David H. Verity
Dr. Verity is a Consultant Ophthalmic Surgeon, and Adnexal Service Director, Moorfields Eye Hospital, London.He is a Honorary Senior Clinical Lecturer, University College London.Trained in ophthalmology in the UK, Dr. Verity undertook two Fellowships in ophthalmic adnexal disease before joining the Consultant staff at Moorfields Eye Hospital in 2004.He is a Full Member of the British Oculoplastic Surgery Society (current Treasurer), Treasurer Elect for the European Society of Ophthalmic Plastic and Reconstructive Surgery, and Full Member of the International Society of Ocular Oncology.In 2009, he was elected to the Orbital Society, in 2010 became Editor-in Chief of the journal ORBIT, and in 2016 became Oculoplastic Section Editor for the Journal of Ophthalmic and Vision Research.His medical publications in peer-reviewed journals and books include 90(+) publications, and with a wide range of research interests, he is an active national and international teacher and surgical trainer.Dr. Verity also has a life-long interest in the charitable work of the St John Eye Hospital Group. In 2014, he was invested in the Order of St John, and in 2016 he joined the Board as Trustee of the Hospital Group.
Geoffrey E. Rose
Geoffrey Rose graduated BSc Pharmacology, MBBS, and MRCP. His postgraduate ophthalmic training culminated in award of FRCS in 1985 and FRCOphth at its foundation in 1988. In 1990, the University of London granted an MS doctorate for corneal research and, in 2004, a Doctor of Science in Ophthalmology and Ophthalmic Surgery.
Professor Rose was appointed consultant to Moorfields Eye Hospital, London, in 1990 and is also a Senior Research Fellow of the NIHR Biomedical Research Centre of the Institute of Ophthalmology. He served as the British Council member of the European Society of Ophthalmic Plastic and Reconstructive, is a Past-President of the British Oculoplastic Surgical Society, and is President of the European Society of Oculoplastic and Reconstructive Surgeons.
Introduction
The acute medical management of non-thyroid orbital and lid inflammation depends on the likely aetiology, this either being deduced from the history and clinical findings or – where there is an intention to treat with immunosuppressants or immunomodulators – from the clinical features and histology of representative tissue(s). Since many diseases can manifest with orbital inflammation (Table 30.1) – including both benign and neoplastic disorders1 – the clinician should almost always obtain a tissue biopsy before starting treatment with glucocorticoids or other immunomodulatory agents.2 The hackneyed term ‘pseudotumour’ is misleading and should no longer be used, chiefly because suppression of orbital inflammatory signs without a tissue diagnosis can mask more sinister pathology and delay appropriate treatment: for example, empirical steroid suppression of the inflammation of an aggressive B-cell lymphoma delays staging and appropriate chemotherapy and/or radiotherapy. Thus, treatment with glucocorticoids without a histological diagnosis is rarely indicated, and, with few exceptions, a tissue biopsy should precede treatment.
Table 30.1
Causes of orbital inflammation
(A) Infectious disease |
Bacterial, viral, fungal and parasitic disease |
(B) Idiopathic inflammations |
Thyroid eye disease |
Myositis |
Scleritis |
Sarcoidosis |
Granulomatous polyangiitis (GPA; previously termed Wegener’s granulomatosis). |
IgG4-related disease |
Reactive lymphoid hyperplasia |
Inflammatory bowel disease |
Histiocytic disease [1] |
Rarer vasculitic causes of orbital inflammation: |
Churg–Strauss syndrome |
Polyarteritis nodosa |
Systemic lupus erythematosus and rheumatoid arthritis |
Temporal arteritis |
Dermatomyositis |
(C) Neoplastic disease |
Rhabdomyosarcoma |
Necrosis of primary or secondary orbital neoplasia |
Haematological malignancy |
Lymphoma |
(D) Miscellaneous |
Cystic lesions: |
Congenital dermoid cyst |
Congenital conjunctival cyst |
Encysted foreign body (including parasitic disease) |
Lymphangioma |
Retained foreign body |
In this chapter, the acute medical management of common eyelid, periorbital and orbital inflammations is summarised. Emphasis is given throughout to the importance of avoiding empirical treatment, and management should be based on representative histopathology to avoid dangerous delays in diagnosis.
Preseptal Inflammation and Infection
Idiopathic Eyelid Inflammation
Severe lipogranulomatous (non-infective) lid inflammations can be particularly difficult to manage, and where the underlying pathology is in doubt with a prolonged ‘cellulitis’, a full-thickness tissue biopsy should be performed to exclude dermatomyositis, discoid lupus erythematosus and neoplasia – with sebaceous cell carcinoma being a recognised cause of recalcitrant lid inflammation (or ‘unilateral blepharitis’). The inflammatory changes seen in severe lipogranulomatous disease are due to retained sebaceous secretions from the glands of Zeiss or Meibom, with acne rosacea and/or seborrhoeic dermatitis frequently being present in such severe cases. Histology shows focal or diffuse inflammation which can involve the entire lid and lead to formation of localised abscesses. Treatment with topical steroid drops and oral doxycycline or lymecycline is generally effective, with topical and oral antibiosis being added where there is evidence for secondary infection; biopsy should be reserved for resistant cases.
Preseptal Infections
Acute staphylococcal meibomitis and bacterial infections of the lash follicles or associated glands of Zeiss or Moll present as tender localised swellings that usually resolve spontaneously or discharge; medical management in the form of topical antibiosis usually suffices, with drainage reserved for refractory cases. Acutely infected lacrimal dacryocoeles are best managed by marsupialising the dilated ductule into the conjunctival sac and evacuation of pus, this leading to rapid resolution. Actinomyces is a common cause of canaliculitis and is reliably cured with canaliculotomy and evacuation of debris and stones, antibiotic drops being largely ineffective. Meibomian ducts per se rarely become infected, causing chronic discharge of oil onto the ocular surface, and may require evacuation of debris and any associated calcific concretions. In the presence of major underlying meibomian gland dysfunction or rosacea, a prolonged (3–6 month) course of oral doxycycline or lymecycline should be considered.
Infections of brow hair follicles and epidermoid cysts can also lead to local abscess formation (Fig. 30.1), with local antibiotic preparations being insufficient to prevent a wider cellulitis developing. In such cases, prompt drainage of localised loculations of pus usually leads to rapid resolution.
Fig. 30.1
Acute brow abscess rapidly resolving with surgical drainage
The presentation of herpes zoster ophthalmicus (HZO; Fig. 30.2) is well known: treatment with an oral antiviral such as acyclovir (800 mg five times a day), famciclovir (500 mg tid) or valaciclovir (1 g tid) is initiated immediately and continued for 7–10 days. Rarely, herpes zoster can also cause orbital inflammation, this including apical involvement with visual loss. Oral corticosteroids reduce the duration of acute pain and accelerate cutaneous healing, but do not decrease the incidence of postherpetic neuralgia, and should be reserved for patients who are relatively healthy and who have no contraindication to their use.
Fig. 30.2
Acute herpes zoster ophthalmicus with vesicle formation above the brow. Note dependent oedema in the lower lid
Necrotising Fasciitis (NF)
Rapidly progressive periorbital inflammation with tissue necrosis strongly suggests a diagnosis of necrotising fasciitis (NF) and is a medical emergency (Fig. 30.3) [2]. A proportion of patients are diabetic or have a history of local injury, but many have no identifiable risk factors. NF carries a high risk of septicaemia, organ failure, disseminated intravascular coagulation (DIC) and death. The immediate management involves demarcation of the area to determine the extent of disease and its progression, very high-dose antibiosis to treat streptococcal species3 and surgical debridement down to healthy tissues (with repeat tissue excision where there is progression at any stage) unless there is prompt response to initial medical treatment. The management of this emergency is shared between the ophthalmologist, internist and microbiologist, and the importance of early and aggressive therapy cannot be overstated, with these authors having treated a number of patients whose late presentation resulted in dire systemic complications and even death.
Fig. 30.3
Acute necrotising fasciitis of the left upper lid
Systemic Inflammatory Disease with Eyelid Involvement
Idiopathic inflammations which can affect the eyelids include eczema (Fig. 30.4a), discoid lupus erythematosus (Fig. 30.4b), sarcoidosis (typically presenting with cutaneous plaques; Fig. 30.4c), xanthogranuloma and dermatofibrosis. Where the underlying aetiology is uncertain, tissue biopsy and discussion with the histopathologist or dermopathologist are essential. The advice of a dermatologist should be sought in refractory cases or where there is dermatopathy elsewhere, with treatment being directed at the underlying aetiology – this typically being in the form of topical glucocorticoid or tacrolimus for severe eczema and similar systemic treatment for sarcoidosis and xanthogranuloma where there is evidence either for deeper orbital disease or peripheral involvement.
