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Case Report

Anaesthetic implications in a patient with Morquio A

syndrome

Charu Mahajan, KN Adarsha1, Bhagya R. Jena2, Devendra Gupta3, Shashi Srivastava3

Abstract
In patients with Morquio A syndrome, almost all the systems of body are affected and this has a bearing on their
anaesthetic management. Herein, we describe the management of child with Morquio A syndrome with atlanto‑axial
dislocation who underwent posterior fixation under general anaesthesia.
Key words: Anaesthesia, atlanto‑axial dislocation, Morquio A syndrome, mucopolysaccharidosis

INTRODUCTION involvement of temporo‑mandibular joints result in a

difficult airway state.
Mucopolysaccharidosis (MPS) refer to an inherited group of
diseases caused by deficiency of different lysosomal enzymes We hereby report a case of MPS IV‑A with atlanto‑axial
responsible for degradation of glycosaminoglycans. dislocation (AAD) scheduled for transoral odontoidectomy
Intracellular accumulation of these partially metabolized and posterior fixation.
glycosaminoglycans hinder normal functioning of the
tissues involved. Morquio A syndrome (MPS IV‑A) is an CASE REPORT
autosomal recessive MPS caused by deficiency of N‑acetyl
galactosamine‑6‑sulfatase enzyme resulting in deposition A 6‑year‑old female child known case of MPS IV‑A,
of large amount of keratan sulphate and chondroitan weighing 14 kg was admitted with history of gradually
sulphate in various tissues of body.[1] The manifestations are increasing weakness of all limbs for last 5  years and
primarily due to mesenchymal abnormalities and mental increased frequency of micturition for last 7  months.
intelligence is normal. Patient had a complete cessation of growth for last
2  years and at present, height was 120  cm. She had
Nearly every system of body may be involved normal developmental milestones and intellect with no
affecting anaesthetic preparation and management. history of obstructive sleep apnoea.
Cervico‑vertebral junction anomaly, deposition of
mucopolysaccharides in various airway structures and On examination, the child had a short stature with
dolichocephaly, short neck, kyphosis at thoraco‑lumbar
Access this article online region, pectum excavatum with prominent costal margins,
Quick Response Code:
genu valgum and equino valgum  [Figure  1]. Central
Website: nervous system examination revealed a power of 4/5 in
www.jnaccjournal.org all limbs with spasticity and brisk reflexes. Computed
tomography (CT) scan of craniovertebral junction showed
DOI: AAD with obliteration of normal cervical lordosis with
10.4103/2348-0548.154238 occipitalisation of C1 posterior arch. Patient was planned
for occipitocervical fusion with sublaminar wiring.

Department of Neuroanaesthesiology, All India Institute of Medical Sciences, New Delhi, 1Departments of Anaesthesiology,
Muslim Educational Society Medical College and Hospital, Kerela, 2Institute of Medical Sciences Banaras Hindu University,
Varanasi, 3Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Address for correspondence:
Dr. Charu Mahajan, Department of Neuroanaesthesiology, All India Institute of Medical Sciences, New Delhi ‑ 110 029, India.
E‑mail: [email protected]

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Mahajan, et al.: Anaesthetic management of child with morquio a syndrome

Preanaesthesia assessment revealed a systolic murmur fibreoptic bronchoscope. Maintenance of anaesthesia

at apical area on auscultation which was confirmed by was done with sevoflurane in air/oxygen mixture and
echocardiographic finding of myxomatous degeneration intermittent boluses of rocuronium.
of heart valves with moderate mitral regurgitation with
normal left ventricular function. Airway examination Monitoring included electrocardiography, pulse
revealed adequate mouth opening with Mallampati oximetry, invasive arterial blood pressure, urinary output
grade  II with large tongue and short neck. A  cervical and endtidal CO2 and anaesthetic gas measurement.
collar was placed around neck. X‑ray neck revealed Heart rate was kept between 90 and 100/min to decrease
enlarged soft tissue shadow in prevertebral area the regurgitant flow across mitral valve. Patient was
with apparently normal laryngeal and tracheal carefully positioned prone for surgery. Surgery lasted
contour [Figure 2]. Possibility of difficulty in securing for an uneventful period of 4 hours.
airway was anticipated in view of presence of congenital
At end of posterior fixation, patient was turned supine
AAD, large tongue, short neck, soft tissue deposit and
anda check larngoscopy was performed to assess the
cervical collar in situ. Preoperative complete haemogram,
laryngeal view. A complete glottic view was visualized
renal function test, lung fields on chest X‑ray were
[Figure 3] and subsequently, neuromuscular blockade
normal. Bedside pulmonary function tests  (PFT) of
was reversed.[15] When child became fully conscious with
the patient were equivocal as the child could not
resumption of normal breathing, trachea was extubated
cooperate to maximum ability. We did not administer
without any complication. She was then transferred to the
infective endocarditis  (IE) prophylaxis, as there is no
neuro‑intensive care unit (NICU), where she recovered
recommendation regarding IE prophylaxis in a patient
uneventfully and was monitored overnight. Duration of
with congenital mitral regurgitation, according to the
intensive care unit (ICU) stay and hospital stay was 1 and
guidelines.[2]
5 days, respectively. At the time of discharge, spasticity
Patient was premedicated with intravenous had decreased with power 4/5 power in all limbs and
glycopyrrolate 0.1  mg, 10  min before induction of glasgow outcome scale (GOS) was 4.
anaesthesia. Difficult airway cart was prepared in view
of anticipated difficult intubation. It comprised oral DISCUSSION
airways, stylets and bougie, laryngoscopes with Miller
The multisystemic involvement[3‑7] in a patient with
and Macintosh blades, different sizes of endotracheal
MPS IV‑A makes thorough preoperative assessment
tubes, laryngeal mask airway  (LMA), paediatric
imperative. Based on the findings  [Table  1], adequate
fibreoptic bronchoscope, glidescope, equipment
preparation should be done to avoid any sudden crisis.
for cricothyrotomy and surgical tracheostomy. The
neurosurgeon was present in the operating room and Several authors have highlighted airway abnormalities
was asked to be prepared for performing tracheostomy, in these patients. Not only it is difficult to intubate
in case need arises. Anaesthesia was induced with trachea but also structural changes may lead to
sevoflurane following which a peripheral venous laryngeal stenosis and distorted lower airway resulting
access was secured. Intravenous (iv) fentanyl 2 µg/kg in ventilation problems. History of obstructive sleep
was administered and after ensuring adequate mask apnoea  (OSA) has been found to be associated with
ventilation, rocuronium 10  mg iv was given. Patient
was intubated successfully with 5.5  mm cuffed, oral,
flexometallic tracheal tube with the help of paediatric

Figure 1: Image of the patient showing pectum excavatum with Figure 2: X-ray neck showing soft tissue mass in prevertebral area
prominent costal margins and short neck with normal laryngeal and tracheal contour

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Mahajan, et al.: Anaesthetic management of child with morquio a syndrome

delineate airway anatomy and assist in decision‑making

regarding intubation and extubation.[12] Recently, use of
ultrasound for assessment of airway anatomy has also
been highlighted.[12] This simple method not only allays
the harmful effects of radiation but can also give a real
time picture.[13]

Rigid instruments like glidescope, lighted stylet etc.,

are preferred for intubation in these patients as they
can displace soft tissue more easily than flexible
fibrescope.[10] We opted for flexible bronchoscope as the
first choice as it causes no movement at atlanto‑axial
joint. Several authors recommend awake fibreoptic
Figure 3: Glidescopic view clearly showing full view of glottis intubation and avoiding muscle relaxants for this
purpose. As our patient was a child, we did not resort to
Table 1: Multisystemic involvement in awake intubation. Though the intellect is normal in these
Morquio A syndrome patients, behavioural abnormalities like anxiety and
System depression are known to occur.[14] We were successful
in intubating the patient with fibreoptic bronchoscope
Cardiac
under anaesthesia in the first attempt. Literature
Valvular regurgitant or stenotic lesions, reduced search reveal reports of augmented difficult intubation
myocardial compliance, hypertension, coronary disease, once the patient is paralysed. Loss of muscular tone
arrhythmias, cardiac failure
and floppy soft tissues after administration of muscle
Respiratory relaxants, makes fibreoptic intubation after induction of
Macroglossia, dental abnormalities, short neck, anaesthesia even more difficult.[7,15] Supraglottic airway
subglottic narrowing, hanging epiglottis, oro‑pharyngeal device such as I‑gel has also been used successfully
deposits, large adenoids/tonsils, restricted opening used as a channel for fibreoptic‑guided tracheal
of temporomandibular joints, restrictive and even intubation in a patient with Hunter syndrome.[16] The
obstructive respiratory pattern
ability to mask ventilate and back‑up of glidescope,
Vertebral cricothyrotomy and tracheostomy made us go ahead
Cranio‑vertebral junction anomaly, odontoid hypoplasia, with muscle relaxation. The choice of muscle relaxant
cervical cord compression is debatable and many will straightway negate the
Skeletal use of rocuronium in such cases and will prefer to use
Short stature, short diaphysis, curved metaphysis, poorly succinylcholine if at all. But we want to emphasize
developed epiphysis, wide acetabula, hypoplastic femoral that succinylcholine is contraindicated in such cases of
heads, pigeon chest, genu valgum, equino valgum upper motor neuron paresis and should not be used.
Others Life‑threatening hyperkalaemia may complicate the
anaesthetic management and even cardiac arrest may
Prognathism, hepatomegaly, corneal clouding, glaucoma,
ensue. However, another safer alternative could have
degenerative retinal lesions, hearing loss
been fibreoptic intubation under sevoflurane induction
along with ‘spray as you go technique’ omitting the
difficult airway.[8] Such children are prone to respiratory administration of muscle relaxant. The inhalational
obstruction during induction of anaesthesia and post induction also allows rapid awakening in case intubation
extubation. The incidence of difficult intubation in fails. Fibreoptic intubation under dexmedetomidine
patients with Morquio syndrome varies from 0–50%.[9‑11] sedation with preservation of spontaneous breathing
In a retrospective review of 17 patients of MPS, overall is another alternative. Fibreoptic intubation under
incidence of difficult intubation was 25%; highest in influence of muscle relaxants requires expertise and
MPS VI (86.7%) and 0% in MPS IV.[9] However, small should only be attempted if one is proficient and has
number of MPS IV patients in study population may be back‑up help. In a difficult airway situation, we should
responsible for this finding. use the technique we are comfortable with.

The decision regarding airway management can be based Posterior fixation renders the airway more difficult.
on radiology findings. In our patient, the preoperative So at the end of surgery, we did a check laryngoscopy
X‑ray neck did not show any evidence of laryngeal or with glidescope to assess the laryngeal view [Figure 3].
tracheal stenosis. Another modality like preoperative An indirect glottis visualization with help of
multidetector computed tomography film can also videolaryngoscope can help in assessing the airway at

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Mahajan, et al.: Anaesthetic management of child with morquio a syndrome

time of extubation in cases where posterior fixation of options for patients with morquio A syndrome. Ther Clin Risk
cervical spine has been done. If reintubation is required Manag 2013;9:45‑53.
in cases where extension of cervical spine is absent, at 5. Walker PP, Rose E, Williams JG. Upper airway
abnormalities and tracheal problems in morquio’s disease.
least we can be sure of being able to visualize glottis
Thorax2003;58:458‑9.
with videolaryngoscopes. With additional help of 6. Muenzer J. Overview of the mucopolysaccharidosis.
stylet/bougie, we expect to be able to reintubate 100% Rheumatology (Oxford) 2011;50 Suppl 5:v4‑12.
of patients. 7. Walker R, Belani KG, Braunlin EA, Bruce IA, Henrik H,
Harmatz PR, et al. Anaesthesia and airway management in
Absence of OSA, uneventful intubation at start and mucopolysaccharidosis. J Inherit Metab Dis 2013;36:211‑9.
visibility of full glottis on check video laryngoscopygave 8. Apfelbaum JL, Haqberg CA, Caplan RA, Blitt CD, Connis RT,
Nickinovich DG, et al. American society of anesthesiologists
an assurance that we can go ahead with extubation of
task force on management of the difficult airway. Practice
trachea. Patients may require continuous positive airway guidelines for management of the difficult airway: An updated
pressure in the postoperative period and should be report by the American society of anesthesiologists task
closely watched for any desaturation episodes. force on management of the difficult airway. Anesthesiology
2013;118:251‑70.
9. Frawley G, Fuenzalida D, Donath S, Yaplito‑Lee J, Peters H.
CONCLUSION A retrospective audit of anesthetic techniques and
complications in children with mucopolysaccharidoses.
MPS IV‑A affects multiple systems and patient should be Paediatr Anaesth 2012;22:737‑44.
assessed in total. Then, depending on the expertise and 10. Theroux MC, Nerker T, Ditro C, Mackenzie WG. Anesthetic
resource availability, an individual anaesthetic plan can care and perioperative complications of children with
be chalked out for these patients. Decision to extubate Morquio syndrome. Pediatr Anesth 2012;22:901‑7.
the patient can be reinforced by a check laryngoscopy 11. Walker RW, Darowski M, Morris P, Wraith JE. Anaesthesia
and mucopolysaccharidoses. A review of airway problems in
with help of video laryngoscope. children. Anaesthesia 1994;49:1078‑84.
12. Ingelmo PM, Parini R, Grimaldi M, Mauri F, Romagnoli M,
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