A 75-year-old man with a recent history of pulmonary embolism, presented with collapse followed by a gran mal seizure and right-sided non-pulsatile proptosis. On recovery, he had diplopia on lateral and upward gaze and signs of congestive cardiac failure. Further pulmonary embolism was proven by lung scintigraphy. Computed tomography of his orbits confirmed a contrast-enhancing space-occupying lesion of the medial wall of the right orbit, with no intracranial abnormality. The patient was investigated for metastatic tumour as a possible cause of the space-occupying lesion and the unprovoked thromboembolic event, but no evidence of malignancy was found. The orbital lesion was not biopsied because of the risk of bleeding from anticoagulation. Three weeks later, the patient re-presented with recurrent cardiac failure, proptosis, and diplopia. A transorbital ultrasound confirmed an encapsulated, well-defined vascular lesion, with typical appearances and Doppler flow characteristics of a cavernous haemangioma. Diuretic therapy abolished the proptosis and diplopia in tandem with relief of the cardiac failure. This is the first description of recurrent proptosis with diplopia due to recurrent congestive expansion of an orbital cavernous haemangioma.
- cardiac failure
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Acute unilateral proptosis causing diplopia is usually the result of acute orbital haemorrhage, cavernous sinus thrombosis, or orbital sepsis. It may, however, result from acute congestive expansion of orbital tumours. A resolving, relapsing cause of unilateral proptosis with diplopia occurring in tandem with recurrent right-sided cardiac failure should suggest a vascular lesion communicating with the major veins of the head and neck, such as cavernous haemangioma, as this case illustrates.
A 75-year-old man was brought to hospital as an emergency following collapse followed by a gran mal seizure. On admission to the Accident & Emergency department, he was still unconscious but no longer fitting. He had rapid atrial fibrillation (110 beats/min) and marked cyanosis (arterial oxygen saturation 77%), moderately elevated blood pressure (180/100 mmHg) but no signs of respiratory distress. Signs of chronic lower limb venous insufficiency, a left lower parasternal heave and distended neck veins were noted. There was obvious non-pulsatile proptosis of the right eye without conjunctival suffusion or an overlying bruit. Both the optic disc and fundus looked normal. His neurological status was considered consistent with a post-ictal state. A non-contrast cranial computed tomography (CT) scan was performed in order to exclude intracranial haemorrhage and cavernous sinus thrombosis. The appearances suggested reduced venous drainage from the right orbit but no other abnormalities. A chest X-ray showed cardiac enlargement and a right basal pleural effusion, but no other abnormalities. Later, collateral history from the patient's daughter revealed that he had been hospitalised 2 months previously with an extensive right leg deep venous thrombosis associated with pulmonary embolism (confirmed on lung ventilation/perfusion scintigraphy) and that he had been taking warfarin since then with doubtful compliance.
The patient was treated with intravenous heparin infusion and 40% inspired oxygen. Five hours after the ictus, the patient had recovered consciousness, and complained of new-onset painless double vision and dyspnoea at rest. Over the next 4 days, his overall clinical status improved, but he continued to have right-sided proptosis and double vision on extremes of lateral gaze. He remained cyanosed with signs of right-sided cardiac failure. Because of persisting right-sided proptosis, on the fourth hospital day the patient had a further cranial orbital CT scan with intravenous contrast. This showed a well-defined, contrast-enhancing retrobulbar mass on the medial side of the right orbit, medial to the medial rectus muscle, stretching along the ethmoid plate and extending into the retrobulbar space, displacing the medial rectus muscle (figure). The differential diagnosis was tumour mass or haemangioma. Clinical, laboratory and radiological investigation for extraorbital malignancy proved negative, and the patient was referred for a clinical ophthalmological assessment. Arising from this, an orbital duplex ultrasound confirmed an ovoid vascular tumour (21 × 18 × 12 mm) with scan appearances and Doppler blood flow characteristics highly consistent with cavernous haemangioma.
Over the next 2 weeks, the patient continued to improve with warfarin, digoxin and diuretics. As the signs of right heart failure regressed, so did the proptosis, and the diplopia eventually resolved completely. The patient was discharged from hospital 22 days after admission on warfarin, digoxin 125 μg daily and bumetanide 1 mg daily. Unfortunately, 3 weeks later, he was readmitted to hospital as an emergency with a 4-day history of relapsing cardiac failure, with recurrent right-sided proptosis and diplopia. Cardiac failure with right-sided pleural effusion was confirmed on chest radiograph. He was treated with intravenous frusemide for 3 days with good effect. Once again, the patient's proptosis receded and the diplopia resolved with stabilisation of his cardiac failure. After treatment of the acute heart failure, an echocardiogram confirmed biventricular systolic dysfunction, bilateral AV valve regurgitation and a mildly elevated pulmonary artery pressure (26 mmHg).
At out-patients follow-up, it was not possible to reproduce the proptosis and diplopia by means of head-down tilt testing or internal jugular venous obstruction by compression at the root of the neck on the affected side. With supervised drug compliance, the patient's cardiac failure has remained stable and the proptosis has not recurred. This, coupled with the patient's guarded prognosis due to his marked degree of heart failure, has meant that surgery has not been considered necessary.
Right-sided cardiac failure presenting with diplopia due to congestive expansion of a retrobulbar cavernous haemangioma has not been described in the literature previously. Cavernous haemangioma is the most common benign orbital tumour in adults (‘cavernous’ refers to the macroscopic appearance of the tumour, and does not imply any anatomical association with the cavernous sinus). Typical appearances are those of large ectatic or ‘cavernous’ spaces, lined by flattened endothelial cells overlying a variable mantle of closely aligned spindle cells. Although cavernous haemangiomas may occur in children, they are generally tumours of adulthood, with most cases occurring between 30 and 60 years of age. From a clinicopathological viewpoint, there is no evidence that cavernous haemangiomas arise from capillary haemangiomas of childhood. They can occur anywhere in the orbital cavity, but most typically in the soft tissues behind the globe, as in this case. The mass is usually sited within the muscle cone, most often inferior and lateral to the optic nerve. Cavernous haemangiomas usually present with slowly progressive unilateral proptosis, and may be associated with optic disc oedema and choroidal folds visible on funduscopy. Visual disability may result from a high degree of relative hypermetropia or from optic disc compression. Features encountered in some other orbital vascular lesions, like intermittent and fluctuating proptosis, postural variation of proptosis and visual distortion, pulsation or bruit, haemorrhagic cyst or phlebolith formation are usually not found with orbital cavernous haemangioma.1
Transorbital duplex ultrasound, which demonstrates the well-defined, capsular, septate structure of the tumour and its highly vascular nature, is currently the method of choice for diagnosis. Because of the high degree of definition of the lesion with ultrasound, tumour angiography or venography is now seldom required. Occasionally, however, in cases of suspected difficult surgical access, direct puncture angiography may be necessary to define the extent of the lesion and its relations to surrounding structures, but there are risks of bleeding and trauma. The availability of high resolution ultrasound and contrast CT or magnetic resonance imaging means that direct puncture angiography is no longer undertaken as part of the routine pre-operative assessment of orbital cavernous haemangiomas.
Definitive treatment is surgical resection, since the natural history is generally that of slowly progressive expansion and worsening proptosis with visual symptoms. In the present case, surgery was not considered necessary because of the resolution of the diplopia concurrent with improvement of the patient's cardiac failure, and the fact that the patient has had no further visual acuity or other ophthalmic symptoms after 9 months of follow-up.
Recurrent congestive expansion of orbital cavernous haemangiomas causing proptosis and diplopia has not been described in the literature before. Acutely raised pulmonary artery pressure may be transmitted to the veins of the head and neck, including the facial vein. The facial vein lacks valves, so that raised facial venous pressure may in turn be transmitted to the orbit via its anastomosis with the superior ophthalmic vein, the principle venous drainage channel of the orbit.2 This is the probable anatomical basis for the congestive expansion of the cavernous haemangioma in this case. The pressure within the cavernous haemangioma was not measured. However, the close temporal association of proptosis/diplopia with the episodes of right-sided cardiac failure and the clearcut resolution of the diplopia simultaneous with resolution of the right heart failure strongly supports a causal link. Contrary to expectation, however, at out-patient follow-up, transient obstruction of venous blood flow by neck root compression did not reproduce proptosis or diplopia in this patient. It may be that sustained, prolonged, elevation of venous pressure (as occurred with the two episodes of right heart failure) is required to alter the compliance characteristics of the tumour sufficient for congestive expansion. In addition, the patient was unable to tolerate neck root compression in the supine posture for more than 2 minutes because of marked orthopnoea. Although fluctuating proptosis is occasionally a feature of cavernous haemangioma,3 accentuation of proptosis by venous flow obstruction in the root of the neck is not documented in the literature as a reliable physical sign. Similarly, change in proptosis with change in posture has not been described in a large series of cases.1
The cause of the gran mal seizure in association with the initial presentation of this patient's diplopia was not thought to be related to the orbital lesion. The CT scan did not show any evidence of cerebral infarction or other abnormality that would readily explain the ictal event. The seizure did occur in association with marked systemic hypoxia (due to pulmonary embolism), which lowers seizure threshold. The patient has been hospitalised subsequently on two occasions with seizures. He has recovered completely on both occasions, without signs of residual neurological deficit.
cavernous haemangioma is the most common benign orbital tumour in adults
the natural history of these tumours is one of slow expansion and gradual onset of symptoms
rarely, subclinical cavernous haemangiomas may undergo acute congestive expansion with associated acute proptosis and visual distortion, as with acute right heart failure
We are not aware of any reported case of right heart failure presenting with recurrent diplopia due to recurring acute congestive expansion of a cavernous haemangioma. Recurrent congestive expansion of a cavernous haemangioma in association with right-sided cardiac failure should be considered as a cause of recurrent proptosis.