Persistent Hyaloid Artery Research Articles

Optical coherence tomography findings in persistent hyaloid artery: A prospective study

Aims/Purpose: To describe the features of peripapillary persistent hyaloid artery and its effects on disc parameters using Swept‐source optical coherence tomography (SS‐OCT) and SS‐OCT angiography (SS‐OCT A).Methods: Seven eyes of 7 patients diagnosed with Persistent hyaloid artery were prospectively reviewed at the department of Ophthalmology in the Habib Thameur Hospital, Tunisia, between January 2024 and June 2024. All patients were assessed for peripapillary retinal nerve fibers layer (pRNFL) thickness by SS‐OCT, and blood flow by papillary OCT‐A, 4.5 mm*4.5 mm cubes.Results: Mean age was 36.33 years, with 4 women and 3 men. SS‐OCT showed peripapillary hyperreflective tubular material of the persistent hyaloid artery in the right eye of 3 patients and in the left eye of 4 patients. On analysis of the pRNFL thickness, the mean average thickness was 110.8, Inferior quadrant (150.8), superior quadrant (134), nasal quadrant (83.4) and temporal quadrant (74.8). pRNFL were thickened in one eye, altered in one eye in the superior quadrant, and preserved in five eyes. SS‐OCT A revealed no blood flow at the lesion in all eyes.Conclusions: A persistent hyaloid artery is an uncommon lesion whose diagnosis, evaluation and monitoring have become easily possible using SS‐OCT and SS‐OCT A. Evaluating the vascular flow at the level of the optic nerve head would be of great importance in order to prevent certain complications, notably vitreous hemorrhage and retinal detachment.References Jeon, H., Kim, J., & Kwon, S. OCT angiography of persistent hyaloid artery: a case report. BMC Ophthalmol. 2019 Jul 4; 19(1): 141. Gamidov AA, Durzhinskaya MH, Makashova NV, Sakalova ED, Velieva IA. Persistent hyaloid artery in an adult (a case report). Vestn Oftalmol. 2020; 136(4): 214.

Optical coherence tomography findings in persistent hyaloid artery: A prospective study

Optical coherence tomography findings in persistent hyaloid artery: A prospective study

Microcephaly with or without chorioretinopathy, lymphedema, or mental retardation (MCLMR)- the new lacunae: a case report

BackgroundMicrocephaly with or without chorioretinopathy, lymphedema, or mental retardation is a rare autosomal dominant disease caused by mutations in KIF11 which disrupt EG5 protein function, impacting the development and maintenance of retinal and lymphatic structures due to its expression in the retinal photoreceptor cilia. The primary ocular finding in MCLMR is chorioretinopathy. Additional features can include microphthalmia, angle-closure glaucoma, persistent hyperplastic primary vitreous, cataract, pseudo-coloboma, persistent hyaloid artery, and myopic or hypermetropic astigmatism. The appearance of the chorioretinal lesions as white to pinkish, round, non-elevated atrophic areas devoid of blood vessels resembles the lacunae in Aicardy syndrome. Due to the lack of systematic description of the lesions and significant phenotypical variability, there is an impending need for a detailed report of each case.Case presentationA child with microcephaly detected in the third trimester of gestation began her following in the ophthalmology department due to a non-visually significant cataract. Shortly after, she developed nystagmus and large-angle alternating esotropia with cross-fixation. Her fundus initially showed a pallid optic disc and pigmentary changes, developing thereafter retinal lacunae and a retinal fold. Her differential diagnosis accompanied the dynamic changes in her fundus, which included congenital infections, Leber´s Congenital Amaurosis and Aicardy syndrome. At 19 months old, genetic testing identified a heterozygous mutation (c.1159 C > T, p.Arg387*) in the KIF11 gene. The patient underwent bilateral medial rectus muscle recession surgery at 2 years old for persistent esotropia, with significant improvement. Refraction revealed a hyperopic astigmatism in both eyes (+ 0.25 -2.50 × 180 OD and + 0.75 -2.00 × 170 OS). She continues to require right eye patching for 2 hours daily.ConclusionsThis case report expands the phenotypic spectrum of MCLMR by demonstrating a unique combination of retinal features which sheds new light on differential diagnosis from Aicardy syndrome. Our findings emphasize the significant phenotypic variability associated with MCLMR, particularly regarding ocular involvement. This underscores the importance of detailed clinical evaluation and comprehensive reporting of cases to improve our understanding of the disease spectrum and genotype-phenotype correlations.

Bilateral persistent hyaloid artery

Bilateral persistent hyaloid artery

OCT angiography findings in posterior persistent fetal vasculature

Aims/Purpose: To report optical coherence tomography angiography findings in a patient presenting unilateral posterior persistent fetal vasculature (PVF).Methods: We report the case of a 19‐year‐old female patient with longstanding visual impairment of the left eye (LE). Patient was assessed with multimodal imaging including color fundus photography, swept source optical coherence tomography (SS‐OCT) and SS‐OCT angiography (SS‐OCTA) of the macula and the optic disc.Results: The patient had a history of unilateral visual impairment of unknown aetiology and consulted for a routine examination. Best corrected visual acuity was 10/10 in the right eye (RE) and limited to counting fingers in the LE. Ophthalmological examination of the LE revealed a mild exophoria, a quiet anterior segment, a vestigial capsular bag and an inferonasal whitish spot at the posterior lens capsule corresponding to Mittendorf's dot. Dilated fundus examination of the LE showed a lack of foveal reflex, retinal vessel contraction associated to the presence of a fibrovascular stalk extending from the OD to the crystalline lens via the vitreous humour, corresponding to fibrovascular contraction of the persistent and hyperplastic Cloquet's canal. No associated retinal detachment was noted. The clinical diagnosis of posterior PVF was confirmed. Macular SS‐OCT revealed retinal thinning with absence of foveal pit. Optic disc SS‐OCT highlighted a hyporeflective tubular structure overlying a Bergmeister'papilla. Cross‐sectional SS‐OCTA detected a flow signal at the level of Bergmeister's papilla, but no confirmed flow signal was identified at the level of the fibrovascular stalk.Conclusions: PVF is a rare condition characterized by fetal remnant from developmental vascular system defects, clinically recognized in its three forms (anterior, posterior and combined). OCT‐angiography is a useful non‐invasive tool to confirm of persistent hyaloid artery patency and assess risk of vascular complications. Contraction of the fibrovascular mass is prone to tractional retinal detachment and repeated episodes of intravitreal or intracapsular haemorrhage. Monitoring is therefore recommended even in adulthood.

Rare coexistence of Mittendorf dot with persistent hyaloid artery in a young Asian child

Rare coexistence of Mittendorf dot with persistent hyaloid artery in a young Asian child

Intraoperative OCT Angiography in Pediatric Patients with Persistent Fetal Vasculature

To describe abnormalities of the optic nerve microvasculature in patients with persistent fetal vasculature (PFV) and their fellow eyes using OCT angiography (OCTA). Cross-sectional study. Fifty eyes of 25 patients with PFV who underwent prospective imaging using supine OCTA during examination under anesthesia at Bascom Palmer Eye Institute from March 1, 2019, to December 31,2022. OCT angiography images of the optic nerve of the included patients were analyzed with a primary focus on blood flow. Demographic, clinical, and treatment factors were compared with morphologic changes in the optic disc microvasculature. Prevalence of optic nerve microvascular abnormalities on OCTA in the affected and fellow eyes of patients with PFV. A total of 50 eyes from 25 patients were reviewed, and 28% (7/25) met image quality criteria for OCTA analysis. Optic nerve OCTA showed a persistent hyaloid artery (PHA) in all (7/7) PFV eyes analyzed. Of these, flow on OCTA was detectable in 57% (4/7). A Bergmeister papilla was evident in 100% (25/25) fellow eyes, of which flow was detected in 68% (17/25). Fluorescein angiography (FA) demonstrated blood flow within the stalk in 40% (10/25) of PFV eyes and within the Bergmeister papilla in 25% (6/25) of fellow eyes. Similar findings of abnormal blood flow and presence of fibrovascular stalk were seen in both treatment-naïve and treated groups. OCT angiography allows for high-resolution visualization of subtle vascular abnormalities that are not readily apparent using RetCam FA and may serve as a useful noninvasive test to confirm the patency of the PHA and Bergmeister papilla in children. The results of the present study suggest that PFV may be a bilateral and asymmetric process. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Central retinal artery and vein occlusion as a complication of persistent hyaloid artery \u2013 a case report

BackgroundIn this case report, we present for the first time central retinal artery occlusion (CRAO) and central retinal vein occlusion (CRVO) as a complication of persistent hyaloid artery (PHA).Case presentationIn August 2019, a six-year-old male patient manifested right eye (RE) excessive tearing, conjunctival injection and pain. On examination, RE demonstrated light perception and intraocular pressure of 36 mmHg. The diagnoses of neovascular glaucoma, CRVO and CRAO were established as affirmed with fluorescein angiography (FA). PHA was not reported. Extensive work-up and family history were unremarkable. The child was born on term after uncomplicated twin pregnancy. In December 2019, he was referred to our Centre. Transillumination revealed fully dilated, non-reactive RE pupil, clear lens and tubular remnant of HA containing blood cells in its lumen freely rotating in the anterior vitreous.ConclusionsPHA results from failure of apoptosis during gestation. It can easily be observed during the red reflex screening at neonatal wards. We hypothesized that PHA twisting led to torsion of the residual primordial common bulb, branching off to HA and CRA with CRAO occurring first. The consequential CRVO presumably advanced by venous stasis due to decrease in arterial inflow. Liquid vitreous appears as early as 4 years of age enabling PHA to whirl more freely. Thus, in case of PHA, we advocate FA to be performed and if connection with retinal artery is proven, parents should be informed on the possible devastating complications and prompt surgical treatment should be considered.

Spontaneous Ocular Abnormalities in Sprague-Dawley Rats.

We collected historical control data derived from pretreatment ophthalmologic examinations of young (4 to 7 wk of age) Sprague-Dawley (Crl:CD[SD]) male, (2033, 42 lots) and female (1322, 32 lots) rats used in toxicity studies at our facility from 2004 through 2015. Ophthalmologic examination of male and female rats by using a binocular indirect ophthalmoscope and slit lamp revealed high incidences of corneal opacity (61% and 60%, respectively), lenticular opacity (43% and 47%), persistent hyaloid artery (21% and 17%), and retinal folds (27% and 27%). All other ocular abnormalities of the globe, conjunctiva, cornea, anterior chamber, lens, iris, vitreous, and choroid or retina occurred at incidences of less than 5%. Corneal opacities were localized mainly in the corneal nasal (38% and 37%) and paracentral (32% and 33%) areas, and lenticular opacities predominantly occurred in the nuclear area (31% and 34%). We then compared the incidences of spontaneous ocular abnormalities between the first (2004 through 2009) and second (2010 through 2015) 6-y periods. Corneal opacity and persistent hyaloid artery in male and female rats occurred more frequently during the second 6-y than during the first (corneal opacity, second period: male, 68%; female, 66%; corneal opacity, first period: 49% and 51%; persistent artery, second period, 26% and 23%; persistent artery, first period; 12% and 10%). These results support the importance of updating historical control data regularly and providing useful information for toxicologists and ophthalmologists to differentiate treatment-related changes from spontaneous lesions.

Posterior segment eye lesions: prevalence and associations with ocular and systemic parameters: results from the Gutenberg Health Study.

To estimate the prevalence of posterior segment eye lesions and to identify their ocular and systemic associations within the Gutenberg Health Study (GHS) in Germany. Assessment and grading of fundus images as well as physical examination and history taking were performed in the cross-sectional analysis of 15,010 subjects (aged 35-74years) using standardised procedures to determine the prevalence and associations of various posterior segment eye lesions. Fundus photographs of both eyes were available for 12,782 (85.2%; 50% female) subjects. The prevalence weighted to the region of Mainz and Mainz-Bingen in Germany was for choroidal nevi 2.4%, drusen of the optic nerve head 0.2%, tilted discs 1.5%, chorioretinal scars suggestive of toxoplasmosis 0.2%, retinitis pigmentosa 0.04% and persistent hyaloid artery 0.02%. Choroidal nevi were positively associated with a history of myocardial infarction (OR = 2.7, 95% confidence interval 1.2-6.2, p value = 0.017). Tilted discs were positively associated with increased intraocular pressure (OR = 1.09 per mm Hg (1.02-1.16), p = 0.011) and negatively associated with smoking (OR 0.4 (0.3-0.7), p = 0.0022). Participants with tilted discs had a mean spherical equivalent of - 3.6dioptres (standard deviation 4.0) compared with - 0.4dioptres (2.4) to those without. Our study is-to the best of our knowledge-the first to determine the prevalence of drusen of optic nerve head among Caucasians, to show a positive association between tilted discs and increased intraocular pressure and questions a possible link between choroidal nevi and myocardial infarction. It also showed that participants with tilted discs had a lower mean spherical equivalent than those without.

OCT angiography of persistent hyaloid artery: a case report

BackgroundA persistent hyaloid artery is a rare fetal remnant. Several complications such as amblyopia, vitreous hemorrhage, and retinal detachment have been reported. Here, we present a case of vitreous hemorrhage with a persistent hyaloid artery.Case presentationA healthy 16-year-old male presented with blurred vision in his left eye. Vitreous hemorrhage occurred and absorbed spontaneously. Slit-lamp examination demonstrated a Mittendorf’s dot and fundus examination revealed a persistent hyaloid artery. Optical coherence tomography (OCT) showed a Bergmeister’s papilla. The blood flow of the persistent hyaloid artery via the Bergmeister’s papilla was found by OCT angiography.ConclusionThe persistent hyaloid artery should be considered as a cause of spontaneous vitreous hemorrhage of young healthy patient. The OCT angiography will be a useful noninvasive approach to confirm the patency of the persistent hyaloid artery.

Persistent hyaloid artery with posterior polar cataract in a young male: A rare presentation

Persistent fetal vasculature (PFV) is a rare congenital developmental malformation of the eye which results from a failure of the fetal hyaloid artery to involute. PFV is usually characterized by microphthalmia, a shallow anterior chamber, elongated ciliary processes, a posterior subcapsular cataract, and a fibrovascular stalk that extends from the optic disc to the lens. It is a rare and sporadic disease with an overall prevalence of 0.064%. We report a case of a young male who presented with decreased vision in the right eye. On examination, it was diagnosed with complete persistent hyaloid artery. The patient was advised regular follow-up.

Idiopathic pigmented vitreous cyst without autofluorescence: a case report

BackgroundVitreous cysts are rare clinical findings and seldom cause visual disturbance. They are generally classified as congenital or acquired and are considered idiopathic when the etiology can not be determined. A previous electron microscopic observation on an idiopathic pigmented vitreous cyst has confirmed its pigment epithelial origin. However, the specific kind of pigment epithelium involved remains unclear.Case presentationA 39-year-old female presented with a round-shaped floater causing frequent visual disturbance in the left eye. A pigmented, non lobulated and freely mobile vitreous cyst was observed in the anterior vitreous by slit lamp examination and anterior segment optical coherence tomography. The pigment clumps on the cyst wall showed no autofluorescence. No persistent hyaloid artery or connection between the cyst and ocular structures was found by fundus fluorescein angiography and B-scan ultrasound. Serum tests for cysticercoids, sparganosis and toxoplasma were negative. A diagnosis of idiopathic vitreous cyst was made and no intervention was given. The cyst sank to the inferior part of the vitreous and the patient felt less visual disturbance during one-year follow-up.ConclusionsWe described the features of a pigmented vitreous cyst revealed by autofluorescence and anterior segment optical coherence tomography for the first time. The intact retina, the absence of lipofuscin of the cyst and its location in the anterior vitreous led to the hypothesis that the cyst may originate from the ciliary pigment epithelium rather than the retinal pigment epithelium.

Severe ocular phenotypes in Rbp4-deficient mice in the C57BL/6 genetic background

Retinol-binding protein 4 (RBP4) is a specific carrier for retinol in the blood. In hepatocytes, newly synthesized RBP4 associates with retinol and transthyretin and is secreted into the blood. The ternary transthyretin–RBP4–retinol complex transports retinol in the circulation and delivers it to target tissues. Rbp4-deficient mice in a mixed genetic background (129xC57BL/6J) have decreased sensitivity to light in the b-wave amplitude on electroretinogram. Sensitivity progressively improves and approaches that of wild-type mice at 24 weeks of age. In the present study, we produced Rbp4-deficient mice in the C57BL/6 genetic background. These mice displayed more severe phenotypes. They had decreased a- and b-wave amplitudes on electroretinograms. In accordance with these abnormalities, we found structural changes in these mice, such as loss of the peripheral choroid and photoreceptor layer in the peripheral retinas. In the central retinas, the distance between the inner limiting membrane and the outer plexiform layer was much shorter with fewer ganglion cells and fewer synapses in the inner plexiform layer. Furthermore, ocular developmental defects of retinal depigmentation, optic disc abnormality, and persistent hyaloid artery were also observed. All these abnormalities had not recovered even at 40 weeks of age. Our Rbp4-deficient mice accumulated retinol in the liver but it was undetectable in the serum, indicating an inverse relation between serum and liver retinol levels. Our results suggest that RBP4 is critical for the mobilization of retinol from hepatic storage pools, and that such mobilization is necessary for ocular development and visual function.

The diseased vitreous body: Malformations, developmental disorders and opacities

Remnants of a persistent hyaloid artery can occasionally cause cataracts and traction on the retina at the posterior pole of the eye.It is the task of the ophthalmologist to weigh up the risk of amblyopia against the risks of vitrectomy and lensectomy. The retina is primarily intact. This is different from the group of hereditary vitreoretinal dystrophies where defects in the retina and vitreous body contribute equally to the overall clinical manifestations. Familial exudative vitreoretinopathy (FEVR) of childhood is sometimes misdiagnosed as uveitis. Retinal exudates leak from enormously permeable retinal vessels and it is necessary to completely remove such abnormal vessels to stop progression. The peculiar vitreous opacities consist of paper-like veils which are stacked like onion skins. The FEVR simulates retinopathy of prematurity and other exudative vitreoretinopathies. Stickler and Wagner syndromes are associated with a high risk of rhegmatogenous retinal detachment, similar to giant retinal tears. Occasionally, extraocular manifestations, such as hyperextensive joints are indicative of a systemic connective tissue disease.The peripheral retina is mechanically weak and susceptible to tears and giant or multiple tears can occur. The prognosis is further aggravated by an elevated risk of proliferative vitreoretinopathy (PVR). It is, therefore, essential to identify the risk profile in this constellation and bear the risk of PVR in mind when selecting a treatment regimen, similar to that for giant tear retinal detachment.

Bilateral persistent hyaloid artery in the adult patient

Introduction: Hyaloid artery occurs in the third week of gestation and reaches the lens in the fourth or fifth week of gestation to form the tunica vasculosa lentis. Atrophy of the hyaloid artery usually ends before birth. The existence of the hyaloid artery with the active blood flow is rare, e specially if bilateral. There are several reports about vitreous hemorrhages (spontaneous and traumatic) and tractional retinal detachments that are associated with persistent hyaloid artery. Patient and Methods: A male patient, aging 25, reported to an ophthalmologist because of subjective complaints of visual impairment. Visual acuity in both eyes was 1.0 determined by Snellen, intraocular pressure of 12 mmHg. Refractometry showed ROD + 1.50 diopters / +0.25 Dcyl (103 °), ROS +1.50 diopters / + 0.50 Dcyl (38 °). The bio microscopy study showed the opalescence in the space of the vitreous body behind a transparent lens on both eyes. Fundus examination showed that both optic nerves were large and with a large central coloboma. It was observed as a. hyaloidea persistens, which comes from the optic disc, surrounded by primary vitreous and at the front end visible as opaque vitreous body behind the clear lens. B -scan confirmed the echogenic visible linear structure, medium to higher reflectivity, in the back of t he vitreous body, which starts with the optic nerve head and extends towards the lens (a. hyaloidea persistens). In addition, fluorescein angiography was performed, which showed a slow and sustained filling of the narrow persistent hyaloid artery. Computerized perimetry showed enlargement of the blind spots on both sides. Optical coherence tomography of the optic nerve of both eyes showed a tubular structure on the optic nerve with hiporeflectivity interior. Conclusion: Persistent hyaloid artery must be con side red as a possible etiologic factor for bleeding in the vitreous body, particularly in healthy young men. Its presence can warn us of possible complications during cataract surgery and other surgical procedures. Ultrasound provides essential information when making a diagnosis, in view to determine the presence of lesion, its provision and the involvement of the retina and optic disc head. The review should include the fluorescein angiography and optical coherence tomography.

Bilateral persistent hyaloid artery. A case report

Case reportA 5-year-old male presented with bilateral poor vision, esotropia and a previous diagnosis of cataract since he was 1 year old. The physical examination revealed bilateral posterior paracentric capsule opacification, vitreous cavity with a permeable pulsatile blood filled hyaloid artery in both eyes. He was kept under observation. DiscussionPersistent hyaloid artery is an uncommon faulty primary vitreous regression, often unilateral (although it may be bilateral) and sporadic, associated with microphthalmos. It may be complicated with glaucoma and phthisis bulbi. Vitrectomy plus lensectomy or simple observation is the accepted treatment option.

Combined central retinal artery and vein occlusion secondary to cataract surgery in a patient with persistent hyaloid artery

Case reportA 72-year-old woman referred for cataract surgery in her right eye. Biomicroscopy revealed a retrocapsular fibrotic tissue in communication with the optic nerve, suggesting a persistent hyaloid artery (PAH). A posterior capsule rupture unexpectedly occurred during lens hydrodissection. One day after surgery, fundus examination showed a combined central retinal artery and vein occlusion. ConclusionPAH is uncommon, but its presence may alert us of this possible complication during cataract surgery.

Precocious synapses in 13.5-week fetal holoprosencephalic cortex and cyclopean retina

Background: Genetic programming of cerebral development involves tissue morphogenesis and also timing of developmental processes. Precocious synaptogenesis in the neocortical plate was previously demonstrated in 5 of 6 fetuses of 20–31weeks gestation. Materials and methods: Neuropathological examination was performed of a 13-week-5-day fetus with trisomy-13, alobar holoprosencephaly, hydrocephalus, cyclopia and absence of ears. Immunocytochemical demonstration of the synaptic vesicle protein synaptophysin was performed in the brain and retina, along with other neuronal markers. Results: Synaptophysin reactivity in the cortical plate was patchy and precocious. Radial glial fibres, demonstrated by vimentin, were oriented parallel to the cortical plate rather than perpendicular, probably because of hydrocephalus. A corpus striatum was not identified, but the poorly formed thalamus exhibited synaptophysin reactivity around many neurones. The cyclopean eye had ocular features of maturational delay including persistent hyaloid artery; ganglion cells were reduced in number, but retinal synaptophysin reactivity was paradoxically precocious. Conclusions: Holoprosencephaly exhibits abnormal patchy synapse distribution in the neocortex and retina; synaptogenesis was precocious, as we previously described in older fetuses. Too soon an onset of synapse formation may promote early epileptic circuitry, leading to severe infantile epilepsies postnatally. The visual system is the last of the special sensory systems to mature, yet in this case showed too early synapse formation. In HPE, cyclopia and in trisomy 13, total absence of external ears has not been reported; it results from faulty craniofacial induction by neural crest.

Ciliary Body Medulloepithelioma: Analysis of 41 Cases

To describe the clinical features, histopathology, treatment, and outcomes of ciliary body medulloepithelioma. Retrospective study. Forty-one patients with medulloepithelioma. Cryotherapy, plaque radiotherapy, external beam radiotherapy, tumor removal by partial lamellar sclerouvectomy (PLSU), or enucleation. Metastasis and death. Of 41 patients with ciliary body medulloepithelioma, the median age at diagnosis was 5 years. The mean tumor basal diameter was 11 mm, and the mean tumor thickness was 7 mm. Related features included secondary glaucoma (n = 18, 44%), iris neovascularization (n = 21, 51%), cataract (n = 19, 46%), lens subluxation (n = 11, 27%), lens coloboma (n = 8, 20%), retrolental neoplastic cyclitic membrane (n = 21, 51%), intratumoral cysts (n = 25, 61%), and extraocular extension (n = 4, 10%). There was systemic association with pleuropulmonary blastoma in 2 cases (5%). Primary tumor treatment included enucleation (n = 21, 60%), tumor removal by PLSU (n = 8, 23%), plaque radiotherapy (n = 3, 9%), external beam radiotherapy (n = 1, 3%), cryotherapy (n = 1, 3%), or palliative chemotherapy (n = 1, 3%). In 1 case, medulloepithelioma was diagnosed histopathologically after inadvertent evisceration for blind painful eye. Subsequent treatment for residual or recurrent tumor in cases treated conservatively/inappropriately (n = 15) was necessary in 7 cases (47%). Histopathology disclosed benign features in 6 cases (20%), malignant features in 24 cases (80%), teratoid features in 11 cases (37%), and nonteratoid features in 19 cases (63%). In the 26 enucleated eyes, other features included retrolental neoplastic cyclitic membrane (n = 18, 69%), neoplastic epiretinal membrane (n = 6, 23%), and persistent hyaloid artery (n = 6, 23%). Systemic metastasis occurred in 3 cases (8%) over a mean follow-up of 49 months, all of whom presented with extrascleral extension of tumor due to mean delay in diagnosis by 39 months. Medulloepithelioma most commonly occurs in children. Systemic association with pleuropulmonary blastoma rarely is found. Patients with extrascleral medulloepithelioma are at risk for metastasis.