CLINICAL SIGNS IN ORBITAL DISEASE
1.
Bony
- •
Hypertelorism.
- •
Dystopia in which the orbits are not level.
- •
Shallow orbits.
2.
Soft tissue
- •
Lid oedema.
- •
Ptosis.
- •
Chemosis.
3.
Globe malposition
- •
Proptosis.
- •
Enophthalmos.
- •
Displacement.
4.
Ophthalmoplegia
- •
By an orbital mass.
- •
Restrictive myopathy.
- •
Ocular motor nerve palsy.
- •
Muscle entrapment in a fracture.
5.
Posterior segment
- •
Disc oedema.
- •
Optic atrophy.
- •
Chorioretinal folds.
- •
Retinal vascular congestion.
BONY ABNORMALITIES
Hypertelorism
Signs
- •
Wide separation of the orbits ( Fig. 2.1 ).
Fig. 2.1
Causes
- •
Facial trauma.
- •
Associated with congenital systemic abnormalities (see below).
Differential diagnosis
- •
Telecanthus in which there is increased distance between the medial canthi as a result of abnormally long medial canthal tendons.
Look for systemic
- •
Craniosynostosis (see below).
- •
Frontonasal dysplasia.
- •
Basal encephalocele with wide nasal bridge.
- •
Syndromic conditions – Hurler, Noonan, Roberts, Gorlin–Goltz and Meckel–Gruber.
Dystopia
Signs
- •
Orbits are not level.
Causes
- •
See Table 2.1 .
Table 2.1
Causes of orbital dystopia
- 1.
Congenital
- •
Idiopathic
- •
Goldenhar syndrome
- •
Hemifacial microsomia
- •
Coronal craniosynostosis
- •
Facial clefting syndromes ( Fig. 2.2 )
- •
- 2.
Acquired
- •
Fractures of orbital floor or rim
- •
Fibrous dysplasia
- •
Meningioma en plaque
- •
Fig. 2.2
- 1.
Shallow orbits associated with craniosynostoses
Oxycephaly
Signs
- •
High, narrow, pointed skull, high forehead and superior prognathism ( Fig. 2.3 ).
Fig. 2.3
Crouzon syndrome
Signs
- •
Midfacial hypoplasia, orbital axes are rotated laterally (exorbitism), flat nasal bridge, parrot’s beak nose, prominent lower jaw and V pattern exotropia ( Fig. 2.4 ).
Fig. 2.4
Apert syndrome
Signs
- •
Similar to Crouzon but with less severe proptosis and hypertelorism ( Fig. 2.5 ).
Fig. 2.5
Look for
- •
Broad thumbs and great toes, and syndactyly.
Pfeiffer syndrome
Signs
- •
Acrocephaly (peaked skull), midfacial hypoplasia, orbital rotation and down-sloping palpebral fissures ( Fig. 2.6 ).
Fig. 2.6
Other causes
- a.
Chromosomal abnormalities
- •
Trisomy 13 (Patau syndrome).
- •
Trisomy 18 (Edward syndrome).
- •
- b.
Other bony defects
- •
Osteogenesis imperfecta.
- •
Osteopetrosis.
- •
Hyperphosphataemia.
- •
ENOPHTHALMOS
In enophthalmos the globe is recessed within the orbit. It may be caused by the following conditions.
Small globe
- a.
Phthisis bulbi ( Fig. 2.7 ).
Fig. 2.7
- b.
Microphthalmos in which the net volume of the globe is reduced; it may be simple ( Fig. 2.8 ) or colobomatous.
Fig. 2.8
- c.
Nanophthalmos which is characterised by a small eye, high hypermetropia, weak but thick sclera, predisposition to angle closure glaucoma and uveal effusion.
Structural bony abnormalities
- a.
After blow-out fracture of orbital floor ( Fig. 2.9 ).
Fig. 2.9
- b.
Congenital bony defects as in NF1.
Atrophy of orbital contents
- a.
Following radiotherapy .
- b.
Parry–Romberg hemifacial atrophy (see Fig. 8.95 ).
- c.
Scleroderma .
- d.
Chronic maxillary sinusitis .
- e.
Long-standing orbital varices ( Fig. 2.10 ).
Fig. 2.10
- f.
Eye poking (oculo-digital sign) in blind children ( Fig. 2.11 ).
Fig. 2.11
Cicatrising orbital lesions
- a.
Metastatic scirrhous breast carcinoma .
- b.
Chronic sclerosing inflammatory orbital disease .
Duane retraction syndrome
Although this is usually characterised by retraction of the globe on adduction rarely there may be true enophthalmos in the primary position (see Fig. 14.17A ).
PROPTOSIS
Signs
- •
The globe is pushed forward – best detected by looking down from above ( Fig. 2.12 ).
Fig. 2.12
Direction
- •
Axial proptosis is caused by lesions within the muscle cone such as cavernous haemangioma, optic nerve tumours and thyroid eye disease.
- •
Eccentric proptosis is caused by extraconal lesions in which the direction of proptosis is determined by the site of the lesion.
Causes of pseudo-proptosis
- •
Ipsilateral large globe as in buphthalmos ( Fig. 2.13 ) or very high myopia.
Fig. 2.13
- •
Ipsilateral lid retraction.
- •
Contralateral enophthalmos.
ORBITAL DISEASE IN CHILDREN
Inflammatory
Preseptal cellulitis
Definition
- •
An infection of the subcutaneous tissues anterior to the orbital septum.
Signs
- •
Unilateral, tender and red periorbital oedema ( Fig. 2.14A ).
Fig. 2.14A
- •
Normal ocular motility and visual acuity.
- •
CT shows opacification anterior to the orbital septum ( Fig. 2.14B ).
Fig. 2.14B
Orbital cellulitis
Definition
- •
A life-threatening infection of soft tissues posterior to the orbital septum, which in children, is usually secondary to ethmoiditis.
Signs
- •
Very unwell patient with a high temperature.
- •
Rapid onset of painful proptosis (usually down and out), chemosis, lid oedema and ophthalmoplegia ( Fig. 2.15A ).
Fig. 2.15A
- •
Optic nerve dysfunction in severe cases.
- •
CT shows preseptal and orbital opacification ( Fig. 2.15B ).
Fig. 2.15B
Idiopathic orbital inflammatory syndrome (pseudo-tumour)
Definition
- •
An uncommon, non-neoplastic and non-infectious space-occupying condition that may present in childhood or adult life. Acute orbital myositis is a subtype which primarily affects one or more of the extraocular muscles.
Signs
- •
Subacute onset of proptosis associated with lid oedema and displacement of the globe ( Fig. 2.16A ).
Fig. 2.16A
- •
In acute orbital myositis, pain and diplopia are increased on attempted gaze into the field of the affected muscle.
- •
CT shows a poorly-defined opacification with ill-defined orbital structures ( Fig. 2.16B ).
Fig. 2.16B
Look for systemic disease in bilateral cases
- •
Wegener granulomatosis.
- •
Polyarteritis nodosa.
- •
Sarcoidosis.
- •
Tuberculosis.
- •
Waldenström macroglobulinaemia.
Thyroid eye disease
Thyroid eye disease may rarely present in children as young as 10 years and cause proptosis and lid retraction ( Fig. 2.17 ).
Benign tumours
Capillary haemangioma
Presentation
- •
At birth or early infancy.
Signs
- •
Anterior orbit lesion that may appear dark blue or purple through overlying skin and may be associated with cutaneous lesions ( Fig. 2.18 ).
