Abstract
Background/Objectives
To analyze the ophthalmic characteristics of congenital prepapillary vascular loop (PVL) and to propose a new morphologic classification dividing the loops into six types.
Subjects/Methods
Collaborative multinational multicentre retrospective study of PVL cases.
Results
There was a total of 49 cases (61 eyes), 37 unilateral (75.5%) and 12 bilateral (24.5%), 32 arterial type (65.3%) and 18 venous type (36.7%) (one patient had either kind in each eye). The mean number of loops per eye was 2.7 (range, 1–7). The loops were asymptomatic in 42 cases (85.7%). Other findings included: the presence of cilioretinal artery (14 cases), retinal vascular tortuosity (26 cases), amaurosis fugax (1 case), branch retinal artery occlusion (1 case) and vitreous haemorrhage (3 cases). Six morphologic loop types could be discerned based on elevation (flat vs. elevated), shape (figure of 8 or corkscrew with hyaline sheath), number (multiple or single), location (central or peripheral), lumen size (arterial vs. arteriolar) and presence of vascular tortuosity or vitreous traction.
Conclusions
PVL are usually asymptomatic and can be divided into six morphologic types with different pathogenesis during early embryogenesis.
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References
Awan KJ. Arterial vascular anomalies of the retina. Arch Ophthalmol. 1977;95:1197–202.
Awan KJ. Anomalies of the retinal veins and their incidence. J Pediatr Ophthalmol. 1976;13:353–9.
Goldstin I, Wexler D. The preretinal artery. An anatomic study. Arch Ophthalmol. 1929;1:324–34.
Merrill UH, Wagener HP. Anomalous spiral looping of a retinal vein. Am J Ophthalmol. 1924;7:177–9.
Francis LM. Anomalous spiral looping of retinal artery. Am J Ophthalmol. 1921;4:202–3.
Liebreich R. Demonstrations of diseases of the eye: persistent hyaloid artery and vein. Trans Pathol Soc Lond. 1871;22:221–4.
Kaimbo Wa Kaimbo D. Frequency of prepapillary vascular loops in Congolese patients. J Fr Ophtalmol. 2016;39:711–5.
Degenhart W, Brown GC, Augsburger JJ, Magargal L. Prepapillary vascular loops. Ophthalmology. 1981;88:1126–31.
Hsieh YT, Yang CM. The clinical study of congenital looped/coiled peripapillary retinal vessels. Eye. 2005;19:906–9.
Nishimura T, Uyama M. Retinal vascular loop formation on the optic disc. Jpn J Clin Ophthalmol. 1982;36:1371–5.
Tamura M, Atsumi O, Yoshimoto H. Retinal vascular loop formation on the optic disc: retrospective study of eleven cases. Folia Ophthalmol Jpn. 1991;42:304–10.
Teramoto S, Ohno-Matsui K, Tokoro T, Ohno S. Peripapillary loops of venous origin are extremely rare. Jpn J Ophthalmol. 1999;43:422–5.
Makino S. Prevalence of prepapillary vascular loop. Scholars J Appl Med Sci. 2015;3:693–4.
Shakin EP, Shields JA, Augsburger JJ, Brown GC. Clinicopathologic correlation of a prepapillary vascular loop. Retina. 1988;8:55–8.
Mireskandari K, Aclimandos WA. Probably the longest prepapillary loop in the world. Retina. 2001;21:393–5.
Regenbogen L, Godel V. Spiral looping of retinal artery. J Pediatr Ophthalmol. 1977;14:117–9.
Romano PE. Prepapillary vascular loops. Clin Exp Ophthalmol. 2001;29:90–1.
Grossniklaus H, Thall E, Annable W. Familial prepapillary vascular loops. Arch Ophthalmol. 1986;104:1755–6.
Lambert HM, Sipperley JO, Shacklett DE. Autosomal dominant preretinal vascular loops. Retina. 1983;3:258–60.
Morán M. [Inherited (Pre)retinal arterial loop (author’s transl)]. Cesk Oftalmol. 1981;37:436–43.
Sugiuchi K, Mori K, Deguchi T, Yoneya S. [Chorioretinal malformation in vascular loop formation on the optic disc]. Nippon Ganka Gakkai Zasshi. 1998;102:215–20.
Punja K, Sharma S. Ophthaproblem. Prepapillary vascular loops. Can Fam Physician. 2002;48:41–50.
Soltau JB, Olk RJ, Gordon JM. Prepapillary arterial loop associated with vitreous hemorrhage and venous retinal macrovessel. Retina. 1996;16:74–5.
Giuffrè G, Lodato G. Prepapillary venous loops and choroidal veins tortuosity. Acta Ophthalmol. 2003;81:665–6.
Walland M. Prepapillary vascular loop associated with persistent hyperplastic primary vitreous. JAMA Ophthalmol. 2015;133:362.
Brown GC, Magargal L, Augsberger JJ, Shields JA. Preretinal arterial loops and retinal arterial occlusion. Am J Ophthalmol. 1979;87:646–51.
Brucker AJ, Michels RG, Fine SL. Congenital retinal loops and vitreous haemorrhage. Am J Ophthalmol. 1977;84:220–3.
Rahimy E, Rayess N, Talamini CL, Kaiser RS. Traumatic prepapillary loop torsion and associated branch retinal artery occlusion. JAMA Ophthalmol. 2014;132:1376–7.
Codenotti M, Fogliato G, De Benedetto U, et al. Simultaneous vitreous hemorrhage and branch retinal artery occlusion after prepapillary arterial loop rupture. J Fr Ophtalmol. 2013;36:e63–5.
Ding PC, Chen MT. Peripapillary arterial loop-case report. Kaohsiung J Med Sci. 1999;15:510–2.
Misra A, Flanagan DW, Martin KR. Recurrent transient visual loss due to intermittent occlusion of a prepapillary vascular loop. Br J Ophthalmol. 2008;92:431–2.
Fujiwara T, Machida S, Herai T, Tazawa Y. Case of subretinal hemorrhage that developed from a prepapillary vascular loop. Jpn J Ophthalmol. 2004;48:175–7.
Youssoufou Souley AS, Alsubari A, Chammout FZ, et al. [Prepapillary vascular loop with vitreous traction]. J Fr Ophtalmol. 2018;41:1002.
Owen CG, Rudnicka AR, Mullen R, et al. Measuring retinal vessel tortuosity in 10-year-old children: Validation of the computer-assisted image analysis of the retina (CAIAR) program. Investig Ophthalmol Vis Sci. 2009;50:2004–10.
Sutter FKP, Helbig H. Familial retinal arteriolar tortuosity: a review. Surv Ophthalmol. 2003;48:245–55.
Lee KE, Jeong EH, Yu HJ, et al. Cerebral infarction caused by a tortuous subclavian artery: a case report. Neurointervention. 2014;9:53–5.
Beyens A, Albuisson J, Boel A, et al. Arterial tortuosity syndrome: 40 new families and literature review. Genet Med. 2018;20:1236–45.
Fruttiger M. Development of the retinal vasculature. Angiogenesis. 2007;10:77–88.
Toma N. Anatomy of the ophthalmic artery: embryological consideration. Neurol Med Chir. 2016;56:585–91.
McLeod DS, Hasegawa T, Prow T, et al. The initial fetal human retinal vasculature develops by vasculogenesis. Dev Dyn. 2006;235:3336–47.
Raman R, Gella L, Kazi MS. Congenital preretinal arterial loop: Is it a misnomer? Oman J Ophthalmol. 2017;10:54–5.
Han HC. Twisted blood vessels: symptoms, etiology and biomechanical mechanisms. J Vasc Res. 2012;49:185–97.
Vedantham V, Ramasamy K, Namperumalsamy P, Cunningham ET. Double prepapillary arterial loops associated with superior branch macular artery occlusion. Ind J Ophthalmol. 2005;53:126–8.
Mansour AM, Walsh JB, Henkind P. Arteriovenous anastomoses of the retina. Ophthalmology. 1987;94:35–40.
Zaret CR, Choromokos EA, Meisler DM. Cilio-optic vein associated with phakomatosis. Ophthalmology. 1980;87:330–6.
Cohen SY. Acquired prepapillary arterial loop after central retinal artery obstruction. Arch Ophthalmol. 1998;116:1398–9.
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Mansour, A.M., Kozak, I., Saatci, A.O. et al. Prepapillary vascular loop-a new classification. Eye 35, 425–432 (2021). https://doi.org/10.1038/s41433-020-0859-3
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DOI: https://doi.org/10.1038/s41433-020-0859-3
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