Proptosis by Dr.Ashwin Menon

Facts in Nutshell regarding proptosis from otorhinolaryngology point of view.

1. DEPT. OF ENT,ACME PROPTOSIS BY, DR.ASHWIN MENON

2. Forward displacement or bulging of the eye . The word Proptosis is derived from Latin: propiptein pro- forward + piptein - to fall DEPT. OF ENT,ACME DEFENITION

3. If protrusion of globe is 18 mm / less it is known as proptosis . If protrusion of globe is more than 18 mm it is known as exophthalmos. Proptosis + lid lag = exopthalmos . DEPT. OF ENT,ACME

4. This is caused due to decrease in the volume of orbit causing the orbital contents to protrude anteriorly. Usually bilateral. Should be differentiated from proptosis / exophthalmos. DEPT. OF ENT,ACME EXORBITISM

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6. Volume of orbit is fixed - 30 ml. Increase in soft tissue volume of 5 ml will cause 5 mm of proptosis . DEPT. OF ENT,ACME ANATOMY OF ORBIT

7. Resembles a four sided pyramid Rim is 40 mm horizontally and 35 mm vertical in an adult male Medial walls are parallel and 25 mm apart in adults Lateral orbital walls angle about 90 degrees from each other DEPT. OF ENT,ACME ANATOMY OF ORBIT

8. Superior orbital rim - Frontal bone. Inferior rim - Maxillary bone (medially) Zygomatic bone (laterally) Lateral orbital rim – Zygoma . Superior rim contains a notch at the junction of medial and lateral thirds (supraorbital notch ). Medial portion of the rim is formed by frontal process of maxilla. DEPT. OF ENT,ACME ORBITAL RIM

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10. Lodges the lacrimal sac This fossa is formed by maxillary and lacrimal bones Bounded by anterior and posterior lacrimal crests Anterior crest is formed by maxillary bone Posterior lacrimal crest is formed by lacrimal bone DEPT. OF ENT,ACME LACRIMAL FOSSA

11. Lies anterior to lacrimal fossa. Also known as sutura longitudinalis imperfecta . This suture runs parallel to anterior lacrimal crest. Infraorbital nerve & artery pass through it to supply nasal mucosa. Bleeding occurs from these vessels during lacrimal sac surgeries. DEPT. OF ENT,ACME WEBER'S SUTURE

12. 7 bones involved in the formation of orbit are derived from neural crest cells. Ossification of orbit is complete at birth except its apex. Lesser wing of sphenoid is cartilaginous. Other bones undergo membranous ossification. DEPT. OF ENT,ACME

13. Formed by frontal bone. Posterior 1.5cms of the roof is formed by lesser wing of sphenoid. Optic foramen contains optic nerve. Optic nerve enters orbit at an angulation of 45 degrees. Lacrimal gland is located at the lateral end of orbital roof. The trochlear fossa is located in the antero medial portion of the orbital roof. DEPT. OF ENT,ACME ORBITAL ROOF

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15. The medial wall of the orbit is formed from anterior to posterior by: F rontal process of maxilla Lacrimal bone Ethmoid bone Lesser wing of sphenoid bone DEPT. OF ENT,ACME MEDIAL ORBITAL WALL

16. The thinnest portion of the medial wall is the lamina papyracea which separates the ethmoidal sinuses from the orbit . The medial wall of the orbit is thicker posterior where the sphenoid bone is present and anteriorly where the posterior lacrimal crest is present. DEPT. OF ENT,ACME MEDIAL ORBITAL WALL

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18. Infections from ethmoidal sinus can easily breach lamina papyracea and affect the orbital contents . Lacrimal bone at the level of lacrimal fossa is very thin. This bone can easily be penetrated during endoscopic DCR. If the maxillary component is predominant then it is really difficult to breach this bone during endoscopic DCR since this bone is rather thick. DEPT. OF ENT,ACME Medial wall of orbit applied anatomy

19. Very important surgical landmark. Marks the approximate level of ethmoidal roof. Dissection above this line will expose the cranial cavity. Anterior and posterior ethmoidal foramina are present in this suture line. Anterior and posterior ethmodial arteries pass throught these foramina. DEPT. OF ENT,ACME FRONTO ETHMOIDAL SUTURE LINE

20. It is the shortest of all the walls. Bounded laterally by infra orbital fissure. Medially bounded by maxilloethmoidal strut of bone. Almost entirely formed by orbital plate of maxilla with minor contribution from orbital plate of palatine bone posteriorly. Floor is thin medial to infra orbital groove. Infraorbital groove becomes infraorbital foramen anteriorly. DEPT. OF ENT,ACME FLOOR OF ORBIT

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22. Formed by greater wing of sphenoid. Zygoma & zygomatic process of frontal bone – minor contribution. Recurrent meningeal branch of middle meningeal artery is seen in this wall. 4-5 mm behind the lateral orbital rim and 1 cm inferior to the fronto zygomatic suture line lie the whitnall's tubercle. DEPT. OF ENT,ACME LATERAL WALL OF ORBIT

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24. Lateral canthal tendon Lateral rectus check ligament Suspensory ligament of lower eyelid (Lockwood's ligament) Orbital septum Lacrimal gland fascia DEPT. OF ENT,ACME WHITNALL'S TUBERCLE (STRUCTURES ATTACHED)

25. Orbit is related to paranasal sinuses in two ways: 1. Anatomically by its location. 2. Venous drainage (They both share the same venous drainage). DEPT. OF ENT,ACME RELATIONSHIP WITH PARANASAL SINUSES

26. The superior wall of the orbit is shared by the floor of the frontal sinus, the floor of the orbit is shared by the roof of the maxillary sinus, the medial wall of the orbit is shared by the lateral wall of ethmoidal sinus. These shared bones are really thin enabling infections to travel from either direction. DEPT. OF ENT,ACME

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28. Entire venous system is devoid of valves – hence two way communication between orbit and sinuses is a reality. Superior ophthalmic vein connects facial vein to cavernous sinus – causing spread of infections from face to cavernous sinus. Inferior ophthalmic vein communicates with pterygoid venous plexus and cavernous sinus by its two branches. DEPT. OF ENT,ACME PECULIARITIES OF ORBITAL VENOUS DRAINAGE

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30. High myopia. Enophthalmos of one eye may cause apparent proptosis of the other one. DEPT. OF ENT,ACME PSEUDOPROPTOSIS

31. Proptosis can be divided into following clinical groups : 1. Unilateral proptosis. 2 . Bilateral proptosis. 3. Acute proptosis. 4. Intermittent proptosis. 5. Pulsatile proptosis. DEPT. OF ENT,ACME CLASSIFICATION

32. Causes of unilateral proptosis include - Congenital conditions. Dermoid cyst, congenital cystic eyeball and orbital teratoma . Traumatic lesions Inflammatory lesions Circulatory disturbances and vascular lesions Cysts of orbit Tumors of the orbit Mucoceles of paranasal sinuses DEPT. OF ENT,ACME ETIOLOGY

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34. Causes of bilateral proptosis include- Developmental anomalies of the skull: craniofacial dysostosis e.g., oxycephaly (tower skull). Osteopathies . Inflammatory conditions: Mikulicz’s syndrome L ate stage of cavernous sinus thrombosis. Endocrinal exophthalmos ( eg ; thyrotoxicosis ) DEPT. OF ENT,ACME

35. DEPT. OF ENT,ACME OXYCEPHALY EXOPHTHALMOS

36. DEPT. OF ENT,ACME MIKULICZ’S SYNDROME

37. DEPT. OF ENT,ACME CAVERNOUS SINUS THROMBOSIS

38. 5. Tumours : These include symmetrical lymphoma or lymphosarcoma . 6. Systemic diseases: Histiocytosis S ystemic amyloidosis Xanthomatosis Wegener’s granulomatosis DEPT. OF ENT,ACME

39. DEPT. OF ENT,ACME Lymphoma – Bilateral involvement Wegener’s granulomatosis

40. . It develops with extreme rapidity (sudden onset). Its common causes are : O rbital emphysema F racture of the medial orbital wall O rbital hemorrhage R upture of ethmoidal mucocele . DEPT. OF ENT,ACME Causes of acute proptosis-

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42. This type of proptosis appears and disappears of its own. Its common causes are: O rbital varix P eriodic orbital oedema R ecurrent orbital haemorrhage H ighly vascular tumors. DEPT. OF ENT,ACME Causes of intermittent proptosis :

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45. It is caused by pulsating vascular lesions such as Carotico cavernous fistula. S accular aneurysm of ophthalmic artery. Pulsating proptosis also occurs due to transmitted cerebral pulsations in conditions associated with deficient orbital roof. These include congenital meningocele or meningoencephalocele , neurofibromatosis and traumatic or operative hiatus. DEPT. OF ENT,ACME Causes of pulsating proptosis :

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49. Mnemonic – VEIN V – Vascular causes E – Endocrine causes I – Inflammatory causes N – Neoplastic causes DEPT. OF ENT,ACME ENT - Causes

50. Classified into arterial and venous. Venous causes are due to dilated veins – Positional proptosis is the classical feature in these patients. It can also be induced by valsalva maneuver . Initially there may be atrophy of fat in these pts causing enophthalmos . CT scan after jugular vein compression is diagnostic. Surgery is disastrous in these patients. Conservative management is the best modality. DEPT. OF ENT,ACME VASCULAR CAUSES

51. Graves’ disease, the most common cause of bilateral proptosis should be ruled out. These patients show clear clinical evidence of thyrotoxicosis like: 1. Bilateral proptosis 2. Lid retraction 3. Lid lag 4. Descent of eyelid cheek complex 5. Gritty sensation in the eye 6. Retrobular pain 7. Tearing 8. Palpitation 9. Sleeplessness 10. Diarrhea 11. Menstrual disturbances (in case of females) DEPT. OF ENT,ACME ENDOCRINE CAUSES

52. Idiopathic inflammation – Pseudo tumor of orbit Due to specific causes of orbital inflammation These pts have pain during ocular movement Associated dacryoadenitis Perioptic neuritis can cause blindness Steroids may be helpful. DEPT. OF ENT,ACME INFLAMMATORY CAUSES

53. Inverted papilloma Fungal infections Mucoceles of paranasal sinuses Fibrous dysplasia of maxilla Osteomas involving frontal / ethmoidal sinuses JNA DEPT. OF ENT,ACME NEOPLASTIC LESIONS INVOLVING NOSE AND SINUSES

54. I. Clinical evaluation (A) History. It should include: age of onset, nature of onset, duration, progression, chronology of orbital signs and symptoms. ( B) Local examination. It should be carried out as follows: DEPT. OF ENT,ACME INVESTIGATION OF A CASE OF PROPTOSIS

55. 1. Inspection . ( i) To differentiate proptosis from pseudoproptosis which is seen in patients with buphthalmos , axial high myopia , retraction of upper lid and enophthalmos of the opposite eye. ( ii) to ascertain whether the proptosis is unilateral or bilateral . (iii ) to note the shape of the skull. (iv ) to observe whether proptosis is axial or eccentric . DEPT. OF ENT,ACME

56. 2. Palpation . It should be carried out for retrodisplacement of globe to know compressibility of the tumour, for orbital thrill , for any swelling around the eyeball, regional lymph nodes and orbital rim. 3 . Auscultation . It is primarily of value in searching for abnormal vascular communications that generate a bruit, such as carotico cavernous fistula . DEPT. OF ENT,ACME

57. 4. Transillumination. It is helpful in evaluating anterior orbital lesions. 5 . Visual acuity. 6 . Pupil reactions . 7. Fundoscopy . DEPT. OF ENT,ACME

58. 8. Ocular motility. It is restricted in thyroid ophthalmopathy, extensive tumour growths and neurological deficit . DEPT. OF ENT,ACME

59. The simplest instrument to measure proptosis is Luedde’s exophthalmometer . the Hertel’s exophthalmometer ( is the most commonly used instrument. The distance between the lateral orbital rim and the corneal apex is used as a measure for proptosis. Normal values vary between 10 and 21 mm and are symmetrical in both eyes. DEPT. OF ENT,ACME EXOPHTHALMOMETERY

60. A difference of more than 2 mm between the two eyes is considered significant . Its advantage is that it measures the two eyes simultaneously. DEPT. OF ENT,ACME

61. C) Systemic examination . A thorough examination should be conducted to rule out systemic causes of proptosis such as thyrotoxicosis , histiocytosis and primary tumors elsewhere in the body (secondaries in orbits ). Otorhinolaryngological examination is necessary when the paranasal sinus or a nasopharyngeal mass appears to be a possible etiological factor.

62. II. Laboratory investigations These should include: Thyroid function tests Haematological studies (TLC, DLC, ESR,VDRL test) Casoni’s test (skin test to rule out hydatid cyst),. Stool examination for cysts and ova. Urine analysis for Bence Jones proteins for multiple myeloma.

63. III. Imaging Technique (A) Non-invasive techniques 1. Plain X-rays. 2 . Computed tomography scanning 3 . Ultrasonography 4 . Magnetic resonance imaging (MRI).

64. CT / MRI may help in identifying the cause Fat in the orbit serves as a contrast medium 3 mm cuts is ideal Ultrasound – A mode / B mode can be done to identify the cause Otolaryngology online IMAGING

65. Imaging helps to identify: Presence of sinusitis. Septal deviation. Hypoplastic maxillary sinuses . Presence of inflammatory / mass lesions in the nose and paranasal sinuses . DEPT. OF ENT,ACME

66. MRI is sensitive in identifying extra ocular muscle oedema. Increased T2 relaxation time indicates extra ocular muscle oedema, these patients respond well to steroid therapy. Patients with normal T2 relaxation levels need orbital decompression. Otolaryngology online ROLE OF MRI

67. (B) Invasive procedures 1 . Orbital venography 2 . Carotid angiography. 3 . Radioisotope studies .

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70. High flow shunts Can occur spontaneously / trauma Subjective bruit / proptosis / chemosis / vision loss Arterolization of conjunctival vessels causing corkscrew pattern Intractable cases – shunt must be closed using balloon / carotid artery ligation DEPT. OF ENT,ACME CAROTID CAVERNOUS FISTULA

71. IV. Histopathological studies The exact diagnosis of many orbital lesions cannot be made without the help of histopathological studies,which can be accomplished by following techniques. 1 . Fine-needle aspiration biopsy (FNAB). 2. Incisional biopsy. 3. Excisional biopsy.

72. Low dose irradiation (rarely used) Surgery DEPT. OF ENT,ACME MANAGEMENT

73. Visual disturbance due to proptosis Failure of steroids to improve vision If steroids are necessary on a long term basis for maintaining vision To prevent exposure keratitis Diplopia Cosmetic DEPT. OF ENT,ACME Indications for orbital decompression

74. Lateral orbitotomy – Dollinger (1911) Superior orbitotomy – Naffziger (1931) Medial orbitotomy – Sewall (1936) Inferior orbitotomy – Hirsch (1950 ) Combined medial and inferior decompression – Walsh – Ogura(1957) Endoscopic Technique – Kennedy(1990) DEPT. OF ENT,ACME

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76. Diplopia Intractable strabismus Hypoglobus Injury to optic nerve due to prolonged globe retraction Retrobular hematoma – this can cause blindness Injury to infraorbital nerve Epistaxis DEPT. OF ENT,ACME Risks of orbital decompression

77. To enlarge the confining space of orbit by removing 1-4 of its walls 15 mm of decompression can be achieved by removing all 4 walls of the orbit Usually successful surgery causes 3-7 mm decompression of orbit DEPT. OF ENT,ACME Orbital decompression (Goals)

78. Naffzeiger technique (1931) Superior wall decompression Complete un roofing of orbit – frontal craniotomy Large amounts of bone can be removed creating more space Craniotomy may be needed Used in pts with orbital trauma DEPT. OF ENT,ACME Superior orbital decompression

79. In collaboration with neurosurgeon Optic nerve should be visualized to begin with The roof of the orbit is removed starting from the optic foramen to the anterosuperior orbital rim Periosteum should be left intact to prevent injury to levator muscle H shaped incision is made over superior periosteum allowing orbital fat to prolapse through it Titanium mesh can be used to cover orbital roof DEPT. OF ENT,ACME NAFFZEIGER (Contd.)

80. Also known as Sewell procedure (1936) Coronal incision / external ethmoidectomy incision Medial canthal tendon is identified and divided Anterior and posterior ethmoidal arteries identified and clipped Complete ethmoidectomy is performed starting from lacrimal fossa DEPT. OF ENT,ACME Medial orbital decompression

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83. Medial canthal tendon can be left intact Ethmoidectomy is performed from above Lacrimal sac and trochlea should not be damaged Medial periosteum is incised and orbital fat is allowed to prolapse into the nasal cavity DEPT. OF ENT,ACME Bicoronal incision for medial orbital decompression

84. Hisch and Urbanek procedure (1950) Artificial creation of blow out fracture of orbital floor sparing infra orbital nerve Trans conjunctival / subciliary incision plus Caldwell Luc procedure Laterally floor can be removed up to zygoma and medially up to lacrimal fossa Posteriorly bone is thick – 3 cms of bone can be removed from this area DEPT. OF ENT,ACME Inferior decompression

85. Periosteum is incised to allow orbital fat to prolapse into the maxillary antrum Forced duction test should be performed to ensure orbital muscles are not entrapped . The forced duction test is performed in order to determine whether the absence of movement of the eye is due to a neurological disorder or a mechanical restriction . The anesthetized conjunctiva is grasped with forceps and an attempt is made to move the eye ball in the direction where the movement is restricted. If a mechanical restriction is present, it will not be possible to induce a passive movement of the eye ball. DEPT. OF ENT,ACME Inferior decompression ( Contd )

86. Dollinger (1911) - removal of lateral orbital wall for decompression into the temporal fossa ( Kroenlein’stechnique ). Coronal incision, and lateral extension of subciliary incision Extended lateral canthotomy Lateral orbital rim periosteum is exposed from zygomatic arch to zygomatico frontal suture Periosteum incised along lateral orbital rim and orbital fat is teased out DEPT. OF ENT,ACME Lateral decompression

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92. Any of the above said approaches can be combined for optimal benefit Combination of approaches reduces the surgical risk and provides more increase of space than one procedure alone Combined medial and inferior decompression ( Walsh – Ogura ): This approach like inferior one involves Caldwell – Luc / Transantral approach. This technique was the most preferred one during 1990’s. This procedure managed to achieve 5mm proptosis reduction. DEPT. OF ENT,ACME Combination of approaches

93. DEPT. OF ENT,ACME TRANSNASAL ENDOSCOPIC DECOMPRESSION

94. Kennedy (1990) N ose decongested and injected with 1% lidocaine with 1:100,000 epinephrine. middle turbinectomy performed Uncinectomy performed maxillary ostium enlarged anteriorly ethmoid bulla opened anterior and posterior ethmoidectomies performed Sphenoidotomy performed lamina papyracea identified and cleaned of mucosa DEPT. OF ENT,ACME TRANSNASAL ENDOSCOPIC DECOMPRESSION

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96. lamina papyracea penetrated and fragments elevated superiorly to ethmoid roof posteriorly to face of sphenoid sinus inferiorly to orbital floor anteriorly to frontal process of maxilla orbital floor removed laterally to infraorbital canal Periorbita incised with sickle knife any existing bands of periorbita that might be hidden between lobules of fat are taken down DEPT. OF ENT,ACME TRANSNASAL ENDOSCOPIC DECOMPRESSION

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99. Nose blowing is to be avoided for 2 weeks following surgery Bilateral decompression should be done within an interval of a week For mild exophthalmos 2-3 mm any of the approaches would suffice For moderate – 3-5mm inferior decompression is sufficient For severe ones – 5-7 mm three wall decompression is preferred DEPT. OF ENT,ACME TIPS

100. DEPT. OF ENT,ACME THANK YOU…