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Abducens nerve palsy and ipsilateral Horner syndrome as the presenting finding of carotid cavernous fistula

Published:April 18, 2019DOI:https://doi.org/10.1016/j.jcjo.2019.02.003

      Case Presentation

      An 84-year-old female with history of hypertension and hyperlipidemia presented with nontraumatic left eyelid edema, erythema, and ptosis followed by acute painless binocular horizontal diplopia. Best corrected visual acuity was 20/20 OD and 20/30 OS. The OD pupil measured 4 mm in the dark and 2 mm in the light, and the OS pupil measured 3 mm in the dark and 2 mm in the light. No relative afferent pupillary defect was detected. There was a 50 prism diopter esotropia in primary gaze and an abduction deficit of –4 OS (Fig. 1). The OD moved normally. Intraocular pressure measured 17 mm Hg OD and 16 mm Hg OS. Anterior segment examination of OD was normal, while OS showed dilated and tortuous deep episcleral vessels with arterialization of vessels. Fundus examination showed no disc edema, and the macula and vessels looked normal. There was no proptosis of the eye or bruit auscultated.
      Fig 1
      Fig. 1Left abducens nerve palsy and ptosis. Esotropia and limitation of abduction in the OS consistent with abducens nerve palsy.
      Cranial magnetic resonance imaging and a standard catheter angiogram confirmed a left Barrow type D carotid cavernous fistula (CCF) supplied principally by the contralateral right external carotid artery (ECA) with involvement of the ipsilateral left internal carotid artery (ICA) intercavernous branches (Fig. 2A and B). There also appeared to be inferior petrosal sinus occlusion and bilateral cortical venous drainage.
      Fig 2
      Fig. 2Cerebral angiography of type D carotid cavernous fistula (CCF) before and after endovascular embolization. A, Right external carotid artery to left CCF with cortical venous drainage before embolization. B, Left internal carotid artery dural branches to the CCF before embolization. C, Occlusion of the CCF after embolization.
      Endovascular embolization via the inferior petrosal sinus was attempted twice, but the microcatheter could not be advanced due to sinus occlusion. Embolization was then attempted via the left superior ophthalmic vein, but the vein was too small to advance the microcatheter. A neurosurgical approach via craniotomy to the sphenoparietal sinus was then considered, but ultimately an ultrasound-guided left internal jugular vein retrograde approach was performed. A guide catheter was placed into the left jugular bulb and directed to the left inferior petrosal sinus and the posterior aspect of the cavernous sinus. Under fluoroscopic control, multiple coils were placed first in the most anterior aspect of the fistula and then in the inferior and posterior aspects of the cavernous sinus, respectively, to disconnect the fistula and the branches of the cortical venous drainage. Liquid Onyx was then instilled for complete obliteration of the CCF (Fig. 2C). The patient's abducens nerve (cranial nerve [CN] VI) palsy with ipsilateral Horner syndrome (Parkinson sign) persisted after the procedure.

      Discussion

      CCF is an abnormal connection between the ICA or ECA (or its branches) and the cavernous sinus (CS) that can be classified based on etiology (e.g., traumatic vs spontaneous), hemodynamics (e.g., high flow vs low flow), and anatomy (e.g., direct vs indirect). The Barrow classification system divides CCFs into 4 types: Type A fistulas are direct shunts between ICA and CS with high velocity blood flow, while types B–D are indirect dural shunts between the meningeal branches of the ICA or ECA and CS with low velocity blood flow.
      • Barrow D.L.
      • Spector R.H.
      • Braun I.F.
      • Landman J.A.
      • Tindall S.C.
      • Tindall G.T.
      Classification and treatment of spontaneous carotid-cavernous sinus fistulas.
      Type B involves ICA branches, type C involves ECA branches, and type D involves both ICA and ECA branches.
      • Barrow D.L.
      • Spector R.H.
      • Braun I.F.
      • Landman J.A.
      • Tindall S.C.
      • Tindall G.T.
      Classification and treatment of spontaneous carotid-cavernous sinus fistulas.
      Traumatic CCFs account for the majority of total CCFs and are almost always type A fistulas, while spontaneous CCFs tend to be types B–D.
      Our patient had a type D CCF, which presented with a CN VI palsy with an ipsilateral Horner syndrome. This association was described by Parkinson in 1979
      • Parkinson D.
      • Bernard M.
      Horner syndrome and others?.
      and localizes to the ipsilateral cavernous sinus. The CS structure contains CN III, CN IV, and the V1, V2 divisions of the trigeminal nerve in its lateral wall, while CN VI is embedded with substance of the sinus itself. In this location, the sympathetic nerve runs a short course close to CN VI on the ICA before it jumps onto the V1 segment of the trigeminal nerve. Although isolated CN VI palsy or Horner syndrome are nonlocalizing, the presence of both is a precise localizing sign that helps in narrowing down the location of the pathology and the diagnostic differentials based on other relevant patient demographics and history.
      • Johnston J.
      • Parkinson D.
      Intracranial sympathetic pathways associated with the sixth cranial nerve.
      The ocular sympathetic nerve plexus in this location is the third order, postganglionic portion of a 3-neuron chain.
      Parkinson sign has been reported with numerous different etiologies, including intracavernous carotid aneurysms,
      • Abad J.M.
      • Alvarez F.
      • Blazquez M.G.
      An unrecognized neurological syndrome: sixth-nerve palsy and Horner's syndrome due to traumatic intracavernous carotid aneurysm.
      • Gutman I.
      • Levartovski S.
      • Goldhammer Y.
      • Tadmor R.
      • Findler G.
      Sixth nerve palsy and unilateral Horner's syndrome.
      • Striph G.G.
      • Burde R.M.
      Abducens nerve palsy and Horner's syndrome revisited.
      metastatic tumours,
      • Gutman I.
      • Levartovski S.
      • Goldhammer Y.
      • Tadmor R.
      • Findler G.
      Sixth nerve palsy and unilateral Horner's syndrome.
      • Tsuda H.
      • Ishikawa H.
      • Asayama K.
      • Saito T.
      • Endo S.
      • Mizutani T.
      Abducens nerve palsy and Horner syndrome due to metastatic tumor in the cavernous sinus.
      • Tsuda H.
      • Ishikawa H.
      • Kishiro M.
      • Koga N.
      • Kashima Y.
      Abducens nerve palsy and postganglionic horner syndrome with or without severe headache.
      meningiomas,
      • Ebner R.N.
      • Ayerza D.R.
      • Aghetoni F.
      Sixth nerve palsy + ipsilateral Horner's Syndrome = Parkinson's Syndrome.
      and posttrauma CCFs.
      • Fujisawa H.
      • Marukawa K.
      • Kida S.
      • Hasegawa M.
      • Yamashita J.
      • Matsui O.
      Abducens nerve palsy and ipsilateral horner syndrome: A predicting sign of intracranial carotid injury in a head trauma patient.
      • Kal A.
      • Ercan Z.E.
      • Duman E.
      • Arpaci E.
      Abducens nerve palsy and ipsilateral horner syndrome in a patient with carotid-cavernous fistula.
      To our knowledge however, this is the first case of Parkinson sign as the presenting finding of a type D CCF in the English language ophthalmic literature.

      Footnotes and Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

      Appendix. Supplementary materials

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