Permanent primitive vitreous hyperplasia is caused by the failure of the primitive vitreous to recede normally during the embryonic period. It is detected by parents as a result of the white pupil that can be present after birth. In addition to the white pupil, there is a small eye, small cornea, shallow anterior chamber, and small lens. The grayish-white membranous tissue covers the posterior capsule of the lens, with a thicker central portion and occasional vitreous artery residue. The ciliary process can also be seen around the lens. Once the posterior lens capsule is ruptured, the lens cortex becomes swollen and cloudy, blocking the atrial aqueous pathway and causing glaucoma. The lens cortex is absorbed and replaced by a fibrous membrane (membranous pseudophakic lens). If the fundus is still visible, intravitreal mechanized cords are often detected; traction folds of the anterior optic papilla and its marginal retina. Diagnosis 1. Full-term newborns with white pupils already seen after birth. It is more common in males. 2, monocular onset, mostly with microphthalmia, the other eye is normal. 3, Clinical examination. Visual acuity is reduced, the pupil area is grayish, the lens is transparent but small and flat, and a white membranous substance is visible behind the lens, thicker in the center and thinner at the periphery, often accompanied by neovascularization. A long ciliary process can be seen after pupil dilatation. 4. Proliferating fibrous membrane traction can lead to retinal detachment, and in severe cases, corneal clouding, secondary glaucoma, vitreous hemorrhage, and ocular atrophy. Identification of white pupil 1, retinoblastoma: ultrasound, MRI 2, retinopathy of prematurity: prematurity, oxygenation, history of low weight, fundus examination. 3, congenital cataract: ultrasound The above three are without microphthalmia, and the axial length of the eye is in the normal range by ultrasound. Pathogenesis The primordial vitreous is the common product of surface ectoderm, neuroectoderm and mesoderm As the vitreous artery grows into the optic cup a capillary component is added to the primordial vitreous. After the 6th week of embryonic life, the lens capsule forms and the relationship between the vitreous and the lens is interrupted. The development of the primordial vitreous terminates in perpetual primordial vitreous hyperplasia, mainly due to the abnormal persistence and excessive growth of the primordial vitreous and the persistent proliferation of intrinsic vessels and branching vessels from the ciliary vessels within the vitreous. The ciliary process is associated with the peripheral portion of the membranous tissue and is pulled centrally as the eye develops, resulting in the formation of a dense posterior lens fibrovascular membrane mass within the secondary vitreous. This can cause an immune response and granulation due to the tension and cellular proliferation of the proliferating membrane, which can rupture the posterior lens capsule in almost every case of PHPV. The proliferative material grows into the lens causing secondary cataracts, resulting in total clouding of the lens. The crystal gradually expands until the anterior chamber becomes shallow and even abuts the cornea, causing secondary corneal changes, edema, clouding, and corneal banding. The blood vessels grow through the rupture, and hemorrhagic mechanized masses are seen in the crystal, with subsequent thickening of the mechanized membrane. The lens may also gradually resorb until only a layer of mechanized membrane remains. Spontaneous hemorrhage from the posterior fibrovascular membrane mass may enter the membrane tissue, vitreous and lens, and the fibrovascular tissue and vessels may extend into the defect of the posterior capsule. The peripheral portion of the retina may be continuous with the fibrovascular membrane mass. The pathogenesis of closed-angle glaucoma includes the expansion of the lens or contraction of the posterior fibrous membrane due to rupture of the posterior lens capsule, resulting in anterior displacement of the lens-iris septum, pupil atresia, etc. This causes pupillary block, closure of the atrial angle and impaired atrial aqueous drainage, and increased intraocular pressure. The pathogenesis of open-angle glaucoma includes chronic uveitis and persistent intraocular hemorrhage from the vessels of the fibrovascular membrane. After extensive histological studies, 56% of permanent primary vitreous hyperplasia with immature atrial angle development were found to be associated with the development of glaucoma as well. Clinical manifestations The eye is mostly smaller than normal, with 90% of cases being monocular, seen in infants and children showing partial or total white pupil, shallow anterior chamber, and white membranous tissue visible behind the lens, sometimes containing blood vessels within the membrane tissue. A dilated pupil with an elongated ciliary process around the lens is a characteristic feature of this condition. In some eyes, the posterior lens capsule ruptures and swells, leading to secondary angle-closure glaucoma. Rupture of the anterior lens capsule and, uncommonly, iris angiogenesis may also be seen. Complications of this condition include intraocular hemorrhage secondary to glaucoma and corneal clouding. High intraocular pressure can cause the corneoscleral rim of the infant’s eye to swell and enlarge, eventually resulting in a “bull’s eye. Complications Spontaneous hemorrhage can occur within the retina, vitreous and lens when fibrous masses adhere to and pull on the retina, and can result in retinal detachment. Other less common complications include rupture of the anterior lens capsule, iris vascular proliferation, lens ectasia, and iris, choroidal, and optic nerve defects. PHPV is often associated with congenital anomalies such as strabismus, nystagmus, and microphthalmia. Examination Imaging tests such as ultrasound, CT, and MRI are performed to help clarify the diagnosis; IOP measurement: performed at the onset of glaucoma symptoms. Treatment Treatment for this condition includes lens removal posterior lens fovectomy vitrectomy. Early surgery is expected to yield better visual and cosmetic results Early lens removal and peripheral iridotomy, and often vitrectomy, are performed in patients with complicated closed-angle glaucoma. Due to the small size of this eye great care should be taken when making the incision to avoid intraocular comorbidities.