Bilateral endogenous endophthalmitis complicated by scleral perforation: an unusual presentation

Abstract

Endogenous endophthalmitis complicated by necrotising scleritis has rarely been reported in the literature. We, hereby, report a case of bilateral scleral perforation with endogenous endophthalmitis in an 87-year-old female patient with diabetes who presented as bilateral orbital cellulitis. Systemic workup ruled out autoimmune aetiology. The culture and sensitivity of exudates exuding from the scleral perforation showed Escherichia coli. The Patient was managed conservatively with parenteral and topical antibiotics along with steroid, but the vision could not be salvaged. The report emphasizes on atypical presentation of endogenous endophthalmitis. In old and immunosuppressed individuals presenting with a clinical picture of bilateral orbital cellulitis with profound vision loss, endogenous endophthalmitis should be ruled out.

Background

Endophthalmitis is an ophthalmic emergency with potentially devastating consequences. Depending on the route of infection, it can be either exogenous (more common) or endogenous. Endogenous endophthalmitis comprises 2%–8% of all cases of endophthalmitis of which around 12% have been reported to be bilateral.^{1 2} There are only a few reported cases of bilateral bacterial endogenous endophthalmitis where Klebsiella, Proteus, meningococcus, Pseudomonas, pneumococcus and Escherichia coli have been isolated as causative organisms.³ On the other hand, spontaneous scleral perforations without a prior history of trauma or surgery are very rare but have been reported in atypical cases of some ocular conditions (scleritis), systemic disorders (Marfan’s syndrome, Axenfeld syndrome, craniofacial dysmorphism and scleroderma), whereas some remain idiopathic.⁴ In nearly 40%–50% of patients, this is associated with systemic collagen vascular diseases. Infectious scleritis is a rare entity and accounts for just 5%–10% of all cases of scleritis.⁵ Scleral infections from Pseudomonas, Staphylococcus, or Herpes zoster virus can cause necrotising scleritis, which is clinically identical to systemic autoimmune disease.⁶ Endophthalmitis has rarely been reported to be complicated by necrotising scleritis leading to a scleral abscess and subsequent perforation.

Case presentation

An 87-year-old woman presented in the ophthalmic emergency with severe pain, complete drooping of lids along with oedema and loss of vision of both eyes for 5 days. She had fever, generalised fatigue and loss of appetite for around 15 days before developing ocular symptoms. She was a known case of controlled type 2 diabetes mellitus. There was no history of trauma or any intraocular/periocular surgery in the past.

On examination, she denied the perception of light in both eyes. There were oedema and erythema of lids along with complete drooping and limited extraocular movements in all gazes. The globe appeared tense on digital palpation. Anterior segment examination on retraction of lids showed congested and chemosed conjunctiva, a clear cornea, and anterior chamber reaction. The pupillary area showed fibrinous exudate and posterior synechiae along with a cataractous lens in both eyes. Posterior segment examination was obscured by the fibrinous exudate and it showed only a yellowish glow on indirect ophthalmoscopy. Based on clinical examination and history of the patient, a provisional diagnosis of bilateral orbital cellulitis was made (figure 1).

Figure 1.

Clinical picture of the patient showing lid oedema and erythema (thin arrow) and purulent discharge at the suspected site of scleral perforation (thick arrow).

Investigations

Routine blood and urine investigations were sent, and she was started on broad-spectrum intravenous antibiotics. As the globe appeared tense during initial examination and the posterior segment details were not visible due to hazy media, an ultrasonogram B scan was done to look for the posterior segment changes, which showed bilateral retinal detachment with subretinal collection and defect in the superotemporal aspect of the globe with scleral abscesses along with extensive debris and organised exudates in the vitreous cavity of both the eyes (figure 2).

Figure 2.

Ultrasonogram B scan of both eyes shows bilateral retinal detachment (thin arrow) with subretinal collection and defect in the superotemporal aspect of the globe (thick arrow) with scleral abscesses (asterisks). There is extensive debris and organised exudates in the vitreous cavity.

Systemic workup along with routine blood and urine investigations revealed a raised total white blood cell count (WBC) of 20.80 ×10⁹/L, erythrocyte sedimentation rate (60 mm/hour), a deranged serum electrolytes (serum chloride 89.41 mEq/L, serum potassium 2.81 mEq/L, serum sodium 153.1 mEq/L) and 10–12 WBC/high power field (HPF) in urine routine microscopy. Though, blood and urine cultures turned out to be inconclusive. Electrolyte disturbance was corrected after consultation with internal medicine.

Considering that the patient was a diabetic, an otolaryngologist opinion was sought and mucormycosis was ruled out. On the 2nd day of admission, the patient developed copious purulent exudation from the temporal aspect of each eye and the eyes became hypotonus. The exudate was sent for culture and sensitivity. A vitreous tap in view of endophthalmitis could not be taken due to hypotony after scleral perforation. MRI of the brain and orbit were done keeping in mind the differential diagnosis of cavernous sinus thrombosis (which was planned due to the toxic look at presentation of the patient and bilateral ophthalmoplegia) which revealed no evidence for the same (figures 3 and 4). Meanwhile, improvement in the general condition of the patient was noted on the parenteral and topical antibiotics and topical steroids. Looking at the intense inflammatory process involved, she was also started on systemic steroids (1 mg/kg/day).

Figure 3.

(A) Axial T2-weighted image of the eye shows bilateral retinal detachment (thin arrow) with T2 hypointense subretinal (asterisks) and scleral (thick arrow) collection/abscess. Bilateral lateral recti are thickened and mildly hyperintense suggestive of inflammatory changes. (B) Axial T2-weighted images of the eye show a defect in the superotemporal aspect of bilateral globe involving the uveoscleral layer (thin arrow) with adjacent scleral collection (asterisks). There are bilateral scleral inflammatory changes (thick arrow). (C) Coronal T2-weighted images of the eye show a defect in the superotemporal aspect of bilateral globe involving the uveoscleral layer (thin arrow) with adjacent scleral collection (asterisks). There are bilateral scleral inflammatory changes (thick arrow).

Figure 4.

(A) Axial diffusion-weighted imaging showing diffusion restriction in bilateral subretinal and scleral collection s/o exudates or abscesses. (B) Apparent diffusion coefficient image showing diffusion restriction in bilateral subretinal and scleral collection suggestive of exudates or abscesses.

Systemic workup for any underlying connective tissue disorder was also undertaken to find the aetiology. Antinuclear antibody screening, rheumatoid arthritis factor, thyroid profile were negative and C reactive protein (CRP) was raised (107.7 mg/dL). The reports of culture and sensitivity of the purulent discharge/exudate showed moderately significant growth of E. coli.

Differential diagnosis

Bilateral orbital cellulitis like picture made cavernous sinus thrombosis as one of the differentials but MRI of the brain and orbit ruled out the same. Other differential diagnoses for our case include infectious and autoimmune causes of necrotising scelritis; however, autoimmune causes were ruled out. The exudates exuding from temporal scleral wound was sent for direct microscopy and culture and sensitivity. Microbiological workup revealed E. coli sensitive to gentamicin, imipenem, meropenem, tigecycline and moderately sensitive to amikacin. The absence of history of trauma and intraocular surgery ruled out the exogenous source of endophthalmitis. The presence of concurrent subclinical urinary tract infection suggested the primary source of infection, and hence a diagnosis of endogenous endophthalmitis was made.

Treatment

She was started on broad-spectrum intravenous antibiotics that included amoxycillin and clavulanic acid 1.2 gm (1×2), amikacin 500 mg (1×2), metronidazole 400 mg (1×3), pantoprazole 40 mg (1×1), and topical moxifloxacin 0.5%, prednisolone acetate 1% and cycloplegic (homatropine 2%) in both the eyes. Based on the culture and sensitivity reports of the conjunctival discharge, the topical drugs were changed to fortified gentamicin 1.4% and parenteral injection meropenem 1 gm (1×3).

Outcome and follow-up

Following treatment, the patient showed improvement in the ocular signs with improvement in extraocular involvement, a decrease in conjunctival chemosis and lid oedema but still denying perception of light in both the eyes. She was systemically stable on subsequent follow-up. The patient was discharged on oral as well as topical antibiotics and steroids. She was followed up in the outpatient department on day 7, 1 month and 3 months. In all the subsequent visits, the patient had progressively decreasing conjunctival congestion, anterior chamber reaction with non-reacting pupil, and poor glow on the posterior segment examination. At the final follow-up, she had regained complete extraocular motility with a quiet anterior chamber; however, she continued to deny perception of light in both the eyes (figure 5).

Figure 5.

Follow-up picture showing resolved lid oedema, mild conjunctival congestion (1 month follow-up).

Discussion

Endogenous endophthalmitis rarely complicates the course of E. coli septicemia.⁷ The endogenous or secondary type results from bacteremia caused by infection at other organ sites and the genitourinary tract accounts for most of these reported cases.³

Metastatic septic endophthalmitis was first recognised by Shammas in 1856.⁸ A classification system has been proposed by Greenwald et al. This system operates with five categories of endogenous bacterial endophthalmitis: anterior and posterior focal infection, anterior and posterior diffuse infection, and panophthalmitis. The last category comprises cases with the affection of the entire eye and orbit, clinically presenting with a proptosed inflamed eye, marked eyelid oedema, and restricted mobility.⁹ Our patient falls in the last category. Previous reports of endogenous endophthalmitis have revealed systemic associations like endocarditis, lumbar abscess, hip alloplasty and urinary tract infections where mostly the blood culture revealed the causative organism.¹⁰

Gruener et al have reported a case of exogenous Aspergillus fumigatus endophthalmitis complicated by necrotising scleritis in a vitrectomised eye of a patient with diabetes.¹¹ Our patient had no prior history of any ocular surgery or trauma. A recently published article by Shegaonkar reports a case of toxic epidermal necrolysis where the patient presented with bilateral panophthalmitis which progressed rapidly and the eye could not be salvaged.¹² There was no history of any mucous membrane involving lesion in the course of the disease in our patient. Scleral infection with Pseudomonas aeruginosa has been reported to cause scleral perforation by Francois et al in 1981 and more recently by Marta et al in patients with no prior history of ocular surgery or trauma,^{13 14} but these cases were not complicated by endophthalmitis as was seen in our case. More so, our patient did not have any history of any connective tissue disorder or Marfan’s syndrome, or any dermatological involvement.

The challenge in our case was in reaching a diagnosis because the patient presented to us after more than a week of symptom onset after having taken treatment from a local practitioner. The disease was already very advanced with poor outcomes and the sclera perforation precluded the vitreous tap leaving us with only the purulent discharge/exudation from the abscess as a source of etiological diagnosis. As the culture of the discharge from scleral abscess showed E. coli, the possible course of infection can be traced to the patient developing probably subclinical urinary tract infection (as the urine routine microscopy showed 10–12 WBCs/HPF though with a normal blood urea and serum creatinine values, deranged electrolytes and inconclusive urine culture) leading to septicemia (a raised WBC count in blood of around 20.80 ×10⁹/L and a CRP of 107.7 mg/dL) and finally metastatic endogenous endophthalmitis. The fact that our patient was elderly with diabetes makes it likely that she had a weak immune system which led to such a devastating consequence.

The uniqueness of our case lies in the fact that there was simultaneous involvement of both the eyes with the scleral abscess giving way, behind the lateral rectus muscle insertion in both the eyes probably because the sclera is the thinnest just behind the recti-insertion.

Patient’s perspective.

I had developed fever and was having malaise for some time. I had consulted the nearest local practitioner. He prescribed some medicines, but my symptoms did not improve. I started developing swelling around my eyes and pain. I visited the hospital where the team of doctors was prompted in examining my condition and ordered several tests including an MRI of the brain and orbit and ultrasound of the eyes on admission. After their examination, they had informed me about the grievous condition of my eyes and explained the guarded visual prognosis to me. I was devastated but they counselled me properly about the importance of coming to a diagnosis and treating the underlying condition which had caused it. They had planned to take some sample from my eyes as they were suspecting an infectious cause, but I was later informed that due to the perforation in my globe it was not possible to get a sample. There was exudation from both the eyes, so they had sent that for culture and sensitivity. Meanwhile, I was also started on various antibiotics. My condition was being properly monitored by an able team of doctors and they explained that though infection was mostly suspected but an autoimmune condition needs to be ruled out. Hence, they ran blood and urine cultures to rule out infectious cause and did a few more blood tests to rule out autoimmune causes. From all the reports that the doctors obtained from the tests, I was informed that I had a urinary tract infection, which had led to the devastating condition of my eyes. They changed my antibiotics according to the culture and sensitivity reports following which my condition gradually started improving. Though the doctors tried their best to save my eyes but they could not, due to the advanced stage of the disease with which I presented to them. I had never thought that urinary tract infection could cause my vision loss and eye infection. I was really thankful to them for having found the source of infection and treating it promptly and saving my life.

Learning points.

• Bilateral orbital cellulitis with profound vision loss in elderly uncontrolled diabetics should raise suspicion for endogenous endophthalmitis. A thorough evaluation is needed to locate the primary source of infection and needs to be treated.

• Delayed presentation of cases with bilateral endophthalmitis in an elderly patient with diabetes can be challenging for salvaging vision.

• A multidisciplinary approach should be sought in cases of an elderly or immunosuppressed individual with other comorbidities presenting with bilateral orbital cellulitis like picture.

• In cases presenting with necrotising scleritis like picture, autoimmune aetiology must be ruled out.

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