Torticollis in children - differential diagnosis approach

Transcription

1 Torticollis in children - differential diagnosis approach Poster No.: C-2101 Congress: ECR 2015 Type: Educational Exhibit Authors: A. Eran, A. Ilivitzki; Haifa/IL Keywords: Neoplasia, Infection, Education, Ultrasound, MR, CT, Pediatric, Neuroradiology spine, Neuroradiology brain DOI: /ecr2015/C-2101 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 20

2 Learning objectives Familiarity with the normal anatomy of the cervical spine and cranio-cervical junction in children. Knowledge of the imaging features of common causes of torticollis in children. Knowledge of the diagnostic approach to torticollis in children including advantages and disadvantages of each diagnostic modality. Background Torticollis is a clinical symptom characterized by lateral head tilt with rotation of the chin to the contralateral side [1]. The main differential diagnosis split is between congenital and acquired torticollis. Congenital torticollis mainly results from muscular causes (congenital muscular torticollis) and skeletal abnormalities. Acquired torticollis is mostly related to trauma, infection and tumors [2]. Many cases of acquired torticollis will resolve spontaneously and have no radiographic abnormalities. It is important to recognize the clinical scenarios in which imaging work-up is needed. Normal C spine anatomy in the pediatric age The pediatric cervical spine and craniocervical junction are notable by the presence of ossification centers, incomplete ossification and increased flexibility. On CT the epiphyseal plates are depicted as smooth and regular in shape, with predictable locations, and sclerotic margins (fig 1). On MRI, during the first months of life the spinal column goes through changes in vertebral body shape and signal as the spine mature; from biconvex vertebral bodies with low T1 signal to rectangular vertebral bodies and high T1 signal (fig 2). Hypermobility of the pediatric C spine is due to ligamentous laxity, shallow and angled facet joints, underdeveloped spinous processes, and physiologic anterior wedging of vertebral bodies (fig 1). Additionally the relatively large head size compared to the neck and weak neck musculature also contribute to the instability of the pediatric C spine [3]. Page 2 of 20

3 The anatomic and functional distinctiveness of the cervical spine play a role in the preferential development of torticollis in children. Images for this section: Fig. 1: C spine images of a 2 year old child in sagittal (A) and axial (B) planes. Arrows point to the normal synchondreses of the dense (A) and atlas (B). Page 3 of 20

4 Fig. 2: Sagittal T1 weighted images through the lumbar spine that show vertebral maturation. From low signal and bullet shaped vertebral bodies at 2 months of age (A), to high signal and rectangular shape at 11 months (B). Page 4 of 20

5 Findings and procedure details Congenital Torticollis Congenital torticollis has a frequency of 1-2 % and is most commonly caused by congenital muscular torticollis (CMT) [2]. See Table 1 for differential diagnosis list of congenital torticollis. Congenital muscular torticollis CMT refers to muscular disorders causing torticollis at birth or shortly after due to unilateral shortening of the sternocleidomastoid muscle. More common in males and on the right side. The affected muscle develops fibrotic changes which can be associated with a mass (fibromatosis colli) or without a mass [4]. Presentation is usually during the first 4 weeks of life with torticollis and / or nontender neck mass. Thought to be caused by intrauterine and perinatal events. Risk factors for CMT include overcrowding environments such as: first-born, oligohydramnios, breech presentation and multiple deliveries as well as difficult delivery [2]. Ultrasound (US) is the imaging modality of choice for initial investigation. There is diffuse or focal enlargement of the sternocleidomastoid muscle. Focal mass is usually hypoechoic and homogenous (fig 3). Ill-defined margins, extension beyond the sternocleidomastoid muscle and prolonged clinical symptoms are indications for further work up with MRI to exclude the possibility of a mass. On MRI CMT has a variable appearance from normal appearance to hyper or hypo-intense muscle on T2 which enhances similar to normal muscles or heterogeneously. CT should be avoided if there is no suspicion for skeletal abnormality. The mass usually resolve within the first year of life with conservative treatment. Skeletal malformations Vertebral and craniocervical junction fusion alter the normal biomechanics of the C spine and predispose to torticollis, disc herniation and instability. Page 5 of 20

6 There is disc space narrowing at the level of the fused vertebrae. Slanting of the anterior and posterior borders of the fused vertebrae towards the center is typical. Fusion of the facet joints supports the diagnosis (fig 4, 5). Work-up with CT and MRI is needed for better definition of the abnormality and to delineated associated CNS (central nervous system) abnormalities (fig 4, 5). Klippel-Feil syndrome is defined as failure of segmentation of 2 or more C spine vertebrae. In 75% of cases the upper 3 cervical vertebra are involved (fig 4). Chiari malformation (fig 6) and additional congenital CNS malformation may also cause torticollis. Acquired torticollis Typically occurs between the ages of 5 and 12 years. Trauma, infections and in#ammatory conditions, and central nervous system tumors or lesions are the most common etiologies [2]. See table 2 for detailed differential diagnosis list. Trauma Occipital condyle fracture and facet dislocation may present with torticollis (fig 7). Atlanto-axial rotatory fixation (AARF) is a rotatory fixation of the atlas and C2 with resultant neck deformity. It could be secondary to trauma, post infectious (Grisel syndrome) or associated with predisposing conditions such as Down syndrome, Marfan syndrome etc. Spontaneous spinal epidural hematoma is a rare disorder which might manifest with painful torticollis followed by weakness and sensory loss and is mostly common at the cervico-thoracic level [5](fig 8). CT is the modality of choice in most trauma cases. MRI is indicated in any case of concern for ligamentous injury or when there is a neurologic deficit. Infection Head and neck and spinal column infections may cause torticollis either by muscular or ligamentous irritation or from direct spinal disease. Lateral neck radiograph will show increased soft tissue thickness anterior to the C spine in retropharyngeal abscess (fig 9A). US may show superficial lymphadenitis and abscess. CT is used to visualize the deep neck spaces and for pre-surgical planning. MRI is useful in spinal column infections due to its increased sensitivity and its ability to show soft tissue and epidural extension (fig 10). Page 6 of 20

7 Tumors Tumors of the CNS, spine and neck may cause torticollis (fig 11, 12, 13). CNS tumors are usually in the posterior fossa or C spine. The common presentation of C spine tumor is pain due to dural irritation. Posterior fossa tumors may also have signs of increased intracranial pressure. In any case of insidious development of torticollis the possibility of a tumor should be considered. MRI is the imaging modality of choice due to its exquisite sensitivity. Inflammatory conditions Symptomatic intervertebral disc calcification syndrome is a rare disease characterized by calcification of the nucleus pulposus (fig 14). The etiology is thought to be inflammatory and presentation is usually with systemic signs of inflammation and neck pain. The cervical spine is the most common site of involvement. Imaging shows calcification of the intervrtebral disc. Juvenile idiopathic arthritis preferentially involve the upper C spine, with inflammatory synovial changes and erosions. Images for this section: Table 1 Page 7 of 20

8 Fig. 3: Transverse US images through the right (A) and left (B) sternocleidomastoid muscles and longitudinal (C) image through the right muscle, that shows focal isoechoic thickening of the right muscle. Page 8 of 20

9 Fig. 4: Fusion of C2, C3 vertebrae (A, C), assimilation of the atlas to the basi-occiput (arrows, B, C) and mild basilar invagination. Note torticollis on the coronal image. Images findings are compatible with Klippel-Feil syndrome. Fig. 5: Coronal and sagittal T2 weighted images that show butterfly vertebra at C4 level (arrow, A) and fusion of C4, C5 vertebra (B) in a 15 months old baby with congenital torticollis. Page 9 of 20

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11 Fig. 6: Sagittal T2 weighted image shows crowding of the foramen magnum and downward displacement of the cerebellar tonsild compatible with Chiari type I malformation. Table 2 Page 11 of 20

12 Fig. 7: Occipital condyle fracture dislocation on the right side (arrows, A, B). Page 12 of 20

13 Fig. 8: Sagittal T1 (A) and T2 (B) weighted images that show a heterogeneous, mostly hyper-intense, lesion in the posterior epidural space (asterisks) with compression of the spinal cord. An epidural hematoma was evacuated on surgery. Page 13 of 20

14 Fig. 9: Lateral retropharyngeal abscess. Lateral radiograph shows increased soft tissue thickness (A, asterisks). Axial, sagittal and coronal CT images demonstrate the abscess (B, D, asterisks) and retropharyngeal edema (C). Page 14 of 20

15 Fig. 10: Epidurits in a 2 year old child who presented with fever and torticollis. Sagittal and axial contrasted T1 weighted images at presentation (A, B) show enhancement of the epidural space with mild compression of the thecal sac. Those resolved following treatment with antibiotics (C, D). Page 15 of 20

16 Fig. 11: Axial (A) and sagittal (B) T2 weighted images that show an exophytic cerebellar mass bulging towards the cerebello-pontine angle in a 2 year old boy who presented with 2 months history of increasing torticollis. Surgical exision revealed pilocytic astrocytoma. Page 16 of 20

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18 Fig. 12: Vetebra plana at C4 level in a child with known Langerhans cell histiocytosis and new toticollis. Page 18 of 20

19 Fig. 13: Extensive Neurofibromas in the neck in a child with a known diagnosis of Neurofibromatosis type 1 and torticollis. Fig. 14: Sagittal and coronal C spine CT images of a 6 year old child who presented with acute torticollis. Images show calcification of the intervertebral disc at C4-5 level compatible with symptomatic intervertebral disc calcification syndrome. Page 19 of 20

20 Conclusion Torticollis is a clinical sign that might signify an underlying disorder. In newborn infants with CMT, ultrasound is preferred and often diagnostic. In older children CT is used to diagnose traumatic insult, neck infection and vertebral anomalies. MRI is used to diagnose inflammatory and infectiouc spinal disorders and in cases in which CNS or neck malignancy is suspected. Personal information References 1. Herman MJ, Wolf M. Torticollis in children. Curr Orthoped Practice 2013;24: Haque S, Bilal Shafi BB, Kaleem M. Imaging of torticollis in children. Radiographics 2012;32: Lustrin ES, Karakas SP, Ortiz AO, Cinnamon J, Castillo M, Vaheesan K, et al. Pediatric Cervical Spine: Normal Anatomy, Variants, and Trauma. Radiographics 2003;23: Tatli B, Nur A, Mine Ç, Meral O, Feride B, Gonul A. Congenital Muscular Tort#collis: Evaluation and Classification. Ped neurol 2006; 34: Patel, JC Boaz, JP Phillips, BP Garg. Spontaneous spinal epidural hematoma in children. Ped neurol 1998;19: Page 20 of 20