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An Approach to Visual Loss in a Child
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
Muhammad Hassaan Ali, Stacy L. Pineles
Another congenital anomaly in which the primary vitreous persists and extends from the optic disc to the vitreous base at the posterior pole of the lens is called persistent hyperplastic primary vitreous (PHPV) or more recently persistent fetal vasculature (PFV) (35). It may be unilateral or bilateral and is seldom associated with microphthalmos. Early vitrectomy can be attempted in such cases; however, the visual outcomes are suboptimal (36).
Ophthalmology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Ocular associations include glaucoma, cataract, Axenfeld–Rieger syndrome, aniridia, microphthalmia, persistent hyperplastic primary vitreous (PHPV) and retinal dysplasia. Systemic abnormalities include craniofacial anomalies, central nervous system abnormalities, fetal alcohol syndrome, chromosomal abnormalities andPeters plus syndrome (a rare autosomal recessive disorder comprising short-limbed dwarfism, cleft lip and/or palate, brachydactyly and learning difficulties).
Vitreoretinal Surgery in Rare Conditions
Published in Pradeep Venkatesh, Handbook of Vitreoretinal Surgery, 2023
Intraocular fetal vasculature is a complex network of blood vessels that supply and nourish the developing anterior, intermediate, and posterior regions of the eye during intrauterine growth. Components of the fetal vasculature include the hyaloid artery, tunica vasculosa lentis, and a pupillary network [iridohyaloid and capsulopupillary artery]. These vessels develop by the process of vasculogenesis and, in the large majority of cases, undergo spontaneous dissolution at specific time intervals, long before the newborn is delivered. In some, however, their remnants could be discernible as Mittendroff’s dot and Bergmeister’s papillae on the posterior capsule of the lens and optic nerve head, respectively. These are detected incidentally, minor in nature, and do not lead to any pathological sequelae. In rare instances, the fetal vasculature fails to dissolute and leads to major sequelae, jeopardizing postnatal development of the eyeball as well as VA. This condition is referred to as persistent fetal vasculature [PFA]. In the past, a commonly used terminology for this was persistent hyperplastic primary vitreous (PHPV). In almost 90% of infants, the condition is unilateral and may be associated with persistent pupillary membrane, congenital cataract, macular ectopia, retinal drag, retinal fold, vitreous opacification, coloboma, optic disc hypoplasia, retinal detachment, and retinal disorganization. Some genetic anomalies [trisomies 13, 15, and 18] and systemic associations [neurofibromatosis] may be present in bilateral cases but not in typical unilateral PHPV. In bilateral cases, a rare variant called MPPC syndrome [microcornea, posterior megalolenticonus, PFA, and coloboma] must be considered. In this syndrome, the posterior lenticonus is sometimes said to be so large as to occupy the majority of the vitreous cavity. The usual presenting features are facial asymmetry [orbital], leukocoria, and microphthalmos. Important differential diagnoses to consider are ROP and FEVR. PFV has been classified into anterior, posterior, and combined forms, and recognizing this is important because it has a bearing on the long-term prognosis and in making management decisions. Each of these anatomical types has been further subdivided based on severity into mild and severe. Anterior PFV is characterized by an isolated dense retrolental plaque [cataract may or may not be present] and is termed mild when there is no involvement of the ciliary process and severe if there is. In posterior PFV, there is a stalk of hyaloid artery remnant running anteroposteriorly within the vitreous cavity and no attachment to the crystalline lens. It is mild when optic nerve traction and macular distortion are negligible and severe when it is significant. Combined anterior–posterior has features of both types and could again be either mild or severe. In some children, spontaneous vitreous haemorrhage and complex retinal detachment may be the presenting features.
A de novo mutation in PITX2 underlies a unique form of Axenfeld-Rieger syndrome with corneal neovascularization and extensive proliferative vitreoretinopathy
Published in Ophthalmic Genetics, 2020
Stephanie N. Kletke, Ajoy Vincent, Jason T. Maynes, Uri Elbaz, Kamiar Mireskandari, Wai-Ching Lam, Asim Ali
The role of PITX2 in retinal development is not fully elucidated and the association of ARS with bilateral progressive proliferative vitreoretinopathy has not been reported. Pitx2 knockout zebrafish have disorganized, irregular branching and patterning of the hyaloid vessels and small retinas (26). A case of persistent hyperplastic primary vitreous (PHPV) has also been reported in a patient with Peters anomaly caused by a c.649 C > A mutation in PITX2 (27). There is evidence that PITX2 drives the differentiation of the periocular mesenchyme (POM), a specific neural crest cell population that plays a role in optic stalk differentiation, embryonic fissure closure and vascular angiogenesis (28). Pitx2 expression has been demonstrated in the mouse POM at embryonic stage E10.5 and in the mesenchyme around the retina and cornea at E15.5 (29). PITX2 also regulates neural crest-derived pericytes, which stabilize ocular blood vessels during development (30). PITX2 downstream effectors may similarly play a role in normal retinal angiogenesis; however, the mechanism by which this leads to proliferative vitreoretinopathy is unclear. It is notable that proliferative vitreoretinopathy was observed in the left eye at 1 year prior to any posterior segment surgery. In comparison, it may be argued that retinal surgical procedures in the right eye contributed to the progression of the vitreoretinopathy to some extent.
The Demography and Etiology of Pediatric Enucleation in a Tertiary Eye Center in North China, 2001–2015
Published in Ophthalmic Epidemiology, 2019
Jing Zhang, Lei Wan, Yunhai Dai
Information tabulated from the records included each patient’s demographic characteristics, primary clinical diagnosis, surgical procedures, hospital charges, and duration of hospitalization. The patient data also included mechanism of injury, cause of injury, and other disease or infection. The primary clinical diagnoses were divided into four main categories: trauma, retinoblastoma (RB), congenital anomalies, and other diseases. Congenital anomalies included congenital glaucoma, congenital cataract, congenital microphthalmia, congenital corneal opacity, familial exudative vitreoretinopathy (FEV), and persistent hyperplastic primary vitreous (PHPV).
Anterior Segment Optical Coherence Tomography in Pediatric Ocular Pathology: Imaging Study of 115 eyes
Published in Expert Review of Medical Devices, 2023
A detailed assessment of the lens is possible by moving the cursor further to center the lens on the dashed lines. Any lens pathology, absence, precise cataract morphology and location, anterior and posterior capsules as well as posterior capsular presence or absence or lenticonus, intraocular lens position, and even Elschnig’s pearls, anterior persistent hyperplastic primary vitreous (PHPV) could be studied. IOL placement or tilt was also assessed.