Case Reports

Mild Grisel Syndrome: Expanding the Differential for Posttonsillectomy Adenoidectomy Symptoms

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Background: Tonsillectomy with or without adenoidectomy is the second most common pediatric surgical procedure in the United States with up to 97% performed as an outpatient. 1,2 While it is largely a safe procedure, several complications have been described and are encountered in the emergency department and primary care setting.

Presentation: A 29-month child presented to the emergency department with neck stiffness 10 days after tonsillectomy and adenoidectomy. A computed tomography scan of the neck limited by motion artifact was unrevealing, but a consult to the pediatric otolaryngologist generated concern for Grisel syndrome, the atraumatic rotary subluxation of the atlantoaxial joint. While surgical intervention can be required, the patient had an uncomplicated clinical course and the anomalous neck posture resolved with time and anti-inflammatories alone.

Conclusions: Keeping a broad differential for posttonsil-lectomy and adenoidectomy patient concerns is important for the clinician. Serious, life-threatening complications can arise from Grisel syndrome while good functional outcomes can be achieved with timely and appropriate treatment.


 

References

Tonsillectomy with or without adenoidectomy (T&A) is the second most common pediatric surgical procedure in the United States.1 It is most often performed during childhood between 5 and 8 years of age with a second peak observed between 17 and 21 years of age in the adolescent and young adult populations.2 While recurrent tonsillitis has been traditionally associated with tonsillectomy, sleep disordered breathing with obstructive sleep apnea is now the primary indication for the procedure.1

Up to 97% of T&As are performed as an outpatient same-day surgery not requiring inpatient admission.2 Although largely a safe and routinely performed surgery, several complications have been described. Due to the outpatient nature of the procedure, the complications are often encountered in the emergency department (ED) and sometimes in primary care settings. Common complications (outside of the perioperative time frame) include nausea, vomiting, otalgia, odynophagia, infection of the throat (broadly), and hemorrhage; uncommon complications include subcutaneous emphysema, taste disorders, and Eagle syndrome. Some complications are rarer still and carry significant morbidity and even mortality, including mediastinitis, cervical osteomyelitis, and Grisel syndrome.3 The following case encourages the clinician to expand the differential for a patient presenting after T&A.

Case Presentation

A child aged < 3 years was brought to the ED by their mother. She reported neck pain and stiffness 10 days after T&A with concurrent tympanostomy tube placement at an outside pediatric hospital. At triage, their heart rate was 94 bpm, temperature was 98.2 °F, respiratory rate, 22 breaths per minute, and oxygen saturation, 97% on room air. The mother of the patient (MOP) had been giving the prescribed oral liquid formulations of ibuprofen and acetaminophen with hydrocodone as directed. No drug allergies were reported, and immunizations were up to date for age. Other medical and surgical history included eczema and remote cutaneous hemangioma resection. The patient lived at home with 2 parents and was not exposed to smoke; their family history was noncontributory.

Since the surgery, the MOP had noticed constant and increasing neck stiffness, specifically with looking up and down but not side to side. She also had noticed swelling behind both ears. She reported no substantial decrease in intake by mouth or decrease in urine or bowel frequency. On review of systems, she reported no fever, vomiting, difficulty breathing, bleeding from the mouth or nose, eye or ear drainage, or rash.

On physical examination, the patient was alert and in no acute distress; active and playful on an electronic device but was notably not moving their head, which was held in a forward-looking position without any signs of trauma. When asked, the child would not flex or extend their neck but would rotate a few degrees from neutral to both sides. Even with moving the electronic device up and down in space, no active neck extension or flexion could be elicited. The examination of the head, eyes, ears, nose, and throat was otherwise only remarkable for palpable and mildly tender postauricular lymph nodes and diffuse erythema in the posterior pharynx. Cardiopulmonary, abdominal, skin, and extremity examinations were unremarkable.

With concern for an infectious process, the physician ordered blood chemistry and hematology tests along with neck radiography. While awaiting the results, the patient was given a weight-based bolus of normal saline, and the home pain regimen was administered. An attempt was made to passively flex and extend the neck as the child slept in their mother’s arms, but the patient immediately awoke and began to cry.

All values of the comprehensive metabolic panel were within normal limits except for a slight elevation in the blood urea nitrogen to 21 mg/dL and glucose to 159 mg/dL. The complete blood count was unrevealing. The computed tomography (CT) scan with contrast of the soft tissues of the neck was limited by motion artifact but showed a head held in axial rotation with soft tissue irregularity in the anterior aspect of the adenoids (Figure 1). There was what appeared to be normal lymphadenopathy in the hypopharynx, but the soft tissues were otherwise unremarkable.

Sagittal Computed Tomography in Soft Tissue Window Showing Artifact in the Hypopharynx

The on-call pediatric otolaryngologist at the hospital where the procedure was performed was paged. On hearing the details of the case, the specialist was concerned for Grisel syndrome and requested to see the patient in their facility. No additional recommendations for care were provided; the mother was updated and agreed to transfer. The patient was comfortable and stable with repeat vitals as follows: heart rate, 86 beats per minute, blood pressure, 99/62, temperature, 98.3 °F, respiratory rate, 20 breaths per minute, and oxygen saturation, 99% on room air.

On arrival at the receiving facility, the emergency team performed a history and physical that revealed no significant changes from the initial evaluation. They then facilitated evaluation by the pediatric otolaryngologist who conducted a more directed physical examination. Decreased active and passive range of motion (ROM) of the neck without rotatory restriction was again noted. They also observed scant fibrinous exudate within the oropharynx and tonsillar fossa, which was normal in the setting of the recent surgery. They recommended additional analgesia with intramuscular ketorolac, weight-based dosing at 1 mg/kg.

With repeat examination after this additional analgesic, ROM of the neck first passive then active had improved. The patient was then discharged to follow up in the coming days with instructions to continue the pain and anti-inflammatory regimen. They were not started on an antibiotic at that time nor were they placed in a cervical collar. At the follow-up, the MOP reported persistence of neck stiffness for a few days initially but then observed slow improvement. By postoperative day 18, the stiffness had resolved. No other follow-up or referrals related to this issue were readily apparent in review of the patient’s health record.

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