Download PDF

Document downloaded from http://www.elsevier.es, day 03/05/2017. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(6):235–238
ARCHIVOS DE LA SOCIEDAD
ESPAÑOLA DE OFTALMOLOGÍA
www.elsevier.es/oftalmologia
Short communication
Bilateral persistent hyaloid artery. A case report夽
A.M. Borbolla-Pertierra a,∗ , C.K. Martínez-Hernández b , J.C. Juárez-Echenique a
a
b
Instituto Nacional de Pediatría, Mexico City, Mexico
Hospital General Manuel Gea González, Mexico City, Mexico
a r t i c l e
i n f o
a b s t r a c t
Article history:
Case report: A 5-year-old male presented with bilateral poor vision, esotropia and a previous
Received 11 December 2012
diagnosis of cataract since he was 1 year old. The physical examination revealed bilateral
Accepted 27 May 2013
posterior paracentric capsule opacification, vitreous cavity with a permeable pulsatile blood
Available online 13 August 2014
filled hyaloid artery in both eyes. He was kept under observation.
Keywords:
often unilateral (although it may be bilateral) and sporadic, associated with microphthal-
Persistent hyaloid artery
mos. It may be complicated with glaucoma and phthisis bulbi. Vitrectomy plus lensectomy
Cataract
or simple observation is the accepted treatment option.
Discussion: Persistent hyaloid artery is an uncommon faulty primary vitreous regression,
Microphthalmos
© 2012 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights
reserved.
Vitreous body
Congenital abnormality
Persistencia bilateral de la arteria hialoidea. Reporte de un caso
r e s u m e n
Palabras clave:
Caso clínico: Varón de 5 años de edad con mala visión bilateral, endotropía y diagnóstico
Persistencia de la arteria hialoidea
previo de catarata desde el año de edad. En la exploración, se observa en ambos ojos opacidad
Catarata
capsular posterior paracentral, cavidad vítrea con arteria hialoidea permeable, ocupada por
Microftalmos
sangre y con pulso. Se dejó en vigilancia.
Cuerpo vítreo
Discusión: La persistencia de la arteria hialoidea es infrecuente y es una falla en la involución
Anomalía congénita
del vítreo primario. Comúnmente es unilateral (aunque hay casos bilaterales), esporádico y
asociado a microftalmos. Puede complicarse con glaucoma y pthisis bulbi. Vitrectomía más
lensectomía u observación son opciones de tratamiento según el caso.
© 2012 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. Todos
los derechos reservados.
Please cite this article as: Borbolla-Pertierra AM, Martínez-Hernández CK, Juárez-Echenique JC. Persistencia bilateral de la arteria
hialoidea. Reporte de un caso. Arch Soc Esp Oftalmol. 2014;89:235–238.
∗
Corresponding author.
E-mail address: [email protected] (A.M. Borbolla-Pertierra).
夽
2173-5794/$ – see front matter © 2012 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
Document downloaded from http://www.elsevier.es, day 03/05/2017. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.
236
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(6):235–238
Case report
Male patient, age 5, whose mother reported having chickenpox in the fifth month of pregnancy, consulted for poor vision,
inward ocular deviation and bilateral cataract since he was
12 months old.
Physical examination produced a visual acuity of no light
perception in right eye (RE) and 20/200 in left eye (LE). The
following were observed: Refraction: RE +2.00 with −7.25 × 90,
LE +1.00 with −5.25 × 70, without achieving visual improvement; endotropia of 25 prismatic diopters, normal duction and
version; ocular posterior capsular opacity in both lenses and
retrolental tissue in RE (Fig. 1); open iridocorneal angles and
intraocular pressure of 15 mmHg in both eyes (BE); vitreous
cavity of BE with functional vascular canal from the papilla to
the lens, presence of pulse, small and pale optic nerves with
coloboma-type defect, hypopigmented macular chorioretinal
scar of about 2 papillar diameters in RE (Figs. 2 and 3).
Fluorangiography: fetal vessels filled with fluorescein,
demonstrating functionality (Figs. 4 and 5).
Magnetic resonance: hypotensive central hyaloid canal in
T2 projection (Fig. 6). It was decided to maintain the patient
under observation.
Discussion
The persistence of fetal vessels, also known as persistence
of hyaloid artery or persistent hyperplastic primary vitreous
(PHPV), is a rare congenital anomaly generally of unknown
cause. Most cases exhibit retrolental plate in a microphthalmic
eye.1 It was initially described as a syndrome by Reese in 1955.2
The defect consists in failed primary vitreous regression.2
The hyaloid artery is formed on the basis of mesenchymatous cells which gained access through the optic fissure.3 This
artery surrounds the lens contributing to form the tunica vasculosa lentis.3 Complete primary vitreous regression must take
place between the seventh and eighth month of gestation.3 It
is anatomically classified on the basis of anterior or posterior
primary vitreous persistence.
PHPV is the second most common cause of acquired
cataract during the first year of life.4 It is most frequently
Fig. 2 – Right eye ocular fundus. Tissue beginning in the
papilla and partially occupying the vitreous cavity, macular
chorioretinal scar with central hyperpigmentation areas
and RPE atrophy at the lesion edges. Choroidal vessels can
be seen.
diagnosed during the first year of life and no relevant familial
history has been identified.5
With greater frequency it is unilateral although it can
express bilaterally in up to 11%2,5 of cases and is associated
to microphthalmos in 2/3 of patients5 .
It expresses mainly with leukocoria, which requires differential diagnosis with cataracts, retinoblastoma, retinopathy
of prematurity, posterior uveitis and Coats disease, among
others.4
Natural history is toward recurring vitreous hemorrhage,
retina detachment, uncontrollable secondary glaucoma and
phtisis bulbi, for which reason its only treatment on some occasions is enucleation4 .
Diagnostic can be clinical. However, A-B mode echography
is essential particularly in the presence of opacity, exhibiting a stem from the posterior pole to the lens, in addition
to discarding or confirming the presence of microphthalmos
or associated retina detachment.5 Color Doppler echography helps to confirm the vascular nature of the lesion.4
Fig. 1 – Microscopic image of anterior segment of both eyes. Superior opacity behind both lenses and presence of retrolental
tissue visible in the right eye (left side).
Document downloaded from http://www.elsevier.es, day 03/05/2017. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(6):235–238
Fig. 3 – Left eye ocular fundus. Tissue beginning in the
papilla and partialLy occupying the vitreous cavity.
Computerized tomography is useful for discarding calcification although this is extremely rare4 in PHPV. Magnetic resonance (MR) enables a clearer image of PHPV and is excellent
for differential diagnostics. In MR, the retrolental membrane
is hyperintense after the application of contrast and shows a
tube-shaped image which represents the hyaloid vessel.6
Treatment is controversial and has three objectives: (1) to
prevent glaucoma, (2) pupil esthetics and (3) visual
improvement.5 Pollord4 and Haddad5 reported long series
including vitrectomy and lensectomy in some cases, concluding that the patients who only exhibited the anterior
primary vitreous persistence variety have good results, in
contrast with those having the posterior variety who, despite
all efforts, did not obtain visual improvements and exhibited
complications with greater frequency. Mittra7 concluded that
good results are obtained with the latest vitreoretinal techniques for surgery and intensive treatment for amblyopia.
Sisk8 treated 81 cases with surgery and observed greater
incidence of glaucoma in operated patients.
Fig. 5 – Left eye fluorangiography at 4:17 min. Persistence
of hyaloid artery hyperfluorescence with contrast diffusion
toward surrounding tissue.
Fig. 6 – Orbitary magnetic resonance, T2 projection, axial
section, showing hypointense central hyaloid canal
vis-à-vis the vitreous in both eyes.
There are very few reported bilateral cases. Hunt9 reported
five patients with bilateral anomaly out of 55 with PHPV,
and reported only two patients with isolated hyaloid artery
as sole expression of PHPV, who were managed conservatively. Said author also recommended avoiding surgery when
the visual axis is free, anatomic anomalies are not progressive and the chamber angle is not compromised.9 In turn,
Kumar10 reported 11 cases of bilateral fetal vessel persistence and indicated this could represent a separate clinical
entity.
In the case reported herein it was decided to treat the
patient conservatively due to the presence of blood flow in the
remaining vessel (vitreous hemorrhage risk), absence of progression during the observation period, lack of alteration of
iridocorneal angles and the certainty of poor visual prognosis
(RE macular scar, LE amblyopia).
Conflict of interest
Fig. 4 – Right eye fluorangiography at 40 s.
Hyperfluorescence of the macular chorioretinal scar edge.
237
No conflict of interest was declared by the authors.
Document downloaded from http://www.elsevier.es, day 03/05/2017. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.
238
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(6):235–238
references
1. Lambert SR. Chilhood cataracts. In: Hoyt C, Taylor D, editors.
Pediatric ophthalmology and strabismus. 4th ed. Elsevier;
2013. p. 341–2.
2. Reese AB. Persistent hyperplastic primary vitreous. Jackson
Memorial Lecture. Trans Am Acad Ophthalmol Otolaryngol.
1955;59:271–86.
3. Cook CS. Embriology. In: Wright KW, editor. Handbook
of pediatric eye and systemic disease. 2nd ed. New York:
Springer; 2006. p. 19–20.
4. Pollord Z. Persistent hyperplastic primary vitreous: diagnosis,
treatment and results. Trans Am Ophthalmol Soc.
1997;XCV:487–549.
5. Haddad R, Font RL, Reeser F. Persistent hyperplastic primary
vitreous. A clinicopathologic study of 62 cases and review
of the literature. Surv Ophthalmol. 1978;23:123–34.
6. Sun MH, Kao LY, Kuo YH. Persistent hyperplastic primary
vitreous: magnetic resonance imaging and clinical findings.
Chang Gung Med J. 2003;26:269–76.
7. Mittra RA, Huynh LT, Ruttum MS, Mieler WF, Connor TB, Han
DP, et al. Visual outcomes following lensectomy and
victrectomy for combined anterior and posterior persistent
hyperplastic primary vitreous. Arch Ophthalmol.
1998;116:1190–4.
8. Sisk RA, Berrocal AM, Feuer WJ, Murray TG. Visual and
anatomic outcomes with or without surgery in persistent
fetal vasculature. Ophthalmology. 2010;117:
2178–83.
9. Hunt A, Rowe N, Lam A, Martin F. Outcomes in persistent
hyperplastic primary vitreous. Br J Ophthalmol.
2005;89:859–63.
10. Kumar A, Jethani J, Shetty S, Vijayalakshmi P. Bilateral
persistent fetal vasculature: a study of 11 cases. J AAPOS.
2010;14:345–8.