Bergmeister Papilla

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Article initiated by:

Andrew Thomson, MD, Kaitlyn Hunter, Kieley Trempy, Eric J. Niespodzany, MD

All contributors:

Andrew Thomson, MD, Kaitlyn Hunter, Kieley Trempy, Leo A. Kim, MD, PhD, Koushik Tripathy, MD (AIIMS), Eric J. Niespodzany, MD

Assigned editor:

Koushik Tripathy, MD (AIIMS)

Review:

Assigned status Up to Date

 by Koushik Tripathy, MD (AIIMS) on September 22, 2024.

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Disease Entity

ICD-10 Q14.2

Figure 1. Optical coherence tomography through Bergmeister papilla

Disease

The Bergmeister papilla was first reported by Austrian ophthalmologist Dr. Otto Bergmeister in 1877. Also referred to as an epipapillary veil, it is a remnant of the fibrous sheath surrounding the fetal hyaloid artery during embryological development.

Etiology

In the fetus, blood is supplied to various parts of the eye by the hyaloid artery. The hyaloid artery travels through the optic nerve head to the posterior aspect of the lens, where it branches into the tunica vasculosa lentis. During normal development at around 30 weeks of gestational age, this blood supply is resorbed and develops into Cloquet’s (hyaloid) canal. Incomplete resorption of these structures during development can result in persistent fetal vasculature, such as the Mittendorf dot and the Bergmeister papilla. It is suspected that defects in genes guiding cell apoptosis result in persistent fetal vasculature.

Figure 2. Illustration of fetal vasculature, consisting of the hyaloid artery running through Cloquet’s canal.

Diagnosis

History

Patients with Bergmeister papillae are often born premature, though many are born full term.

Symptoms

Bergmeister papillae are typically asymptomatic and found incidentally. Changes to vision would depend upon association with secondary effects on the retina or macula.

Diagnostic procedures

Bergmeister papillae can be observed with ophthalmoscopy and fundus photography (Figure 3), but they are more likely to be identified through optical coherence tomography (OCT) (Figure 1). Visualization reveals a cluster of veil-like glial or fibroglial tissue emerging anteriorly, centrally, and nasally from the optic nerve head. Presence of papillae can influence the size of the optic nerve head cup. Bergmeister papillae can be unilateral or bilateral.

Figure 3. Fundus photo of an optic nerve with a Bergmeister papilla.

Papillae can be vascularized by a retinal vessel, otherwise known as a prepapillary vascular loop. If it is vascularized, this can be visualized on fluorescein angiography (FA). Without vascularization, the FA of a patient with Bergmeister papilla would be unremarkable.

Differential diagnosis

The most important differential diagnosis is regressed (fibrosed) neovascularization of disc (NVD). Examination of the retina usually reveals retinal vascular disease (ischemic NVD) or inflammation (inflammatory NVD) in cases with NVD. FA is useful in such cases to confirm NVD and to evaluate the retinal vascular status.

Management

General treatment

None indicated if Bergmeister papillae are without associated complications.

Prognosis

Prognosis is good in the vast majority of cases as the papilla itself is clinically insignificant. There have been reports of Bergmeister papilla associated with vitreoretinal adhesion and macular retinoschisis. If a prepapillary vascular loop exists within the papilla, thrombosis of this vessel could cause vitreous hemorrhage, hyphema, branch retinal artery occlusion or branch retinal vein occlusion, though reports of these complications are rare.