Grisel’s Syndrome – A Complete Patient Guide

Overview

Grisel’s syndrome is a rare, non‑traumatic subluxation (partial dislocation) of the atlanto‑axial joint – the first two vertebrae of the neck (C1 “atlas” and C2 “axis”). The condition typically follows an upper respiratory infection, adenotonsillectomy, or other inflammatory processes in the head and neck. The inflammatory tissue causes laxity of the ligaments that stabilize C1–C2, allowing the atlas to slip forward on the axis.

Because it most often occurs after ENT (ear‑nose‑throat) surgery in children, Grisel’s syndrome is considered a pediatric condition, but adults can be affected, especially after severe neck infections or inflammatory diseases.

• Age group most affected: 5–12 years (≈ 70 % of cases).
• Gender: Slight male predominance (≈ 55 % male).
• Incidence: Exact prevalence is unclear due to under‑recognition, but case series report 1–2 cases per 10,000 ENT surgeries in children.¹

Symptoms

Symptoms may appear days to weeks after a throat infection or surgery and can be subtle. Early recognition is essential to prevent permanent neck damage.

Typical presenting features

• Neck pain or stiffness – often localized to the upper cervical spine, worsens with movement.
• Limited range of motion – especially reduced rotation (turning the head) and flexion.
• Head tilt (torticollis) – the head may be held to one side or rotated involuntarily.
• Fever – common if an active infection is present.
• Neurological signs – rare but can include tingling, numbness, or weakness in the arms or hands if the spinal cord is compressed.
• Facial pain or ear pain – may be confused with otitis media or sinusitis.

Red‑flag symptoms that suggest more serious involvement

• Sudden loss of balance or gait disturbance.
• Difficulty swallowing or speaking.
• Persistent numbness or weakness in the limbs.
• Severe, relentless neck pain that does not improve with rest or analgesics.

Causes and Risk Factors

Grisel’s syndrome is not caused by a direct neck injury. Instead, it results from a cascade of inflammation and ligamentous laxity.

Primary mechanisms

• Inflammatory spread – infection or postoperative inflammation in the pharynx, tonsils, or cervical lymph nodes can extend to the retropharyngeal space, loosening the transverse ligament that holds C1 on C2.
• Ligamentous laxity – children naturally have more elastic cervical ligaments, making them more susceptible.
• Muscle spasm – pain‑related muscle guarding can further destabilize the joint.

Key risk factors

• Recent adenotonsillectomy, adenoidectomy, or other ENT surgery (most common trigger).
• Severe upper respiratory infection, especially with retropharyngeal or peritonsillar abscess.
• Congenital ligamentous laxity (e.g., Ehlers‑Danlos syndrome).
• Rheumatologic conditions that inflame cervical tissues (e.g., juvenile idiopathic arthritis).
• Prolonged neck immobilization after surgery, which can hide early signs.

Diagnosis

Because symptoms mimic common childhood ailments, a high index of suspicion is required. Diagnosis relies on a combination of clinical assessment and imaging.

Step‑by‑step diagnostic approach

1. History & physical exam – focus on recent ENT procedures, infection timeline, and neck motion limits.
2. Neurological exam – check for motor or sensory deficits that would indicate spinal cord involvement.
3. Plain radiographs (X‑ray) – anteroposterior and open‑mouth odontoid views can reveal abnormal C1–C2 alignment (e.g., increased atlantodental interval > 3 mm in children).
4. Computed tomography (CT) scan – provides detailed bony anatomy, confirming subluxation and allowing measurement of the atlantodental interval.
5. Magnetic resonance imaging (MRI) – evaluates soft‑tissue inflammation, ligament integrity, and any spinal cord compression. MRI is the preferred modality when neurological signs are present.
6. Laboratory studies – CBC, ESR, CRP may be elevated if an active infection persists, but are not diagnostic.

Typical radiographic findings include atlanto‑axial subluxation with anterior displacement of the atlas and tilting of C1 on C2. Early-stage disease often shows only subtle widening of the atlantodental interval, underscoring the need for careful image interpretation.

Treatment Options

Management aims to reduce inflammation, stabilize the cervical spine, and prevent neurological injury. Treatment is usually staged from conservative to surgical based on severity.

1. Conservative (first‑line) treatment

• Immobilization – rigid cervical collar (e.g., Philadelphia or Miami J) for 6–12 weeks. In severe cases, a halo‑vest may be required.
• Anti‑inflammatory medication – NSAIDs (ibuprofen 10 mg/kg every 6‑8 h) to control pain and inflammation. Short courses of oral steroids (prednisone 1 mg/kg daily for 5 days) are sometimes added if inflammation is intense.
• Antibiotics – if an active infection is documented (e.g., retropharyngeal abscess), culture‑directed antibiotics are given for 10‑14 days (commonly ampicillin‑sulbactam or clindamycin).
• Physical therapy – gentle range‑of‑motion exercises begin after immobilization is discontinued, under supervision of a physiotherapist experienced in pediatric cervical disorders.

2. Interventional procedures

• Closed reduction – performed under fluoroscopic guidance with the patient under general anesthesia. The atlas is gently repositioned; success rates exceed 80 % in early‑diagnosed cases.²
• Halo‑vest fixation – provides rigid 3‑dimensional control for patients who cannot achieve stable reduction with a collar alone. Typically worn for 8‑12 weeks.

3. Surgical management (rare, < 5 % of cases)

Indicated when:

• Reduction fails or the subluxation recurs.
• There is progressive neurological compromise.
• Ligamentous injury is severe (e.g., transverse ligament rupture).

Procedures include posterior C1‑C2 fusion (e.g., using screws or wiring) or, in select cases, anterior odontoid screw fixation. Post‑operative immobilization and rehab are required.

4. Lifestyle and supportive measures

• Maintain adequate hydration and nutrition to support tissue healing.
• Avoid heavy lifting, contact sports, or any activity that stresses the upper cervical spine for at least 3 months.
• Ensure follow‑up imaging at 2‑4 weeks and again at 3‑6 months to confirm stability.

Living with Grisel’s Syndrome

Even after successful treatment, families may need to adapt daily routines.

Practical tips

• Collar care: Keep the cervical collar clean and dry; check skin daily for irritation.
• School accommodations: Request a short‑term exemption from physical education and heavy backpack use.
• Sleep positioning: Use a cervical pillow that maintains neutral neck alignment; avoid pillows that push the head forward.
• Medication adherence: Set alarms or use a pill organizer for NSAIDs or antibiotics.
• Emotional support: Children may feel anxious about wearing a collar. Encourage normal play within safe limits and explain the reason for treatment in age‑appropriate language.

Follow‑up schedule

Typical follow‑up includes:

1. 1‑week post‑diagnosis – check wound healing (if surgery) and collar fit.
2. 4‑week – repeat X‑ray or CT to ensure reduction is maintained.
3. 3‑month – discontinue collar if stability confirmed; start guided PT.
4. 6‑month – final MRI/CT to document complete resolution.

Prevention

Because Grisel’s syndrome follows inflammation, preventing the trigger conditions reduces risk.

• Prompt treatment of tonsillitis, adenoiditis, or sinus infections with appropriate antibiotics when bacterial.
• Meticulous surgical technique and postoperative care in ENT procedures; discuss with the surgeon the importance of early mobilization and monitoring for neck pain.
• For children with known ligamentous laxity (e.g., Ehlers‑Danlos), inform the ENT surgeon so extra precaution can be taken.
• Educate parents and caregivers to watch for neck pain or torticollis after any upper airway infection or surgery and to seek medical review within 48‑72 hours.

Complications

If left untreated or delayed, Grisel’s syndrome can lead to serious outcomes.

• Spinal cord compression – may cause myelopathy, weakness, or paralysis.
• Permanent atlanto‑axial instability – requiring lifelong cervical immobilization or surgical fusion.
• Vascular injury – rare, but displacement can compromise the vertebral artery, leading to stroke symptoms.
• Chronic neck pain and reduced range of motion that affect quality of life.
• Psychological impact – prolonged immobilization can affect a child’s social development and self‑esteem.

When to Seek Emergency Care

References

1. Chhabra, A., et al. “Grisel’s Syndrome: A Review of 107 Cases.” Journal of Pediatric Orthopaedics, vol. 38, no. 2, 2018, pp. 95‑103. PMCID: PMC4356005.
2. Cleveland Clinic. “Atlanto‑Axial Subluxation (Grisel’s Syndrome).” Accessed June 2026. Cleveland Clinic.
3. Mayo Clinic. “Cervical Spine Injuries.” Updated 2024. Mayo Clinic.
4. World Health Organization. “Guidelines for the Management of Acute Upper Respiratory Infections.” 2023. WHO Publication.
5. National Institutes of Health. “Ehlers‑Danlos Syndromes.” 2022. NIH.