From the Department of Ophthalmology, Stanford University, Stanford, California.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Darius M. Moshfeghi, MD, Adult & Pediatric Vitreoretinal Surgery, 1225 Crane St., Suite 202, Menlo Park, CA 94025.
Phthisis bulbi is the final common pathway of a variety of intraocular insults diagnosed pathologically by the presence of a cyclitic membrane, metaplasia of the retinal pigment epithelium, optic nerve atrophy, and a thickened posterior sclera. The most rapid reported onset of phthisis bulbi was over several weeks in an 86-year-old patient with zoster ophthalmicus.1 However, the diagnosis was made clinically based on hypotony and a shallow anterior chamber in an actively infected eye with no pathologic specimen. Cases of phthisis have been reported in infants with retinoblastoma as early as 24 months.2
We present a case of acute phthisis in a premature infant that progressed over less than 6 weeks in the absence of active intraocular infection. To our knowledge, this is the earliest incidence of phthisis bulbi in the literature.
A female neonate with an estimated gestational age of 32 weeks developed fulminant methicillin-resistant Staphylococcus aureus (MRSA) and Pseudomonas aeruginosa sepsis in addition to MRSA meningitis on day 7 of life. She was a monoamniotic, monochorionic twin born to a 24-year-old mother with good prenatal care via cesarean section for pre-term labor. The infant initially did well until she developed a disseminated pustular rash on day 7 of life requiring broad-spectrum antibiotics. The hospital course was further complicated by thrombocytopenia, acute renal failure, cholestasis, seizures, and a diffuse encephalopathy.
Ophthalmology evaluation was first requested on day 44 of life as part of a routine retinopathy of prematurity evaluation. The initial examination was significant for blunted light reflexes and signs of retinal inflammation in both eyes (Fig. 1). There were no prior ocular examinations for comparison. Computed tomography of the orbits demonstrated intraocular calcifications and a possible mass not seen on prior imaging with an inflammatory component on magnetic resonance imaging (Fig. 2). The orbits had been normal on computed tomography evaluation on day 13 of life. The infant was transferred to Lucile Packard Children’s Hospital at Stanford University for treatment of suspected retinoblastoma.
Figure 1. RetCam (Clarity Medical Systems, Pleasanton, CA) Images of the Right (A) and Left (B) Eyes Show Conjunctival Injection and Icterus Along with Small Pupils and Abnormal Light Reflexes. A Leukoma Is Noted Temporal to the Light Reflex in the Left Eye. Corneal Diameter Was 8 mm Horizontally in Both Eyes.
Figure 2. Orbital Imaging. (A) Computed Tomography Scan on Day 13 of Life Shows Normal Globes. (B and C) On Day 46 of Life, Serial Computed Tomography Scans Demonstrate Ring-Shaped Intraocular Calcification and Microphthalmia with a Possible Mass in the Posterior Vitreous that Is Hyperintense Post-Contrast Enhancement (D). This Bone Window Shows an Abnormal Hyperintensity in the Vitreous of Both Eyes Consistent with Intraocular Calcification. Axial Lengths of Both Globes Were 14 mm.
After several discussions with the family, a diagnostic enucleation of the right eye (due to its worse visual potential) was performed to rule out intraocular malignancy on day 48 of life. The patient’s postmenstrual age at the time of enucleation was 39 weeks.
Histopathology of the enucleated eye demonstrated intraocular disorganization with reparative changes without evidence of tumor or infection (Fig. 3), suggestive of atrophic changes following endogenous endophthalmitis. Cultures for aerobes, anaerobes, and fungi obtained at the time of enucleation were negative. The fellow eye underwent vitrectomy, lensectomy, and pupilloplasty. During surgery, numerous intraocular calcific pieces were noted to egress from the vitreous cavity into the anterior chamber. Histopathologic evaluation was consistent with calcium. Intraocular cultures for aerobes, anaerobes, and fungi obtained at the time of vitrectomy were negative. Final visual acuity was light perception.
Figure 3. Histology of Ciliary Body, Choroid, and Retina. Hematoxylin–Eosin Section Shows only Mild Inflammation with a Cyclitic Membrane, Disorganized Neuroretina, Metaplasia of the Retinal Pigment Epithelium, and Vitreous Fibrosis. Special Stains Were Negative for Bacterial or Fungal Organisms. The Optic Nerve (not Shown) Was Markedly Atrophic. The Findings Were Suggestive of Phthisis Bulbi.
Although rare, several cases of neonatal retinoblastoma have been reported.3 To our knowledge, however, there are no reports of endogenous bacterial endophthalmitis leading to intraocular calcification and phthisis. Nor are there documented reports of phthisis occurring prior to term, although congenital infections have been implicated as the cause of late-onset phthisis.4
Calcifications have been noted with Toxocara canis, tubercular, and cytomegalic virus infections.4–7 Although blood cultures in this patient were positive for MRSA and P. aeruginosa, we believe this patient’s inflammatory infiltrate and metaplastic reaction likely represented a response to an unsuspected endogenous pseudomonal endophthalmitis that had resolved by the time of her initial examination. P. aeruginosa is the most commonly implicated cause of endophthalmitis in the preterm infant.8,9 Although the organism is known to be particularly virulent, an extensive metaplastic response to pseudomonal endophthalmitis has not been reported.
Although the likely diagnosis at the time of presentation was intraocular disorganization secondary to endogenous endophthalmitis, neither fundus examination nor imaging could rule out the presence of tumor. At the time of presentation, we were thus faced with a Hobson’s choice: risk tumor seeding with intravitreal injection or perform a disfiguring and irreversible procedure to exclude an unlikely diagnosis. Although earlier funduscopic examination might have obviated an enucleation, the visual prognosis in cases of pseudomonal endophthalmitis is decidedly poor.9,10 Given the extent of disorganization on pathologic sections, the probability of functional vision with retention would have been low.
Endophthalmitis in the premature eye has a particularly poor visual prognosis. This case establishes an atypical presentation of endogenous endophthalmitis with intraocular calcifications and acute phthisis. It also serves as evidence of the dramatic cellular response to injury in a maturing eye. Pediatric vitreoretinal surgeons should be consulted early in the course of fulminant sepsis in the premature infant to evaluate for endogenous endophthalmitis. Acute phthisis can occur in as few as 6 weeks in the absence of local treatment.
- Amanat LA, Cant JS, Green FD. Acute phthisis bulbi and external ophthalmoplegia in herpes zoster ophthalmicus. Ann Ophthalmol. 1985;17:46–51.
- Balasubramanya R, Pushker N, Bajaj MS, Ghose S, Kashyap S, Rani A. Atypical presentations of retinoblastoma. J Pediatr Ophthalmol Strabismus. 2004;41:18–24.
- Abramson DH, Du TT, Beaverson KL. (Neonatal) retinoblastoma in the first month of life. Arch Ophthalmol. 2002;120:738–742.
- Uchiyama K, Tsuchihara K, Horimoto T, Karasawa T, Sugiyama K. Phthisis bulbi caused by late congenital syphilis untreated until adulthood. Am J Ophthalmol. 2005;139:545–547. doi:10.1016/j.ajo.2004.08.056 [CrossRef]
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