Pigmented paravenous retinochoroidal atrophy is an uncommon fundus disorder with a striking clinical presentation. [ncbi.nlm.nih.gov]

In the present case the visual acuity at presentation was poor caused by the macular involvement. There was no evidence of ocular inflammation. [ojoonline.org]

ORPHA:251295 Synonym(s): PPRCA Prevalence: Inheritance: Autosomal dominant or Not applicable Age of onset: All ages ICD-10: H35.5 OMIM: 172870 UMLS: C1868310 MeSH: - GARD: - MedDRA: - The documents contained in this web site are presented for information [orpha.net]

• Asymptomatic

  The maculas are spared of clinically observable abnormalities, and the vast majority of patients with this disorder are asymptomatic. [ncbi.nlm.nih.gov]

  […] commonly bilateral and symmetric retinal disease characterized by non-progressive or slowly progressive chorioretinal atrophy, peripapillary pigmentary changes and accumulation of ''bone-corpuscle'' pigmentation along the retinal veins and which is usually asymptomatic [orpha.net]

  These patients are often asymptomatic, but with careful testing, scotomas corresponding to the areas of degeneration can be identified. Typically, the ERG is minimally reduced and disease is thought to be, at most, slowly progressive. [aibolita.com]

• Pallor

  Note the pallor of the optic nerve, the vascular attenuation, atrophy of the pigment epithelium, and bone-spicules. Reproduced from Weleber, R. G., Butler, N. S., Murphey, W. H., Sheffield, V. C., and Stone, E. M. (1997). [aibolita.com]

  Figure 1: Fundus photograph of the right eye showing waxy pallor of the disc, attenuated arterioles, and bony spicule pigmentation characteristic of retinitis pigmentosa (a). [ijo.in]

  Fundus examination of the right eye showed clear ocular media and a normal optic disc without pallor. There was no vascular attenuation (Figure 1 and 2). [westcoastretina.com]

  Macular displacement Macular halo Macular infarction Macular pigment deposit Macular subretinal fibrosis Macular traction syndrome Nerve fiber layer infarct Neurocutaneous syndrome Noncystic peripheral retinal tuft On examination - retinal pigmentation Pallor [icd9data.com]

  The classic clinical triad of RP is arteriolar attenuation, retinal pigmentary changes (could be either hypopigmentation and/or hyperpigmentation in form of bone-spicule and pigment clumpings), and waxy disc pallor. [picuki.com]

• Alopecia

  PPCA was first described in 1937 in a 47 year old man with alopecia areata whose fundus was incidentally noted to have the increased pigmentation along peripheral veins associated with chorioretinal atrophy. 1 The appearance was termed retinochoroiditis [westcoastretina.com]

  The initial case was a 47 year old male with past exposure of tuberculosis (multiple family members) was admitted for alopecia areata. [eyewiki.org]

• Angiomatosis

  Convert to ICD-10-CM : 362.89 converts approximately to: 2015/16 ICD-10-CM H35.89 Other specified retinal disorders Approximate Synonyms Acute retinal necrosis Angiomatosis of retina Atrophic macular change Bull's eye maculopathy Carcinoma-associated [icd9data.com]

• Retinal Pigmentation

  Funduscopic examination revealed bone-spicule pigmentation and choroidal and retinal pigment epithelium atrophy along the vascular arcades, with macular involvement in right eye. [ncbi.nlm.nih.gov]

  Keywords Public Health Genetic Study Retinal Pigment Epithelium Retinal Pigment Pigment Epithelium These keywords were added by machine and not by the authors. [link.springer.com]

• Night Blindness

  The left eye of the same patient revealed typical PPRCA phenotype, with no night blindness, normal field, and normal ERG. RP and PPRCA phenotypes are part of the same spectrum of genetic disorder. [ncbi.nlm.nih.gov]

  As a consequence, by the time the patient recognizes the symptom of night blindness, a reduction in cone sensitivity can be significant. [institut-vision.org]

  However, unlike this case, loss of visual acuity and photopsia within the scotomata are common ocular symptoms associated with AZOOR patients, and night blindness and progressive visual field loss are observed in RP patients, respectively. [benthamopen.com]

• Blurred Vision

  The majority of patients are asymptomatic at presentation or have mild blurred vision. The exception is patients with macular involvement, where vision can be severely reduced on presentation. [eyewiki.org]

• Suggestibility

  Our patients, and the preponderance of males in previously reported cases of this disease, suggest the possibility of Y-chromosome mediated male-to-male transmission. [ncbi.nlm.nih.gov]

• Irritability

  Mechanical centurion syndrome A 73-year-old woman with epiphora and ocular irritation resistant to medical treatment. The lacrimal pathway was permeable and there was no eyelid malposition. [infona.pl]

Systemic workup revealed QuantiFERON-gold positive. This is the first report of unilateral PPRCA secondary to presumed ocular tuberculosis. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:345-349.]. [ncbi.nlm.nih.gov]

Results of laboratory studies, including a vasculitis workup, were within normal limits. A tuberculin skin testing result was 20 mm, erythrocyte sedimentation rate was 23 mm/hr, and a Quantiferon TB gold test (Qiagen) result was normal. [retinatoday.com]

Laboratory test Laboratory studies should be aimed towards narrowing the differential diagnosis depending on the presentation and given the overlap with inflammatory degenerative diseases a workup can include: comprehensive metabolic panel, complete and [eyewiki.org]

We referred the patient for a medical workup and asked her to return in six to eight weeks for close observation. The patient returned two years later with complaints of vision loss OS (20/100; OD remained 20/20). [reviewofoptometry.com]

No effective treatment is available. [ncbi.nlm.nih.gov]

276 1552 Symptomatic Treatment 277 156 Differential Diagnosis 278 1563 Congenital Fibrosis of the Extraocular Muscles 279 Treatment of Specific Types of Nystagmus 283 161 Introduction 284 16211 Etiology 286 16212 Therapeutic Recommendations 287 1622 [books.google.com]

Treatment Treatment Options: No effective treatment is available although low vision aids are likely to be helpful in selected patients. References References Tsang SH, Burke T, Oll M, Yzer S, Lee W, Xie YA, Allikmets R. [disorders.eyes.arizona.edu]

Treatment - monotherapy: ciclosporin or triple therapy: systemic steroids, azathioprine and ciclosporin; stem cell treatment [ 10 ]. [patient.info]

The visual prognosis for patients with pigmented paravenous retinochoroidal atrophy is quite good. [ncbi.nlm.nih.gov]

Prognosis - this condition recurs over months and frequent foveal involvement gives rise to a relatively poor prognosis. Multiple evanescent white dot syndrome (MEWDS) Nature - uncommon, idiopathic and self-limiting condition. [patient.info]

Prognosis Patients with PPRCA have good visual prognosis with slowly progressive disease. In patients without macular involvement, visual acuity is minimally adversely affected. [eyewiki.org]

Ischemic Visual Loss 235 1335 Orbital Manifestations 236 1343 LargeVessel Vasculitis 237 135 Laboratory Investigations in GCA 238 1352 CReactive Protein 239 1355 Anemia 240 1361 Temporal Artery Biopsy 241 1363 Role of Ultrasound 243 137 Treatment and Prognosis [books.google.com]

WFAF may also be helpful in determining prognosis in patients with AMD, although this needs confirmation in larger trials (i.e., AREDS2). [reviewofophthalmology.com]

Etiology The etiology of PPRCA remains unknown with a presumed initial inflammatory insult that leads to the slow developmental of the retinal findings. [eyewiki.org]

The etiology in this case could be inflammatory, infectious, or both. To the best of our knowledge after an extensive literature search, PPCRA with vasculitis has not been previously reported. [retinatoday.com]

Although earlier reports have suggested a possible post-inflammatory etiology such as tuberculosis, rubeola and syphilis as possible causes, a definite etiology remains elusive. [2] Typically, bone corpuscle pigmentation is seen along the distribution [ojoonline.org]

The cause of the condition may be unknown or idiopathic, although a dysgenetic, degenerative, hereditary etiology or even an inflammatory cause has been hypothesized. [ingentaconnect.com]

2013/61/10/586/121083 PPCRA is an uncommon, asymptomatic, slowly progressive disease of unknown etiology, although some authors have attributed it to be the sequela of ocular inflammation. [ijo.in]

Questions involved clinicians' perceptions of the epidemiology, diagnosis, treatment and prophylaxis of invasive candidosis. P < 0.0 Rev. Soc. Bras. Med. Trop.. Publicado em: 23/07/2013 3. [academicoo.com]

Ocular symptoms were analyzed using a validated ocular surface... article Adenoviral keratoconjunctivitis: An update The objective of this review is to describe the clinical and epidemiological characteristics of adenoviral conjunctivitis, as well as [infona.pl]

Relevant External Links for CRB1 Genetic Association Database (GAD) CRB1 Human Genome Epidemiology (HuGE) Navigator CRB1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: CRB1 No data available for Genatlas for CRB1 Gene Clinical and molecular [genecards.org]

BACKGROUND Describe clinical and epidemiological characteristics of pediatric patients diagnosed with acute diarrhea and shock, admitted to the pediatric intensive care unit, in order to compare the evolution of clinical data between the survival and [pe.anoxia.us]

This case might provide preliminary information regarding the pathophysiology of PPRCA and improve our understanding of the nature of this disease. [ncbi.nlm.nih.gov]

Offers the most comprehensive content available on retina, balancing the latest scientific research and clinical correlations, covering everything you need to know on retinal diagnosis, treatment, development, structure, function, and pathophysiology. [books.google.com]

[…] ophthal #medicine #surgery #eyespecialist #eyemedicine #eyesurgeon #myophthalmologyresidency Retinitis Pigmentosa (RP) The term retinitis pigmentosa (RP) is semantically inaccurate: inflammation (implied by the suffix -itis) is not a prominent part of the pathophysiology [picuki.com]

Prevention the secondary degeneration of cones in RP represents a very promising approach. This because such a therapy could be applied in a wide range of mutations expressed in rods, and even at late stages of the disease. [institut-vision.org]

BACKGROUND AND OBJECTIVES The presence of adverse reactions, inherent to all treatments, justifies the necessity of deep knowledge, by the medical team of the prevention and treatment of occasional organic dysfunctions, reducing its impact. [pe.anoxia.us]