David H. Verity
Dr. Verity is a Consultant Ophthalmic Surgeon, and Adnexal Service Director, Moorfields Eye Hospital, London.He is a Honorary Senior Clinical Lecturer, University College London.Trained in ophthalmology in the UK, Dr. Verity undertook two Fellowships in ophthalmic adnexal disease before joining the Consultant staff at Moorfields Eye Hospital in 2004.He is a Full Member of the British Oculoplastic Surgery Society (current Treasurer), Treasurer Elect for the European Society of Ophthalmic Plastic and Reconstructive Surgery, and Full Member of the International Society of Ocular Oncology.In 2009, he was elected to the Orbital Society, in 2010 became Editor-in Chief of the journal ORBIT, and in 2016 became Oculoplastic Section Editor for the Journal of Ophthalmic and Vision Research.His medical publications in peer-reviewed journals and books include 90(+) publications, and with a wide range of research interests, he is an active national and international teacher and surgical trainer.Dr. Verity also has a life-long interest in the charitable work of the St John Eye Hospital Group. In 2014, he was invested in the Order of St John, and in 2016 he joined the Board as Trustee of the Hospital Group.
Usha R. Kim
Geoffrey E. Rose
Geoffrey Rose graduated BSc Pharmacology, MBBS, and MRCP. His postgraduate ophthalmic training culminated in award of FRCS in 1985 and FRCOphth at its foundation in 1988. In 1990, the University of London granted an MS doctorate for corneal research and, in 2004, a Doctor of Science in Ophthalmology and Ophthalmic Surgery.
Professor Rose was appointed consultant to Moorfields Eye Hospital, London, in 1990 and is also a Senior Research Fellow of the NIHR Biomedical Research Centre of the Institute of Ophthalmology. He served as the British Council member of the European Society of Ophthalmic Plastic and Reconstructive, is a Past-President of the British Oculoplastic Surgical Society, and is President of the European Society of Oculoplastic and Reconstructive Surgeons.
Dr. Usha Kim received her MBBS from Bharathiar University, Coimbatore, in 1991, Diploma in Ophthalmology and MNAMS (Oph), from Aravind Eye Hospital, Madurai, in the year 1994 and 1995, respectively. She completed her specialty training under the guidance of Dr. William B. Stewart at University of California, San Francisco, and at Salt Lake City, Utah, in the United States of America. She has established and is currently heading the Orbit, Oculoplasty and Ocular Oncology department at Aravind Eye Hospital since 1998. Under her efficient leadership, the clinic has seen several innovations such as the addition of Ocular Oncology Services and an Ocular Prosthesis Centre. She has trained nearly 55 national and 16 international candidates as fellows in the field of Orbit, Oculoplasty and Ocular Oncology. She has also trained 41 Ocularists both at the national and international level.She is also The Director of the MLOP program which forms the backbone of the Aravind Eye Care System. She has been a member of Aravind Research Committee since March 1998 and is a Principle investigator for many studies conducted at Aravind Eye Care System. She is actively involved in various research activities and currently focuses on genetics of Retinoblastoma. She has 43 publications to her credit – 23 international and 18 national. She has also written chapters in many books and coauthored a book on “Imaging in Orbit and Neuroophthalmology.” She established “The Ring of Hope” in 2004, a program that provides free service to patients, children, and adults who have life-threatening cancers in the eye like retinoblastoma. The Ring of Hope Fund, since its inception, has supported 1600 ocular cancer patients, who would otherwise not have been able to receive treatment.
Introduction
Orbital vascular disease can present with acute symptoms and, if untreated, can lead to loss of visual functions. In general, such a presentation is due to orbital haemorrhage, acute arteriovenous shunting or subacute thrombosis with resultant local changes in orbital perfusion. On occasion, the clinical presentation heralds the presence of previously unrecognised disease, whereas in others, it occurs against a background of a known diagnosis of vascular disorder.
Pathogenesis
Visual loss occurs when the elevation of orbital pressure causes decrease in central retinal artery perfusion or compression of the long and short posterior ciliary arteries resulting in retina and optic nerve ischaemia, respectively. Increased orbital pressure can also cause vision loss secondary to central retinal vein occlusion or compressive optic neuropathy [1].
Clinical Features
Typical symptoms and signs of an acute vascular event within the orbit include sudden onset of proptosis, usually occurring overnight (Fig. 49.1), painful periorbital oedema, decreased or double vision and nausea or vomiting. Any pathology at the orbital apex leads to impairment of the venous drainage manifesting as episcleral venous congestion and a secondary rise in intraocular pressure. If acute extravascular haemorrhage or intravascular pathology occurs at the orbital apex, embarrassment of the posteriorly directed venous drainage occurs and manifests as a episcleral venous congestion and a secondary rise in intraocular pressure. Periocular bruising appears after a delay, which depends on the depth of the haemorrhage within the orbit. The rapid expansion of the intraorbital volume and sudden stretching of the extraocular muscles can lead to severe pain, nausea and vomiting; nausea and vomiting are mediated through the oculo-cardiac vagal reflex and are particularly troublesome in children, in whom the symptoms can be incorrectly attributed to presumed intracranial injury (a scenario that can also occur following blow-out fractures in children; see Chap. 14). In cases of significant arteriovenous shunting, the intraocular pressure will often also show an abnormally high variation during the cardiac cycle (as high as 10 mmHg), and, when suspected, this sign should actively be elicited on applanation tonometry. A secondary effect of such vascular congestion and fluid leakage is an increased intercellular fluid volume, resulting in oedematous extraocular muscles (manifest as a global reduction in eye movements) and caruncular or subconjunctival oedema. Very rarely, frank bleeding can occur from the orbit and on occasion patients can be aware of a sudden onset of a ‘pulsation’ or ‘rushing sound’ within their head in high-flow carotid-cavernous fistula.
Fig. 49.1
Sudden onset of proptosis and partial visual loss due to acute haemorrhage in a patient with a mixed orbital venous-lymphatic malformation
Vascular Lesions of the Orbit
Acute Orbital Haemorrhage [2–6]
Spontaneous haemorrhage from a normal orbital vessel is exceptionally rare. It may occur during an acute increase in the orbital venous pressure during a significant Valsalva manoeuvre (e.g. during delivery, exertional sports, such as weightlifting, severe vomiting or strangulation). Bleeding can also occur following an orbital fracture if the small vessels which perforate the orbital walls are sheared; in some cases, the site of blow can be outside the orbit (particularly with frontal fractures), and this should be considered where there is loss of vision in the absence of direct injury to the globe.
In contrast to the rarity of orbital haemorrhage from healthy vessels, bleeding from fragile vessels in the elderly patient is relatively common (and the most common cause of all orbital haemorrhages). Associated risk factors are the concurrent use of anti-platelet agents (aspirin and other nonsteroidal anti-inflammatory agents, warfarin and clopidogrel), long-standing (or uncontrolled) hypertension and chronic diabetes in younger patients. In these patients, a precipitating event, such as a severe bout of coughing, can result in a rapid onset of unilateral proptosis, usually with minimal reduction of vision or ocular ductions. Imaging with CT typically identifies such spontaneous haemorrhages in the posterior half of the infero-temporal quadrant of the orbit, these sometimes described as having a ‘beached whale’ configuration (Fig. 49.2).
Fig. 49.2
T2-weighted MR showing typical appearance of an acute haemorrhage, biassed inferolaterally within the left orbit, it having a hyperintense ‘capsule’ surrounding the lower signal blood products
In both congenital and acquired orbital vascular lesions, haemorrhage usually occurs with low-pressure venous or venous-lymphatic (‘lymphangioma’) anomalies and not, as might be expected, with high-pressure (or high-flow) arteriovenous malformations or fistulas. In patients with orbital lymphangiomas, spontaneous haemorrhages tend to occur in the first decade of life (Fig. 49.3) and seem to become less frequent with advancing years – this possibly occurring as a result of perivascular fibrosis in regions of prior haemorrhage. Infrequently, haemorrhage in adults can arise from a previously unrecognised venous anomaly (Fig. 49.4) and can also occur in orbital tumours undergoing necrosis.
Fig. 49.3
Preoperative image showing a 12-year-old patient with a known deep orbital venous-lymphatic malformation, presenting with sudden onset of proptosis and partial visual loss due to acute orbital haemorrhage
Fig. 49.4
Acute orbital haemorrhage in a young male presenting with proptosis and early optic neuropathy; haemorrhage related to a bi-lobed varix was identified at surgery and the haemorrhage drained and venous anomaly excised
Finally, spontaneous haemorrhage can also occur into the fibrous sheath around long-standing silicone implants (such as floor implants); again, such patients present with acute proptosis – and often with hyperglobus – but the appearance of periocular bruising can be delayed for up to a week.
Orbital haemorrhage following trauma [7] can be accidental or iatrogenic. Haemorrhage within the orbit may occur as a postoperative complication of orbital or periorbital surgeries, certain oculoplastic and lacrimal procedures, most commonly orbitotomies, fracture repairs, blepharoplasties and dacryocystorhinostomies [8, 9]. Retrobulbar haemorrhage occurs in less than 2 % of retrobulbar and peribulbar anaesthetic injections and may result in devastating visual loss if not promptly managed [10] (See Chap. 11).
Diagnosis is clinical and should be suspected in patients presenting with acute proptosis, rapid vision loss, ophthalmoplegia and relative afferent pupillary defect [11]. Evaluation and treatment must be prompt to ensure the best possible outcome. Determining the location of the haemorrhage and the etiologic mechanism is essential for providing the most appropriate intervention and requires a thorough understanding of the orbital and periorbital anatomy.
Treatment of Spontaneous Orbital Haemorrhage [12–14]
Irrespective of the cause, the hydrostatic pressure generated by an arterial haemorrhage can be sufficient to arrest vascular perfusion at the orbital apex or in the peripapillary area, both carrying a dire visual prognosis. In the presence of dramatic proptosis, marked loss of eye movements and increased resistance to retropulsion, immediate treatment is imperative if visual function is to be saved. Treatment should be directed to drainage of the haemorrhage or creation of more space for the orbital contents by disrupting the orbital septum. Once a rupture of the globe has been excluded, intermittent firm pressure should be applied to tamponade the orbit until the bleeding subsides (due to vasospasm, platelet action and coagulation) and whilst secondary treatment is being organised. The firm tamponade should be released at regular intervals to permit optic nerve perfusion, and urgent arrangements made to drain the haemorrhage or reduce the intraorbital pressure by disrupting the orbital septum. In the case of acute postoperative haemorrhage, the incision should be widely reopened, and, with blunt dissection, the tissues are spread to permit release of the haematoma; the clinician should not be concerned if orbital fat prolapses from the incision, as this will result in further lowering of the orbital pressure.
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