Endoscopic Vitrectomy for Microcornea, Posterior Megalolenticonus, Persistent Fetal Vasculature, Coloboma Syndrome

Bowe, Theodore; Rahmani, Safa; Yonekawa, Yoshihiro

doi:10.1016/j.ophtha.2017.07.028

Cited by 6 publications

(3 citation statements)

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“…58 Endoscopic vitrectomy has been used successfully in complex cases, including microcornea, posterior megalolenticonus, PFVS, and coloboma syndrome. 59 Endoscopic vitrectomy is currently only available in 20-gauge and 23-gauge platforms.…”

Section: Endoscopic Vitrectomymentioning

confidence: 99%

<p>Diagnostic and Management Strategies in Patients with Persistent Fetal Vasculature: Current Insights</p>

Prakhunhungsit¹,

Berrocal²

2020

OPTH

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Persistent fetal vasculature (PFV), previously known as persistent hyperplastic primary vitreous, is a developmental malformation of the eyes that is caused by a failure of the hyaloid vasculature to regress in utero. PFV has been reported for decades; however, our understanding of the pathophysiology/pathogenesis of PFV, and the diagnostic and treatment modalities for PFV have evolved over time, and these advancements have improved diagnosis, treatment, and outcomes. However and in spite of these advancements, the heterogeneity of this disease continues to make PFV a diagnostic challenge. Here, we review what is currently known about various important aspects of PFV to update and enhance the knowledge of ophthalmologists who encounter and manage PFV in clinical practice.

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Section: Endoscopic Vitrectomymentioning

confidence: 99%

<p>Diagnostic and Management Strategies in Patients with Persistent Fetal Vasculature: Current Insights</p>

Prakhunhungsit¹,

Berrocal²

2020

OPTH

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“…Endoscopy also allows the application of laser photocoagulation to the far periphery to surround focal retinal tractions and defects as needed. 50 Lack of adequate visualisation and treatment of the peripheral retina predisposes these eyes for retinal detachments complicated by retinal dialyses and proliferative vitreoretinopathy. The combined lensectomy-vitrectomy approach with anterior and posterior surgeons is advantageous over pars plana lensectomy and vitrectomy in that it often allowed for maintenance of capsular support for a future IOL.…”

Section: Discussionmentioning

confidence: 99%

Outcomes and surgical management of persistent fetal vasculature

2021

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ObjectiveTo analyse outcomes in different forms of persistent fetal vasculature (PFV).Methods and analysisRetrospective cohort study at a university-based practice of children presenting with PFV between 2011 and 2020. Exclusion criteria was surgical management outside of our institution and follow-up less than 1 month. Wilcoxon and Student’s t-tests were used for statistical analysis.ResultsForty-six eyes of 45 patients presented with PFV at 16.7±31.3 (median 2.8) months old with 32.6±29.8 (median 22.5) months of follow-up. Types of PFV included: mild combined anterior-posterior (23 eyes, 50%), severe combined anterior-posterior (18 eyes, 39%), severe anterior (3 eyes, 7%), mild anterior (1 eye, 2%) and posterior (1 eye, 2%). Thirty-two eyes (70%) underwent PFV surgical correction; lensectomy (13 mild combined), vitrectomy (3 mild combined), sequential lensectomy then vitrectomy (3 severe combined), combined lensectomy-vitrectomy (11 severe anterior or severe combined), laser retinopexy (1 mild combined). Five eyes required additional vitrectomy surgery for retinal detachment, fold or cyclitic membrane. Nine eyes developed glaucoma, six requiring Intraocular pressure (IOP)-lowering surgery. At final follow-up, 32 eyes had at least form vision and 6 eyes were aversive to light. Eight eyes, all which were severe combined, and four that did not undergo PFV surgery, were unable to detect light due to phthisis bulbi (7) and optic nerve hypoplasia (1).ConclusionsClassification of PFV is important in determining surgical approach with severe cases often requiring both lensectomy and vitrectomy for optimal anatomic and functional outcomes.

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“…We add to this syndromic description of MPPC syndrome, the presence of retrobulbar cysts. A short report by Bowe et al similarly mentioned the presence of retrobulbar cysts in association with these ocular findings, but does not discuss management 10 . The presence of retrobulbar cysts further worsens the patient's final visual acuity and increases the risk of retinal detachment 5 …”

Section: Discussionmentioning

confidence: 99%

Microcornea, posterior megalolenticonus, persistent fetal vasculature, chorioretinal coloboma (MPPC) syndrome: Case series post vitrectomy

Rothfield

Cernichiaro-Espinosa

Alabiad

et al. 2019

American Journal of Ophthalmology Case Reports

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PurposeMPPC syndrome has been described as a syndrome that presents with chorioretinal coloboma, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma. The purpose of our study is to report three patients who present with a variation of MPPC syndrome who each underwent pars plana vitrectomy, pars plana lensectomy, and amblyopic management. Clinical characteristics, ancillary test findings, and post-surgical functional results are compared to what is reported in the literature.MethodsRetrospective review of medical records of patients who presented with microcornea, persistent fetal vasculature, chorioretinal coloboma, and microphthalmia who underwent surgical correction at Bascom Palmer Eye Institute.Results3 patients (6 eyes) were included, two males and one female. All patients were born full term, vaginally, and had no family history of genetic abnormalities affecting the eye. All patients had color fundus images, fluorescein angiography (FA) and echography. Four eyes underwent surgery. Following surgical intervention, patients demonstrated improved visual acuity, and improved functional status.Conclusion and ImportancePatients with microcornea, PFV, chorioretinal coloboma, and microphthalmos, can benefit from surgical intervention when functional decline or media opacities are noted. Though difficult to assess accurate visual acuity and visual improvement pre-operatively and post-operatively, it is evident that our patients demonstrated improvement in functionality and vision following surgical intervention.

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Endoscopic Vitrectomy for Microcornea, Posterior Megalolenticonus, Persistent Fetal Vasculature, Coloboma Syndrome

Cited by 6 publications

References 0 publications

<p>Diagnostic and Management Strategies in Patients with Persistent Fetal Vasculature: Current Insights</p>

<p>Diagnostic and Management Strategies in Patients with Persistent Fetal Vasculature: Current Insights</p>

Outcomes and surgical management of persistent fetal vasculature

Microcornea, posterior megalolenticonus, persistent fetal vasculature, chorioretinal coloboma (MPPC) syndrome: Case series post vitrectomy

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