Comparison study of clinical features between persistent hyperplastic primary vitreous and congenital fibrovascular pupillary membrane / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To analyze the similarities and differences of the clinical features between persistent hyperplastic primary vitreous(PHPV)and congenital fibrovascular pupillary membrane(CFPM).METHODS: Retrospectively analyze the ocular biometric parameters, clinical features and morphological changes in children with PHPV(PHPV group)and CFPM(CFPM group)who received surgery at the department of ophthalmology, Xijing Hospital from March 2006 to December 2021.RESULTS: The study included 56 cases(61 eyes)of PHPV and 24 cases(25 eyes)of CFPM. There were no differences on the gender and age of onset between PHPV and CFPM, and both of them were mainly unilaterally affected, with the ratio of 91% and 96%. Children with PHPV and cataract combined with other complications and ocular developmental abnormalities. CFPM was mainly presented different degrees of blockage and morphological abnormalities of pupillary area. In unilateral cases of PHPV and CFPM, the anterior chamber depth(ACD)of affected eyes was smaller than that of the fellow eyes, and in subgroups with age of operation ≤24mo, the axial length(AL)of affected eyes was smaller than that of the fellow eyes(P<0.05). The corneal diameter(CD)of the affected eyes in PHPV group was smaller and the intraocular pressure(IOP)was higher than those of the fellow eyes(all P<0.05); while there were no significant differences on CD and IOP between affected eyes and the fellow eyes in CFPM group(P>0.05). The ACD of affected eyes in PHPV group was significantly smaller than that of CFPM group(P<0.05). The fibrovascular membrane in PHPV group was located in the posterior part of the lens and vitreous cavity; while the fibrovascular membrane in CFPM group was located between the iris and the anterior capsule of the lens, rarely involving the lens.CONCLUSION: PHPV and CFPM had the similar clinical features, suggesting that they may belong to the different variants of persistent fetal vasculature(PFV). However, PHPV had a wider range of lesions and more complex conditions.

Ambliopia severa na infância relacionada à persistência da vasculatura fetal / Severe childhood amblyopia associated to persistence of fetal vasculature

RESUMO A persistência do vítreo primário hiperplásico, atualmente referida como persistência da vasculatura fetal, é uma anomalia congênita que resulta da não regressão do vítreo vascular primário e do sistema da artéria hialoide durante a embriogênese. Trata-se de uma anomalia unilateral na maioria dos casos, esporádica e comumente não associada a nenhum outro achado sistêmico. Clinicamente, essa condição pode ser classificada em persistência anterior e em persistência posterior da vasculatura fetal. A condição anterior está relacionada ao sistema da artéria ciliar, enquanto a persistência da vasculatura posterior associa-se à artéria hialoide e pode apresentar anormalidades, com desfecho visual desfavorável. A detecção da persistência do vítreo primário hiperplásico é de suma importância, visto que é um diagnóstico diferencial para retinoblastoma. O relato de caso a seguir descreve o acompanhamento ambulatorial em um Serviço de Oftalmologia de uma criança do sexo masculino com persistência da vasculatura fetal unilateral e sem alterações sistêmicas.

ABSTRACT Hyperplastic primary vitreous persistence, currently referred to as fetal vasculature persistence, is a congenital anomaly that results from non-regression of the primary vascular vitreous and hyaloid artery system during embryogenesis. It is a unilateral anomaly in the vast majority of cases, sporadic and commonly not associated with any other systemic finding. Clinically, this condition can be classified into anterior and posterior persistence of fetal vasculature. The anterior condition is related to the ciliary artery system, while the persistence of the posterior vasculature is associated with the hyaloid artery, which may present abnormalities with an unfavorable visual outcome. Detecting persistent hyperplastic primary vitreous is of paramount importance, as it is a differential diagnosis for retinoblastoma. The following case report describes the outpatient follow-up at the ophthalmology service of the Federal University of Triângulo Mineiro (UFTM) of a male child with persistent unilateral fetal vasculature and no systemic changes.

Surgical treatment and current advances of persistent fetal vasculature syndrome / 中华眼底病杂志

Persistent fetal vasculature syndrome (PFVS) is a rare congenital vitreous dysplasia, which is classified as anterior, posterior and combined types according to the location of the vascular abnormalities. The clinical manifestations of PFVS are diverse, and early surgical intervention is very important. The main objective of surgical treatment is to remove the anterior and posterior traction between fibrovascular membranes and retina as well as lens, and to reconstruct clear visual axis. Surgical treatments include pupilloplasty, lensectomy with or without intraocular lens implantation and vitrectomy via limbal or scleral approach. For new technologies, the applications of ophthalmic viscosurgical device and femtosecond lasers have desirable results . In addition to focusing on improving the success rate of surgery, it is also necessary to systematically and comprehensively assess the overall preoperative condition and postoperative visual function of the patients. PFVS eyes have limited improvement in postoperative vision, which is related to the extent of lesion involvement and the occurrence of complications. Eyes with macular dysplasia and tractional retinal detachment, as well as elongated ciliary process, have a poor prognosis of vision after surgery. How to improve postoperative vision in the eye affecting the posterior segment of the eye with PFVS from the microscopic anatomical relationship between the fibrous vascular pedicle and the retina is worth further study. On the other hand, reducing surgical trauma and optimizing surgical procedures in order to improve postoperative visual acuity and reduce postoperative complications are also the key research directions of future PFVS treatments.

Hemorragia vítrea como causa de pérdida de la visión en una paciente con Dengue / Vitreous hemorrhage as a cause of vision loss in a patient with dengue

RESUMEN La hemorragia vítrea secundaria a una arteria hialoidea persistente íntegra es una anomalía infrecuente. Se presenta el caso de una mujer de 65 años con antecedentes personales conocidos de esta anomalía congénita en el ojo derecho, la cual presentó disminución de visión de ese ojo de forma repentina, 10 días después de haber presentado cuadro febril agudo con diagnóstico de Dengue. La biomicroscopía posterior reveló una arteria hialoidea persistente rellena de sangre con inserción en la cristaloides posterior. En la oftalmoscopia indirecta se observó una hemorragia vítrea parcial, que permitía visualizar las estructuras de la retina. Se realizaron como pruebas diagnósticas y de seguimiento la ecografía ocular y la retinofoto.

ABSTRACT Vitreous hemorrhage secondary to a complete persistent hyaloid artery is an uncommon anomaly. The case of a 65-year-old woman with a known personal history of this congenital anomaly in the right eye is presented, who had a sudden decrease in vision of that eye, ten days after presenting an acute febrile condition with a diagnosis of dengue. Posterior biomicroscopy revealed a persistent hyaloid artery filled with blood with insertion into the lens posterior portion or hyaloid fossa. In indirect funduscopy a partial vitreous hemorrhage was observed, which allowed visualizing the structures of the retina. Ocular ultrasound and retinophoto were performed as diagnostic and follow-up tests. The vitreous hemorrhage was completely reabsorbed at 15 days and the patient recovered her vision at 0.5 due to the presence of an ongoing cataract. There was no recurrence of bleeding in six months' follow-up.

A clinicopathological study of persistent fetal vasculature

Purpose: To study the clinicopathological findings of Persistent Fetal Vasculature (PFV) in patients with congenital cataract and PFV. Methods: Six eyes with anterior or combined PFV with cataract underwent phacoaspiration with primary posterior capsulotomy with anterior vitrectomy with intraocular lens implantation followed by histopathological evaluation of the PFV stalk and membrane. Results: Four and two patients had combined and anterior PFV respectively. There was no postoperative hyphema, vitreous haemorrhage, glaucoma or retinal detachment in six months. Haematoxylin and eosin staining showed inflammatory cells predominantly with extramedullary hematopoeisis and vascularisation. Conclusion: We recommend IOL implantation in PFV, with early and aggressive amblyopia therapy.

Aqueous misdirection syndrome in persistent fetal vasculature (PFV) / Síndrome de mau direcionamento do humor aquoso em persistência da vasculatura fetal (PVF)

ABSTRACT We report a case of a 5-year-old girl with persistent fetal vasculature who presented with unilateral acute angle closure caused by aqueous misdirection syndrome that was apparently associated with regression of cycloplegia. Initial treatment with topical steroids, anti-glaucomatous drops, and atropine showed insufficient control of the intraocular pressure (IOP). Surgical treatment with lensectomy and vitrectomy resulted in satisfactory IOP control and no complications at the 6-month follow-up.

RESUMO Relatamos o caso de uma criança de 5 anos, sexo feminino com persistência do vítreo primário que apresentou crise de fechamento angular por síndrome do mau direcionamento do humor aquoso, aparentemente associado a regressão da cicloplegia. Tratamento inicial com corticoide tópico, colírios antiglaucomatosos e atropina mostraram controle insuficiente da pressão intraocular (PIO). Tratamento cirúrgico com lensectomia e vitrectomia anterior permitiu o controle satisfatório da PIO, sem intercorrências no seguimento de 6 meses.

Clinical Characteristics of Retinoblastoma Patients whose Diagnosis was Difficult due to Atypical Ocular Manifestation

PURPOSE: To report the clinical characteristics of retinoblastoma patients whose diagnosis was difficult due to atypical ocular manifestations. METHODS: Among retinoblastoma patients who were diagnosed and treated from January 1999 to December 2014 at Seoul National University Children's Hospital, 6 patients whose diagnosis was difficult were retrospectively reviewed. Factors including age, sex, family history, initial findings, time to final diagnosis, histopathologic examination, additional treatment, and survival rate were evaluated. RESULTS: Among 6 patients, 5 were male, and the mean age at the initial visit was 32.9 ± 19.1 months. None of the patients had family history, and all presented with unilateral lesion at the initial visit. The initial diagnoses were Coats' disease and uveitis in 2 patients, respectively, and persistent hyperplastic primary vitreous and traumatic hyphema in 1 patient, respectively. During an intensive short-term follow-up of 8.3 ± 5.3 weeks, 2 patients showed malignant cells after external subretinal fluid drainage procedure, and 4 patients demonstrated increasing ocular size or calcification in imaging. These patients received enucleation under suspicion of malignancy and were finally diagnosed with retinoblastoma after histopathologic examination. There were 2 patients with optic nerve involvement, and 3 patients underwent additional systemic chemotherapy. Five patients were followed-up for 7.6 ± 6.3 years after enucleation, and the mean age at final follow-up was 10.6 ± 7.4 years. CONCLUSIONS: Retinoblastoma is one of the diseases in which early diagnosis and treatment are important. However, some cases are difficult to diagnose, even for experienced clinicians. If there are no typical manifestations such as mass or calcification and early findings show retinal detachment, glaucoma, pseudohypopyon, or hyphema, intensive short-term follow-up to exclude retinoblastoma is needed.

Ophthalmic Doppler in persistent hyperplastic primary vitreous atypical presentation: case report / Doppler oftálmico na persistência hiperplásica do vítreo primário de apresentação atípica: relato de caso

ABSTRACTThe purpose is to document a case of persistent hiperplastic primary vitreous (PHPV) with atypical Bergmeister's papilla and patent hialoid artery that had its conclusive diagnosis done by doppler ultrasound. We report a case of child, who had unilateral leucokoria. This child's fundoscopic examination showed a white mass on the optic nerve that extended over to adjacent retina. Performed ultrasound that remained a doubt with the following diagnostic hypotheses: persistent hiperplastic primary vitreous, granuloma (toxocara), astrocytic hamartoma and retinoblastoma. The diagnosis was only established when the doppler ultrasound showed a blood flow inside of the membrane, thus confirming the diagnosis of persistent hiperplastic primary vitreous associated with the Bergmeister'spapilla.

RESUMOO objetivo é documentar um caso de persistência hiperplásica do vítreo primário (PHPV) com papila de Bergmeister atípica e artéria hialóide pérvia que teve seu diagnóstico conclusivo feito pelo exame ultrassonográfico com Doppler colorido. Relatamos um caso de uma criança com leucocoria unilateral, apresentando massa branca sobre o disco óptico observada à fundoscopia, que se estendia à retina adjacente. Após a realização do exame ultrassonográfico foi mantida a dúvida com as seguintes hipóteses diagnósticas: persistência hiperplásica do vítreo primário, granuloma (toxocaríase) e retinoblastoma. O diagnóstico foi estabelecido após a utilização do Doppler colorido que evidenciou fluxo sanguíneo no interior da membrana, confirmando o diagnóstico de persistência hiperplásica do vítreo primário associada à papila de Bergmeister.

Persistent fetal vasculature: ocular features, management of cataract and outcomes / Persistência da vasculatura fetal: características oftalmológicas, manuseio da catarata e resultados cirúrgicos

PURPOSES: To describe ocular features, management of cataract and functional outcomes in patients with persistent fetal vasculature. METHODS: Retrospective, descriptive case series of patients with persistent fetal vasculature. Data were recorded from the Congenital Cataract Section of Federal University of São Paulo, Brazil from 2001 to 2012. All patients were evaluated for sex, age at diagnosis, systemic findings, laterality, age at surgery, and initial and final follow-up visual acuities. Follow-up and complications after cataract surgery were recorded. Ultrasound was performed in all cases and ocular eco-Doppler was performed in most. RESULTS: The study comprised 53 eyes from 46 patients. Age at diagnosis ranged from 5 days of life to 10 years-old (mean 22.7 months). Twenty-seven patients were male (58.7%). Persistent fetal vasculature was bilateral in 7 patients (15.2%). Forty-two eyes (79.2%) had combined (anterior and posterior forms) PFV presentation, 5 eyes (9.4%) had only anterior persistent fetal vasculature presentation and 6 eyes (11.3%) had posterior persistent fetal vasculature presentation. Thirty-eight eyes (71.7%) were submitted to cataract surgery. Lensectomy combined with anterior vitrectomy was performed in 18 eyes (47.4%). Phacoaspiration with intraocular lens implantation was performed in 15 eyes (39.5%), and without lens implantation in 5 eyes (13.2%). Mean follow-up after surgery was 44 months. Postoperative complications were posterior synechiae (3 cases), retinal detachment (2 cases), phthisis (3 cases), posterior capsular opacification (8 cases), inflammatory pupillary membrane (5 cases), glaucoma (4 cases), intraocular lens implantation displacement (1 case) and vitreous hemorrhage (2 cases). Complications were identified in 19 (50%) of the 38 operated eyes. Visual acuity improved after cataract surgery in 83% of the eyes. CONCLUSIONS: Patients with persistent fetal vasculature have variable clinical presentation. There is an association of persistent fetal vasculature with congenital cataract. Severe complications are related to cataract surgery in patients with persistent fetal vasculature, but 83% of the operated eyes improved visual acuity.

OBJETIVOS: Descrever as características oftalmológicas, o tratamento da catarata e os resultados funcionais em pacientes com o diagnóstico de persistência da vasculatura fetal. MÉTODOS: Estudo retrospectivo e descritivo de série de casos de pacientes com persistência da vasculatura fetal. Dados foram obtidos dos arquivos do Setor de Catarata Congênita da Universidade Federal de São Paulo, Brasil, durante o período entre 2001 a 2012. Todos os pacientes foram avaliados quanto ao sexo, idade ao diagnóstico, achados sistêmicos, lateralidade, idade à cirurgia e acuidade visual inicial e final ao seguimento. Complicações após a cirurgia da catarata foram analisadas. Ultrassom foi realizado em todos os casos e eco-Doppler foi realizado na maioria dos pacientes. RESULTADOS: O estudo incluiu 53 olhos de 46 pacientes. Idade ao diagnóstico variou de 5 dias de vida até 10 anos (média 22,7 meses). Vinte e sete pacientes eram masculinos (58,7%). A persistência da vasculatura fetal foi bilateral em 7 pacientes (15,2%). Quarenta e dois olhos (79,2%) apresentaram formas combinadas (anterior e posterior) da persistência da vasculatura fetal, 5 olhos (9,4%) tinham somente a forma anterior da persistência da vasculatura fetal e 6 olhos (11,3%) tinham a forma posterior de apresentação da persistência da vasculatura fetal. Trinta e oito olhos (71,7%) foram operados de catarata. Lensectomia com vitrectomia anterior foi realizada em 18 olhos (47,4%). Facoaspiração com implante de lente intraocular foi realizada em 15 olhos (39,5%) e sem implantação de lente em 5 olhos (13,2%). O seguimento médio após cirurgia foi de 44 meses. Complicações pós-operatórias foram: sinéquias posteriores (3 casos), descolamento da retina (2 casos), atrofia do globo ocular (3 casos), opacificação da cápsula posterior (8 casos), membrana pupilar inflamatória (5 casos), glaucoma (4 casos), deslocamento da lente implantada (1 caso) e hemorragia vítrea (2 casos). Complicações foram identificadas em 19 (50%) dos 38 olhos operados. Acuidade visual melhorou após a cirurgia da catarata em 83% dos olhos. CONCLUSÕES: Pacientes com persistência da vasculatura fetal tem apresentações clínicas variáveis. Existe uma associação da persistência da vasculatura fetal com catarata congênita. Complicações graves são associadas com a cirurgia da catarata nesses pacientes, mas 83% dos olhos operados melhoraram a acuidade visual nesse estudo.

Therapeutic effects of vitrectomy for persistent fetal vasculature / 中华眼底病杂志

ObjectiveTo observe the effects of vitrectomy for persistent fetal vasculature (PFV).Methods The clinical data of 11 patients (14 eyes) with PFV, including posterior PFV (one eye) and combined PFV (13 eyes), were retrospectively analyzed. Vitrectomy was performed for all patients,lensectomy was performed for seven eyes, retinotomy was performed for five eyes, retinotomy and scleral buckling was performed for one eye. Intraoperative and postoperative complications were observed. The follow-up period ranged from eight to 74 months, with the mean of 44 months. The visual acuity (VA) and intraocular pressure before and after surgery were comparatively analyzed. Results There were no intraoperative complications in all the patients. The surgery was performed successfully in 12 eyes (86.0 %)and failed in two eyes (14. 0%). The postoperative VA of five eyes (35.7%) increased, four eyes (28. 6%)didn't change and five eyes (35.7%) decreased. The postoperative intraocular pressure of two eyes (14. 3%) increased, 11 eyes (78. 6%) didn't change and one eye (7. 1%) decreased. There was one eye with persistent hypotension and one eye with recurrent retinal detachment after surgery. Conclusion Vitrectomy can prevent complications and improve visual acuity in some PFV patients.

Clinical Findings and Prognosis of Persistent Hyperplastic Primary Vitreous

PURPOSE: To evaluate the clinical findings, visual improvement and preservation of eye according to treatment modality among children with persistent hyperplastic primary vitreous (PHPV) METHODS: A retrospective study, from 1982 to 2001, at Seoul National University Children's Hospital RESULTS: The subjects for investigation were 126 PHPV eyes in 114 children. The mean age at first exam was 3.6 +/- 3.5months, and the mean age at operation was 13.3 +/- 12.1months. Combined type (67%) was most common, followed by posterior (24%), and anterior (9%) types. The chief complaints in order of frequency were are leukocoria (35%), no fixation (23%), strabismus (21%), and microophthalmia (12%). Percentages of eye preservation and visual improvement over 20/1000 were 77%, and 57%, respectively. Nevertheless, the percentage of visual improvement over 20/200 in the anterior type was 25%. CONCLUSIONS: Combined type was most common in PHPV. Clinical findings and treatment modality varieds according to the type. Visual prognosis was poor. However, the post-treatment status was good in view of the treatment purpose. Therefore we can optain a good outcome through active treatment after the exact classification of PHPV eyes and by establishing the treatment purpose.

Ultrasonographic Findings of Persistent Hyperplastic Primary Vitreous

We analyzed 33 patients (39 eyes) who were surgically diagnosed as persistent hyperplastic primary vitreous (PHPV) with standardized B-scan ultrasonography. The eyes were evaluated for vitreous stalk or membrane, retrolental proliferation, retinal fold, vitreous opacity, and retinal detachment (64.1 %, 48.7 %, 15.4 %, 15.4 %, and 5.1 %, respectively) with the results. We classified these findings of PHPV into 5 types as follows, type 1: simple retinal fold or preretinal fold alone; type 2: retrolental proliferation alone; type 3: vitreous stalk or membrane; type 4: retrolental proliferation connected with vitreous stalk or membrane; type 5: complicated retinal detachment. The incidence of these types were 15.4%, 15.4%, 28.2 %, 33.3 %, and 5.1 %, respectively. Ultrasonographic findings would be used as a useful parameters to evaluate, document, and plan the management in PHPV.

Clinical Evaluation in Persistent Hyperplastic Primary Vitreous

We analysed a retrospective study of 23 patients (29 eyes) who were clinically and radiologically diagnosed as persistent hyperplastic primary vitreous from January 1984 to June 1992 at St. Mary's Hospital, Catholic University Medical College, by observing the clinical findings, results of CT or MRI, incidence of complications and postoperative visual acuity. The results were as follows: 1. The unilateral ocular involvement was 73.9% and bilateral ocular involvement was 26.1%. 2. The common presenting clinical signs were shallow anterior chamber, lens opacity, vitreous opacity and microphthalmos. 3. The main features of CT or MRI were lens defcrmity, shallow anterior chamber, vitreous opacity and microphthalmos. 4. The complications were glaucoma (31.0%), intraocular hemorrhages (10.3%), posterior synechiae (17.2%) and phthisis bulbi (6.9%). 5. The postoperative visual acuity was not improved than preoperative levels.

A Case of Bilateral Persistent Hyperplastic Primary Vitreous

Persistent hyperplastic primary vitreous(PHPV) is a congnital abnormality of the embryonic intraocular vasculature, resulting from the failure of regression of the primary vitreous in a full-term infant. PHPV is usually unilateral and characterized by leukocoria, elongated ciliary process, microphthalmia, cataract, rubeosis iridis, shallow anterior chamber, and secondary glaucoma. PHPV finally develops into retinal detachment, intractable glaucoma, and pthisis bulbi. Surgical treatment, involving lensectomy and vitrectomy, is not successful and poor visual result is obtained due to retinal abnormalities. A one-month-old girl was found to have persistent hyperplastic primary vitreous in both eyes, and she expired of unknown etiology at home 2 months after diagnosls.