Eur Arch Otorhinolaryngol (2000) 257 : 533–536
© Springer-Verlag 2000
RHINOLOGY
Paolo Castelnuovo · S. Mauri · M. Bignami
Spontaneous compressive orbital emphysema of rhinogenic origin
Received: 3 April 2000 / Accepted: 5 September 2000
Abstract We report the case of a young patient who developed spontaneous compressive orbital emphysema after an attack of coughing. At admission the patient presented left proptosis, diplopia, vision impairment and headache. Computer tomography showed air in the lateral part of left orbit compressing the eyeball and the optic nerve medially. It also revealed a sphenoid bone dysplasia with hyperpneumatization of the left greater wing and with two dehiscences in its wall. It was very intriguing to discover that this sphenoid dysplasia and the flap of mucosa covering one dehiscence were causing a ball-valve effect, allowing air to enter but not leave the orbit. Endoscopic sinus surgery was successfully used to treat this case. Keywords Orbital emphysema · Sphenoid dysplasia · Endoscopic sinus surgery
Case report An otherwise healthy 15-year-old white man was referred to our hospital with spontaneous compressive orbital emphysema. He presented with diplopia, left proptosis, vision impairment and left frontal headache. Symptoms had started spontaneously 3 days earlier after a violent attack of coughing, getting worse every time he blew his nose. Coronal and axial computed tomography (CT) scans showed air in the lateral part of the left orbit, compressing the eyeball, optic nerve and ocular muscles medially. No orbital fractures were found. CT scans also revealed sphenoid bone dysplasia in the form of abnormal hyperpneumatization of the left greater wing, continuing, at the level of the squamous suture of the greater wing of sphenoid, with the squamous part of the temporal bone; there were two dehiscences in its walls, one anteriorly, opening into the orbit, and one laterally, opening towards the temporalis muscle (Fig. 1). CT scans showed a small dehiscence at the left lateral sphenoidal
Introduction Compressive orbital emphysema is a very uncommon yet potentially serious complication of orbital surgery or trauma; air that enters the orbit but does not leave it may cause visual loss through optic nerve compression, ischaemia or central retinal artery occlusion [3, 8]. Because of the potential for severe visual loss, rapid diagnosis and management of this condition is essential. We present a case report of a 15-year-old boy who developed spontaneous compressive orbital emphysema after an attack of coughing.
P. Castelnuovo () · S. Mauri · M. Bignami Department of Otorhinolaryngology, University of Pavia, IRCCS Policlinico S. Matteo, P. le Golgi 2, 27100 Pavia, Italy e-mail:
[email protected], Tel.: +39-0382-526218, Fax: +39-0382-528184
Fig. 1 CT scan (axial plane) showing left proptosis, sphenoid bone dysplasia, in the form of abnormal hyperpneumatization of the left greater wing and the two dehiscences in its wall, one anteriorly, opening into the orbit (larger arrow) and one laterally, opening towards the temporalis muscle (smaller arrow)
534 wing wall would not explain the air compressing the temporalis muscle through the lateral dehiscence (Fig. 4). The same situation could have been created by two ball-valve effects at the dehiscence in the greater wing, but this seemed very unlikely. Finally, the CT scan revealed hyperpneumatization of the squamous part of the left temporal bone, apparently asymptomatic, and hyperplasia of the left maxillary sinus mucosa. We treated this case using an endoscopic approach. We decided on the transethmoidal approach to the sphenoid because of the anatomical size of the sphenoethmoidal recess in our patient (a transnasal approach to the sphenoid would have damaged the middle turbinate and it would not have been possible to reach the lateral sphenoidal wall). This method would also be useful in treating left maxillary sinusitis. The first stage of our surgical procedure was a left partial uncinectomy with middle meatotomy to treat the concomitant disease of the left maxillary sinus. We then proceeded to remove the inferiomedial part of the ethmoid bulla and of the basal lamella of the middle turbinate. We penetrated the posterior ethmoid, the mucosa of which was hyperplastic. After identifying and lateralizing the superior conchae, we identified the ostium of the sphenoid si-
Fig. 2 CT scan (coronal plane) showing the dehiscence (arrow) at the left lateral sphenoidal sinus wall, creating a communication between the left hyperpneumatized greater wing and the sphenoidal sinus
A
B Fig. 3 CT scan (axial plane) showing the air passage from the sphenoid sinus to the orbit sinus wall, creating a communication between the left hyperpneumatized greater wing and the sphenoidal sinus (Fig. 2). It was suggested that the pathological event was a ball-valve effect: during intranasal events which involve high pressure (noseblowing, coughing, etc.) air passes through the valve, reaching the lateral part of the orbit by the greater wing (path of least resistance) (Fig. 3). After analysing the CT images, we were quite sure that the ball-valve effect was at the level of the sphenoidal sinus wall, because a valve mechanism at the level of the anterior left greater
Fig. 4 Scheme of etiopathogenetic interpretation. A Sphenoid bone anatomy predisposing to spontaneous compressive orbital emphysema (1 dehiscence in left lateral sphenoid sinus wall; 2 dehiscence in left lateral greater wing wall; 3 dehiscence in anterior greater wing wall). B Situation with regard to air flow, on presentation: an inflamed mucous membrane, creating a ball-valve effect at the level of the lateral sinus wall (1), permitted air to enter but not to leave the hyperpneumatized greater wing during high-pressure events (nose-blowing, coughing). Dehiscences in the wall of the greater wing allowed air to enter the orbit through a pathway of least resistance (in the temporal fossa, the temporal muscle and fascia oppose the air pressure)
535 sema. Some hours after surgery the patient had normal visual acuity and no diplopia left. Two days later the patient went home. He was instructed not to blow his nose, and to cough gently. At 1 year follow-up, orbital emphysema had not recurred and nasal breathing was excellent. No headache remained. CT scans showed complete resolution of the emphysema (Fig. 5), and good ventilation of the paranasal sinuses. CT also confirmed the dysplasia of the lateral sphenoid wing with complete resolution of pneumorbita (Fig. 6).
Discussion
Fig. 5 CT scan (axial plane) at 1-year follow-up showing the complete resolution of orbital emphysema
Fig. 6 CT scan (coronal plane) showing scars (arrow) at the left sphenoidal sinus wall and at the previously hyperpneumatized greater wing
nus with a sinus ostium seeker; the ostium was covered by hyperplastic mucosa, which was removed with a circular cutting punch, and we then enlarged the ostium with a Citelli punch and entered the sphenoidal sinus. Sinus endoscopy revealed an inflamed area of mucosa at the left lateral wall, just behind the bone covering the anulus tendineus communis. Concomitant digital palpation of the left eyeball revealed simultaneous medialization of the inflamed mucosa. While this mucosa was being removed with BlakesleyWeil forceps, air bubbles entered the sinus through the hole, confirming that the obstruction had been cleared. Further air bubbles were released by applying pressure to the left eyeball, with complete and immediate resolution of the compressive orbital emphy-
The sphenoid sinuses start to develop at the third fetal month [1] and usually reach their full size at the age of 15 years. The degree of pneumatization of the sphenoid bone varies considerably. From a recent anatomical study of 200 patients [2], it emerges that the anterior clinoid process is pneumatized in 6% of patients, and the pterygoid in 16%. In his studies, Stammberger [7] found that the bony canal covering the internal carotid artery is partially dehiscent in 25% of patients. So it is not surprising to find a child with a hyperpneumatized greater wing and dehiscence in the walls, even though this has not been reported in the literature. However, it was very intriguing to discover that this sphenoid dysplasia and the flap of mucosa covering the dehiscence were causing a ball-valve effect, allowing air to enter but not leave the orbit. Cases of paranasal sinus disease involving the orbit have been reported, but spontaneous compressive orbital emphysema is a very rare complication [3–6, 8, 9]. Orbital emphysema is usually a benign transient phenomenon. Trauma is the most frequent cause [4, 9] but orbital emphysema may also occur spontaneously, usually after an increase in nasal pressure from nose-blowing, coughing or vomiting. Generally it is a benign self-limited condition. In contrast, compressive orbital emphysema is a very rare but serious event, because of the potential risk of severe visual loss [3, 8]. Understanding the pathophysiology of spontaneous compressive orbital emphysema is important in developing a rational approach for its management. When a patient has spontaneous compressive orbital emphysema, it is essential to consider orbital compression via a ball-valve effect, allowing air to enter but not leave the orbit. This is an emergency situation which requires a CT scan to investigate where the air is coming from. With regard to treatment, intraorbital needle aspiration [3] may relieve compression, with improvement in visual acuity, but this procedure has the limitation of not removing the ball-valve effect. This is why we decided on a surgical method for treating our patient. Endoscopic sinus surgery allowed us to reach and remove the mucosal flap responsible for the ball-valve effect. We decided not to close the defect in the sphenoidal sinus for the following main reasons: (a) the hole was very small and it was situated in the inferior lateral angle of the sphenoid sinus: to fix a graft in this position would have required a larger endoscopic approach; (b) considering that the dehiscence was very small and that the mucosa around the hole was surgically traumatized we thought that spontaneous healing would have created a scar strong enough to prevent re-
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currences. This approach seemed the easiest and the less invasive for the patient’s nasal anatomy. After surgery the patient was instructed to blow his nose gently. The symptoms resolved immediately, and no external permanent scars were left. One year after surgery, the patient’s nasal respiration is normal and he enjoys good general health.
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