Background/aims To describe our experience of patients presenting to a tertiary referral adnexal department with orbital or periocular tuberculosis (TB) over a 10-year period.
Methods We reviewed all patients with a diagnosis of orbital or periocular TB from 2001 to 2011 in Moorfields Eye Hospital.
Results Nine patients were identified over the 10-year period. Three cases of cutaneous TB, two cases of TB dacryocystitis and four cases of diffuse orbital TB were identified. All patients lived in the UK, but were born in the African or Asian subcontinents. Three patients had known prior (and treated) pulmonary TB and all were immunocompetent.All patients presented with periocular discomfort. After tissue diagnosis, all patients were referred for triple antituberculous therapy (ATT); all patients completed their course of ATT, with resolution of all orbital and lacrimal masses. There were no recurrences at a median follow-up of 26 weeks (range 1 month–5 years). One patient, who required later evisceration, was the only case with loss of vision.
Conclusions Orbital and periocular TB can be difficult to diagnose and lead to diagnostic delays, with emphasis on clinical suspicion rather than a positive culture result; the management of such cases is not only surgical, but also medical and social. Although surgical intervention can alleviate symptoms and prevent visual loss, the use of a complete course of ATT is paramount for disease management and the patient and their family need to be counselled about the associated public health issues.
- Eye Lids
- Public health
- Lacrimal drainage
- Lacrimal gland
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Mycobacterium tuberculosis usually causes pulmonary disease, but many people become symptom-free carriers of the bacterium, and lymphatic or haematogenous spread allows bacterial dissemination to distant parts, such as the periocular tissues, but may also arise through direct spread from the adjacent paranasal sinuses.1 In the UK, orbital and periocular tuberculosis (TB) are rare presenting forms of extrapulmonary TB.
The WHO has estimated that about one-third of the world’s population is infected with M. tuberculosis and 9 million new cases of tuberculous disease occur each year, accounting for nearly 2 million deaths annually.2 Although most cases occur in the developing world, a recent resurgence of TB has occurred in the ‘developed’ world, with the UK having one of the highest incidence rates of TB in Western Europe.3 Indeed, in the UK, 8483 new cases of TB were reported in 2010—a rate of 13.6 cases per 100 000 population—with London accounting for the highest proportion of such cases (39%), and having the highest incidence (42/100 000).4
We report our experience of patients presenting to the Adnexal Service at Moorfields Eye Hospital with orbital or periocular TB over a 10-year period.
Patients and methods
A retrospective non-comparative review of all patients with a presumed diagnosis of orbital or periocular TB from 2001 to 2011 was performed; all cases had tuberculous infection confirmed on cultures of biopsied tissues. Ethical committee approval was sought and obtained before commencing the study. Consecutive patients were identified from the orbital and pathology databases at Moorfields Eye Hospital and the clinical notes were reviewed. Patients were categorised as having either cutaneous, lacrimal or orbital TB. The main outcome measures were number of disease-free years and amount of visual loss sustained.
Nine patients with this rare condition were identified over the 10-year period, with three cases of cutaneous TB, two cases of TB dacryocystitis and four cases of diffuse orbital TB (table 1).
Clinical case reports
Orbital TB—Case 1
A 59-year-old Indian woman, with a history of pulmonary TB treated 30 years ago in India, presented with 3 months of left retrobulbar ache which was worse with eye movement. Soft swelling was present in the left upper eyelid sulcus, visual functions were normal and CT showed a diffuse mass across the anterosuperior part of the left orbit (figure 1). Biopsy showed a chronic granulomatous mass with fibrosis and the microbiology supported a diagnosis of TB. The patient was referred to chest physicians and commenced on triple therapy and later oral steroids. The orbital pain and mass resolved and her optic nerve functions remained intact. She had no visual loss and remained disease free for 5 years.
Lacrimal TB—Case 2
An Indian woman presented at age 73 years with a 1-year history of a right lower lid mass and associated epiphora; over the preceding year she had lacrimal drainage surgery which was complicated 1-month post-op by a fistula on the incision line. Six months later, she had a violaceous lump in the lower part of her right orbit extending into the dermis of the lid and also in the area of the previous dacryocystorhinostomy and was thought to originate from the previous lacrimal sac infection. There were fistulae on each of these masses (figure 2). TB in these lesions was confirmed on biopsy and a positive QuaniFERON-TB Gold test. The patient denied any significant medical, ophthalmic or family history, had no prior exposure to chronic infections and was a non-smoker.
CT showed destruction of the lacrimal bone and a soft tissue mass in that area. She was commenced on quadruple antituberculous therapy (ATT) and her lesions remain quiescent (figure 3) for 2 years and she experienced no visual loss.
Cutaneous TB—Case 3
A 53-year-old North African man was referred to our department for management of his destructive midline facial disease, having had 13 years of treatment for lupus vorax and four prior eyelid operations. At presentation, he had gross bilateral corneal exposure due to heavily scarred lids with marked forniceal shortening. He was treated with urgent bilateral lower lid reconstructions with mucous membrane grafting, and biopsies revealed necrotising palisading granulomas, typical of TB. He was retreated with triple antituberculous therapy, but, with a painful blind eye secondary to infected exposure keratitis and endophthalmitis, underwent a left evisceration 1 year after presentation. With a destroyed midface, special consideration was given to anaesthetic induction, this requiring preoxygenation through a plastic drape placed over the face, and anaesthesia was induced with propofol and rocuronium before intubation could be achieved with a preformed tracheal tube. He subsequently required a large forehead transposition flap to reform the lower eyelid to protect the right ocular surface, this severely compromised by exposure.
All patients originated from the Asian or African continents (four North African and five Indian), with a female predominance (7/9 patients) and a wide age range at presentation (19–78 years) (table 1). Three patients had known prior (and treated) pulmonary TB and had been discharged as ‘cured’. Only one patient had known BCG vaccination (a 19-year-old African woman) and all patients gave a history of TB contact within the last 5 years; none were known to have been immunocompromised. All patients presented with periocular discomfort, this being worse with eye movement in all cases of orbital involvement. Visual loss was present only in the one case with severe keratopathy due to destruction of his eyelids (Case 3), and vision was normal in all cases with orbital and lacrimal diseases. One patient had chest symptoms, with typical changes in pulmonary TB on X-ray, none had night sweats and two patients had newly discovered lymphadenopathy.
After tissue diagnosis, all patients were referred for triple ATT and one patient with orbital disease also received oral corticosteroids following respiratory assessment. No corticosteroid treatment was deemed necessary to maintain visual integrity. All patients completed their course of ATT and the orbital and lacrimal masses resolved in all cases, with no recurrence at a median follow-up of 26 weeks (range 1 month–5 years); visual loss (with later evisceration) occurred in one patient (Case 3; table 1).
The incidence of TB differs worldwide and is influenced by several modifiable risk factors such as exposure to TB, immunosuppression, drug resistance to TB and low socioeconomic status. TB still poses a public health problem in the UK, with increasing numbers of immigrants from endemic areas and the appearance of more drug-resistant organisms.5 Extrapulmonary TB is rare, but has become more prevalent with the greater prevalence of comorbidities such as HIV.6 TB in the head and neck area mainly manifests as cervical lymphadenitis,7 and intraocular TB mainly presents as an asymptomatic choroiditis.8 We note from our cohort of patients that there is no typical presentation of periocular and orbital TB; all our patients gave a history of TB exposure and were immunocompetent. The main presenting symptom was ocular discomfort on eye movement and there was no evidence of intraocular disease or lymphadenopathy.
Cutaneous TB or lupus vulgaris can manifest itself in a number of ways, such as ‘recurrent chalazia’, ulcerated skin nodules, diffuse lid swelling and cicatricial eyelid changes (Case 4; table 1).1 TB invasion of the lacrimal drainage system is more common in the developing world, with only two isolated case reports from the UK.9 ,10 In no other cases are blood-stained tears reported, although epiphora is usually present and the diagnosis commonly only made with biopsy at the time of surgery.11 ,12 Primary TB of the lacrimal sac remains exceedingly rare, with secondary causes of dacryocystitis—such as sarcoidosis or sac tumours—being commoner. Orbital presentations of TB can be clinically divided into periostitis, soft tissue tuberculoma with or without bony involvement, orbital spread from the paranasal sinuses or TB dacryoadenitis.1
All patients with suspected extrapulmonary TB should undergo further investigation with a chest X-ray, and if the clinical picture and histology are strongly suggestive of TB, the appropriate treatment regime commenced while awaiting culture results.13 Growth of M. tuberculosis from a specimen is the ‘gold standard’ for diagnosis of TB and allows drug sensitivity to be assessed. A negative culture—with no staining of acid fast bacilli (AFB)—should not dissuade the clinician from considering a diagnosis of TB, but merely prompts the use of supportive tests such as tuberculin skin tests, PCR-based analysis and interferon-γ release assays (IGRA). The diagnosis of orbital TB is more often based on clinical suspicion than positive cultures, with only 18/59 cases having positive cultures for AFB.1 Our four orbital cases and three cutaneous cases had positive cultures on the first biopsy; however, our one patient with dacryocystitis needed a second biopsy to confirm the diagnosis. National Institute of Health and Care Excellence (NICE) guidelines also suggest that biopsied tissues for TB culture should be taken into a formalin-free dry pot in order to maximise growth of AFB.
TB is renowned for mimicking other diseases and for false-negative testing—with cultures, AFB smears and PCR all having low sensitivities.14 Diagnosis of TB on biopsy alone is difficult, as the widespread chronic inflammation with caseating granulomas may be misinterpreted as sarcoidosis or Wegener's granulomatosis. Standard tuberculin testing (Mantoux test) involves the intradermal injection of tuberculin purified protein derivative and measurement of the induration 72 h later; false positives can, however, occur due to previous BCG vaccination. IGRA (these including the QuantiFERON-TB Gold test and T-SPOT.TB assay) quantify the release of interferon from lymphocytes after stimulation in vitro by M. tuberculosis antigen, the test being more specific than the Mantoux and not confounded by previous BCG vaccination.15
Although anti-TB medication has improved the outcome of many patients affected by the disease, as demonstrated by our cohort of patients (table 1), patient education is paramount in controlling spread of the disease and decreasing the development of drug-resistant strains. Once referred to the TB service, each patient should be assigned to key worker responsible for facilitating education of the patient and ensuring adherence to the treatment plan. If this individual is concerned about non-adherence to treatment, they can instigate directly observed therapy, but this is more commonly reserved for patients with adverse risk factors such as known prior non-adherence, or those with active TB who are street-dwelling or shelter-dwelling.14 Primary care health organisations in areas of high incidence of TB should recommend neonatal BCG vaccination (before 6 months of age), but elsewhere the routine BCG vaccination is now not recommended for children between the ages of 10–14.13 Notably, all of our patients were born outside the UK and only one patient underwent vaccination on arrival into the UK (table 1).
Orbital and periocular TB can be difficult to diagnose, with emphasis on clinical suspicion rather than a positive culture result and the management of such cases is not only surgical, but also medical and social. Although surgical intervention may alleviate symptoms and prevent visual loss, the use of a complete course of ATT is essential for treatment of this disease and both the patient and their family need to be counselled about the associated public health issues.
Professor Geoffrey Rose receives some funding from the Department of Health's NIHR Biomedical Research Centre for Ophthalmology at Moorfields Eye Hospital and UCL Institute of Ophthalmology.
Contributors All authors contributed equally in the researching and writing of this article.
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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