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Show detailsContinuing Education Activity
White-centered retinal hemorrhages, also known as Roth spots, are retinal hemorrhages that can be seen in a variety of medical conditions. Roth spots are most commonly associated with infective endocarditis and have been detected in 80 percent of cases of subacute bacterial endocarditis. They are also seen in association with conditions including leukemia, anemia, hypertensive retinopathy, preeclampsia, diabetic retinopathy, and anoxia. This activity describes the evaluation and management of Roth spots and highlights the role of the interprofessional team in improving care for affected patients.
Objectives:
- Describe the appearance and clinical significance of Roth spots.
- Identify additional evaluations that should be done for patients that present with Roth spots.
- Identify treatment considerations for patients with Roth spots.
- Describe the importance of improving care coordination among the interprofessional team members to identify Roth spots with their common associating diseases and provide treatment of the underlying condition which will enhance the delivery of care.
Introduction
White centered retinal hemorrhages, also known as Roth spots, were first described by Moritz Roth, a Swiss physician in 1872. However, it was not until 1878 that this condition was assigned the name "Roth spot" by Moritz Litten. Litten reported that these white-centered retinal hemorrhages had been detected in 80% of cases associated with subacute bacterial endocarditis. Roth spots are most commonly associated with infective endocarditis. However, they can be seen in a wide variety of medical conditions including leukemia, anemia, hypertensive retinopathy, pre-eclampsia, diabetic retinopathy, and anoxia.[1][2][2][3]
Etiology
Historically, Roth spots were considered to be a pathognomonic finding for bacterial endocarditis secondary to septic emboli within the retina. Given recent histological data, it is now believed that Roth spots are the result of retinal capillary rupture and intraretinal hemorrhage. Roth spots occur in a multitude of disease processes in which retinal blood vessel injury and subsequent bleeding and hemorrhage occur. A common thread found in these conditions is a predisposition for endothelial dysfunction allowing for retinal capillary disruption. Histological examination reveals that the white centered lesions are composed mostly of fibrin, which represents a fibrin-platelet plug at the site of vessel rupture.[4]
Epidemiology
White centered retinal hemorrhages are a nonspecific ophthalmologic finding seen in multiple systemic conditions of various etiologies. Roth spots are seen on fundoscopic examination and tend to be located in the peripheral or posterior poles of the eyes. Retinal endothelial dysfunction appears to be a common thread among the diverse causes of white centered retinal hemorrhage. The differential diagnosis of Roth spots is vast and includes:
- Bacterial endocarditis
- Anemia/thrombocytopenia
- Collagen vascular disease
- Leukemia
- Hypertensive retinopathy
- Diabetic retinopathy
- Pre-eclampsia
- Human Immunodeficiency Virus (HIV)
- Anoxia
- Shaken baby syndrome
Pathophysiology
While multiple theories have been proposed to explain the pathogenesis of white centered retinal hemorrhages, the most widely accepted is that of retinal capillary rupture and intraretinal hemorrhage. Retinal capillary rupture occurs as a result of endothelial cell dysfunction. Extravasation of red blood cells follows vessel rupture with subsequent activation of the clotting cascade and formation of a platelet-fibrin plug at the site of the damaged endothelium. Histological studies revealed the whites lesions are composed primarily of platelet-fibrin thrombi.
History and Physical
Retinal hemorrhage is commonly seen in patients with underlying disease processes that predispose to retinal endothelial dysfunction and rupture. Roth spots may be seen on a routine ophthalmologic exam in asymptomatic patients prompting further diagnosis and evaluation. However, patients may also present with signs and symptoms of systemic illness and retinal hemorrhages noted during acute evaluation. The presence of Roth spots on physical examination should prompt the physician to evaluate for an underlying systemic disease. The clinician should take a thorough history and complete a careful physical exam.[5][6]
A detailed history with a full review of the system can help to narrow the differential diagnosis and identify the underlying disease process. Attention must be paid to symptoms associated with the more common etiologies of Roth spots. Symptoms of endocarditis and infection including fever, chills and night sweats must be reviewed. Symptoms commonly seen in hematologic disease should also be assessed such as unexplained weight loss, fatigue, abnormal bleeding or bruising and shortness of breath. Review of personal past medical history, as well as family history, may also be helpful in determining the etiology.
Roth spots are seen on fundoscopic examination. A complete ophthalmologic examination should be performed as other retinal abnormalities may accompany Roth spots. After Roth spots are identified, the physician must carefully examine the patient to assess for physical signs of systemic illness. It is important to note the patient's vital signs, particularly blood pressure, temperature, and pulse. Several etiologies of white-centered retinal hemorrhages have classical physical examination findings associated with them; therefore, the physician must have a high index of suspension during the examination. In patients that present with concerns for endocarditis and Roth spots, the clinician must carefully examine for other stigmata of endocarditis including Janeway lesions, Osler nodes, splinter hemorrhages, petechiae and cardiac murmurs. Assessment of digital clubbing, the presence or absence of splenomegaly and respiratory abnormalities is also helpful.[7][8]
Evaluation
The initial evaluation is based largely on results of a thorough history and physical examination. A complete blood count with differential to assess for leukocytosis, anemia, and thrombocytopenia along with other hematologic abnormalities should be completed in all patients. A fasting plasma glucose or hemoglobin A1C to assess for diabetes mellitus should be performed. If infectious etiology is suspected erythrocyte sedimentation rate, C-reactive protein and blood cultures may be indicated. A transthoracic echocardiogram is an important initial diagnostic test if endocarditis is suspected. Additional tests such as HIV antibody testing, imaging studies or peripheral blood smear should be considered if indicated based on presenting symptoms and physical examination findings.
Treatment / Management
Roth spots are most often asymptomatic and detected during a routine dilated eye examination. Visual disturbance due to Roth spots is rare but can occur with macular involvement. The majority of white centered retinal hemorrhages will resolve without intervention. Treatment is aimed at the underlying cause and is often supportive. The most important management consideration in patients with Roth spots is the identification of the causative etiology to prevent possible ocular and systemic complications that may occur due to the underlying disease. Follow-up with an ophthalmologist is an important consideration in patients in whom Roth spots are discovered during acute illness. [9]
Differential Diagnosis
- Anaemia
- Anoxia
- CO Poisoning
- Complicated Labor
- Diabetic Retinopathy
- HIV Retinopathy
- Hypertension
- Intracranial Hemorrhage
- Preeclampsia
- Shaken Baby Syndrome
- Subacute Bacterial Endocarditis
Pearls and Other Issues
- Roth spots although classically associated with infective endocarditis are a nonspecific ophthalmologic finding with multiple etiologies.
- Retinal vessel rupture with whole blood extravasation and fibrin plug development at the site of vessel rupture is the mechanism by which Roth spots occur.
- A thorough history and physical examination are paramount in diagnosing the underlying disease and guiding treatment in patients with Roth spots.
- If Roth spots are found on routine ophthalmologic examination referral to a primary care physician for evaluation and work-up of systemic disease is suggested.
- Identification of the underlying etiology is important in patients found to have Roth spots so that management can be guided to prevent any systemic or ocular complications that might occur.
- Roth spots are usually asymptomatic and resolve with treatment of the underlying condition.
Enhancing Healthcare Team Outcomes
When the opthalmologist makes a diagnosis of roth spots, the patient should be referred to other relevant specialists for work up because there are many causes. The initial referral should be to the primary care physician, nurse practitioner and the internist. The evaluation is based largely on results of a thorough history and physical examination. A complete blood count with differential to assess for leukocytosis, anemia, and thrombocytopenia along with other hematologic abnormalities should be completed in all patients. A fasting plasma glucose or hemoglobin A1C to assess for diabetes mellitus should be performed. If infectious etiology is suspected erythrocyte sedimentation rate, C-reactive protein and blood cultures may be indicated. A cardiologist should perform a transthoracic echocardiogram to rule out endocarditis. The infectious disease expert should conduct HIV antibody testing, imaging studies or peripheral blood smear, if indicated based on presenting symptoms and physical examination findings.
References
- 1.
- Wang L, Petrak M, Holz FG, Müller A, Krohne TU. Retinal Hemorrhages in Shaken Baby Syndrome. J Pediatr. 2019 Apr;207:256. [PubMed: 30922497]
- 2.
- Ścisłowicz A, Piejko P, Nowak M, Renke P, Chaniecki P, Rucka A. [Infective endocarditis in a patient with vision disorders]. Pol Merkur Lekarski. 2018 Nov 28;45(269):198-200. [PubMed: 30531670]
- 3.
- Sabrane I, Belkhadir K, Saoudi S, Benchekroun S, El Ikhloufi M, Cherkaoui O. [Roth spots suggestive of myeloid leukemia: Case report]. J Fr Ophtalmol. 2018 Nov;41(9):e423-e424. [PubMed: 30458927]
- 4.
- Zhang J, Chen Y, Yu Z, Liu L. Bilateral Hemorrhagic Retinopathy with Roth Spots in Pediatric-Onset Systemic Lupus Erythematosus and Associated Thrombocytopenia: A Case Report and Review of Literature. Ocul Immunol Inflamm. 2018;26(8):1150-1153. [PubMed: 29020480]
- 5.
- Lin AL, Burnham JM, Pang V, Idowu O, Iyer S. OCULAR MANIFESTATIONS OF PRIMARY MYELOFIBROSIS. Retin Cases Brief Rep. 2016 Fall;10(4):364-7. [PubMed: 26836259]
- 6.
- Furtado JM, Toscano M, Castro V, Rodrigues MW. Roth Spots in Ocular Toxoplasmosis. Ocul Immunol Inflamm. 2016 Oct;24(5):568-70. [PubMed: 26472315]
- 7.
- Singh R, Singh SP, Mittal SK, Rana SK. Roth Spots in Chronic Myeloid Leukaemia. J Assoc Physicians India. 2014 Sep;62(9):836-7. [PubMed: 26259322]
- 8.
- Dell'Arti L, Barteselli G, Pinna V, Invernizzi A, Mapelli C, Viola F. Sudden occurrence of Roth spots and retinal hemorrhages following endoscopic adhesiolysis: an SD-OCT evaluation. Eur J Ophthalmol. 2015 Dec 01;26(1):e11-3. [PubMed: 26428215]
- 9.
- Mahroo OA, Graham EM. Images in clinical medicine. Roth spots in infective endocarditis. N Engl J Med. 2014 Jun 19;370(25):e38. [PubMed: 24941198]
Disclosure: Stephanie Ruddy declares no relevant financial relationships with ineligible companies.
Disclosure: Reece Bergstrom declares no relevant financial relationships with ineligible companies.
Disclosure: Vijai Tivakaran declares no relevant financial relationships with ineligible companies.
- PubMedLinks to PubMed
- Roth Spots: A Rare Finding in Sickle Cell Anemia.[Cureus. 2024]Roth Spots: A Rare Finding in Sickle Cell Anemia.Kothari M, Chiwhane A, Kumar S, Wanjari A, Reddy H. Cureus. 2024 Apr; 16(4):e59047. Epub 2024 Apr 26.
- Roth spots in pernicious anaemia.[BMJ Case Rep. 2011]Roth spots in pernicious anaemia.Macauley M, Nag S. BMJ Case Rep. 2011 Apr 19; 2011. Epub 2011 Apr 19.
- Review Roth spots seen on ophthalmoscopy: diseases with which they may be associated.[Conn Med. 1995]Review Roth spots seen on ophthalmoscopy: diseases with which they may be associated.Falcone PM, Larrison WI. Conn Med. 1995 May; 59(5):271-3.
- White-centred retinal haemorrhages (Roth spots).[Postgrad Med J. 1998]White-centred retinal haemorrhages (Roth spots).Ling R, James B. Postgrad Med J. 1998 Oct; 74(876):581-2.
- Review Retinal lesions as clues to disseminated bacterial and candidal infections: frequency, natural history, and etiology.[Medicine (Baltimore). 2003]Review Retinal lesions as clues to disseminated bacterial and candidal infections: frequency, natural history, and etiology.Rodríguez-Adrián LJ, King RT, Tamayo-Derat LG, Miller JW, Garcia CA, Rex JH. Medicine (Baltimore). 2003 May; 82(3):187-202.
- Roth Spots - StatPearlsRoth Spots - StatPearls
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