Ain-Shams Journal of Anaesthesiology

CASE REPORT
Year
: 2015  |  Volume : 8  |  Issue : 4  |  Page : 683--685

Crouzon syndrome: an anesthetic challenge


Minal Harde1, Varsha Suryavanshi1, Swati Chhatrapati1, Mahadevan Vaidyanathan1, Rakesh Bhadade2,  
1 Department of Anaesthesia, T.N.M.C. & B.Y.L. Nair Ch. Hospital, Mumbai, Maharashtra, India
2 Department of Medicine, T.N.M.C. & B.Y.L. Nair Ch. Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Minal Harde
Flat No 15, Anand Bhavan, Nair Hospital, Mumbai Central 08, Maharashtra, 440011
India

Abstract

Crouzon syndrome is a rare hereditary disorder, characterized by craniosynostosis. Management of difficult airway is challenging because of craniofacial abnormalities. Presentation during adulthood is rare. We describe the case of a 37-year-old man with a known case of Crouzon syndrome who was posted for fixation of humerus fracture. The patient was unique because of the presence of kyphoscoliosis with severe restrictive lung functions, atlantoaxial dislocation, and mental retardation, having almost all features of the syndrome. Anesthesia plan A was to use laryngeal mask airway proseal as the patient had adequate mouth opening. Nasal fiberoptic bronchoscopy was contraindicated and regional nerve block was not a feasible option. Plan A was not successful because of obstructed breathing pattern. Plan B of oral fiberoptic bronchoscopy-guided intubation with good airway anesthesia and sedation was successfully managed in the next attempt.



How to cite this article:
Harde M, Suryavanshi V, Chhatrapati S, Vaidyanathan M, Bhadade R. Crouzon syndrome: an anesthetic challenge .Ain-Shams J Anaesthesiol 2015;8:683-685


How to cite this URL:
Harde M, Suryavanshi V, Chhatrapati S, Vaidyanathan M, Bhadade R. Crouzon syndrome: an anesthetic challenge . Ain-Shams J Anaesthesiol [serial online] 2015 [cited 2022 Dec 6 ];8:683-685
Available from: http://www.asja.eg.net/text.asp?2015/8/4/683/172770


Full Text

 Introduction



Crouzon syndrome is an autosomal dominant disorder also known as the brachial arch syndrome that usually presents during childhood [1] . These syndromes with craniofacial deformities when present in adulthood are very challenging because of difficult airway management [2] . The patient was unique because of the presence of kyphoscoliosis with severe restrictive lung functions, atlantoaxial dislocation (AAD), and mental retardation, having almost all features of the syndrome. This report highlights the management of this patient at various fronts.

 Case report



A 37-year-old 70 kg man was posted for an open reduction and internal fixation of upper 1 / 3 humerus fracture. He was a known case of Crouzon syndrome with classical features of skull and facial dysmorphism and exophthalmos [Figure 1]. The patient had a history of delayed developmental milestones and convulsions during childhood, with the last episode of convulsion at the age of 7 years. The patient had mild conductive deafness and blindness, optic atrophy, kyphoscoliosis, and mental retardation.{Figure 1}

The patient had frontal bossing, beaked nose, and compressed nasal bridge with deviated nasal septum. On airway examination, mouth opening was greater than three fingers with a large tongue and high-arched palate. Mallampati class was IV with multiple missing teeth and abnormal dentition. The patient had maxillary hypoplasia, permanent underbite, and prognathism. He had a short neck with restricted neck movements and thyromental distance less than 4 cm [Figure 2]. There was severe kyphoscoliosis with Cobbs angle less than 15°, and pulmonary function test (PFT) showed severe restrictive changes.

Patient's vitals, all blood investigations, arterial blood gases, and two-dimensional echo were within normal limits. Chest radiograph showed crowding of rib spaces on the right side. Computed tomography of the skull showed craniosynostosis, hydrocephalus, and AAD, for which neurological opinion was taken, minimal manipulation was advised to avoid rise in intracranial tension (ICT).{Figure 2}

Preoperative optimization using incentive spirometry was started with counseling the patient.

Our plan was to use laryngeal mask airway (LMA) ProSeal (The Laryngeal Mask Company Ltd', LeRocher, Victoria, Mahe, Seychells) size 4 with spontaneous ventilation keeping a difficult airway cart with fiberoptic bronchoscopy (FOB) (Pentax FB-18V' Montvale, New Jersey, insertion tube diameter 6 mm; Japan) ready.

On the day of surgery, valid, written informed high-risk consent was obtained from parents. Standard monitors were attached, preoxygenation was carried out for 5 min, and premedication with intravenous glycopyrrolate 0.2 mg, ondansetron 4 mg, midazolam 0.5 mg, and fentanyl 40 µg was administered.

On induction of anesthesia with oxygen and sevoflurane, he developed partial respiratory obstruction, which worsened after oropharyngeal airway insertion. Mask ventilation was difficult and so LMA was inserted immediately. SpO 2 was 99%; however, there was increasing resistance to the reservoir bag and the capnogram showed a rising ETCO 2 . Hence, LMA was removed and we attempted direct laryngoscopy, which revealed only the tip of the epiglottis (Cormack and Lehane grade 3) and intubation was not successful. Hence, the patient was awakened and planned for FOB-guided intubation. Airway anesthesia with lignocaine nebulization, superior laryngeal nerve, and transtracheal block was induced. Bilateral superior laryngeal nerve block was induced with a 25-G needle at the level of the thyrohyoid membrane inferior to the cornu of the hyoid bone after negative aspiration for air and blood, and 2 ml of 2% lidocaine was injected. Transtracheal block was induced perpendicular to the axis of the trachea with a 20-G needle syringe with continuous aspiration until air was freely aspirated and then 4 ml of 4% lidocaine was injected.

On guiding FOB through the oral cavity, secretions were hampering the field of vision and the patient had become very uncooperative and so the procedure was abandoned on that day.

The patient was again posted for surgery on the third day, after proper counseling. Gargling with viscous lignocaine was carried out outside the operation theater with the help of relatives. Premedication and sedation as mentioned above were given and airway preparation was carried out in the form of lignocaine nebulization and superior laryngeal and transtracheal nerve blocks.

Oral FOB was performed through the bite block (to prevent accidental biting), during the course of which a large overhanging epiglottis was detected, vocal cords could not be appreciated, and the tracheal rings could not be visualized with clarity, but glow was seen in the anterior neck and the carina was appreciated. A portex cuffed endotracheal tube no 7.5 mm ID was guided past the vocal cords. Correct placement was confirmed by means of auscultation and by means of the capnogram findings. The patient was given standard balanced general anesthesia (in the form of thiopental sodium 5 mg/kg intravenously, vecuronium bromide 0.1 mg/kg, and repeat fentanyl 0.5 µg/kg intravenously and was maintained with oxygen and sevoflurane) with careful positioning. Extreme care was taken with minimal movement at the cervical spine; adequate padding and measures to prevent rise in ICT were also taken. The intraoperative course was uneventful and the surgical time was 4 h. At the end of the procedure, the patient was reversed but not extubated and shifted to the postanesthesia care unit on T-piece with minimal sedation (intravenous midazolam 0.5 mg/h) for observation. He was extubated after 4 h when he was fully awake and had no complications postoperatively.

 Discussion



Crouzon described the hereditary syndrome of craniofacial dysostosis with the triad of calvarial deformities, facial anomalies, and exophthalmos. Crouzon syndrome is an autosomal dominant disorder with complete penetrance and variable expressivity [3],[4] . Prevalence is one case per 60 000 live births [1] . It is characterized by craniosynostosis and is detected during the newborn or infantile period because of dysmorphic features [3],[5],[6] . Other clinical features include hypertelorism, shallow orbits, exophthalmos, strabismus, beaked nose, short upper lip, hypoplastic maxilla, mandibular prognathism, midfacial hypoplasia, and upper airway obstruction [1],[5] .

Managing airway is complicated because of high-arched palate, limited neck extension nasopharynx narrowing, and tracheal ring abnormality [5],[6],[7] .

If cranial reconstruction is performed early in age, intelligence is normal. Our patient was 37 years of age, mentally retarded, and had almost all features of Crouzon's syndrome. Unique problems in our patient were hydrocephalus, kyphoscoliosis with severe restrictive lung functions, and AAD.

General anesthesia was planned as the patient had mental retardation, anticipated difficult airway, AAD, and surgical position being lateral decubitus. Regional nerve block was not a feasible option due to restricted neck movements, AAD not permitting proper positioning for block, and difficulty in managing uncooperative patient under regional, as excessive sedation was risky because of difficult airway. Awake nasal FOB was contraindicated because of nasal deformity.

As the patient had adequate mouth opening we planned LMA proseal under sevoflurane with spontaneous ventilation. There was one case report of successful use of LMA-Fastrach in Crouzon's syndrome [8] . Inhaled induction of anesthesia is useful in difficult airway conditions as spontaneous ventilation is maintained and rapid recovery is possible.

In our patient, LMA placement was not successful because of distorted anatomy and obstructed breathing and so the patient was awakened and planned for oral FOB intubation with good airway anesthesia and sedation in the next attempt, which was successfully managed.

Reported mortality in this syndrome is because of upper airway obstruction leading to acute respiratory distress or increased intracranial pressure [6],[9] .

Anesthetic management is very challenging in Crouzon syndrome. Anticipation, patient counseling, complete difficult airway cart with alternative plans, and multidisciplinary approach goes a long way in managing such patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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