While congenital muscular torticollis (CMT) can occur along with other conditions, such as clavicle fracture or brachial plexus injury, these conditions exist outside the sternocleidomastoid muscle (SCM). We present a rare case with concurrence of CMT and a malignant tumor inside the same SCM, along with serial clinical and radiological findings of the atypical features of CMT. The malignant tumor was in fact a low-grade fibromyxoid sarcoma. To the best of our knowledge, the current case is the first of a concurrent condition of CMT inside the SCM. This case suggests that concurrent conditions could exist either inside or outside the SCM with CMT. Therefore, a thorough evaluation of SCM is required when subjects with CMT display atypical features, such as the increase of mass or poor response to conservative therapy. In that case, appropriate imaging modalities, such as ultrasonogram or magnetic resonance imaging, are useful for differential diagnosis.
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To introduce the Korean Database of Cerebral Palsy (KDCP) and to provide the first report on characteristics of subjects with cerebral palsy (CP).
The KDCP is a nationwide database of subjects with CP, which includes a total of 773 subjects. Characteristics such as demography, birth history, onset and type of CP, brain magnetic resonance imaging (MRI) findings, functional ability and accompanying impairments, were extracted and analyzed.
Preterm delivery and low birth weight were found in 59.51% and 60.28% of subjects, respectively. Postnatally acquired CP was 15.3%. The distribution of CP was 87.32%, 5.17%, and 1.81% for spastic, dyskinetic, and ataxic types, respectively. Functional ability was the worst in dyskinetic CP, as compared to other types of CP. Speech-language disorder (43.9%), ophthalmologic impairment (32.9%), and intellectual disability (30.3%) were the three most common accompanying impairments. The number of accompanying impairments was elevated in subjects with preterm birth and low birth weight. Brain MRI showed normal findings, malformations, and non-malformations in 10.62%, 9.56%, and 77.35% of subjects, respectively. Subjects with normal MRI findings had better functional ability than subjects with other MRI findings. MRI findings of a non-malformation origin, such as periventricular leukomalacia, were more common in subjects with preterm birth and low birth weight.
The KDCP and its first report are introduced in this report, wherein the KDCP established agreement on terminologies of CP. This study added information on the characteristics of subjects with CP in South Korea, which can now be compared to those of other countries and ethnicities.
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Grisel syndrome is a rare, non-traumatic atlanto-axial subluxation associated with an inflammatory or infectious process in the upper neck. According to the two-hit hypothesis, which is widely accepted for the pathogenesis of Grisel syndrome, preexisting ligamentous laxity of the atlanto-axial joint is regarded as the first hit. An inflammatory or infectious process of the atlanto-axial joint acts as the second hit, resulting in non-traumatic atlanto-axial subluxation. We report on a 6-year-old girl with atlanto-axial subluxation following retropharyngeal and cervical lymphadenitis. She was diagnosed with Grisel syndrome, for which an initial computed tomography did not show any preexisting ligamentous laxity of the atlanto-axial joint. A literature review found only 4 case reports on Grisel syndrome with an initially normal atlanto-axial joint. The present case offers some evidence that a single hit, such as inflammatory changes in the atlanto-axial joint, might cause Grisel syndrome, even without underlying ligamentous laxity.
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To present our experience with ear splint therapy for babies with ear deformities, and thereby demonstrate that this therapy is an effective and safe intervention without significant complications.
This was a retrospective study of 54 babies (35 boys and 19 girls; 80 ears; age ≤3 months) with ear deformities who had received ear splint therapy at the Center for Torticollis, Department of Physical Medicine and Rehabilitation, Ajou University Hospital between December 2014 and February 2016. Before the initiation of ear splint therapy, ear deformities were classified with reference to the standard terminology. We compared the severity of ear deformity before and after ear splint therapy by using the physician's ratings. We also compared the physician's ratings and the caregiver's ratings on completion of ear splint therapy.
Among these 54 babies, 41 children (58 ears, 72.5%) completed the ear splint therapy. The mean age at initiation of therapy was 52.91±18.26 days and the treatment duration was 44.27±32.06 days. Satyr ear, forward-facing ear lobe, Darwinian notch, overfolded ear, and cupped ear were the five most common ear deformities. At the completion of therapy, the final physician's ratings of ear deformities were significantly improved compared to the initial ratings (8.28±1.44 vs. 2.51±0.92; p<0.001). There was no significant difference between the physician's ratings and the caregiver's ratings at the completion of ear splint therapy (8.28±1.44 vs. 8.0±1.61; p=0.297).
We demonstrated that ear splint therapy significantly improved ear deformities in babies, as measured by quantitative rating scales. Ear splint therapy is an effective and safe intervention for babies with ear deformities.
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To identify the correlation between change in spinal deformities after surgical release and age at the time of surgery, and the effectiveness of surgical release in patients with neglected congenital muscular torticollis (CMT).
This was a retrospective study of 46 subjects with neglected CMT who had undergone surgical release at age ≥5 years at a tertiary medical center between January 2009 and January 2014. Spinal deformities were measured on anteroposterior plain radiographs of the cervical and whole spine, both preoperatively and postoperatively, to assess 3 parameters: cervicomandibular angle (CMA), lateral shift (LS), and Cobb angle (CA). We analyzed the change in spinal deformities after surgical release in consideration of age at the time of surgery.
The median age at the time of surgery was 12.87 years. All 3 parameters showed significant improvement after surgical release (median values, pre- to post-surgery: CMA, 12.13° to 4.02°; LS, 18.13 mm to 13.55 mm; CA, 6.10° to 4.80°; all p<0.05). There was no significant correlation between age at the time of surgery and change in CMA (R=0.145, p=0.341) and LS (R=0.103, p=0.608). However, CA showed significant improvement with increasing age (R=0.150, p=0.046).
We assessed the correlation between change in spinal deformities after surgical release and age at the time of surgery. We found that that surgical release is effective for spinal deformities, even in older patients. These findings enhance our understanding of the effectiveness and timing of surgical release in patients with neglected CMT.
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To evaluate the craniofacial asymmetry in adults with neglected congenital muscular torticollis (CMT) by quantitative assessment based on craniofacial three-dimensional computed tomography (3D-CT).
Preoperative craniofacial asymmetry was measured by 3D-CT for 31 CMT subjects ≥18 years of age who visited a tertiary medical center and underwent 3D-CT between January 2009 and December 2013. The relationship between the age and the severity of craniofacial asymmetry was analyzed in reference to anteroposterior length asymmetry of the frontal bone and zygomatic arch, vertical and lateral displacements of the facial landmarks, and mandibular axis rotation.
The age at CT was 27.71±7.02 years (range, 18-44 years). All intra-class correlation coefficients were higher than 0.7, suggesting good inter-rater reliability (p<0.05) of all the measurements. The frontal and the zygomatic length ratio (i.e., the anteroposterior length asymmetry on the axial plane) was 1.06±0.03 and 1.07±0.03, respectively, which was increased significantly with age in the linear regression analysis (r2=0.176, p=0.019 and r2=0.188, p=0.015, respectively). The vertical or lateral displacement of the facial landmarks and rotation of the mandibular axis did not significantly correlate with age (p>0.05).
Craniofacial asymmetry of neglected CMT became more severe with age in terms of anteroposterior length asymmetry of the ipsilateral frontal bone and zygomatic arch on the axial plane even after growth cessation. This finding may enhance the understanding of therapeutic strategies for craniofacial asymmetry in adults with neglected CMT.
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To quantify magnetic resonance imaging (MRI) findings of congenital muscular torticollis (CMT) and to demonstrate the usefulness of quantitative MRI findings in the management of CMT.
This was a retrospective study of 160 subjects with CMT who had undergone neck MRI at the age of 48 months or younger at a tertiary medical center. Among the 160 subjects, 54 had undergone surgical release of CMT and 106 subjects had not undergone surgery. For the quantitative analysis, the ratios of area and intensity of the MRI findings were measured and compared between the two groups (ratio of area = the largest cross-sectional area of the SCM with CMT - the cross-sectional area of the contralateral SCM without CMT / the cross-sectional area of the contralateral SCM without CMT; ratio of intensity = the mean gray color intensity of the contralateral SCM without CMT - the lowest mean gray color intensity of the SCM with CMT / the mean gray color intensity of the contralateral SCM without CMT). Receiver operating characteristic (ROC) curve analysis was conducted for the ratios of area and intensity in order to find the optimal cutoff value for determining the need for surgery in CMT cases.
The ratios of area and intensity were significantly higher in the surgical group than in the non-surgical group (p≤0.001), suggesting that the sternocleidomastoid muscle (SCM) was thicker and darker in the surgical group than in the non-surgical group. The optimal cutoff value for the ratio of area was 0.17 and that for the ratio of intensity was 0.05. All subjects with a ratio of intensity less than 0.03 belonged to the non-surgical group, and all subjects with a ratio of intensity greater than 0.16 were categorized in the surgical group.
The quantitative MRI findings, i.e., ratios of area and intensity, may provide a guideline for deciding the need for surgical intervention in CMT patients. Further prospective studies are required to verify these findings.
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Congenital muscular torticollis (CMT) and craniosynostosis are diseases that cause plagiocephaly and craniofacial asymmetry in children. In our literature review, we did not find any report of concurrent manifestation of CMT and craniosynostosis. A 41-month-old boy visited our hospital with left torticollis, right laterocollis, and craniofacial asymmetry as the main findings. During clinical examination, prominent right sternocleidomastoid muscle and limited range of motion of the neck were noted, and right CMT was confirmed by magnetic resonance imaging of the neck. Three-dimensional computed tomography of the skull, which was conducted due to the unusual appearance of the skull with a large head circumference, mild brachycephaly, as well as left plagiocephaly, revealed premature closure of the sagittal suture. Thus, we report the first case that showed concurrence of CMT and sagittal synostosis. We recommend that concurrently manifested craniosynostosis needs to be examined if the subject with CMT displays unusual craniofacial asymmetry to a greater extent than deformational plagiocephaly.
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To compare effectiveness on correcting cranial and ear asymmetry between helmet therapy and counter positioning for deformational plagiocephaly (DP).
Retrospective data of children diagnosed with DP who visited our clinic from November 2010 to October 2012 were reviewed. Subjects ≤10 months of age who showed ≥10 mm of diagonal difference were included for analysis. For DP treatment, information on both helmet therapy and counter positioning was given and either of the two was chosen by each family. Head circumference, cranial asymmetry measurements including diagonal difference, cranial vault asymmetry index, radial symmetry index, and ear shift were obtained by 3-dimensional head-surface laser scan at the time of initiation and termination of therapy.
Twenty-seven subjects were included: 21 had helmet therapy and 6 underwent counter positioning. There was no significant difference of baseline characteristics, head circumferences and cranial asymmetry measurements at the initiation of therapy. The mean duration of therapy was 4.30±1.27 months in the helmet therapy group and 4.08±0.95 months in the counter positioning group (p=0.770). While cranial asymmetry measurements improved in both groups, significantly more improvement was observed with helmet therapy. There was no significant difference of the head circumference growth between the two groups at the end of therapy.
Helmet therapy resulted in more favorable outcomes in correcting cranial and ear asymmetry than counter positioning on moderate to severe DP without compromising head growth.
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To examine whether the clinical severity of cervical dystonia (CD) significantly correlates with 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) findings as well as to determine the threshold of the clinical severity of CD for positive 18F-FDG PET/CT study findings.
Forty-seven subjects with torticollis as one of the symptoms of CD were included. The clinical severity of CD was evaluated with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) at the time of 18F-FDG PET/CT. The correlation between the clinical severity of CD and the highest SUVmax was examined. The threshold of the clinical severity of CD necessary for positive 18F-FDG PET/CT findings was determined using receiver operating characteristics curve analysis.
Thirty-three of the 47 subjects (70.21%) showed positive 18F-FDG PET/CT findings. The ipsilateral splenius capitis/cervicis, oblique capitis inferior, and longus colli/capitis were the rotators most frequently involved. The highest SUVmax of 18F-FDG PET/CT was significant correlated with the TWSTRS. Subjects with a total TWSTRS exceeding 39 showed positive 18F-FDG PET/CT findings, with those having a total TWSTRS ≤22 showing negative 18F-FDG PET/CT results. The cutoff value of the total TWSTRS for positive 18F-FDG PET/CT findings was set at 27.5 with 90.9% sensitivity and 64.3% specificity.
A significant correlation was evident between the clinical severity of CD and 18F-FDG PET/CT findings, providing a threshold of the clinical severity of CD for acquisition of positive 18F-FDG PET/CT findings.
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To evaluate the usefulness of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) in the management of cervical dystonia (CD) with botulinum toxin type A (BoNT-A) injection.
Thirty two subjects with CD were included. A BoNT-A injection was provided either by clinically targeting method (group 1) or by 18F-FDG PET/CT-assisted, clinically targeting method (group 2). In group 2, selection of target muscles and dosage of BoNT-A were determined according to the increased 18F-FDG uptake, in addition to physical examination and functional anatomy. The outcomes of BoNT-A injection was compared between the two groups, in terms of the number of subjects who had reinjection before and after 6 months, the number of reinjections, the interval of reinjections, the duration to the minimal Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), the number of adverse events, the reduction rate of TWSTRS at 1-3 months and 3-6 months after injection, and the probability of reinjection-free living.
The number of subjects who had reinjection within 6 months was significantly lower in group 2 than in group 1 (10 in group 1 vs. 3 in group 2). The reduction rate of TWSTRS after 3-6 months (37.8±15.7% of group 1 vs. 63.3±28.0% of group 2) and the probability of reinjection-free living were significantly higher in group 2 than in group 1.
These findings suggest that 18F-FDG PET/CT study could be useful in management of CD in terms of the identification of dystonic muscles if there is an increase in the 18F-FDG uptake in the cervical muscle of the images.
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(1) To present the magnetic resonance imaging (MRI) findings of congenital muscular torticollis (CMT) of subjects who underwent surgical release and subjects who showed a good prognosis with stretching exercises and (2) to correlate the MRI findings with the histopathologic findings of CMT for subjects who underwent surgical release in order to examine the hypothesis that the MRI findings of CMT can be used as a determinant to perform surgical release of CMT.
The neck MRI findings of 33 subjects who underwent surgical release for CMT were compared with those of 18 subjects who were successfully managed only with conservative management. The MRI findings were correlated with the histopathologic sections of the CMT mass.
All 33 subjects (100%) who underwent surgical release showed one or more low signal intensities within the involved sternocleidomastoid muscle (SCM) on the T1- and T2-weighted images of neck MRI. The eighteen non-surgical candidates showed only enlargement of the SCM without low signal intensity within the SCM. The histopathologic findings showed interstitial fibrosis and/or the presence of aberrant tendon-like excessive dense connective tissue that was either well-arranged or disorganized.
The histopathologic findings and MRI findings showed good correlation in terms of the amount of fibrosis and aberrant dense connective tissue within the SCM. If multiple or large low signal intensities within the SCM are noted, we think that surgical release should be considered.
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To compare the clinical severity of congenital muscular torticollis (CMT) based on the method of child birth.
Children diagnosed with CMT and who were < 6-years-of-age at the time of their first visit at the Center for Torticollis, Ajou Medical Center, were included in this study. The medical records were retrospectively reviewed with reference to the method of child birth and the clinical severity of CMT. The clinical severity of CMT was determined either by whether stretching exercises were needed for the children <6-month-of-age or whether surgical release was required for the children ≥6-months-of-age at the time of the first visit.
One hundred seventy eight subjects with CMT were enrolled. There was no significant difference in the rate of surgical release according to the method of child birth. For 132 patients <6-month-of-age there was also no significant difference in the rate of stretching exercises.
There was no significant difference in the clinical severity of CMT based on the method of child birth. This finding suggests that prenatal factors alone could be a cause of CMT and that the clinical severity of CMT in children delivered by Cesarean section is not different when compared with the severity of CMT in children born through vaginal delivery.
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To examine whether the thickness of the sternocleidomastoid muscle (SCM) could be used as a prognostic factor for congenital muscular torticollis (CMT).
This was a retrospective study conducted in a pediatric rehabilitation service at a tertiary medical center. Fifty-two children who met the following inclusion criteria were included: 1) children who were 3 month-old or younger, 2) children diagnosed with CMT, 3) passive rotation of the face toward the shoulder of the tilted side ≤60°, 4) children who had been managed according to the clinical pathway for CMT, 5) children who had been followed up for 6 months or more after the end of treatment. The duration and total number of stretching exercise sessions were reviewed with reference to the thickness of the SCM.
Among the 52 children with CMT, 46 children were successfully managed with only stretching exercise of the SCM for 1-6 weeks (group 1: 88.5%) and 6 children were managed with botulinum toxin A injection, surgical release or both in addition to stretching exercise (group 2: 11.5%). The difference in the SCM thickness between the affected and normal sides was significantly greater in group 2 than that in group 1 (p=0.026). A strong correlation was found between the total duration of stretching exercise and the difference in the SCM thickness in group 1 (Pearson' γ=0.429; p=0.003).
Children with a thicker SCM seem to require a longer duration of stretching exercise and other therapeutic interventions in addition to stretching exercise for CMT. Therefore, the thickness of the SCM may be one prognostic factor for CMT treatment.
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