Research progress of stellate multiform amelanotic choroidopathy_Chinese Journal of Ocular Fundus Diseases

Authors：

Abdulla Sawut , Su Yu ,  Chen Changzheng

Eye Center, Renmin Hospital of Wuhan University, Wuhan 430061, China;

Corresponding author：

Chen Changzheng, Email: whuchenchzh@163.com

Keywords：

Stellate multiform amelanotic choroidopathy; Serous maculopathy due to aspecific choroidopathy; Review

DOI：

10.3760/cma.j.cn511434-20241216-00491

Video：

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Abstract Full text Figures/Tables Video References Cited by

Stellate multiform amelanotic choroidopathy (SMACH) is a rare choroidal disease that can cause persistent subretinal fluid (SRF). It is more common in young individuals, with a similar male-to-female ratio, it is most often unilateral, and its clinical manifestations are diverse. The pathogenesis of SMACH is not well understood, but it may be a form of congenital choroidal dysplasia. The progressive impact of the lesion on the choroidal capillaries and the retinal pigment epithelium may be the cause of SRF. Its characteristic multimodal imaging changes include optical coherence tomography showing hyperreflective fibrous-like changes located in the inner choroidal stroma. Typical finger-like projections arranged in a stellate configuration are best seen on near-infrared imaging, indocyanine green angiography, and en face optical coherence tomography. The lesion is stable, with no progressive changes, and is unresponsive to treatment. Clinicians have limited knowledge about SMACH, which can lead to patients undergoing unnecessary or inappropriate treatments. Therefore, it is necessary to understand and recognize SMACH early in clinical practice.

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9.	Gajdzik-Gajdecka U, Dorecka M, Nita E, et al. Indocyanine green angiography in chronic central serous chorioretinopathy[J]. Med Sci Monit, 2012, 18(2): 51-57. DOI: 10.12659/msm.882455.
10.	Hansraj S, Chhablani J, Behera UC, et al. Inner choroidal fibrosis: an optical coherence tomography biomarker of severity in chronic central serous chorioretinopathy[J]. Am J Ophthalmol, 2024, 264: 17-24. DOI: 10.1016/j.ajo.2024.02.025.
11.	李海东, 沈丽君. 脉络膜色素痣的研究现状及进展[J]. 中华眼底病杂志, 2018, 34(2): 190-193. DOI: 10.3760/cma.j.issn.1005-1015.2018.02.024.Li HD, Shen LJ. Research status and progress of pigmented nevus of choroid[J]. Chin J Ocul Fundus Dis, 2018, 34(2): 190-193. DOI: 10.3760/cma.j.issn.1005-1015.2018.02.024.
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1. Ramtohul P, Pellegrini M, Pichi F, et al. Stellate multiform amelanotic choroidopathy: clinical and multimodal imaging features[J]. Retina, 2023, 43(9): 1448-1461. DOI: 10.1097/IAE.0000000000003826.
2. Fine SL, Owens SL. Central serous retinopathy in a 7-year-old girl[J]. Am J Ophthalmol, 1980, 90(6): 871-873. DOI: 10.1016/s0002-9394(14)75205-5.
3. Kim YC, Kim SD, Kim KS. A case of idiopathic central serous chorioretinopathy in a 12-year-old male treated with bevacizumab[J]. Korean J Ophthalmol, 2012, 26(5): 391-393. DOI: 10.3341/kjo.2012.26.5.391.
4. van Dijk EHC, Boon CJF. Serous business: delineating the broad spectrum of diseases with subretinal fluid in the macula[J/OL]. Prog Retin Eye Res, 2021, 84: 100955[2021-04-11]. https://pubmed.ncbi.nlm.nih.gov/33716160/. DOI: 10.1016/j.preteyeres.2021.100955.
5. van Dijk EHC, Ossewaarde-van Norel J, Vingerling JR, et al. Serous maculopathy due to aspecific choroidopathy (SMACH)[J]. Asia Pac J Ophthalmol (Phila), 2023, 12(5): 496-498. DOI: 10.1097/APO.0000000000000550.
6. Yu MD, Dalvin LA, Ancona-Lezama D, et al. Choriocapillaris compression correlates with choroidal nevus-associated subretinal fluid: OCT analysis of 3431 cases[J]. Ophthalmology, 2020, 127(9): 1273-1276. DOI: 10.1016/j.ophtha.2020.03.025.
7. Carrera W, Liang MC. A 19-year-old asymptomatic woman[J/OL]. Retin Cases Brief Rep, 2025, 2025: E1(2025-01-14)[2024-12-16]. https://pubmed.ncbi.nlm.nih.gov/39820360/. DOI: 10.1097/ICB.0000000000001716.[published online ahead of print].
8. 尹心恺, 戴荣平. 肥厚型脉络膜疾病研究现状及展望[J]. 中华实验眼科杂志, 2021, 39(1): 78-83. DOI: 10.3760/cma.j.cn115989-20200706-00478.Yin XK, Dai RP. Research progress on pachychoroid disease[J]. Chin J Exp Ophthalmol, 2021, 39(1): 78-83. DOI: 10.3760/cma.j.cn115989-20200706-00478.
9. Gajdzik-Gajdecka U, Dorecka M, Nita E, et al. Indocyanine green angiography in chronic central serous chorioretinopathy[J]. Med Sci Monit, 2012, 18(2): 51-57. DOI: 10.12659/msm.882455.
10. Hansraj S, Chhablani J, Behera UC, et al. Inner choroidal fibrosis: an optical coherence tomography biomarker of severity in chronic central serous chorioretinopathy[J]. Am J Ophthalmol, 2024, 264: 17-24. DOI: 10.1016/j.ajo.2024.02.025.
11. 李海东, 沈丽君. 脉络膜色素痣的研究现状及进展[J]. 中华眼底病杂志, 2018, 34(2): 190-193. DOI: 10.3760/cma.j.issn.1005-1015.2018.02.024.Li HD, Shen LJ. Research status and progress of pigmented nevus of choroid[J]. Chin J Ocul Fundus Dis, 2018, 34(2): 190-193. DOI: 10.3760/cma.j.issn.1005-1015.2018.02.024.
12. Francis JH, Pang CE, Abramson DH, et al. Swept-source optical coherence tomography features of choroidal nevi[J]. Am J Ophthalmol, 2015, 159(1): 169-176. DOI: 10.1016/j.ajo.2014.10.011.
13. Jonna G, Daniels AB. Enhanced depth imaging OCT of ultrasonographically flat choroidal nevi demonstrates 5 distinct patterns[J]. Ophthalmol Retina, 2019, 3(3): 270-277. DOI: 10.1016/j.oret.2018.10.004.
14. Shields CL, Arepalli S, Atalay HT, et al. Choroidal osteoma shows bone lamella and vascular channels on enhanced depth imaging optical coherence tomography in 15 eyes[J]. Retina, 2015, 35(4): 750-757. DOI: 10.1097/IAE.0000000000000376.
15. 宣懿, 王敏, 常青, 等. 脉络膜骨瘤扫频源光相干断层扫描影像特征[J]. 中华眼底病杂志, 2020, 36(6): 435-441. DOI: 10.3760/cma.j.cn511434-20200323-00126.

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