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LETTERS TO EDITOR
Year : 2022  |  Volume : 23  |  Issue : 1  |  Page : 77-79
 

Caught between the devil and deep sea: Anesthetic management of a patient with hereditary cerebellar ataxia and iatrogenic median and ulnar entrapment neuropathy posted for fixation of both bone forearm


1 Department of Anaesthesiology, Sree Balaji Medical College and Hospital, Chennai, Tamilnadu, India
2 Department of Microbiology, Sree Balaji Medical College and Hospital, Chennai, Tamilnadu, India

Date of Submission21-Dec-2021
Date of Decision25-Jan-2022
Date of Acceptance26-Jan-2022
Date of Web Publication23-Mar-2022

Correspondence Address:
Prof. Vinod Krishnagopal
Sree Balaji Medical College and Hospital, Chrompet, Chennai - 600 044, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/TheIAForum.TheIAForum_168_21

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How to cite this article:
Krishnagopal V, Murugan R, Priya A S, Krishnakumar S. Caught between the devil and deep sea: Anesthetic management of a patient with hereditary cerebellar ataxia and iatrogenic median and ulnar entrapment neuropathy posted for fixation of both bone forearm. Indian Anaesth Forum 2022;23:77-9

How to cite this URL:
Krishnagopal V, Murugan R, Priya A S, Krishnakumar S. Caught between the devil and deep sea: Anesthetic management of a patient with hereditary cerebellar ataxia and iatrogenic median and ulnar entrapment neuropathy posted for fixation of both bone forearm. Indian Anaesth Forum [serial online] 2022 [cited 2022 Oct 3];23:77-9. Available from: http://www.theiaforum.org/text.asp?2022/23/1/77/340479




Sir,

Hereditary cerebellar ataxias (HCA) are genetically inherited disorders characterized by atrophy of the cerebellum which results in gradual worsening of coordination, movement, and speech. In addition, these patients may suffer from ophthalmoplegia, spasticity, neuropathy, cognitive impairment, and parkinsonism.[1] Regional anesthesia (RA) may edge over general anesthesia (GA) in patients with HCA as a technique of choice as delayed recovery following GA may predispose the patient to the risk of aspiration and ventilatory depression. On the contrary presence of entrapment neuropathy raises the question of safety of RA as it can worsen the pre-existing neuropathy due to double crush phenomenon.[2] We would like to highlight the successful anesthetic management of a patient with HCA with entrapment neuropathy scheduled for both bone forearm fracture fixation.

A 38-year-old woman with HCA, mitral valve prolapse, hypothyroidism, and obesity (body mass index 43.28) presented with both bone forearm fracture (right). The patient was treated initially by a traditional bone setter by cast application following which she developed right-sided median and ulnar nerve palsy. Nerve conduction study (NCS) revealed demyelinating axonal neuropathy [Figure 1]. Ulnar nerve palsy resulted in hypothenar muscle wasting [Figure 2]. Ultrasound study of the forearm revealed increase in cross-sectional area of the median (20.91 vs. 10.20 mm2) and ulnar nerve (13.73 vs. 10.69 mm2) on the affected side. Central nervous system examination revealed Mini-mental state score of 24/30, nystagmus, increased muscle tone, exaggerated reflexes, bilateral cerebellar signs positive for ataxia and past pointing. Magnetic resonance imaging brain revealed diffuse cerebellar atrophy. The patient was taken up for surgery under Ultrasound guided (USG) guided supraclavicular brachial plexus block with 7 ml of 2% lignocaine and 8 ml of 0.5% ropivacaine. The procedure lasted for 3 h. The intra-operative and postoperative periods were uneventful and the patient complained of pain after 8 h. The patient regained the baseline motor and sensory function and was discharged after 3 days and there was no progression of the neurological symptoms.
Figure 1: Nerve conduction study of both upper limbs

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Figure 2: Hypothenar muscle wasting following ulnar nerve palsy

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GA has been administered to patients with HCA successfully in the past but one has to be mindful of delayed recovery, unpredictable response to anesthetic agents, risk of atrioventricular blocks, and postoperative delirium.[3] The challenges faced in employing a regional block in these patients would be fear of worsening the pre-existing injury, lack of substantiating evidence or guidelines favoring safety of regional block, and the possibility of facing a medico-legal suit. Despite above mentioned challenges, traditionally local anesthetics with steroid injections have been employed successfully in alleviating symptoms in patients with entrapment neuropathies.[4] HCA can present with neuropathy, but in our case, the median and ulnar neuropathy were due to entrapment of nerves following cast application by traditional bone setters which was apparent from the NCS and ultrasound measurement of nerve diameter. The post-operative nerve injuries can be caused by blunt trauma, drug neurotoxicity, compressive injury, stretch injury, and nerve ischemia.[5] We incorporated ultrasound guidance, avoided tourniquet, lesser volume of plain local anesthetics, and optimal limb placement to negate the aforementioned factors.

We feel that supraclavicular brachial plexus block can be used as a safe alternative to GA in patients with HCA and ipsilateral entrapment neuropathy without the fear of worsening the preexisting neuropathy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Jayadev S, Bird TD. Hereditary ataxias: Overview. Genet Med 2013;15:673-83.  Back to cited text no. 1
    
2.
Upton AR, McComas AJ. The double crush in nerve entrapment syndromes. Lancet 1973;2:359-62.  Back to cited text no. 2
    
3.
Levantesi L, De Cosmo G, Logroscino G, Saracco M. Recurrent postoperative delirium in spinocerebellar ataxia type 2: A case report. J Med Case Rep 2019;13:112.  Back to cited text no. 3
    
4.
Gronbeck C, Wolf J, Rodner CM. Ultrasound-guided cubital tunnel injection: A review and exploration of utility as a diagnostic aid in mild or nonclassic cubital tunnel patients. Tech Orthop 2021;36:301-6.  Back to cited text no. 4
    
5.
Sorenson EJ. Neurological injuries associated with regional anesthesia. Reg Anesth Pain Med 2008;33:442-8.  Back to cited text no. 5
    


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