Benign Lobular Inner Nuclear Proliferations (BLIPs)

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 by Michael Javaheri, MD on January 14, 2025.


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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

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Primary prevention

None

Diagnosis

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Physical examination

  • Ophthalmic Examination
    • Visual Acuity: Most cases show no visual impairment.
    • Funduscopic Examination

Signs

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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

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Complications

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Prognosis

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Additional Resources

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References

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  1. 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.
  2. 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
  3. 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.
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