POSTSURGICAL BACTERIAL ENDOPHTHALMITIS PRESENTING AS FROSTED BRANCH ANGIITIS: A CASE REPORT Dhanashree Ratra, MD, FRCSEd,* Shafiq Jafferji, MD,* Jyotirmay Biswas, MD†
Purpose: To report a case of unilateral postsurgical bacterial endophthalmitis, which presented in an unusual manner with frosted branch angiitis. Methods: We reviewed the chart of a patient with acute postoperative bacterial endophthalmitis who presented initially as frosted branch angiitis and subsequently developed full-fledged endophthalmitis. Results: The patient was treated with conventional treatment for postsurgical endophthalmitis along with oral steroids. He recovered full vision with complete resolution of the frosted branch appearance as well as the endophthalmitis. Discussion: Primary frosted branch angiitis is a rare form of vasculitis seen in otherwise healthy young children. It has also been reported secondary to autoimmune disorders, malignancies such as lymphoma, and various infective agents. Commonly associated infective agents are viruses such as cytomegalovirus, herpes simplex, varicella zoster, and human immunodeficiency virus. There are isolated case reports of associated rubella and toxoplasma infections. One case has been reported in association with endogenous Fusarium endophthalmitis. No case has been reported in association with bacterial endophthalmitis. RETINAL CASES & BRIEF REPORTS 4:20 –22, 2010
From the *Department of Vitreoretinal Diseases; and †Department of Ocular Pathology, Medical Research Foundation, Sankara Nethralaya, Chennai, India.
unaided vision of 20/25. Forty-eight hours later, he presented with sudden diminution in vision to counting fingers at 1 m with mild ocular pain. Anterior segment examination was unremarkable with occasional cells in the vitreous cavity. Fundus examination showed perivascular sheathing of the veins resembling FBA with mild disk pallor, one fluffy exudate over the disk, and a few intraretinal hemorrhages (Figure 1). A diagnosis of FBA was made and high-dose oral steroids were started. A fluorescein angiography was advised; however, within a span of 2 hours, the vitreous exudates increased rapidly obscuring fundus details. Fluorescein angiography could not be done. He was referred for further specialized management. At this juncture, examination of his right eye revealed a vision of light perception, corneal haze of 2⫹, 2.5-mm hypopyon, patent YAG peripheral iri-
W
e report a case of acute postoperative bacterial endophthalmitis presenting initially as frosted branch angiitis (FBA) and subsequently leading to full-fledged endophthalmitis. A 32-year-old well controlled diabetic of Asian origin who was otherwise healthy underwent uneventful phakic intraocular lens surgery for the correction of myopia in the right eye. A similar procedure was done in the left eye 2 weeks earlier. He was noted to be doing well on the first postoperative day with
Reprint requests: Dhanashree Ratra, MD, FRCSEd, Bhagavan Mahavir Department of Vitreoretinal Services, Medical Research Foundation, Sankara Nethralaya, 18, College Road, Chennai 600006, India; e-mail: [email protected]
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FROSTED BRANCH ANGIITIS IN ENDOPHTHALMITIS
Fig. 1. Fundus picture showing perivascular sheathing typical of frosted branch angiitis and early vitreous exudates.
Fig. 2. Normal fundus with complete resolution of frosted branch angiitis as well as endophthalmitis at 6 weeks.
dotomy, and an exudative membrane over the pupillary area. Ultrasound examination showed multiple low to moderate reflective dot-like echoes in the vitreous cavity. An impression of rapidly progressing severe endophthalmitis was made. The patient underwent urgent lensectomy with core vitrectomy and intraocular lens removal. Intraoperative visualization was poor as a result of corneal edema; however, the frosted branch appearance of the perivascular sheathing was noted. The vitreous sample was subjected to routine microbiologic tests. Additional polymerase chain reaction tests for viruses were ordered in view of the frosted branch picture. Methicillin-resistant Staphylococcus epidermidis was grown from the vitreous sample. Culture for fungus was negative. However, polymerase chain reaction for cytomegalovirus (CMV) was positive. Polymerase chain reaction was also done for herpes simplex virus and varicella zoster virus, which was negative. The patient was thoroughly investigated for immunosuppresion. However, he was found to be immunocompetent with HIV-1 and -2 both being negative and CD 4 and 8 counts within normal limits. His white blood cell count was mildly elevated at 11,600 cells/mm3 with a neutrophilic picture consistent with a possible bacterial pathology. The lymphocyte morphology was reported as normal. Titers for toxoplasmosis and cryptococcus were within normal range. He was treated with intravitreal and systemic antibiotics. The oral steroids were continued in a tapering fashion. Subsequently, the hypopyon cleared. A revitrectomy was done to remove the residual cortical matter and peripheral vitreous exudates. At 6 weeks, the visual acuity was 20/20 with a correction of ⫹3.50 D. The fundus was normal with complete resolution of the sheathing (Figure 2).
Discussion Frosted branch angiitis is increasingly being recognized in diverse clinical settings making it unclear whether it is a distinct clinical syndrome as first described by Ito et al1 or merely a clinical sign seen in various inflammatory and infective conditions. Kleiner has in fact suggested three separate subgroups of patients with the similar retinal appearance of FBA.2 First is the group of patients with lymphoma or leukemia who have a frosted branch-like appearance resulting from infiltration with malignant cells. Second is the group of patients who have associated viral infections or autoimmune disease, which produce the clinical signs of FBA resulting from immune complex deposition. Third is the group of otherwise healthy patients described initially. In this group also, the typical onset of FBA after a multifactorial prodromal illness has led to the suggestion of a hypersensitivity reaction to various infective agents, which may initiate FBA through a common pathway, possibly of immune complex deposition.3 Its association with autoimmune disorders such as Crohn disease, sarcoidosis, systemic lupus erythematosus, and rapid resolution with systemic steroids further corroborates the hypothesis of immune complex deposition. However, Biswas et al4 have reported a case of FBA associated with CMV retinitis in a patient with acquired immunodeficiency syndrome, which resolved with antiviral therapy. No steroids were given as a result of concurrent multiple infections. The FBA showed causal relationship with the CMV retinitis, reappearing with relapse of the retinitis. The vascular sheathing was thought to be caused directly by CMV. Only one case of endophthalmitis with FBA has been reported.5 The causative agent was Fusarium
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dimerium. The authors noted that the vitreous exudates were aligned along the vessels giving the frosted branch appearance. These exudates were easily peeled off during posterior vitreous excision. In our case, however, the perivascular exudates did not peel off along with vitrectomy. However, more aggressive attempts were not made as a result of poor visualization during surgery. By the time of revitrectomy, the vascular sheathing had completely disappeared. No consistent etiologic agent has been identified. A presumed viral illness has preceded the onset in 33% of cases.3 The most common association has been with CMV. However, positive serology results have been reported for herpes simplex, varicella zoster, Epstein-Barr virus, measles, rubella.6 Bacterial agents have been rarely seen in association with FBA. Tuberculoprotein and antistreptolysin O were thought to be associated by the early reporters.3 In our patient, methicillin-resistant Staphylococcus epidermidis was grown from the vitreous sample. It is possible the bacterial infection may have initiated this pathway in our case. We therefore propose a possibility of the same pathway being initiated by bacterial antigens other than those previously proposed. Interestingly, in our patient, polymerase chain reaction done on the vitreous sample was positive for CMV. However, on thorough investigations, no evidence of immunosuppression was found. Cytomegalovirus is the most frequent cause of infectious retinitis in patients with
●
2010
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VOLUME 4
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AIDS but is rare in immunocompetent individuals.4 Because the clinical picture was not consistent with the CMV etiology, we chose not to administer ganciclovir. The present case showed a typical clinical picture of FBA with complete resolution of perivascular sheathing and recovery of normal vision. Frosted branch angiitis has so far not been reported to be associated with bacterial endophthalmitis. Our patient tested positive for methicillin-resistant S. epidermidis in the culture and he responded very well to the combined therapy. Key words: bacterial endophthalmitis, frosted branch angiitis, vitrectomy. References 1. 2. 3. 4.
5.
6.
Ito Y, Nakano M, Kyu N, Takeuchi M. Frosted-branch angiitis in child. Jpn J Clin Ophthalmol 1976;30:797– 803. Kleiner RC. Frosted branch angiitis: clinical syndrome or clinical sign? Retina 1997;17:370 –372. Walker S, Iguchi A, Jones NP. Frosted branch angiitis: a review. Eye 2004;18:527–533. Biswas J, Raizada S, Gopal L, Kumarasamy N, Solomon S. Bilateral frosted branch angiitis and cytomegalovirus retinitis in acquired immunodeficiency syndrome. Indian J Ophthalmol 1999;47:195–197. Gabriele P, Hutchins RK. Fusarium endophthalmitis in an intravenous drug abuser. Am J Ophthalmol 1996;122:119 – 121. Biswas J, Fogla R, Madhavan HN. Bilateral frosted branch angiitis in an 8-year old Indian girl. Retina 1996;16:444 – 445.
Purpose: To report a case of unilateral postsurgical bacterial endophthalmitis, which presented in an unusual manner with frosted branch angiitis. Methods: We reviewed the chart of a patient with acute postoperative bacterial endophthalmitis who presented initially as frosted branch angiitis and subsequently developed full-fledged endophthalmitis. Results: The patient was treated with conventional treatment for postsurgical endophthalmitis along with oral steroids. He recovered full vision with complete resolution of the frosted branch appearance as well as the endophthalmitis. Discussion: Primary frosted branch angiitis is a rare form of vasculitis seen in otherwise healthy young children. It has also been reported secondary to autoimmune disorders, malignancies such as lymphoma, and various infective agents. Commonly associated infective agents are viruses such as cytomegalovirus, herpes simplex, varicella zoster, and human immunodeficiency virus. There are isolated case reports of associated rubella and toxoplasma infections. One case has been reported in association with endogenous Fusarium endophthalmitis. No case has been reported in association with bacterial endophthalmitis. RETINAL CASES & BRIEF REPORTS 4:20 –22, 2010
From the *Department of Vitreoretinal Diseases; and †Department of Ocular Pathology, Medical Research Foundation, Sankara Nethralaya, Chennai, India.
unaided vision of 20/25. Forty-eight hours later, he presented with sudden diminution in vision to counting fingers at 1 m with mild ocular pain. Anterior segment examination was unremarkable with occasional cells in the vitreous cavity. Fundus examination showed perivascular sheathing of the veins resembling FBA with mild disk pallor, one fluffy exudate over the disk, and a few intraretinal hemorrhages (Figure 1). A diagnosis of FBA was made and high-dose oral steroids were started. A fluorescein angiography was advised; however, within a span of 2 hours, the vitreous exudates increased rapidly obscuring fundus details. Fluorescein angiography could not be done. He was referred for further specialized management. At this juncture, examination of his right eye revealed a vision of light perception, corneal haze of 2⫹, 2.5-mm hypopyon, patent YAG peripheral iri-
W
e report a case of acute postoperative bacterial endophthalmitis presenting initially as frosted branch angiitis (FBA) and subsequently leading to full-fledged endophthalmitis. A 32-year-old well controlled diabetic of Asian origin who was otherwise healthy underwent uneventful phakic intraocular lens surgery for the correction of myopia in the right eye. A similar procedure was done in the left eye 2 weeks earlier. He was noted to be doing well on the first postoperative day with
Reprint requests: Dhanashree Ratra, MD, FRCSEd, Bhagavan Mahavir Department of Vitreoretinal Services, Medical Research Foundation, Sankara Nethralaya, 18, College Road, Chennai 600006, India; e-mail: [email protected]
20
21
FROSTED BRANCH ANGIITIS IN ENDOPHTHALMITIS
Fig. 1. Fundus picture showing perivascular sheathing typical of frosted branch angiitis and early vitreous exudates.
Fig. 2. Normal fundus with complete resolution of frosted branch angiitis as well as endophthalmitis at 6 weeks.
dotomy, and an exudative membrane over the pupillary area. Ultrasound examination showed multiple low to moderate reflective dot-like echoes in the vitreous cavity. An impression of rapidly progressing severe endophthalmitis was made. The patient underwent urgent lensectomy with core vitrectomy and intraocular lens removal. Intraoperative visualization was poor as a result of corneal edema; however, the frosted branch appearance of the perivascular sheathing was noted. The vitreous sample was subjected to routine microbiologic tests. Additional polymerase chain reaction tests for viruses were ordered in view of the frosted branch picture. Methicillin-resistant Staphylococcus epidermidis was grown from the vitreous sample. Culture for fungus was negative. However, polymerase chain reaction for cytomegalovirus (CMV) was positive. Polymerase chain reaction was also done for herpes simplex virus and varicella zoster virus, which was negative. The patient was thoroughly investigated for immunosuppresion. However, he was found to be immunocompetent with HIV-1 and -2 both being negative and CD 4 and 8 counts within normal limits. His white blood cell count was mildly elevated at 11,600 cells/mm3 with a neutrophilic picture consistent with a possible bacterial pathology. The lymphocyte morphology was reported as normal. Titers for toxoplasmosis and cryptococcus were within normal range. He was treated with intravitreal and systemic antibiotics. The oral steroids were continued in a tapering fashion. Subsequently, the hypopyon cleared. A revitrectomy was done to remove the residual cortical matter and peripheral vitreous exudates. At 6 weeks, the visual acuity was 20/20 with a correction of ⫹3.50 D. The fundus was normal with complete resolution of the sheathing (Figure 2).
Discussion Frosted branch angiitis is increasingly being recognized in diverse clinical settings making it unclear whether it is a distinct clinical syndrome as first described by Ito et al1 or merely a clinical sign seen in various inflammatory and infective conditions. Kleiner has in fact suggested three separate subgroups of patients with the similar retinal appearance of FBA.2 First is the group of patients with lymphoma or leukemia who have a frosted branch-like appearance resulting from infiltration with malignant cells. Second is the group of patients who have associated viral infections or autoimmune disease, which produce the clinical signs of FBA resulting from immune complex deposition. Third is the group of otherwise healthy patients described initially. In this group also, the typical onset of FBA after a multifactorial prodromal illness has led to the suggestion of a hypersensitivity reaction to various infective agents, which may initiate FBA through a common pathway, possibly of immune complex deposition.3 Its association with autoimmune disorders such as Crohn disease, sarcoidosis, systemic lupus erythematosus, and rapid resolution with systemic steroids further corroborates the hypothesis of immune complex deposition. However, Biswas et al4 have reported a case of FBA associated with CMV retinitis in a patient with acquired immunodeficiency syndrome, which resolved with antiviral therapy. No steroids were given as a result of concurrent multiple infections. The FBA showed causal relationship with the CMV retinitis, reappearing with relapse of the retinitis. The vascular sheathing was thought to be caused directly by CMV. Only one case of endophthalmitis with FBA has been reported.5 The causative agent was Fusarium
22
RETINAL CASES & BRIEF REPORTSℜ
dimerium. The authors noted that the vitreous exudates were aligned along the vessels giving the frosted branch appearance. These exudates were easily peeled off during posterior vitreous excision. In our case, however, the perivascular exudates did not peel off along with vitrectomy. However, more aggressive attempts were not made as a result of poor visualization during surgery. By the time of revitrectomy, the vascular sheathing had completely disappeared. No consistent etiologic agent has been identified. A presumed viral illness has preceded the onset in 33% of cases.3 The most common association has been with CMV. However, positive serology results have been reported for herpes simplex, varicella zoster, Epstein-Barr virus, measles, rubella.6 Bacterial agents have been rarely seen in association with FBA. Tuberculoprotein and antistreptolysin O were thought to be associated by the early reporters.3 In our patient, methicillin-resistant Staphylococcus epidermidis was grown from the vitreous sample. It is possible the bacterial infection may have initiated this pathway in our case. We therefore propose a possibility of the same pathway being initiated by bacterial antigens other than those previously proposed. Interestingly, in our patient, polymerase chain reaction done on the vitreous sample was positive for CMV. However, on thorough investigations, no evidence of immunosuppression was found. Cytomegalovirus is the most frequent cause of infectious retinitis in patients with
●
2010
●
VOLUME 4
●
NUMBER 1
AIDS but is rare in immunocompetent individuals.4 Because the clinical picture was not consistent with the CMV etiology, we chose not to administer ganciclovir. The present case showed a typical clinical picture of FBA with complete resolution of perivascular sheathing and recovery of normal vision. Frosted branch angiitis has so far not been reported to be associated with bacterial endophthalmitis. Our patient tested positive for methicillin-resistant S. epidermidis in the culture and he responded very well to the combined therapy. Key words: bacterial endophthalmitis, frosted branch angiitis, vitrectomy. References 1. 2. 3. 4.
5.
6.
Ito Y, Nakano M, Kyu N, Takeuchi M. Frosted-branch angiitis in child. Jpn J Clin Ophthalmol 1976;30:797– 803. Kleiner RC. Frosted branch angiitis: clinical syndrome or clinical sign? Retina 1997;17:370 –372. Walker S, Iguchi A, Jones NP. Frosted branch angiitis: a review. Eye 2004;18:527–533. Biswas J, Raizada S, Gopal L, Kumarasamy N, Solomon S. Bilateral frosted branch angiitis and cytomegalovirus retinitis in acquired immunodeficiency syndrome. Indian J Ophthalmol 1999;47:195–197. Gabriele P, Hutchins RK. Fusarium endophthalmitis in an intravenous drug abuser. Am J Ophthalmol 1996;122:119 – 121. Biswas J, Fogla R, Madhavan HN. Bilateral frosted branch angiitis in an 8-year old Indian girl. Retina 1996;16:444 – 445.
