Retina Associates’ Case of the Month: April 2013

Apr 13 Colour Montage

Author: Samantha Fraser-Bell

Editor: Adrian Fung

Figure 1.
The right fundus shows subretinal lesions of different ages and sizes. The left fundus was normal.

A 27-year-old female was referred complaining of spots in her right vision.

 Case History

A 27-year-old woman presented complaining of one week of right visual disturbance, with ‘spots in her vision’. Her optometrist referred her after noting creamy subretinal lesions in the right fundus (Figure 1). Past ophthalmic history was unremarkable. She was 30 weeks pregnant, but had no significant medical history and was not taking any medications. There was no history of illicit drug use, nor family history of eye disease. Born in Pakistan, she had lived in Australia for 3 years.

Visual acuities were 6/7.5 right eye (OD) and 6/6 left eye (OS). Intraocular pressures were 12mmHg in each eye. There were 1+ cells in the right anterior chamber but minimal vitritis. The left eye was quiet. Dilated examination of her right fundus revealed creamy subretinal lesions that appeared to radiate out from the optic disc. There was a lesion close to the fovea and several more peripherally. The lesions were of different sizes and ages but not associated with haemorrhage or retinal oedema. The left fundus was normal.

 

What is your diagnosis?

 Differential diagnosis

The differential diagnoses for multifocal subretinal lesions include:

Inflammatory causes

  • Multifocal choroiditis (idiopathic aetiology)
  • Serpiginous choroiditis (idiopathic aetiology)
  • Sarcoidosis

Infective causes

  • Tuberculous serpiginouslike choroiditis
  • Blood borne infections/endogenous endophthalmitis
  • Syphilis
  • Lyme
Additional history and investigations 
Figure 2. Right optical coherence tomography image demonstrating subretinal lesions in the areas corresponding to the creamy white lesions.

Figure 2.
Right optical coherence tomography image demonstrating subretinal lesions in the areas corresponding to the creamy white lesions.

 

Figure 3.Right fundus autofluorescence demonstrating hypo- and hyperautofluorescent lesion

The patient was referred to the local hospital’s chest clinic for Mantoux (tuberculin) tuberculosis (TB) skin testing, which was positive. QuantiFERON TB Gold (>4.0iu/ml) blood test was also positive, consistent with prior exposure to Mycobacterium tuberculosis. Angiotensin-converting enzyme (ACE, for sarcoidosis) was within normal limits and syphilis serology was negative. Full blood count, liver function tests and tests for kidney function were normal. No chest imaging was performed due to the pregnancy.

Tuberculous serpiginouslike choroiditis.

Clinical Course

The patient was referred to the chest clinic at her local hospital for quadruple anti-TB treatment and commenced on oral prednisone (1mg/kg). The prednisone was tapered over 6 months while TB treatment was ongoing. Her vision initially dropped over the first 2 weeks to 6/24 due to expansion of the lesion close to the fovea but returned to 6/7.5 as the choroidal lesions became inactive (Figure 4).

Figure 4.Colour fundus photograph 3 months after presentation. The choroidal lesions are now inactive, and vision had returned to 6/7.5.

 

Discussion

The main differential diagnoses in this case are serpiginous choroiditis1-4 and tuberclulous serpiginouslike choroiditis.5-7 Serpiginous choroiditis is a rare, idiopathic inflammatory disease involving the inner choroid and retinal pigment epithelium. It is usually bilateral, spreading in a snake like (serpiginoid) fashion from the optic nerve. It typically affects middle-aged persons and is progressive with multiple exacerbations. Tuberculous serpinouslike choroidits typically affects younger adults of Asian-Indian origin, is more likely to be multifocal early in the course with vitritis, has a relatively low complication and recurrence rate and is less commonly bilateral. It is usually associated with good visual outcome. Differentiating the tuberculous entity from classic serpiginous choroiditis is critical because the heavy immunosuppressive treatments usually required for serpiginous choroiditis have several potential adverse effects and such treatment may reactivate concomitant tuberculous infection.

A diagnosis of tubercular serpiginouslike choroiditis depends on the appropriate clinical history and signs and evidence of tuberculous infection (such as a positive Mantoux skin test, QuantiFERON gold TB result8 and chest imaging). Entities such as syphilis and sarcoidosis that may similarly involve the retinal pigment epithelium (RPE) and choriocapillaris should be excluded. A positive therapeutic response to antituberculous medication supports the association with Mycobacterium tuberculosis.

As in our patient, the lesions characteristically evolve from an active hyperautofluorecent stage to a healed inactive hypoautofluorescent one.9 Complications of serpiginous and tuberculous serpiginouslike choroiditis include choroidal neovascularisation (13-35% of patients), subretinal fibrosis and RPE atrophy.10,11 In 75% of cases the central fovea is spared, and most eyes return to at least 6/12 vision.

The pathophysiology of presumed tuberculous serpiginouslike choroiditis is unknown. In the absence of active systemic TB infection, a hypersensitivity reaction to Mycobacterium tuberculosis manifesting as inflammation of the RPE, choriocapillaris, and choroid may be present.However, an active infectious component in these cases cannot be ruled out. The finding of tuberculous bacilli at the level of the RPE may indicate this site as a possible sanctuary for dormant bacilli.12 Either reactivation of dormant bacilli or even choroidal seeding due to reactivation elsewhere in the body may lead to local inflammation. This hypothesis of an infectious component is reinforced by the favourable response after initiation of anti-tuberculous drug treatment.

In conclusion, tubercular serpiginouslike choroiditis commonly affects young to middle-aged persons from areas endemic for TB. The lesions usually begin as discrete, multifocal lesions that spread in a serpiginoid pattern and tend to spare the fovea and have a good final visual acuity. Identification of the clinical spectrum of serpiginous choroiditis is of major relevance to target specific tailored investigations and treatment.

 

Take home points
  • Anti-tuberculous treatment and immunosuppression is needed to treat this condition and to reduce recurrence.
  • Active lesions are hyperautofluorescent, but often become inactive and hypoautofluorescent.
  • With prompt treatment, visual prognosis can be good, since in most cases the fovea is spared.

Want to subscribe to the Retina Associates Case of the Month? Click here!

 

References
  1. Lim WK, Buggage RR, Nussenblatt RB. Serpiginous choroiditis. Surv Ophthalmol 2005;50:231-44
  2. Cordero-Coma M, Benito MF, Hernández AM, Antolin SC, Ruíz JM. Serpiginous choroiditis. Ophthalmology 2008;115:1633
  3. Abrez H, Biswas J, Sudharshan S. Clinical profile, treatment, and visual outcome of serpiginous choroiditis. Ocul Immunol Inflamm 2007;15:325-35
  4. Fung AT, Nicolò M, Yzer S, Traverso CE, Yannuzzi LA. Eales dieases associated with serpiginous choroiditis. Arch Ophthal. 2012;130:1484-6
  5. Gupta V, Gupta A, Rao NA. Intraocular tuberculosis—an update. Surv Ophthalmol 2007;52:561-87
  6. Vasconcelos-Santos DV, Rao PK, Davies JB, Sohn EH, Rao NA. Clinical features of tuberculous serpiginous-like choroiditie in contrast to classic serpiginous choroiditis. Arch Ophthal 2010;128:853-8
  7. Bansal R, Gupta A, Gupta V, Dogra MR, Sharma A, Bambery P. Tubercular Serpiginous-Like Choroiditis Presenting as Multifocal Serpiginoid Choroiditis. Ophthalmol 2012;119:2334-42
  8. Mackensen F, Becker MD, Wiehler U, Max R, Dalpke A, Zimmermann S. QuantiFERON TB-Gold—a new test strengthening long-suspected tuberculous involvement in serpiginous-like choroiditis. Am J Ophthalmol 2008;146:761-6
  9. Gupta A, Bansal R, Gupta V, Sharma A. Fundus autofluorescence in serpiginouslike choroiditis. Retina 2012;32:814-25
  10. Christmas NJ, Oh KT, Oh DM. Long-term follow-up of patients with serpiginous choroiditis. Retina 2002;22:550-6
  11. Chisholm IH, Gass JD, Hutton WL. The late stage of serpiginous (geographic) choroiditis. Am J Ophthalmol 1976;82:343-51
  12. Rao NA, Saraswathy S, Smith RE. Tuberculous uveitis: distribution of Mycobacterium tuberculosis in the retinal pigment epithelium. Arch Ophthalmol 2006;124:1777-9

 

Disclaimer

Care has been taken to confirm the accuracy of the information present and to describe generally accepted practices. However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication. Application of this information in a particular situation remains the professional responsibility of the practitioner; the clinical treatments described and recommended may not be considered absolute and universal recommendations.g”> Figure 1.
The right fundus shows subretinal lesions of different ages and sizes. The left fundus was normal.[/caption]

A 27-year-old female was referred complaining of spots in her right vision.

 Case History

A 27-year-old woman presented complaining of one week of right visual disturbance, with ‘spots in her vision’. Her optometrist referred her after noting creamy subretinal lesions in the right fundus (Figure 1). Past ophthalmic history was unremarkable. She was 30 weeks pregnant, but had no significant medical history and was not taking any medications. There was no history of illicit drug use, nor family history of eye disease. Born in Pakistan, she had lived in Australia for 3 years.

Visual acuities were 6/7.5 right eye (OD) and 6/6 left eye (OS). Intraocular pressures were 12mmHg in each eye. There were 1+ cells in the right anterior chamber but minimal vitritis. The left eye was quiet. Dilated examination of her right fundus revealed creamy subretinal lesions that appeared to radiate out from the optic disc. There was a lesion close to the fovea and several more peripherally. The lesions were of different sizes and ages but not associated with haemorrhage or retinal oedema. The left fundus was normal.

 

What is your diagnosis?

 Differential diagnosis

The differential diagnoses for multifocal subretinal lesions include:

Inflammatory causes

  • Multifocal choroiditis (idiopathic aetiology)
  • Serpiginous choroiditis (idiopathic aetiology)
  • Sarcoidosis

Infective causes

  • Tuberculous serpiginouslike choroiditis
  • Blood borne infections/endogenous endophthalmitis
  • Syphilis
  • Lyme
Additional history and investigations 
Figure 2. Right optical coherence tomography image demonstrating subretinal lesions in the areas corresponding to the creamy white lesions.

Figure 2.
Right optical coherence tomography image demonstrating subretinal lesions in the areas corresponding to the creamy white lesions.

 

Figure 3.Right fundus autofluorescence demonstrating hypo- and hyperautofluorescent lesion

The patient was referred to the local hospital’s chest clinic for Mantoux (tuberculin) tuberculosis (TB) skin testing, which was positive. QuantiFERON TB Gold (>4.0iu/ml) blood test was also positive, consistent with prior exposure to Mycobacterium tuberculosis. Angiotensin-converting enzyme (ACE, for sarcoidosis) was within normal limits and syphilis serology was negative. Full blood count, liver function tests and tests for kidney function were normal. No chest imaging was performed due to the pregnancy.

Tuberculous serpiginouslike choroiditis.

Clinical Course

The patient was referred to the chest clinic at her local hospital for quadruple anti-TB treatment and commenced on oral prednisone (1mg/kg). The prednisone was tapered over 6 months while TB treatment was ongoing. Her vision initially dropped over the first 2 weeks to 6/24 due to expansion of the lesion close to the fovea but returned to 6/7.5 as the choroidal lesions became inactive (Figure 4).

Figure 4.Colour fundus photograph 3 months after presentation. The choroidal lesions are now inactive, and vision had returned to 6/7.5.

 

Discussion

The main differential diagnoses in this case are serpiginous choroiditis1-4 and tuberclulous serpiginouslike choroiditis.5-7 Serpiginous choroiditis is a rare, idiopathic inflammatory disease involving the inner choroid and retinal pigment epithelium. It is usually bilateral, spreading in a snake like (serpiginoid) fashion from the optic nerve. It typically affects middle-aged persons and is progressive with multiple exacerbations. Tuberculous serpinouslike choroidits typically affects younger adults of Asian-Indian origin, is more likely to be multifocal early in the course with vitritis, has a relatively low complication and recurrence rate and is less commonly bilateral. It is usually associated with good visual outcome. Differentiating the tuberculous entity from classic serpiginous choroiditis is critical because the heavy immunosuppressive treatments usually required for serpiginous choroiditis have several potential adverse effects and such treatment may reactivate concomitant tuberculous infection.

A diagnosis of tubercular serpiginouslike choroiditis depends on the appropriate clinical history and signs and evidence of tuberculous infection (such as a positive Mantoux skin test, QuantiFERON gold TB result8 and chest imaging). Entities such as syphilis and sarcoidosis that may similarly involve the retinal pigment epithelium (RPE) and choriocapillaris should be excluded. A positive therapeutic response to antituberculous medication supports the association with Mycobacterium tuberculosis.

As in our patient, the lesions characteristically evolve from an active hyperautofluorecent stage to a healed inactive hypoautofluorescent one.9 Complications of serpiginous and tuberculous serpiginouslike choroiditis include choroidal neovascularisation (13-35% of patients), subretinal fibrosis and RPE atrophy.10,11 In 75% of cases the central fovea is spared, and most eyes return to at least 6/12 vision.

The pathophysiology of presumed tuberculous serpiginouslike choroiditis is unknown. In the absence of active systemic TB infection, a hypersensitivity reaction to Mycobacterium tuberculosis manifesting as inflammation of the RPE, choriocapillaris, and choroid may be present.However, an active infectious component in these cases cannot be ruled out. The finding of tuberculous bacilli at the level of the RPE may indicate this site as a possible sanctuary for dormant bacilli.12 Either reactivation of dormant bacilli or even choroidal seeding due to reactivation elsewhere in the body may lead to local inflammation. This hypothesis of an infectious component is reinforced by the favourable response after initiation of anti-tuberculous drug treatment.

In conclusion, tubercular serpiginouslike choroiditis commonly affects young to middle-aged persons from areas endemic for TB. The lesions usually begin as discrete, multifocal lesions that spread in a serpiginoid pattern and tend to spare the fovea and have a good final visual acuity. Identification of the clinical spectrum of serpiginous choroiditis is of major relevance to target specific tailored investigations and treatment.

 

Take home points
  • Anti-tuberculous treatment and immunosuppression is needed to treat this condition and to reduce recurrence.
  • Active lesions are hyperautofluorescent, but often become inactive and hypoautofluorescent.
  • With prompt treatment, visual prognosis can be good, since in most cases the fovea is spared.

Want to subscribe to the Retina Associates Case of the Month? Click here!

 

References
  1. Lim WK, Buggage RR, Nussenblatt RB. Serpiginous choroiditis. Surv Ophthalmol 2005;50:231-44
  2. Cordero-Coma M, Benito MF, Hernández AM, Antolin SC, Ruíz JM. Serpiginous choroiditis. Ophthalmology 2008;115:1633
  3. Abrez H, Biswas J, Sudharshan S. Clinical profile, treatment, and visual outcome of serpiginous choroiditis. Ocul Immunol Inflamm 2007;15:325-35
  4. Fung AT, Nicolò M, Yzer S, Traverso CE, Yannuzzi LA. Eales dieases associated with serpiginous choroiditis. Arch Ophthal. 2012;130:1484-6
  5. Gupta V, Gupta A, Rao NA. Intraocular tuberculosis—an update. Surv Ophthalmol 2007;52:561-87
  6. Vasconcelos-Santos DV, Rao PK, Davies JB, Sohn EH, Rao NA. Clinical features of tuberculous serpiginous-like choroiditie in contrast to classic serpiginous choroiditis. Arch Ophthal 2010;128:853-8
  7. Bansal R, Gupta A, Gupta V, Dogra MR, Sharma A, Bambery P. Tubercular Serpiginous-Like Choroiditis Presenting as Multifocal Serpiginoid Choroiditis. Ophthalmol 2012;119:2334-42
  8. Mackensen F, Becker MD, Wiehler U, Max R, Dalpke A, Zimmermann S. QuantiFERON TB-Gold—a new test strengthening long-suspected tuberculous involvement in serpiginous-like choroiditis. Am J Ophthalmol 2008;146:761-6
  9. Gupta A, Bansal R, Gupta V, Sharma A. Fundus autofluorescence in serpiginouslike choroiditis. Retina 2012;32:814-25
  10. Christmas NJ, Oh KT, Oh DM. Long-term follow-up of patients with serpiginous choroiditis. Retina 2002;22:550-6
  11. Chisholm IH, Gass JD, Hutton WL. The late stage of serpiginous (geographic) choroiditis. Am J Ophthalmol 1976;82:343-51
  12. Rao NA, Saraswathy S, Smith RE. Tuberculous uveitis: distribution of Mycobacterium tuberculosis in the retinal pigment epithelium. Arch Ophthalmol 2006;124:1777-9

 

Disclaimer

Care has been taken to confirm the accuracy of the information present and to describe generally accepted practices. However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication. Application of this information in a particular situation remains the professional responsibility of the practitioner; the clinical treatments described and recommended may not be considered absolute and universal recommendations.