© Springer Nature Switzerland AG 2021
C. S. Foster et al. (eds.)Uveitishttps://doi.org/10.1007/978-3-030-52974-1_24

24. Punctate Inner Choroidopathy

Karen Wingartz Small1  
(1)
Professional Eye Care, Mission of Sight, Jamaica, Chattanooga, TN, USA
 
Keywords
UveitisPunctate inner choroidopathy (PIC)

Overview

  • Definition

    • Inflammatory multifocal chorioretinal disorder of unknown etiology affecting mainly young, healthy, myopic women

    • May be a subset of multifocal choroiditis and panuveitis (MCP)

  • Symptoms

    • Loss of central acuity

    • Scotoma

    • Photopsia

    • Metamorphopsia

    • Photophobia

  • Laterality

    • 80% bilateral, but may be asymmetric

  • Course

    • Self-limited over several months

    • Recurrence is less likely compared to MCP

    • New lesions are rare

    • Visual prognosis typically good in absence of CNV and subretinal fibrosis

    • Some cases progress to MCP

  • Age of onset

    • Median of 30 years

  • Gender/race

    • Predominantly female with varying degree of myopia

    • Caucasian

  • Systemic association

    • Affected individuals are usually healthy

Exam: Ocular

Anterior Segment

  • No AC reaction

Posterior Segment

  • Little to no vitritis

  • Small (100–200 μm diameter), discrete, yellowish lesions at inner choroid and RPE:

    • Limited to posterior pole, rarely extend to midperiphery

    • Become atrophic and punched out

    • New lesions are rare

  • CNV:

    • Very common (40–75%)

    • Major cause of visual impairment

  • CME is uncommon

Exam: Systemic

  • None

Imaging

  • OCT

    • RPE elevation with sub-RPE collections and compression of photoreceptor IS/OS junctions

  • FA

    • Active lesions: early hyperfluorescence followed by variable late staining/leakage

    • Inactive lesions: window defects

  • ICG

    • Hypolucent, corresponding to lesions on exam

  • ERG

    • Normal

  • Visual field/microperimetry

    • Helpful in monitoring for scotomata

Laboratory and Radiographic Testing

None specific to PIC - Rule out masqueraders

  • Chest Xray

  • ACE/lysozyme

  • FTA-ABS/RPR

  • PPD/QuantiFERON-TB Gold

Differential Diagnosis

  • Multifocal choroiditis and panuveitis (MCP)

    • Chronic/recurrent course

    • More intraocular inflammation

    • Lesions more variable in size and extend beyond posterior pole

    • CME more common

    • ERG often abnormal

  • Presume Ocular histoplasmosis syndrome (POHS)

  • Multiple evanescent white dot syndrome (MEWDS)

  • Acute posterior multifocal placoid pigment epitheliopathy (APMPPE)

  • Subretinal fibrosis and uveitis (SFU)

  • Sarcoidosis

  • Myopic degeneration maculopathy

  • Toxoplasmosis

  • Lyme disease

  • Vogt-Koyanagi-Harada (VKH) syndrome

Treatment

  • Systemic and regional corticosteroids if lesion threatens fovea

  • IMT in rare, recurrent cases

  • CNV can be effectively treated with intravitreal anti-VEGF, PDT/verteporgin +/− intravitreal triamcinolone

Referral/Comanagement

  • None