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Year : 2020  |  Volume : 14  |  Issue : 4  |  Page : 551-552

Anesthetic management of a patient with Susac syndrome: A rare neurological disorder

Department of Neuroanaesthesiology and Critical Care, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Dr. Navdeep Sokhal
Department of Neuroanaesthesiology and Critical Care, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/sja.SJA_66_20

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Date of Submission22-Jan-2020
Date of Acceptance26-Jan-2020
Date of Web Publication24-Sep-2020

How to cite this article:
Khandelwal A, Prasad C, Sokhal N, Chaturvedi A. Anesthetic management of a patient with Susac syndrome: A rare neurological disorder. Saudi J Anaesth 2020;14:551-2

How to cite this URL:
Khandelwal A, Prasad C, Sokhal N, Chaturvedi A. Anesthetic management of a patient with Susac syndrome: A rare neurological disorder. Saudi J Anaesth [serial online] 2020 [cited 2021 Mar 4];14:551-2. Available from:


A 35-year-old, 70 kg male presented with a history of bifrontal headache associated with nausea and vomiting, sensorineural hearing loss (L > R), unsteady gait, bilateral partial visual loss, diplopia, and bilateral optical disc edema with peripapillary exudation. The patient had already received intravenous (IV) methylprednisolone, five cycles of plasma exchange therapy, cyclophosphamide, and intratympanic dexamethasone and was now scheduled for duramater and brain biopsy. In the operating room, the patient complained of severe headache and nausea. As such, prior to induction of anesthesia, we examined the optic nerve sheath diameter (ONSD) using ultrasound and found an average of 5.2 mm (right eye) and 5.1 (left eye) suggesting raised intracranial pressure (ICP). The patient had a stable heart rate (80/min), noninvasive blood pressure (130/80 mm Hg) and oxygen saturation (99%). Induction of anesthesia was done with fentanyl (2 mcg/kg) and propofol (2mg/kg). Rocuronium (1 mg/kg) was used for tracheal intubation. After 5 mins of induction, we again performed ONSD which showed approximately 4.5 mm in both eyes. Maintenance of anesthesia was done with oxygen and air (1:1) along with intravenous propofol infusion titrated to maintain BIS of 50–60. Intermittent rocuronium and fentanyl were used for muscle relaxation and pain alleviation, respectively. No volatile anesthetic agent was used. Ventilation was adjusted to maintain PaCO2 between 30–35 mm Hg. Mannitol (0.5mg/kg) was administered at the beginning of minicraniotomy. Around 1100 ml of normal saline (0.9% NS) was used as maintenance fluid during surgery. The intraoperative course was uneventful. Tracheal extubation was done successfully at the end of the surgery. After 15 mins of extubation, we again performed the ONSD which showed 4.3 mm (right eye) and 4.2 mm (left eye).

Susac syndrome (SS) is an autoimmune endotheliopathy, consisting of a clinical triad of encephalopathy with or without focal neurological signs, branch retinal artery occlusions, and hearing loss. It is characterized by micrangiopathy affecting the precapillary arterioles of retina, inner ear, and cerebral parenchyma and, thus, is also known as SICRET (small infarctions of cochlear, retinal, and encephalic tissue) syndrome.[1] The MR scans in SS show a rather distinctive pattern of supratentorial white matter lesions that always involve the corpus callosum. There is often deep gray matter, posterior fossa involvement, and frequent parenchymal with occasional leptomeningeal enhancement.[2]

In our case, both induction and maintenance of anesthesia were done with intravenous propofol known to cause cerebral vasoconstriction and reduced ICP.[3] Nitrous oxide was avoided as it has negative effects on cerebral hemodynamics.[4] We also avoided halogenated volatile anesthetics as they cause cerebral vasodilatation. Utmost importance was given to maintaining adequate depth of anesthesia, skeletal immobilization, and pain alleviation. Mild hyperventilation was done to a level not below PaCO2 of 30 mm Hg. Hypotonic solutions were avoided intraoperatively. In our case, we utilized ONSD as an important noninvasive diagnostic tool for intracranial hypertension. The optic nerve sheath is an anatomical extension of the duramater, and the subarachnoid space around the optic nerve is continuous with the intracranial subarachnoid space. Dilatation of optic nerve sheath has been shown to be a much earlier manifestation of ICP rise.[5]

Thus, SS being an extremely rare neurological disorder, improved understanding of this disease is crucial. Baseline neurological status including fundoscopic and audiometric examination should be documented. A proper history of concomitant drug therapy and its associated interaction with anesthetic drugs should also be kept in mind. In our case, we had administered propofol and instituted mild hyperventilation only to negate the effects of raised ICP; however, it should be kept in mind that in the absence of raised ICP, this anesthetic technique may not be advisable as it can worsen cerebral-retinal-cochlear perfusion in an already existing microangiopathic state. Volatile anesthetics with normocapnia would be desirable in the absence of raised ICP. Thus, anesthetic technique in SS should be modified based on the presence or absence of intracranial hypertension. Since no literature has previously described anesthetic management in SS, we believe that our report brings some insight into the pathophysiology of this disorder and, thus, understanding perioperative anesthetic implications.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Grygiel-Górniak B, Puszczewicz M, Czaplicka E. Susac syndrome-clinical insight and strategies of therapy. Eur Rev Med Pharmacol Sci 2015;19:1729-35.  Back to cited text no. 1
Susac JO, Murtagh FR, Egan RA, Berger JR, Bakshi R, Lincoff N, et al. MRI findings in Susac's syndrome. Neurology 2003;61:1783-7.  Back to cited text no. 2
Petersen KD, Landsfeldt U, Cold GE, Pedersen CB, Mau S, Hauerberg J, et al. ICP is lower during propofol anaesthesia compared to isoflurane and sevoflurane. Acta Neurochir Suppl 2002;81:89-91.  Back to cited text no. 3
Reinstrup P, Ryding E, Algotsson L, Berntman L, Uski T. Effects of nitrous oxide on human regional cerebral blood flow and isolated pial arteries. Anesthesiology 1994;81:396-402.  Back to cited text no. 4
Soldatos T, Karakitsos D, Chatzimichail K, Papathanasiou M, Gouliamos A, Karabinis A. Optic nerve sonography in the diagnostic evaluation of adult brain injury. Crit Care 2008;12:1.  Back to cited text no. 5


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