British Journal of Plastic Surgery (1989), 47~122-123 0 1989 The Trustees of British Association of Plastic Surgeons

Rotatory atlantoaxial subluxation following pharyngoplasty

P. A. EADIE, R. MORAN, E. E. FOGARTYand G. E. EDWARDS

Departments of Plastic Surgery and Orthopaedic Surgery, Our Lady’s Hospital for Sick Children, Dublin

Summary-Atlantoaxial rotatory subluxation often presents in childhood as a stiff, painful neck with associated tortico!lis. It can occur spontaneously following minor trauma or an inflammatory process in the cervical tissues. Failure to recognise the condition may result in a fixed rotatory subluxation which can be difficult to treat.

The authors report on two cases of atlantoaxial rotatory subluxation which occurred following pharyngoplasty and discuss the clinical presentation and management of these cases. The literature on this subject is briefly reviewed.

Pharyngoplasty requires hyperextension of the neck and maintenance of that position for the duration of the procedure. It also causes a local inflammatory response in the tissues. Both of these factors have been implicated as causes of atlantoaxial subluxa- tion (Sullivan, 1949; Zook and Salmon, 1977).

The authors report on two cases of this condition associated with pharyngoplasty in order to draw attention to a rare complication which, untreated, can have severe consequences for both the patient and the surgeon.

Case reports

Case I

A 6-year-old girl with Pierre Robin syndrome was admitted for pharyngoplasty. Her cleft palate had been repaired uneventfully at 11 months. Her tonsils and adenoids were removed at 5 years, after which she developed hypernasality. Endotracheal intubation prior to pharyngoplasty was difficult and required the use of an introducer.

A superiorly based pharyngeal flap was elevated for the pharyngoplasty. Postoperatively she complained of some neck pain and had a torticollis. Initially it was felt that this was due to discomfort post-surgery. Plain films of the cervical spine, tomography and cineroentgenogra- phy showed no abnormality. Two weeks postoperatively a C-T scan was performed which showed atlantoaxial subluxation.

The patient was commenced on halter traction and gradually the torticollis resolved over the subsequent 2 weeks. X-ray revealed Chat the subluxation had been reduced. The patient was then immobilised in a hard cervical collar for a further 6 weeks. At follow-up her symptoms and signs had resolved completely.

Case 2

An &year-old girl was admitted for pharyngoplasty. Surgery was uneventful. She had a low grade pyrexia on the first postoperative day. On the 2nd day she complained of neck pain and clinically had a torticollis with marked restriction of neck movement. X-ray confirmed rotatory subluxation of the cervical spine (Fig. 1).

Her symptoms and signs settled over a 4-day period in halter traction and X-ray revealed that the subluxation had been reduced (Fig. 2). She was then immobilised in a hard cervical collar for one month. She was taken out of the collar at that stage but complained of pins and needles in both hands and had the collar reapplied for a further 4 weeks. She made a fulI recovery.

Discussion

The articulation between the atlas and axis com- prises of three synovial joints: the lateral atlanto- axial joints, and an articulation between the dens and the anterior arch and transverse ligament of the atlas. Atlantoaxial stability is maintained primarily by the transverse ligament which prevents anterior shift of the atlas on the axis and secondarily by the alar ligaments which limit the rotatory movement (Gray’s Anatomy, 1973).

Rotatory atlantoaxial subluxation in childhood has a known association with inflammatory condi- tions of the cervical tissues (Sullivan, 1949). It has been described following tonsillitis, pharyngitis and juvenile rheumatoid arthritis. It is thought that these inflammatory conditions result in hyperaemia and laxity of the ligamentous structures, permitting the atlas to rotate anteriorly on the axis (Martin, 1942). The degree of rotatory shift can vary from

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ROTATORY ATLANTOAXIAL SUBLUXATION FOLLOWING PHARYNGOPLASTY 723

Fig. 1 Fig. 2

Figure I-Atlantoaxial rotatory subluxation. There is obliteration of the right lateral atlantoaxial joint (arrow) due to subluxation. The left lateral mass of Cl has rotated anteriorly while the right lateral mass has rotated posteriorly. Figure 2-The subluxation has been reduced following a period of halter traction.

simple rotatory displacement without anterior shift of the atlas, to complete dislocation of the lateral atlantoaxial joints (Fielding and Hawkins, 1977).

There is a known association between congenital cervical anomalies and cleft palate (Zook and Salmon, 1977). Cleft palate repair in patients with Klippel-Feil syndrome is often difficult because reduced neck extension hinders intubation and surgical exposure is often limited. Rotatory atlanto- axial subluxation is another cervical problem associated with cleft palate and pharyngeal surgery.

Typical clinical features include neck pain, stiffness and torticollis. The torticollis has been likened to a robin listening for a worm-the “cock- robin” position (Rockwood et al., 1984).

Diagnosis can be made by open mouth antero- posterior and lateral views of the cervical spine. Fielding and Hawkins (1977) found lateral cinero- entgenography the most useful way to make the diagnosis. However, in a young child with a stiff, sore neck all of these procedures can be difficult. C-T scan of the cervical spine has been shown to be valuable, providing the appropriate information with minimal patient discomfort (Geehr et al., 1978). It was especially useful in our first case.

Most cases will respond quickly to halter traction followed by a period of immobilisation in a cervical collar. If the diagnosis is missed, these patients may develop a fixed rotatory subluxation for which spinal fusion is often necessary. Awareness of this condition by surgeons involved in cleft palate and pharyngeal surgery should lead to its early recog- nition and treatment, preventing a fixed deformity occurring.

References

Fielding, J. W. and Hawkins, R. J. (1977). Atlanto-axial rotatory fixation. Journal of Bone and Joint Surgery. 59A, 37.

Geehr, R. B., Rothman, S. L. G. and Kier, E. L. (1978). The role of computed tomography in the evaluation of upper cervical spine pathology. Computerized Tomographv, 2,79.

Gray’s Anatomy (1973). 35th Edition. Edited by Warwick, R. and Williams, P. L. Edinburgh: Churchill Livingstone.

Martin, R. C. (1942). Atlas-axis dislocation following cervical infection. Journal of the American Medical Association, 118, 874.

Rockwoad, C. A., Wilkins, K. E. and King, R. E. ( 1984). Fractures in Children. 2nd Edition. Philadelphia : J. B. Lippincott.

Sullivan, A. W. (1949). Subluxation of the atlanto-axial joint, sequel to inflammatory process in the neck. Journal qf Pediatrics, 35,45 1.

Zook, E. G. and Salmon, J. H. (1977). Anomalies of the cervical spine in the cleft palate patient. Plastic and Reconsfructice Surgery, 31, 179.

The Authors

Patricia A. Eadie, FRCSI, Registrar in Plastic Surgery, St James’s Hospital and Our Lady’s Hospital for Sick Children, Dublin.

Raymond Moran, FRCSI, Senior Registrar in Orthopaedic Surgery, St Mary’s Orthopaedic Hospital, Dublin.

Esmond E. Fogarty, FRCSI, FRACS, Consultant Orthopaedic Surgeon, Our Lady’s Hospital for Sick Children, Dublin.

Gerald E. Edwards, FRCSI, Consultant Plastic Surgeon, St James’s Hospital and Our Lady’s Hospital for Sick Children, Dublin.

Requests for reprints to: MS P. A. Eadie, Department of Plastic Surgery, St James’s Hospital, Dublin 8, Ireland.

Paper received 27 September 1988. Accepted 23 January 1989.