Craniofacial deformity in patients with uncorrected congenital muscular torticollis: an assessment from three-dimensional computed tomography imaging

Congenital muscular torticollis is caused by idiopathic fibrosis of the sternocleidomastoid muscle that restricts movement and pulls the head toward the involved side. Deformation of the craniofacial skeleton will develop if the restriction is not released and result in aesthetic and functional problems. The purpose of this study was to use three-dimensional computed tomography imaging for qualitative and quantitative evaluation of the craniofacial deformity in a series of patients with uncorrected congenital muscular torticollis, and to assess age as a precipitating factor for severity of the deformity. A total of 14 patients from 1 month to 24 years of age were included. The skull images were rotated into standard orientation and reconfigured for evaluation of the cranium, endocranial base, and facial skeletal structures. The midlines of cranial base and facial bone, angle of midline deviation, width of each hemicranium and hemiface, and the orbital index were defined and measured. The results showed that the cranium and cranial base deformation took place as early as in infant stage, with the most prominent change occurring in the posterior cranial fossa. Facial bone asymmetry started to appear after 5 years of age, at which time the mandibular and occlusal abnormalities were observed. The deformity of the orbits and maxilla occurred at an older age, characterized by the deviation and decreased vertical height on the affected side. The severity of the observed deformities increased with age. The angle of midline deviation was 2.48 +/- 1.68 degrees in the cranial base and 3.26 +/- 3.28 degrees on the facial bone. Both of the midline deviations were significantly correlated with age. Compared with the contralateral side, the width of the ipsilateral posterior hemicranium was longer (54.36 +/- 6.72 mm versus 50.81 +/- 6.55 mm), and the width of the ipsilateral lower hemiface was shorter (35.30 +/- 7.27 mm versus 43.49 +/- 11.34 mm). Both differences were statistically significant. Measurement of the orbital index demonstrated a significantly flatter orbit on the ipsilateral side (89.48 +/- 0.11 versus 92.74 +/- 0.08). This study showed that the cranium and cranial base deformity occurred early in patients with uncorrected torticollis, while the facial bone deformity occurred in childhood stage. The cranial and facial deformity became more severe with age. Early release of the muscle restriction is advised to prevent craniofacial deformation.     

EMG biofeedback treatment of torticollis: a controlled outcome study.

Successful treatment of torticollis with electromyographic (EMG) biofeedback has been reported in a number of single case and single group studies. The present investigation represents the first controlled outcome study. Twelve torticollis patients were randomly assigned to EMG biofeedback or relaxation training and graded neck exercises (RGP). The procedure involved three sessions of baseline assessment, 15 sessions of EMG BF or RGP, 6 sessions of EMG BF or RGP plus home-management, 6 sessions of home-management alone, and follow-up 3 months after the end of treatment. A variety of outcome measures were used including physiological (EMG from the two sternocleidomastoid muscles, skin conductance level), behavioral (angle of head deviation, range of movement of the head), and self-report (depression, functional disability, body concept), therapist and "significant other" reports and independent observer assessment of videos. In both groups, neck muscle activity was reduced from pre- to posttreatment. This reduction was greater in the EMG biofeedback group. There was evidence of feedback-specific neck muscle relaxation in the EMG biofeedback group. Therefore, the outcome was not due to nonspecific factors and could be attributed to feedback-specific effects. Changes in skin conductance level showed that neck muscle relaxation was not simply mediated by a general reduction of "arousal." Significant improvements of extent of head deviation, and range of movement of the head, as well as reductions of depression were present, which were not different in the two groups. At the end of treatment, no patient was asymptomatic. Any therapeutic benefit was generally maintained at follow-up. The results and the procedural simplicity of RGP make the issue of cost-efficacy of EMG biofeedback a pertinent one. Further controlled outcome studies of EMG biofeedback treatment of torticollis with larger samples are required.     

Botulinum A toxin for the treatment of adult-onset spasmodic torticollis

Thirty-five patients with adult-onset idiopathic torticollis were treated by local injections of botulinum A toxin into dystonic cervical muscles. Substantial improvement with respect to reduction and elimination of pain was found in 81 percent, improvement in posture deformity and involuntary spasms in 70 percent, increased range of motion of the neck in 78 percent, reduction in visible sternocleidomastoid hypertrophy in 86 percent, and improvement in tremor in 65 percent. The syndrome was divided into four subtypes based on pattern of dystonic muscle groups involved in the dystonia, head and shoulder posture, and sternocleidomastoid muscle hypertrophy. Injection strategy based on this subdivision is described.     

EMG biofeedback treatment of torticollis: A controlled outcome study

Successful treatment of torticollis with electromyographic (EMG) biofeedback has been reported in a number of single case and single group studies. The present investigation represents the first controlled outcome study. Twelve torticollis patients were randomly assigned to EMG biofeedback or relaxation training and graded neck exercises (RGP). The procedure involved three sessions of baseline assessment, 15 sessions of EMG BF or RGP, 6 sessions of EMG BF or RGP plus home-management, 6 sessions of home-management alone, and follow-up 3 months after the end of treatment. A variety of outcome measures were used including physiological (EMG from the two sternocleidomastoid muscles, skin conductance level), behavioral (angle of head deviation, range of movement of the head), and self-report (depression, functional disability, body concept), therapist and significant other reports and independent observer assessment of videos. In both groups, neck muscle activity was reduced from pre- to posttreatment. This reduction was greater in the EMG biofeedback group. There was evidence of feedback-specific neck muscle relaxation in the EMG biofeedback group. Therefore, the outcome was not due to nonspecific factors and could be attributed to feedback-specific effects. Changes in skin conductance level showed that neck muscle relaxation was not simply mediated by a general reduction of arousal. Significant improvements of extent of head deviation, and range of movement of the head, as well as reductions of depression were present, which were not different in the two groups. At the end of treatment, no patient was asymptomatic. Any therapeutic benefit was generally maintained at follow-up. The results and the procedural simplicity of RGP make the issue of cost-efficacy of EMG biofeedback a pertinent one. Further controlled outcome studies of EMG biofeedback treatment of torticollis with larger samples are required.This work was funded by grants from the Medical Research Council and the Dystonia Society.     

Tenotomy of the avian anterior latissimus dorsi muscle. II. Can regeneration from the stump occur in the pigeon?

Because the chick's anterior latissimus dorsi muscle (ALD) regenerates a fast-twitch muscular connection after tenotomy, the pigeon's ALD was tenotomized, either at the origin or through the muscle 0.5 cm from the origin, to determine whether this muscle behaves similarly to the chick muscle. These procedures were compared in pigeons operated upon at 7 weeks, versus 5 to 9 months of age. The pigeon's ALD did not regenerate a new connection, and other differences were observed between the pigeon and chick ALD. The pigeon ALD has only a single slow muscle-fiber type, has fewer fast fibers, and transforms to a fast-twitch muscle more readily than the chick ALD after tenotomy. The transformation of muscle fiber types occurred more readily in the older pigeons than those tenotomized at 7 weeks of age. Tenotomy induced morphological alterations of the muscle fiber structure in all of the pigeons, which is in contrast to the absence of changes in the tenotomized chick ALD. Therefore the pigeon and chick ALD respond completely differently to tenotomy.     

Antigraviceptive neck muscle responses to "moving up and moving down" in human

The responses of neck muscle to sudden transit from one 'g' to hyper 'g', work to support the head and remain the relative position of head on trunk as common observed: i.e. in sudden acceleration or deceleration by car or ejection of pilot from aircraft. Accordingly it is highly possible that the neck muscle responses to moving up may be important to prevent the neck injury due to sudden linear acceleration such as moving up against gravity. However little is known about the evaluation of mechanism of this reflex. Therefore the present study was conducted with two aims. The first aim was to investigate the neck muscle responses to vertical linear acceleration bv 0.4 g produced with an electro-hydraulic servo-system. We chose the vertical linear acceleration because it activates mainly sacculus, from which afferents have been demonstrated to be connected directly to sternocleidomastoid muscle in animals and human. The second aim was to determine whether there is a difference of neck muscle response to moving down and moving up.     

Venous air embolism during a craniofacial procedure

The possibility of venous air embolism exists whenever the craniofacial operative field is above the level of the heart. Craniotomy with the high-torque craniotome is hypothesized to have produced venous air embolism in the patient described in this report. The diagnosis of venous air embolism is determined by transesophageal Doppler probe, transesophageal echocardiogram or external echocardiogram, and end-tidal N2 and CO2 determinations. Treatment includes control of the air entry sites, aspiration of air from the right atrium via a catheter placed prior to operation, and discontinuing nitrous oxide. If these measures are unsuccessful, the operative field should be transposed below heart level and the procedure terminated. In the event of significant hemodynamic compromise, closed cardiac massage should be tried; if that fails, open cardiac massage and direct aspiration are necessary. The true incidence of venous air embolism in craniofacial operations may be much higher than previously suspected. We therefore recommend placement of appropriate monitoring equipment to detect intracardiac air in those major craniofacial procedures in which there is a potential for intravascular air ingress.     

Some hormonal factors (hypophysectomy, castration and testosterone administration) modifying the course of "compensatory" muscle hypertrophy in the rat.

1. Maximum compensatory hypertrophy of the soleus and plantaris muscle in male rats is attained seven days after tenotomy of the gastrocnemius muscle (39% and 9% respectively). When tenotomy of the gastrocnemius was performed seven days ater hypophysectomy, hypertrophy in these two muscles was aproximately half that found in control animals. 2. After 81-day castration of young male rats the weight of the saleus and plantaris was reduced and hypertrophy following tenotomy of the gastrocneumius muscle did not develop. 3. Chronically castrated rats received testosterone two weeks prior to tenotomy of the gastrocnemius and a week during the muscle hypertrophy phase. Hypertrophy of the soleus in castrated rats which had received testosterone seven days after tenotomy of the gastrocnemius was 25% as compared with muscles of castrated animals. The corresponding value in the plantaris muscle was 10%. 4. These results indicate that even calf muscles of the rat, namely the soleus and plantaris muscles, are significantly affected by testosterone under these conditions, although it is not, as yet, clear whether its action is direct or indirect.     

Influence of test position on neck muscle fatigue in healthy controls

BackgroundIt has been suggested that increased fatigue of neck muscles could be related to neck pain. However, studies on the matter present contradicting results which could be explained by the different test positions used.PurposeThe purpose of this study was to investigate the influence of test position on muscle fatigue of neck flexor and extensor muscles in healthy controls.MethodsTwenty-five women without neck pain sustained neck flexion and neck extension isometric contractions at 25% and 75% of their maximal voluntary contraction (MVC) in two test positions: sitting and supine lying. Using surface electromyography, the change over time of the median frequency of the power spectrum (MDF slope) of the myoelectric signal of the sternocleidomastoid and splenius capitis muscles was measured and compared between both positions.ResultsAt 75% MVC, splenius capitis muscles presented higher fatigue in lying compared to sitting, while sternocleidomastoid demonstrated no difference between positions. No statistically significant effect of test position was found at 25% MVC for both sternocleidomastoid and splenius capitis muscles as they generally did not present myoelectric manifestations of fatigue.ConclusionThese results underline the need to standardise the test position when investigating neck muscle fatigue, especially for neck extensors at high loads.     

Pain-induced changes in cervical muscle activation do not affect muscle fatigability during sustained isometric contraction

This study investigated whether pain-induced changes in cervical muscle activation affect myoelectric manifestations of cervical muscle fatigue. Surface EMG signals were detected from the sternocleidomastoid and splenius capitis muscles bilaterally from 14 healthy subjects during 20-s cervical flexion contractions at 25% of the maximal force. Measurements were performed before and after the injection of 0.5 ml of hypertonic (painful) or isotonic (control) saline into either the sternocleidomastoid or splenius capitis in two experimental sessions. EMG average rectified value and mean power spectral frequency were estimated throughout the sustained contraction. Sternocleidomastoid or splenius capitis muscle pain resulted in lower sternocleidomastoid EMG average rectified value on the side of pain (P < 0.01). However, changes over time of sternocleidomastoid EMG average rectified value and mean frequency (myoelectric manifestations of fatigue) during sustained flexion were not changed during muscle pain. These results demonstrate that pain-induced modifications of cervical muscle activity do not change myoelectric manifestations of fatigue. This finding has implications for interpreting the mechanisms underlying greater cervical muscle fatigue in people with neck pain disorders.     

Giant congenital nevi: a 20-year experience and an algorithm for their management.

A variety of treatment options exists for the management of giant congenital nevi. Confusion over appropriate management is compounded because not all giant congenital nevi are pigmented, and malignant potential varies between different types. The present study sought to define factors in the presentation of giant congenital nevi that could provide an algorithm for their management, with respect to both the extent of resection and subsequent reconstructive options.A retrospective review of all patients who presented with a congenital nevus of 20 cm2 or greater since 1980 was performed, distinguishing among nevi involving the head and neck, the torso, and the extremities. Sixty-one patients with giant congenital nevi were evaluated (newborn to age 16 years), of which 60 nevi in 55 patients have been operated on.Giant congenital nevi having malignant potential were pigmented nevi (53 patients) and nevus sebaceus (four patients). Those not having malignant potential were verrucous epidermal nevi (three patients) and a woolly hair nevus (one patient). Of the 60 giant congenital nevi operated on, expanded flaps were used in 25, expanded full-thickness skin grafts were used in 10, split-thickness or nonexpanded full-thickness skin grafts were used in 13, and serial excision was used in 30. After 1989, operations tended to use multimodality treatment plans, with an increased use of expanded full-thickness grafts and immediate serial tissue expansion. The use of serial excision, particularly in the extremities, also increased after 1989. Serial excision was the treatment of choice when it could be completed in two procedures or less, which occurred in more than 80 percent of cases using serial excision alone. Expanded flaps were the most common mode of reconstruction in the head and neck region and were used in 49 percent of these procedures. Serial excision was the most common form of treatment in the extremities, used in 50 percent of procedures. Tissue expansion in the extremities was infrequently used to provide an expanded flap (8 percent of procedures), whereas it was frequently used to provide expanded full-thickness skin grafts harvested from the torso (used in 31 percent of procedures).On the basis of these data, algorithms for the extent of resection and subsequent reconstructive options for giant congenital nevi were developed. Their management should be formulated relative to pigmentation, malignant potential, and anatomic location of the respective lesions.     

A novel passive neck orthosis for patients with degenerative muscle diseases: Development & evaluation

The current study evaluated the effect of a passive neck orthosis, developed for patients suffering from progressive muscular diseases, on neck muscle activity in 10 adult healthy participants.The participants performed discrete head movements involving pure neck flexion (−10 to 30°), pure neck rotation (up to 30° left and right) and combined neck flexion-rotation (−10 to 30°) in steps of 10° by moving a cursor on a screen to reach predefined targets and staying on target for 10 s. Surface electromyography (EMG) was recorded from upper trapezius and sternocleidomastoid muscles and amplitudes were averaged over the static phases in trials with and without the orthosis. Moreover, the variability in head position and time required to perform the tasks were compared between conditions.Wearing the orthosis caused significant reductions (p = 0.027) in upper trapezius activity (a change of 0.2–1.5% EMGmax) while working against gravity. The activity level of the sternocleidomastoid muscle increased (p ≤ 0.025) by 0.3–1.0% EMGmax during pure and combined rotations without any pain reported.The orthosis showed potential to reduce the activity level of the upper trapezius muscle, the main load bearing muscle of the neck. Further study will be carried out to evaluate the effect in different patient groups.     

Bilateral thymus found in association with unilateral cleft lip and palate

Ectopic localizations of the thymus have been reported in a variety of unusual sites, but not on the surface of the skin of the neck. As one can see in our case, the left neck mass (1.5 x 2 x 0.5 cm) and the right neck mass (4 x 4 x 0.5 cm), when added together in weight, also equal the total weight of the thymus at birth. Also, not being able to encounter the "sail sign" of the thymus gland on the chest x-ray proved to us that no other thymus gland was present. Because of these unusual findings, prior to excision of any congenital lesion in the neck, a biopsy should be done, and only after that should one proceed with the necessary surgical intervention. We are presenting this case because it is unique in this category.     

Electromyographic investigations in torticollis.

In 13 patients with torticollis, the electromyographic (EMG) activity in the neck muscles at rest was investigated before, during and after stimulation of the H1 bundle (in horizontal torticollis) and inner part of oral ventral nucleus (in the rotatory form), as well as after coagulation. Three EMG types of torticollis-spasmodic, myoclonic and mixed - could be differentiated. During the 8-, 25- and 50-Hz stimulations, a diminution in the amplitude and frequency of muscle potentials, followed by electric silence, was most often obtained; in general, the electric silence was preceded by a rhythmic activity of grouped potentials. Following coagulation, no spontaneous EMG activity was any longer recorded in the neck muscles, in the majority of the cases.     

Spinal cord stimulation in the treatment of spasmodic torticollis

This report presents our observations in 63 patients undergoing chronic spinal cord stimulation for treatment of spasmodic torticollis. In this series there were 23 patients (36.5%) who demonstrated marked improvement, characterized by no evidence of torticollis, full range of motility of the head and neck and no pain. Moderate improvement was found in 20 patients (31.8%) who showed minimal residual torticollis, but had full motility and no pain. There were 5 patients (7.9%) considered mildly improved who demonstrated decrease in their torticollic position, spasms and pain, but retained some element of torticollis and/or some limitation of motility. Correlations were made demonstrating the effect on the results of age, sex, electrode array, the configuration of the applied field and the parameters of stimulation.     

Neuronal mechanisms of motor signal transmission in the thalamic ventrooral nucleus in spasmodic torticollis patients

A microelectrode technique was used to study the neuronal mechanisms of motor signal transmission in the ventrooral internus nucleus (Voi) of the motor thalamus during voluntary and involuntary pathological (dystonic) movements in patients with spasmodic torticollis. Voi cell elements proved highly reactive to various functional (mostly motor) tests. An activity analysis of 55 Voi neurons detected during nine stereotactic operations revealed, first, a difference in neuronal mechanisms of motor signal transmission for voluntary movements that do or do not involve the affected axial muscles of the neck and for passive and abnormal involuntary dystonic movements. Second, a sensory component was found to play a key role in the mechanisms of sensorimotor interactions during voluntary and involuntary dystonic head and neck movements activating the axial muscles of the neck. Third, rhythmic and synchronized activity of Voi neurons was shown to play an important role in motor signal transmission during voluntary and passive movements. The Voi nucleus was directly implicated in the mechanisms of involuntary head movements and tension of the neck muscles in spasmodic torticollis. The results can be used to identify the Voi nucleus of the thalamus during stereotactic neurosurgery in order to select the optimal destruction or stimulation target and to reduce the postoperative effects in spasmodic torticollis patients.     

Combined effects of tenotomy and denervation on the dynamic properties of soleus muscle of the rat

Isometric contraction time (CT), half relaxation time (1/2 RT), tetanus fusion frequency (TFF) and tetanus: twitch ratio (T : t ratio) were measured in the denervated (D) and tenotomized-denervated (TD) Soleus muscle of the rat. In D muscle there was an apparent speeding effect at the 2nd day after denervation, with a significant decrease of CT, which was followed by the usual slowing process of denervated muscle. In TD muscle, denervation was performed a week after tenotomy. Tenotomy "per se" was ineffective in modifying dynamic properties of muscle, but it accentuated the early shortening of CT caused by denervation, while reducing and delaying the subsequent slowing process. The results are discussed in the light of the hypothesis that muscle disuse has a speeding effect which counteracts the slowing effect of denervation, and/or that tenotomy modifies the effects of denervation by changing the pattern of fibrillation development.     

Use of dermal regeneration template in contracture release procedures: a multicenter evaluation

Integra dermal regeneration template (Integra Life Sciences, Plainsboro, N.J.) is an effective treatment for full-thickness burns. It can also be useful in contracture release procedures; however, the clinical utility of a dermal regeneration template in contracture release procedures has not been adequately characterized. In this multicenter investigation, the outcomes of release procedures incorporating a dermal regeneration template for 89 consecutive patients, who underwent a total of 127 contracture releases, were retrospectively evaluated. The procedures involved the application of Integra, which includes a temporary silicone epidermal substitute and an artificial dermal layer. After formation of a neodermis, the silicone layer is removed and replaced with an epidermal autograft. Data on patient and contracture site history, treatment methods, physician assessments of range of motion or function, patient satisfaction, recurrence, and adverse events were collected with a standardized questionnaire. Release procedures for the study patients involved the neck, axilla, trunk, elbow, knee, hand, and other anatomical sites. The mean postoperative follow-up period was 11.4 months. At 76 percent of the release sites, range of motion or function was rated as good (significant improvement in range of motion or function) or excellent (maximal range of motion or function possible) by physicians. Responding patients expressed satisfaction with the overall results of treatment at 82 percent of the sites. No recurrence of contracture at 75 percent of the sites was observed during follow-up monitoring. Patient age and prior surgical treatment at the site did not significantly affect the results of treatment. However, outcomes were superior at mature sites, i.e., those for which more than 12 months had elapsed since the original injury. Postoperative complications rarely necessitated regrafting. These results indicate that a dermal regeneration template provides a useful alternative technique for contracture release procedures. The study data indicate that this approach leads to favorable functional outcomes and a high rate of patient satisfaction. This modality also seems to be versatile, because a range of anatomical sites are amenable to treatment with a dermal regeneration template, regardless of prior surgical treatment, and both pediatric and adult patients respond well to this form of therapy. Furthermore, Integra confers functional and cosmetic benefits similar to those of full-thickness grafts but without comparable potential for donor-site morbidity.     

Design and validation of a novel method to measure cross-sectional area of neck muscles included during routine MR brain volume imaging

Introduction

Low muscle mass secondary to disease and ageing is an important cause of excess mortality and morbidity. Many studies include a MR brain scan but no peripheral measure of muscle mass. We developed a technique to measure posterior neck muscle cross-sectional area (CSA) on volumetric MR brain scans enabling brain and muscle size to be measured simultaneously.

Methods

We performed four studies to develop and test: feasibility, inter-rater reliability, repeatability and external validity. We used T1-weighted MR brain imaging from young and older subjects, obtained on different scanners, and collected mid-thigh MR data.

Results

After developing the technique and demonstrating feasibility, we tested it for inter-rater reliability in 40 subjects. Intraclass correlation coefficients (ICC) between raters were 0.99 (95% confidence intervals (CI) 0.98–1.00) for the combined group (trapezius, splenius and semispinalis), 0.92 (CI 0.85–0.96) for obliquus and 0.92 (CI 0.85–0.96) for sternocleidomastoid. The first unrotated principal component explained 72.2% of total neck muscle CSA variance and correlated positively with both right (r = 0.52, p = .001) and left (r = 0.50, p = .002) grip strength. The 14 subjects in the repeatability study had had two MR brain scans on three different scanners. The ICC for between scanner variation for total neck muscle CSA was high at 0.94 (CI 0.86–0.98). The ICCs for within scanner variations were also high, with values of 0.95 (CI 0.86–0.98), 0.97 (CI 0.92–0.99) and 0.96 (CI 0.86–0.99) for the three scanners. The external validity study found a correlation coefficient for total thigh CSA and total neck CSA of 0.88.

Discussion

We present a feasible, valid and reliable method for measuring neck muscle CSA on T1-weighted MR brain scans. Larger studies are needed to validate and apply our technique with subjects differing in age, ethnicity and geographical location.