Injury Extra 41 (2010) 41–44

Case report

New Eagle’s syndrome variant complicating management of intracranial pressureafter traumatic brain injury

Brian Callahan a, Jennifer Kang a, Anwar Dudekula a, Vincent Eusterman b, Craig H. Rabb a,c,*a Department of Neurosurgery, Denver Health Medical Center, University of Colorado School of Medicine, Denver, CO, USAb Department of Otolaryngology, Denver Health Medical Center, University of Colorado School of Medicine, Denver, CO, USAc Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA

A R T I C L E I N F O

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1. Introduction

Eagle’s syndrome is a rare condition consisting of throat pain,pharyngeal foreign body sensation, otalgia, and neck pain.4 It is dueto abnormally long styloid processes or by progressive miner-alisation of Reichert’s cartilage in the stylohyoid ligamentouscomplex.4 The styloid process may also compress the internal orexternal carotid arteries and the perivascular sympathetic fiberscausing neck and face pain or syncope with head rotation.1,2,4,6 Wereport a case of factitiously increased intracranial pressure aftertraumatic brain injury due to unilateral traumatic sigmoid sinusocclusion in combination with contralateral jugular venouscompression by an elongated and posteriorly positioned styloidprocess.

2. Case report

This 47-year-old male was found down unresponsive in hishome. At presentation, the patient’s Glasgow Coma Scale score was7. A CT scan revealed bilateral temporal contusions, left greaterthan right, a large left frontal contusion, right sided temporal bonefracture, and 4 mm of midline shift (Fig. 1a). The basal cisternswere visible. A fiberoptic intracranial pressure (ICP) monitor wasplaced, with an opening pressure of 35 mmHg.

Over the next 24 h, the intracranial pressure became increas-ingly resistant to sedation, paralytics, mannitol and pentobarbital.His pupils remained reactive. He was taken to surgery and a partialleft frontal lobectomy with debridement of necrotic tissue wasperformed. An external ventricular drain was also placed.Immediately postoperative, the ICP was normal, but promptly

Article history:

Accepted 20 January 2010

* Corresponding author at: Denver Health Medical Center/University of Colorado

School of Medicine, 777 Bannock, MC-0206, Denver, CO 80204-4507, USA.

Tel.: +1 303 436 6558; fax: +1 303 436 6572.

E-mail addresses: Craig.Rabb@dhha.org, craig-rabb@ouhsc.edu (C.H. Rabb).

1572-3461 � 2010 Elsevier Ltd.

doi:10.1016/j.injury.2010.01.109Open access under the Elsevier OA license.

began to rise. Repeat imaging demonstrated significant radio-graphic improvement (Fig. 1b). His pupils remained small andreactive. These elevations in ICP were managed with sedation,paralytics, mannitol, and hypertonic saline. Despite thesemaneuvers, he continued to have unprovoked ICP elevations intothe 50s. Follow up CT scans continued to show open basilarcisterns and decreased cerebral oedema. The patient waspharmacologically paralysed, and his pupils did not dilate withthe spikes of ICP.

During the postoperative course, a CT angiogram (CTA) wasperformed as part of a standardised institutional protocol toscreen all patients with basilar skull fractures for blunt carotidand vertebral arterial injuries. This demonstrated the presence ofright sigmoid thrombosis underlying the mastoid fracture, whichwas thought to be contributing to his ongoing elevated ICP(Fig. 2a).

Five days later, a subsequent review of the CTA noted thepresence of the elongated styloid processes (4 cm), withassociated compression of the residual patent jugular veinbetween the styloid and the lateral mass of C1 (Fig. 2b). Toconfirm this finding, a CT venogram was performed, whichdemonstrated bilateral venous compression was confirmed(Fig. 2c and d). This also demonstrated some early recanalisationof the occluded sigmoid sinus, as well as significant venousoutflow via the vertebral venous plexus (Fig. 2e). At this time, abrain tissue oxygen monitor (Licox, Integra Neuroscience,Plainsboro, New Jersey) was placed to help guide treatment.The patient’s PbO2 was found to be normal, even when there werespikes in the ICP. We felt this provided reassurance that the ICPfigures were factitious, and not requiring any further aggressivetherapy. Concurrently, the elevated intracranial pressures alsobegan to diminish, and he was weaned off the sedation andparalytics. Over the next few days, these monitors were removed,and he began following simple commands. Heparin was theninstituted to further facilitate dissolution of the sigmoidthrombus. He was then transferred to an inpatient rehab center,awake and alert, following commands in all his extremities, andstarting to speak simple phrases. During his inpatient rehabilita-tion stay, it was also discovered that the patient had sarcoidosis.Three months after discharge, the patient’s Glasgow OutcomeScore was 4.

Fig. 1. (a) Preoperative CT Left frontal haemorrhagic contusion with mass effect and midline shift. (b) Postoperative CT demonstrating improvement in mass effect and

normalisation of midline shift.

B. Callahan et al. / Injury Extra 41 (2010) 41–4442

3. Discussion

The styloid process is an elongated protrusion of the temporalbone.10 It arises from the second brachial arch (Reichert’scartilage).14 It is near the carotid artery, the internal jugular vein,and cranial nerves VII, IX, X, and XII.10 Eagle’s syndrome consists ofsymptoms of recurrent throat pain, pharyngeal foreign bodysensation, dysphagia, referred otalgia, and neck pain.4 In 1937,Eagle defined stylalgia related to an elongated styloid process,defining an elongated styloid process as >25 mm.4,10 Eagledescribed 2 separate syndromes associated with an elongatedstyloid process: classic stylohyoid syndrome and stylocarotidsyndrome.4,14 The classic syndrome occurs almost always aftertonsillectomy, involving dull pain in the tonsillar fossa thatradiates to the ear.4 It is also associated with facial-cervical pain.4

The stylocarotid syndrome is due to compression of the internaland external carotid arteries and the associated sympathetic nervefibers.10 Symptoms are exacerbated by turning the head to theextreme lateral position. If the ECA is compressed, pain is in theinfraorbital, temporal and mastoid regions, and if the ICA iscompressed the pain is in the ophthalmic area.4

Pressure on the ICA resulting from elongated styloids can alsointerrupt blood flow and cause symptoms of brain ischaemia, suchas aphasias, visual disturbances, weakness and syncope.1,2,6 Therehas even been a description of carotid dissection secondary to anelongated styloid process.17 Avitia et al. described an airline pilotwho had syncopal episodes when turning his head to the extremeright.1 He was later found to have an elongated styloid. Morerecently, Chuang et al. reported a type of Eagle’s syndrome inwhich a 77-year-old male developed left hemispheric ischaemicsymptoms when turning his head to the left and down.6 CT of theneck showed a calcified left stylohyoid ligament. More compelling,however, was the CT angiogram of the neck done with the patient’shead in the down and left position. The authors demonstrated theleft styloid process compressing the ICA and absence of flow. Ittherefore seems appropriate to consider any symptomatic condi-tion resulting from elongated styloid processes to be an Eagle’ssyndrome variant.

Venous outflow obstruction in the form of dural sinusthrombosis should be investigated in cases in which intracranialpressures appear out of proportion to the radiographic findings. Ingeneral, dural sinus thrombosis has rarely been associated withclosed head injury. However, there are several series thatdocument its occurrence.3,5,7–9,12,13,15,16 Satch et al. in 1993presented two cases where the presenting sign was increasedintracranial pressure.11 The mechanism of dural sinus thrombosisafter closed injury is not known. It may be caused by bonefragments, sinus dissection, or sinus distortion that may initiatethrombus formation. Treatment may be complicated, as it was inour patient, by haemorrhagic injuries to the brain. Thus, the risksand benefits of anticoagulation must be carefully balanced.

In our patient, it appears that the compression of his internaljugular veins complicated the measurement and management ofhis intracranial pressure. Despite the improved appearance of thepostoperative CT scans, his measured ICP continued to besignificantly elevated and resistant to medical management. Webelieve that this was due to the obstruction of venous outflow fromthe combination of the sigmoid sinus occlusion and the bilateraljugular vein compression by elongated styloid processes. It is ouropinion that this patient likely lived in a compensated state prior tohis injury as a result of venous collateral drainage. Loss of onejugular vein as a result of the sigmoid thrombosis likely threw thepatient into an uncompensated state, leading to the factitiouslyelevated ICP. Moreover, we feel that aggressive treatment ofelevated ICP in the presence of venous compression is likelyunwarranted, absent other causes that would normally requireeither medical or surgical intervention, or absent obvioussymptoms. In this case, such a cause (contusion with shift) wastreated, and ICP would likely have been normal had there not beenvenous outflow restriction.

It is interesting to note that the etiology of our patient’s fall isunknown. He was not alone in the house at the time he was found,yet was found unconscious and lying in a pool of emesis. There isno history suggestive of any known trauma prior to this. We havehypothesised that his styloid processes may have compressed oneof his internal carotid arteries when he turned his head. This may

Fig. 2. (a) Axial CTA with right sigmoid sinus occlusion (black arrow) and dye present in the left sigmoid sinus (white arrow). (b) CT venogram confirming the left internal

jugular vein compressed between the styloid process (black arrow) and lateral mass of C1 (white arrow). (c) 3D CTA demonstrating the left internal jugular vein compressed

between the styloid process (black arrow) and lateral mass of C1 (white arrow). (d) Left internal jugular vein, both compressed between the styloid and C1 lateral mass. (e)

Sagittal view of subsequent CTA demonstrating compression of the right internal jugular vein, but some early recanalisation.

B. Callahan et al. / Injury Extra 41 (2010) 41–44 43

B. Callahan et al. / Injury Extra 41 (2010) 41–4444

have lead to a brief episode of ischaemia, and then syncope. Fig. 2cdemonstrates the proximity of the styloid to the carotid artery, inaddition to the jugular compression. In questioning the patient’sfamily, he had at least one prior episode of unexplained loss ofconsciousness. Thus, this variant of Eagle’s syndrome may explainnot only his unusual course, but also may explain the cause of thefall, as well. We doubt that his diagnosis of sarcoidosis had anyinfluence on his clinical course at this time, as there was noevidence of neurosarcoidosis present during his hospitalisation.

4. Conclusions

Eagle’s syndrome is a rare condition that usually presents withpain syndromes or vascular insufficiency by elongated styloidprocesses. In our patient, this anatomical feature complicatedmeasurement and management of his intracranial pressure, bypresumably contributing to his elevated venous pressure. Webelieve that this demonstration of venous compression constitutes anew variant of Eagle’s syndrome. We conclude that, when managingpatients with unexplained ICP elevations, one should attempt tocorrelate the CT findings with the clinical picture and vigorouslysearch for various causes of venous outflow disturbance in cases inwhich the measured ICP appears out of proportion to the imagingfindings. This variant of Eagle’s syndrome may represent a rare causeof such venous insufficiency that should be in the differentialdiagnosis of unexplained or disproportionately elevated ICP.

References

1. Avitia S, Hamilton J, Osborne RF. Styloid-carotid artery syndrome. Ear NoseThroat J 2006;85:228–9.

2. Bafaqeeh SA. Eagle syndrome: classic and carotid artery types. J Otolaryngol2000;29:88–94.

3. Barbati G, Dalla MG, Coletta R, Blasetti AG. Post-traumatic superior sagittalsinus thrombosis. Case report and analysis of the international literature.Minerva Anestesiol 2003;69:919–25.

4. Beder E, Ozgursoy OB, Karatayli OS. Current diagnosis and transoral surgicaltreatment of Eagle’s syndrome. J Oral Maxillofac Surg 2005;63:1742–5.

5. Carrie AW, Jaffe FA. Thrombosis of superior sagittal sinus caused by traumawithout penetrating injury. J Neurosurg 1954;11:173–82.

6. Chuang WC, Short JH, McKinney AM, Anker L, Knoll B, McKinney ZJ. Reversibleleft hemispheric ischemia secondary to carotid compression in Eagle syndrome:surgical and CT angiographic correlation. AJNR Am J Neuroradiol 2007;28:143–5.

7. Crimmins TJ, Rockswold GL, Yock Jr DH. Progressive posttraumatic superiorsagittal sinus thrombosis complicated by pulmonary embolism. Case report. JNeurosurg 1984;60:179–82.

8. D’Alise MD, Fichtel F, Horowitz M. Sagittal sinus thrombosis following minorhead injury treated with continuous urokinase infusion. Surg Neurol1998;49:430–5.

9. Ferrera PC, Pauze DR, Chan L. Sagittal sinus thrombosis after closed head injury.Am J Emerg Med 1998;16:382–5.

10. Fini G, Gasparini G, Filippini F, Becelli R, Marcotullio D. The long styloid processsyndrome or Eagle’s syndrome. J Craniomaxillofac Surg 2000;28:123–7.

11. Fuentes S, Metellus P, Levrier O, Adetchessi T, Dufour H, Grisoli F. Depressedskull fracture overlying the superior sagittal sinus causing benign intracranialhypertension. Description of two cases and review of the literature. Br JNeurosurg 2005;19:438–42.

12. Hesselbrock R, Sawaya R, Tomsick T, Wadhwa S. Superior sagittal sinus throm-bosis after closed head injury. Neurosurgery 1985;16:825–8.

13. Kuether TA, O’Neill O, Nesbit GM, Barnwell SL. Endovascular treatment oftraumatic dural sinus thrombosis: case report. Neurosurgery 1998;42:1163–6.

14. Mendelsohn AH, Berke GS, Chhetri DK. Heterogeneity in the clinical presenta-tion of Eagle’s syndrome. Otolaryngol Head Neck Surg 2006;134:389–93.

15. Ochagavia AR, Boque MC, Torre C, Alonso S, Sirvent JJ. Dural venous sinusthrombosis due to cranial trauma. Lancet 1996;347:1564.

16. Stringer WL, Peerless SJ. Superior sagittal sinus thrombosis after closed headinjury. Neurosurgery 1983;12:95–7.

17. Zuber M, Meder JF, Mas JL. Carotid artery dissection due to elongated styloidprocess. Neurology 1999;53:1886–7.