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Clinical Image Persistent hyaloid artery with an aberrant peripheral retinal attachment: A unique presentation

Jay U. Sheth, Ashish Sharma, Santonu Chakraborty1

Departments of Vitreoretina, 1Paediatric Ophthalmology, Lotus Care Hospital, Coimbatore, Tamilnadu, India

Persistence of the hyaloid vascular system is seen in 3% of full corneal diameters were 11.00 horizontally and 10.50 vertically by term infants and 95% of premature infants.[1] It may be present callipers. B‑scan ultrasound showed the presence of a moderately as Mittendorf’s dot located at the posterior capsule or as echogenic band extending from the to the peripheral Bergmeister’s papilla located at the optic disc.[1] Persistent hyaloid . On kinetic examination, the band had minimal mobility artery extending from the disc upto the posterior lens capsule is with no after movements [Figure 3]. The posterior lens capsule uncommon.[1] We hereby present a patient with persistent hyaloid did not reveal the presence of any remnant of the hyaloid vessel. artery extending from the optic disc to the superior part of retina Fundus fluorescein angiography showed the absence of blood flow with no remnant at the posterior lens capsule. in the hyaloid artery remnant, along with late hyperfluorescence at the site of attachment indicating staining [Figure 2c and d]. A 15‑year‑old male patientpresented to the out‑patient department Spectral domain – optical coherence tomography (SD‑OCT) at for routine eye examination. Present and past ocular history, and the level of the optic disc revealed the presence of a hollow tubule past medical history were unremarkable. He was born at eighth along with perivascular glial tissue [Figure 4]. With the help of months of gestation. There was no history of developmental delay. all these investigative modalities, a diagnosis of left eye persistent General examination was normal. hyaloid artery with an abnormal attachment to the superior retina was made. The patient has been advised to continue the current On ocular examination, the best‑corrected visual acuity (BCVA) glasses and follow‑up annually. with − 1.00 spherical power was 20/20, N6 in both . Intraocular pressure was 16 mmHg with applanation tonometry The hyaloid artery is a branch of the , which is a in both eyes. Examination of the right eye was normal. Slit lamp branch of . It is present in the optic canal and examination of the left eye revealed a normal anterior segment extends from the optic disc to the crystalline lens via the vitreous with clear posterior lens capsule [Figure 1]. Fundus examination humour. It is most prominent around ninth week of gestation showed the presence of a greyish‑white rounded fibrous tubule and slowly regresses by the seventh month.[1,2] Atrophy of the extending from the optic disc and attaching to the superior retina vessels begins in the posterior region (vasa hyloidea propria) and at 12 O’clock position, about 1 disc diameter posterior to the ora gradually progresses toward the anterior part (tunica vasculosa serrata. The site of attachment was greyish white in colour with lentis).[2] Mitchell and colleagues proposed that the regression the presence of glial tissue [Figure 2a and b]. Rest of the fundus is facilitated by the lens development that separates the fetal examination was normal. vasculature from the vascular endothelial growth factor leading to a reduction in the level of this survival factor culminating in On A‑scan biometry, the AC depth was 3.06 mm, the lens endothelial cell apoptosis.[2] Regression of the hyaloid artery thickness was 3.42 mm, and the axial length was 23.35 mm. The leaves behind a clear central zone in the vitreous humor, called the hyaloid or Cloquet’s canal. Access this article online Quick Response Code: Occasionally, the artery may not fully regress, leading to a [1] Website: condition persistent hyaloid artery. This may be either partial www.ojoonline.org or complete. Partial remnant of the anterior portion is present at the former site of anastomosis of the hyaloid artery and tunica DOI: vasculosa lentis.[1] It is called Mittendorf’s dot and is usually located 10.4103/0974-620X.111924 on the posterior lens capsule, inferonasal to the posterior pole of the lens.[1]

Copyright:  2013 Sheth JU, et al. This is an open‑access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Correspondence: Dr. Ashish Sharma, Consultant, Retina and Research, Lotus eye Care Hospital, Coimbatore, Tamilnadu, India. E‑mail: [email protected]

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Sheth and Sharma: Anatomical variation of persistent hyaloid artery – abnormal attachment to the peripheral retina

a b

c d Figure 1: Slit-lamp photo of the left eye in retroillumination showing clear posterior Figure 2: (a) Fundus photo of the left eye showing the persistent hyaloid artery arising lens capsule from the optic disc. (b) Fundus photo of the left eye showing the aberrant attachment of the persistent hyaloid artery at the peripheral retina. (c) FFA of the left eye showing the occluded hyaloid artery. (d) FFA of the left eye showing hyperfluorescense at the site of retinal attachment

Figure 3: Ultrasound B-scan showing the persistent hyaloid artery extending from the optic disc to the peripheral retina

Figure 4: SD-OCT image showing a tubular structure at the optic disc along with Remnant of the posterior portion is present at the optic disc perivascular glial tissue usually associated with some amount of glial tissue. It is called Bergmeister’s papillae and appears as a greyish linear structure lentis without much or any posterior hyaloid component.[4] anterior to the optic disc and adjacent to the retina.[1] Prepapillary Posterior PHPV is very rare in which opaque connective tissue loops that were earlier thought to be an exaggeration of Bergmeister’s arises from the Bergmeister’s papillae and persistent hyaloid papillae are now thought to be developmental anomaly of the vessels.[4] They can cause congenital falciform folds of the mesenchymal tissue that was destined to form the mature retinal retina and in severe cases, can cause tentlike retinal folds vasculature within the Bergmeister’s papillae.[1,3] Vitreous cysts are that may rarely lead to tractional or rhegmatogenous retinal benign lesions containing remnants of the hyaloid vascular lesions detachment.[4] resulting from abnormal regression.[3] Vitreous cysts are generally not symptomatic and thus do not require surgical intervention. In our case, the hyaloid vessel extended from the optic disc to the superior retina along with a clear posterior lens capsule. Rarely, the entire hyaloid artery may be present extending from Embryologically, it may be explained by an abnormality in the the optic disc to the posterior lens capsule. This hyaloid artery fetal vasculature regression pattern. There has been a case report may be patent or occluded. In patent hyaloid vessels, it may be of connections between the branches of persistent hyaloid supplying a part of the retinal tissue. This fact has to be kept in artery and peripheral retinal vessels.[5] However, after a thorough mind before planning to excise the vessel. PUBMED/MEDLINE search, an abnormal retinal attachment of anterior end of persistent hyaloid artery has not yet been described. Anterior persistent hyperplastic vitreous (PHPV) has In our case report, we have utilized sophisticated modalities like predominant features of persistent anterior tunica vasculosa B‑scan and SD‑OCT to validate the findings. The patient requires

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Sheth and Sharma: Anatomical variation of persistent hyaloid artery – abnormal attachment to the peripheral retina

regular follow‑up to look for early signs of complications such 3. Francois J. Pre‑papillary cyst developed from remnant of the hyaloid as tent‑like retinal folds, tractional or rhemnatogenous retinal artery. Br J Ophthalmol 1950;34:365. 4. Cockburn DM, Dwyer PS. Posterior persistent hyperplastic primary detachment, or any other unforeseen complications. vitreous. Am J Optom Physiol Opt 1988;65:316‑7. 5. Schwab S, Schriever D. Connections between the branches of a persistent References hyaloid artery and the peripheral retinal vessels. Ber Zusammenkunft Dtsch Ophthalmol Ges 1977;74:793‑4.

1. Jones H. Hyaloid remnants in the eyes of premature babies. Br J Ophthalmol 1963;47:39‑44. Cite this article as: Sheth JU, Sharma A, Chakraborty S. Persistent hyaloid 2. Mitchell CA, Risau W, Drexler HC. Regression of vessels in the tunica artery with an aberrant peripheral retinal attachment: A unique presentation. vasculosa lentis is initiated by coordinated endothelial apoptosis: A role Oman J Ophthalmol 2013;6:58-60. for vascular endothelial growth factor as a survival factor for endothelium. Sources of Support: Lotus vision research fund, None declared. Dev Dyn 1998;213:322‑33. Conflict of Interest:

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