Delayed atlantoaxial rotatory dislocation in a child with Crohn’s disease: illustrative case

Abstract

BACKGROUND

Atlantoaxial rotatory dislocation (AARD) in children may be caused by neck trauma or an upper respiratory tract infection. Here the authors describe the very rare association between inflammatory bowel disease and AARD in a child.

OBSERVATIONS

A 7-year-old girl presented with an 11-month history of torticollis that occurred spontaneously without a trauma context. Her history revealed a recent diagnosis of Crohn’s disease. Physical exam of the cervical spine revealed a “cock-robin” posture. Neck radiography and three-dimensional computed tomography reconstruction established the diagnosis of AARD. Given the duration of the symptoms and failure of previous conservative treatments, the patient was taken to the operating room and underwent open reduction through posterior approach with a C1–2 fusion according to the Harms technique. The torticollis resolved with no recurrence at the last follow-up and minimal restriction of rotation.

LESSONS

This is the third report to describe the very rare association between inflammatory bowel disease and AARD but at a very early age, the youngest in the literature. One should be aware of such association as early diagnosis may prevent aggressive surgical management.

Atlantoaxial rotatory dislocation (AARD) is defined as a subluxation or dislocation of the atlantoaxial joint. It is an example of torticollis and is commonly found in children who may present with neck pain, reduced range of motion, and head tilt.¹ It may occur after a minor trauma or an upper respiratory tract infection; however, its association with inflammatory bowel diseases has been rarely reported.

We report the third pediatric case of a delayed AARD associated with Crohn’s disease in a 7-year-old child, which has never been recorded at such a young age. The cause-effect relationship between the 2 pathologies is discussed and the different lines of management are outlined.

Illustrative Case

A 7-year-old girl presented with an 11-month history of torticollis that occurred spontaneously without a trauma context; in fact, the family denied any fall or accident and no upper respiratory tract infection context could be retrieved either. However, a long history of abdominal pain was mentioned with a recent diagnosis of Crohn’s disease. Physical examination of the cervical spine revealed a “cock-robin” posture with left inclination of the head and right-sided rotation (Fig. 1A). Anteroposterior open-mouth and lateral radiograph were performed (Fig. 1B and C) and computed tomography (CT) scans with three-dimensional (3D) reconstruction confirmed left C1–2 articular facet dislocation (Fig. 1D–F). Given the duration of the dislocation and the failure of conservative management, including muscle relaxants, cervical collar, and physical therapy with traction, the patient was taken to the operating room and a closed reduction was attempted, first with the use of the transoral technique where the index finger of the dominant hand applies direct pressure to the subluxated lateral mass of C1 (Fig. 2A), followed by traction using Gardner-Wells tongs. However, the dislocation was fixed and external maneuvers failed.

FIG. 1.

Clinical photograph depicting the cock-robin posture with left inclination of the head and right-sided rotation (A). Radiograph confirming the left lateral tilt and rotation of the head to the right side in the anterior-posterior open-mouth view with suspicious asymmetry of the odontoid–C1 lateral mass interspace (B). Lateral radiograph showing C1–2 kyphosis with compensatory hyperlordosis in the subaxial cervical spine (C). CT scans (D and E) with 3D reconstruction (F) confirming the atlantoaxial dislocation on the left side.

FIG. 2.

Intraoperative fluoroscopy showing the attempt at using the transoral technique to reduce the dislocation (A). Intraoperative images showing patient positioning with the Mayfield clamp (B), C1 and C2 exposure after muscles dissection (C), removal of the posterior part of the C1 arch on the left side to access the lateral mass (D, arrow), reduction of the dislocation on the left side with a reducer (E), and the final construct with the posteriorly attached iliac crest bone graft (F).

Therefore, surgical management was decided in the same setting with C1–2 posterior reduction and fixation with pedicle screws. The patient was installed in a Mayfield clamp (Fig. 2B). After muscles dissection, the upper cervical spine was exposed subperiosteally from C1 to C2 (Fig. 2C). Under C-arm guidance, C2 pedicle screws and a C1 right lateral mass screw were inserted. A C1 left lateral mass screw was inserted after removal of the inferior part of the C1 arch on the left side to gain proper access (Fig. 2D). To release the fixed dislocation, facet manipulation was performed and the flat edge of a small osteotome was inserted into the space between the C1 and C2 joints and rotated (turned vertically) to achieve a distraction effect and open the interfacet space; during this maneuver, an audible crack could be sensed. After fixation of a titanium rod on the C2 left screw, a rod reducer was applied on the left C1 screw and the dislocation was progressively reduced under direct macroscopic vision (Fig. 2E). After the left C1 screw was tightened, the construct was completed by a second rod on the right side. Monocortical autologous bone graft from the iliac crest was harvested and shaped to fit on the C2 arch and posterior to the C1 arch and was attached to the construct with strong sutures (Fig. 2F). Total blood loss was 100 mL, and no subfascial drain was inserted.

The patient could walk on day 1 with a neck collar. Clinically, her torticollis resolved and a postoperative CT scan demonstrated reduction of the dislocation confirmed on 3D images (Fig. 3A). A 6-month CT scan showed solid C1–2 fusion (Fig. 3B). She showed a stable radiological result at 2 years (Fig. 3C) with a normal neck posture (Fig. 3D–F).

FIG. 3.

Postoperative 3D CT scan confirming reduction of the dislocation and showing the print (or depression) left by the C1 lateral mass on the deformed C2 facet (A, arrows). Six-month CT scan demonstrating solid C1–2 fusion at the level of the posterior elements (B). Radiograph at 2 years showing normal sagittal alignment (C). Clinical photographs at 2 years showing normal neck posture with satisfactory range of motion (D–F).

Discussion

Atlantoaxial rotatory dislocation is the most common pediatric cervical spine injury, causing torticollis and neck pain, which is due to several factors, including spinal ligamentous hyperlaxity and a proportionally larger head with minimally developed neck musculature.² It can occur after a minor trauma or be associated with an infection of the upper respiratory tract (Grisel’s syndrome); however, its association with an inflammatory bowel disease has been rarely reported.³

Presumably, any condition that causes inflammation in the head or neck may be implicated in the onset of AARD, whether coming from a local etiology such as pharyngitis, tonsillitis, cervical adenitis, or from a more global pathology such as autoimmune disorders including juvenile idiopathic arthritis, ulcerative colitis, and HLA-B27–positive seronegative spondylarthropathy.⁴ In fact, arthritis associated with inflammatory bowel disease may cause chronic inflammation and erosive changes in the ligaments and associated joints leading to ligamentous laxity and joint instability, increasing the susceptibility to torsion-related injuries.⁵

Depending on the time of presentation, AARD can be categorized into 3 types: acute when presentation is less than 1 month, subacute when presentation is between 1 and 3 months, chronic when presentation is more than 3 months. It is usually classified according to Fielding and Hawkins.⁶

Most studies agree that the treatment of acute AARD should be conservative, including medication, closed manipulation, transoral technique,⁷ or cervical traction followed by halo-vest bracing.⁸ However, in chronic AARD, the aforementioned methods are often unsuccessful, with a high risk of developing recurrent subluxation after an initial closed reduction and therefore requiring surgical management.³

The gold standard of surgical management consists of C1–2 posterior arthrodesis either after closed reduction or open reduction in case of rigid or fixed dislocation. A variety of posterior fusion techniques have been advocated, such as posterior wiring method described by Gallie,⁹ the transarticular screw technique reported by Magerl, and the C1–2 lateral mass fixation described by Goel and Laheri¹⁰ and later by Harms and Melcher.¹¹

Our patient is the third case of AARD associated with inflammatory bowel disease in the literature. She presented with a chronic AARD that went undiagnosed for 11 months, but the recent diagnosis of Crohn’s disease after a long history of abdominal pain triggered the possible association between the 2 pathologies after discussion with the pediatrician. Unfortunately, being resident in a rural area, the patient’s access to medical care was limited, with an initial diagnosis of muscular torticollis that was treated conservatively with no investigations done. This explains the delay in establishing the different diagnosis, which subsequently delayed its proper management.

The association of AARD with inflammatory bowel disease has been reported only twice in the literature in children and once in adults. For the latter,¹² the AARD was the initial manifestation of Crohn’s disease and the patient’s symptoms resolved with corticosteroids and sulfasalazine administration. With regard to the 2 pediatric cases, the first one¹³ was a 9-year-old boy with previously diagnosed Crohn’s disease and an acute AARD presentation of 1 week; he was managed conservatively with head traction, which enabled complete torticollis recovery. Our patient, diagnosed at age 7, is currently the youngest reported case in the literature combining Crohn’s disease and AARD; in addition, it was a delayed presentation requiring surgical management at a very young age. The second one³ was a 17-year-old boy with a known diagnosis of ulcerative colitis. He was discovered with an AARD that went undiagnosed for over 1 year, similarly to our case; therefore, surgical management was performed with C1–2 posterior fusion using C1 lateral mass screws and C2 hooks (unlike our case where C2 pedicle screws were used), achieving partial reduction because of facet fusion with a clinically remaining torticollis. Both cases are summarized in Table 1.

TABLE 1.

Previous reports of atlantoaxial dislocation associated with inflammatory bowel disease in the literature

Authors & Year	Age (yrs)	Sex	Duration of Sxs (days)	Associated Pathology	Treatment	Correction
Majahan et al., 200113	9	M	7	Crohn’s disease	Traction	Complete
Tauchi et al., 20133	17	M	385	Ulcerative colitis	C1–2 fusion	Partial
Present study	7	F	330	Crohn’s disease	C1–2 fusion	Complete

Sxs = symptoms.

Papers in the literature could not describe an approximation on how long the inflammatory process may take in order to cause damage leading to joint instability; in fact, the pathogenesis of extraintestinal manifestations of inflammatory bowel disease is not well understood. In the current report, long discussion with the pediatrician led to the possible association between the 2 pathologies (Crohn’s and AARD), given their concomitant development, the fact that no etiology for the AARD such as trauma or infection could be found, and the unlikelihood of an eventual random correlation for such entities in children.

In the case of our patient, given the duration of the dislocation, its fixed character, and failure of conservative treatments including manipulation in the operating room, surgical reduction and fusion were performed, which enabled satisfactory clinical recovery and radiological outcome at 2 years. Such an outcome could not be achieved in the other similar case in the literature³ where surgical management was performed, as the fixed facet could not be fully released.

Once the diagnosis of Crohn’s disease has been established, early detection of an eventual atlantoaxial dislocation may be done through education of the parents about the clinical signs that may be seen in the case of AARD (neck pain, head tilt or rotation, limited range of motion) with the advice of coming to the spine clinic whenever any suspicious sign is noticed. Also, regular visits to the spine clinic (e.g., every 6 months) with clinical exam looking for signs of AARD and eventual radiographs to rule out any onset of dislocation could help in early detection of the pathology. And when detected early, conservative treatment should represent the first line of management of AARD with the use of muscle relaxants, cervical collar, and physical therapy with traction. In case of no response after 5 days, closed reduction under general anesthesia followed by rigid collar immobilization for 4 weeks should be performed.

Treatment of inflammatory bowel disease and its musculoskeletal manifestations comprises a combination of physiotherapy, corticosteroids (intraarticular/systemic), anti-inflammatory, and anti–tumor necrosis factor medication.¹⁴ Therapies may prevent the development of extraintestinal manifestations or may help stabilize them, avoiding their worsening with specific protocols being applied in the pediatric population.^15,16

Observations

Trauma remains the major cause of AARD in children, but this entity may be also associated with an infection of the upper respiratory tract.³ In reality, any condition that results in inflammation in the head or neck may be implicated in the onset of AARD, whether coming from a local etiology or from a more global pathology such as Crohn’s disease,⁴ as observed in the current case. The C1–C2 joint presents a complex anatomy and inflammatory bowel disease may initiate chronic inflammation and erosive changes in the ligaments and associated joints in the upper cervical area, leading to ligamentous laxity and joint instability, increasing the susceptibility to subsequent dislocations.

Lessons

Early recognition and treatment of an inflammatory pathology such as Crohn’s disease during childhood may prevent development of a concomitant uncommon musculoskeletal disease such as atlantoaxial dislocation, with its subsequent aggressive management when a delayed diagnosis is established. Considering the limitations associated with a single case report for such a condition, a more important number of patients is warranted in order to better understand the pathophysiology of such a complex association between 2 rare diseases and the true effectiveness of treating one in order to prevent the occurrence of the other.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: Bourghli, Al Araki, Konbaz, Almusrea, Obeid. Acquisition of data: Al Araki, Almusrea. Analysis and interpretation of data: Boissière, Obeid. Drafting of the article: Bourghli, Konbaz, Boissière. Critically revising the article: Konbaz, Almusrea, Boissière, Obeid. Approved the final version of the manuscript on behalf of all authors: Bourghli. Administrative/technical/material support: Al Araki.