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LETTER TO EDITOR
Year : 2019  |  Volume : 20  |  Issue : 2  |  Page : 107-109
 

Videolaryngoscopy-assisted intubation in a patient of Pendred syndrome with a pendulous goiter


Department of Anaesthesiology, Heritage Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

Date of Web Publication28-Aug-2019

Correspondence Address:
Dr. Reena
Department of Anaesthesiology, Heritage Institute of Medical Sciences, NH-2, Bhadwar, Varanasi - 221 311, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/TheIAForum.TheIAForum_25_19

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How to cite this article:
Gupta K, Reena, Gangwar P, Agarwal S, Kumar A. Videolaryngoscopy-assisted intubation in a patient of Pendred syndrome with a pendulous goiter. Indian Anaesth Forum 2019;20:107-9

How to cite this URL:
Gupta K, Reena, Gangwar P, Agarwal S, Kumar A. Videolaryngoscopy-assisted intubation in a patient of Pendred syndrome with a pendulous goiter. Indian Anaesth Forum [serial online] 2019 [cited 2019 Sep 22];20:107-9. Available from: http://www.theiaforum.org/text.asp?2019/20/2/107/265644




Sir,

Pendred syndrome is a genetic disorder with bilateral congenital sensorineural deafness, with a progressively enlarging goiter. It was first recognized by Pendred in 1896.[1] Pendred syndrome accounts for about 4%–10% of all cases of hereditary deafness.[2] Thus, it may be the most frequent cause of autosomal recessive syndromic deafness.[3]

We encountered a 62-year-old woman with congenital sensorineural deafness and a huge goiter posted for total thyroidectomy [Figure 1]. The diagnosis of Pendred syndrome was made clinically as genetic mutation study was not feasible. Both computerized tomography scan of the neck and X-ray neck revealed a large colloid goiter, with trachea grossly deviated to the right side with moderate tracheal compression [Figure 2] and [Figure 3]. Awake fiberoptic intubation (FOI) is the safest and most recommended technique to secure airway in patients with difficult airway.[4] Patient's cooperation is, however, necessary in awake FOI, which may be difficult to obtain from a deaf-mute patient. A videolaryngoscopy (VL)-assisted intubation following inhalational induction is a better alternative in such patients. In the preoperative visit, we tried to establish communication with the patient by learning simple sign language used between her and her relatives. With the help of her relatives, she was counseled regarding the mode of anesthesia, perioperative analgesia, and postoperative outcome.
Figure 1: Pendulous goiter

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Figure 2: Neck X-ray showing rightward deviation of the trachea

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Figure 3: Computerized tomography neck

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On the day of surgery, the patient was taken to the operation theater and all standard monitoring was applied. VL was done with King Vision VL after local anesthetics nebulization and sevoflurane induction while maintaining spontaneous respiration. A cuffed 6.5 flexometallic tube could only be negotiated after leftward tracheal manipulation. Rest of the anesthetic management was uneventful. The patient was extubated awake after confirming bilateral vocal cords movements with videolaryngoscope.

The literature search for planning anesthesia in a case of deaf and mute patient results in surprisingly limited data. A thorough preoperative assessment plays an important role to understand the patient's expressing capabilities, educational background, and basic understanding ability. The presence of a family member during the preanesthetic checkup as well as induction of anesthesia can be helpful and has been recommended. Some authors also recommend involvement of a sign language specialist who can train and communicate with the patient.[5] Attention should be paid to the clinical observation and objective assessment for postoperative pain. Early mobilization and allowing a family member to visit and communicate as soon as the patient is stable can be helpful in early recovery in such patients.

VL has been used in difficult airway situations where an awake inspection or intubation is warranted. The American Society of Anesthesiologists has incorporated VL as an adjunct to “Alternative Difficult Intubation Approaches” in their practice guidelines for the management of the difficult airway.[4] Topical application of local anesthetics markedly reduces the gag reflex which, along with mild sedation, helps patients to tolerate VL.

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.
Pendred V. Deaf mutism and goiter. Lancet 1896;2:532.  Back to cited text no. 1
    
2.
Fraser GR. Association of congenital deafness with goitre (Pendred's syndrome) a study of 207 families. Ann Hum Genet 1965;28:201-49.  Back to cited text no. 2
    
3.
Wémeau JL, Kopp P. Pendred syndrome. Best Pract Res Clin Endocrinol Metab 2017;31:213-24.  Back to cited text no. 3
    
4.
Apfelbaum JL, Hagberg CA, Caplan RA, Blitt CD, Connis RT, Nickinovich DG, et al. Practice guidelines for management of the difficult airway: An updated report by the American Society of Anesthesiologists task force on management of the difficult airway. Anesthesiology 2013;118:251-70.  Back to cited text no. 4
    
5.
Bhalotra AR, Kakkar B. Anesthesia for the deaf and mute. Korean J Anesthesiol 2017;70:654-5.  Back to cited text no. 5
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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