Persistent hyperplastic primary vitreous phpv is an ocular developmental disorder resulting from incomplete apoptosis of the embryonic hyaloid vasculature. Bilateral persistent hyperplastic primary vitreous a rare case. Persistent hyperplastic primary vitreous or persistent fetal vasculature is considered to be a congenital, nonheritable malformation caused by the failed regression of the primitive. We report the mri features of phpv of the dorsal type to facilitate differential diagnosis from other causes of leukocoria, namely retinoblastoma, which may have major therapeutic consequences.
Computed tomography in the evaluation of patients with persistent hyperplastic primary vitreous phpv. We recently encountered a patient with the anterior variant of phpv who had mr imaging of the orbits. The space at the back of the eye, behind the lens, is normally filled with a clear jelly. To report outcomes in cases of persistent hyperplastic primary vitreous phpv and examine factors predictive of visual acuity in patients selected for surgery. Changes in the contralateral eye in uncomplicated persistent.
It is divided into anterior and posterior types and is characterized by the presence of a vascular membrane located behind the lens. Find out information about primary persistent hyperplastic vitreous. The imaging features of persistent hyperplastic primary vitreous phpv affecting the posterior eye are well known. Persistent hyperplastic primary vitreous phpv is a rare congenital developmental malformation of the eye that commonly manifests in a fullterm infant with leukocoria and microphthalmos 1,2. Clinical presentation clinically, this condition usually manifests as unilateral. It happens due to a failure in the regression of foetal hyaloid vasculature and primary vitreous, which remains inside the hyaloid canal in the vitreous chamber leading to fibrosis and several abnormalities 1. The most important factor in the prognosis of patients with persistent hyperplastic primary vitreous is the extent of the membrane. Persistent hyperplastic primary vitreous was first described by reese in 1955 in his jackson memorial lecture. Persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature pfv, is a rare congenital developmental anomaly of the eye that results following failure of the embryological, primary vitreous and hyaloid vasculature to regress.
Pdf while the great majority of patients with persistent hyperplastic primary vitreous never obtain useful vision, it is encouraging that 18. Ophthalmic doppler in persistent hyperplastic primary. Primary persistent hyperplastic vitreous article about. We report a child with unilateral phpv who underwent cataract extraction, primary. Persistent hyperplastic primary vitreous phpv is a developmental malformation of the eye due to the presence of a retrolental fibrovascular membrane with persistence of the posterior portion of the tunica vasculosa lentis and hyaloid artery. Persistent hypertrophic primary vitreous radiology. Diagnosis, treatment and results article pdf available in transactions of the american ophthalmological society 95. The anterior and posterior hyaloid vascular systems regress independently. Unilateral persistent hyperplastic primary vitreous. Emphasis is placed on early operative intervention in order to preserve the globe, as well as. Families should be told that if their child has only an anterior presentation, then surgical and amblyopia therapies offer a good chance for useful vision. Variation in persistent hyperplastic primary vitreous. Persistent hyperplastic primary vitreous phpv is a rare congenital developmen tal malformation of the eye that commonly manifests in a fullterm infant with leukocoria and microphthalmos 1,2.
Outcomes in persistent hyperplastic primary vitreous article pdf available in british journal of ophthalmology 897. The primary vitreous forms around the seventh week of life and begins involuting. Pdf persistent hyperplastic primary vitreous phpv is a pathologic entity resulting from the abnormal persistence of the fetal fibrovascular. This rare ocular abnormality has already been described described in man, several dog breeds, transgenic mice, labor rats and cats. Persistent hyperplastic primary vitreous findings in an adult. Phpv pathology usually occurs unilaterally, but a rare bilateral condition has also been described goldberg, 1997. Typically, there is a retrolental fibrovascular mass 3 caused by persistence of the primary vitreous that normally should regress 4. Persistent hyperplastic primary vitreous an overview. Neogeninloss in neural crest cells results in persistent. Diseases of the vitreous are uncommon but, as in the case of persistent hyperplastic vitreous phpv described here, can have severe vision consequences. Pdf unilateral persistent hyperplastic primary vitreous. Persistent hyperplastic primary vitreous is a rare benign developmental disorder of the globe in which the embryonic hyaloid artery fails to regress normally, resulting in abnormal lenticular development and secondary changes of the retina and globe. Persistent hyperplastic tunica vasculosa lentis persistent hyperplastic primary vitreous phtvlphpv is a congenital eye anomaly, which in severe cases leads to cataract. Three cases of persistent hyperplastic primary vitreous phpv showing a ruptured lens capsule and a phacoanaphylactoid reaction are presented.
Persistent hyperplastic primary vitreous involving the. However, the doberman 1 and the staffordshire bull terrier 2 are exceptions to this. It is a rare abnormality in animals as well as in humans. A rare case of binocular persistent hyperplastic primary. Persistent hyperplastic primary vitreous hereditary. Persistent hyperplastic primary vitreous phpv is thought to be a congenital anomaly in which the normal regression of the primary vitreous body and hyaloid. We discuss nine cases of persistent hyperplastic primary vitreous seen recently, including mode of presentation, management and postsurgical followup. Phpv is unilateral in more than 80% of cases, and bilateral in about 20%. It contains the hyaloid vessels and fibrillar ectodermal tissues, then gradually disappears in the fifth to sixth month of gestation and is replaced by the secondary vitreous, which is the final vitreous. Persistent hyperplastic primary vitreous phpv is also referred to as persistent fetal vasculature pfv or persistent fetal vasculature syndrome pfvs, omim 618 autosomal dominant phpv or omim 6111 autosomal recessive phpv. Persistent fetal vasculature, previously known as persistent hyperplastic primary vitreous, is a spectrum of disease and can present with no clinical effects or with severe morbidity. This study confirms that retinal dysplasia and persistent hyperplastic primary vitreous is a congenital abnormality that is inherited as an autosomal recessive condition in miniature schnauzers.
Phacoanaphylactoid reaction in persistent hyperplastic primary. Posterior hyperplastic primary vitreous american journal of. Highfrequency ultrasonography findings in persistent hyperplastic primary vitreous. Persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a congenital anomaly in which the normal regression of the primary vitreous and hyaloid vasculature does not occur. We report a child with unilateral phpv who underwent cataract extraction. Get a printable copy pdf file of the complete article 15m, or click on a page. Persistent hyperplastic primary vitreous phpv, a developmental cause of leukocoria, is due to incomplete regression of the fetal blood supply to the eye. Persistent hyperplastic primary vitreous in transgenic mice. Persistent hyperplastic primary vitreous wikipedia. Persistent hyperplastic primary vitreous phpv request pdf. Emphasis is placed on early operative intervention in order to preserve the globe, as well as to prevent irremedial stimulus deprivation amblyopia.
Persistent hyperplastic primary vitreous is a congenital condition characterized by retrolenticular fibrovascular tissue formation and potential retention of the hyaloid vasculature. Persistent hyperplastic primary vitreous springerlink. Persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a congenital anomaly of the eye that results from failure of the embryological, primary vitreous, and the hyaloid vasculature to regress. Here we report a case of nonsurgical unilateral anterior phpv that was managed by amblyopia treatment and resulted in an improvement of visual acuity and regression of the fetal vasculature. Persistent hyperplastic primary vitreous phpv is a rare congenital developmental ocular disorder caused by incomplete regression of the embryonic hyaloid vasculature. Inherited retinal dysplasia and persistent hyperplastic. Visual outcomes following lensectomy and vitrectomy for. How to treat persistent fetal vasculature successful treatment requires meticulous followup by pediatric ophthalmologists, and retina and contact lens specialists. It typically presents unilaterally, without associated. Infants, eyes and orbits persistent hyperplastic primary vitreous phpv is a pathologic entity resulting from abnormal hypertrophy of the fetal fibrovascular primitive stroma hyaloid system of the eye 1. Bilateral persistent hyperplastic primary vitreous.
Persistent hyperplastic tunica vasculosa lentis and. Persistant hyperplastic primary vitreous phpv was described in detail as a clinical entity by reese1 in 1955 in his jackson memorial lecture. Feb, 2017 persistent hyperplastic tunica vasculosa lentis phtvl and persistent hyperplastic primary vitreous phpv are congenital eye anomalies that results from hyperplastic development of the hyaloid system and primitve vitreous combinened with incomplete regression. Persistent fetal vasculature pfv, formerly known as persistent hyperplastic primary vitreous phpv, is a congenital anomaly, usually unilateral and occurring in the fullterm infant. Extended report outcomes in persistent hyperplastic. Often it is only one of a group of ocular anomalies which appear to result from a posterior form of persistent or hy perplastic primary vitreous. Persistent hyperplastic primary vitreous phpv, nowadays referred to as persistent fetal vasculature pfv, is a rare congenital anomaly of. Persistent hyperplastic primary vitreous phpv emconsulte. Extended report outcomes in persistent hyperplastic primary. We present the clinical and imaging findings of this unusual entity and discuss the therapeutic options available for its management. Outcomes in persistent hyperplastic primary vitreous.
Magnetic resonance imaging and clinical findings minghui sun, md. Pdf persistent hyperplastic primary vitreous the martini. This study presents a very rare case of a patient with three consecutive pregnancies, in which abortion was induced twice due to a finding of binocular persistent hyperplastic primary vitreous. While the great majority of patients with persistent hyperplastic primary vitreous never obtain useful vision, it is encouraging that 18. Pdf highfrequency ultrasonography findings in persistent. Persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a rare congenital developmental malformation of the eye, caused by the failure of regression of the primary vitreous.
Persistent hyperplastic primary vitreous phpv must be considered in the differential diagnosis of leukocoria. Petra meier, peter wiedemann, in retina fifth edition, 20. Persistent hyperplastic primary vitreous phpv, a potentially blinding childhood disease, is one of the most common congenital malformation syndromes of the eye. Persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a congenital anomaly of the eye that results from failure of the. Retrospective case series of 55 eyes in 50 patients presenting between 1990 and 2001 at the childrens hospital, westmead. Persistent hyperplastic primary vitreous phpv is a congenital anomaly that usually debuts during the first weeks after birth. In this variant of persistent hyperplastic primary vitreous, retrolental mass was absent and use of color doppler was diagnostic. Persistent hyperplastic primary vitreous of the eye. Abstract persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a rare congenital developmental. Persistent hyperplastic primary vitreous phpv, also termed persistent fetal vasculature, is a developmental malformation of the eye in which the primary vitreous fails to regress in utero, resulting in the presence of a retrolental fibrovascular membrane with persistence of the posterior portion of the tunica vasculosa lentis and hyaloid artery. Congenital, due to incomplete regression of fetal vasculature of vitreous humor, producing a retrolental mass usually unilateral associated with small eye, white reflex behind pupil leukokoria, fibrous tissue behind lens, often cataract. The incidence of phtvlphpv in doberman and the results of. Prenatal ultrasonographic diagnosis of persistent hyperplastic primary vitreous e. The posterior chamber of the eye contains the retina the tissue that responds to light and is filled with a transparent jellylike substance called the vitreous humor.
Pdf outcomes in persistent hyperplastic primary vitreous. Children with phpv are born with a hazy, scarred vitreous. Achiron the ultrasound unit, department of obstetrics and gynecology and the goldschleger eye institute, the chaim sheba medical center. Persistent hyperplastic primary vitreous phpv, also known as the persistent fetal vasculature, refers to a rare congenital developmental malformation of the eye. The primary vitreous lies between the lens and the retina. Persistent fetal vasculature pfv, also known as persistent hyperplastic primary vitreous phpv is a constellation of ophthalmic findings typically consisting of microphthalmia, cataract figs. Murad radiology department, aga khan university hospital, karachi. Persistent hyperplastic primary vitreous radiology. Persistent hyperplastic tunica vasculosa lentispersistent hyperplastic primary vitreous phtvlphpv is a congenital eye anomaly, which in severe cases leads to cataract. What is persistent hyperplastic primary vitreous phpv. Regression of fetal vasculature and visual improvement in. This study presents magnetic resonance imaging mri findings of both the anterior and posterior types of persistent hyperplastic primary vitreous. Discussion persistent hyperplastic primary vitreous was first described by reese in 1955 in his jackson memorial lecture.
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