Moran CORE

Open source ophthalmology education for students, residents, fellows, healthcare workers, and clinicians. Produced by the Moran Eye Center in partnership with the Eccles Library

Search Moran CORE

Punctate Inner Choroidopathy (PIC)

Home / Retina and Vitreous / Focal and Diffuse Choroidal and Retinal Inflammation

Title: Punctate Inner Choroidopathy (PIC)
Author: Braedon Murdock, MSIV George Washington School of Medicine; Marissa Larochelle, MD
Photographers: James Gilman, CRA, FOPS, Moran Eye Center and Glen Jenkins
Date: 8/14/2019
Keywords/Main Subjects: PIC, Punctate Inner Choroidopathy, OCT Macula, Auto-fluorescence, White dot syndromes, MEWDS

Diagnosis: Punctate Inner Choroidopathy

A 33-year-old previously healthy female presented to our clinic with a two-week history of a persistent “light spot” in the nasal aspect of her central vision in the left eye. The spot was associated with new-onset light sensitivity and generalized discomfort of the eye but was not associated with increased flashes, floaters, headaches, or other systemic symptoms. She endorsed no significant past medical history. She denied recent travel and exposure to cats.

One week after the onset of symptoms, she was seen by an outside provider where an optical coherence tomography (OCT) of the macula was obtained of the left eye showing a pigmented epithelial detachment temporal to the fovea with possible underlying subretinal fluid. Color photos showed ovoid, hazy, yellowish spots temporal to the fovea with fluorescein angiogram showing two, ovoid, hyperfluorescent lesions in laminar phase in the left eye. At that time, an infectious cause was a concern and so the patient was started on a two-week course of Bactrim DS. The following week, repeat color imaging showed multiple new punctate lesions temporal to the macula. Repeat fluorescein angiogram showed many new punctate hyperfluorescent spots temporal to the macula in laminar phase and the patient was referred for further evaluation of a possible multifocal choroiditis.

On presentation, her exam revealed (OD = right eye, OS = left eye, OU = both eyes):
⦁ Vision: 20/20 OD, 20/25-2 OS
⦁ Intraocular pressure: Normal OU
⦁ Slit-lamp examination: Normal OD. Rare vitreous cell OS.
⦁ Dilated Exam:
⦁ OD: Normal
⦁ OS: Two ovoid yellowish lesions temporal to the fovea

Imaging:

Figure 1.

 

Figure 2: Autofluorescence (Figure 1 & 2): Diffuse punctate areas of hyper-autofluorescence in the temporal fovea spanning from the superior to inferior arcades with an area of hypo-autofluorescence just temporal to the fovea.

 

Figure 3: Macula OCT of the left eye –Pigmented epithelial detachment (PED) with sub-RPE accumulation and overlying heterogeneous hyperreflective material with possible sub-retinal fluid temporal to the fovea with disruption of the IS/OS junction.

Laboratory testing for possible causes including sarcoid, tuberculosis, syphilis, and toxoplasma were negative. The patient was diagnosed with Punctate Inner Choroidopathy and received anti-VEGF therapy (Eylea) and oral prednisone with one-week repeat imaging showing resolved pigmented epithelial detachment and edema (Figure 4). VA showed no improvement and intraocular pressure remained within normal limits.

Figure 4: Macula OCT of the left eye showing improvement in PED

Discussion:
Punctate Inner Choroidopathy is a rare cause of posterior uveitis with an estimated incidence of 1.6/1,000,000. Initially described by Watzke et. al. in 1984, this disease typically presents in healthy Caucasian women in their third decade of life with symptoms of blurred central vision, photopsia, metamorphopsia and paracentral scotoma. Exam findings typically include yellow-gray lesions at the junction of the inner choroid and retinal pigmented epithelium (RPE). In the identified cases, most patients presented with bilateral posterior pole involvement without signs of anterior or intermediate ocular inflammation.
PIC has been classified within the spectrum of white dot syndromes – syndromes of idiopathic, white spots located in the retina/choroid. It has been hypothesized that PIC occurs as an autoimmune response following polygenic environmental stimulation from triggers such as infection, immunization, or stress.
The diagnosis of PIC must rule out possible known causes of posterior uveitis including infection, auto-immune disease, trauma, or genetic syndromes. PIC has been separated from other causes of posterior uveitis as having the following features:

⦁ Absence of anterior or intermediate uveitis
⦁ Small punctate lesions involving the choroid and retina of the posterior pole without peripheral involvement
⦁ Retinal scar and CNVM formation

Although the diagnosis is based on clinical and physical findings, imaging has been used to guide management and better understand this disease process. Indocyanine Green Fluorescence has been used to help elucidate subclinical hypofluorescence and choroidal damage typical of this disease. Treatment for PIC, while not well-studied,, varies based on location, severity, progression, and potential impact on vision. Treatment may include observation only, steroids, immunomodulating therapy, Anti-Vascular Endothelial Growth Factor (Anti-VEGF), and photodynamic therapy. Visual outcomes for patients with PIC without choroid neovascular membranes (CNVM) are excellent—greater than 50% of patients end up seeing 20/25 or better. The disease may be self-limiting, lasting a few years, or may persist for decades.

References:

  1. Ahnood, Dana & Madhusudhan, Savitha & D. Tsaloumas, Marie & K. Waheed, Nadia & Keane, Pearse & Denniston, Alastair. (2016). Punctate Inner Choroidopathy: A Review. Survey of Ophthalmology. 62. 10.1016/j.survophthal.2016.10.003.
  2. A. T. Gerstenblith, J. E. Thorne, L. Sobrin et al., “Punctate inner choroidopathy: a survey analysis of 77 persons,” Ophthalmology, vol. 114, no. 6, pp. 1201.e4–1204.e4, 2007.
  3. Brown J, Jr., Folk JC, Reddy CV, et al. Visual prognosis of multifocal choroiditis, punctate inner choroidopathy, and the diffuse subretinal fibrosis syndrome. Ophthalmology 1996; 103: 1100–1105.
  4. F Spaide, Richard & Goldberg, Naomi & Freund, K. (2013). Redefining multifocal choroiditis and panuveitis and punctate inner choroidopathy through multimodal imaging. Retina (Philadelphia, Pa.). 33. 10.1097/IAE.0b013e318286cc77.
  5. Turkcuoglu P, Chang PY, Rentiya ZS, Channa R, Ibrahim M, Hatef E, Sophie R, Sadaka A, Wang J, Sepah YJ, Do DV, Foster CS, Nguyen QD. Mycophenolate mofetil and fundus autofluorescence in the management of recurrent punctate inner choroidopathy. Ocul Immunol Inflamm. 2011 Aug;19(4):286-92
  6. Watzke RC, Packer AJ, Folk JC, Benson WE, Burgess D, Ober RR. Punctate inner choroidopathy. Am J Ophthalmol. 1984;98(5): 572-84
  7. Zhang X, Wen F, Zuo C, Li M, Chen H, Huang S, Luo G. Clinical features of punctate inner choroidopathy in Chinese patients. Retina. 2011 Sep;31(8):1680-91
  8. Zhang X, Zuo C, Li M, Chen H, Huang S, Wen F. Spectral-domain optical coherence tomographic findings at each stage of punctate inner choroidopathy

Faculty Approval by: Marissa Larochelle, MD
Identifier: Moran_CORE_28335
Copyright Statement: Copyright: Braedon Murdock, ©2019. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/