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Year : 2019  |  Volume : 20  |  Issue : 1  |  Page : 42-45

Anesthesia challenges in a case of Crouzon syndrome for corrective rigid external distraction frame insertion

Department of Anesthesiology, Grant Government Medical College, Mumbai, Maharashtra, India

Date of Submission07-Oct-2018
Date of Acceptance25-Dec-2018
Date of Web Publication6-May-2019

Correspondence Address:
Dr. Paulomi Dey
Flat No. 13, LXN3, Sai Krupa CHS, Kashish Park, LBS Marg, Thane West - 400 604, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_57_18

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Crouzon syndrome is an autosomal dominant disease occurs in approximately 1 in 25,000 births, due to a mutation in the fibroblast growth factor receptor 2 gene on chromosome 10 characterized by craniosynostosis, mid-face hypoplasia, hypertelorism, high-arched palate, skull base abnormalities, and exophthalmos. Patients present in early childhood for cranial reconstructive surgery. We describe here the successful management of a 5-year-old child with Crouzon syndrome with hypertrophic adenoids and Arnold-Chiari malformation type 1 posted for bifrontal craniotomy with fronto-facial advancement and application of a rigid external distraction frame.

Keywords: Craniosynostosis, Crouzon, difficult airway, pediatric anesthesia, rigid external distraction frame

How to cite this article:
Ganeriwal V, Dey P, Gore B, Hujare T. Anesthesia challenges in a case of Crouzon syndrome for corrective rigid external distraction frame insertion. Indian Anaesth Forum 2019;20:42-5

How to cite this URL:
Ganeriwal V, Dey P, Gore B, Hujare T. Anesthesia challenges in a case of Crouzon syndrome for corrective rigid external distraction frame insertion. Indian Anaesth Forum [serial online] 2019 [cited 2022 Oct 6];20:42-5. Available from: http://www.theiaforum.org/text.asp?2019/20/1/42/257682

  Introduction Top

Crouzon syndrome is a congenital disorder characterized by premature closure (synostosis) of the coronal sutures, and less frequently sagittal or lambdoidal skull sutures.[1] Crouzon occurs in approximately 1 in 25,000 births and is due to a mutation in the fibroblast growth factor receptor 2 gene on chromosome 10.[2] It may be inherited in an autosomal dominant fashion or as a spontaneous mutation with a male:female predominance of 3:1.[2] The challenges in anesthetic management include the presence of a difficult airway, history of obstructive sleep apnea, congenital cardiac disorders, hypothermia, blood loss, and venous air embolism.[2],[3],[4]

  Case Report Top

A 5-year-old female child weighing 12 kg with Crouzon syndrome was referred from peripheral hospital to our institute for corrective surgery. She had delayed developmental milestones and protrusion of eyeballs with abnormal skull growth. During preoperative assessment, the parents gave a history of snoring episodes with mouth breathing and inability to lie in the supine position. There were no complaints signifying any cardiac condition. An ENT opinion was sought and she was diagnosed with adenoid hypertrophy. There was no evidence of syndactyly in limbs or any spinal deformity. Airway examination revealed a short neck, large tongue, hypoplastic maxilla with high-arched palate and enlarged adenoids. Temporomandibular joints were bilaterally mobile [Figure 1].
Figure 1: Preoperative appearance

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All routine blood investigations including coagulation profile were normal. Thyroid profile done to rule out exophthalmic goiter was also normal. Radiological workup detected maxillary hypoplasia,  Arnold- Chiari malformation More Details More Details type 1, and a reduced oropharyngeal airway diameter [Figure 2]. CT scan of the brain and two-dimensional echocardiogram were normal.
Figure 2: Xray neck

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On the day of surgery, intravenous access was secured with 22G cannula and the child was premedicated with 4 μg/kg glycopyrrolate and 0.5 mg/kg ketamine. After connecting the standard monitors (peripheral capillary oxygen saturation, non-invasive blood pressure, electrocardiography, and end-tidal carbon dioxide), the child was preoxygenated in the lateral position with an oropharyngeal airway in situ in view of preoperative history of obstructive sleep apnea (OSA). A further dose of 0.5 mg/kg ketamine was given and using the straight blade no. 2 C-MAC video laryngoscope a check laryngoscopy was done. After visualization of the vocal cords, depth of anesthesia was deepened with 2 mg/kg propofol and 2 μg/kg fentanyl, and the patient was intubated with flexometallic uncuffed endotracheal tube of 5 mm diameter while maintaining spontaneous respiration. After confirming the placement of the endotracheal tube (ETT), the patient was paralyzed with 0.5 mg/kg atracurium and positive pressure ventilation was initiated. The ETT was secured and taped to the chin for ease of surgical manipulation [Figure 3]. A 22G invasive arterial line, 5 Fr right IJV central venous pressure line, and 20G additional peripheral line were inserted.
Figure 3: After intubation

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Anesthesia was maintained with air, oxygen (FiO2 0.5) and sevoflurane (1–1.6 Mac) and 5 ug/kg/min of atracurium. Surgery lasted for 4 h with 150 ml of blood loss which was replaced with crystalloids and 100 ml of blood targeting a hematocrit between 30% and 35%. Tranexamic acid was administered to minimize blood loss. Temperature was maintained between 36.3°C and 36.9°C throughout the procedure. The child received of 5 μg/kg of fentanyl and paracetamol (15 mg/kg) during the procedure. At the end of the procedure, the flexometallic ETT was secured at the angle of the mouth and the child was shifted to the intensive care unit with rigid external distraction (RED) frame in situ.

In the intensive care unit (ICU), paralysis and sedation were given with dexmedetomidine infusion at 0.4 μg/kg/h and atracurium at 5 μg/kg/min and analgesia with paracetamol 15 mg/kg 8 hourly. The child was successfully weaned and extubated after 24 h when she was conscious and hemodynamically stable and had postoperative pain and discomfort due to the RED frame was judiciously controlled. She was then shifted to the ward on day 2 for further recovery [Figure 4].
Figure 4: After extubation

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  Discussion Top

Crouzon syndrome is characterized by premature closure of the cranial sutures and distinctive facial abnormalities, resulting in brachycephaly, trigonocephaly or scaphocephaly. In addition, it may also be associated with frontal bossing; a curved nose; mid-face hypoplasia; and a short upper lip. Hypoplastic maxilla with relative mandibular prognathism may also occur.[5] Proptosis along with hypertelorism and strabismus has been reported. Approximately 30% of individuals with Crouzon syndrome develop hydrocephalus while some may also present with sensorineural hearing loss. Extended dysostosis of facial and cervical bones and subsequent soft tissue abnormalities may comprise the upper airways and OSA is common in Crouzon Syndrome.[1] Spine abnormalities may be present, which can reduce cervical movement and together with nasal and pharyngeal obstructions, a difficult airway scenario has to be anticipated.[1],[5] Crouzon syndrome may be associated with a patent ductus arteriosus and aortic coarctation, and hence, a cardiac evaluation is mandatory. However, no such anomaly was detected in our patient. Complex hypoplasia of the cranial vault, orbits, and mid-face may require fronto-facial advancement and/or distraction osteogenesis with application of a RED frame as was performed in our patient.[4],[6]

The perioperative concerns in such patients include potentially difficult airway, exposure keratitis, hypothermia, blood loss, venous air embolism, and long duration of surgery.[3],[7] Achieving a good mask seal may be difficult. As our patient had no features of syndactyly securing, intravenous access was not an issue.[8] We were successful in preoxygenating with oropharyngeal airway and a size 2 mask in the lateral position. In anticipated difficult airway C-MAC video laryngoscope has been shown to perform better in terms of shorter intubation time, higher success rate, and less number of optimizing maneuvers.[9] As our patient had Arnold-Chiari malformation, the use of C-MAC facilitated intubation with the neck in the neutral position.

It is essential to ensure that the endotracheal tube is not subjected to kinking and to prevent accidental dislodgement. A south-facing Ring-Adair-Elwyn tube can be used successfully for fronto-orbital advancement and remodeling (FOAR) and nasal cuffed tubes for total calvarial surgery.[7]

The patients may be positioned in supine, prone, or modified prone (sphinx) positions.[4] Lubricating eye drops were applied and temporary tarsorrhaphy was done to protect the eyes from corneal abrasions and exposure keratitis.[1] Direct orbital pressure may cause damage to the optic nerve and retinal ischemia. An acute presentation of such pressure may be a vagally mediated bradycardia, most likely seen in FOAR surgery.[1] Our patient was positioned supine with the head on the horseshoe support with operating table in neutral position.

Faberowski et al. found the incidence of VAE during craniectomy for craniosynostosis to be 82.6%, as detected by precordial Doppler.[10] In some centers, they perform a tracheostomy2 weeks before the midfacial surgery if a RED frame is planned.[7] However in our patient the frame being supra-labial we did not opt for tracheostomy. The child was extubated after 24 hours of ICU stay; during extubation, the C-MAC laryngoscope and tracheostomy tube were kept standby. Postextubation, the patient was further monitored for 24 h and then shifted to the ward after proper counseling of the parents regarding care of the child with the RED device in situ. The plan was to maintain distraction with the frame for 4 weeks after which further surgical course would be determined.

  Conclusion Top

Crouzon syndrome being a rare genetic disorder, complete evaluation and strict vigilance perioperatively enable safe anesthetic management, recovery, and rehabilitation.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Bajwa SJ, Gupta SK, Kaur J, Singh A, Parmar SS. Anaesthetic management of a patient with Crouzon syndrome: Case study. South Afr J Anaesth Analg 2012;18:270-2.  Back to cited text no. 1
Reardon W, Winter RM, Rutland P, Pulleyn LJ, Jones BM, Malcolm S, et al. Mutations in the fibroblast growth factor receptor 2 gene cause Crouzon syndrome. Nat Genet 1994;8:98-103.  Back to cited text no. 2
Pearson A, Matava C, Anaesthetic management for craniosynostosis repair in children. BJA Educ 2016;16:410-6.  Back to cited text no. 3
Bajwa SS, Haldar R. Craniosynostosis surgery-anaesthetic challenges and implications. Med J DY Patil Univ 2015;8:364-6.  Back to cited text no. 4
  [Full text]  
Sculerati N, Gottlieb MD, Zimbler MS, Chibbaro PD, McCarthy JG. Airway management in children with major craniofacial anomalies. Laryngoscope 1998;108:1806-12.  Back to cited text no. 5
Hughes C, Thomas K, Johnson D, Das S. Anesthesia for surgery related to craniosynostosis: A review. Part 2. Paediatr Anaesth 2013;23:22-7.  Back to cited text no. 6
Thomas K, Hughes C, Johnson D, Das S. Anesthesia for surgery related to craniosynostosis: A review. Part 1. Paediatr Anaesth 2012;22:1033-41.  Back to cited text no. 7
Kumar N, Arora S, Bindra A, Goyal K. Anesthetic management of craniosynostosis repair in patient with apert syndrome. Saudi J Anaesth 2014;8:399-401.  Back to cited text no. 8
[PUBMED]  [Full text]  
Aziz MF, Dillman D, Fu R, Brambrink AM. Comparative effectiveness of the C-MAC video laryngoscope versus direct laryngoscopy in the setting of the predicted difficult airway. Anesthesiology 2012;116:629-36.  Back to cited text no. 9
Faberowski LW, Black S, Mickle JP. Incidence of venous air embolism during craniectomy for craniosynostosis repair. Anesthesiology 2000;92:20-3.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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