Nontraumatic orbital hemorrhage

s u r v e y o f o p h t h a l m o l o g y 5 9 ( 2 0 1 4 ) 1 6 6 e1 8 4

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Major review

Nontraumatic orbital hemorrhage Alan A. McNab, MB, BS, FRANZCO, DMedSc* Director, Orbital Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital, Melbourne, Australia This work was presented in part as the Peter Rogers Lecture at the Annual Scientific Congress of the Royal Australian and New Zealand College of Ophthalmologists, Melbourne, November, 2012.

article info

abstract

Article history:

Nontraumatic orbital hemorrhage (NTOH) is uncommon. I summarize the published

Received 29 April 2013

reports of NTOH and offer a classification based on anatomic and etiologic factors.

Received in revised form

Anatomic patterns of NTOH include diffuse intraorbital hemorrhage, “encysted” hemor-

6 July 2013

rhage (hematic cyst), subperiosteal hemorrhage, hemorrhage in relation to extraocular

Accepted 9 July 2013

muscles, and hemorrhage in relation to orbital floor implants. Etiologic factors include

Available online 18 December 2013

vascular malformations and lesions, increased venous pressure, bleeding disorders, infection and inflammation, and neoplastic and nonneoplastic orbital lesions. The majority

Keywords:

of NTOH patients can be managed conservatively, but some will have visual compromise

orbit

and may require operative intervention. Some will suffer permanent visual loss, but a large

hematoma

majority have a good visual outcome. ª 2014 Elsevier Inc. All rights reserved.

hemorrhage blood breakdown products nontraumatic orbital hemorrhage spontaneous orbital hemorrhage nontraumatic subperiosteal orbital hemorrhage vascular malformation

1.

Introduction

Nontraumatic orbital hemorrhage (NTOH) is uncommon. There are a large number of single case reports and small case series of NTOH, but no systematic review of the subject. I have summarized the previously reported cases of NTOH and offer a classification system based on the anatomic pattern of hemorrhage and the etiologic factors involved.

2.

Terminology

NTOH has been referred to by a number of terms, including spontaneous orbital hemorrhage, idiopathic orbital hemorrhage, orbital hematoma, and orbital hematic cyst. The term nontraumatic orbital hemorrhage seems to be the most logical to cover all types of bleeding in the orbit occurring in the absence of trauma. Many of these patients have underlying reasons for

* Corresponding author: Dr. Alan A. McNab, MB, BS, FRANZCO, DMedSc, Suite 216-218, 100 Victoria Parade, East Melbourne, 3002, Victoria, Australia. E-mail address: [email protected]. 0039-6257/$ e see front matter ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.survophthal.2013.07.002

s u r v e y o f o p h t h a l m o l o g y 5 9 ( 2 0 1 4 ) 1 6 6 e1 8 4

the development of the hemorrhage, such as a vascular malformation, a sudden rise in cranial and orbital venous pressure, or a bleeding diathesis. Therefore, the term spontaneous is not appropriate. The terminology hematic cyst has been used by a number of authors to describe a range of clinical entities. These include an apparently “encysted” hemorrhage (a localized hemorrhage surrounded by a nonepithelialized fibrous capsule) occurring with or without an underlying vascular malformation, nontraumatic subperiosteal orbital hemorrhage (NTSOH) and orbitofrontal cholesterol granuloma. The term hematic cyst should be confined to those intraorbital lesions characterized by a collection of blood and blood breakdown products within a cystlike structure without an epithelial lining and without an underlying vascular malformation. These are quite rare.

3.

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with possible liver disease and a clotting disorder. The anatomic site of the bleeding cannot be determined from the report. In 1912, Gruenig reported a case of “idiopathic hematoma of the orbit” that may have occurred in relation to an underlying vascular malformation, but there is no way of establishing this from the report.76 Kundert described the case of a newborn infant with bilateral exophthalmos apparently the result of hemorrhage that resolved, but the site of the bleeding cannot be established from the description.112 In 1951, Law described spontaneous orbital hemorrhage in 2 fit young men, possibly related to mild exertion, that could have been intraorbital or subperiosteal.117 Both recovered fully. With the availability of CT and MRI, patients with apparent NTOH can be imaged, and the clinical and imaging features of the majority of these patients allows a confident diagnosis of hemorrhage without the need for operative exploration.

Historical aspects 4.

In older textbooks, NTOH is described as largely being the result of hemorrhagic diatheses such as hemophilia or scurvy, or “congestion” with venous engorgement caused by thoracic compression, strangulation, violent coughing, or lifting heavy weights.58 The example of scurvy provides an interesting introduction to the subject of NTOH. Historically, scurvy has been responsible for the deaths of large numbers of sailors on long voyages from the start of the great Age of Discovery up to the early parts of the 19th century. The preventive role of foods with vitamin C or ascorbic acid became known in the late 18th century; however, scurvy still occurred in large armies on campaign and in groups such as polar explorers. Then, in the latter half of the 19th century in developed countries, infantile scurvy became a common condition as affluent parents fed their infants sterilized infant formula or boiled milk devoid of vitamin C.160 Around this time, there are numerous references to exophthalmos in infantile scurvy.51,228 By 1931, when Dunnington reported a case, he could find 22 previously published definite single cases of proptosis in childhood scurvy.59 The American Pediatric Society conducted a large survey of its members’ experience of infantile scurvy and published its report in 1898.74 They described in detail 379 cases. In 110 of these cases, swelling or protrusion of the eyes was noted as absent, but was present in 49. This suggests that up to 31% (49/159) had orbital hemorrhage as a manifestation of their scurvy. Interestingly, orbital hemorrhage is known to be a rare manifestation of scurvy in adults, even though bleeding elsewhere is a common feature. In 1905, Snow reported the post-mortem findings in an infant who died from scurvy with an orbital hemorrhage.185 Dissection of the orbit showed that the bleeding had occurred into the subperiosteal space in the roof of the orbit. As we will see, NTSOH nearly always occurs in the superior orbit. In reports of NTOH that preceded the advent of computed tomography (CT) or magnetic resonance imaging (MRI), it is often difficult to know what if any underlying cause existed in each case and where anatomically the hemorrhage occurred. Friedenwald, for example, described 2 cases in 1894 of “exophthalmus” owing to orbital hemorrhage.62 One was traumatic and the other occurred spontaneously in a patient

Classification

NTOH can be classified on the basis of the anatomic site of the hemorrhage as well as the underlying cause. There are several distinct anatomic patterns of NTOH. In parallel with this, there are a number of underlying clinicopathologic causes of NTOH. Some anatomic variants, such as NTSOH, do not occur in the presence of all types of underlying clinicopathologic causes; similarly, some underlying causes do not lead to hemorrhage in all of the defined anatomic spaces. The classification is useful, however, in thinking about an individual patient presenting with NTOH. The following anatomic patterns of NTOH are welldescribed: 1. 2. 3. 4. 5.

Diffuse intraorbital Localized intraorbital (hematic cyst) Subperiosteal Related to extraocular muscle (EOM) Related to orbital floor implants.

Underlying clinicopathologic factors in the development of NTOH include: 1. 2. 3. 4. 5. 6. 7.

Vascular lesions Increased cranial venous pressure Bleeding disorders Infection Inflammation Neoplasms Other orbital lesions.

In some cases, it may be impossible to assign an underlying cause to the NTOH and these patients can be truly termed idiopathic; however, with modern investigation and imaging, these patients are very rare.

5.

Diffuse intraorbital hemorrhage

Diffuse intraorbital hemorrhage, or hemorrhage that is not localized to 1 anatomic site, may occur in several clinical

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settings, including underlying vascular lesions, inflammation, and neoplasms. These are covered in subsequent sections. There are case reports, however, of diffuse intraorbital hemorrhage in the absence of these underlying factors.

5.1. Diffuse intraorbital hemorrhage with increased cranial venous pressure Just as in NTSOH, a sudden increase in cranial venous pressure can lead to intraorbital hemorrhage. Corl and associates described a case of bilateral orbital hemorrhage in a patient who strained to move her bowels and felt “a pop” behind the right eye and rapidly developed proptosis with loss of vision. Imaging showed bilateral orbital hemorrhages, much worse on the right.43 After evacuation of the hematoma on the right, her vision recovered. Geyer and colleagues described a case of acute diffuse intraorbital hemorrhage in a woman giving birth for the 8th time with no effect on the vision and spontaneous resolution with no sequelae.65 Intraconal hemorrhage has also been reported in a scuba diver suffering barotrauma where, rather than a rise in cranial or orbital venous pressure, there is a reduction in pressure inside the diving mask leading to a suction effect on the orbital contents.25 Ocular hemorrhage is a well-recognized complication of subarachnoid hemorrhage, and the mechanism may be an increase in intracranial venous pressure. Rarely, diffuse intraorbital hemorrhage has been reported in association with subarachnoid hemorrhage from ruptured intracranial aneurysm.26,138 One condition that can lead to a significant increase in orbital venous pressure is carotid cavernous sinus fistula. Despite this, orbital hemorrhage has not been reported in this entity, but intracranial hemorrhage and epistaxis are recognized complications.

5.2. Diffuse intraorbital hemorrhage occurring with general anesthesia and surgery Several cases of diffuse intraorbital hemorrhage have occurred during general anesthesia. The procedures have included arthroplasty, removal of a pharyngeal foreign body, and gastroscopy.1,10,201 All had minimal effect on vision and resolved with conservative treatment. In 1 case, there are insufficient data to determine whether the hemorrhage was intraorbital or subperiosteal.78 None of these patients had any underlying pathology, and it is likely that an increase in cranial venous pressure during anesthesia or at the time of extubation lead to the bleeding.

5.3. Diffuse intraorbital hemorrhage occurring with bleeding disorders Various bleeding disorders may lead to diffuse intraorbital hemorrhage, including endogenous disorders such as hemophilia, thrombocytopenia, leukemia, and aplastic anemia.17,75,162,169,214,222,229 In the majority of more recent reports, the hemorrhages developed with anticoagulation, including warfarin,198 heparin,38,174 and systemic thrombolytic agents used after acute myocardial infarction or coronary

artery angioplasty.42,46,121 A case of massive subtenon’s hemorrhage occurred in an elderly patient on warfarin.36 A case of orbital hemorrhage has also been described in the setting of purpura fulminans secondary to meningococcal septicemia.180

5.4.

Idiopathic diffuse intraorbital hemorrhage

In several cases of diffuse intraorbital hemorrhage, no obvious cause could be identified.31,176,188 Two cases of diffuse intraorbital hemorrhage were in patients who were hypertensive at presentation, but had no other risk factors.111,175

6.

Orbital hemorrhage and vascular lesions

A large proportion of NTOH cases occurs in the presence of an orbital vascular malformation or other vascular lesion. The nomenclature of orbital vascular lesions has been confused, but recent attempts have been made to simplify the classification on the basis of their flow characteristics.84 The types of vascular lesions reported to lead to hemorrhage within the orbit include: 1. No-flow vascular malformations (“lymphangiomas”) 2. Low-flow vascular malformations (venous anomalies) 3. High-flow vascular malformations (arteriovenous malformations [AVM] and fistulas) 4. Cavernous hemangiomas 5. Intraorbital arterial aneurysms 6. Epithelioid hemangioma (angiolymphoid hyperplasia and eonsinophilia) 7. Intravascular papillary endothelial hyperplasia. A large majority are in the first 2 groups. Hemorrhage from cavernous hemangiomas and intraorbital aneurysms is rare. The largest study of NTOH is that of Sullivan and Wright.192 They describe 115 patients with NTOH seen in 1 institution over 24 years. Associated vascular malformations were present in 104 (90%). No predisposing cause could be found in 6 (5%); in 13 (11%), there were additional causative factors such as increased venous pressure, hypertension, or bleeding diatheses. The commonest presentation was acute-onset painful proptosis. In 37 (32%), there was reduced visual acuity at presentation. Eight (7%) underwent surgery for optic nerve compromise, and excluding these, 62% had a complete resolution of their visual compromise, 27% had a partial resolution, and 4% had no improvement. Final visual acuity was reduced in 23 (20%). The majority of patients presented with their first hemorrhage in the first 2 decades of life (Fig. 1). If presenting in the first 2 decades, the likelihood of a vascular malformation being present was very high (97%), and much less when the patient presented first after the age of 50 (62%). Repeated hemorrhages (from 2 to 10) occurred in 41 patients, and was commonest in those with low-flow (venous) anomalies, where 36 of 97 (37%) had recurrent hemorrhages in the follow-up period. In the high-flow lesions (AVM), 4 of 7 (57%) had recurrent bleeding. Where no vascular malformation could be identified, recurrent bleeding was rare (only 1 of 11 [9%]).

s u r v e y o f o p h t h a l m o l o g y 5 9 ( 2 0 1 4 ) 1 6 6 e1 8 4

Fig. 1 e (A) A 13-year-old girl underwent surgical debulking of the right medial orbital extraconal component of a lowflow vascular malformation at the age of 7 years. She represented with an orbital hemorrhage and reduced vision at age 13. A localized hemorrhagic collection was aspirated and the vision recovered. (B) Axial computed tomography (CT) showed a well-circumscribed lesion with layering of blood products within it in the intraconal space. (C ) Lower axial cut on the orbital CT scan showed a multiloculated, low-flow vascular malformation throughout the intraconal space.

An earlier study from the same institution of 17 patients with NTOH found that 11 of the 17 had an underlying low-flow vascular malformation.110 This study was of interest in that it preceded routine CT, and most patients were investigated by orbital venography, which showed venous anomalies in 9 of 10 patients on whom this study was performed. Looked at another way, patients with low-flow (venous) malformations have been reported to have presented initially with a hemorrhage in 59 of 158 (37%) cases in another large series from Moorfields Eye Hospital.217 Another study of 22 patients with orbital venous malformations found that deep orbital lesions were more likely to present with acute-onset

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proptosis owing to hemorrhage or thrombosis, and these lesions were nondistensible.14 Conversely, a large study of 42 perioribital lymphatic (no-flow) malformations found 52% had episodes of intralesional bleeding in the study period.72 In Lacey and colleagues’ 30 patients with distensible venous orbital malformations, 8 (27%) presented with hemorrhage.115 Hemorrhage was commoner in deeper lesions and in those with combined lymphatic and venous features. The same group has reported a group of 12 patients with venous or combined venous lymphatic malformations of the orbit presenting acutely with some features consistent with orbital hemorrhage (acute-onset pain, proptosis, and nausea) who were found instead to have thrombosis of part of the lesion rather than hemorrhage.134 Four of the 5 with combined venous lymphatic lesions also had some signs of bleeding. A study from Korea reported 8 patients presenting for the first time with orbital hemorrhage in the presence of a distensible venous anomaly.106 The authors pointed out that MRI at the time of presentation and CT at follow-up after resolution of acute hemorrhage confirmed the diagnosis without the need for surgery. In some reports, the vascular malformation is discovered at surgery for an acute hemorrhage.158,164,195 Orbital AVM are rare and difficult-to-treat lesions that may present with hemorrhage, which may also be recurrent. Sullivan and Wright included 7 cases of AVM in their large series and 4 of these experienced recurrent bleeding.192 Another series of 4 orbital AVMs reported hemorrhage in only 1 of the 4.88 Moin and associates have also reported a case of spontaneous hemorrhage in an orbital AVM,142 but a series of 8 cases of orbital AVM did not have any with hemorrhage as a clinical feature.207 A single case of recurrent orbital hemorrhage occurring in a patient with multiple AVM and Wyburn-Mason syndrome has been described.212 One of the commonest orbital vascular lesions is cavernous hemangioma. Three large series of these have been reported with total number of 365 cases, and hemorrhage did not occur in any.85,136,220 There have, however, been scattered case reports of cavernous hemangioma presenting with acute hemorrhage confirmed histopathologically.15,28,218,219,227 Epithelioid hemangioma or angiolymphoid hyperplasia with eosinophilia is a rare orbital lesion. It has also been reported to present with spontaneous hemorrhage, but this is an even rarer event.32 Ophthalmic artery aneurysms are rare and even more rarely present with massive hemorrhage.137 A case of intraorbital hemorrhage secondary to an aneurysm of the maxillary artery in the inferior orbital fissure has been reported.173 This was treated by ligation of the maxillary artery in the pterygopalatine fissure. Another rare orbital lesion is intravascular endothelial hyperplasia and a recent case report has described hemorrhage as part of the presenting features.2

7.

NTSOH

Probably the second most frequent cause of NTOH after vascular malformations is NTSOH, which can occur in a number of settings. The commonest is where there is a sudden increase in cranial venous pressure, as with vomiting, straining during parturition, or with strangulation.16

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Other groups are those with bleeding disorders, paranasal sinus infection or mucocele, and during or after surgery with general anesthesia. A distinct group occurs in young patients with sickle cell crises who develop infarction of orbital bone and an adjacent subperiosteal hemorrhage. In some patients, a cause cannot be identified. Patients with NTSOH typically present acutely, having developed symptoms over minutes to hours, or wake with symptoms in the morning. These presentations include proptosis, globe displacement, upper lid swelling, pain, nausea and vomiting, vertical diplopia, and, in some cases, reduced vision. Findings include reduced acuity and, less commonly, signs of optic nerve compromise (reduced color vision, afferent pupil defect, visual field defect, and altered visual evoked potentials). Most have proptosis and hypoglobus, lid and conjunctival swelling, and reduced eye movements, particularly in upgaze. CT typically shows a well-demarcated, biconvex mass in the superior orbit lying against the orbital roof, with inferior displacement of adjacent orbital soft tissues. The mass is homogeneous, but can show heterogeneity with layering of blood products in some cases. The lesion is slightly denser than brain on CT (Figs. 2 and 3). MRI is useful in confirming the presence of blood in the lesion (Fig. 4). The signal intensity varies depending on the age of the hemorrhage.30,A The evolution of the hemorrhage can be divided into 5 stages, which reflect the steady breakdown of blood: 1. Hyperacute - intracellular oxyhemoglobin - isointense on both T1- and T2-weighted imaging

Fig. 2 e (A) A 57-year-old man presented with acute-onset right hypoglobus and diplopia on upgaze the morning after an episode of vomiting. Symptoms and signs resolved completely after several weeks without treatment. (B) Coronal computed tomography shows a lenticular shaped collection in the right superior orbit representing subperiosteal hemorrhage.

Fig. 3 e (A) A 30-year-old woman collapsed suddenly with a massive pulmonary embolus. She was resuscitated, intubated, and anticoagulated with warfarin. At day 2 in the intensive care unit, she was noted to have bilateral proptosis and right chemosis. (B) Coronal computed tomography (CT) of the orbits showed bilateral nontraumatic subperiosteal orbital hemorrhages superiorly with some globe compression. Both lesions were surgically drained and confirmed as blood clot. (C ) Sagittal CT showed the typical lenticular shaped subperiosteal collection with layering of the blood products within the lesion and marked globe compression.

2. Acute (1e2 days) - intracellular deoxyhemoglobin - T2 signal intensity drops (T2 shortening) - T1 remains intermediate to long 3. Early subacute (2e7 days) - intracellular methemoglobin - T1 signal gradually increases (T1 shortening) to become hyperintense 4. Late subacute (7 to 14e28 days) - extracellular methemoglobin - an increase in the T2 signal also 5. Chronic (longer than 14e28 days) - periphery - intracellular hemosiderin - low signal on both T1- and T2-weighted imaging - center - extracellular hemichromes - isointense on T1- and hyperintense on T2-weighted imaging.

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Table 1 e Underlying causes for nontraumatic subperiosteal orbital hemorrhage with a sudden rise in venous pressure in 33 reported cases Cause

N

Vomiting Scuba diving/barotrauma Parturition (mother) Birth (infant) Strangulation/headlock Weightlifting Thoracoabdominal crush injury Total

Fig. 4 e (A) T1-weighted coronal magnetic resonance imaging (MRI) of the orbits in a 70-year-old woman who presented with acute right proptosis an diplopia after heavy lifting in the garden. There is a high signal intensity biconvex lesion in the right orbital roof representing a nontraumatic subperiosteal orbital hemorrhage. (B) T2weighted coronal MRI of the orbits shows a high signal lesion with some adjacent opacification of the ethmoid sinus.

These changes on MRI are best seen before the administration of gadolinium contrast, which can mask the signal features. The source of bleeding in NTSOH has usually been stated to be from small veins crossing the subperiosteal space between the bone of the orbital roof and the periorbita. At surgery when the periorbita is reflected from the orbital roof, small vessels may be encountered, and these may be the source of the bleeding. Whittnall in his classic textbook on orbital anatomy describes bony foramina in the orbital roof, usually in the region of the lacrimal gland fossa, that are present in some skulls, and have been termed cribra orbitalia.210 He cites several studies which found cribra orbitalia in between 11 and 25% of dried skulls. Whittnall ascribed these foraminae to the presence of venous channels passing from the diploe of the skull to the periorbita, and if that is so, then these could also be a source of the bleeding in NTSOH.

7.1. NTSOH with a sudden increase in cranial venous pressure Of the 33 cases summarized here, the underlying causes are listed in Table 1.9,16,18,20,21,23,24,29,33,39,44,53,60,65,70,83,90,97,104, 105,109,116,125,145,168,173,197,215 Vomiting was the most common. The clinical features are summarized in Table 2. There is a strong female preponderance, which is unexplained. The age range is broad, from birth to 70 years. The 4 infants with NTSOH all presented at or soon after birth with clinical and imaging signs of NTSOH. It is not clear whether NTSOH in the

11 7 6 (1 also postpartum hemorrhage) 4 3 1 1 33

newborn is owing to raised venous pressure or direct birth trauma, but given the position of the hemorrhage in the orbit and its characteristic appearance in patients with an increase in cranial venous pressure, it seems reasonable to assume that such an increase in venous pressure is at least part of the reason for the development of the hemorrhage. Several cases of NTSOH have been reported in scuba divers suffering barotrauma.9,24,33,39,70,116,168,215 This form of barotrauma is not from a sudden increase in cranial and orbital venous pressure, but rather a fall in pressure in the diver’s mask leading to a suction effect on the orbital contents and an increase in the pressure differential between the veins and the orbital soft tissues. One case of diffuse intraorbital hemorrhage has also been reported with this type of barotrauma.23 Most cases were unilateral; only 4 of the 33 were bilateral. In 31 cases, the subperiosteal collection was in the superior orbit; in only 2 cases did it occur in the medial orbit. There were no cases in the lateral or inferior orbit. Vision at presentation was normal in most, with 4 having a mild reduction in acuity of 20/30, and 1 patient having hand movements vision only. All recovered to normal, except this latter patient who recovered from hand movements to 20/40. Treatment was conservative in the majority with complete recovery. One patient underwent canthotomy and cantholysis, and 6 underwent surgical drainage of the subperiosteal collection. There have been no recorded cases of recurrent NTSOH.

7.2.

NTSOH with bleeding disorders

NTSOH occurs in patients with bleeding disorders.4,7,16,52,71, 73,77,79,91,126,128,141,143,154,161,170,172,184,193,206,224 The underlying disorders are listed in Table 3 and the clinical details of 23 reported cases plus 1 additional unpublished case of the author are summarized in Table 2. Again, patients of all ages are in this group, but notably all patients with scurvy were children (aged 1.5, 2, 3, 5, 5, and 13 years).79,172,184,193,206 Males and females are more equally affected in this group. All 24 cases had their hemorrhage in the superior orbit. Bilateral cases were much commoner (12 of 24 cases), and vision was more often severely affected, with 2 patients not regaining any vision. Some of these patients were very ill. Two had disseminated intravascular coagulation.16,71 The first patient with disseminated intravascular coagulation developed this after massive postpartum hemorrhage, transfusion, cardiac arrest, and

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Table 2 e Clinical features of 124 cases of nontraumatic subperiosteal orbital hemorrhage (NTSOH) of various causes NTSOH with rise in venous pressure (N ¼ 33)

NTSOH with sinus infection/mucocele (N ¼ 21)

NTSOH idiopathic (N ¼ 13)

NTSOH with surgery and general anesthesia (N ¼ 7)

NTSOH with sickle cell crisis (N ¼ 26)

23 (70)

13 (59)a

14 (67)

11 (85)

0e70 31 30

2e65 33 28

4e70 57 47

1.5e81.0 43 41

31 (94) 2 (6) 0 0

22 (100)b 0 0 0

21 (100) 0 0 0

10 (77) 2 (15) 1 (8) 0

7 (100) 0 0 0

15 (48)d 12 (38) 4 (13)

4 (17) 8 (33) 12 (50)

9 (43) 12 (57) 0

8 (62) 5 (38) 0

2 (29) 4 (57) 1 (14)

11 (42) 4 (15) 11 (42)

3 (27)h 4 (20/25e20/100) 4 (CF, CF, HM, HM)

2 (29)i 4 (20/25e20/40) 1 (20/200)

13 (46) 13 (20/25e20/80) 2 (20/200, 20/800)

30 (86)e 4 (20/30) 1 (HM)

9 (41)f 5 (20/25e20/100) 8 (20/200 e NPL (3 eyes)

7 (39)g 8 (20/25e20/70) 3 (CF, CF, NPL)

4 (57)

8 (31)

43e74 51 57

2e35 11 13 7 (35)c 0 9 (45) 4 (20)

36 (97) 1 (20/40) 0

25 (81)b 4 (20/30e20/100) 2 (both NPL)

17 (89)j 2 (20/25, 20/30) 0

10 (100)k 0 0

8 (100) 1 1

35 (100) 0 0

26 (79) 6 (18) 1 (cantholysis)

16 (67) including vitamin C 7 (29) 1 (aspiration)

1 (5) 20 (95) 2 by craniotomy 0

2 (15) 10 (77) 5 by craniotomy 1 (aspiration)

7 (100) 0 0

22 (85) 4 (15)

CF ¼ counting fingers; HM ¼ hand motions; NLP ¼ no light perception; VA ¼ visual acuity. Vision at follow-up not recorded in 3 eyes; 1 (bilateral) had improved vision, 1 died postoperatively (scurvy with NTSOH and extradural hematoma). Vision at presentation not recorded in 9 eyes. Vision at follow-up not recorded in 1 case. a Gender not specified in 2 cases. b Location not specified in 2 cases. c Location not recorded in 6 cases. d Laterality not specified in 2 cases. e Vision at presentation not recorded in 1 bilateral case. f Vision at presentation not recorded at presentation, and 2 eyes had “reduced vision.” g Vision at presentation not recorded in 2 cases, and “reduced” in 1 case. h Vision at presentation not recorded in 2 cases. i Vision at presentation not recorded in 1 case. j Vision at follow-up not recorded at in 2 cases. k Vision at follow-up not recorded in 1 case, and “improved” in 2 cases.

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Female gender, N (%) Age (yrs) Range Median Mean Location, N (%) Superior Medial Lateral Superolateral Laterality, N (%) Right Left Bilateral VA at presentation (eyes) Normal, N (%) Mild-moderate red Severe reduction VA at last visit (eyes) Normal, N (%) Mild-moderate reduction Severe reduction Management (%) Conservative Surgical Other

NTSOH with bleeding disorders (N ¼ 24)

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resuscitation. Bilateral NTSOH was only detected when the patient regained consciousness 1 week after admission to the intensive care unit. One eye lost all vision and the other regained 20/30 vision with a small residual island of field with an altitudinal defect. The visual loss may have been the result of ischemic optic neuropathy secondary to hypotension and anemia rather than the orbital hemorrhages.16 The other patient who lost all vision in 1 eye had disseminated intravascular coagulation and disseminated carcinoma of the prostate. The orbital hemorrhage was not drained because of the risk of operating with poor clotting and the patient’s poor prognosis. He died 4 months later, but with good vision in the contralateral eye.71 Two of the children with scurvy who presented with NTSOH had concurrent large extradural hemorrhages, which required surgical drainage.193,206 One of these children died immediately postoperatively.193 All of the remaining children recovered well with the addition of vitamin C to their diet. One of the reported cases of NTSOH in scurvy also had thalassemia.184 Sickle cell disease and sickle-thalassemia are known to cause orbital bone infarction and in some cases adjacent NTSOH, and it is possible that the hemoglobinopathy in this patient contributed to the development of the subperiosteal bleeding. Another patient had thalassemia with severe anemia and thrombocytopenia and presented with bilateral NTSOH.161 In this case, it seems more likely the thrombocytopenia was the main factor in leading to the NTSOH.

7.3.

NTSOH with paranasal sinusitis or mucocele

A report from 1968, before the advent of CT, described a case of spontaneous orbital hemorrhage in association with frontal and ethmoid sinusitis but the description of the surgical details does not make it possible to be sure whether the hemorrhage was subperiosteal or elsewhere within the orbit.96 Since then, there have been 20 cases of NTSOH reported in association with paranasal sinus infection or mucocele,5,13,16,41,86,94,107,118,122,130,155,156,173,187,189,204,216,226 and the author has cared for 1 additional patient (Fig. 5). The clinical details are summarized in Table 2. Patients with NTSOH arising in association with paranasal sinus infection or mucocele tended to be somewhat older than in the previous 2 groups, but the overall age range remains broad. There is a female preponderance in this group as well,

Table 3 e Underlying causes for 24 cases of nontraumatic subperiosteal orbital hemorrhage occurring in patients with bleeding disorders Cause

N

Liver disease Scurvy Thrombolysis/anticoagulation Disseminated intravascular coagulation Christmas disease Chronic myeloid leukemia Henoch-Schonlein purpura Von Willebrand disease Thalassemia, thrombocytopenia Total

7 6 4 2 1 1 1 1 1 24

Fig. 5 e (A) A 14-year-old boy developed signs of right orbital cellulitis over 48 hours. Several hours before presentation, there was a sudden worsening of the right proptosis and upper eyelid swelling, and worse vertical diplopia. (B) Marked limitation of elevation of the right eye. (C ) coronal computed tomography (CT) of the orbits and paranasal sinuses shows ethmoidal sinus opacification and some thickening if the maxillary sinus mucosa. There is a small orbital collection adjacent to the right medial orbital wall and a larger separate lesion in the orbital roof. (D) A more posterior coronal CT of the orbits shows the superior orbital lesion to extend well posteriorly. The orbit was explored via a medial transconjunctival approach. The medial collection was a small subperiosteal abscess. The superior collection was a subperiosteal hemorrhage. The patient made a full recovery.

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similar to that occurring with NTSOH with increased cranial venous pressure. A variety of sinuses have been affected, but the majority had ethmoid or frontal sinus pathology, usually bacterial infection, but there has been a small number with mucoceles. Of the 3 cases of NTSOH occurring in association with mucoceles (frontal or fronto-ethmoidal), one also had associated infection.13 Vision may be moderately affected. Some patients have had associated orbital cellulitis as well as NTSOH, which may have contributed to the reduced vision. One patient had no light perception vision at presentation, but improved to 20/30 at 3 weeks after surgical drainage.5 It has not always been apparent whether the subperiosteal collection has been infective or hemorrhagic, and this has only become clear at surgery. The history of acute, sudden worsening of proptosis and the signal characteristics on MRI are helpful in pointing toward the diagnosis of NTSOH. Probably because of the associated paranasal sinus infection and uncertainty about the nature of the collection in these patients, surgical drainage has almost always been performed. Two of the patients underwent craniotomy to drain the superior NTSOH, an unnecessarily invasive technique when simpler anterior orbital approaches are more than adequate.13,204 One patient in this group had an associated extradural hemorrhage.189 In the neurosurgical literature, there are reports of a number of cases of spontaneous extradural hemorrhage occurring in association with paranasal sinus infection or mucocele, and the mechanism is presumably similar to that occurring with NTSOH and sinus infection or mucocele. What this mechanism is remains obscure. The case reported by Spennato and colleagues is the only one with both NTSOH and extradural hemorrhage occurring concurrently in association with paranasal sinus infection or mucocele.189 A number of other cases cited by Spennato and colleagues claim this association, but careful assessment shows that, rather than NTSOH, these patients only had orbital cellulitis.

7.4.

NTSOH with no identifiable cause (idiopathic)

There have been 12 reports of NTSOH where no identifiable cause was found8,12,16,35,81,92,114,131,147,159,171,211 (Table 2). Women were again far more commonly affected. The age range was broad. Vision was reduced in the majority at presentation, some severely, but all recovered or improved with treatment. Nine of the 12 underwent surgical drainage and of these, 6 underwent craniotomy. As demonstrated in numerous other case reports, these collections can be readily drained via an anterior orbital approach without craniotomy.

7.5. NTSOH occurring in association with general anesthesia and operative procedures Seven cases of NTSOH have been reported after surgery and general anesthesia11,19,55,61,93,157,221 (Tables 2 and 4). A majority were undergoing cardiac or vascular procedures, and there may have been a contribution from anticoagulants. One occurred in a patient in the prone position, and increased

Table 4 e Operative procedures performed in 7 cases of nontraumatic subperiosteal orbital hemorrhage Occurring after surgery and general anesthesia Procedure

N

Cardiac surgery Coronary angioplasty Carotid aneurysm coiling Skin grafting in prone position Phacoemulsification (other eye)

2 2 1 1 1

cranial venous pressure may have contributed.221 Cranial venous pressure is also often increased at the time of extubation after a general anesthetic, and this is another potential mechanism. All had a good visual outcome.

7.6.

NTSOH occurring with sickle cell crises

A distinct clinical entity may occur in young patients with sickle cell disease that mimics the more typical patient with NTSOH. Sickle cell patients are susceptible to “crises” that can be brought on by systemic acidosis, cold, or hypoxia, when their red blood cells change to a crescent shape and are relatively rigid. This can lead to vasoocclusion, which typically affects the long bones, but any bone with marrow can be affected. The bones of the orbit may still have active marrow in the orbital roof and greater wing of the sphenoid. This bone becomes ischemic, and adjacent to this ischemic bone, swelling occurs, leading to painful proptosis. In some cases, blood can collect adjacent to the infarcted bone in the subperiosteal space. Sokol and coworkers summarized 27 previously reported cases, and in 20 where sufficient data were available, subperiosteal hematoma was reported in 12 (60%).186 In the same 20, intracranial hematoma occurred in 7 (35%). Table 2 summarizes the clinical details of 26 cases of sickle cell crisis with orbital bone infarction complicated by NTSOH.6,47,54,56,57,63,64,66,103,108,124,127,148,150,153,178,186,200 Patients in this group are nearly all children or young adults; boys are more commonly affected for no obvious reason. Bilateral subperiosteal collections are common (42% of cases) in sickle cell patients with NTSOH. The locations of the subperiosteal hemorrhage in patients with sickle cell disease differs somewhat from the other groups of NTSOH patients in whom a high proportion occur in the superior orbit. With sickle cell disease, the hematomas are often superolateral or purely lateral. This occurs because the hematoma occurs adjacent to the infarcted bone, which is either the frontal bone or the greater wing of the sphenoid, which form part of the lateral wall of the orbit. There may be components in these collections other than just blood, with some references describing “fluid collections” and “exudate”.66,186 The lateral or superolateral localization is a useful distinguishing clinical sign, but there are other pointers to the diagnosis of sickle cell disease in a patient presenting with NTSOH. These include race, young age, pain, and marked lid and conjunctival swelling. On MRI, there is a different signal in the adjacent bone, indicating loss of blood flow to the bone.

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Table 5 e Clinical features in 25 patients with nontraumatic orbital hemorrhage occurring in relation to extraocular muscles (19 published cases, 6 additional cases managed by the author) Feature Gender, N (%) Male Female Age (yrs) Range Median Mean Laterality Right Left Bilateral Muscle affected IR MR LR SR Vision at presentation (eyes) Normal Mild-moderate reduced Severely reduced Vision at last visit (eyes) Normal Mild-moderate reduced Severely reduced Management Conservative Surgical drainage Aspiration

Value 13 (52%) 12 (48%) 13e84 68 62.5 12 11 2 15 5 4 3 26 1 d 27 d d 23 1 1

IR ¼ inferior rectus; LR ¼ lateral rectus; MR ¼ medial rectus; SR ¼ superior rectus.

Many are known to have sickle cell disease before presentation. Vision was often mildly or moderately reduced, but in all cases returned to normal with appropriate medical care, which usually involved systemic corticosteroids and supportive measures including blood transfusion. A few underwent surgical drainage. This group of patients is another that may present with simultaneous extradural hematomas. An interesting overlap in etiologic factors concerns the possible role of low levels of vitamin C in sickle cell patients. Khouri and associates reported a case of bilateral superior NTSOH in a patient with sickle cell disease.108 The patient had severe vitamin C deficiency as well. It has been reported that up to 50% of sickle cell patients have vitamin C levels within the range regarded as deficient.40 The mechanism may relate to iron overload leading to decreased gastrointestinal absorption of vitamin C.

8. Orbital hemorrhage in relation to extraocular muscles NTOH may occur in direct anatomic relation to one of the EOMs. The clinical details of 19 reported cases and 6 additional patients cared for by the author are summarized in Table 5.22,25,49,82,124,151,181,182,199,205,213,230 Their underlying medical problems are listed in Table 6.

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Table 6 e Underlying medical problems in 25 patients with nontraumatic orbital hemorrhage in relation to extraocular muscles Condition

N

Hypertension (treated) Oral antiplatelet agents Hypercholesterolemia (treated) Acute myeloid leukemia Atrial fibrillation Autoimmune hepatitis Chronic obstructive airway disease Hypothyroidism (treated) Mitral regurgitation

5 3 2 1 1 1 1 1 1

NTOH in relation to EOMs is a distinct subgroup. Patients typically present with acute onset of unilateral proptosis often first evident on waking in the morning, diplopia, pain, and sometimes nausea and vomiting. They are generally older (median, 68 years). The inferior rectus muscle is the most commonly affected (13/25 muscles; 52%). In no case has there been any other underlying orbital pathology identified, and none had a sudden increase in cranial venous pressure as a possible causative factor. Three patients were on antiplatelet agents, and one had thrombocytopenia related to acute myeloid leukemia. Five had treated hypertension. The hemorrhage has been a single event in nearly all patients, although 1 patient has been reported with 3 episodes of bleeding in the same lateral rectus muscle within 7 months.199 In nearly all cases, these patients settle spontaneously over several weeks with no sequelae. Surgery is rarely required, except when vision is threatened. The imaging features of NTOH in relation to EOMs are typical (Fig. 6). A well-defined mass is seen either within the belly of the muscle or, more commonly, apparently within the muscle sheath with displacement of the muscle belly to 1 side of the lesion. The lesion is usually rounded anteriorly and tapering toward the orbital apex. Rose and Verity include this entity in their review of acute presentations of orbital vascular disease166 but do not comment on the relationship of the hemorrhage to the EOMs or their sheath, and described the typical shape of the lesion as resembling a “beached whale.” The hemorrhages typically occur in the elderly, who usually develop a more diffuse hemorrhage because of attenuation of the orbital connective tissue septa. Because the hemorrhages, which seem to occur in relation to the EOMs, are wellcircumscribed, it seems that the hemorrhage is confined anatomically by a structure, and this appears to be the sheath of 1 EOM. Follow-up scans do show some minor changes in relation to the affected muscle (Fig. 7). The lesion is usually homogeneous, but in some cases layering of the hematoma can be seen as the blood products separate. The MRI signal characteristics vary depending on the age of the hematoma. The lesion gradually reduces in size as the hematoma resorbs, and after several months there is only some minor residual thickening of the muscle (Fig. 7). In long-term follow-up, no other orbital pathology has become evident in any of these cases. The source of the bleeding in these patients is obscure. Given the size of the hematoma and the acute onset, the

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Fig. 6 e (A) A-72 year-old woman with treated hypertension awoke with left orbital pain, nausea, and proptosis with vertical diplopia and marked limitation of left eye elevation. (B) Coronal computed tomography (CT) of the orbits shows a large lesion in the inferior orbit. The belly of the inferior rectus muscle seems to be on the medial aspect of the lesion. (C ) Axial CT of the orbit shows the typical appearance of an acute hemorrhage in relation to the inferior rectus muscle. The anterior border of the lesion is rounded and well-circumscribed. The belly of the inferior rectus muscle runs along the medial aspect of the lesion. The patient’s symptoms and signs resolved spontaneously over several weeks.

bleeding likely comes from the arterial circulation. The arterial branches to the EOMs may be the source, and in the inferior orbit the orbital branch of the infraorbital artery is another possibility. Certainly, the inferior rectus is the most commonly affected.

9.

Hemorrhage in orbital neoplasms

The question often arises in a patient presenting acutely with what might be a spontaneous orbital hemorrhage as to

Fig. 7 e (A) A 68-year-old woman awoke with right orbital pain, diplopia, and nausea and vomiting. There was marked restriction of right eye elevation. Coronal computed tomography (CT) showed a presumed hemorrhage in relation to the inferior rectus muscle with the suggestion of layering of blood products. She was treated conservatively. (B) Repeat CT at 6 weeks shows the lesion has reduced significantly in size. (C ) Orbital magnetic resonance image at 6 months shows the right inferior orbital mass has almost completely resolved. There is some minor residual change in the inferior rectus muscle. She was completely asymptomatic at this stage.

whether there may be an underlying malignancy. Despite this concern, the numbers of patients reported with orbital neoplasms that present with hemorrhage is small. Certain neoplasms are known to present with bleeding more commonly and the classical example is metastatic neuroblastoma in children. Ocular involvement in neuroblastoma has been studied by Musarella and associates.146 They examined the records of 405 patients seen at the Hospital for Sick Children, Toronto, with neuroblastoma. Ophthalmic involvement was seen in 80 (20%). Orbital disease with proptosis was present in 60 (75%) and, in these, periorbital bruising occurred in 22 patients, often bilaterally. In a much smaller series, 2 of 6 patients with metastatic neuroblastoma presenting to a pediatric

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ophthalmologist had periorbital bruising.3 Occasionally, the bruising may be the presenting sign of neuroblastoma.144 The commonest orbital malignancy is lymphoma. Bleeding in lymphoma is distinctly rare, but there have been case reports of bleeding as the presenting feature.89 In the largest series describing the clinical features of patients with ocular adnexal lymphoma, none of 326 patients had hemorrhage.99 Orbital granulocytic sarcoma (chloroma or extramedullary acute myeloid leukemia) may occasionally present with bleeding. In a series of 7 cases presenting in childhood, 1 had bleeding into the eyelid.177 One often highly vascular orbital neoplasm is hemangiopericytoma. In a series of 30 cases, eyelid swelling with or without ecchymosis of the eyelid was a presenting feature in 3 cases (10%).45 A small number of individual case reports have described hemorrhage occurring with orbital neoplasms. These have included rhabdomyosarcoma,27,98 primary orbital melanoma,119 anaplastic carcinoma of the lacrimal gland,165 and metastatic renal cell carcinoma.87 The feature these tumors have in common is relatively rapid growth and possible tumor necrosis. Bleeding may be commoner with highly malignant orbital tumors than the small number of case reports suggests, but is presumably a relatively minor component of the clinical presentation. Benign orbital lesions very rarely present with bleeding. One case of bleeding within an atypical pleomorphic adenoma of the lacrimal gland is reported.140

10.

Other hemorrhagic orbital lesions

10.1.

Hematic cyst

The term hematic cyst has been used to describe a number of clinical entities, including a localized apparently encysted hemorrhage in the presence of a vascular malformation,209 orbitofrontal cholesterol granuloma, and NTSOH. These are specific entities, and the term hematic cyst probably should be confined to those lesions where there is an intraorbital collection of blood surrounded by a nonepithelialized capsule in the absence of other pathologies. Such lesions are rare. Amrith and colleagues reported 3 cases of “spontaneous hematic cyst.”8 Their first case fits this definition of hematic cyst, but the second is possibly in association with a vascular malformation and the third case was a subperiosteal hemorrhage. Cameron and coworkers described a case that would fit with hematic cyst,34 and Matsuura and colleagues also reported a single case with evidence of old and recent hemorrhage in a fibrocollagenous capsule.132 Yoshikawa and associates reported 3 cases of “hematic cyst” of the orbit, and 2 of these fit that definition, but the third looks more like an orbitofrontal cholesterol granuloma.225 They produced some evidence of increased fibrinolysis and tissue plasminogen activator function that might explain how these lesions can continue to expand. Travis and colleagues reported 8 cases of intraconal orbital hematic cysts in young people, but it is likely also that a proportion of these occurred in patients with vascular malformations.202 There are insufficient data to determine

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this. In one of their cases, the cyst was large with bony orbital enlargement, and in others adjacent to the orbital wall, there was some bone remodeling. Spontaneous intraorbital hemorrhages in younger patients tend to be localized,191 and some of these have been termed hematic cysts, but most probably have occurred in the presence of vascular malformations. Occasionally, an encysted or localized hemorrhage in a very young child may occur in the absence of evidence of a vascular malformation.149 Many of these hematic cysts have required surgical excision because of associated morbidity and the prognosis has been generally good.

10.2.

Orbital amyloidosis

Another orbital disease that may be associated with nontraumatic hemorrhage is orbital amyloidosis. A review of 100 cases of various types of generalized amyloidosis has shown that bleeding is a common manifestation of amyloidosis, occurring in 41.223 The bleeding is most likely owing to direct infiltration of blood vessels by amyloid. There have been several case reports of ocular adnexal amyloidosis presenting with periorbital bleeding. This may occur in amyloidosis secondary to multiple myeloma,69 in familial transthyretin amyloidosis,152 in AA amyloidosis causing renal disease,113 and after diagnostic proctoscopy in primary systemic amyloidosis183 or general anesthesia.208 A large series of 24 cases of periocular and orbital amyloidosis found periocular hemorrhage as a presenting feature in 3 cases (12.5%).120 Another 3 developed subconjunctival hemorrhage. The hemorrhages in patients with amyloidosis are within the eyelid, rather than the orbit, but it is worth considering the diagnosis of amyloidosis in patients presenting with orbital signs and eyelid bruising.

10.3.

Hemorrhage in idiopathic orbital inflammation

There is a small number of cases of orbital hemorrhage reported in association with idiopathic orbital inflammation. In 2 of these cases, the clinical presentation was one of orbital myositis.163 In the other cases, the inflammatory process was less well-localized. A biopsy was obtained in 3 of the 5 cases, and the diagnosis in the other cases was based on clinical, imaging, and laboratory findings, as well as response to corticosteroids, so an element of doubt exists as to the true nature of the underlying pathology.123,191,203 All cases, however, made a complete recovery with normal follow-up imaging.

11. Hemorrhage in relation to alloplastic orbital floor implants A well-recognized clinical syndrome is delayed orbital hemorrhage within the capsule surrounding orbital floor implants used to repair orbital floor fractures. Patients present acutely or subacutely with swelling, proptosis, hyperglobus, and often diplopia. CT or MRI shows a lenticular shaped mass in relation to the orbital floor implant (Fig. 8). With MRI, the

178

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Fig. 8 e (A) A 40-year-old man presented with acute onset of right hyperglobus and vertical diplopia. He had undergone repair of a right orbital floor fracture with a thin silicone sheet implant 17 years earlier. (B) Coronal computed tomography (CT) showed a lenticular-shaped collection above the orbital floor implant. The area was surgically explored. An old hematoma was drained and the orbital floor implant removed along with part of its fibrous capsule.

overrepresentation in series of trauma. The age range is broad. The hemorrhage typically occurs many years after the surgical repair of an orbital floor fracture with an implant. In most cases, patients present with the first hemorrhage, with a small number having recurrent episodes before presentation or after a conservative approach to management. In 34 cases, the implant has been removed, sometimes in conjunction with removal of part or all of the surrounding fibrous capsule. In only 1 case, this capsule has had respiratory epithelium lining it100; all others showing a nonepithelialized, fibrous capsule. After removal of the implant, no case of recurrent hemorrhage has occurred. The type of alloplastic orbital implant in these cases has been either silicone, supramid, or Teflon (Table 8). There have been no reported cases of delayed peri-implant hemorrhage with porous implants such as porous polyethylene, or metallic mesh implants, and none with autogenous implants (bone or cartilage grafts). Whether this is related to the relative frequency of the use of these materials or that the risk of delayed hemorrhage is commoner with some materials cannot be determined. Curiously, there have been no cases of peri-implant hemorrhage occurring with medial wall implants or with implants used after enucleation or evisceration, although similar materials are used in this type of surgery, and the implant is quite mobile. The cause and source of the hemorrhage in these patients is unclear. Some authors have concluded that there is bleeding from small vessels in the capsule surrounding these implants, but what initiates this bleeding remains obscure.

12. mass has signal characteristics of blood or blood breakdown products. The clinical features of 37 reported cases are summarized in Table 7.37,48,50,67,68,80,95,100e102,129,133,135,139,167,179,190,194,196 A majority occurred in males, in keeping with their

Table 7 e Clinical features in 37 cases of delayed hemorrhage occurring in relation to orbital floor implants Feature Gender, N (%) Male Female Age* (yrs) Range Median Mean Years after initial surgery Range Median Mean Episodes of bleeding Single Recurrent Management Removal of implant Conservative * Age not mentioned in 2 cases.

Value 20 (57) 15 (43) 17e89 40 41.0 2 weeks e 25 yrs 12 11.4 31 6 (2, 3 or “several”) 34 3

Clinical and management issues

Most patients presenting with NTOH give a history of fairly sudden onset of symptoms, which may include visual disturbance, diplopia, globe displacement, and eyelid swelling, sometimes with visible bruising appearing in the lids and conjunctiva later. In many patients, there are no signs in the eyelids or conjunctiva of bruising or edema at presentation or later (Fig. 6A). There may be pain, nausea, and vomiting.166 The different patterns of NTOH outlined determines the exact nature of the symptoms and signs, but the best clue to the diagnosis of NTOH is the suddenness of onset. Subacute presentations may occur, but are less frequent. Imaging features conform to the patterns as outlined. If the clinical presentation and imaging features are consistent with a diagnosis of NTOH, and the vision is not compromised, then a conservative approach can be adopted because in many instances the blood resorbs without

Table 8 e Orbital implant material in 37 cases of delayed peri-implant orbital floor hemorrhage Material

N

Silicone Supramid Teflon Unknown

15 11 6 5

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significant sequelae. If the vision is compromised or proptosis is severe with risk of corneal exposure, then drainage of collections can be undertaken. This is safe in cases of NTSOH, NTOH in relation to EOMs, and with bleeding around orbital floor implants, but is risky with intraorbital hemorrhage occurring with vascular malformations, where a conservative approach is favored unless vision is significantly compromised. To avoid recurrence of bleeding with orbital floor implants, the collection should be drained and implant removed.

13.

Conclusion

NTOH is uncommon, but presents in one of several distinct clinical patterns. Anatomically, the hemorrhage may occur diffusely or localized within the orbit, extraperiosteally, in relation to EOMs and their sheath, or surrounding orbital floor implants. Predisposing factors include underlying vascular malformations, a sudden increase in cranial venous pressure, bleeding disorders, sickle cell crises, or adjacent paranasal sinus infection or mucocele. Some orbital neoplasms may present with hemorrhage, as may ocular adnexal amyloidosis. Knowledge of these patterns of NTOH allows the clinician to make a firm diagnosis in a majority of cases and allow an appropriate course of management, which in many cases is conservative.

14.

Method of literature search

A PubMed search of the terms “orbital hemorrhage,” “spontaneous orbital hemorrhage,” “nontraumatic orbital hemorrhage,” “hematic cyst,” “nontraumatic subperiosteal orbital hemorrhage,” “orbital sickle cell infarction,” “extraocular muscle hemorrhage,” “orbital tumor hemorrhage,” and combinations was performed. The same search terms were used for Google searches to find publications not included on PubMed. The bibliography of all publications was examined for appropriate publications for inclusion. Full manuscripts were examined and publications in foreign languages were translated.

15.

Disclosure

The author has no conflicts of interest to declare.

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