A 7-week-old boy was referred to the pediatric ophthalmology department for irregular red reflex in the left eye. He was born at 40 weeks' gestation and weighed 3,827 grams, and there was no family history of syndromic disease. On examination, external appearance was notable for a dull red reflex of the left eye. At examination under anesthesia, intraocular pressure was normal in both eyes. Anterior and posterior segment examination of the right eye was normal. Anterior segment examination of the left eye revealed fine blood vessels at the surface of the iris, extending past the pupillary margin onto the anterior lens surface. The crystalline lens was clear with a dense membranous network of blood vessels just behind the posterior lens capsule (Figure 1A). There was no view of the posterior pole. Ultrasound biomicroscopy of the left eye revealed a prominent retrolenticular membrane with a small attenuated stalk emanating from the nasal aspect of the membrane and no ciliary body mass (Figure 1B). Ultrasound B-scan demonstrated attached retina and no intraocular mass (Figure 1C). Axial lengths were 20 and 17 mm in the right and left eyes, respectively.
What's Your Diagnosis?
For the correct answer, see page 372.
The answer for What's Your Diagnosis? is anterior persistent fetal vasculature (PFV). PFV is usually diagnosed within the first year of life, presenting commonly as leukocoria and/or strabismus.1–3 Persistent tunica vasculosa lentis is often seen in the anterior or combined form of PFV as a network of persistent perilenticular vessels.4 This case presented in characteristic fashion with unilateral findings in an eye with decreased axial length. Management and prognosis vary on the presentation of PFV. Anterior disease can be treated with lensectomy and membranectomy, whereas posterior or combined cases may necessitate vitrectomy with retinal detachment repair. Outcomes for anterior PFV are comparable to those of congenital cataract, whereas outcomes in combined or posterior PFV are variable.5–8 In this case, the lens was removed with generous posterior capsulotomy. Minimal bleeding was encountered at the time of surgery. Postoperative care included aphakic contact lens and amblyopia treatment.
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- Reese AB. Persistent hyperplastic primary vitreous. The Jackson Memorial Lecuture. Am J Ophthalmol. 1955;40(3):317–331. doi:10.1016/0002-9394(55)91866-3 [CrossRef]13248898
- Goldberg MF. Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV). LIV Edward Jackson Memorial Lecture. Am J Ophthalmol. 1997;124(5):587–626. doi:10.1016/S0002-9394(14)70899-2 [CrossRef]9372715
- Mutulu F, Leopold IH. The structure of fetal hyaloid system and tunica vasculosa lentis. Arch Ophthalmol. 1964;71:102–110. doi:10.1001/archopht.1964.00970010118019 [CrossRef]
- Sisk RA, Berrocal AM, Feuer WJ, Murray TG. Visual and anatomic outcomes with or without surgery in persistent fetal vasculature. Ophthalmology. 2010;117(11):2178–2183. doi:10.1016/j.ophtha.2010.03.062 [CrossRef]20619897
- Walsh MK, Dresner KA, Capone A Jr, Trese MT. Early vitrectomy effective for bilateral combined anterior and posterior persistent fetal vascular syndrome. Retina. 2010;30(4 suppl):S2–S8. doi:10.1097/IAE.0b013e3181d34a9e [CrossRef]20224462
- Hunt A, Rowe N, Lam A, Martin F. Outcomes in persistent hyperplastic primary vitreous. Br J Ophthalmol. 2005;89(7):859–863. doi:10.1136/bjo.2004.053595 [CrossRef]15965167
- Solebo AL, Russell-Eggitt I, Cumberland P, Rahi JS. Congenital cataract associated with persistent fetal vasculature: findings from IoL-under2. Eye (Lond). 2016;30(9):1204–1209. doi:10.1038/eye.2016.159 [CrossRef]