Anesthetic issues and Difficult Airway Management in a Case of Grisel's Syndrome.

Grisel's syndrome is a nontraumatic subluxation of atlanto-axial joint which is associated with inflammatory conditions of head and neck and occurs primarily in children. Anesthetic management is such cases constitute a multitude of challenges, especially related to the airway management. We presented here a case of 16-year-old male child weighing 23 kg, came to our hospital for the treatment of torticollis who was previously treated with intravenous antibiotics for rhinopharyngitis and diagnosed as a case of Grisel's syndrome. The child was operated on for unilateral resection of sternocleidomastoid muscle under general anesthesia. This case report pertains to the successful airway and anesthetic management in the background of difficult airway.

INTRODUCTION

Grisel's syndrome is nontraumatic subluxation of atlanto-axial joint following nasopharyngeal inflammation although it is first described by Sir Charles Bell in 1830 in a patient with syphilis, pharyngitis, and lethal spinal compression.[1] The name originated from the Grisel, who described two cases of pharyngitis and subluxation of atlanto-axial joint in 1951.[1] It is a rare disease, usually affect children and classically have torticollis associated with neck stiffness or pain on the neck movement. The condition often associated with pharyngitis, tonsillar or cervical abscess, otitis media, and adenotonsillitis.[2] Diagnosis is based on clinical and radiological findings. Presented here a case of Grisel's syndrome which seemed to be case of torticollis on the first look, on elicitation of past medical history as well as radiological examination diagnosis was made possible.

CASE REPORT

A 16-year-old male patient was brought to the Orthopaedics Outpatient Department by his parents with chief complaint of deformity in the neck region and pain on the neck rotation [Figures 1 and 2]. Surgery was planned for the resection of the distal end of sternocleidomastoid muscle. On the first inspection, it seemed to be a case of torticollis. However, on elicitation of past medical history during preanesthetic check-up, it was revealed that the child was treated with intravenous ceftriaxone and clindamycin for 1 week after pediatric consultation for the complaint of rhinopharyngitis and difficulty in the neck movement. The patient was discharged home as fever resolved and prescribed intravenous clindamycin therapy for 4 weeks. There was no history of trauma, vomiting, diarrhea, cough, nasal congestion or voice changes, and any neurological symptom.

Figure 1

Frontal view of the patient showing neck contracture and difficult airway

Figure 2

Lateral view showing significant neck contracture

After 1-month, the patient was readmitted for complaint of difficulty in the neck rotation and fatigue. He was afebrile, hemoglobin level was 12.2 g/dL and serum electrolytes, blood urea, serum creatinine, serum bilirubin, aspartate aminotransferase, alanine aminotransferase, and random blood glucose was within normal limits. There were no coagulation and platelets defects. Chest X-ray (posterior-anterior view) was normal.

On clinical examination, pulse rate was 82/min, regular with adequate volume, and blood pressure was recorded to 108/70 mm Hg. Mallampati Grade 3 with mouth opening of approximately 1.5 cm and weight of child was 23 kg. Lateral view cervical spine showed subluxation of atlanto-axial joint [Figure 3] and contrast enhanced computed tomography (CT) scan showed marked anterior subluxation of C1 on C2 measured 10 mm [Figure 4]. The electrocardiogram (ECG) was of sinus rhythm with no any abnormality. We had discussed with neurosurgeon regarding the extent of allowable neck movements, it was agreed that a slight degree of extension at the atlanto-axial joint would be permissible.

Figure 3

Lateral view of cervical spine showing subluxation of atlanto-axial joint

Figure 4

Computed tomography scan showing marked anterior subluxation of C1 on C2

On the day of surgery, child received 75 mg of ranitidine and 12.5 mg of oral promethazine syrup 1 h before surgery. Peripheral intravenous access was secured using 20-gauge cannula. Resuscitation and difficult airway management trolley were kept by side, and the equipment for tracheostomy was made available. The child was preoxygenated with 100% oxygen for 3 min.

Anesthesia was induced with fentanyl 50 μg, glycopyrrolate 0.1 mg, and propofol 50 mg. After the loss of consciousness, checked for ventilation then a bolus of 50 mg succinylcholine was administrated to facilitate tracheal intubation. After one min of ventilation and cessation of fasciculation, fiberoptic intubation was carried out. It was little difficult, but we were able to view the glottis and trachea was intubated with cuffed endotracheal tube 5.5 mm internal diameter. Throughout the induction and intubation, the neck was held in the neutral position with complete avoidance of flexion and allowing only minimal extension. Anesthesia was maintained with oxygen, nitrous oxide, 0.5% halothane, and vecuronium bromide. The entire procedure vital monitoring was carried out strictly by heart rate, pulse oximetry, ECG, noninvasive blood pressure, and end-tidal carbon dioxide (EtCO2). The entire procedure lasted for 2 h. Position of the patient was supine, and head rotated on the opposite side. At the end of surgery, neuromuscular blockade was reversed with neostigmine and glycopyrrolate. The child was extubated after the return of airway reflexes and rhythmic breathing. During the recovery period, our aim was to keep airway patent, to prevent aspiration and to keep neck stable by avoiding any flexion or extension.

Postoperatively, oxygen was given for 10 min and kept under monitoring in postanaesthesia care unit for 2 h. Postoperative period was uneventful. On 7th, postoperative day, the patient was discharged without any complication.

DISCUSSION

Grisel's syndrome is nontraumatic subluxation of atlanto-axial joint following the head or neck infection or after common surgical procedures such as adenoidectomy, mastoidectomy, tonsillectomy, cleft palate repair, and choanal atresia repair.[3] Which usually presents with torticollis, cervical pain, head tilt and restricted painful neck movements, and later with neurological complication[4] such as mild paresthesia, exaggerated reflex, clonus, bladder dysfunction to quadriplegia, and acute respiratory failure to death.[5] Pathophysiology of this disease consists of relaxation of the transverse and alar ligament of the atlanto-axial joint. Spread of inflammation to the atlanto-axial ligaments on the head or neck infection or surgical procedure causes hyperemia which weakens the transverse and alar ligaments and results in instability of atlanto-axial joint.[6] Symptom begins as a typical torticollis which is caused by spasm of irritated neck muscles. Diagnosis is made possible by patient's history, clinical signs, and ultimately by radiological findings. Lateral cervical spine X-ray may show an increase in atlanto-dens interval, i.e., subluxation. The normal atlanto-dens interval is ≤3 in adults and ≤4.5 mm in children.[7] CT is the investigation of choice for the evaluation of bony cervical spine. Biochemical tests are nondiagnostic, blood cultures are often negative, and an erythrocyte sedimentation rate is mostly raised. The primary treatment of early detected Grisel's syndrome is conservative which includes bed rest, antibiotic therapy, muscle relaxants, anti-inflammatory therapy, and external fixation.

The main challenges in our case were the airway management due to laxity of transverse ligament. Extension of the head in these patients may cause subluxation of atlas over axis with resultant compression of the spinal cord by the odontoid process. Agarwal et al. reported the case of a child who, following adentonsillectomy, developed quadriplegia and acute respiratory distress due to previously undetected atlanto-axial instability.[8] Patients with atlanto-axial instability at risk of serious neurological injury during general anesthesia, particularly at the time of laryngoscopy and tracheal intubation.

We had planned for fiberoptic bronchoscopy after neck stabilization to prevent any neurological complication. The airway trolley was made ready for difficult airway management. Anesthesia was induced with propofol. We checked for ventilation and after establishing successful bag and mask ventilation. We administered succinylcholine for muscle relaxation, trachea was then intubated. On 5-point auscultation and by EtCO2, we confirmed the position of endotracheal tube and fixed. For achieving analgesia, fentanyl was used because of its lesser propensity for respiratory depression, myocardial depression, and relatively shorter duration of action.

CONCLUSION

Successful airway and anesthetic management in case of Grisel's syndrome, one should thorough and deep knowledge about the anatomic changes and pathophysiology as to prevent any clinical disaster in operation theater, especially for elective surgery. Grisel's syndrome must be taken under consideration in patients with acute torticollis following either an infection or operative procedure in upper aerodigestive tract to minimize the risk of neurological complication.

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.

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Conflicts of interest

There are no conflicts of interest.