Ординатура / Офтальмология / Английские материалы / Moorfields Manual of Ophthalmology_Jackson_2007

Orbital vascular lesions

Lesions may ache, and cause pain and proptosis if they bleed. Superficial lesions have visibly dilated veins (Fig. 3.11). Repeated haemorrhages and scarring may cause enophthalmos due to fat atrophy. There may be involvement of contiguous structures (CNS, face, sinuses, temporalis fossa). Static CT or MRI shows multiple irregular masses, often along the course of a vein ± signs of prior bleeding. There may be a thin hyperdense rim on CT. Angiography is rarely required. Dynamic CT, MRI, or USS may show filling with Valsalva. USS shows cystic spaces. Management may involve amblyopia therapy or surgical excision for unremitting pain, severe cosmetic disfigurement, or massive expansion. Surgical risks include haemorrhage and recurrence. Drainage of acute haemorrhage (‘chocolate cysts’) is possible. Interventional radiologic techniques and injection of a sclerosants such as glue may reduce lesion size.

No-flow malformations (a.k.a. ‘lymphangiomas’)

Superficial lesions (Fig. 3.12) produce intermittent swelling, ecchymoses, and disfigurement of the lids and conjunctiva; deeper lesions produce intermittent haemorrhage or thrombosis, with episodic swelling, visual disturbance, or dysmotility. May be extraorbital, especially involving the face and oropharynx. Imaging and treatment are similar to low-flow malformations, but lesions are nondistensible and more infiltrative.

Orbital lymphoproliferative disorders

Orbital Lymphoproliferative

Disorders

Background Orbital lymphoproliferations are divided into reactive lymphoid hyperplasia, intermediate lymphoproliferation (atypical lymphoid hyperplasia), and lymphoma. Orbital T-cell lymphoma is extremely rare. B-cell lymphoma is histologically divided into low and high grade, the former being much more common. The most common B-cell lymphoma is marginal zone type. The orbit may be involved secondarily from adjacent or distant B-cell lymphoma. Conversely, orbital lymphoma may spread to extraorbital sites, e.g. CNS.

Clinical features The typical presentation is a gradual onset of a painless infiltrative mass in the sixth and seventh decades, often with nonaxial proptosis. Disturbed ocular motility may occur, but neurovascular or visual compromise is uncommon (Fig. 3.13). Conjunctival involvement (≈20%) is classically ‘salmon patch’ in colour and texture. Lacrimal gland involvement is common. Highgrade tumours produce a more rapid, infiltrative course.

Investigation Distinguish from non-neoplastic or idiopathic inflammatory disorders (p. 87). CT typically shows a well-defined, enhancing, ‘moulding’, extraconal, homogeneous, soft-tissue mass. Calcification is uncommon. Biopsy, sometimes repeated, is mandatory, and immunohistochemistry ± gene rearrangement studies (Ig heavy chain PCR) are usually required. A fresh specimen may be required for flow cytometry.

Management

■Reactive lymphoid hyperplasia : Extraorbital, especially lymph node, involvement is not uncommon. Progression to lymphoma

occurs in ≈10% of cases. The response to prednisolone is generally excellent; low-dose radiotherapy (XRT) may be required for functional disturbance; debulking or decompressive surgery may be required for gross mass effect or proptosis.

■Intermediate lymphoproliferaton : Behaves similar to low-grade B-cell lymphoma. A systemic immunoregulatory disorder may coexist. The response to prednisolone may be poor, requiring immunosuppression or XRT.

■B-cell lymphoma : Refer all patients to a haematologist/

oncologist for systemic investigation. The risk of systemic disease varies with site (eyelid > orbit > conjunctiva) and is higher with bilateral disease. About 25% have prior or

concurrent systemic disease, and 50% subsequently. Lowgrade lymphoma is treated with XRT (≈24 Gy), or systemic disease with chemotherapy (e.g. chlorambucil) often with good response. Higher-grade, systemic disease generally requires chemotherapy and or XRT (30–35 Gy). Alternatives exist, e.g. monoclonal antibodies and allogeneic transplants. As the prognosis is generally good, consider the risk versus benefit of treatment.

ORBIT 3 Chapter

Lacrimal gland tumours

Lacrimal Gland Tumours

Pleomorphic adenoma

Background The commonest benign lacrimal gland tumour. May involve the orbital (majority) or palpebral lobe of the lacrimal gland.

Clinical features Classically presents in the second to fifth decades as very slowly progressive proptosis, inferomedial globe displacement, and superotemporal quadrant lid swelling. Ocular indentation may cause blurred vision, choroidal folds, and a hypermetropic shift. Ocular dysmotility may occur. Pain, sensory disturbance, and inflammation are uncommon and suggest malignancy.

Investigations CT typically reveals a round or bosselated, well-circumscribed, enhancing mass indenting the globe and lacrimal fossa. Calcification occurs in 3%.

Differential diagnosis (of superotemporal quadrant masses) Other benign lacrimal gland tumours (rare), malignant lacrimal gland tumours, lymphoproliferation, neural tumours (e.g. neurofibroma), idiopathic orbital inflammatory disease, Sjögren’s syndrome, sarcoidosis, Wegener’s granulomatosis, dermoid or lacrimal cysts, extrinsic tumours.

Management Refer routinely to an experienced orbital surgeon. Clinicoradiologic features should suggest the diagnosis. Incisional biopsy is contraindicated as it increases the risk of recurrence and malignant transformation. Complete excision with an intact pseudocapsule is required, often via a lateral orbitotomy. Keratoconjunctivitis sicca may occur postoperatively, even with preservation of the palpebral lobe.

Follow-up: 5–10 years to exclude malignant transformation that may occur despite histological clearance. If histological foci of malignant change are found, extend follow-up and consider radiotherapy as for lacrimal gland carcinoma.

Differential diagnosis Similar to pleomorphic adenoma (see previous section), which must itself be excluded clinicoradiologically. Consider carcinoma in cases of nonresolving dacryoadenitis.

Clinical features Typically, a brief (<1 year) history of a superotemporal quadrant mass with pain, sensory loss, and paraesthesiae. Inflammation may occur.

Investigations CT classically shows an infiltrative tumour moulding to the globe with finely irregular bony erosion, and calcification in 33%.

Management Refer to an experienced orbital surgeon for urgent incisional biopsy to confirm the diagnosis. Treatment of most well-defined tumours then involves complete local excision, occasionally exenteration or radical craniofacial resection, followed by radiotherapy.

Follow-up Review lifelong to treat the inevitable dry eye, radiation-induced cataract, and monitor for local or systemic recurrence, particularly pulmonary.

ORBIT 3 Chapter

Fibrous histiocytoma

Rhabdomyosarcoma

Background The commonest primary malignant mesenchymal tumour of childhood. May be spontaneous (majority) or familial.

Clinical features Orbital involvement typically presents in the first decade with rapid proptosis (weeks), superotemporal swelling with globe displacement, lid swelling, erythema, and ptosis. Pain, visual loss, and watering are uncommon. A small number arise from extraorbital sites with secondary orbital spread. May rarely present in the elderly.

Differential diagnosis Consider inflammatory disorders (e.g. orbital cellulitis), haemangioma, lymphangioma, aggressive neurofibroma, and poorly differentiated tumours (e.g. neuroblastoma, Ewing’s and other sarcomas).

Investigations CT usually shows a homogeneous, welldefined, enhancing, soft tissue mass without bony destruction – typically not including the muscles. Biopsy (incisional or excisional) is required for histologic typing.

Management Treat with excisional biopsy. Refer to a paediatric oncologist for staging and multiagent chemotherapy, which generally results in a good prognosis. Radiotherapy may be required.

Fibrous Histiocytoma

The commonest adult mesenchymal orbital tumour. Usually presents in middle age with a slow-growing, firm, superonasal mass, proptosis, and visual loss. Dysmotility, pain, lid swelling, and ptosis are less common. Infiltration and local recurrence are typical but metastases rare. Treatment involves complete local resection, and occasionally radical excision or exenteration to prevent local recurrence. Radiotherapy is unhelpful and the role of chemotherapy unclear.

Orbital Trauma

Background The commonest orbital injury is a ‘blowout’ fracture, typically involving the floor and/or medial wall. If the injury results from an alleged assault or workplace injury, make detailed notes documenting timing and circumstances of the injury: measure or preferably photograph all injuries. For traumatic optic neuropathy see page 661.

History Ask about the nature of the injury, any visual symptoms including diplopia, blurred vision or field loss, periorbital sensory loss, dental malocclusion, and trismus (inability to open the mouth).

Examination Check VA, RAPD, colour vision, infraorbital sensation, ocular motility, exophthalmometry (note any nonaxial globe dystopia), and bony orbital rims. Perform a careful head, ocular, and general assessment to exclude other injuries.

Mangement Consider the following diagnoses:

■Orbital floor fracture Features include: periorbital ecchymoses; subconjunctival haemorrhage (SCH); enophthalmos; limited eye movement, especially upgaze (from inferior rectus entrapment, bruising, oedema or neurapraxia); infraorbital nerve hypoaesthesia/numbness/pain; subcutaneous emphysema. Children may have a ‘greenstick’ fracture with significant muscle entrapment but minimal bruising (‘white eye blowout’) and nausea and vomiting, especially on upgaze.

ORBIT 3 Chapter

Orbital trauma

Request CT (same or next day) (Fig. 3.14) with fine cuts of orbits and face (axial, coronal ± sagittal). Consider plain X- rays if CT is unavailable, and skull and cervical X-rays as indicated. Ban nose-blowing for 10 days.

Surgery is required in the following situations:

■Child: a white-eye blowout requires surgery within 48–72 hours.

■Enophthalmos >2 mm and symptomatic: this may become apparent weeks after injury.

■Greater than 50% of the floor area involved, as there is a high risk of late enophthalmos.

■Diplopia that fails to resolve after 2–3 weeks.

Monitor diplopia carefully with serial Hess charts. Some diplopia on extreme upor downgaze may persist, but if restriction affects the primary position or the field of binocular vision at 2–3 weeks then consider orbital exploration, and fracture repair. Persistent diplopia may require inferior rectus recessions, and/or Faden procedures of the superior rectus and inferior rectus of the unaffected eye. Ipsilateral inverse Knapp procedure can be helpful after about 6 months if improvement has stopped. In general, results are often disappointing. Consider decompressing the infraorbital canal if there is significant pain in the inferior orbital nerve territory. Options for repair include alloplastic material (e.g. porous polyethylene) or autogenous material (e.g. cranial bone).

■Medial orbital wall fracture Often has subcutaneous emphysema, variable ecchymoses, medial rectus (MR) dysfunction, enophthalmos, or proptosis. Repair if there is

significant MR entrapment causing pain, diplopia, or if enophthalmos >2 mm. Otherwise observe. Ban nose blowing for 10 days.

■Lateral orbital wall and roof fracture Isolated lateral wall fractures are rare and are usually associated with zygomaticoorbitomaxillary complex fractures. Small, undisplaced roof fractures may be monitored for neurologic and ophthalmic complications with broad-spectrum antibiotic cover (e.g. Co-amoxiclav).

■Orbital haematoma May occur in blunt trauma, bleeding diatheses, or vascular anomalies. Features include: severe