Periocular intravascular papillary endothelial hyperplasia: A retrospective study

Area of Science:

• Ophthalmology
• Pathology
• Vascular Biology

Background:

• Intravascular papillary endothelial hyperplasia (IPEH) is a rare endothelial cell proliferation of uncertain etiology, often associated with thrombus formation.
• While IPEH has been documented in the periocular region, its occurrence in the conjunctiva has not been previously reported.
• Diagnosis of IPEH is typically confirmed through histopathological examination.

Purpose of the Study:

• To report the first documented cases of conjunctival Intravascular papillary endothelial hyperplasia (IPEH).
• To describe the clinical, histopathological, and management characteristics of periocular IPEH, including new conjunctival cases.
• To emphasize the association of IPEH with underlying vascular malformations.

Main Methods:

• A retrospective case series involving seven patients with histopathologically confirmed IPEH.
• Data collected included demographics, clinical presentation, radiological findings, histopathological features (including IHC), suspected vascular etiology, management, and follow-up.
• Analysis focused on identifying commonalities and differences in periocular and conjunctival IPEH cases.

Main Results:

• Seven cases of histologically confirmed IPEH were analyzed, with a median age of 36 years (range 6-69).
• Three cases (42.8%) involved the eyelid and three (42.8%) occurred in the conjunctiva.
• All patients had pre-existing vascular lesions (five malformations, two lymphangiectasis); all were treated with excisional biopsy with no recurrence at a mean 7-month follow-up.

Conclusions:

• Periocular Intravascular papillary endothelial hyperplasia (IPEH) is a rare entity frequently coexisting with vascular lesions and thrombi.
• This study provides the first evidence of IPEH occurring in the conjunctiva.
• Pathologists should recognize the histopathological spectrum of IPEH, especially in the context of periocular and conjunctival lesions.