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Permanent visual loss in a child with a rash
  1. P A Berry,
  2. J K Dart,
  3. E Graham,
  4. M Matheson,
  5. E M E MacMahon
  1. St Thomas's Hospital, London UK
  1. Correspondence to: Philip Berry, St Thomas's Hospital, London UK; philaberry{at}hotmail.com

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Endophthalmitis is an unusual but well described complication of bacteraemia. Here we report endophthalmitis arising in the course of a mild illness resembling chickenpox.

CASE REPORT

A previously healthy 6 year old white girl awoke in the early hours feeling hot and complaining of headache. She was given ibuprofen and she went back to sleep. Spots appeared on her limbs later the same day but they blanched beneath a glass bottle and several developed into small blisters. A classmate was known to have chickenpox. Next day some new spots appeared on her buttocks, and some of the older lesions appeared to be crusting over. She was noticed rubbing her right eye and later said she felt warm, and vomited. Next morning she complained of pain in the right eye and could not open it. Following assessment by her general practitioner she was referred urgently to an eye unit.

On examination she appeared well and was afebrile. The right eye was chemotic, with an absent red reflex and a large hypopyon (Fig 1). Erythematous maculopapular lesions were present on her arms, legs, and face.

Figure 1

Right eye showing panophthalmitis. There is a diffuse corneal infiltrate and a purulent exudate in the anterior chamber leading to the opaque appearance of the cornea and the absence of any view of the iris or pupil. The sclera is exposed in the upper temporal quadrant and is infiltrated and thinned.

Investigations included a white cell count of 8.0 × 109/l (neutrophils 4.0 × 109/l) and C reactive protein 22 mg/l. Material was collected from the base of the sole remaining vesicle, on her shin. Ciprofloxacin was commenced parenterally at a dose of 100 mg twice daily (10 mg/kg/day). Examination under general anaesthesia revealed a panophthalmitis with total corneal abscess and hypopyon. There was no evidence of a puncture wound to suggest exogenous infection. Aqueous humour was aspirated, and intravitreous amikacin and vancomycin administered. Vitrectomy could not be performed because of the severe anterior segment inflammation.

Electron microscopy, immunofluorescence staining and polymerase chain reaction (PCR) testing of the vesicular material was negative for varicella zoster virus (VZV) and herpes simplex virus. Neither IgM nor IgG against VZV was detected in the blood. Cultures of blood and aqueous humour were negative but urgent Gram stain of the aqueous humour revealed scanty intracellular Gram negative diplococci. Meningococcus C DNA was detected in the aqueous and in the skin scraping, but not in blood, by PCR testing.

The patient was apyrexial and remained clinically well during her hospital admission. At discharge after 5 further days of parenteral antibiotics (ceftriaxone) the rash had completely resolved. In the absence of a positive culture sensitivities could not be confirmed, although all the stated antibiotics except vancomycin are generally effective against meningococcus. At follow up the eye had become phthisical, with visual acuity reduced to perception of light only.

COMMENT

In this case meningococcal endophthalmitis, usually seen in association with clinically apparent septicaemia and meningitis,1 occurred in the setting of a mild illness thought to be chickenpox. Although the usual hallmarks of meninogococcal sepsis were absent, evidence for seeding of the eye in the course of a transient meningococcaemia was provided by Gram negative intracellular diplococci in the aqueous humour and detection of meningococcal DNA in both the aqueous humour and cutaneous material. Reports of meningococcal endophthalmitis in clinically well, non-bacteraemic patients are very rare, with just four published cases in English. The patients were 13 months,2 15 years,3 17 years,4 and 23 years5 old. Both eldest and youngest had no skin lesions, but were pyrexial at presentation. The 15 year old had a low grade fever and a generalised macular rash misdiagnosed as scarlet fever. The 17 year old appeared completely “non-septic” with a 4 day history of myalgia, arthralgia, sore throat, and transient fever. A vesicular rash on the lower extremities appeared vasculitic on histological examination. In all of these cases only one eye was involved. Meningococcus type C was isolated from the eye in two cases, type B in a third, and in the fourth case the type was not stated. The outcome was bad in each case. In the three older patients vision was reduced to the perception of light only, with dense cataracts forming in two, and pthisis in the 23 year old who underwent vitrectomy. The 13 month old underwent vitrectomy and lensectomy, and the plan at publication was to provide the patient with a new lens.

Vesicles are not generally regarded as lying within the spectrum of rashes caused by meningococcal infection. The acute ocular complications of varicella infection include spread of vesicles to the eyelid, papillary conjunctivitis and, uncommonly, dendritic keratitis, acute retinal necrosis, and optic neuritis.6 Hypopyon, observed in this case, is not a feature of primary VZV infection.

This report serves to emphasise the broad clinical spectrum of meningococcal disease, and highlights the potential for severe sequelae despite an otherwise effective host response to bacteraemia. Seeding of group C meningococci resulted in visual loss in this young patient for whom the UK conjugate vaccine programme came just a year too late.

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