Ординатура / Офтальмология / Английские материалы / Surgical Atlas of Orbital Diseases_Mallajosyula_2009
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22 Surgical Atlas of Orbital Diseases
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Clinical Approach |
to Proptosis |
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C H A P T E R |
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Subrahmanyam Mallajosyula |
Before we discuss about proptosis let me remind you that we have to distinguish it from certain conditions which resemble proptosis (pseudo-proptosis) like unilateral high myopia, buphthalmos, unilateral lid retraction, unilateral minimal ptosis of contralateral eye.
Though advances in imaging techniques have revolutionized the diagnosis of orbital diseases, proper clinical evaluation of proptosis is still very important because it gives us the insight into the disease process and helps in evaluating the CT/MRI and arriving at a correct diagnosis. I follow the
9 “P”s, an extension of the 6 “P”s of Krohel’s. The 9 Ps are: Pain, Progression (from history), Proptosis, Pulsations, Pupil, PBCT, Perception of color vision, Periorbital changes (inspection) and Palpation.
Pain: When severe pain is the presenting symptom in proptosis, we have to consider the following conditions: infection and inflammatory lesions like orbital cellulitis, orbital abscess (Figure 2.1), myocysticercosis, vascular conditions like lymphangioma (Figure 2.2), high flow carotidcavernous fistula (Figure 2.3). Metastatic lesions (Figure 2.4) are also very painful.
Figure 2.1: Orbital abscess |
Figure 2.2: Lymphangioma |
Figure 2.3: Carotid-cavernous fistula |
Figure 2.4: Metastasis from thyroid carcinoma |
24 Surgical Atlas of Orbital Diseases
Retinoblastoma (Figure 2.5), and rhabdomyosarcoma (Figure 2.6) can be very painful and mimic orbital cellulitis. Thyroid orbitopathy which is usually chronic, can rarely present acutely and can be very painful. (Figures 2.7 and 2.7A) Moderate pain is a feature of idiopathic orbital inflammatory syndrome, myocysticercosis, (Figures 2.8 and 2.8A) ruptured dermoid cyst, while dull boring pain is associated with bone-erosion usually due to neoplastic tumors (Figures 2.9 and 2.9A). Pain can be a feature of proptosis following trauma (Figures 2.10 and 2.10A)
Proptosis following trauma can be immediate (due to retrobulbar hemorrhage or surgical emphysema) or delayed due to carotid cavernous fistula. I came across a single case of pulsatile proptosis following trauma, due to herniation of brain through fractured roof of orbit.
Figure 2.5: Retinoblastoma |
Figure 2.6: Rhabdomyosarcoma |
Figure 2.7: Acute thyroid orbitopathy with chemosis and exposure keratopathy. Notice the lid retraction of left eye
Figure 2.7A: CT showing enlarged recti with sparing of tendons
Figure 2.8: Myocysticercosis presenting as ptosis and proptosis with pain. Note the periocular inflammatory response
Figure 2.8A: CT scan of the orbit showing cystic lesion involving
SR-LPS complex with hyper dense spot in the cyst (Scolex)
Clinical Approach to Proptosis 25
Figure 2.9: Eccentric proptosis with globe pushed down. Note fullness of lacrimal gland region
Figure 2.10: Acute proptosis following Trauma. Note severe chemosis, exposure keratitis with hypopyon
Figure 2.9A: CT scan orbit showing a mass in the fossa of lacrimal gland. Note the bony erosion and heterogenisity of the mass (Adenoidcystic carcinoma of lacrimal gland)
Figure 2.10A: After anterior orbitotomy shows complete recovery. Vision improved to 20/30
Progression: The onset of proptosis can be acute (hours to week), subacute (1 to 4 weeks) or chronic (more than 1 month). Acute proptosis can be due to infections, inflammations, parasitic infestations, trauma, metastatic lesions or lymphangioma. Subacute presentation is common in inflammations, parasitic infestations or metastatic neoplasia.
(Figure 2.11) Chronic presentation is commonly due to thyroid associated orbitopathy (Figure 2.12), orbital varices or benign neoplasia like cavernous hemangioma, (Figures 2.13 to 2.13B), Neurofibroma (Figures 2.14 to 2.14E), Schwannoma (Figures 2.15 to 2.15B), Glioma of Optic Nerve, (Figures 2.16 to 2.16C). Chronic presentation is characteristic of most of the primary neoplasia of the orbit, both benign and malignant. However, if the presentation is less than 6 months, consider the possibility of a malignant lesion.
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Figure 2.12: Thyroid associated orbitopathy with chronic presenta- |
Figure 2.11: Metastatic orbital lesion presenting subacutely. |
tion. Note the lid retraction and lateral flare of right upper lid. Note the |
Note the inflammatory changes |
absence of congestion and edema |
26 Surgical Atlas of Orbital Diseases
Figure 2.13: Axial proptosis of left eye 3 yrs. Notice |
Figure 2.13A: CT scan showing well encapsulated tumor |
how quiet was the globe |
(Cavernous hemangioma) |
Figure 2.14: M 52, RE proptosis since 3 yrs,
Def. Vision 1yr. RAPD +, VA : 20/200
Figure 2.13B: Excised tumor
Figure 2.14A: CT shows a large well defined mass with bony expansion
Figure 2.14B: Extension of tumor into superior peripheral space pushing the globe down
Clinical Approach to Proptosis 27
Figure 2.15: F 30, presented with proptosis of 3 yrs.
Defective vision(20/800) RAPD +ve
Figure 2.14C: Well encapsulated tumor on gross exam
Figure 2.15A: CT scan showing a well encapsulated intraconal mass. Note the excavation of lateral wall
Figure 2.14D: Spindle-cells of neurofibroma
Figure 2.14E: Postoperative status recovery from proptosis. VA |
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improved to 20/40 |
Figure 2.15B: Excised tumor, proved to be schwannoma |
Intermittent proptosis is usually due to idiopathic orbital inflammatory syndrome, lymphangioma
(Figure 2.17), orbital varices and myocysticercosis (in endemic areas). (Figure 2.18)
28 Surgical Atlas of Orbital Diseases
Figure 2.16: M 14 yrs, proptosis of right eye since 8 years.
RAPD + Vision absent PL
Figure 2.16B: Excised tumor
Figure 2.16A: CT scan orbit showing optic nerve glioma with cystic degeneration
Figure 2.16C: Postoperative status. No proptosis
Figure 2.17: Girl of 12 years presenting with recurrent episodes of proptosis (3 in 5 yrs). Note the subconjunctival hemorrhage in the proptosed LE (Lymphangioma)
Proptosis: Proptosis or protrusion of the eye ball depends on the location of the orbital lesion. A lesion in the intraconal space pushes the globe forwards to cause “axial proptosis” (Figures 2.19 to 2.19B), where as a lesion in the peripheral surgical space pushes the globe to the opposite side and causes “eccentric
Figure 2.18: CT scan showing an enlarged superior rectus muscle with a cyst showing hyper-dense spot within (Myocysticercosis)
proptosis.” However, since these surgical compartments are not strictly water-tight, a large intraconal lesion can enter peripheral surgical space and cause eccentricity to an otherwise axial proptosis (Figure 2.14 series).
Axial proptosis: Lesion is in the intraconal space. In my experience the most common lesion of intraconal space causing axial proptosis is cavernous hemangioma followed by Schwannoma, and neuro-fibroma. Optic nerve glioma and meningioma of optic nerve sheath are important but not very common lesions. Other lesions include orbital varix, lymphangioma. The most common intraconal cystic lesion is hydatid cyst (Figures 2.20 and 2.20A).
One has to remember that most of the intraconal lesions can compress optic nerve and lead to visual loss. Hence loss of vision in axial proptosis does not necessarily mean that the patient had either optic nerve glioma or meningioma of optic nerve sheath. It can be due to any other lesion of intraconal space like hemangioma, schwannoma, neurofibroma, hydatid cyst or even idiopathic orbital inflammatory syndrome. Optic nerve can also be compressed by enlarged extraocular muscles as in thyroid associated orbitopathy.
Clinical Approach to Proptosis 29
Down and out proptosis is due to lesions of superomedial space (pushing the globe down and out). Frontoethmoidal lesions are the most common cause of such eccentric proptosis. Mucocele (Figures 2.21A to C), fungal granuloma, neoplastic lesions and fibrous dysplasia (Figures 2.22A and B) are the common lesions. Lesions of supero-medial space like dermoids, hemangiomas can also present with eccentric proptosis with globe displaced down and out (Figures 2.23A and B). Osteoma of ethmoid (Figures 2.24A to E) is another rare cause.
Figure 2.19: Axial proptosis of left eye due to intraconal lesion
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B |
Figures 2.19A and B: CT scans of orbit showing intraconal tumor arising from optic nerve (Optic nerve glioma)
Figure 2.20: Intraconal hydatid cyst of orbit |
Figure 2.20A: Cyst excised with cryo after aspirating fluid |
30 Surgical Atlas of Orbital Diseases
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B |
C
Figures 2.21A to C: Eccentric proptosis with the eyeball pushed down and out. Notice the gross outward displacement with fullness in the superomedial aspect. CT scan shows a huge fronto-ethmoid mucocele
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Figures 2.22A and B: Eccentric proptosis with globe pushed down |
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and out due to fibrous dysplasia of frontal bone as demonstrated by |
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the CT scan (B) |
Clinical Approach to Proptosis 31
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Figures 2.23A and B: Coronal and axial sections of CT scan orbit showing a very large cystic lesion in the superomedial peripheral space
(blue arrow), displacing the Globe (green pentagon) down and out. Compare the size of the cyst with that of opposite eyeball. Note the bony excavation of the roof and medial wall (red arrow). This is a case of Hydatid cyst of orbit
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Figures 2.24A to C: This young male of 22 yrs presented with recurrent, eccentric proptosis of right eye since 1year, and defective vision since 3 months. He underwent surgery elsewhere for similar lesion 2 years back. Note the periocular fullness, and lateral displacement of the globe. He had RAPD and the vision was 20/40. CT scan of orbits revealed a huge osteoma involving the ethmoid bone (arrow)
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Figures 2.24D and E: Excised ivory osteoma. The postoperative |
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D |
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recovery was uneventful. The patient's vision improved to 20/20 |
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