacles. The patient's best corrected visual acuity was 0.6 with -8.0sph/-4.0cyl/ax15 in the right eye and 0.7partim with -8.0sph/-4.0cyl/ax155 in the left eye. The autorefractometer values were -6.25sph/-6.75cyl/ax17 in the right eye and -6.75sph/-6.5cyl/ax155 in the left eye. During the eye examination we found a number of ocular manifestations that are typical of Alport syndrome. On the cornea there were opacities as a residue of corneal erosions, and at one of the following check-ups we also found a newly developed corneal erosion. Subsequently, we found an anterior lenticonus and incipient cataract. Upon performing OCT, a typical temporal macular atrophy was evident. Fundus examination in artificial mydriasis showed just a minimal manifestation of fleck retinopathy. Due to the clinical manifestation we decided to perform cataract surgery and implant a monofocal toric intraocular lens in both eyes. There were no complications during the operations, however the surgeon registered a non-standard structure of the lens capsule. The capsule was more fragile, and performing capsulorhexis was much more complicated. A week after the surgery, higher cylinder diopters were still present. A decrease of the higher diopters was noticeable one month after surgery. The time interval between the first operation and the second operation was one month. The patient was highly satisfied with result, and uncorrected visual acuity improved by over four lines. After surgery the patient needed low diopters for near as well as far distance. In the case of this patient, the ocular manifestations were detected and treated in adulthood. Nevertheless, early detection of ocular symptoms of Alport syndrome in young patients before renal failure could lead to timely start of the treatment and delay a possible renal transplant. In case of any suspicion of Alport syndrome it is advised to send the patient to a pediatrician, and at an older age to an internal medicine specialist, for further examination.</p>\",\"PeriodicalId\":39839,\"journal\":{\"name\":\"Ceska a Slovenska Oftalmologie\",\"volume\":\"80 Ahead of print\",\"pages\":\"332-337\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Ceska a Slovenska Oftalmologie\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.31348/2024/28\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Ceska a Slovenska Oftalmologie","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.31348/2024/28","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
摘要
作者介绍了一例三十八岁的阿尔波特综合征患者。患者有多种眼部疾病症状,从十五岁起就开始接受与阿尔波特综合征有关的全身性治疗。在那个年纪,患者还接受了肾移植手术,以治疗肾功能不全。至今,他仍在使用免疫抑制剂和降压药。此外,患者还患有感知性耳聋。患者于 2021 年到我院就诊,要求解决他的高度屈光不正问题,因为他的屈光度数太高,无法配戴眼镜。患者右眼最佳矫正视力为-8.0sph/-4.0cyl/ax15,视力为 0.6;左眼最佳矫正视力为-8.0sph/-4.0cyl/ax155,视力为 0.7partim。自动屈光仪的数值为右眼-6.25sph/-6.75cyl/ax17,左眼-6.75sph/-6.5cyl/ax155。在眼部检查中,我们发现了一些阿尔波特综合征的典型眼部表现。角膜上有角膜侵蚀残留的不透明,在随后的一次检查中,我们还发现了新出现的角膜侵蚀。随后,我们又发现了前皮孔和初期白内障。在进行 OCT 检查时,我们发现了典型的颞黄斑萎缩。在人工昏迷状态下进行的眼底检查显示,仅有轻微的斑片状视网膜病变。根据临床表现,我们决定为他实施白内障手术,并在双眼植入单焦点散光人工晶体。手术过程中没有出现任何并发症,但医生发现晶状体囊的结构不符合标准。晶状体囊更加脆弱,进行囊外摘除术也更加复杂。手术一周后,仍有较高的圆柱度数。术后一个月,较高的屈光度数明显减少。第一次手术和第二次手术之间的时间间隔为一个月。患者对手术效果非常满意,未矫正视力提高了四行多。手术后,患者在近距离和远距离都需要低度数的屈光度。在这名患者的病例中,其眼部表现在成年后才被发现并得到治疗。不过,如果能在肾功能衰竭之前及早发现年轻患者的阿尔波特综合征眼部症状,就能及时开始治疗,并推迟可能的肾移植。如果怀疑患者患有阿尔波特综合征,建议将其送往儿科医生处做进一步检查,如果年龄较大,则送往内科专科医生处做进一步检查。
Refractive Surgery in a Patient with Alport Syndrome. A Case Report.
The authors present a case of a thirty-eight-year-old patient with Alport syndrome. The patient had several ocular symptoms of the disease and has been treated for systemic problems in connection with Alport syndrome since he was fifteen years old. At that age the patient also underwent a kidney transplant in order to deal with renal insufficiency. To date, he still uses immunosuppressants and antihypertensives. Furthermore, the patient suffers from perceptive deafness. The patient visited our clinic in 2021 with a request to solve his high refractive error, in which the diopters were so high that it was not possible to place them in spectacles. The patient's best corrected visual acuity was 0.6 with -8.0sph/-4.0cyl/ax15 in the right eye and 0.7partim with -8.0sph/-4.0cyl/ax155 in the left eye. The autorefractometer values were -6.25sph/-6.75cyl/ax17 in the right eye and -6.75sph/-6.5cyl/ax155 in the left eye. During the eye examination we found a number of ocular manifestations that are typical of Alport syndrome. On the cornea there were opacities as a residue of corneal erosions, and at one of the following check-ups we also found a newly developed corneal erosion. Subsequently, we found an anterior lenticonus and incipient cataract. Upon performing OCT, a typical temporal macular atrophy was evident. Fundus examination in artificial mydriasis showed just a minimal manifestation of fleck retinopathy. Due to the clinical manifestation we decided to perform cataract surgery and implant a monofocal toric intraocular lens in both eyes. There were no complications during the operations, however the surgeon registered a non-standard structure of the lens capsule. The capsule was more fragile, and performing capsulorhexis was much more complicated. A week after the surgery, higher cylinder diopters were still present. A decrease of the higher diopters was noticeable one month after surgery. The time interval between the first operation and the second operation was one month. The patient was highly satisfied with result, and uncorrected visual acuity improved by over four lines. After surgery the patient needed low diopters for near as well as far distance. In the case of this patient, the ocular manifestations were detected and treated in adulthood. Nevertheless, early detection of ocular symptoms of Alport syndrome in young patients before renal failure could lead to timely start of the treatment and delay a possible renal transplant. In case of any suspicion of Alport syndrome it is advised to send the patient to a pediatrician, and at an older age to an internal medicine specialist, for further examination.
期刊介绍:
- Redakce přijímá pouze práce vyhovující po odborné stránce, které jsou na odpovídající profesionální a formální úrovni. - Uveřejněná práce se stává majetkem časopisu, přetisknout její část nebo obrázek lze jen s citací původu. - Rukopis zasílejte v originále a dobře čitelné kopii (je nutná také kopie tabulek, legend, podle možnosti i obrázků). - Listy číslujte v pravém horním rohu a spojujte svorkou, nesešívejte. Přijímáme práce psané na jedné straně kvalitního bílého nelesklého papíru formátu A4 (neprůklepový) na psacím stroji nebo počítači s obvyklými typy.