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LETTER TO EDITOR
Year : 2018  |  Volume : 34  |  Issue : 3  |  Page : 413-414

Epiglottic cyst in von Hippel-Lindau syndrome: Shared pathology or a separate entity?


Department of Neuroanaesthesiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Date of Web Publication11-Oct-2018

Correspondence Address:
Appavoo Arulvelan
Department of Neuroanaesthesiology, 401, Fourth Floor, Surgical Block, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/joacp.JOACP_360_15

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How to cite this article:
Lionel KR, Arulvelan A, Manikandan S. Epiglottic cyst in von Hippel-Lindau syndrome: Shared pathology or a separate entity?. J Anaesthesiol Clin Pharmacol 2018;34:413-4

How to cite this URL:
Lionel KR, Arulvelan A, Manikandan S. Epiglottic cyst in von Hippel-Lindau syndrome: Shared pathology or a separate entity?. J Anaesthesiol Clin Pharmacol [serial online] 2018 [cited 2018 Dec 19];34:413-4. Available from: http://www.joacp.org/text.asp?2018/34/3/413/243172



Madam,

Von Hippel-Lindau (VHL) syndrome is a genetic disorder characterized by hemangioblastomas involving central nervous system and cysts in the liver, kidney, and pancreas.[1] We encountered a cyst in the upper airway of a patient with VHL syndrome that led to difficulty in airway management.

A 43-year-old man with VHL syndrome was planned for excision of a recurrent cerebellar hemangioblastoma. Ultrasound of the abdomen revealed multiple cysts involving pancreas and kidneys. He gave no history of voice change, snoring, difficulty in swallowing or breathing. He had limited neck movements, which we attributed to previous two midline suboccipital craniotomies. Previous anesthesia records revealed no difficulty in airway management. The patient was induced with intravenous propofol, fentanyl, and vecuronium. Laryngeal structures could not be visualized during initial intubation attempt with Macintosh size 4 blade. Hence, mask ventilation was continued and intubation attempted with C-Mac Video Laryngoscope (Karl Storz, Tuttlingen, Germany). It revealed a pedunculated, 2.0 cm × 2.0 cm, smooth surfaced lesion in the vallecula obstructing the view of glottis [Figure 1]. With gentle manipulation of the scope, posterior part of the glottis could be visualized. Using a gum-elastic bougie, trachea was intubated avoiding injury to the lesion. Extubation and postoperative period were uneventful.
Figure 1: Video laryngoscopic view of the epiglottic cyst

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The pathogenesis of VHL syndrome involves mutation in chromosome 3p. This leads to abnormalities in tumor suppression, microtubule stability, cilia formation, and assembly of collagen (Type IV) and fibronectin in extracellular matrix, resulting in visceral cysts. Although not described previously, there is theoretical possibility that the pathology can involve airway mucosa as it also consists of various types of epithelial cells, cilia, Type IV collagen, and fibronectin.[2] Asymptomatic airway cysts can cause difficulty in mask ventilation by ball valve mechanism, difficulty in endotracheal intubation by obscuring glottic view, and difficulty in supraglottic airway placement by altering the anatomy of laryngeal inlet.[3] Aggressive airway manipulation can cause rupture, and spillage of its contents bleeding from the cyst can cause aspiration and may even precipitate a “cannot ventilate – cannot intubate” scenario. In such scenarios, the attending anesthesiologist should be prepared to maintain ventilation and oxygenation of the patients. The alternative plans for securing the airway after induction of anesthesia will depend on the degree of difficulty, anesthetic agents used, availability of alternative airway gadgets, and the experience of the anesthesiologist in using them. The epiglottic cyst found in our case could be a casual coexistence or part of the syndrome itself. Preoperative endoscopic airway examination (PEAE) has shown to affect the clinical examination-based airway management plan in patients presented with airway pathology.[4] However, the advantage of routine preoperative use of PEAE in asymptomatic patients is questionable considering the rare incidence of unanticipated difficult airway. Since VHL syndrome is associated with cysts involving various organs such as the pancreas, liver, and kidney, it would be wise on the part of the anesthetist to expect cysts in the airway as well. Obtaining careful history related to airway symptoms and using indirect laryngoscopy or any other endoscopic or fiberoptic technique during pre-anesthesia assessment may help rule out airway pathology and help planning airway management in these patients.

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.
Friedrich CA. Genotype-phenotype correlation in von Hippel-Lindau syndrome. Hum Mol Genet 2001;10:763-7.  Back to cited text no. 1
    
2.
Hoshino M, Nakamura Y, Sim J, Shimojo J, Isogai S. Bronchial subepithelial fibrosis and expression of matrix metalloproteinase-9 in asthmatic airway inflammation. J Allergy Clin Immunol 1998;102:783-8.  Back to cited text no. 2
    
3.
Moorthy SS, Gupta S, Laurent B, Weisberger EC. Management of airway in patients with laryngeal tumors. J Clin Anesth 2005;17:604-9.  Back to cited text no. 3
    
4.
Rosenblatt W, Ianus AI, Sukhupragarn W, Fickenscher A, Sasaki C. Preoperative endoscopic airway examination (PEAE) provides superior airway information and may reduce the use of unnecessary awake intubation. Anesth Analg 2011;112:602-7.  Back to cited text no. 4
    


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