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Persistent hyperplastic primary vitreous is a disease whose spectrum has continued to widen throughout the years; in fact, it probably comprises a number of nosological entities considered as isolated symptoms until today. Our study has shown that the disease can be congenital and affect both eyes in the same individual. The possibility of an etiopathogenic connection with retrolental fibroplasia cannot be excluded.
PURPOSE: Persistent hyperplastic primary vitreous (PHPV) is a rare developmental malformation of the eye. This anomaly is usually unilateral and unassociated with other disease. PHPV may have clinical variations: an anterior and/or a posterior one. Diagnosis and treatment are exposed in our paper. METHODS: We study 38 eyes in 34 patients. All patients had an anterior form of PHPV. Nine of them associated a posterior form. Eighteen eyes underwent surgery, the mean postoperative follow-up is 4.7 years. Treatment is discussed, depending on the initial form of PHPV. We report the evolution of the surgical eyes and the untreated eyes. RESULTS: Slit lamp examination and echographic findings (A-scan and B-scan) support the diagnosis. In some cases computed tomography or RMN can be useful. If the cataract is mild in the anterior form of PHPV, treatment of amblyopia and frequent clinical examination can be sufficient. If the cataract is dense, a lensectomy must to be performed. The surgical technique can be difficult. For the eyes with posterior PHPV, the surgical treatment is to be avoided, because of high risk of retinal detachment. CONCLUSION: PHPV may have clinical variations, mostly with cataract as major sign. In purely anterior presentation, in absence of marked microphthalmos, lensectomy can be useful.
This study examines the results of research on the surgical treatment of children with various vitreous diseases. Vitrectomy was performed on 44 eyes in children, from four months to fifteen years of age, suffering from the following diseases: lensectomy complications as vitreocorneal adhesions or immersion of lens mass into the vitreous; persistent hyperplastic primary vitreous (PHPV), trauma consequences, uveitis and eyeball subatrophy. Preoperational examination included measurement of visual acuity, biomicroscopy, direct and indirect ophthalmoscopy, tonography, ultrasonography and electrophysiological examinations. Vitreotome 'ocutome' was used to perform the operations. A transcyliar approach was used in all but 14 cases, for which the operations were performed by limbal incision. Improvement of visual functions was noted in 28 eyes (63.3%), and relatively good visual function was achieved in 7 eyes (16.9%). Visual acuity remained unchanged in 6 eyes (13.6%) because of severe amblyopia, although the transparency of optic zone was restored and the fundus did become visible. IN 10 (22.7%) of the children visual acuity could not be measured because of too young an age. One day after surgery a cellular suspension in the vitreous was observed in many eyes. This however, resolved within 3 days. Intraocular pressure usually returned during the first week after surgery. Hypotonia remained for 1-2 months after subtotal vitrectomy. The transcyliar extraction of pathological vitreous in children is generally a less traumatic and more convenient means of intravitreal manipulation, but 2 cases of intraoperational hemophthalmia suggested that iridiocyclitis is a contraindication for transcyliar approach. Transcorneal extraction is a less traumatic surgical method in such cases.
Pars plana vitrectomy requiring a two-instrument technique successfully cleared the pupillary axis of dense persistent hyperplastic primary vitreous in a 29-year-old patient. To find PHPV in an adult eye with vision is so unusual that distinguishing it from the ocular sequelae of traumatic injury can be challenging. Hyperplastic primary vitreous persists with a wide variety of clinical features in both anterior and posterior segments of the eye.
BACKGROUND: The pathogenesis of open-angle glaucoma and ocular hypertension in patients who have undergone surgical correction of their congenital cataracts remains undetermined. This study examines the prevalence of glaucoma and ocular hypertension in a population of patients who did not undergo surgical correction of their pediatric cataracts. METHODS: Fifty-eight eyes of 41 patients had cataracts before 2.5 years of age and were followed up until at least 5 years of age without operative correction. The patients were studied for the following parameters: age at diagnosis, type of cataract, etiology, bilaterality, optic nerve head cup-to-disc ratio, intraocular pressures, and reason why the patient did not undergo an operation. Glaucoma was defined as the presence of glaucomatous optic nerve head cupping with intraocular pressures of greater than 22 mm Hg. Ocular hypertension was defined as intraocular pressures greater than 22 mm Hg with no optic nerve changes. RESULTS: Nine of the 58 eyes had cataracts caused by persistent hyperplastic primary vitreous. The average age to the last intraocular pressure measurement was 19 years (range 5 to 48 years). Closed-angle glaucoma developed in two patients with persistent hyperplastic primary vitreous. Neither open-angle glaucoma nor ocular hypertension developed in any patients. CONCLUSION: Pediatric cataracts not of the persistent hyperplastic primary vitreous type were not associated with ocular hypertension or glaucoma in the absence of surgical cataract correction. In eyes with persistent hyperplastic primary vitreous cataracts, spontaneous closed-angle glaucoma developed in two of nine patients and open-angle glaucoma developed in none. Surgical cataract correction, or the aphakic state that follows such operations, may be responsible for pediatric aphakic glaucoma.
From 1985 till 1990 twenty-three children aged seven weeks to six years presented with a retrolental white mass. Six children had retinoblastoma, six Coats' disease, five retinopathy of prematurity, four persistent hyperplastic primary vitreous, one cysticercosis and one retinal detachment. Retinoblastoma and Coats' disease was only seen in children over eight months, whereas persistent hyperplastic primary vitreous and retinopathy of prematurity all presented before the age of six months. Differentiation between Coats' disease and retinoblastoma is not always easy. Most helpful for the diagnosis is to perform funduscopy under general anaesthesia with special attention to the vasculature. Ultrasounds are helpful, but can give confusing results. Cytological analysis of subretinal fluid is useful in Coats' disease.
The clinical study done on 19 cases with posterior primitive vitreous persistency and hyperplasia showed the predilection affectation of youth (average age: 11.2 year old), the frequent lateral localization (17 cases), microcornea (7 eyes), hypermetropia (average value + 3D). The visual acuity was between 1 and unregistered device values, being a function of the papillar ant retinal lesion extension. The ophthalmoscopical aspect was balanced, from a simple prepapillar veil to complex ophthalmological syndromes. Etiopathogenetically, the affection is considered to be a embryogenesis flaw, appeared in the development of the primary hyaloid-vitreous complex, described by an incomplete resorption and a hyperplasia of its elements. The problems of differential diagnosis and treatment are extensively presented.
Two cases of persistent hyperplastic primary vitreous have been managed by the vitreous cutter Sukon "Alternating" with a pars-plana approach.
A key tumor suppressor mechanism that is disrupted frequently in human cancer involves the ARF and p53 genes. In mouse fibroblasts, the Arf gene product responds to abnormal mitogenic signals to activate p53 and trigger either cell cycle arrest or apoptosis. Recent evidence indicates that Arf also has p53-independent functions that may contribute to its tumor suppressor activity. Using Arf(-/-) and p53(-/-) mice, we have discovered a p53-independent requirement for Arf in the developmental regression of the hyaloid vascular system (HVS) in the mouse eye. Arf is expressed in the vitreous of the eye and is induced before HVS regression in the first postnatal week. In the absence of Arf, failed HVS regression causes a pathological process that resembles persistent hyperplastic primary vitreous, a developmental human eye disease thought to have a genetic basis. These findings demonstrate an essential and unexpected role for Arf during mouse eye development, provide insights into the potential genetic basis for persistent hyperplastic primary vitreous, and indicate that Arf regulates vascular regression in a p53-independent manner. The latter finding raises the possibility that Arf may function as a tumor suppressor at least in part by regulating tumor angiogenesis.
We describe a newborn with congenital cystic eye, contralateral persistent hyperplastic primary vitreous, and cerebrocutaneous abnormalities. The cerebrocutaneous abnormalities consisted of agenesis of the corpus callosum, midbrain deformity, malformed sphenoid bone, right upper eyelid coloboma, and a left periocular hamartoma. The results of karyotype analysis of the patient and his parents were normal. The association of congenital cystic eye with contralateral persistent hyperplastic primary vitreous has not been previously reported, to our knowledge. Although no unifying diagnosis exists for the collection of anomalies demonstrated in this patient, the term cranial ectodermopathy broadly classifies most of the defects.
Oculo-palato-cerebral syndrome is an extremely rare disorder consisting of low birth weight, microcephaly, short stature, persistent hyperplastic primary vitreous, microphthalmia, large ears, small hands and feet, cleft palate, joint hypermobility, developmental delay, and cerebral atrophy. There has been one report of a consanguineous family with three affected children, suggesting autosomal recessive inheritance. We report on the second case of this disorder. Our patient, a 2-year-old boy, had growth delay, microcephaly, bilateral persistent hyperplastic primary vitreous with right microphthalmia, long ears with thickened helices, small hands and feet, highly arched palate, joint hypermobility, hypoplastic nails, frontal cerebral atrophy and thinning of the corpus callosum on brain magnetic resonance imaging, and mild developmental delay. He has much milder features than those seen in the previously reported cases.
The occurrence of retinoblastoma in an eye with persistent hyperplastic primary vitreous (PHPV) is reported. These are both rare lesions. The absence of previous reports of their association has led to the clinical impression that the occurrence of PHPV in a microphthalmic eye precludes the presence of retinoblastoma. The coexistence of these lesions in the present case may represent a coincidence. Their occurrence in the same eye is felt to be noteworthy, nontheless, because of its clinical implications and the possibility of a common underlying etiology.
A 4-month old child with findings of persistent hyperplastic primary vitreous was also noted to have an ocular communicating orbital cyst in the same eye. The ocular component of the cyst was observed to move.
Ultrastructural study of two cases of persistent hyperplastic primary vitreous. Behind posterior lens capsule we find a connective tissue where fibroblasts present an intense activity. Numerous vessels and neuro-ectodermal tissue were noted.
The authors describe the case of two brothers afflicted with persistent anterior hyperplasic primary vitreous and hyaloid artery. The occurrence of combination in two members of the same family is unusual, also moreover one of the patients was affected by macular retinoschisis. The two cases were clinically different; the first was typical, complicated by cataract; the second presented alterations mostly located in the posterior vitreous. In one eye fluorescein angiography showed a very slow and delayed filling of the hyaloid artery. Blood flow was directed from its anterior portion towards the optic disk. Nd: YAG laser photoresection of the hyaloid artery, performed in order to obtain a better visualization of the macula did not lead to remarkable hemorrhages. In an other eye fluorescein angiography could be performed after Nd: YAG laser photoresection of a vitreous membrane located in front of the posterior pole: remnants of the hyaloid vascular system with retrograde blood filling were present also in this case. The mechanisms leading to this reverse filling of the vessels are discussed.
PURPOSE: To evaluate Color Doppler Imaging (CDI) in the value of Persistent Hyperplastic Primary Vitreous(PHPV). METHODS: 3 cases (4 eyes) of PHPV were reproted and were examinated by high resolution CDI, the blood flow pattern was evaluated in basic of two dimensional grayscale image. RESULTS: CDI shows a band vascular structure coursing through the band-shaped moderately echogenic structure in the central portion of vitreous chamber, with the apex located near the optic disk and the base extending toward the lens. Spectral display of the Doppler signal indicated arterial flow. CONCLUSION: Central vessel exhibiting arterial flow is compatible with a prominent persistent hyaloid vascular structure. This CDI imaging feature, in addition to microphthalmia and lack of calcification, is virtually pathogenomonic of PHPV and aids in enabling distinction between PHPV and simulating lesions.
Observations on (postnatal) persistent hyperplastic tunica vasculosa lentis/persistent hyperplastic primary vitreous (PHTVL/PHPV) in man and dog have been published previously. Up to the present, no evidence on the etiology of this entity was available. The hereditary occurrence of the disease in the Dobermann pinscher dog and the similarity of ocular development in mammals has provided a useful model in providing ontogenetic data. The present study deals with the early morphogenesis of PHTVL/PHPV, from day 25 to 44 post-coitum (D25-D44), in genetically affected dog fetuses. Normal beagle dog fetuses served as reference material, which has been described separately. At D30, the hyaloid system, including the tunica vasculosa lentis posterior, had developed further than in the reference fetuses. From that stage onward, a retrolental fibrovascular membrane developed. In some of the eyes of D37, posterior polar subcapsular cataracts and preretinal glial proliferations were observed. Capsular anomalies and distortions of the lens shape as seen in clinical PHTVL/PHPV were not observed, and are believed to be secondary entities. Extrapolation of some of the obtained data from dog to man is possible by the use of comparable gestational time scales. The anterior form of (PHTVL/PHPV) in man probably develops its main features in the period of approximately 43 to 66 days of pregnancy. Recently, anti-angiogenetic properties of normal vitreous have been described. This, and the fact that overdevelopment and subsequent incomplete regression of the hyaloid system plays a major role in the pathogenesis of PHTVL/PHPV, gives rise to the hypothesis that a changed amount or effectiveness of such (humoral) factors is an important factor in the etiology of this disease.
To elucidate the underlying developmental mechanisms of persistent hyperplastic primary vitreous (PHPV) in humans, we investigated a mouse model for PHPV induced by retinoic acid. We treated C57BL/6NJcl mice at various stages of pregnancy (gestation days 7, 8, 9, 10, 11, or 12) with the teratogen retinoic acid, which affects the migration of neural crest cells. Untreated pregnant mice served as a control group. The eyes of the fetuses were examined histologically on day 18 on gestation. Developmental abnormalities of the vitreous were defined as the presence of excessive mesenchymal tissue in the vitreous cavity. The incidence of developmental abnormalities of the vitreous in all groups, except for those treated on day 12 of pregnancy, significantly exceeded that in the control group (P<0.01). The histological characteristics of the observed vitreous abnormalities in mice resembled those found in PHPV clinically. Retinoic acid-induced abnormalities in mice can serve as an experimental model for PHPV by environmental factors. Results suggest that the critical period for these retinoic acid-induced abnormalities was during days 7 to 11 of gestation, which corresponds to a critical period of 2.5 to 7 weeks of gestation for PHPV in humans.