Endophthalmitis is an extremely infrequent complication after intraocular surgery and an even more rare complication after strabismus surgery, with a reported incidence as low as 1:3,500 to 1:185,000.1,2
Due to the low incidence, the most effective management of endophthalmitis following strabismus surgery has yet to be elucidated. When left untreated, endophthalmitis can progress to panophthalmitis, meningitis, and even death.3 Typical treatment options include antibiotics (eg, topical, intravitreal, or systemic) and vitrectomy. Despite aggressive treatment, the visual prognosis of these patients is poor and most cases result in eventual enucleation.4
We present the case of a 9-month-old male infant with a history of Down syndrome who developed leukocoria 2 weeks after bilateral medial rectus recession. We discuss the presentation, diagnosis, management, and treatment of this case of a histopathologically confirmed scleral perforation leading to endophthalmitis.
A 9-month-old male infant with a history of Down syndrome and no other significant medical history underwent bilateral medial rectus recession for intermittent alternating esotropia. The surgery was uneventful and no complications were noted on the operative report or on verbal recollection by the surgeon. Postoperatively, the patient was given moxifloxacin (Vigamox; Novartis AG, Basal, Switzerland) eye drops four times a day in both eyes, which is the typical regimen used after strabismus surgery. One week after the surgery, the patient was diagnosed as having a pyogenic granuloma of his left eye (Figure 1). This resolved after 1 week of treatment with prednisolone acetate 1% ophthalmic eye four times a day.
External photograph of the left eye showing a medical conjunctival swelling 1 week after medial rectus recession.
Subsequently, the family noted that the child had abnormal red reflex in his left eye. He returned to his operating surgeon, where he was noted to have leukocoria on examination and transferred to the Children's Medical Center for further evaluation.
On arrival, he was noted to have absence of redness, irritation, or drainage from either eye. A review of systems was negative for cough, fevers, chills, vomiting, diarrhea, eyelid swelling, lethargy, altered mental status, or rashes. His vision in the right eye was central, steady, and maintained with appropriate fix and follow. His left eye had no light perception visual acuity. Pupillary examination demonstrated a relative afferent pupillary defect of the left eye.
The patient underwent examination under anesthesia. Intraocular pressure was 12 and 8 mm Hg in the right and left eyes, respectively. Slit-lamp examination of his right eye was unremarkable aside from iris Brushfield spots (Figure 2A). His left eye was noted to have trace conjunctival injection, a quiet anterior chamber with posterior synechiae at the 2-o'clock position, engorged iris vessels, Brushfield spots, and a white opacity posterior to the lens (Figures 2B).
Anterior segment photograph of (A) the right eye showing iris Brushfield spots and (B) the left eye showing iris Brushfield spots, engorged iris vessels, and a prominent retrolental white mass.
On dilated eye examination, the right fundus was noted to have a small, horseshoe-shaped subretinal lesion in the post-equatorial region of the nasal periphery and five additional pinpoint discrete lesions 1 to 2 mm posterior to the ora (Figure 3A). None of these lesions were associated with retinal hemorrhages, necrosis, or overlying vitritis. The fundus of the left eye demonstrated a white, creamy mass with numerous undulations. Necrosis and hemorrhages in the retrolental space were present with no obvious retinal vessels observed (Figure 3B). An ultrasound examination found extensive retinal detachment and multiple areas of loculated debris with one small hyperechoic area centrally concerning for calcification within his left eye.
Fundus photographs of (A) the right eye showing periphery with one large horseshoe-shaped subretinal lesion and multiple, smaller discrete white lesions, and (B) the left eye showing a white, creamy mass with areas of hemorrhage filling the retrolental space.
To further delineate the etiology of the left eye intraocular mass, magnetic resonance imaging of the orbits with and without contrast were completed. This revealed an irregular noncalcified lesion within the left vitreous body abrupting the posterior margin of the lens, a shallow anterior chamber, and adhesions from the inflammatory mass to the optic nerve head and medical ocular sclera (Figure 4). The post-contrast magnetic resonance image showed thickening of the uvea and intense pathologic enhancement within the anterior chamber, vitreous humor, and choroid. There were no signal abnormalities within the optic nerve.
Axial T2 magnetic resonance imaging scan of the orbits demonstrating a bright lesion within the left vitreous body and adhesions from this lesion to the medical sclera.
The clinical picture was suggestive of endophthalmitis in the left eye. However, the possibility of retinoblastoma could not be conclusively excluded. The patient was admitted for initiation of broad-spectrum intravenous antibiotics. Although the eye remained white and comfortable 48 hours later, the intraocular mass in the left eye appeared enlarged. No treatment was prescribed for the right eye.
Extensive discussions were held with the family regarding the differential diagnosis, which included endophthalmitis and retinoblastoma. Due to the patient's distance from tertiary medical care, limited follow-up capacity at home, and non-zero risk of retinostic and therapeutic enucleation of the left eye.
Histopathologic analysis revealed neutrophils and macrophages with areas of necrosis and exudates within the vitreous. There was an area of sclera that showed a tract partially filled with suture material surrounded by multinucleated giant cells and inflammation. This tract was connected to the retina and vitreous (Figure 5). Stains for fungus, mycobacteria, and bacteria were negative. The patient had a repeat examination under anesthesia at 2 and 6 weeks postoperatively, in which the retinal lesions in the remaining right eye were stable. At 18 months, the patient continues to do well and there have been no changes in the right eye.
Hematoxylin–eosin stain (original magnification ×40) of the left eye demonstrating (A) vitreous inflammation and necrosis, an area of the sclera with suture material surrounded by multinucleated giant cells extending toward the retina and vitreous and (B) an enlarged view of the suture tract.
We have described the first pediatric case of endophthalmitis status following strabismus surgery secondary to scleral perforation as confirmed by histopathologic diagnosis. Strabismus surgery was complicated by a “pyogenic granuloma” that progressed to leukocoria of his left eye 2 weeks later and resulted in the ultimate diagnosis of endophthalmitis.
Although the differential diagnosis for leukocoria is broad, two important etiologies to consider in the setting of a rapidly developing leukocoria are retinoblastoma and endophthalmitis. Our patient had a history of Down syndrome that was confirmed by trisomy 21 on chromosome analysis; studies suggest Down syndrome may be associated with an increased incidence of retinoblastoma.5 Leukocoria and strabismus can also be a presenting feature of retinoblastoma.6 Due to the fact that the initial examination 3 months prior to the surgery performed by the outside ophthalmologist was reportedly unremarkable and a dilated ophthalmic examination was not repeated prior to surgery, we could not definitively confirm the preexistence of the small peripheral lesions of the right eye or the interim development of lesions in the left eye prior to surgery. Although these lesions were not the typical presentation for retinoblastoma, the presence of a white mass with possible calcification on ultrasound examination only heightened anxiety about the risk of a “missed retinoblastoma.”
Endophthalmitis in children poses a unique problem because young children are typically unable to either recognize or verbalize symptoms.4 The presentation and temporality of the patient's leukocoria to recent strabismus surgery was highly suggestive of postoperative endophthalmitis as the more likely of the two major etiologies on the differential diagnosis. However, his eye was unusually “white and quiet” and lacked classic inflammatory signs with only trace conjunctival injection of the left eye. He lacked significant anterior chamber inflammatory reaction and symptoms of lethargy, eyelid swelling, or fever.4
Based on these factors, we could not definitely rule out retinoblastoma. Therefore, the family chose to proceed with a diagnostic and therapeutic enucleation due to the small chance that a diagnosis of retinoblastoma would prompt immediate and aggressive treatment of his remaining right eye.
Traditionally, a pars plana vitrectomy with intravitreal antibiotics is the treatment of choice for light perception endophthalmitis after cataract surgery.7 No clear treatment modalities have been established for treating endophthalmitis after strabismus surgery in a young child. Given that we could not conclusively rule out retinoblastoma, we elected against a pars plana vitrectomy with intravitreal antibiotics due to the risk of intraocular perforation leading to the seeding of any malignant lesions. For similar reasons, intravitreal antibiotics without a pars plana vitrectomy were not used.
Globe perforation (defined by retinal and/or choroidal injury visible with indirect ophthalmoscopy) has been reported to occur in 0.13% to 1.8% of strabismus procedures.8 Bacterial entry into the vitreous cavity via inadvertent scleral perforation is a postulated mechanism for endophthalmitis following strabismus surgery.9 Suture contamination via eyelash or skin contact may be the source of the bacterial pathogen.10 As many as 28% of strabismus sutures have been found to be contaminated.10
Our case is unique in that the right eye demonstrated multiple retinal scars suggestive of possible scleral perforation and that pathology of the left eye revealed suture tract perforation surrounded by inflammation and giant cells connecting to the retina and vitreous. These findings suggest that globe perforation in this region leads to microorganism entry into the vitreous.
Virulent organisms such as Streptoccocus pneumoniae, Haemophilus influenzae, and Staphylococcus aureus were cultured in previously reported cases of endophthalmitis following pediatric strabismus surgery.3 Although rare in adults, Streptoccocus pneumoniae and H. influenzae are more frequent causes of postoperative endophthalmitis in children, likely due to high rates of pediatric colonization by these organisms.3 The respiratory tract of healthy children is colonized by H. influenzae 98% of the time and by Streptoccocus pneumoniae transiently 45% of the time.11 Recchia et al.4 recommended systemic antibiotics effective against H. influenzae until cultures indicate that it is not present given the high incidence that they found of H. influenzae–positive cases of endophthalmitis and the risk of periorbital and orbital disease due to H. influenzae. We gave our patient broad-spectrum intravitreal antibiotics to prevent the spread of potential infection. Due to concern for retinoblastoma, we did not attempt to perforate the eye for culture prior to histopathologic diagnosis. Unfortunately, we were unable to obtain cultures after enucleation. Therefore, the microbiological cause of endophthalmitis remains unclear.
In the largest series of endophthalmitis following strabismus surgery, Recchia et al.4 reported six cases that were treated with pars plana vitrectomy and intravitreal and systemic antibiotics. Despite this treatment, all eyes eventually had no light perception visual acuity and several became pre-phthisical and/or were enucleated. Devastating visual acuity outcome is the rule rather than the exception in cases of endophthalmitis following pediatric strabismus surgery, although there are cases in which patients return to preoperative baseline vision after aggressive treatment.12 This poor visual outcome is not surprising because visual and anatomical outcomes have been poor in clinical and experimental infections caused by virulent organisms such as Streptococcus species and Gram-negative species, including H. influenzae.13–16 Similar to our case, 5 of the 6 reported cases in the Recchia et al.4 series occurred following surgery on the left medial rectus muscle. Surgery on the left medial rectus muscle is theorized to pose more of a surgical challenge for a right-handed surgeon.
Endophthalmitis prophylaxis and treatment in pediatric strabismus surgery has not been evaluated by a large prospective series due to its rarity and the large sample size needed for such an investigation. Questions remain regarding the use of subconjunctival antibiotics, intraoperative eye preparation, prophylaxis with oral antibiotics, and the use of postoperative topical antibiotics.3 In addition, management of a severe, vision-threatening infection poses its own unique challenges. This case demonstrates the importance of a thorough and complete preoperative eye examination, proper operative preparation, precise surgical technique, and close postoperative follow-up after pediatric strabismus surgery. Although these were all true for the above case, endophthalmitis as a complication is not impossible. This case serves as a stark reminder that, although rare, complications following strabismus surgery can be devastating.
- Simon JW, Lininger LL, Scheraga JL. Recognized scleral perforation during eye muscle surgery: incidence and sequelae. J Pediatr Ophthalmol Strabismus. 1992;29:273–275.
- Knobloch R, Lorenz A. On serious complications after strabismus surgery [article in German]. Klin Monbl Augenheildk Augenarztl Fortbild. 1962;141:348–353.
- Wu ZH, Chan RP, Luk FO, et al. Review of clinical features, microbiological spectrum, and treatment outcomes of endogenous endophthalmitis over an 8-year period. J Ophthalmol. 2012;2012:265078.
- Recchia FM, Baumal CR, Sivalingam A, Kleiner R, Duker JS, Vrabec TR. Endophthalmitis after pediatric strabismus surgery. Arch Ophthalmol. 2000;118:939–944.
- Ayed W, Gouas L, Penault-Llorca F, Amouri A, Tchirkov A, Vago P. Trisomy 21 and cancers [article in French]. Morphologie. 2012;96:57–66. doi:10.1016/j.morpho.2012.10.001 [CrossRef]
- Aerts I, Lumbroso-Le Rouic L, Gauthier-Villars M, Brisse H, Doz F. Retinoblastoma update [article in French]. Arch Pediatr. 2016;23:112–116. doi:10.1016/j.arcped.2015.09.025 [CrossRef]
- Endophthalmitis Vitrectomy Study Group. Results of the endophthalmitis vitrectomy study: a randomized trial of immediate vitrectomy and of intravenous antibiotics for the treatment of postoperative bacterial endophthalmitis. Arch Ophthalmol. 1995;113:1479–1496. doi:10.1001/archopht.1995.01100120009001 [CrossRef]
- McNeer KW. Three complications of strabismus surgery. Ann Ophthalmol. 1975;7:441–446.
- Huang S, Crawford JB, Rutar T. Pathological findings in poststrabismus surgery endophthalmitis. J AAPOS. 2011;15:98–100. doi:10.1016/j.jaapos.2010.11.018 [CrossRef]
- Eustis HS, Rhodes A. Suture contamination in strabismus surgery. J Pediatr Ophthalmol Strabismus. 2012;49:206–209. doi:10.3928/01913913-20110920-01 [CrossRef]
- Katosova LK. The features of Haemophilus influenza and Streptococcus pneumoniae carriage and the comparative characteristics of strains isolated from healthy children and from patients with acute and chronic respiratory infections [article in Russian]. Zh Mikrobiol Epidemiol Immunobiol. 1994;suppl1:55–60.
- Alniemi ST, Bakri SJ, Cherfan C, Mohney BG. Successfully managed endophthalmitis following strabismus surgery. J AAPOS. 2016;20:263–266. doi:10.1016/j.jaapos.2016.01.008 [CrossRef]
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- Mao LK, Flynn HW Jr, Miller D, Pflugfelder SC. Endophthalmitis caused by streptococcal species. Arch Ophthalmol. 1992;110:798–801. doi:10.1001/archopht.1992.01080180070030 [CrossRef]
- Schmidt ME, Smith MA, Levy CS. Endophthalmitis caused by unusual gram-negative bacilli: three case reports and review. Clin Infect Dis. 1993;17:686–690. doi:10.1093/clinids/17.4.686 [CrossRef]
- Myerowitz RL, Klaw R, Johnson BL. Experimental endogenous endophthalmitis caused by Haemophilus influenzae type b. Infect Immun. 1976;14:1043–1051.