Orbital Conditions | Jimmy Uddin | Oculoplastic Surgeon London

Mr Uddin specialises in the diagnosis and treatment of orbital disorders, treating a large number of patients both adults and children. He sees patients from all over the UK as well as internationally, accepting referrals from many countries across the globe.

Over the 20 years as a specialist orbital surgeon at Moorfields, he has performed many orbital biopsies and excision of tumours, which require very specialised expertise.

He has performed over 500 orbital decompression procedures, for thyroid eye disease, over the last ten years. He has an extensive thyroid eye disease (TED) practice and runs the TED service at Moorfields.

Mr Uddin has set up the multidisciplinary orbital service at St George’s Hospital Medical School, where he regularly manages orbital trauma, infections, tumours and deformity with an excellent, highly specialised, well-established team including specialists in maxillofacial surgery, ENT, neurosurgery, head & neck oncology, endocrinology, rheumatologist, radiology and pathology.

He has been pivotal in training over 50 specialists who have become consultants in the United Kingdon, Europe, Asia, USA, Canada, Australia, New Zealand and Africa.

“The orbit supports the function and form of eye and eyelids...serving vision and cosmesis. It is a complex structure with a myriad of internal and adjacent structures ..... Any of which can go wrong resulting with problems with eyesight, pain and appearance”

What is the orbit?

The orbit or eye socket is the bony cavity which contains the eyeball and accompanying muscles, soft tissues, blood vessels and nerves. Its purpose is to protect and serve the eye and associated structures. Due to its proximity to the sinuses, brain, and a myriad of other delicate structures, it is vulnerable to many types of inflammation, infection, tumour, injury and deformity.

MDT (multidisciplinary team working)

Many orbital conditions are initially investigated and diagnosed by the orbital, oculoplastic ophthalmologist (who have been specially trained and experienced in this particular field of medicine). Many conditions are managed by the orbital specialist.

However, there are many conditions where the patient is best served with a team. Mr Uddin has developed and worked closely with many world-class teams for the various orbital conditions that he manages regularly.

Types of MDTs

At Moorfields, Mr Uddin works with experienced orbital and radiologists and pathologists.

At St George’s Hospital Medical School, he has set up a 20-year orbital service of maxillofacial, ENT and Neurosurgery with input to head & neck oncology and the rest of the hospital services. Orbital trauma, facial nerve and tumours are commonly managed here, as well as Guys & St Thomas’ and St Bart’s team

Endocrinology and medical physician input include doctors from St Thomas’ Hospital, UCL and others.

Orbital inflammation

Thyroid eye disease and other inflammatory conditions need experienced radiologists and pathologists to assist in diagnosis. Endocrinologists, rheumatologists, medical ophthalmologists and immunologists help with the medical care

Orbital Trauma

Mr Uddin and his team have 20 years’ experience of managing orbioi-facial trauma. Central to this is the function of the eye, but also the form and appearance of the person, the whole person. As well as dealing with acute trauma, Mr Uddin specialises in late reconstructive trauma and rehabilitation to restore eye function and excellent cosmesis where possible.

Orbital tumours

Excellent radiology points to the likely diagnosis but sometimes a diagnosis can be ambiguous, so an experienced orbital pathologist is necessary to make a definitive diagnosis. These are managed in the appropriate MDT. For example, orbital lymphoma is the most common orbital malignancy and needs highly specialised treatment options with radiotherapy and chemotherapy.

Orbital “deformity”

This is a poor term used to describe conditions that change the appearance of the orbit and face including congenital and progressive conditions (eg fibrous dysplasia) as well as post-trauma or tumour. This often affects the bones of the orbit, skull and face, and is best served within an experienced MDT

Varied symptoms of orbital disease

Symptoms often point to the most likely diagnoses

Timing of symptoms

Sudden progression of symptoms is often seen with acute bleeding, acute inflammation and aggressive tumours, as well as trauma.

Slow and progressive changing symptoms may indicate tumours, inflammation and fibrosis.

Old photographs are useful to compare longstanding changes.

Orbital swelling
When any of the tissues in the orbit or surrounding structures increase in size, the eye may be pushed forward and look bigger. Localised swelling may indicate which structure is likely to be involved. There may be swelling of the upper or lower eyelids which sometimes can be misdiagnosed as “just age-related puffiness”.

Orbital pain
Pain behind the eye can indicate inflammation or infection. Sometimes rapidly growing tumours, bleeding or swelling can cause pain. Pain with eye movements may indicate inflammation of the muscle in myositis or TED

Nerve pain
Numbness or nerve pain is a serious indicator of disease.

Double vision
Eye movement problems and double vision indicates muscle involvement, swelling of adjacent tissue and or damage to the motor nerve.
Vision and eyesight

Reduction in vision may be due to orbital swelling so must always be carefully assessed. Reduction in colour vision (especially loss of red colour perception) may indicate optic nerve damage.

Gaze-evoked (looking in a particular direction) visual disturbance can indicate tumours near the optic nerve

Investigations

Imaging is the primary investigation in orbital disease

CT scans (Computed Tomography) is quick, effective and inexpensive. It gives good definition of bone (esp for trauma) and soft tissue, and is diagnostic for many orbital conditions.
It is used sparingly with children to reduce radiation dose.
MRI scans (Magnetic Resonance Imaging) is good for soft tissue of the orbit. It can help identify water content, cellular density, the orbital apex, thrombosis and other positive findings. It takes longer to acquire.
Colour doppler ultrasound is very useful to look how vascular a lesion is and whether it is high or low flow.
Blood test can investigate thyroid problems, autoimmune disorders, GPA, sarcoid etc.
Chest x-ray/CT may be helpful to associated conditions including sarcoid and TB
Angiography is used for vascular lesions including fistulae.
PET scans (Positron Emission Tomography) is helpful to look at “active” tissues and local and distant involvement.

Disease Tissue typing (biopsy)

A biopsy or sampling of the affected tissue is sometimes the best way of making a diagnosis, by examining with special stains, microscopy and immunostaining (Histology).

Tissue is taken with an operation under local or general anaesthesia

Incisional biopsy: An incision is made, and a piece of tissue is removed and sent for histology. This way, you have a larger, representative sample to examine, thus getting a more accurate result and diagnosis.

Excisional biopsy: With some conditions (eg pleomorphic adenoma/solitary fibrous tumour/ dermoid/ well defined tumours) a complete, intact removal of the tumour is preferable to reduce the chance of re-operation or complications.

FNA (fine needle biopsy/tru cut biopsy). A needle or wider bore needle is placed into the tissue to remove cells of “core-biopsy” tissue. It is often performed under local or no anaesthesia.

It may be guided by ultrasound or CT scan.

Orbital swelling

When any of the tissues in the orbit or surrounding structures become increase in size, the eye may be pushed forward and look bigger. Localised swelling may indicate which structure is likely to be involved. There may be swelling of the upper or lower eyelids which sometimes can be misdiagnosed as “just age-related puffiness”. Pain behind the eye can indicate inflammation or infection. Numbness or nerve pain is a serious indicator of disease. Eye movement problems and double vision indicate muscle involvement. Reduction in vision may be due to orbital swelling so must always be carefully assessed. Slow and progressive changing symptoms may indicate tumours.

Orbital inflammation

Hallmarks of inflammation are pain, redness, and swelling. This is not always apparent if the inflammation is deeper in the orbit. Loss of function of the structures involved indicates which anatomical structures may be involved. For example, weakening orbital muscles can result in double vision, and sight problems may indicate optic nerve involvement

What causes the orbit to become inflamed?

The tissues in the orbit can become inflamed, which can cause the eye to be pushed forward and appear as if they’re bulging (proptosis). In some cases, the bulging prevents the eyelids from closing, which can also cause problems with the cornea (the protective outer layer of the eye). Pain is a common feature.

This inflammation can be caused by a cyst or an abscess (collection of pus) or a result of an infection such as sinusitis. It can also be a symptom of thyroid eye disease. Autoimmune conditions or in rare cases a tumour could be responsible.

Occasionally, the inflammation may occur spontaneously without any obvious cause. This is referred to as Idiopathic Orbital Inflammatory Syndrome (IOI). People with IOI may experience proptosis, red-eye, pink eyelid swelling, double vision and vision loss. In children, IOI may also cause a fever, headaches, vomiting and abdominal pain.

Investigations including blood tests and imaging (CT/MRI) often indicate the cause. Sometimes a biopsy is required to look at the tissue to make a diagnosis; this is especially important when diagnosing a tumour.

Dacryoadenitis: Tear gland inflammation

The lacrimal glands which produce tears can also become inflamed, this is referred to as dacryoadenitis. It is usually caused by specific or non-specific inflammation (eg GPA/sarcoid) or viral infection such as mumps or the Epstein-Barr virus (the organism that causes glandular fever). A person with dacryoadenitis may experience eye pain, redness and eyelid swelling (in the upper outer part of the eyelid) causing a “ptosis, often with watery eye and discharge. Blood tests and CT scan is usually required. A biopsy is sometimes needed.

GPA: Granulomatosis with Polyangiitis (Wegener's)

IgG4 disease/sclerosing inflammation

Myositis

Idiopathic orbital inflammation

What causes an orbital infection?

The orbit can become infected; this is known as orbital cellulitis. It can be very serious with spreading, invasive infection which impairs vision. It is usually caused by bacterial infection spreading from a sinusitus. Symptoms include proptosis, red eye, pain, blurred or double vision and swelling around the eyes. Some also experience nasal congestion or discharge, sinus pressure, headaches and tooth pain. It is usually treated with antibiotics, occasionally surgery is needed if vision is impaired.

Can tumours form in the orbit?

Although orbital tumours are very rare, Mr Uddin sees over 100 cases each year, due to his referal pattern. These tumours can either be benign (may grow locally and not spread to other tissues) or malignant (cancerous, may invade other tissues or spread to other parts of the body). Sometimes the tumour can be an indication of a cancer that originated from another part of the body (metastasis)

Symptoms of an orbital tumour are similar to many other conditions these include proptosis, pain, double or loss of vision and droopy eyelids. Numbness around the skin is a sinister feature.

It’s important to reach a diagnosis first. Sometimes this can be done with just the clinical finding and imaging in the form of a computed tomography (CT) or magnetic resonance imaging (MRI) scans and ultrasound.

Often a biopsy (to take a piece of tissue and examine under a microscope to make a diagnosis) is required. Sometimes, it is better to removal the whole tumour.

If it turns out to be malignant this may require surgery, MDT work, and treatment including drugs to treat it and/or radiation therapy.

Dermoid (cysts) and dermolipoma

Orbital lymphoma and lymphoproliferative

Orbital lymphoma is the most common malignant tumour of the orbit, There are different types. The least aggressive is slow-growing, painless and following imaging to locate it, a biopsy is performed to make the diagnosis. Radiotherapy and/or “mild” chemotherapy often is the only treatment necessary.

More aggressive forms grow more quickly, may have pain and need more aggressive oncology input and treatment

Lacrimal gland tumours

Tumours may be benign or malignant.

Pleomorphic adenomas are usually slow growing and can be seen in old photographs.

Sudden swelling or pain may indicate malignant transformation.

Diagnosis is made with imaging and treatment is complete excision.

Lacrimal gland carcinoma
Often presenting with sudden swelling and the eye may be pushed down. There may be
numbness.

Imaging with CT and MRI followed by a biopsy.

Management is complex and controversial and options for treatment needs to be explained
carefully.

Cavernous haemangioma/Haemangiopericytoma

Orbital Vascular Conditions

Sometimes disorders affecting the blood vessels within the orbit and brain can cause orbital problems.

Usually bleeding from arteries or veins or blockages, can cause sudden symptoms such as proptosis (eye protruding), sometimes with accompanying bruising around the eye. If this happens, the person should be seen urgently.

Other vascular abnormalities can cause symptoms more gradually. The person may an underlying condition from birth. They involve abnormal connections between blood vessels such as aterio venous malformations or vascular fistulae. These are managed a specialised vascular MDTs and Mr Uddin is part of very experienced teams for this, including interventional radiologists.

Sometimes abnormalities within blood vessel walls cause dilatation or enlargement of blood vessels due to an acquired fistula. These sometimes have a “whooshing” sound in the head. They can also be mistaken for TED.

Vascular malformations causing orbital disease include capillary, arteriovenous as well as lymphatic malformations.

Capillary haemangioma presents very soon agates birth, enlarge in the first few months to years. They may be an arable to medical therapy such as beta-blockers. Occasionally surgery or steroids are necessary.

There are also syndromes associated with more diffuse malformations such as Sturge Weber syndrome, OslerWeber Rendu and Wyburn Masson Syndrome.

These conditions require very careful investigation and management to provide the most appropriate treatments.

Menigiomas
Nerve sheath tumours
Histiocytic tumours
Rhabdomyosarcoma

Orbital invasion of skin tumours (BCC/SCC/Sabaceous cell carcinoma

Breast cancer

Other orbital tumours

Orbital lymphoma and lymphoproliferative

Orbital trauma, fractures & deformity

Orbital and facial trauma can result in immediate that need urgent treatment (for example sight loss or entrapment of eye muscles).
There can be many problems that become evident once the trauma has settled. This can be related to visual problems of the optic nerve, eye itself or double vision. Appearance and lid malpositions can be difficult to quantify, such as a sunken eye appearance and droopy eyelids. This will be as a result of underlying bony changes, soft tissue changes and functional changes of eyelid muscles and skin.
These need to be assessed, often in teams including the oculoplastic/orbital surgeon and maxillofacial team.

How are orbital fractures treated?

Injury or trauma to the head can cause an orbital bone fracture. Symptoms include pain and swelling behind and around the eye, as well as bruising or bleeding. Double or impaired vision may also occur due to the swelling or if the eyeball is damaged. A computed tomography (CT) scan is usually performed and surgery may be necessary if the vision is affected, the fracture is large or the eyeball sunken.

If there is pain and sickness especially in under 20-year-olds, there may be entrapment of the muscle and needs urgent care.

Orbital floor fracture is a common fracture where the bottom wall or floor of the orbital is blown out. This can be repaired effectively.

When other bones are affected, the orbital surgeon assists maxillofacial surgeons to get the most effective assessment of the eye, muscles and soft tissues to decide about whether surgery is required and timing of surgery.

Orbital deformity

Sunken eye appearance: Enophthalmos

Sunken eye or enophthalmos can occur for a variety of reasons. The eye appears to be sunken down in the orbit and may therefore look smaller than the other side. Usually, one side is affected but sometimes both sides can be involved.

Enophthalmos can be associated with lid drooping (ptosis) or double vision. However, sometimes the other eye appears to stand out in comparison to the other side and people are referred because of this. The aesthetic appearance of the sunken eye may be the main issue for others looking for surgery to improve appearance.

The most common cause of enophthalmos is trauma and orbital fractures but there are other conditions that need to be considered if no injury has occurred.

Shrinking of normal fatty tissue within the orbit can also result in the sunken eye appearance. This may be a normal age-related process but can occur in the presence of abnormalities of blood vessels of the orbit, or even some glaucoma drops.

Conditions causing shrinking of muscles or connective tissues (fibrosis) can also cause the eye to sink back into the orbit. In these cases, there are also problems with eye movement and may be due to tumours (eg breast cancer or sclerosing inflammation)