South Korea c Department of Physical & Rehabilitation Medicine, Samsung Medical Center, Sungkyunkwan University, School of Medicine,

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1 Journal of Pediatric Surgery (2011) 46, Clinical features and outcome of physiotherapy in early presenting congenital muscular torticollis with severe fibrosis on ultrasonography: a prospective study Yong-Taek Lee a, Kyungjae Yoon a,, Yong-Bum Kim b, Pil-Wook Chung b, Ji Hye Hwang c, Young Sook Park d, Seung Hyun Chung e, Soo Kyung Cho f, Byung Hee Han g a Department of Physical & Rehabilitation Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University, School of Medicine, Seoul , South Korea b Department of Neurology, Kangbuk Samsung Hospital, Sungkyunkwan University, School of Medicine, Seoul , South Korea c Department of Physical & Rehabilitation Medicine, Samsung Medical Center, Sungkyunkwan University, School of Medicine, Seoul , South Korea d Department of Physical & Rehabilitation Medicine, Samsung Changwon Hospital, Sungkyunkwan University, School of Medicine, Changwon , South Korea e Department of Physical & Rehabilitation Medicine, National Cancer Center, Gyeonggi-do , South Korea f Department of Physical & Rehabilitation Medicine, Samyook Hospital, Gyeonggi-do , South Korea g Department of Radiology, Cheil General Hospital, Kwandong University, School of Medicine, Seoul , South Korea Received 4 November 2010; revised 13 February 2011; accepted 14 February 2011 Key words: Congenital muscular torticollis; Clinical features; Severity of fibrosis on ultrasonography; Outcome of physiotherapy Abstract Background: It has been reported that ultrasonography (US) can detect the severity of congenital muscular torticollis (CMT), and severe fibrosis of the sternocleidomastoid (SCM) muscle noted on US is irreversible and likely to require surgery. Clinical outcome of CMT depends mainly on the patient's age, which is also associated with the severity of fibrosis as determined by US. However, there has been no well-designed study to elucidate the true relationship among these factors nor a definite consensus on treatment of young infants with severe fibrosis in the SCM compared with well-documented reports that late cases require surgery. Purpose: The purpose of the current study was to investigate whether severity of SCM fibrosis on US is correlated with clinical severity and outcome of standardized physiotherapy in early presenting CMT. Methods: Fifty patients with a palpable neck mass, initial deficit of passive neck rotation (ΔROT) more than 10, and age less than 3 months were classified into 4 US types according to the severity of fibrosis in the SCM and underwent standardized physiotherapy and regular assessment. Relationship between US types and 2 variables (ΔROT and treatment duration) and success rate of physiotherapy was assessed. Results: None of the cases was classified as type 4.Type 3 showed greatest ΔROT and longest mean treatment duration. Both variables showed a significant linear trend of association with US Corresponding author. Tel.: ; fax: address: kint999@nate.com (K. Yoon) /$ see front matter 2011 Elsevier Inc. All rights reserved. doi: /j.jpedsurg

2 Clinical features and outcome of physiotheraphy in early CMT 1527 types by P for trend (P =.003, P b.001, respectively). Treatment was successful in 49 patients (98%). Conclusion: In young infants with CMT, US can document severity; and an early and adequate physiotherapy is a good treatment option, particularly even in those with severe fibrosis Elsevier Inc. All rights reserved. The main clinical features of congenital muscular torticollis (CMT) include a characteristic head tilt, limited neck rotation, and/or a palpable neck mass that are caused by unilateral fibrous contracture of the sternocleidomastoid (SCM) muscle [1,2]. The basic abnormality is known as endomysial fibrosis with deposition of collagen and migration of fibroblasts around individual muscle fibers [3]. Although most patients with CMT show rapid improvement during early infancy, a few are still subject to limited neck motion and far-reaching effects on craniofacial or spine growth during their childhood and, subsequently, require surgical release [1,4,5]. Recently, high-resolution ultrasonography (US) has increasingly been used to demonstrate the fibrotic lesion in the SCM muscle. Ultrasonographic classification according to the severity of SCM fibrosis has been reported to correlate with clinical severity and associated with clinical outcomes [6,7]. Patients with CMT with severe fibrosis in the entire SCM muscle on US did not improve after conservative treatment and were more likely to require surgical intervention than those with mild fibrosis, who significantly improved [8,9]. However, for clinical outcome, it is wellknown that the success rate of conservative treatment depends mainly not only on the degree of SCM muscle damage but also on the patient's age at initiation of treatment [10-15]. Furthermore, earlier reports showed that patients with severe fibrosis on US were likely to be older than those with mild fibrosis [6-9]. Thus, to elucidate the true relationship between clinical features and severity of fibrosis on US, the effect of patient age should be considered. In addition, inclusion of various types of torticollis, such as CMT, postural torticollis and SCM imbalance, or different severity of condition before treatment, including degree of deficit in passive neck movement, could alter the outcome. Nevertheless, these factors have rarely been considered in previous studies and, especially, have never been controlled for the investigation of the relationship between US findings and clinical features. The purpose of the current study was to prospectively investigate whether the severity of fibrosis on US correlated with clinical severity using clearly defined inclusion criteria, including age, in early presenting CMT. A new hypothesis that intrauterine constraint leads to limitation of head mobility and progressive degrees of neck contracture has been proposed. Moreover, it has been suggested that the severity of torticollis depends on the duration of prenatal immobility [16-18]. Recently, Lee et al [19] reported that a history of breech presentation or oligohydramnios resulted in a higher likelihood of severe fibrosis on US. The authors suggested that intrauterine mechanical factors restricting fetal movement was associated with US-detected severe fibrosis in early infancy. Thus, severe fibrosis on US is likely to have been initiated during the intrauterine period and, consequently, would be more long-term in nature at the time of diagnosis than mild fibrosis, which might develop around the time of birth [19]. This finding suggests that the long-term nature of severe fibrosis could result in a poor outcome after physiotherapy irrespective of young age. There has been no definite consensus on the treatment strategy for early presenting CMT with severe fibrosis, whereas there are many well-documented reports showing that late cases of CMT with a definite tight fibrotic band require an operative procedure [10,20]. Thus, the second goal of our study was to assess the outcome of conservative treatment and attempt to establish a treatment strategy using a clearly defined inclusion criteria, standardized physiotherapy, and evaluation protocol in early presenting CMT with severe fibrosis. 1. Materials and methods All infants with clinically suspected CMT were seen at the torticollis clinics of Kangbuk Samsung Hospital and Cheil General Hospital & Women's Health Care Center from 2008 to Fifty (32 boys, 18 girls) infants met the inclusion criteria, and the parents agreed to a conservative treatment program. The study protocol was approved by the institutional review boards of the Kangbuk Samsung Hospital. The present study was conducted in accordance with the Declaration of Helsinki. All patients were prospectively enrolled in this study after their parents or caregivers provided informed consent. Inclusion criteria were (1) palpable neck mass or limited neck motion, (2) younger than 3 months of age, and (3) deficit in passive neck rotation (ΔROT) more than 10. Exclusion criteria were as follows: (1) congenital anomalies of the cervical spine; (2) apparent ocular torticollis; and (3) another type of torticollis, such as acute, spasmodic, or neurogenic torticollis. When ocular symptoms such as eye movement abnormality was suspected, we consulted an ophthalmologist to consider exclusion. In addition, we obtained plain spine radiographs and neck US to exclude any spinal abnormalities and to confirm that the mass was located in the SCM muscle in all patients. Clinical characteristics including sex, age at presentation, side of the torticollis, initial ΔROT and initial deficit in passive lateral flexion (ΔLAT) and thickness ratio (affected/unaffected side SCM anteroposterior diameter) were assessed. Initial ΔROT

3 1528 Y.-T. Lee et al. was used as an indicator of clinical severity and treatment parameter. Success rate and duration of conservative treatment at the end point were used as the outcome dependent variable Ultrasonographic classification Ultrasonographic classification was performed using the criteria reported in our previous study [19], which was modified from that of Hsu et al [8] and Tang et al [9] in consensus by 1 radiologist and 1 physiatrist experienced in the examination of CMT. Criteria were defined according to the severity of fibrosis on US as follows: (1) type 1 had a localized well-defined heteroechoic mass in the involved SCM muscle, (2) type 2 represented diffuse hyperechogenecity along the entire muscle with hypoechogenic background muscle, (3) type 3 had diffuse hyperechogenecity along the entire muscle with almost no hypoechoic background, and (4) type 4 had a hyperechogenic band in the entire involved muscle. Ultrasonographic examination was performed with the patient in the supine position with head slightly rotated to the opposite side. Existence of a mass and muscle thickness as well as echo texture was evaluated on both sides with real time high-resolution US (Voluson 730 [GE, Zipf, Austria], Logiq 9 [GE, Milwaukee, WI]) using a 6 to 12 MHz lineararray transducer Treatment and follow-up protocol Treatment and regular follow-up were conducted using the protocol modified from Cheng et al [12]. We followed up our patients from initial assessment to the final assessment when the end point was met. Initial assessment was conducted at the time patients were first seen in our clinic. Follow-up for the clinical assessment was performed once a month during the treatment by 1 physiatrist who was blinded to the US type of CMT. Passive neck range of motion (ROM) was measured using an arthrodial protractor with the patient in the supine position, with the shoulder stabilized, and the head and neck supported by the examiner over the edge of the examination couch so that the neck was free to rotate and move in all directions (Fig. 1). Treatment was started within just a few days after first assessment. A standardized physiotherapy program (manual stretching exercise for 30 minutes 3 times per week) was used and performed by 1 experienced physical therapist. Manual stretching consisted of a standardized protocol (3 repetitions of 15 gentle manual stretches of the tight muscle with sustained force for 1 second and a rest period of 10 seconds in between). The parents were also educated to carry out a home program of active positioning with specific instructions. Treatment duration was defined as the time between the initial assessment and the final assessment. End points of the study were determined at the time when the ΔROT was normalized or less than 5, or treatment was found to have failed. ΔROT more than 6 at the age of 10 Fig. 1 Examination and measurement of passive neck range of motion. A, Rotation. B, Lateral flexion. months or no further improvement after more than 6 months of successive treatment was regarded as failure Statistical analysis The χ 2 test and 1-way analysis of variance with multiple comparison procedures (Bonferroni method) were used for analysis of statistical differences in the clinical characteristics of the participants, particularly in treatment duration, initial ΔROT and ΔLAT, thickness ratio, and age at presentation among the different US types. For statistical analysis, US types were used as an ordinal variable that represented the severity of US detected fibrosis. Correlation between US types and 2 variables (initial ΔROT and treatment duration) were assessed by Spearman rank correlation coefficient and P for trend. All statistical analyses were conducted using PASW Statistics 17.0 (SPSS Inc, Chicago, IL). 2. Results Fifty infants (32 boys, 18 girls) whose ages ranged from 20 days to 3 months (1.51 ± 0.72 months) were included in this study. Among the 50 patients, 25 (50.0%) had type 1,

4 Clinical features and outcome of physiotheraphy in early CMT 1529 Table 1 Clinical characteristics according to US classifications Type P No. of cases 25 (50.0) 8 (16.0) 17 (34.0) Initial ΔROT ( ) 26.6 ± ± ± Initial ΔLAT ( ) 12.0 ± ± ± Age at presentation (mo) 1.3 ± ± ± Thickness ratio 2.39 ± ± ± Treatment 3.2 ± ± ± duration (mo) Male:female 18:7 5:3 9:8.448 Right:Left 15:10 6:2 11:6.742 Values are expresses as number (percentages) and mean ± SD, unless otherwise indicated. ΔROT indicates deficit of passive rotation of affected side compared with unaffected side; ΔLAT, deficit of passive lateral neck flexion of affected side compared with unaffected side; Thickness ratio, affected SCM thickness/unaffected SCM thickness Significantly different compared with type 1 fibrosis (P b.01). 8 (16.0%) had type 2, and 17 (34.0%) had type 3 fibrosis. None of the subjects was classified as type 4. Basic clinical characteristics, including mean age at presentation, thickness ratio, birth weight, and affected side, were statistically similar among the different US types (Table 1). Initial ΔROT of type 3 patients was significantly greater than that of type Fig. 3 Treatment duration according to severity of fibrosis on US. Mean duration of treatment was gradually lengthened as the severity of fibrosis on US increased from type 1 to 3. This showed a significant linear trend of association. P for trend b.001. Values represent mean ± SEM. 1(P =.011, Table 1). Initial ΔROT was gradually increased as the severity of fibrosis on US increased from type 1 to type 3, which showed a significant linear trend of association (P for trend =.003, Fig. 2) and moderate correlation by the Spearman rank correlation coefficient (P =.003, ρ = 0.415). Conservative treatment was successful in 49 (98.0%) of 50 patients. Only 1 patient who had type 3 fibrosis was a treatment failure because the ΔROT was 10 at the age of 10 months. Treatment duration of type 3 CMT was significantly longer than that of type 1 (P =.001, Table 1). Treatment duration was also longer with increased severity of US fibrosis from type 1 to 3, which showed a significant linear trend of association (P for trend b.001, Fig. 3) and moderate correlation by the Spearman rank correlation coefficient (P b.001, ρ = 0.485). 3. Discussion Fig. 2 Initial rotation difference (ΔROT) according to severity of fibrosis on US. ΔROT indicates deficit in passive rotation of affected side compared with unaffected side. Initial ΔROT was gradually enlarged as the severity of fibrosis on US increased from type 1 to 3. This showed a significant linear trend of association. P for trend =.003. Values represent mean ± SEM. Initial ΔROT was greater in cases with severe fibrosis on US than in other milder types. The mean duration of treatment as the clinical outcome parameter was longer in the severe type than other milder type. These 2 variables showed a statistically significant linear trend of association and correlation with severity of fibrosis on US. These findings were consistent with previous reports that showed that the severity of restriction of neck rotation at the beginning of treatment was a predictor of treatment duration [21,22] and that severity of fibrosis on US was intimately associated with clinical outcome [8]. It was reported that severity of fibrosis on US showed a correlation with microscopic findings as well, in which mild type of fibrosis (type 1 or 2) showed a

5 1530 Y.-T. Lee et al. mixture of young proliferating fibroblasts with background normal muscle fibers, whereas severe type (type 3 or 4) showed that the entire SCM muscle was replaced with mature fibroblasts or fibrotic bands without a normal muscle [8]. SCM muscle with type 3 fibrosis appears to be more seriously involved by the pathologic process than type 1 or 2 fibrosis, which supports the findings that these USdetected features correlate with clinical severity in early presenting CMT. Treatment success was achieved in 49 (98%) of 50 patients after our standardized physiotherapy program. The present study showed much better overall results compared with most of the previously reported series where the success rate of conservative treatment ranged from 69.3% to 91.1% [12,23]. The results of present study can be compared with that of the well-designed study involving a large population by Cheng et al [22] that showed an excellent to good outcome in 89% of 538 patients initially seen before 3 months of age. However, we could not completely compare our outcomes with Cheng et al because of the fact that they used a clinical classification criteria instead of a US criteria and enrolled various types of torticollis, including postural torticollis, whereas we included only patients with US-confirmed CMT. In addition, Cheng et al used their own scoring scale as an outcome parameter that included many subjective findings such as degree of craniofacial asymmetry, residual band, head tilt, and subjective assessment by parents, whereas we used only the objective finding ΔROT. Of specific note, the good clinical outcome observed in infants with severe fibrosis in the SCM (type 3) in the present study differed from that of previous reports in which passive rotation of neck for types 3 and 4 fibrosis did not improve after conservative treatment and showed a higher probability for requiring surgical correction [8,9]. In previous reports, patients with severe fibrosis on US were likely to be older than those with mild fibrosis. In the report of Hsu et al [8], subjects' median ages were 0.2 years for type 1; 0.7 years, type 2; and 0.8 years, type 3. Tang et al [9] reported that subjects' mean age and SD were 0.39 ± 0.3 (type 1), 1.09 ± 0.80 (type 2), and 4.41 ± 3.85 (type 3). In both studies, all patients with a fibrotic cord in the entire SCM (type 4) were older than 2 years. It is well established that patients who are younger at the time of diagnosis have a better chance of recovery after conservative treatment alone and rarely require an operation [23,24]. Celayir [25] reported that, when intensive therapy is started within the first 4 months of age, the average length of treatment was only 3.2 months; and none required surgery. Thus, the results of our study showing both a high success rate after conservative treatment, especially in patients with type 3 and absence of type 4, may be attributable to the fact that the subject's age was younger than 3 months. The present study showed that patients with type 3 fibrosis had most severe symptoms and needed a longer duration of physiotherapy among different US types. The 1 patient who failed conservative treatment as defined by predetermined end points (ΔROT N10 at the age of 10 months) had type 3 fibrosis. Although most patients with CMT have a favorable prognosis after conservative treatment, craniofacial asymmetry may persist; and scoliosis can develop in patients with severe CMT who have been treated inadequately [1,4,5,23,26]. Surgical release has been recommended for such refractory cases especially in patients presenting after the age of 1 year [13] or severe US type (type 3 or 4) [7-9]. Patients presenting before 4 months of age [25] or those with mild fibrosis (type 1 or 2) completely recovered early in infancy [27]. These findings suggest that, in some of the patients with severe fibrosis in early infancy, clinical symptom might persist longer and more seriously and, subsequently, requires a surgical procedure if adequate physiotherapy is delayed. In the present study, there were some limitations that should be considered. The first is that only the ΔROT was used as an evaluation parameter. That was chosen because a measurement of rotation shows better interexaminer reliability compared with measurement of lateral flexion [10,20-22]. Furthermore, because all of our subjects were younger than 3 months, they could not control their head actively and were perhaps unwilling to rotate the neck to the affected side. Thus, the evaluation of other clinical features such as active ROM or degree of head tilt was impossible. Additional limitations included a follow-up period that was relatively short, and the small sample size may have adversely affected the statistical power in our study. This suggests that further studies with consideration of these limitations are required. This study demonstrated that US features of the SCM muscle correlate with the clinical severity of CMT in young infants. Even in patients with severe fibrosis in the SCM muscle, an early intensive physiotherapy program with regular checkups appears to be a good treatment strategy. References [1] Porter SB, Blount BW. Pseudotumor of infancy and congenital muscular torticollis. Am Fam Physician 1995;52(6): [2] Robin NH. Congenital muscular torticollis. 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