Orbit & Lacrimal

Systems
Prepared By
Dr. Faraidoon Fatih M.A.
FICMS, CAB. Ophth, FICO
Fellowship at Gulhane military medical academy/ Ankara








Anatomy
   1    The roof consists 0f lesser wing of the sphenoid and frontal. 

   2    The lateral wall consists of the greater wing of the sphenoid and the zygomatic. 

   3    The floor consists of zygomatic, maxillary and palatine. 

   4    The medial wall consists of maxillary, lacrimal, ethmoid and sphenoid. The
lamina papyracea, which forms part of the medial wall, is paper-thin . Orbital cellulitis is
therefore frequently secondary to ethmoidal sinusitis.

   5    The superior orbital fissure is a slit between the greater and lesser wings of
the sphenoid bone, through which pass the following important structures.   
•    The superior portion contains the lacrimal, frontal and trochlear nerves, and the
superior ophthalmic vein.

   •    The inferior portion contains the oculomotor nerve, the abducens and nasociliary
nerves and sympathetic fibres from the cavernous plexus.


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Clinical signs
Soft tissue involvement
      Signs include lid and periorbital oedema, skin discoloration, ptosis, chemosis and epibulbar
injection
Proptosis
It describes an abnormal protrusion of the globe which may be caused by retrobulbar lesions or,
less frequently, a shallow orbit. Asymmetrical proptosis is best detected by looking down at the patient
from above and behind . Exophthalmos specifically describes proptosis of the eye and is
sometimes used to describe the bulging of the eye associated with Graves ophthalmopathy.
Enophthalmos
It implies recession of the globe within the orbit
Dystopia
It implies displacement of the globe in the coronal plane, usually due to an extraconal orbital mass
such as a lacrimal gland tumour.
Ophthalmoplegia
it is defective ocular motility .
Dynamic properties
1    Increasing venous pressure by dependent head position, Valsalva manoeuvre or jugular
compression may induce or exacerbate proptosis in patients with orbital venous anomalies or infants
with orbital capillary haemangiomas.
   2    Pulsation is caused either by an arteriovenous communication or a defect in the orbital roof.   
3 A bruit is a sign of of carotid-cavernous fistula.
     Fundus changes
   1    Optic disc swelling may be the initial feature of compressive optic neuropathy
   2    Optic atrophy
  








Thyroid eye disease (TED)
Risk factors
•Smoking
• Women
•Radioactive iodine used to treat hyperthyroidism .
Clinical manifestations
• soft tissue involvement,
• lid retraction, TED is the most common cause of eyelid retraction.
The upper lid margin normally rests 2 mm below the limbus . Lid
retraction is suspected when the margin is either level with or above the
superior limbus, allowing sclera to be visible ('scleral show’). Likewise, the
lower eyelid normally rests at the inferior limbus; retraction is suspected when
sclera shows below the limbus.
    1    Dalrymple sign is lid retraction in primary gaze .
   2    Kocher sign describes a staring and frightened appearance of the eyes
which is particularly marked on attentive fixation
  3    Von Graefe sign signifies retarded descent of the upper lid on downgaze
• proptosis, TED is the most common cause of unilateral or bilateral proptosis.
• Optic neuropathy
• Restrictive myopathy.
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 Preseptal cellulitis
It is an infection of the subcutaneous tissues anterior to the orbital septum.  In contrast to
orbital cellulitis proptosis and chemosis are absent; visual acuity, pupillary reactions and ocular
motility are unimpaired.
Bacterial orbital cellulitis
Bacterial orbital cellulitis is a life-threatening infection of the soft tissues behind the orbital
septum. It can occur at any age but is more common in children. The most common causative
organisms are S. pneumoniae, S. aureus, S. pyogenes and H. influenzae.
Pathogenesis
   1    Sinus-related, most commonly ethmoidal, typically affects children and young adults.
   2    Extension of preseptal cellulitis through the orbital septum.
   3    Local spread from adjacent dacryocystitis, mid-facial or dental infection.
   4    Haematogenous spread.
   5    Post-traumatic develops within 72 hours of an injury that penetrates the orbital
septum.
   6    Post-surgical may complicate retinal, lacrimal or orbital surgery.
   Presentation is with the rapid onset of severe malaise, fever, pain and visual impairment.
Complications
   1    Ocular complications include exposure keratopathy, raised intraocular pressure,
occlusion of the central retinal artery or vein, endophthalmitis and optic neuropathy.
2    Intracranial complications include meningitis, brain abscess and cavernous sinus
thrombosis
3    Subperiosteal abscess
Antibiotic therapy involves intravenous ceftazidime, pencillin and oral metronidazole .
Vancomycin is a useful alternative in the context of penicillin allergy.
Surgical intervention in which the infected sinus, subperiosteal or intracranial abscess
are drained .
















Idiopathic orbital inflammatory disease
It previously referred to as orbital pseudotumour, is an uncommon disorder
characterized by non-neoplastic, non-infective, space-occupying orbital lesions. The
inflammatory process may involve any or all of the orbital soft tissues, resulting in, for
example, myositis, dacryoadenitis, optic perineuritis or scleritis.

Dermoid cyst
An orbital dermoid cyst is a choristoma (a mass of histologically normal tissue in
an abnormal location) derived from displacement of ectoderm to a subcutaneous
location along embryonic lines of closure. Dermoids are lined by keratinized
stratified squamous epithelium (like skin), have a fibrous wall and contain dermal
appendages such as sweat glands, sebaceous glands and hair follicles. Epidermoid
cysts do not contain such adnexal structures. Dermoids may be (a) superficial or (b)
deep, located anterior or posterior to the orbital septum respectively.

Cavernous haemangioma
It is the most common orbital tumour in adults with a female preponderance
of 70%. Although it may develop anywhere in the orbit, it most frequently occurs
within the muscle cone just behind the globe.
Capillary haemangioma
Capillary haemangioma, a hamartoma, – a disorganized overgrowth of mature tissues
normally present in the involved area – is the most common tumour of the orbit and
periorbital areas in childhood. Girls are affected more commonly than boys.
The tumour is classified according to its location (a) cutaneous, (b) purely preseptal, (c)
preseptal with an extraconal element and (d) combination of preseptal, extraconal and intraconal.
  
Presentation is usually in the first few weeks of life (approximately 30% are present at birth).
The course is characterized by rapid growth 3–6 months after diagnosis , followed by a
slower phase of natural resolution in which 30% of lesions resolve by the age of 3 years and
70% by the age of 7 years.
Indications  of treatment:
•    Amblyopia secondary to induced astigmatism, anisometropia, and occlusion.
   •    Optic nerve compression.
   •    Exposure keratopathy.
   •    A severe cosmetic blemish, necrosis or infection.
1 Beta-blockers. Oral propranolol is now widely used, and seems most effective in the
proliferative stage
   2   Laser
   3   Steroid injection into the lesion which might be complicated by occlusion of central
retinal artery , skin depigmentation and necrosis, bleeding and fat atrophy.
   4    Systemic steroids
   5   Local resection with cutting cautery




















Pleomorphic lacrimal gland adenoma
Pleomorphic adenoma (benign mixed-cell tumour) is the most common
epithelial tumour of the lacrimal gland and is derived from the ducts and
secretory elements including myoepithelial cells.
Presentation is in the 2nd–5th decades with a painless, slowly
progressive proptosis or swelling in the superolateral part of the orbit, usually
of more than a year's duration.
Treatment involves surgical excision
Prognosis is excellent provided excision is complete and without disruption
of the capsule.

Lacrimal gland carcinoma

Lacrimal gland carcinoma is a rare tumour which carries a high morbidity
and mortality. In order of frequency the main histological types are: (a) adenoid
cystic, (b) pleomorphic adenocarcinoma, (c) mucoepidermoid and (d) squamous
cell.  
      Presentation is in the 4th–5th decades with a history shorter than
that of a benign tumour. Pain is a frequent feature of malignancy
Treatment involves excision of the tumour and adjacent tissues.
Radiotherapy and chemotherapy may be utilized in some cases.
Prognosis for life is frequently poor



Rhabdomyosarcoma
It is the most common primary orbital malignancy of childhood. The tumour is
derived from undifferentiated mesenchymal cell s, which have the potential to
differentiate into striated muscle. They do not arise from striated muscle.
Presentation is in the 1st decade (average 7 years) with rapidly progressive
unilateral proptosis which may initially mimic an inflammatory process. Swelling and
injection of overlying skin develop later but the skin is not warm
Treatment involves radiotherapy and chemotherapy
Prognosis for patients with disease confined to the orbit is good. .

Adult metastatic tumours

In order of frequency the most common primary sites are breast, bronchus,
prostate, skin (melanoma), gastrointestinal tract and kidney.

Childhood metastatic tumours

Neuroblastoma
Orbital metastases may be bilateral and typically present with an abrupt onset of
proptosis accompanied by a superior orbital mass and lid ecchymosis
Myeloid sarcoma
Langerhans cell histiocytosis



Surgical procedures

Enucleation (removal of the globe) is indicated in the following

circumstances:
   1    Primary intraocular malignancies .
   2    After severe trauma to avoid sympathetic ophthalmitis .
   3    Blind painful or unsightly eyes .

Evisceration involves removal of the entire contents of the globe leaving

the sclera and extraocular muscles intact.

Exenteration involves removal of the globe and the soft tissues of the
orbit.

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 Lacrimal Drainage System
Anatomy
The puncta are located at the posterior edge of the lid
margin, at the junction of the lash-bearing lateral five-sixths (pars
ciliaris) and the medial non-ciliated one-sixth (pars lacrimalis).
Normally they face slightly posteriorly and can be inspected by
everting the medial aspect of the lids.
Physiology
Tears secreted by the main and accessory lacrimal glands pass
across the ocular surface. A variable amount of the aqueous
component of the tear film is lost by evaporation.
a    Tears flow along the upper and lower marginal strips (A) and
enter the upper and lower canaliculi by capillarity and also
possibly by suction.
b    With each blink, the pretarsal orbicularis oculi compresses
the ampullae, shortens and compresses the horizontal canaliculi
and moves the puncta medially. Simultaneously, the lacrimal part
of the orbicularis oculi, which is attached to the fascia of the
lacrimal sac, contracts and compresses the sac, thereby creating a
positive pressure which forces the tears down the nasolacrimal
duct and into the nose (B and C).
   c    When the eyes open the muscles relax, the canaliculi and
sac expand creating a negative pressure which, assisted by
capillarity, draws the tears from the eye into the empty sac




Causes of a watering eye
Epiphora is defined as the sign of overflow of tears, and may be caused
by the following:   
1    Hypersecretion secondary to ocular inflammation or surface
disease. In these cases watering is associated with symptoms of the
underlying cause and treatment is usually medical.
   2    Defective drainage due to compromise of the lacrimal drainage
system. This tends to be exacerbated by a cold and windy atmosphere,
and is least evident in a warm dry room. It may be caused by:   
a    Malposition of the lacrimal puncta (e.g. secondary to ectropion).
   b    Obstruction along the lacrimal drainage system, from the puncta
to the nasolacrimal duct.
   c    Lacrimal pump failure, which may occur secondarily to lower
lid laxity or weakness of the orbicularis muscle (e.g. facial nerve palsy).

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Congenital nasolacrimal duct obstruction
Duct obstruction is perhaps better termed delayed canalization since it often
resolves spontaneously. The lower end of the nasolacrimal duct (at the valve of Hasner)
is the last portion of the lacrimal drainage system to canalize, complete patency usually
occurring soon after birth. Epiphora affects approximately 20% of neonates, but
spontaneous resolution occurs in 96% of cases within the first 12 months.  
      Signs   
•    Epiphora and matting of lashes may be constant or intermittent.
   •    Gentle pressure over the lacrimal sac causes reflux of purulent material from the
puncta.
   •    Acute dacryocystitis is uncommon
Treatment   
a    Massage of the lacrimal sac increases the hydrostatic pressure and may rupture the
membranous obstruction.
b Probing of the lacrimal system should be delayed until the age of 12–18 months
because spontaneous canalization is likely
Results are usually excellent and 90% of children are cured by the first probing and
more than half of the remainder by the second. If symptoms persist , temporary
intubation with fine silastic tubes with or without balloon dilatation of the nasolacrimal
duct may affect a cure. Patients who fail to respond to such measures can be
treated later with DCR, provided the obstruction is distal to the lacrimal sac.






Dacryocystitis
Infection of the lacrimal sac is usually secondary to obstruction of the
nasolacrimal duct. It may be acute or chronic and is most commonly
staphylococcal or streptococcal.
Acute dacryocystitis
   1    Presentation is with the subacute onset of pain in the medial canthal
area, associated with epiphora.
   2    Signs
   •    Very tender tense red swelling at the medial canthus that may be
associated with preseptal cellulitis .
   •    Abscess formation may occur.
   3    Treatment   
a    Initial treatment involves the application of local warm compresses
and oral antibiotics such as flucloxacillin or co-amoxiclav; irrigation and probing
should not be performed.
   b    Incision and drainage may be considered if pus points and an
abscess is about to drain spontaneously
   c  Dacryocystorhinostomy  (DCR: In principle the operation involves
anastomosing the lacrimal sac to the nasal mucosa of the middle nasal meatus).
It is usually necessary after the acute infection has been controlled. Surgery
should not be delayed in the presence of persistent epiphora because of the
risk of recurrent infection.
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