Benign Lobular Inner Nuclear Proliferations (BLIPs)
All content on Eyewiki is protected by copyright law and the Terms of Service. This content may not be reproduced, copied, or put into any artificial intelligence program, including large language and generative AI models, without permission from the Academy.
Article summary goes here.
Disease Entity
Benign lobular inner nuclear layer proliferations (BLIPs) are novel, benign intraretinal tumors that arise in the inner nuclear layer (INL) of the retina that may be associated with congenital hypertrophy of the retinal pigment epithelium (CHRPE),
History
The term BLIPs was first published by a group led by Aaron Nagiel, MD, PhD, Christian J. Sanfilippo, MD, and Michael Javaheri MD (BLIP consortium), in 2022 who published four cases of a novel intrinsic retinal tumor that in some cases was associated with ipsilateral CHRPE lesions. They characterized these lesions with multimodal imaging and performed whole exome sequencing of the blood in three of the four patients. The areas were thought to be hamartomatous in nature and were believed to benign and not visually significant[1]. Once published, one previous case was found that had been published as a "Diagnostic and Therapeutic Challenge" without a disease entity [2]
Etiology
Unknown
Risk Factors
[1]None have been identified to date. No sex predilection has been described.
General Pathology
None
Pathophysiology
Add text here
Primary prevention
None
Diagnosis
Add text here
Physical examination
- Ophthalmic Examination
- Visual Acuity: Most cases show no visual impairment.
- Funduscopic Examination
Signs
Add text here
Symptoms
Although lesions can be seen in the macula, patients are typically asymptomatic.
Clinical diagnosis
- Fundus Photography: white intraretinal tumors in the consisting of lobules with thin, arching extensions. These lesions are typically in the macula but can extend all the eay to the ora serrata. CHRPE lesions may be seen extending from the macula to ora serrata.
- Fundus Autofluorescence: mild hypoautoflourescence corresponding to the areas of BLIP lesions but severe in areas of CHRPE lesions.
- Fluorescein Angiography: no areas of leakage as the areas of BLIP have no intrinsic vascularity.
- Optical Coherence Tomography: multiple homogenous, hyper-reflective lobular lesions residing in the INL compressing the adjacent outer retinal and inner retinal layers of the retina.
- En Face OCT highlights the "ball and spike" shape of lesions.
- BLIPs are homogenously reflective without intralesional cavitations or hyper-refelective foci.
- BLIPs do not erode into adjacent layers, or cause intraretinal fluid, hemorrhage or exudates.
- Optical Coherence Tomography Angiography: shows absence of flow signal
Laboratory test
Genetic testing has been used but has yet to identify a correlation.
Differential diagnosis
Management
Short term follow up indicates lesion stability[1]. One case demonstrates stability after 30 years of follow up[3].
General treatment
As these lesions are benign, cause no visual impairment, and remain stable, no intervention is warranted.[3][2]
Medical therapy
None
Medical follow up
Add text here
Complications
Add text here
Prognosis
Add text here
Additional Resources
Add text here
References
Add text here
- ↑ Jump up to: 1.0 1.1 1.2 1.3 1.4 Sanfilippo CJ, Javaheri M, Handler S, Berry JL, Cobrinik D, Deardorff MA, Sun M, Schmidt RJ, Barkmeier AJ, Nagiel A. Benign Lobular Inner Nuclear Layer Proliferations of the Retina Associated with Congenital Hypertrophy of the Retinal Pigment Epithelium. Ophthalmology. 2023 Mar;130(3):265-273. doi: 10.1016/j.ophtha.2022.10.011. Epub 2022 Oct 18. PMID: 36270406; PMCID: PMC9974858.
- ↑ Jump up to: 2.0 2.1 Pastor-Idoate S, Heimann H, Keane PA, Balaskas K, Lujan BJ. Diagnostic and Therapeutic Challenges. Retina Phila Pa. 2016;36(9):1796-1801. doi:10.1097/IAE.0000000000000979
- ↑ Jump up to: 3.0 3.1 Shah M, Charbel Issa P. Long-Term Stability of Benign Lobular Inner Nuclear Layer Proliferations. JAMA Ophthalmol. 2024 Jun 1;142(6):e236065. doi: 10.1001/jamaophthalmol.2023.6065. Epub 2024 Jun 20. PMID: 38900199.