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Frontal cephalometric analysis in the evaluation of facial asymmetry in torticollis, and outcomes of bipolar release in patients over 6 years of age.

BACKGROUND: The aim of this study was to investigate the mid-term results of bipolar release in congenital muscular torticollis patients over 6 years of age, and the efficacy of frontal cephalometric analysis in the determination and follow-up of facial asymmetry. METHODS: Twelve patients (9 boys, 3 girls) from 7 to 12 years of age were included in the study. Bipolar release was performed, followed by 5-7 days of traction and 3 months of physiotherapy. Posteroanterior cephalometric radiography was performed at the beginning of and after therapy. The postural symmetry angle (PSA) was used to determine the presence and severity of facial asymmetry. A modified version of Lee et al.'s system was used in evaluating the results. RESULTS: The average follow-up period was 3 years and 5 months. According to the congenital muscular torticollis evaluation system, the outcome was excellent in 2 of the patients, good in 6, fair in 2 and poor in 2. Asymmetry was not severe in all patients at the beginning of therapy according to PSA values, being insignificant in 2, mild in 6, and severe in 4. The PSA results of the last examination indicated that severe facial asymmetry persisted in 3 patients. In 2 of them, PSA values remained within the limits of severe asymmetry despite a slight angular correction. DISCUSSION: Congenital muscular torticollis patients can benefit from surgical treatment over the age of 6 years. Bipolar release is an adequate and complication-free method. Moreover, PSA may be used as an objective method in the determination and follow-up of facial asymmetry in torticollis patients.

Adolescent↗

Transaxillary endoscopic release of restricting bands in congenital muscular torticollis--a novel technique.

Congenital muscular torticollis is due to fibrosis of one or both the heads of sternocleidomastoid muscle. This may also involve the platysma, scalene muscles, and the carotid sheath and may be associated with cervical scoliosis. Conventional surgical procedures leave visible scars. Ramirez, who used the posterior part of the traditional face-lift incision, made perhaps the first attempt at concealing scars. Burstein et al. reported a large series of subcutaneous endoscopic release of torticollis through a hairline approach. Sasaki described an endoscopic two-incision, posterior auricular fold and hairline approach. A technique of transaxillary subcutaneous endoscopy for the release of the sternocleidomastoid muscle in congenital muscular torticollis is described here. This procedure provides direct access to the fibrous bands, enables release without risk of damage to the spinal accessory nerve, external jugular vein, or greater auricular nerve, and leaves no visible neck scars. Two cases of congenital muscular torticollis presenting in adulthood were managed successfully by this technique. The fibrotic part of sternocleidomastoid muscle was released and the normal range of head motion was restored. There were no surgical complications encountered and the patients achieved complete pain free range of movement in six weeks. This technique provides direct and quick access, perpendicular to the line of the fibrotic bands, avoids injury to neurovascular structures and does not leave visible neck scars.

Adolescent↗

Deep brain stimulation for generalised dystonia and spasmodic torticollis.

Dystonia appears distinct from the other tremulous disorders in that improvement following deep brain stimulation frequently appears in a delayed and progressive manner. The rate of this improvement and the point at which no further progress can be expected are presently unknown. The establishment of these parameters is important in the provision of accurate and relevant prognostic information to these patients, their carers, and their treating physicians. We studied 12 consecutive patients with generalised dystonia (n=6) and spasmodic torticollis (n=6) who underwent bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) and were followed up for a minimum of 2 years postoperatively. Standard rating scales were used to quantify their neurological improvement. Both groups experienced a statistically significant improvement in their rating scores at both one and two years following surgery. At 2 years follow-up, the spasmodic torticollis group exhibited a 59% improvement in their total Toronto Western Spasmodic Torticoilis Rating Scale (TWSTRS) rating score and the generalised dystonia group attained a 46% improvement in their overall Burke, Fahn and Marsden Dystonia Rating Scale (BFMDRS) evaluation. Ninety-five percent of the final improvement was attained by 6.4 months in the generalised dystonia group and by 6.6 months in those with spasmodic torticollis. There was no significant improvement after one year postoperatively. These findings add further support to GPi DBS as an effective treatment for generalised dystonia and spasmodic torticollis, and furnish important information as to the expected rate of improvement and the point at which no further gains can be reasonably anticipated.

Adolescent↗

Congenital muscular torticollis: evaluation and classification.

In this investigation of congenital muscular torticollis, 311 infants treated consecutively for congenital torticollis over an 8-year period (1995-2003) at the Pediatric Neurology Clinic of Istanbul Medical Faculty, Istanbul University, Turkey were reviewed retrospectively. The clinical presentation, associated abnormalities, treatment, and outcomes of the overall group and of subgroups divided according to an ultrasonography-based classification were evaluated. All patients were evaluated using a standard approach: cervical ultrasonography was performed, and the patients were divided into two subgroups. Each group was scanned for other anomalies, and outcomes were compared. The mean age at diagnosis was 2.3 months; patients included in this study were 138 males and 173 females. Two clinical subgroups, comprised of sternomastoid tumors 85% and postural torticollis 15%, were identified. Passive range of motion was the initial treatment recommended for all of the patients. Follow-up data were available for all 311 patients; 95% experienced total resolution and 5% experienced subtotal resolution. We conclude that the majority of children with congenital muscular torticollis experience total resolution of symptoms. The success rate of conservative treatment is primarily dependent on the patients' age at the initiation of exercises and ultrasonographic findings.

Female↗

Selective peripheral denervation for torticollis: preliminary results.

Herein we report the preliminary results in nine patients who have undergone selective peripheral denervation for spasmodic torticollis and have been followed up for at least 13 months. All patients had improvement immediately after surgical intervention, and the results have been maintained in five patients. In one patient who had recurrent torticollis, a second procedure in conjunction with injection of botulinum toxin has produced substantial improvement; however, follow-up was brief (6 months). No surgical complications occurred. We believe that selective peripheral denervation is safe and that it can benefit patients with torticollis who have not responded to other types of therapy. These favorable results confirm other published reports on the efficacy of selective peripheral denervation. Long-term follow-up, however, is necessary for determining the role of this procedure in the management of torticollis.

Adult↗

Correlation of ultrasonographic imaging of congenital muscular torticollis with clinical assessment in infants.

Congenital muscular torticollis (CMT) is a common problem affecting infants and children. There is a general lack of standard clinical classification or objective assessment methods. Ultrasonographic imaging of the sternomastoid muscle (SCM) has been carried out in a consecutive series of 436 infants less than 1 y old presenting with CMT over a 5-y period. All patients were classified into three clinical groups: postural torticollis, muscular torticollis and sternomastoid tumor. The severity of the torticollis was also expressed into four subgroups according to the degree of deficits in passive rotation of the neck. The ultrasonographic image of the affected SCM included the echogenicity, texture, motility, softness and the transverse and longitudinal extent of the involvement. The disturbance in the quantitative measurement of the transverse diameter of the lower and upper third of the SCM and the ratio of the measurement to the normal side was recorded. The qualitative and quantitative changes in the SCM image were found to correlate significantly with the clinical typing and severity of rotational deficits of the neck. Ultrasonographic imaging has important potential clinical application in helping the diagnosis, prognostication and monitoring of progress of CMT longitudinally.

Female↗

Pseudotumor cerebri manifesting as stiff neck and torticollis.

Stiff neck and torticollis are significant signs of neurologic disease. Nuchal rigidity is often associated with meningitis, subarachnoid hemorrhage, and posterior fossa tumor. Torticollis may be encountered in inflammatory disorders, such as cervical lymphadenitis, or it can be a sign of spinal cord syrinx or of central nervous system neoplasm. We report on three prepubertal children in whom stiff neck and torticollis were the presenting signs of pseudotumor cerebri. In all, the removal of 6-7 mL of cerebrospinal fluid led to prompt relief of symptoms and signs. We suggest that in the presence of unexplained stiff neck or torticollis in children, the optic discs should be examined to exclude pseudotumor cerebri.

Child↗

Electromyographic study in an infant with benign paroxysmal torticollis.

The findings of surface electromyography (s-EMG) in a female with benign paroxysmal torticollis in infancy (BPT) are reported. This disease is characterized by (1) a self-limiting condition that appears in early infancy and disappears spontaneously, (2) recurrent episodes of sudden, stereotypic torticollis, usually alternating from side to side, and (3) episodes that usually continue for several hours to a few days. The pathogenesis of this disease has not been determined. During episodes in the patient, continuous electrical discharges over the sternocleidomastoid muscle on the same side as the torticollis were observed on s-EMG, indicating that the torticollis was a dystonic phenomenon. The findings are clinically consistent with BPT, and the patient should be categorized as having an idiopathic paroxysmal dystonia in infancy.

Child, Preschool↗

Personality in torticollis: a controlled study.

The Eysenck Personality Questionnaire, the trait scales of the Leyton Obsessional Inventory, the Spielberger State Trait Anxiety Inventory, and a questionnaire assessing marital status and harmony were completed by 100 torticollis patients and a control group of 49 cervical spondylosis sufferers. Information regarding employment status, and events preceding onset of their complaint was also obtained. The two groups did not differ in terms of any of the personality dimensions evaluated, or in their self-reports of events prior to onset of their illness. The groups differed significantly with regards to marital status (a higher proportion of the torticollis patients were single) but not marital harmony. A significantly higher proportion of the torticollis patients were in the permanently sick category of employment status. Findings of previous studies evaluating personality in torticollis and in sufferers of other disabling physical conditions are considered, and the results are discussed in terms of the effects of disabling chronic physical disorders.

Cervical Vertebrae↗

[From the expert's office. Atlanto-axial subluxation with spastic torticollis after adenoid-ectomy resp. tonsillectomy in rose position - malpractice of the surgeon or the anaesthesiologist?].

INTRODUCTION: An arbitration board had to decide whether or not there had been a causal connection between an adenoidectomy or resp. a tonsillectomy and an atlanto-axial dislocation and if so whether this was to be considered a case of malpractice. CASE HISTORIES: In two young girls aged 6 and 11 a torticollis had developed 3 resp. 4 days after the operation. In both cases the proper diagnosis was made only after extensive diagnostic procedures including radiology, neurology, neurosurgery and orthopaedics. The findings are presented in detail. In both cases there was a rotary subluxation between cervical vertebrae C1 and C2 due to a retropharyngeal inflammation. Normal function could only be achieved by surgical reposition and the application of a fixateur externe for quite a long period. DISCUSSION: A non-traumatic torticollis is a very rare complication of an adenoidectomy or tonsillectomy, two of the most common surgical interventions in oto-rhino-laryngology. It is known as Grisel's disease because Grisel in 1930 was the first to describe this sequel following nasopharyngitis and tonsillectomy. A detailed review of the literature and a discussion of the underlying pathology is presented. Predisposing factors might be additional local anaesthesia and electro-haemostatis. CONCLUSIONS: : In both cases evidence for malpractice could not be found, neither concerning the intervention itself nor the handling in the postoperative period. The latency of several days between the operation and the manifestation of the torticollis is regarded as proof that intraoperatively there was no malpractice. In cases where the torticollis is present immediately after the intervention, as has been reported in the literature, a traumatic luxation during the operation or positioning of the patient may be taken into consideration. Because of the extreme rareness of the complication it does not seem compulsory to make it part of the preoperative informed consent.

Adenoidectomy↗

Hypnosis and spasmodic torticollis--report of four cases: a brief communication.

Dystonia and particularly spasmodic torticollis are neuromuscular disorders that are extremely resistant to most therapies (physical, medical, or surgical). Torticollis is a unilateral spasm of the neck muscles, particularly of the sternocleidomastoid, that produces violent, tonic turning of the head to one side. The etiology remains uncertain, although the role of psychogenic factors has been emphasized. This article reviews the literature and reports four cases of spasmodic torticollis treated successfully with hypnosis. In all four cases, psychogenic causes were involved. Postural hypnosis (i.e., hypnosis in the standing position) was employed to counteract and minimize muscle spasms due to postural reflexes. A hypnobehavioral approach was adopted along with hypnotic strategies that included hierarchical desensitization, sensory-imaging conditioning, ego-boosting suggestions, and hypnosis-facilitated differential muscle retraining. In two cases, a combined hypnosis and electromyographic-biofeedback approach was used to equilibrate and retrain affected neck muscles. Although the hypnotherapeutic process took several months to induce and stabilize significant changes, outcome results were good to excellent in all cases, with marked reduction of the torticollis and the hypertrophy of the neck muscles as well as a reduced interference of symptoms in daily living.

Adult↗

Surgical management of torticollis.

Torticollis is a clinical sign of either CNS or musculoskeletal dysfunction. Thorough clinical and neuroradiographic evaluation is indicated for determination of the source, course, and exact nature of the deformity before a considered approach to surgical management is undertaken. Torticollis of neurogenic origin is managed by surgical decompression of the brainstem or cervical spinal cord when indicated, as in the presence of hydrocephalus, the Arnold-Chiari malformation, syringobulbia , syringomyelia, colloid cyst of the third ventricle, or neoplasms in the third ventricle, posterior fossa, or cervicomedullary junction. In progressive and medically refractory cases of spasmodic torticollis, neuroablative procedures, neuroaugmentive procedures, selective muscle excisions, and radical cervical muscle excisions have been used to control the dystonia. Torticollis of musculoskeletal origin is often secondary to or associated with trauma, although congenital craniocervical anomalies may be a predisposing factor. The primary goals of surgery for craniovertebral or upper cervical spine deformity are reduction of the malalignment; decompression of the brainstem, cervical spinal cord, and/or nerve roots if necessary; and stabilization of the upper cervical spine.

Arnold-Chiari Malformation↗

Spasmodic torticollis-like posture and caudate nucleus monoamine in cats: in vivo dialysis study.

In order to study the monoamine mechanism in the caudate nucleus of experimental spasmodic torticollis, the left side of the ventromedial tegmentum (VMT) was electrically destroyed in cats, and in vivo brain dialysis in the caudate nucleus was performed for determining the changes in monoamines and their metabolites. Six out of 12 animals showed the torticollis-like posture in which the head became deviated towards the side of the lesion by rotating around the longitudinal axis. In 4 of these 6 cats, this posture continued for 3 d until brain dialysis was performed. The extracellular 5-HIAA level decreased compared to HVA level in the caudate nucleus of the lesion side. In the other 2 cats, the posture disappeared on the day after the lesion was induced and the change of 5-HIAA level was less prominent or within the range of the control group. The brain dialysis method was very useful for measuring the monoamine metabolite levels of the caudate nucleus while the experimental model was still alive and presenting the torticollis-like posture. These results suggest, although inconclusively, that decreased activity in the ipsilateral caudate nucleus serotonin neuron might play an important role in the manifestation of the torticollis-like posture in this animal model.

3,4-Dihydroxyphenylacetic Acid↗

Venous air embolism and selective denervation for torticollis.

Selective denervation to relieve severe torticollis requires surgery with the patient in the sitting position. Nerve stimulation is used to guide the procedure; therefore, patients undergo general anesthesia without muscle relaxation. Because of the risk of venous air embolism (VAE), monitoring with precordial Doppler and a multiorifice right atrial catheter has been recommended. To document the incidence of VAE and, thus, the effectiveness of monitors to detect it, consecutive patients who underwent selective peripheral denervation for torticollis between June 1993 and July 1994 were studied retrospectively, and those who underwent the procedure between August 1994 and February 1996 were studied prospectively. No VAE was documented in the retrospective group (n = 31). In the prospective group (n = 69), precordial Doppler detected one episode of VAE that lasted < 20 s and had no clinical sequelae. The incidence of complications from right atrial catheter insertion was 8% (carotid puncture, hematoma, inability to cannulate) but with no permanent sequelae. We conclude that VAE is infrequent and self-limited in association with selective denervation for torticollis. While monitoring with precordial Doppler for patients undergoing denervation for torticollis is indicated, the use of a right atrial catheter is of limited value because of associated complications and increased operating room time and cost.

Adult↗

Treatment of recalcitrant idiopathic muscular torticollis in infants with botulinum toxin type a.

Congenital muscular torticollis (CMT) is the most common form of torticollis in children, significantly outnumbering orthopedic, neurologic, and ocular causes. CMT may present as a palpable sternomastoid tumor (SMT) or a simple tightness of the sternocleidomastoid muscle (SCM), designated as idiopathic muscular torticollis (IMT). Muscular torticollis has been associated with positional plagiocephaly in neonates who slept in the supine position. We have had difficulty in treating some of these combined cases by traditional methods such as physiotherapy, stretching exercises, and molding helmets. In November 2000, we began injecting botulinum toxin type A in cases in which there was persistent IMT, despite significant physical therapy input. The 15 patients included in this retrospective study all presented with IMT and positional plagiocephaly; all had responded poorly to conservative treatment, including physiotherapy, stretching exercises, or use of a helmet. In the attempt to avoid progression to surgical release, these patients were treated with botulinum toxin injected into the affected SCM and subsequent additional physiotherapy. All appeared to respond well, and a retrospective analysis of this treatment strategy was undertaken. Information gathered included a questionnaire, skull-shape tracings, and photographs. Independent outcome assessment data were then obtained from the regional child development teams and community physiotherapists. These results show that 14 of 15 children with recalcitrant IMT and positional plagiocephaly treated with botulinum toxin obtained sufficient improvement in neck range of motion and head position as to make surgical release of the muscle unnecessary. Our conclusion is that the use of botulinum toxin is a safe and effective adjunct to physical therapy in treating recalcitrant IMT; in selected cases, it may obviate the need for surgical release of a tight but nonfibrotic SCM.

Botulinum Toxins, Type A↗

Variable co-diagnosis of plagiocephaly and torticollis in Texas health care facilities.

Diagnosis of positional plagiocephaly has increased as babies have been put to sleep supine. The literature varies in reported co-incidence of sternocleidomastoid torticollis, as well as in definition of torticollis as a defect. Statewide data from the Texas Birth Defects registry was analyzed for the diagnosis of plagiocephaly with or without co-diagnosis of torticollis. There were 172 facilities with at least 1 diagnosis of plagiocephaly. Of these, 18 facilities had recorded >20 cases and 3 facilities had recorded >150 cases. In the 18 facilities, percentage of plagiocephaly/torticollis co-diagnosis ranged from 5% to 67%. Two facilities specialize in orthopedic or neurologic problems, but the rest are general pediatric facilities. The three facilities with the most cases showed co-diagnosis of 10% (16 of 159 cases), 29% (108 of 376 cases), and 54% (122 of 228 cases). It is noted that the three largest facilities use different professionals and different medical specialists in evaluation of cases. These data suggest that the wide range in torticollis diagnosis is less likely to reflect true patient variability than an inconsistently applied definition of the defect. Other factors that may play a role are diagnosis by non-MD medical professionals, involvement of different MD specialists in different facilities and diagnosis-dependent but variable insurance reimbursement.

Diagnosis, Differential↗

Spasmodic torticollis due to neurovascular compression of the spinal accessory nerve by the anteroinferior cerebellar artery: case report.

OBJECTIVE AND IMPORTANCE: Spasmodic torticollis is a neuromuscular disorder characterized by uncontrollable clonic and intermittently tonic spasm of the neck muscles. We report a case of spasmodic torticollis attributable to neurovascular compression of the right XIth cranial nerve by the right anteroinferior cerebellar artery (AICA). CLINICAL PRESENTATION: A 72-year-old man with a 2-year history of right spasmodic torticollis underwent magnetic resonance imaging, which demonstrated compression of the right XIth cranial nerve by an abnormal descending loop of the right AICA. INTERVENTION: The patient underwent microvascular decompression surgery. During surgery, it was confirmed that an abnormal loop of the right AICA was compressing the right accessory nerve. Compression was released by the interposition of muscle between the artery and the nerve. CONCLUSION: The patient's postoperative course was uneventful, and his symptoms were fully relieved at the 2-year follow-up examination. This is the first reported case of spasmodic torticollis attributable to compression by the AICA; usually, the blood vessels involved are the vertebral artery and the posteroinferior cerebellar artery.

Accessory Nerve Diseases↗

Recurrent torticollis caused by dissecting vertebral artery aneurysm in a pediatric patient: results of endovascular treatment by use of coil embolization: case report.

OBJECTIVE AND IMPORTANCE: Torticollis is a symptom that can be related to different pathological mechanisms ranging from simple to life-threatening conditions. We report a child with recurrent torticollis caused by an intracranial dissecting vertebral artery aneurysm. This is a very rare condition in childhood, and it was resolved successfully with endovascular treatment. CLINICAL PRESENTATION: The patient was a 10-year-old boy with a 4-year history of left recurrent torticollis, followed by hemiparesis, dysarthria, dysmetria, and tremor. Brain magnetic resonance imaging and digital angiography detected a dissecting aneurysm involving the fourth segment of the left vertebral artery. INTERVENTION: The patient underwent endovascular treatment. Coil embolization, followed by histoacryl injection into the lesion, provided complete obliteration of the aneurysmal sac. CONCLUSION: The patient's postoperative course was characterized by a dramatic disappearance of symptoms and signs within a few hours of the intervention. No relapses of symptoms occurred during a follow-up period of 18 months. This is the first report of a child in whom recurrent torticollis was related to a dissecting vertebral artery aneurysm. Although long-term results of vertebral artery coil embolization remain to be elucidated, the method seems reliable and effective in treatment of these vascular lesions in pediatric patients.

Angiography, Digital Subtraction↗