Hatem A. Tawfik
Dr. Hatem A Tawfik, M.D., graduated from Ain Shams University School of medicine, Cairo, Egypt. He did his research fellowship at Duke University Eye center in 1998. He then served as a Lecturer in Ophthalmology at the University until his resignation in 2011. He is currently a Consultant Ophthalmic Plastic Surgeon at Watany Eye Hospital, and the Eye Subspecialty center in Cairo. He has 48 peer-reviewed international publications, 3 book chapters, and more than 200 oral and poster presentations in numerous international meetings. He is also a semi-professional photographer and a passionate art collector focusing specifically on contemporary Arab artists.
Yousef Ahmed Fouad
graduated from the Faculty of Medicine, Ain Shams University, Cairo, Egypt. He then went on to study Cancer Biology and Therapeutics at Harvard Medical School. As a student, he worked as an assistant researcher on multiple projects, and even served as a principal investigator in one study. He has 7 peer-reviewed papers, 4 of which are related to ophthalmic plastic surgery. His fields of interest include Ophthalmic Plastic Surgery, Cancer Biology, and Medical Oncology.
Yasmin Ashraf Hamza
graduated from the Faculty of Medicine, Ain Shams University, Cairo, Egypt. She is currently halfway into her internship at Ain Shams University Hospitals. Yasmin chose to train in Ophthalmology as it has always been her passion since the start of medical school. She has assisted on multiple research projects and served leading positions in the Ain Shams Medical Students Research Association.
Introduction
The only weapon with which the unconscious patient can immediately retaliate upon the incompetent surgeon is hemorrhage (William Stewart Halsted, 1912)
On casual examination in a dried skull, the orbit looks like an open storage box, although in clinical practice, it behaves more like a watertight closed jewelry box [1]. Of the many nightmares any surgeon has after a busy day at surgery is a retrobulbar hemorrhage (RBH). Bleeding into the confined space of the orbit increases its internal pressure (orbital compartment syndrome or OCS) resulting in retinal ischemia and a subsequent irreversible loss of vision within 60–100 min [2]. However as we shall see, surgery is not the sole cause of a hematoma confined to the retrobulbar space.
Nomenclature
Orbital hemorrhage has been referred to by a number of terms in the literature, including orbital hemorrhage, orbital hematoma, hematic cyst, encysted hemorrhage, RBH, and OCS. Although the term OCS is gaining popularity in recent literature, we still prefer to use the term RBH because OCS may have a heterogeneous etiology and is not a term circumscribed to hemorrhages alone.
Classification
Some authors have attempted a simple anatomical classification based on the location of hemorrhage into intraorbital hemorrhage and subperiosteal hemorrhage [3], while others have attempted a more elaborate classification systems into (1) diffuse intraorbital, (2) localized intraorbital (hematic cyst), (3) subperiosteal, (4) related to extraocular muscle (EOM), and (5) related to orbital floor implants [4]. Because of the inclusion of traumatic retrobulbar hemorrhage in the current discussion, an anatomical classification is not applicable because traumatic hemorrhage may not lend itself to specific anatomical location. In addition, it will be obvious after reading this chapter that there exists a significant overlap between nosological types of RBH, the etiopathogenesis, and the anatomic location; therefore, we will adopt a simple etiological/anatomic classification solely for the purpose of the current discussion.
- A.
Traumatic hemorrhage
- I.
Diffuse
- II.
Localized
- I.
- B.
Surgical hemorrhage
- I.
Diffuse
- II.
Localized
- I.
- C.
Non-traumatic hemorrhage
- I.
Diffuse
- II.
Localized
- I.
- D.
Atypical forms
- (1)
Subperiosteal hemorrhage
- (2)
Hematic cyst
- (3)
Beached whale hematoma
- (1)
Traumatic Hemorrhage
A traumatic RBH (Fig. 18.1) usually presents with an acute unilateral arterial bleed (infraorbital or ethmoidal arteries), which characteristically develops within a few hours following trauma, but may be delayed for days [2]. The incidence of traumatic RBH ranges from 0.45 to 12 % following blunt or penetrating trauma to the orbit or zygoma [5]. RBH is less likely to occur with displaced fractures of the orbit because of the sudden expansion of the tight orbital milieu, but it may still occur [2,6]. On the other hand, undisplaced, blow-in (Fig. 18.1a, b), or even small linear fractures of the orbital walls may cause minimal bleeding that cannot possibly drain into the paranasal sinuses [6]. Patients with displaced fractures however are not immune from developing a blinding RBH. The location of the hemorrhage/fracture is also important. In a recent study in eight patients with traumatic RBH presenting with vision loss, six out of the eight hemorrhages were superior [6].
Fig. 18.1
Traumatic hemorrhage. (a, b) Right frontal bone fracture, subdural hematoma, and massive right RBH with blow-in fracture of the orbital roof. The patient was unconscious on admission with right relative afferent pupillary defect. Urgent canthotomy, cantholysis, and inferior septal release resulted in complete recovery of vision. (c) Massive blow to the face with a high-velocity high-pressure pneumatic grease-gun at point-blank. Patient presented with light perception vision, stone-hard orbit, diffuse subconjunctival hemorrhage, diffuse intraconal RBH on CT, and greasy material coming out of a small superonasal eyelid wound. (d) Patient referred for repair of upper eyelid laceration and canalicular tear. On examination the orbit was stone hard with inability to open the eyelids even by force. Canthotomy/cantholysis under general anesthesia was complicated by persistent bleeding. The patient was finally diagnosed with mild hemophilia after three repeated factor VIII assays. (e, f) 17-year-old male patient presenting 1 week after a blow to the left orbit during a street fight. CT scan showed a well-circumscribed hematoma with layering of blood within it in the medial extraconal space. The right pupil was dilated irreactive with no light perception vision
Surgical Hemorrhage
RBH may also develop as a complication of any surgery on or near the orbit or any anesthesia technique that violates the orbital septum (also referred to as iatrogenic RBH) [5]. Surgeries commonly associated with increased risk of RBH include:
Blepharoplasty
The incidence of post-blepharoplasty orbital hemorrhage (Fig. 18.2a, f) was reported to be 1 in 2,000, with a 1 in 10,000 risk of development of permanent visual loss [7]. Most hemorrhages occur within the first 24 h, but delayed hemorrhage has been documented for as long as 9–15 days postoperatively [7]. Although Hass et al. put this figure at 6 h [7], in our experience the most critical period where close monitoring is mandatory is the first 3 h.
Fig. 18.2
Surgical hemorrhage. (a) RBH in a patient who underwent upper and lower eyelid blepharoplasty. The patient later admitted she was abusing alprazolam for the past 2 months. (b) Bilateral RBH developing after balanced orbital decompression. (c) Unilateral RBH developing after bilateral endonasal nonendoscopic DCR in a 70-year-old female. (d) RBH developing immediately after debulking of hemangiopericytoma. Preoperative visual acuity was hand motion. Bleeding recurred despite immediate decompression through a lateral canthotomy and cantholysis. (e) Periprosthetic bleeding. Acute proptosis and delayed orbital hemorrhage due to prior fracture floor repair 6 years ago with a silicone sheet. (f) Severe unilateral RBH developing 3 h after transconjunctival blepharoplasty in a chronic Khat abuser with malignant hypertension. Patient was admitted to the ICU after canthotomy/cantholysis
We usually avoid any form of orbital fat clamping to avoid tearing of deep orbital vessels. An important source of bleeding in lower eyelid blepharoplasty which manifests by a sudden gush during surgery and may be challenging to manage is hemorrhage due to injury of the inferior oblique muscle. The surgeon must outweigh the benefits of cauterizing the source of bleeding versus the risk of inducing fibrosis and subsequent diplopia with the cautery.
An often overlooked cause of bleeding in upper eyelid blepharoplasty is excessive dissection in and around medial orbital fat compartment which could result in inadvertent injury to the superior root of the superior ophthalmic vein (which represents the orbital continuation of the supraorbital and the supratrochlear veins) [8] or even the superior ophthalmic vein itself, with massive catastrophic bleeding.
Endoscopic Sinus Surgery (ESS)
Endonasal Dacryocystorhinostomy (DCR)
Orbital Surgery
Although fracture floor repair particularly with the use of solid implants (silicone, Teflon, Supramid) [11] is allegedly notorious for the development of RBH and is potentially the most serious complication of the surgery, with a reported incidence rate of 3.4 % [12], we have only encountered a single case so far (Fig. 18.2e).
Certain pathologies occasionally encountered during orbital removal of solid tumors may induce severe intraoperative or postoperative bleeding that may be challenging to manage. Hemangiopericytomas which are radiologically quite similar to venous malformations (previously called cavernous hemangioma) may present with life-threatening hemorrhage (Fig. 18.2d) that may only respond to ligation of the external carotid artery [13]. In our experience, even a simple biopsy for certain metastatic orbital lesions particularly hepatocellular carcinoma may be associated with severe uncontrolled RBH, and extreme care should be exercised if the primary is already well known [14].
Conventional wisdom would dictate that orbital decompression surgery for thyroid-associated orbitopathy should rarely be associated with RBH, and indeed this was the case in the era of inferomedial wall decompression because it created a huge communication with the sinuses. However, as more and more surgeons are moving toward lateral approaches, the risk of an RBH is becoming real regardless of whether the deep lateral wall is decompressed alone or with medial wall decompression (Fig. 18.2b). Because the orbit is divided into compartments and because of the proximity of the deep lateral wall to the orbital apex, a small collection of blood in this area may be critically close to the optic nerve and may rapidly impair vision [12] (Fig. 18.2b).
Strabismus Surgery
Several case reports have appeared in the literature documenting RBH after strabismus surgery even without any predisposing factor [15].
Non-traumatic Hemorrhage
In the absence of trauma or surgery around the orbit, RBH is rare but is occasionally seen in clinical practice (Fig. 18.3). Absolutely spontaneous RBH is extremely rare, and usually a predisposing factor is encountered. In fact several case reports in the literature described as ‘non-traumatic’ are actually cases associated with an unusual form of trauma [20]. A survey of our own medical records as well as a review of the literature has revealed three different patterns of presentation of non-traumatic hemorrhage:
Fig. 18.3
Non-traumatic hemorrhage. (a, b) Subacute presentation of RBH in a patient with chronic liver disease. T2-weighted MRI showed a hyperintense inferior intraocular lesion not compressing the optic nerve. Vision was 20/20 (OU). (c, d) Complete loss of vision immediately following delivery in a multigravida female. Pregnancy was complicated by preeclampsia. A T1-weighted MRI performed at presentation 2 weeks after delivery shows a slightly hyperintensive intraconal collection of blood pushing the optic nerve medially. (e, f) A 73-year-old male on self-prescribed anticoagulants. On examination there was massive RBH, subconjunctival chemosis, and severe resistance to retropulsion. CT ordered at presentation (5 days following onset of symptoms) showed unilateral non-traumatic SOH superiorly with some globe compression. Vision loss was irreversible. (g, h) Inferonasal beached whale hematoma in a 71-year-old hypertensive male after severe bronchospasm. Patient was not anticoagulated. (i, j) Patient with a 4-month history of diplopia and 15-month history of slowly progressing painless proptosis. CT showed a relatively well-circumscribed lesion in the superotemporal quadrant with significant destruction of the frontal bone
- 1.
An elderly hypertensive patient presenting with an acute onset of unilateral proptosis or diplopia typically on waking up in the morning. This patient is usually on aspirin and may or may not recall a precipitating event such as severe coughing, severe straining, or vomiting [3,11,12]. These patients typically present with hemorrhage in the inferotemporal quadrant of the orbit with a “beached whale” configuration, although we have seen one patient with similar findings inferonasally (Fig. 18.3g, h) [12]. Prognosis for vision is usually excellent, but we have observed one such patient with complete amaurosis.
- 2.
A young, probably muscular or athletic patient presenting with acute proptosis or diplopia following a severe bout of emesis, straining, or lifting weights. Location of hemorrhage is usually superior subperiosteal and prognosis for vision is usually excellent.
- 3.
Hemorrhage in a Unique Anatomic Location
Subperiosteal Hemorrhage
Subperiosteal hematomas (SOH) rarely occur in the orbit [4], as a rule develop superiorly near the roof of the orbit [4], and may be recurrent (Fig. 18.3e, f). They usually result from avulsion or rupture of subperiosteal blood vessels but may also be an extension of a subgaleal hematoma, and in such scenario, they may also be bilateral due to the fact that the subgaleal region is continuous with the orbital subperiosteal space [4]. CT usually shows a homogenous (occasionally heterogeneous due to blood layering) mass in the superior orbit with inferior displacement of orbital contents [11].
Hematic Cyst
McNab strictly defined the term hematic cyst as an “intraorbital lesion characterized by a collection of blood and blood breakdown products within a cystlike structure without an epithelial lining and without an underlying vascular malformation” [11]. This specific terminology is used to differentiate it from the lymphangiomatous “chocolate cyst” which bears an epithelial lining [21]. Because these lesions are characteristically long standing, painless, and superior, with associated bone erosion, this has prompted some authors to theorize that they represent neglected SOH [4,21]. CT usually shows a soft tissue mass in the upper temporal quadrant with bone erosion that may simulate a malignant process in the lacrimal gland or a ruptured dermoid cyst (Fig. 18.3i, j). Pathological examination usually reveals a granulomatous or foreign body reaction to blood breakdown products with no epithelial or endothelial lining [21].
Beached Whale Hematoma
To the best of our knowledge, Rose and Verity coined the term “beached whale” hematoma [12] and used it specifically to describe arterial hemorrhages that localize inferotemporally along the orbital floor in elderly patients with a fragile musculature. It is difficult to conclude whether the beached whale is a separate entity or simply a variation of SOH which attributes its specific shape on CT simply due to an inferior location (Fig. 18.3g, h).
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