eISSN 1308-4038 International Journal of Anatomical Variations (2013) 6: 68–70 Case Report Variation in persistent hyperplastic primary vitreous Published online April 21st, 2013 © http://www.ijav.org Deepali Prashant ONKAR [1] Abstract Prashant Madhukar ONKAR [2] Persistent hyperplastic primary vitreous is a result of failure of regression of embryonic hyaloid artery. It leads to abnormal lenticular development and secondary changes in retina and orbit. It presents as leucocoria and the eye is usually microphthalmic. Departments of Anatomy [1] and Radiodiagnostics [2], NKP High frequency ultrasound usually demonstrates an echogenic mass posterior to the lens with Salve Institute of Medical Sciences and Research Center, a hyperechoic band extending from the optic disc to the posterior surface of the retrolental Nagpur, INDIA. mass, corresponding to the hyaloid canal. Visualization of hyaloid artery in this canal with color Doppler imaging is almost pathognomonic. A 3-month-old male infant presented with a unilateral leucocoria. High frequency ultrasound revealed echogenic band extending from optic disc to posterior aspect of lens. No retrolental Deepali Prashant Onkar mass was noted. On color Doppler, this band showed vascular pulsatile color flow in the persistent Associate Professor Anatomy hyaloid artery. Color Doppler was diagnostic in this variant of persistent hyperplastic primary NKP Salve Institute of Medical vitreous where retrolental mass was absent. Sciences and Research Center © Int J Anat Var (IJAV). 2013; 6: 68–70. Nagpur, 440016, INDIA. +91 (712) 2248590 [email protected] Key words [variant persistent hyperplastic primary vitreous] [color Doppler] [hyaloid artery] Received March 5th, 2012; accepted October 21st, 2012 [high frequency ultrasound] Introduction broadband linear probe on My Lab 50 ultrasound and color Persistent hyperplastic primary vitreous commonly presents Doppler machine manufactured by M/S Esaote, Italy. No as leucocoria at birth or early fetal life. This sporadic condition significant retrolenticular mass was noted. An echogenic is usually unilateral. Rare bilateral cases are associated with band extending from optic disc to posterior aspect of lens syndromes. Failure of regression of primary vitreous results was noted (Figure 1). The thickness of the band measured in its proliferation and a retrolental mass is formed. This between 2 to 3 mm. mass is attached to optic disc by a fibrovascular band [1]. The On color Doppler, this echogenic band showed color flow with artery in this primary vitreous if not regressed commonly direction towards the lens. On spectral Doppler the flow was causes vitreous hemorrhage in early life. Retinal detachment is common association. High frequency ultrasound is used found to be pulsatile indicating the persistent hyaloid artery for evaluation. Visualization of artery rules out retinal (Figure 2). No other finding was noted in orbit. Other orbit detachment or retinal hemangioblastoma. Demonstration of was normal on ultrasound. hyaloid artery by color Doppler on the band is pathognomonic Discussion [2]. Complications include hemorrhage and glaucoma. Various surgeries for visual rehabilitation have been described [3]. Persistent hyperplastic primary vitreous is usually noted at We present a variant of persistent hyperplastic primary birth or in early life. Rarely cases manifesting in adults have vitreous where retrolental mass was absent. been reported. The occurrence is sporadic. A predilection for whites is suggested [1]. The condition is unilateral in Case Report more than 90% cases. A case of association with Von-Hipple A 3-month-old male infant presenting with unilateral Lindau disease is reported [2]. Rare bilateral cases have been leucocoria was referred for high frequency ultrasound of left reported with Norrie disease, Warburg syndrome, and other orbit. He did not have any other obvious congenital anomaly. neurological and systemic anomalies [1]. Common clinical High frequency ultrasound was done with 7-12 MHz linear signs are leucocoria (abnormal white reflection from the Variant persistent hyperplastic primary vitreous 69 L OD Figure 2. Pulse Doppler shows pulsatile flow in the blood vessel indicating artery. surface of the optic stalk. The hyaloid artery vascularizes the developing retina and initially also vascularizes the lens vesicle. The lips of the choroidal fissure fuse by day 33, enclosing the hyaloid artery and accompanying vein in a canal in the ventral wall of the optic stalk. When the lens matures during fetal life and ceases to need blood supply, the portion of hyaloid artery that crosses the vitreous body degenerates and is removed by macrophages by the end of 4th month. The proximal part of the hyaloid artery becomes the central retinal artery, which supplies blood to the retina a [5]. When this primitive mesenchymal tissue persists and continues to proliferate, a retrolental mass is formed. The fibrovascular tissue behind the lens varies in extent and thickness. In first few months of life vitreous hemorrhage is common. Retinal detachment is seen in 30%–56% of cases [1]. High frequency ultrasound or B-Scan is usually diagnostic and is the investigation of choice. It demonstrates an echogenic mass posterior to the lens. A hyperechoic band extending from the posterior pole of the globe to the posterior surface of the retrolental mass, corresponding to the hyaloid canal is also seen. Ultrasound also demonstrates other associated findings like retinal detachment, hemorrhage. In our case a thin band of thickness 2-3 mm extending from optic disc to posterior aspect of lens was seen. No significant retrolental mass was found (Figure 1). b The hyaloid artery in this band may be seen with Doppler imaging [6]. The incidence of visualization of hyaloid Figure 1. a) High-resolution ultrasound of left orbit shows artery in persistent hyperplastic primary vitreous by color hyperechoic (bright) band (red arrow) extending from optic disc Doppler is not known. Color Doppler demonstrated a blood (OD) to posterior aspect of lens (L). b) Image shows Color Doppler indicating presence of blood vessel with flow towards lens. vessel with flow direction towards the lens in the echogenic band. On pulse Doppler, the vessel showed pulsatile flow pattern confirming presence of an artery. Visualization retina) and microphthalmia [4]. Sometimes it is associated of artery in this echogenic band is pathognomonic of with other ocular anomalies. persistent hyperplastic primary vitreous and virtually rules out other differentials like retinal detachment or During early embryonic life, hyaloid artery develops from retinal hemangioblastoma [2]. In this variant of persistent a branch of the ophthalmic artery and gains access to the hyperplastic primary vitreous, retrolental mass was absent lentiretinal space via the choroidal fissure on the ventral and use of color Doppler was diagnostic. 70 Onkar and Onkar Complications of this condition include hemorrhage and cataract surgery, intraocular lens placement, treatment of glaucoma, which usually result in enucleation. The treatment glaucoma, vitrectomy and removal of embryonic remnants consists of visual rehabilitation by various surgeries including [3]. References [1] Haddad R, Font RL, Reeser F. Persistent hyperplastic primary vitreous: a clinicopathologic [4] Aironi VD, Gandage SG. Pictorial essay: B-scan ultrasonography in ocular abnormalities. study of 62 cases and review of the literature. Surv Ophthalmol. 1978; 23: 123–134. Indian J Radiol Imaging. 2009; 19: 109–115. [2] Mihmanli I, Albayram MS, Kantarci F, Adaletli I, Islak C, Altug A. 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