A S i n g l e C a s e S t u d y Effectiveness of Cervical Stabilisation Training and Correction of Muscle Imbalance, following reduction of Atlanto-Axial Rotatory Subluxation: A single case study DEBBIE D A V ID S O NA B S T R A C T : A tlanto-axial rotatory subluxation is a rare, con troversial and frequen tly m isdiagn osed condition occurring p rim a rily in children. A single case study design w as u sed to evalu ate the effectiveness o f cervical stabilisation training and correction o f m uscle im balance, fo llo w in g reduction o f this condition, in a nine y e a r o ld boy. The study w as conducted over a six w eek p e r io d during which the su bject m aintained a daily d ia ry to record his sym ptom s. The program m e con sisted o f various m uscle relaxation and lengthening techniques, endurance training o f the deep cervica l fle x o rs an d low er scapu lar stabilisers, as w ell as p o s ­ tural re-education. N o treatm ent w as d irected a t the a rticu la r com ponent. The p a tien ts signs and sym ptom s w ere g reatly redu ced during the study p erio d . This study supports the im portance o f correcting the m uscle dysfunction com ponent in cervicogen ic p a in sufferers. INTRODUCTION Atlanto-axial rotatory subluxation is a rare, poorly understood condition that occurs primarily in children (Fielding & Hawkins, 1977; Phillips et al, 1989; Robert & Hensinger, 1993). The subluxa­ tion occurs when the lateral mass of the atlas rotates forward on the facet o f the axis when turning the head and remains fixed in this position on returning the head to neutral (Henrys et al, 1977). Fielding & Hawkins (1977) classified rotatory subluxation into four types according to the degree of anterior insta­ bility. An illustrated case in each classi­ fication is demonstrated below (fig 1). Although the rotatory subluxation is usually short-lived and easily correctable (Phillips et al, 1989; Robert & Hensinger, 1993), Fielding & H awkins (1977) described a series of patients in whom the diagnosis was made late and the subluxa­ tion was irreducible. They termed this outcome rotatory fixation and recom­ m ended atlanto-axial arthrodesis for relief o f symptoms. The onset is usually associated with an upper respiratory tract infection (URTI) or minor trauma (Fielding & Hawkins, 1977; Phillips et al, 1989; Henrys et al, 1978). The exact aetiology o f the condi­ tion is unknown, but is thought to be due to increased laxity or deficiency of the alar and transverse ligaments and of the capsular structures secondary to inflam­ mation, infection or trauma (Phillips et al, CORRESPONDENCE: Debbie Davidson (Shuter) BSc (Physiotherapy) Witwatersrand Suite 168, Postnet XII, Craighall 2024 (H) 011-880-6979 (W) 011-442-8233 (C) 083-457-7558 1989; Dvorak & Panjabi, 1987; Roach et al, 1984; Aspinall, 1990). The patient presents with a torticollis deformity which is typically likened to a robin listening for a worm, the so-called “cocked robin” position (Fielding & Hawkins, 1977; Robert & Hensinger, 1993; Phillips et al, 1989). Unlike patients who have muscular torticollis, the patient presents with spasm in the sternocleidomastoid muscle on the side to which the chin is rotated, suggesting an attempt to reduce the deformity (Phillips et al, 1989). D iagnosis is prim arily dependant on history, clinical findings and dynamic com puted tom ography (Fielding et al, 1978). Whilst the condition exists, atlanto­ axial stability may be compromised and even minor injury to the neck may pro­ duce catastrophic results including spinal cord com pression and brainstem ischaemia (Phillips et al, 1989; Robert & Hensinger, 1993; Henrys et al, 1977). It is well documented that trauma to the cervical spine is one o f the most com­ monly cited provocative causes of headache (Beeton & Jull, 1994; Bogduk, 1986; Edeling, 1982; Jull 1988). This is supported by the frequent accounts of frontal and occipital headaches following reduction of the subluxation, that were found hidden within the case reports of the literature. It is postulated that these headaches arise from the ligaments and the atlanto-axial joints that are exposed to excessive mechanical stress as a result of the subluxation (Fielding & Hawkins, 1977; Phillips et al, 1989; Robert & Hensinger, 1993). No literature regarding physiotherapy m anagem ent follow ing reduction could be found. Studies by Watson (1990) have shown that cervicogenic headache sufferers dem onstrate weakness and loss of endurance of the deep cervical flexors Figure 1. Vf V T y p e I - r o ta to r y s u b lu x a tio n w i t h n o a n te r io r d is p la c e m e n t. T y p e II - r o ta to r y s u b lu x a tio n w i t h a n te r io r d is p la c e m e n t o f th r e e to fiv e m illim e tre s . T y p e III - r o ta to r y s u b lu x a tio n w ith a n te r io r d is p la c e m e n t o f m o r e th a n fiv e m illim e tre s . T y p e IV - r o ta to r y s u b lu x a tio n w i t h p o s te r io r d is p la c e m e n t. 4 SA J o u r n a l o f Ph ysio t h er a py 1998 V o l 54 No 3 R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) and often exhibit a forward head posture as well as striking muscle imbalance of the ‘proximal crossed syndrome’ type described by Janda (1988). Clinical treat­ ment of muscle imbalance using cervical stabilisation training is giving very encouraging long term results in patients with chronic cervical pain syndromes (Beeton & Jull, 1994; Watson, 1990; Jull e ta l, 1995). On the basis of the above information, this study was designed to: a) Describe this rare condition to other physiotherapists and highlight the impor­ tance of prompt recognition and referral. b) To determine if cervical stabilisation training and correction of muscle imba­ lance would alter the patient’s signs and symptoms following reduction of atlanto­ axial rotatory subluxation. CASE REPORT INITIAL EXAMINATION Subjective Assessment A nine year old boy, actively involved in sports, presented with severe left sided neck pain and continuous headache asso­ ciated with dizzy spells. He described his pain as “vicious” in the nape of his neck, extending as a “dull, dizzy” pain over the top of his head. He had fallen whilst waterskiing two days previously and noticed slight pain on the left side of his neck. He awoke the following morning with a typical torticollis. Four years previously he fell from a 2 metre high wall onto his head, landing on a concrete surface. He sustained a left frontal skull fracture and a chronic sub­ dural haemorrhage. According to the patient’s mother he had since complained of no related symptoms. No medical practitioner had been seen for this condition and no X-rays had been taken. Objective Assessment The fact that there were no X-rays, no doctor’s referral and a history of severe previous trauma indicated the need for extreme caution to be taken in examina­ tion. On observation, the patient had a severe torticollis with lateral flexion to the left, rotation to the right and slight flexion (Figure 2). The right sternocleido­ mastoid muscle appeared to be in severe spasm. Due to the fact that the patient was in acute pain and unwilling to move his neck, the objective assessment was limit­ ed to a neurological examination, that essentially revealed no abnormalities. He was referred urgently to an orthopaedic surgeon. Figure 2. Typical "cocked robin" positio n o f rotatory subluxation The patient was diagnosed as having a type II atlanto-axial rotatory subluxation which was confirmed with dynamic com­ puted tomography. Reduction of the sub­ luxation was achieved with bedrest, anti­ inflammatories and strong muscle relax­ ant medication, followed by immobilisa­ tion in a rigid cervical brace for ten weeks to allow for ligamentous healing. EXAMINATION FOLLOWING REDUCTION AND IMMOBILISATION Subjective assessment The patient now described three dis­ tinct intermittent symptoms which started following removal of the cervical brace three weeks previously (Figure 3). Symptom 1 - was aggravated by sus­ tained postures and occurred mainly at school, playing on the computer or whilst watching TV, lasting anything from two hours to the whole day. Symptom 2 - occurred with awkward quick movements involving extreme flex­ ion, for example, removing a T-shirt over his head. The pain only lasted for a “split” second. Symptom 3 - was aggravated by any­ thing that stretched the left side of his neck, for example turning to the right, serving at tennis and the end of his golf swing. The pain only lasted a few min­ utes. He also commented that his head felt “too heavy for his neck” and his mother added that he frequently supported his head with his left hand, especially when watching TV or doing homework. A soft collar was to be worn at specified risky times during the day and participation in contact sports was not allowed. Objective assessment On observation the patient showed pos­ tural deformities of increased thoracic kyphosis, rounded shoulders and a severe “poking chin”. There was noticeable scapular winging bilaterally. The neck appeared to be held in slight lateral flex­ ion to the left, and shortening and spasm was noted in the left scalenes and upper trapezius area. Both rotation and lateral flexion to the right were limited to half range reproduc­ ing symptom three. Cervical flexion was limited to five centimetres from chin to sternum also reproducing symptom three. Cervical extension and left rotation and lateral flexion were full range and pain free with gentle overpressure. Symptom 1 was not reproduced as it occurred following greater than half-an- hour of a sustained posture. It was also deemed unnecessary to reproduce symp­ tom 2 due to its severity, and the nature of the condition. Neural tension tests and the neurologi­ cal examination were unremarkable. The vertebral artery tests were not completed due to limitation of the patient’s pain free range of motion. On palpation active trigger points, pro­ tective spasm and shortening were found in the left scalenes, levator scapulae, upper fibres of trapezius (UFT) and the suboccipital muscles. Gentle pressure applied to left first rib posteriorly repro­ duced symptom 3. Central, left and right unilateral postero-anterior movements of the second cervical vertebra produced sharp local increases in pain. Intermittent dull headache asso­ ciated with dizziness and drow­ siness. 2/5 Intermittent “Sharp stabbing pain” - “ electric shock” 4,5/5 Intermittent “Burning, straining, pulling” pain. 3/5 SA J o u r n a l o f Ph y sio th era py 1998 V o l 54 No 3 5 R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) TREATMENT The study was carried out for six weeks, during which the subject, with the help of his mother, maintained a daily diary to record his symptoms. Informa­ tion documented included the frequency, duration, intensity and any known causative factor for the symptoms. The endurance capacity of the deep cervical flexors (DCF) was measured as the number of times the patient could hold a chin tuck position for ten seconds in the supine position using a biofeedback pressure sensor (Figure 4). Similarly the lower scapular stabilisers (LSS) were assessed by the number of times the patient could hold a static inner range contraction for ten seconds in the stan­ dard muscle test position. These are the standard clinical tests described by Beeton & Jull (1994) who regarded an endurance limit of a minimum of ten 10- second holds as a normal endurance level. The tests were terminated when the sub­ ject was either unable to maintain the contraction or substitution strategies by other muscles were observed. Treatment was aimed at restoring mus­ cle balance. Weeks 1 and 2 The patient’s endurance capacity mea­ surements of the: a) DCF was four 10-second holds increasing the pressure in the cuff from 20 to 28mm Hg before substitution strate­ gies by the sternocleidomastoid muscles was observed. b) LSS was five 10-second holds before loss of control of the scapular posi­ tion was observed. The patient attended physiotherapy ses­ sions twice weekly. A moist hot pack was applied for fifteen minutes. Following this, gentle muscle lengthening tech­ niques including stretching, trigger point therapy and m yofascial release were applied to the left UFT, scalenes, levator scapulae and the suboccipital extensors. H e was to perform a hom e programme twice daily which included: a) four gentle muscle stretching exercises for the left UFT, scalenes, levator scapu­ lae and the suboccipital extensors. Each stretch was to be done three times and held for thirty seconds. b) five 10-second holds using the chin tuck action, with the assistance of feed­ back from the stabiliser. c) five 10-second static contractions of the LSS. Weeks 3 and 4 Cervical rotation and lateral flexion to the right were greatly improved. They were full range reproducing symptom 3 only at the end of range. Cervical flexion was full range and pain free with gentle overpressure. The subject’s endurance capacity of the: a) DCF improved to seven 10-second holds. b) LSS improved to nine 10-second holds. Physiotherapy sessions continued twice weekly with similar treatment. Muscle lengthening techniques were more vigor­ ous and taken further into range. A hold- relax proprioceptive neuromuscular facil­ itation technique was added for each spe­ cific tight muscle group (Janda 1988). Postural re-education was commenced and correct neutral posture was taught in sitting and standing. Visual and sensory awareness of the new corrected posture was provided. H is hom e program m e was altered to: a) eight 10-second holds with the chin tuck action. b) eight 10-second static contractions of the LSS. c) practising the correct neutral posture at specific times during the day such as in the car, watching TV, in the shower and at school. d) continuation of the four stretching exercises. Weeks 5 and 6 All cervical active movem ents were full range and pain free with gentle overpressure. A complete ver­ tebral artery test was now performed and revealed no abnormalities. On palpation the left upper fibres of trapezius, scalenes, levator scapulae and the deep cervical extensors no longer felt tight or appeared to be in spasm. The subject’s endurance capacity o f the: a) DCF improved to ten 10-sec­ ond holds. b) LSS improved to twelve 10- second holds. Physiotherapy sessions were reduced to once a week and were similar. A postural correction and reversal exercise in prone described by Jull (1988) was taught to the patient in order to gain good facilitation and co-contraction of the lower scapular stabilisers and the deep cervical flexors (Figure 5). H is h o m e p r o g r a m m e w a s p ro g re sse d to : a) ten 10-second holds with the chin tuck action. b) ten 10-second holds of the postural correction and reversal exercise. c) continued practise of the correct neu­ tral posture and muscle lengthening exer­ cises. RESULTS All of the patients symptoms decreased markedly in frequency over the study period. This is clearly depicted in graphi­ cal form below (Figure 6). There was a reduction in intensity of symptom 1 from 2.4/5 in weeks 1 and 2 to 1/5 in weeks 5 and 6. The mean intensi­ ties of symptom 2 and 3 were essentially unchanged. All cervical ranges of active movement were restored to full range and were painfree with gentle overpressure. The left UFT, scalenes, levator scapu­ lae, and the suboccipital extensor muscle groups were felt to be of normal length by the end of the study. The subject’s endurance capacity of the DCF improved from four to twelve 10- second holds. Similarly LFT improved from five to twelve 10-second holds. Telephonic follow-up two months later found the patient to have experienced no symptoms since his last physiotherapy session. One year later he was still clear of all symptoms. DISCUSSION The majority of the patient’s symptoms following reduction and immobilisation were felt to be due to: a) slight persistence of the torticollis causing soft tissue shortening and mus­ cle spasm on the left side. b) marked weakness and instability due to pain inhibition, ligamentous defi­ ciency and prolonged immobilisation. The above factors created a muscle imbalance of the “proximal crossed syn­ drome” type described by Janda (1988) and treatment was therefore aimed at restoring muscle balance. The results show that a six week cervi­ cal stabilisation programme, in a child following reduction of the subluxation, led to a significant improvement in the Figure 4. A ssessing endurance o f deep cervical flexors using pressure sensor. 6 SA J o u r n a l o f Ph y sio th era py 1998 V o l 54 No 3 R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) F ig u re 5. P o stu ra l co rre ctio n a n d re v e rs a l exe rc ise signs and symptoms and an increase in the endurance capacity and lengths of the relevant muscle groups. There was a steady decrease in the frequency of alJ symptoms with the greatest improvement occurring in weeks 5 and 6. Unexpected increases in frequency occurred in symptom 1 in week 2 and symptom 3 in week 4. These were explained on analysis of the subject’s diary, which showed returning to school after vacation in week 2 and a tennis clin­ ic in week 4 which were both major pro­ voking factors. In week 5 the patient reported one “dull, dizzy headache” which appeared to be a reaction to an increase in the number o f repetitions of chin tuck holds in his home programme. This reflects the irri­ tability of the upper cervical region and the fact that care needs to be taken in introducing or increasing muscle re-edu- cation techniques (Beeton & Jull, 1994; Janda, 1988). Symptom 3 was the only other symptom reported in weeks 5 and 6 and only occurred in physiotherapy ses­ sions. There has been no previous attempt to determine the effect of only treating the muscle dysfunction com ponent in patients with chronic cervical pain syn­ dromes (Beeton & Jull, 1994; Jull, 1988; Janda, 1988). Jull (1988) believes that measures to lengthen muscles and to facilitate muscle activity will not be fully successful unless the articular component is directly treated. This study has shown, however, that improving the patients muscle dysfunc­ tion without directly treating the articular component led to significant reduction of the patient’s signs and symptoms. CONCLUSION The presence of an acquired torticollis in a child should arouse suspicion of atlanto-axial rotatory subluxation, partic­ ularly where there is a history of a recent URTI or minor trauma. The vital impor­ tance of recognition and appropriate referral o f this rare condition lies in the fact that it may indicate a compromised atlanto-axial complex, with the potential to cause neural damage or even death. This study has clearly shown that fol­ lowing reduction of the subluxation, a cervical stabilisation training programme that excluded direct treatment of the artic­ ular component, was highly effective in reducing the patient’s signs and symp­ toms. It therefore supports and highlights the important concept of correcting the muscle dysfunction component in cer- vicogenic pain sufferers. REFERENCES Aspinall W. 1990 Clinical Testing for the Craniovertebral Hypermobility Syndrome. The J o u rn a l o f O rthopaedic a n d S ports P hysical Theropy 12:47-54 Beeton K, Jull G. 1994 Effectiveness of Manipulative Physiotherapy in the Management o f Cervicogenic Headache: A Single Case Study. P h ysiotherapy 80: 417- 423. Bogduk N. 1986 Cervical Causes o f Headache and D izziness. In: Grieve GP (ed) M odern M a n u a l Therapy o f the Vertebral C olum n, pp289-302 Edinburgh: Churchill Livingston. Dvorak J, Panjabi M. 1987 Functional Anatomy o f the Alar Ligaments. Spine 12:183-189. Edeling J. 1982 The True Cervical Headache. S o u th A frican M e d ic a l J o u rn a l 62:531-534. Fielding JW, Hawkins RJ. 1977 Atlanto- Axial Rotatory Fixation. T he J o u rn a l o f B one a n d J o in t Surgery 59A :37-44. Fielding JW, Stillw ell WT, Chynn KY, Spyropoulos, EC. 1978 U se o f Computed Tomography for the Diagnosis o f Atlanto- Axial Rotatory Fixation. The J o u rn a l o f B one a n d J o in t Surgery. 6 0 A :1 102-1104. 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The Jo u rn a l o f B o n e a n d J o in t Surgery. 66A :708- 714. Robert N , Hensinger RN. 1993 Cervical Spine Problem s in Children: Upper C ervical Instability. Transactions o f the C ollege o f M ed icin e o f S o u th A frica. 41A :45-57. Watson DH. 1990 Cervical Headache: An Investigation o f Natural Head Posture and Upper Cervical Flexor M uscle Performance. In: G rieve GP (2 nd ed) M o d e rn M a n u a l T herapy o f the V ertebral C olum n, pp249-360. Edinburgh: Churchill Livingston. Figure 6. Frequency o f subject's sym ptom s 12 10 8 6 4 2 0 I ■ Symptom 1 "dull, dizzy headache" ■ Symptom 2 "sharp, electric shock" □ Symptom 3 "burning, strain" _l l ______a SA J o u r n a l o f Ph ysio t h er a py 1998 V o l 54 No 3 7 R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. )