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 Table of Contents  
Year : 2021  |  Volume : 4  |  Issue : 2  |  Page : 121-124

Role of preoperative multidetector computed tomography airway reconstruction in anaesthetic management of mucopolysaccharidosis type IV

1 Department of Anaesthesiology and Critical Care, Seven Hills Hospital, Mumbai, Maharashtra, India
2 Department of Anaesthesiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India

Date of Submission06-Jun-2021
Date of Acceptance24-Jun-2021
Date of Web Publication10-Aug-2021

Correspondence Address:
Dr. Pradeep Tiwari
Department of Anaesthesiology and Critical Care, Seven Hills Hospital, Marol, Andheri East, Mumbai - 400 059, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/arwy.arwy_33_21

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Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders that often present with a difficult airway. The trachea is usually narrowed and flattened, making the choice of correct technique and endotracheal tube (ETT) size crucial. Multidetector computed tomography (MDCT) images have been used to assess the airway of children who are considered at risk for difficult intubation. However, it has not been standardised in preoperative assessment of MPS. We report a case in whom after failed fibreoptic intubation, titrated doses of injection propofol were given and direct laryngoscopy was performed. A 6.0 mm internal diameter (ID) and later a 5.0 mm ID ETT were tried but could not be negotiated. Subsequently, the airway was secured with a 4.5 mm ID ETT. Postoperatively, MDCT airway reconstruction demonstrated that the narrowest part of airway measured 4.3 mm. Anaesthesiologists often face a multitude of challenges in these patients. Our experience gained in retrospect suggests that a detailed preoperative evaluation including MDCT airway reconstruction, followed by meticulous planning, is essential for a safe outcome.

Keywords: Airway reconstruction, difficult airway, mucopolysaccharidoses, multidetector computed tomography

How to cite this article:
Tiwari P, Kotak NM, Shetty AN. Role of preoperative multidetector computed tomography airway reconstruction in anaesthetic management of mucopolysaccharidosis type IV. Airway 2021;4:121-4

How to cite this URL:
Tiwari P, Kotak NM, Shetty AN. Role of preoperative multidetector computed tomography airway reconstruction in anaesthetic management of mucopolysaccharidosis type IV. Airway [serial online] 2021 [cited 2022 Jan 27];4:121-4. Available from: https://www.arwy.org/text.asp?2021/4/2/121/323578

  Introduction Top

Mucopolysaccharidosis type IV, also known as Morquio syndrome, is caused by deficiency of n-acetylgalactosamine-6-sulphate.[1] These patients can have an increased anaesthetic risk secondary to a difficult airway.[2] Multidetector computed tomography (MDCT) image reconstruction has been used to define the central airway and is recommended in assessment of airway in children who are considered at risk for difficult intubation.[3] Anaesthetic management of these patients is challenging and needs extensive planning, in which preoperative MDCT airway reconstruction can play a vital role.[3] We present a case of a 15-year-old male posted for atlantoaxial dislocation surgery in a case of mucopolysaccharidosis type IV.

  Case Report Top

A 15-year-old-male weighing 35 kg presented with difficulty in walking, inability to sit or hold objects in hands. He was mentally retarded, short statured (120 cm) with a short neck and dorsal kyphoscoliosis. Mouth opening was 3.5 cm with a relatively large tongue and base of uvula, Mallampati Class III, neck circumference of 30 cm with restricted neck movements, thyromental distance of 2.5 cm and hyomental distance of 2 cm. Air entry was decreased on the right side, and the cardiovascular system was unremarkable. Power was decreased in all four limbs. Magnetic resonance imaging of the neck depicted shortening of C1 arch with a dystrophic odontoid and severe stenosis of foramen magnum.

Fibreoptic intubation with sevoflurane induction was planned. A difficult airway cart was kept in readiness, and the airway was prepared with 10% lignocaine spray and 2% lignocaine nebulisation and gargle. Injection glycopyrrolate 4 μg/kg was given, and the patient was taken inside the operation theatre. Basic monitoring was established, intravenous access secured and manual-in-line-stabilisation given throughout the procedure. Anaesthesia was induced with sevoflurane in 100% oxygen by facemask and titrated to deepen the anaesthetic while preserving spontaneous respiration. Oxygen saturation and end-tidal carbon dioxide were continuously monitored. After ensuring adequate depth of anaesthesia, fibreoptic intubation with 'spray-as-you-go' technique was attempted, but the vocal cords could not be visualised. Thereafter, titrated dose of injection propofol was given ensuring preservation of spontaneous breathing. Direct laryngoscopy with Macintosh #2 blade revealed Cormack-Lehane Grade 2 view, but tubes of 6.0 and 5.0 mm internal diameter (ID) could not be negotiated. The airway was finally secured with a 4.5 mm ID endotracheal tube (ETT). Dexamethasone 2 mg was given intravenously because of repeated airway manipulation.

Anaesthesia was maintained with 3%–4% desflurane in oxygen-nitrous oxide mixture (50:50) and intermittent boluses of atracurium 0.05 mg/kg. As airway pressures were high because of ventilation through a relatively small tube, the patient was placed on pressure-controlled ventilation. Surgery was uneventful, and the patient was electively ventilated for 24 h and later extubated over a tube exchanger. Retrospectively, MDCT with airway reconstruction illustrated that the narrowest portion of airway was 4.3 mm. The narrowest portion was immediately below the vocal cords in the subglottic region and extended over 2–3 mm [Figure 1].
Figure 1: Postoperative multidetector computed tomography airway reconstruction illustrating the narrowest portion of airway to be 4.3 mm just below vocal cords

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  Discussion Top

Mucopolysaccharidosis type IV is an autosomal recessive disorder caused by deficiency of n-acetylgalactosamine-6-sulphate. Airway problems in these patients are often complicated by unstable cervical spine and the risk of subluxation.[4] Pulmonary functions can be compromised, resulting in restrictive lung disease. Cardiac involvement is usual.[5] In our patient, we noticed coarse facial features such as frontal slanting, narrowed nasal opening, macroglossia, reduced oropharyngeal space and restricted neck movements. There was no cardiac involvement.

Important issues concerning the airway management are related to intracellular accumulation of mucopolysaccharides, resulting in a narrowed and flattened trachea. The peculiar anatomy and extremely sensitive airway may result in failure of intubation and bronchospasm after intubation.[6] MDCT images provide preoperative detailed evaluation of the airway morphology which significantly influences anaesthetic planning.[3] In a study by Ozgul et al.,[7] MDCT was projected as a noninvasive, reliable method that helps to define size, length and type of stent in patients with tracheobronchial stenosis who are scheduled for endobronchial stenting.

Another study by Ingelmo et al.[3] concluded that MDCT images provided clinically important information in 87% of the patients with mucopolysaccharidoses, with reduction of tracheal size being the most common finding. Around 21% of anaesthesiologists switched their primary airway device after reviewing MDCT reconstruction images. In our case, a 6.0 mm ID and 5.0 mm ID ETTs were tried first in the anticipation of narrowed airway. Later, tracheal intubation was successful with a 4.5 mm ID tube, thereby suggesting that there was airway narrowing. Retrospectively, MDCT with airway reconstruction illustrated that the narrowest portion of airway was 4.3 mm. Age-predicted formulae do not work in these patients and appropriate uncuffed ETT is often two or three sizes smaller. It is crucial to decide the appropriate tube size to prevent extubation problems as well.[8] Sedative premedications are best avoided; we used only an antisialogogue.

Being minimally invasive and allowing neutral head position, awake fibreoptic intubation is the gold standard but could be difficult because of airway collapse and an uncooperative adolescent.[9] Walker et al.[10] found the use of a laryngeal mask airway to be extremely useful for short procedures in patients with mucopolysaccharidoses (MPS). However, in our patient, endotracheal intubation was mandated as the surgery was for atlantoaxial dislocation in the prone position.

An inhalational induction with sevoflurane provides a controlled situation with maintenance of spontaneous ventilation until laryngoscopy is performed. In our patient, fibreoptic intubation under spontaneous ventilation was tried but was unsuccessful. The reason could have been altered airway anatomy and a sensitive airway causing soft-tissue collapse. Manual-in-line-stabilisation was applied throughout the process of intubation.

Tracheostomy may be the choice in an emergency situation and cricothyroidotomy is not recommended for MPS patients. As extubation could prove to be problematic, all essential fibreoptic equipment and experienced personnel including an otorhinolaryngologist should be available at the time of extubation. A tube exchanger introduced into the ETT before extubation can be useful to perform immediate reintubation. Extubation should be done when the patient is fully awake, coughing vigorously and breathing adequately.[10]

  Conclusion Top

Patients with MPS pose a major challenge to the anaesthesiologist. The risk of anaesthesia can be reduced considerably if potential problems could be anticipated by the concerned team. A detailed knowledge of cervical and tracheolaryngeal anatomy is essential. In hindsight, we learnt the importance of a preoperative MDCT airway reconstruction to not only complete airway assessment but to also plan an effective airway management strategy in these problematic patients.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Morquio L. Sur une forme de dystrophie osseuse familiale. Arch Med Enfants 1929;32:129-40.  Back to cited text no. 1
Kamin W. Diagnosis and management of respiratory involvement in Hunter syndrome. Acta Paediatr 2008;97:57-60.  Back to cited text no. 2
Ingelmo PM, Parini R, Grimaldi M, Mauri F, Romagnoli M, Tagliabue G, et al. Multidetector computed tomography (MDCT) for preoperative airway assessment in children with mucopolysaccharidoses. Minerva Anestesiol 2011;77:774-80.  Back to cited text no. 3
Kerai S, Saith V, Kumar R, Tewari S. Airway management in Hurler's syndrome: A persistent challenge for anaesthesiologists. Indian J Anaesth 2016;60:599-601.  Back to cited text no. 4
[PUBMED]  [Full text]  
Rentería VG, Ferrans VJ, Roberts WC. The heart in the Hurler syndrome: Gross, histologic and ultrastructural observations in five necropsy cases. Am J Cardiol 1976;38:487-501.  Back to cited text no. 5
Man TT, Tsai PS, Rau RH, Cheng CR, Ko YP, Wu KH. Children with mucopolysaccharidoses–Three cases report. Acta Anaesthesiol Sin 1999;37:93-6.  Back to cited text no. 6
Ozgul MA, Ozgul G, Cetinkaya E, Abul Y, Kirkil G, Seyhan EC, et al. Multiplanar and two-dimensional imaging of central airway stenting with multidetector computed tomography. Multidiscip Respir Med 2012;7:27.  Back to cited text no. 7
Walker RW, Colovic V, Robinson DN, Dearlove OR. Postobstructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses. Paediatr Anaesth 2003;13:441-7.  Back to cited text no. 8
McLaughlin AM, Farooq M, Donnelly MB, Foley K. Anaesthetic considerations of adults with Morquio's syndrome – A case report. BMC Anesthesiol 2010;10:2.  Back to cited text no. 9
Walker RW, Darowski M, Morris P, Wraith JE. Anaesthesia and mucopolysaccharidoses. A review of airway problems in children. Anaesthesia 1994;49:1078-84.  Back to cited text no. 10


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