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Symptoms, if any, can be mild even in the presence of significant swelling or masses.
Lacrimal gland involvement may cause swelling of the upper eyelid, or proptosis if there is severe swelling. Other orbital masses or inflammation can result in visual disturbance (blurred vision, double vision, visual field impairment), restricted eye movements, pain or discomfort, numbness in the distribution of the supraorbital and/or infraorbital nerves, or proptosis.
IgG4-related ophthalmic disease has been estimated to account for approximately 25% of all cases of proptosis, eyelid swelling and other features of orbital swelling.
Affected individuals typically present with sudden painful proptosis, redness, and edema. Proptosis will vary according to the degree of inflammation, fibrosis, and mass effect. Occasionally, ptosis, chemosis, motility dysfunction (ophthalmoplegia), and optic neuropathy are seen. In the setting of extensive sclerosis there may be restriction, compression, and destruction of orbital tissue. Symptoms usually develop acutely (hours to days), but have also been seen to develop over several weeks or even months.Malaise, headaches, and nausea may accompany these symptoms. Other unusual presentations described include cystoid macular edema, temporal arteritis, and cluster headaches.
Pediatric IOI accounts for about 17% of cases idiopathic orbital inflammation. The most common sign is proptosis, but redness and pain are also experienced. Presentation varies slightly compared to adults with bilateral involvement, uveitis, disc edema and tissue eosinophilia being more common in this population. The presence of uveitis generally implies a poor outcome for pediatric IOI. Bilateral presentation may have a higher incidence of systemic disease.
Patients with trochleitis typically experience a dull fluctuating aching over the trochlear region developing over a few days. Some may also feel occasional sharp pains punctuating the ache. In patients with migraines, trochleitis may occur simultaneously with headache. Presentation is usually unilateral with palpable swelling over the affected area supranasal to the eye. The trochlear region is extremely tender to touch. Pain is exacerbated by eye movements looking down and inwards, and especially in supraduction (looking up) and looking outwards, which stretches the superior oblique muscle tendon. Notably, there is no restriction of extraocular movements, no diplopia, and often no apparent ocular signs such as proptosis. However, occasionally mild ptosis is found. The absence of generalized signs of orbital involvement is helpful in eliminating other more common causes of periorbital pain.
Idiopathic orbital inflammatory (IOI) disease, or orbital pseudotumor, refers to a marginated mass-like enhancing soft tissue involving any area of the orbit. It is the most common painful orbital mass in the adult population, and is associated with proptosis, cranial nerve (Tolosa–Hunt syndrome), uveitis, and retinal detachment. Idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, was first described by Gleason in 1903 and by Busse and Hochhmein. It was then characterized as a distinct entity in 1905 by Birch-Hirschfeld. It is a benign, nongranulomatous orbital inflammatory process characterized by extraocular orbital and adnexal inflammation with no known local or systemic cause. Its diagnosis is of exclusion once neoplasm, primary infection and systemic disorders have been ruled-out. Once diagnosed, it is characterized by its chronicity, anatomic location or histologic subtype.
Idiopathic orbital inflammation has a varied clinical presentation depending on the involved tissue. It can range from a diffuse inflammatory process to a more localized inflammation of muscle, lacrimal gland or orbital fat. Its former name, orbital pseudotumor, is derived due to resemblance to a neoplasm. However, histologically it is characterized by inflammation. Although a benign condition, it may present with an aggressive clinical course with severe vision loss and oculomotor dysfunction.
IgG4-related ophthalmic disease (IgG4-ROD) is the recommended term to describe orbital (eye socket) manifestations of the systemic condition IgG4-related disease, which is characterised by infiltration of lymphocytes and plasma cells and subsequent fibrosis in involved structures. It can involve one or more of the orbital structures.
Frequently involved structures include the lacrimal glands, extraocular muscles, infraorbital nerve, supraorbital nerve and eyelids. It has also been speculated that ligneous conjunctivitis may be a manifestation of IgG4-related disease (IgG4-RD).
As is the case with other manifestations of IgG4-related disease, a prompt response to steroid therapy is a characteristic feature of IgG4-ROD in most cases, unless significant fibrosis has already occurred.
Acute periostitis is due to infection, is characterized by diffuse formation of pus, severe pain, constitutional symptoms, and usually results in necrosis. It can be caused by excessive physical activity as well, as in the case of medial tibial stress syndrome (also referred to as tibial periostalgia, soleus periostalgia, or shin splints). Congenital infection with syphilis can also cause periostitis in newborn infants.
Periostitis, also known as periostalgia, is a medical condition caused by inflammation of the periosteum, a layer of connective tissue that surrounds bone. The condition is generally chronic, and is marked by tenderness and swelling of the bone and an aching pain.
The cause of trochleitis is often unknown (idiopathic trochleitis), but it has been known to occur in patients with rheumatological diseases such as systemic lupus erythematosus, rheumatoid arthritis, enteropathic arthropathy, and psoriasis. In his study, Tychsen and his group evaluated trochleitis patients with echography and CT scan to demonstrate swelling and inflammation. Imaging studies showed inflammation of superior oblique tendon/ trochlear pulley. It was unclear whether the inflammation involved the trochlea itself, or the tissues surrounding the trochlea.
Garre's sclerosing osteomyelitis is a type of chronic osteomyelitis also called proliferative periostitis, periostitis ossificans and Garré's sclerosing osteomyelitis.
It is a rare disease. It mainly affects children and young adults. It is associated with a low grade infection, which may be due to dental caries (cavities in the teeth).
The body of the mandible may show irregular lucent/opaque changes with subperiosteal opaque layering along inferior border. It is a chronic osteomyelitis with subperiosteal bone and collagen deposition.
There is no suppuration and sinus formation.
It was first described by the Swiss surgeon Carl Garré.
Orbital cellulitis
is inflammation of eye tissues behind the orbital septum. It most commonly refers to an acute spread of infection into the eye socket from either the adjacent sinuses or through the blood. It may also occur after trauma.
When it affects the rear of the eye, it is known as retro-orbital cellulitis.
It should not be confused with periorbital cellulitis, which refers to cellulitis anterior to the septum.
Common signs and symptoms of orbital cellulitis include pain with eye movement, sudden vision loss, chemosis, bulging of the infected eye, and limited eye movement. Along with these symptoms, patients typically have redness and swelling of the eyelid, pain, discharge, inability to open the eye, occasional fever and lethargy. It is usually caused by a previous sinusitis. Other causes include infection of nearby structures, trauma and previous surgery.
Symptom may include pain in a specific bone with overlying redness, fever, and weakness. Onset may be sudden or gradual.
Individuals with an enchondroma often have no symptoms at all. The following are the most common symptoms of an enchondroma. However, each individual may experience symptoms differently. Symptoms may include:
- Pain that may occur at the site of the tumor if the tumor is very large, or if the affected bone has weakened causing a fracture of the affected bone
- Enlargement of the affected finger
- Slow bone growth in the affected area
The symptoms of enchondroma may resemble other medical conditions or problems. Always consult your physician for a diagnosis.
Symptoms are usually limited to one side of the head, and in most cases the individual affected will experience intense, sharp pain and paralysis of muscles around the eye. Symptoms may subside without medical intervention, yet recur without a noticeable pattern.
In addition, affected individuals may experience paralysis of various facial nerves and drooping of the upper eyelid (ptosis). Other signs include double vision, fever, chronic fatigue, vertigo or arthralgia. Occasionally the patient may present with a feeling of protrusion of one or both eyeballs (exophthalmos).
For a person with arthritis mutilans in the hands, the fingers become shortened by arthritis, and the shortening may become severe enough that the hand looks paw-like, with the first deformity occurring at the interphalangeal and metacarpophalangeal joints. The excess skin from the shortening of the phalanx bones becomes folded transversely, as if retracted into one another like opera glasses, hence the description "la main en lorgnette". As the condition worsens, luxation, phalangeal and metacarpal bone absorption, and skeletal architecture loss in the fingers occurs.
Arthritis mutilans is a rare medical condition involving severe inflammation damaging the joints of the hands and feet, and resulting in deformation and problems with moving the affected areas; it can also affect the spine. As an uncommon arthropathy, arthritis mutilans was originally described as affecting the hands, feet, fingers, and/or toes, but can refer in general to severe derangement of any joint damaged by arthropathy. First described in modern medical literature by Marie and Leri in 1913, in the hands, arthritis mutilans is also known as opera glass hand ("la main en lorgnette" in French), or chronic absorptive arthritis. Sometimes there is foot involvement in which toes shorten and on which painful calluses develop in a condition known as opera glass foot, or "pied en lorgnette".
Some clinically observed signs and symptoms include:
- Orbital pain
- Eyes displaced posteriorly into sockets (enophthalmos)
- Limitation of eye movement
- Loss of sensation (hypoesthesia) along the trigeminal (V2) nerve distribution
- Seeing-double when looking up or down (vertical diplopia)
- Orbital and lid subcutaneous emphysema, especially when blowing the nose or sneezing
- Nausea and bradycardia due to oculocardiac reflex
On physical exam, the fracture appears as a loss of cheek projection with increased width of the face. In most cases, there is loss of sensation in the cheek and upper lip due to infraorbital nerve injury. Facial bruising, periorbital ecchymosis, soft tissue gas, swelling, trismus, altered mastication, diplopia, and ophthalmoplegia are other indirect features of the injury. The zygomatic arch usually fractures at its weakest point, 1.5 cm behind the zygomaticotemporal suture.
Periorbital cellulitis must be differentiated from orbital cellulitis, which is an emergency and requires intravenous (IV) antibiotics. In contrast to orbital cellulitis, patients with periorbital cellulitis do not have bulging of the eye (proptosis), limited eye movement (ophthalmoplegia), pain on eye movement, or loss of vision. If any of these features is present, one must assume that the patient has orbital cellulitis and begin treatment with IV antibiotics. CT scan may be done to delineate the extension of the infection.
Affected individuals may experience the following;
swelling,
redness,
discharge,
pain,
shut eye,
conjunctival injection,
fever (mild), slightly blurred vision, teary eyes, and some reduction in vision.
Typical signs include periorbital erythema, induration, tenderness and warmth.
Diagnosis is suspected by physical exam and history, in which, classically, the hard and soft palate of the midface are mobile with respect to the remainder of facial structures. This finding can be inconsistent due to the midfacial bleeding and swelling that typically accompany such injuries, and so confirmation is usually needed by radiograph or CT.
THS is usually diagnosed via exclusion, and as such a vast amount of laboratory tests are required to rule out other causes of the patient's symptoms. These tests include a complete blood count, thyroid function tests and serum protein electrophoresis. Studies of cerebrospinal fluid may also be beneficial in distinguishing between THS and conditions with similar signs and symptoms.
MRI scans of the brain and orbit with and without contrast, magnetic resonance angiography or digital subtraction angiography and a CT scan of the brain and orbit with and without contrast may all be useful in detecting inflammatory changes in the cavernous sinus, superior orbital fissure and/or orbital apex. Inflammatory change of the orbit on cross sectional imaging in the absence of cranial nerve palsy is described by the more benign and general nomenclature of orbital pseudotumor.
Sometimes a biopsy may need to be obtained to confirm the diagnosis, as it is useful in ruling out a neoplasm.
Differentials to consider when diagnosing THS include craniopharyngioma, migraine and meningioma.
Lefort I - Slight swelling of the upper lip, ecchymosis is present in the buccal sulcus beneath each zygomatic arch, malocclusion, mobility of teeth. Impacted type of fractures may be almost immobile and it is only by grasping the maxillary teeth and applying a little firm pressure that a characteristic grate can be felt which is diagnostic of the fracture. Percussion of upper teeth results in cracked pot sound. Guérin's sign is present characterised by ecchymosis in the region of greater palatine vessels.
Lefort II and Lefort III (common) - Gross edema of soft tissue over the middle third of the face, bilateral circumorbital ecchymosis, bilateral subconjunctival hemorrhage, epistaxis, CSF rhinorrhoea, dish face deformity, diplopia, enophthalmos, cracked pot sound.
Lefort II - Step deformity at infraorbital margin, mobile mid face, anesthesia or paresthesia of cheek.
Lefort III - Tenderness and separation at frontozygomatic suture, lengthening of face, depression of ocular levels (enophthalmos), hooding of eyes, and tilting of occlusal plane, an imaginary curved plane between the edges of the incisors and the tips of the posterior teeth. As a result, there is gagging on the side of injury.
The morphological appearance can be helpful in determining the cause of a periosteal reaction (for example, if other features of periostitis are present), but is usually not enough to be definitive. Diagnosis can be helped by establishing if bone formation is localized to a specific point or generalized to a broad area. The appearance of the adjacent bone will give clues as to which of these is the most likely cause.
Appearances include solid, laminated, spiculated, and Codman's triangle.
An enchondroma may occur as an individual tumor or several tumors. The conditions that involve multiple lesions include the following:
- Ollier disease (enchondromatosis) - when multiple sites in the body develop the tumors. Ollier disease is very rare.
- Maffucci's syndrome - a combination of multiple tumors and angiomas (benign tumors made up of blood vessels).
Swelling may be severe enough to put pressure on the eye and distort vision. Some patients first thought to have dacryoadenitis may turn out to have a malignancy of the lacrimal gland.