j. Cranio-Max.-Fac. Surg, 16 (1988) J.Cranio-Max.-Fac. Surg. 16 (1988) 385-388 © Georg Thieme Verlag Stuttgart • New York
Traumatic Carotid-Cavernous Sinus Fistula Nicholas Zachariades, Demetrius Papavassiliou Oral and Maxillo-FacialDepartment(Head:Dr. N. Zachariades,M. D., D. D. S., Dr. Dent.), PeripheralGeneralHospitalof Attica-K.A. T. (formerly: "ApostlePaul's"AccidentsHospital),Kifissia,Athens, Greece Submitted 18.12.87; accepted 19.2.88
Introduction Traumatic carotid-cavernous sinus fistula is a very rarely encountered complication of cranial or maxillofacial trauma (Roger and Epker, 1977; Kuroi et al., 1987) and is often followed by very serious complications. Occasionally it occurs spontaneously (West, 1980) with 12-15 % of the cases being bilateral (Garland et al., 1977). We present such a case, victim of a motorcycle accident, with multiple maxillofacial injuries and fracture of the sella turcica. Case Report On March 23, 1985, a 23-year-old man was brought to the Casualty Department of our hospital following a motorcycle accident that had occurred when he collided with a wall while riding at low speed and at the same time trying to adjust his helmet. He was comatose, with unequal pupils and would only respond to painful stimuli. He was bleeding from the nose and blood was coming out of his ears. He was immediately intubated and anterior nasal packs were inserted. Radiographic examination revealed a fracture of the sella turcica (Fig. 1), a Le Fort III fracture, and fractures of the right mandibular angle and the left parasymphyseal area. A right carotid angiogram was also performed which was within normal limits. Review of systems and laboratory findings were also within normal limits. The patient was subsequently taken to the Intensive Care Unit and a few hours later a CT scan was performed which showed an epidural haematoma in the right frontal area, bilateral haemorrhagic contusion without deviation of the midline and air in the right lateral ventricle. The Neurosurgical Service decided that no immediate treatment was necessary. The patient's condition indeed improved; by the 6th day the pupils were equal; by the 7th he responded to simple commands. He was extubated on the 9th day while diabetes insipidus, which had previously been noted, gradually disappeared by the 15th day. After 20 days in the Intensive Care Unit he was transferred to the Neurosurgical Ward. By that time the patient was still confused but had decidedly improved. There was ptosis of the right upper lid and there was no light reflex in the right eye; the eye movements on that side were deft-
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Summary Traumatic carotid-cavernous sinus fistula is an uncommon situation resulting from severe trauma, with less than 40 cases having been reported. It is usually associated with a skull base, frontal or mid-facial fracture, but it may also be a spontaneous phenomenon of congenital, infective or degenerative origin. The blood shunts from the internal carotid to the cavernous sinus resulting in pulsating exophthalmos, orbital headache, pain, orbital or frontal bruit, loss of visual acuity, diplopia, ophthalmoplegia and the differential diagnosis should include superior orbital fissure syndrome, orbital apex syndrome and cavernous sinus thrombosis. Several modes of treatment have been proposed. We were recently faced with such a case, who had sustained fractures of the facial skeleton and developed the fistula despite an initial non-contributory angiogram. The patient remained in a permanent coma. Key words Internal carotid - Cavernous sinus fistula - Maxillofacial trauma - Fracture of sella turcica - Exophthalmos - Ophthalmoplegia - Diabetes insipidus - Haemorrhage
nitely restricted and as there was strong evidence that there was also hypo-aesthesia of the right frontal area, a superior orbital fissure syndrome was diagnosed. At that time he was transferred to the Oral and Maxillofacial Clinic. He was operated upon a week later under general anaesthesia. A tracheostomy was performed, arch bars were applied and he was placed in intermaxillary fixation. Subsequently, intraosseous wires were placed in the mandibular fractures and the right frontozygomatico-maxillary suture. Bilateral Roger-Anderson pins were introduced to stabilize the zygomatico-maxillary complexes in relation to the frontal bones. The anterior nasal packs were inserted again, as the patient bled. He tolerated the proce-
Fig. 1
Fracture of the sella turcica.
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Fig. 2 After removai of the pins, the maxillary antra (particulary the right one) are seen to be cloudy. The arch bars and some of the intraosseous wires can also be seen.
Fig. 3 Arrows point to the carotid artery (lower arrow) and the cavernous sinus (upper arrow).
dure well and was taken back to the ward in good condition. Two days later he regained full consciousness. However, his postoperative course was complicated, as his temperature reached 39 ° and remained at that level until the 7th postoperative day, when pus drained from around right and left lower pins and a sinus draining pus was noticed in the right upper mucobuccal fold. The cultures proved negative and as he did not respond to antibiotics, the pins had to be removed (Fig. 2). He was scheduled for surgical exploration of the right maxillary antrum, but the night before, rigidity of the neck was noticed, with his temperature reaching 40 ° . Lumbar puncture revealed 700cells/mm 3 of polymorphonuclear type, 3 9 5 m g % albumin, 5 0 r a g % sugar and, upon culture, meningococcus, pneumococcus and haemophilus. The diagnosis of meningitis was thus established and he was placed on penicillin and chloramphenicol. The surgical exploration was postponed, while the meningitis showed clear signs of improvement within 72 hours. However, sudden bleeding from the mouth, nose and trachea occurred for which he received four units of blood while bilateral anterior and posterior nasal packs were inserted. He was transferred to the Intensive Care Unit again. A C T scan, taken a few hours later, showed an intracranial haematoma of the anterior cranial fossa, particularly localized in the right frontal lobe. Collections of pus were noted in both the frontal and maxillary antra bilaterally. As the patient continued to improve clinically, the Neurosurgical Service decided not to intervene, but 48 hours later, while the patient was recovering and had stabilized, a new, cataclysmic haemorrhage Occurred from the nose, mouth and trachea. He lost consciousness, stopped breathing and cardiac arrest was diagnosed. An emergency thoracotomy was immediately performed in his bed by a thoracic surgeon, who happened to be in the Intensive Care
Unit at the time, and direct cardiac massage was successfully performed. He received fourteen units of blood, most of which was not cross-matched. At the same time the nasal packs were replaced and the mouth was packed open with gauze. However, he did not regain consciousness. Carotid angiography was performed a little later, which showed a right carotid-cavernous sinus fistula (Fig. 3). Following an electroencephalogram an almost isoelectric line was seen and the diagnosis of terminal (depass~) coma was made. A decision was then taken by the Neurosurgical Service to ligate the right common carotid, which was done five days later. The remaining eighteen months were spent in the Neurosurgical Service, where a shunt was made to drain the posttraumatic hydrocephalus. The operation had to be repeated on a number of occasions as it did not function properly. Various CT scans showed subdural hygromas (Fig. 4), which were drained via bur holes. Currently the patient, still in coma, is at home. Discussion and Conclusions
Carotid-cavernous sinus fistula is a very uncommon complication of cranial or facial trauma, with 32 patients having been reported by 1980 (Roger and Epker, 1972; Garland et al., 1977; West, 1980) and sporadic cases since then (Kuroi et al., 1987; Labb8 et al., 1987; Lanigan and Tubman, 1987), not exceeding 40 in total, mostly published in neurosurgical journals. Seventy-two to 77 % of the cases are considered to be traumatic in origin with males outnumbering females in a ratio of 2 : 1 . They are usually associated with skull base, frontal or mid-facial fracture; the intracavernous portion of the arterial wall, surrounded by an irregular plexus of venous channels, is torn or lacerated by a bony spicule or by shearing forces (to which it is susceptible as it is relatively fixed to the carotid canal posteriorly and by the dura anteriorly), its vasa
Traumatic Carotid-Cavernous Sinus Fistula
Fig.4
Arrows point to the areas of subdurat hygromas.
Venus plexus Oculomotor n. Internal carotid Trochlear n. Abducens n. Ophthalmic n. Maxillary n.
Fig.5
The cavernous sinus and its contents,
vasorum may be damaged thus producing a dissecting aneurysm and eventually future into the cavernous sinus ensues (Esposito, 1970; Leopard, 1985). Not uncommonly, there may be no evidence of cranial or maxillofacial injury as a small number of cases are spontaneous (with 12-15 % being bilateral) resulting from a congenitally, infectively or degeneratively weakened arterial wall. They may also appear in pregnancy (Roger and Epker, 1972; Garland et al., 1977; West, 1980; Leopard, 1985; Lanigan and Tubman, 1987). The fistula is established within a few hours to several months, with the blood shunting from the high-pressure lumen of the internal carotid to the low-pressure cavernous sinus. The rise in pressure in this sinus will result in distortion of the venous channels communicating with the sinus and increase in the mean pressure in the orbital veins caused by the retrograde flow of blood from the cavernous sinus. As a result the following signs may be noted: 1. Pulsating exophthahnos with orbital headache and pain that may be felt in the affected temporal and parietal areas, subjective or objective orbital or frontal bruit, syn-
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chronous with the pulse (which is pathognomonic), dilation of superficial veins of the eyelids and forehead, chemosis, pulsating retinal veins, papilloedema, loss of visual acuity due to impaired retinal circulation and possibly, late optic atrophy. 2. Diplopia is the most frequent symptom and is secondary to compression of the oculomotor nerve in the cavernous sinus. The Vlth nerve is the only one free within the sinus and in close relationship to the carotid artery and its involvement will result in limitation of lateral gaze. The IIIrd and IVth nerves may also be affected resuiting in ophthalmoplegia. The ophthalmic and maxillary branches of the Vth nerve, when affected, will result in sensory disturbances, while the VIIth and VIIIth cranial nerves will be involved as the haematoma increases or the intraorbital pressure rises. 3. Loss of sympathetic supply to Miiller's muscle or disruption of the innervation of the levator palpebrae will result in ptosis. 4. Disruption of the parasympathetic innervation may result in a fixed, dilated pupil. The latter, associated with ptosis and anhydrosis, will confirm the presence of Hornet's syndrome (Esposito, 1970; Roger and Epker, 1972; West, 1980; Labb~ et al., 1987; [Fig. 5]). The misleading complexity of signs and symptoms may render the diagnosis difficult. This should be established by cerebral angiography when diffusion of radiopaque material into the cavernous sinus during the filling phase will be noted. The differential diagnosis should include the superior orbital fissure syndrome, orbital apex syndrome, orbital (retrobulbar) haematoma and cavernous sinus thrombosis; the latter is often associated with a recent history of facial or dental infection, while carotid compression will suppress the noise (Roger and Epker, 1972; Garland et al., 1977; Zachariades, 1982; Zachariades et al., 1985, 1987). Although a small percentage of carotid-cavernous sinus fistulae may close spontaneously, the possibility of blindness, significant neurological deficits and fatal epistaxis render the prognosis serious. Treatment consists of common or internal carotid ligation, clipping or clamping, insertion of coils and balloon catheters, infusion of sclerosing liquids, irradiation and embolization of the artery or packing the cavernous sinus with various materials such as gelfoam (alone or admixed with gauze), oxidized cellulose etc. Occasionally, spontaneous thrombosis may take place (Esposito, 1970; Roger and Epker, 1972; Kuroi et al., 1987; Labb~ et al., 1987). A few points of interest should be underlined in this unfortunate case. One is the fact that the original arteriogram, taken on admission, was not indicative of carotidcavernous sinus fistula. A most likely explanation is that it had not been established at that time; and it was the manipulation of fractures that initiated the process either by a shearing force or a bony spicule that injured the arterial wall or by opening up an already established but well-protected fistula. The clinical signs, in the absence of the pathognomonic orbital and frontal bruit, the chemosis and the pulsating exophthalmos associated with the negative arteriograms directed the diagnosis towards the superior orbital fissure syndrome. It is possible that the frequent nasal bleedings averted the establishment of definite clinical signs of the fistula, as the increase in pressure was released through the nose. It is also possible that the fistula was not primarily traumatic but the result rather of meningitis as an infective process in the area and its result up-
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on the arterial wall, as already postulated. Of course, meningitis in this case is also the sequel to trauma and in this sense the fistula is also traumatic in a broader sense, although exceptionally rare in its pathogenesis. Last, but not least, fracture of the sella turcica is a highly u n c o m m o n incident in itself. Its association with diabetes insipidus (Zachariades et al., 1982) is not unexpected; however, the sella turcica is so well protected that it takes a very strong force to result in its fracture, a force which is n o t usually associated with survival and which, in the present case, was not to be expected.
References
Esposito, J.G.: Cavernous sinus-carotid fistula: a complication of maxillofacial injury: report of case. J. Oral Surg. 28 (1970) 537 Garland, S.D., D.L. Maloney, H.C. Doku: Carotid-cavernous sinus fistula after trauma to the head. J. Oral Surg. 35 (1977) 832 Kuroi, M., M. Nagai, T. Shimizu: Traumatic carotid-cavernous fistula associated with a mandibular fracture. J. Oral Maxillofac. Surg. 45 (1987) 526 Labbb, D., P. Courtheoux, M. Rigot-Jolivet, J.-F. Compere, J. Theron: Fistule durale carotido-caverneuse bilaterale. Rev. Stomatol. Chir. maxillofac. 88 (1987) 120
Lanigan, D.T., D.E. Tubman: Carotid-cavernous sinus fistula following LeFortI osteotomy. J. Oral Maxillofac. Surg. 45 (1987) 969 Leopard, P.: Carotid-cavernous fistula. In: Rowe, N. and Williams J. (Eds.): Maxillofacial Injuries, 1985, Churchill Livingstone, p. 759 Roger, R., B.N. Epker: Carotid-cavernous sinus fistula accompanying mid-facial fractures: report of a case. J. Oral Surg. 30 (1972) 432 West, C. G.: Bilateral carotid-cavernous fistulae: a review. Surg. Neurol. 13 (1980) 85 Zachariades, N.: The superior orbital fissure syndrome. Review of the literature and report of a case. Oral Surg. 53 (1982) 237 Zachariades, N., J. Papademetriou, D. Papavassiliou, J. Koundouris: Diabetes insipidus associated with facial fractures. Report of a case. Oral Surg. 53 (1982) 141 Zachariades, N., E. Vairaktaris, D. Papavassiliou, J. Papademetriou, M. Mezitis, D. Triantafyllou: The superior orbital fissure syndrome. J. Max.-Fac. Surg. 13 (1985) 125 Zachariades, N., E. Vairaktaris, D. PapavassiIiou, D. Triantafyllou, D. Mezitis: Orbital apex syndrome. Int. J. Oral Maxillofac. Surg. 16 (1987) 352
Dr. N. Zachariades, M. D., D. M. D. 40 Papadiamantopoulou St. 157 71 Athens Greece