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Rationale: A case of traumatic atlanto-axial rotatory subluxation (AARS), dens fracture, rupture of transverse atlantal ligament (TAL), and subaxial spinal cord injury without radiographic abnormality (SCIWORA) of Brown-Sequard syndrome has never been reported in a child., Patient Concerns: A 7-year-old boy presented to hospital with torticollis, neck pain, and limited neck rotation after a seat-belt injury sustained during a car accident. Neurologic examination revealed right-side motor weakness and left-side sensory abnormality, known as Brown-Sequard syndrome., Diagnosis: Radiologic examinations revealed type II AARS (Fielding and Hawkins classification), increased atlanto-dental interval (ADI) of 4.5 mm due to a type 1B TAL rupture (Dickman classification), a displaced transverse dens fracture along with an ossiculum terminale, and an intramedullary hemorrhage on the right side of the spinal cord at C3-4., Interventions: The patient immediately received methylprednisolone, and his motor weakness and sensory abnormality gradually improved. At the same time, the patient underwent initial halter traction for 2 weeks, but he failed to achieve successful reduction and required manual reduction under general anesthesia., Outcomes: At the 7-month follow-up visit, radiologic examinations showed a corrected type II AARS that was well maintained and normalization of the ADI to 2 mm. The reduced transverse dens fracture was well maintained but still not united. All clinical symptoms were significantly improved, except the remaining motor weakness of the right upper extremity., Lessons: To the best of our knowledge, this is the first report of traumatic AARS, dens fracture, TAL rupture, and subaxial SCIWORA of Brown-Sequard syndrome in a child. Appropriate diagnosis and careful treatment strategy are required for successful management of complex cervical injuries in a child., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Competing Interests: The authors declare no conflict of interest.

Rationale: Pial arteriovenous fistula (PAVF) occurs when intracranial arteries communicate directly with veins. PAVFs are very rare congenital vascular lesions that are commonly seen in infants and children. Arachnoid cysts are congenital cavitation often filled with cerebrospinal fluid. We present a very rare associated occurrence of bilateral pediatric PAVF and a giant arachnoid cyst presenting as torticollis in a child. So far, this is the first case., Patient Concerns: A 6-year-old male child was referred to our facility from a local hospital due to severe torticollis., Diagnoses: An enhanced computed tomography scan revealed 2 slightly high-density masses at the anterior pontine cistern, right circumferential cistern, as well as left posterior occipital region. The same computed tomography scan revealed a giant arachnoid cyst in the left occipital as well as the temporal region with a thin cerebral cortex adjacent to the cyst., Interventions: Digital subtraction angiography confirmed that the 2 high-flow lesions were PAVFs. The patient was treated with a combination of detachable coils and Onyx Liquid Embolic System (Onyx HD-500) (Covidien/ev3 Neurovascular) via the transarterial endovascular route while the giant arachnoid cyst was managed conservatively., Outcomes: The torticollis resolved 2 days after the procedure. He is currently well with no neurologic deficit., Lessons: We advocate that in cases of PAVF with accompanying cyst, the cyst should be managed conservatively if it is not associated with intracranial hemorrhage or focal neurologic deficit.

Objective: This study aimed to assess the spectrum of pathologies responsible for torticollis in children presenting to the emergency department and to evaluate the associated symptoms to determine clinical red flags for hospitalization., Methods: This was a historical retrospective cohort study. Medical records of children evaluated in our emergency department for torticollis from 2008 to 2013 were reviewed., Results: Among 392 identified patients, 61% had postural torticollis,19.4% infection related, 16.3% traumatic, and 3.5% other. Twenty-five patients (6.4%) were hospitalized. Four variables were strongly and independently related to the severe outcome: fever, sore throat, headache, and age., Conclusions: The association of 2 or 3 of these 4 features carried a risk of 32% and 58%, respectively, of having a severe illness.

Torticollis could be the only symptom and sign of craniovertebral junction (CVJ) abnormality. It could be difficult to identify CVJ abnormality as a cause of torticollis due to their rarity, especially for the subjects with torticollis caused by nontraumatic CVJ abnormalities. There has been no report to focus on nontraumatic CVJ abnormalities as a cause of torticollis. The objective of this study was to report 27 patients of torticollis caused by nontraumatic CVJ abnormalities, with the aim of helping clinicians to identify nontraumatic CVJ abnormalities as a cause of torticollis. This is a retrospective cohort study including 27 subjects who had torticollis caused by nontraumatic CVJ abnormalities. The CVJ was examined in terms of atlanto-occipital angle, atlanto-axial angle, and lateral and anterior atlanto-dens intervals for the evaluation of occipital condylar hypoplasia, rotation of atlanto-axial joint, and lateral and anterior shift of the dens, respectively. Abnormalities of the lower cervical or thoracic spine were also evaluated. Occipital condylar hypoplasia, rotation of atlanto-axial joint, and lateral shift of the dens were the most common CVJ abnormalities. The 18.5% of the subjects had concurrent anomalies of lower cervical or thoracic vertebrae along with CVJ abnormalities. Each subject had 2.22 ± 1.10 types of CVJ abnormalities on average. In conclusion, comprehensive evaluation of CVJ abnormalities is recommended for differential diagnosis of subjects with unexplained torticollis. Once CVJ abnormalities are identified, concurrent abnormalities of other vertebrae need to be evaluated.

Cat-scratch disease-associated vertebral osteomyelitis and epidural involvement are rare and may manifest with nonspecific chronic symptoms in children, such as fever or torticollis. We present only the fourth case in the literature describing epidural involvement in an immunocompetent boy presenting with fever, torticollis, and indiscriminate left upper back tenderness. Bartonella henselae serologies are important to consider because such atypical complications often are responsive to conservative management with antibiotics.

Acquired torticollis is a common clinical finding in children evaluated in the pediatric emergency department. It may be the presentation symptom of different illnesses, such as trauma, muscle contraction, infections, or malignancies, and an accurate differential diagnosis is required to correctly identify the cause and choose the right treatment. Spondylodiscitis is a low-grade bacterial infection that involves intervertebral disks and the adjacent vertebral bodies. Spondylodiscitis of the cervical spine is unusual and may be a rare cause of torticollis. We report the case of a 4-year-old male patient admitted to the emergency department for a 5-day history of painful torticollis. Blood tests showed an elevated erythrocyte sedimentation rate. The radiograph of the cervical spine showed a thin fifth cervical soma. The magnetic resonance imaging of cervical spine showed the alteration of cervical vertebral bodies and intervertebral disks, suggesting the diagnosis of cervical spondylodiscitis. The patient recovered after endovenous antibiotic treatment. We suggest that cervical spondylodiscitis should be suspected and investigated by means of an magnetic resonance imaging in every case of unexplained torticollis with persisting symptoms.

Pediatric emergency physicians must have a high clinical suspicion for atlantoaxial rotatory subluxation (AARS), particularly when a child presents with neck pain and an abnormal head posture without the ability to return to a neutral position. As shown in the neurosurgical literature, timely diagnosis and swift initiation of treatment have a greater chance of treatment success for the patient. However, timely treatment is complicated because torticollis can result from a variety of maladies, including: congenital abnormalities involving the C1-C2 joint or the surrounding supporting muscles and ligaments, central nervous system abnormalities, obstetric palsies from brachial plexus injuries, clavicle fractures, head and neck surgery, and infection. The treating pediatrician must discern the etiology of the underlying problem to determine both timing and treatment paradigms, which vary widely between these illnesses. We present a comprehensive review of AARS that is intended for pediatric emergency physicians. Management of AARS can vary widely bases on factors, such as duration of symptoms, as well as the patient's history. The goal of this review is to streamline the management paradigms and provide an inclusive review for pediatric emergency first responders.

Descriptive case report.To report a rare case of post-traumatic torticollis by odontoid fracture in a patient with diffuse idiopathic skeletal hyperostosis (DISH).Cervical fractures in DISH can result from minor trauma, and a delay in presentation often prevents their timely diagnosis. Cervical fractures in patients with spinal DISH usually occur in extension injuries, and almost always occur in the lower cervical spine. Reports of odontoid fractures with torticollis in patients with spinal DISH are rare.A 73-year-old man with DISH presented with severe neck pain and a cervical deformity presenting as torticollis without neurological deficits. He gave a history of a fall while riding a bicycle at a low speed 3 months ago. X-ray showed torticollis in the right side, and computed tomography (CT) showed a type-II odontoid fracture and subluxation at the C1-2 level.We performed a staged treatment because this patient had severe neck pain associated with a chronic course. Initially, the fracture dislocation was reduced under general anesthesia and was stabilized with a halo vest. We then performed posterior occipitocervical in situ fusion after confirming the correction of the cervical deformity by CT. The patient showed significant amelioration of neck symptoms postoperatively, and bony fusion was achieved 1 year after surgery.For post-traumatic torticollis due to an odontoid fracture, plain CT is useful for diagnosis and posterior occipitocervical in situ fusion following correction and immobilization with a halo vest is a safe and an effective treatment.

Torticollis can be due to many different etiologies. Torticollis is not a diagnosis, but a symptom of many conditions. We present a case of persistent torticollis in a child that was misdiagnosed during multiple visits to the pediatrician, otolaryngologist, and 3 different emergency departments. Grisel syndrome must be included in the differential diagnosis of any patient with torticollis after a head and neck infection, upper respiratory infection, or postoperative from head and neck surgery. Early diagnosis allows for appropriate management and can prevent significant morbidity.

Objective: To elucidate the clinical and cellular characteristics of spinocerebellar ataxia type 35 (SCA35), which is caused by mutations in the TGM6 gene encoding transglutaminase 6 (TG6), in a Taiwanese cohort., Methods: Mutations in TGM6 were ascertained in 109 unrelated probands of Chinese descent with molecularly unassigned SCA from 512 pedigrees, in whom mutations responsible for 15 other ataxia syndromes had been excluded. The clinical features of all patients with a TGM6 mutation were systematically analyzed. The biological consequences of the newly identified TGM6 mutations were investigated in HEK293 cells transfected with mutant complementary DNA constructs., Results: Two missense mutations (p.R111C and p.D510H) and one 3-base pair deletion (p.E574del) in TGM6 were identified. Among them, p.R111C and p.E574del were novel. The common features of SCA35 include a slowly progressive clinical course, trunk/limb ataxia, and hand tremors. The age at onset varies from adolescence to the fifth decade. Torticollis and intellectual impairment are rare manifestations. Brain MRI reveals diffuse cerebellar atrophy without involvement of the cerebral hemispheres or brainstem. The 3 mutations identified here attenuated the protein stability and catalytic activities of TG6., Conclusions: SCA35 is an uncommon ataxia syndrome, accounting for 0.6% (3/512) of SCAs among the Han-Chinese descent in Taiwan. This study broadens the mutational spectrum of SCA35 and stresses the importance of TG6 in cerebellar functions., (© 2014 American Academy of Neurology.)

We report the case of a 7-year-old boy who presented with a 1-month history of neck pain, left-sided torticollis, and no neurological deficit. Computed tomography and magnetic resonance imaging revealed an expansile lesion in the axis, with epidural and prevertebral soft tissue components. Histopathologic examination of the biopsy specimen revealed primary vertebral Ewing sarcoma. This is the first case of primary vertebral Ewing sarcoma that has presented with torticollis. It is essential for physicians to be familiar with this condition and the associated imaging findings because early diagnosis of such cases is the key to better prognosis.

Anosognosia or lack of illness awareness is a clinical manifestation of both schizophrenia and right hemispheric lesions associated with stroke, neurodegeneration, or traumatic brain injury. It is thought to result from right hemispheric dysfunction or interhemispheric disequilibrium, which provides a neuroanatomical model for illness unawareness in schizophrenia. Lack of insight contributes to medication nonadherence and poor treatment outcomes and is often refractory to pharmacological and psychological interventions. We present the first report of transient illness awareness (<8 hours) after individual bilateral electroconvulsive therapy treatments in the case of a 39-year-old man with antipsychotic refractory schizophrenia. Electroencephalography demonstrated frontal slow wave activity with shifting frontotemporal predominance, which was concurrent with the patient's transient level of insight. A systematic review of the literature on electroconvulsive therapy-induced illness awareness in schizophrenia and psychotic disorders produced zero relevant results. Future research should focus on the prospective role of focal interventions, such as transcranial magnetic stimulation, in the development of a neurophysiological model for anosognosia reversal in schizophrenia that may, in turn, contribute to novel therapeutic developments targeting lack of illness awareness.

Study Design: A retrospective case series., Objective: To propose a novel treatment strategy for chronic atlantoaxial rotatory fixation (AARF)., Summary of Background Data: Treatment strategy for chronic or recurrent AARF remains controversial. We have previously reported that a deformity of the superior facet of the axis (C2 facet deformity), which is frequently observed in patients with chronic AARFs, is a risk factor for recurrent dislocation. In this article, we report seven consecutive cases of chronic AARF who underwent closed manipulation followed by external halo fixation and maintained good reduction with the remodeling of the C2 facet deformity., Methods: Seven girls with a chronic AARF who sustained torticollis for an average of 4.6 months after the onset were referred to our clinic. Closed manipulation by careful manipulation under general anesthesia followed by external immobilization with a halo vest was performed in all cases. Radiographic findings and clinical courses were retrospectively reviewed with approvals by the institutional review board., Results: Three-dimensional computed tomography images before reduction revealed persistent atlantoaxial subluxation and the C2 facet deformity in the dislocated side in all cases. Follow-up three-dimensional computed tomographic scans demonstrated the remodeling of the C2 facet deformity at an average of 2.8 months after successful reduction of subluxation. Subsequently, the halo vests were removed and gentle neck range of motion exercise was started in all cases. The normal cervical range of motion was obtained 2 weeks after the removal of halo vests in five cases, whereas the range of motion remained limited in two cases. At a mean follow-up of 17.4 months, neither symptoms nor recurrence of subluxation occurred in all cases., Conclusion: Chronic irreducible and recurrent unstable AARF can be managed successfully by careful closed manipulation followed by halo fixation, if the C1 and C2 have not been osseously fused. The remodeling of the C2 facet deformity detected on follow-up CT scans can be a useful radiographic parameter to determine the appropriate period of halo fixation in this new treatment strategy obviating the need for surgical intervention.

A 7-yr-old girl was diagnosed with atlantoaxial rotatory fixation, a serious but treatable cause of acquired torticollis in children and not well known by clinical physicians. Two and a half years after conservative treatment, she had recovered completely. This case report suggests that if the patient has no previous adequate rehabilitation treatment, then conservative treatments are effective for chronic but stable atlantoaxial rotatory fixation.

Unilateral congenital agenesis of sternocleidomastoid and trapezius muscles is a rare occurrence. To date, there are only two case reports in the literature. We present a case of a 7-year-old Caucasian boy who was born with torticollis and a cosmetic defect in his neck. An MRI scan of the cervical spine and shoulder confirmed the absence of the right sternocleidomastoid and trapezius muscles. No other significant congenital anomalies were detected. The patient has been followed-up for 7 years with no functional disabilities.

Study Design: A case report., Objective: We report a novel case of torticollis disorder because of a congenital split atlas after minor trauma., Summary of Background Data: Torticollis experienced after minor trauma in childhood is usually because of atlantoaxial rotatory fixation, which is a common disorder in pediatric patients and is usually diagnosed with computed tomography (CT). CT scanning with 3-dimensional reconstruction, however, showed a unique rotation of split atlas, a congenital anomaly that presented with torticollis., Methods: A female child aged 3 years and 11 months presented to the orthopedic clinic with torticollis after a fall. CT imaging showed no rotatory dislocation of C1-C2. On the 3-dimensional CT reconstruction images, however, anterior and posterior defects in the atlas, the so-called split atlas, and an atypical rotation with malalignment of the posterior arch and asymmetry of the atlantoaxial facet joint were noted. The child was treated with closed reduction using skull traction under general anesthesia., Result: Repositioning of the atlas rotation was confirmed using intraoperative 3-dimensional scanning, and open reduction therapy was avoided. The patient was treated with halo vest for 8 weeks and had full recovery of neck motion with resolution of the torticollis., Conclusion: We present a novel torticollis disorder caused by C1 rotation of a split atlas with closed reduction treatment.

Objective: To describe an infant with Lemierre-like syndrome caused by community-associated methicillin-resistant Staphylococcus aureus whose course was complicated by hemorrhagic pericarditis., Design: Case report., Setting: A 30-bed, pediatric intensive care unit at a tertiary care children's hospital., Patient: A 7-month-old infant presented with fever and torticollis attributable to a retropharyngeal abscess and left internal jugular venous thrombosis. He was treated with antibiotics and anticoagulation, and his course was complicated by hemorrhagic pericarditis and cardiac tamponade., Interventions: Resuscitation of shock; video-assisted thoracoscopic drainage of bilateral empyema with pleural decortication; vancomycin and clindamycin treatment of methicillin-resistant Staphylococcus aureus; incision and drainage of retropharyngeal abscess; treatment of internal jugular venous thrombus with anticoagulation; and treatment of pericardial tamponade by insertion of pericardial drain., Measurements and Main Results: Methicillin-resistant Staphylococcus aureus from blood and pleural fluid peel cultures were multi-locus sequence type 8, Panton-Valentine leukocidin-positive, and contained SCCmec IV, findings consistent with USA300 pulsotype. There was complete recovery from this life-threatening infection., Conclusions: Septic jugular venous thrombophlebitis complicating upper airway infections is a rare but serious acute medical condition. We present an infant with methicillin-resistant Staphylococcus aureus infection and clinical features that overlapped those described by Lemierre, in whom hemorrhagic pericarditis developed while receiving anticoagulation therapy. Anticoagulation, if used, should be closely monitored in this population.

Study Design: A retrospective study of cervical Langerhans cell histiocytosis (LCH)., Objective: To evaluate the safety and efficiency of the present diagnosis and treatment strategy., Summary of Background Data: The diagnosis and treatment protocols are still controversial for the rarity of cervical LCH., Methods: Thirty patients with cervical LCH were diagnosed in the past 10 years. Biopsy was routinely performed to establish the final diagnosis before treatment. Immobilization was usually the first choice. Low-dose radiotherapy was suggested for cases with solitary marked bony erosion and/or soft tissue extension, and chemotherapy for cases with multiple lesions. Surgery was preserved for suspected malignancy, neurologic deficits, severe deformity, and/or instability., Results: The mean age at diagnosis was 14.2 (range: 1.5-41) years old. Neck pain (96.7%) was the most common symptom, followed by restricted motion (70%), neurologic symptoms (36.7%), and torticollis (30%). Four cases had multiple lesions. Fourteen cases had atlantoaxial lesion and 16 cases were subaxial. The lesion extended to paravertebral soft tissue in 40% cases, to epidural space in 30%, to pedicle and/or transverse process in 56.3%. One case had endplate destruction. The accuracy of percutaneous needle biopsy under CT guidance was 91.2%. Eighteen patients had conservative treatment and 12 underwent operation. Three cases involving C2 vertebral body had fixed atlantoaxial anterior dislocation. Another 3 cases with atlantoaxial lateral mass destruction had spontaneous fusion. Eighteen patients had conservative treatment (1 only by immobilization, 13 by radiotherapy, 2 by chemotherapy, and 2 by combined chemotherapy and radiotherapy) and 12 underwent operation. All the initial symptoms were resolved, and there was no recurrence. From retrospective view, the surgical procedure might be avoided in 60% cases. Twenty-five cases had an average 61.6-month follow-up. In cases with severe bony collapse, the vertebral height ratio increased from 20.0% to 44.9% and the lateral mass height ratio from 22.2% to 56.8%., Conclusion: Cervical LCH lesions often extend to paravertebral soft tissue, epidural space, pedicles, and even to the endplate and lamina. Needle biopsy under CT guidance is safe and effective. The prognosis of cervical LCH is generally fair. Conservative treatment is usually enough and surgery should be reserved for major neurologic defects like myelopathy or monoparesis.

The etiologies of torticollis are numerous. We describe a unique patient who presented with torticollis with head tilting to the right after sustaining a minor fall. Computed tomography scan with two and three-dimensional reconstructions revealed an underlying hypoplasia of the right lateral mass of C1 and occipital condyle. Further study with MRI/magnetic resonance angiography showed hypoplasia of the right vertebral artery with compensatory dilatation of the left vertebral artery. We postulate these underlying anomalies predisposed the patient to torticollis, which became apparent after his fall. We discuss the surgical options available, and the rationale for a single C1-C2 transarticular screw with iliac crest bone graft and cable. In the literature, there are four reported cases of vascular anomalies resulting in torticollis. Our patient is the first, to our knowledge, who harbored both bony and vascular anomalies.

We present a case of atlantoaxial rotary subluxation after a minor injury managed by manual reduction under sedation. Atlantoaxial rotary subluxation should be considered in a child with inability or unwillingness to turn their head when history and physical examination do not suggest torticollis of benign etiology. In our discussion, we review etiology, diagnosis, as well as pre- and in-hospital management.

Study Design: Retrospective case review of children with eosinophilic granuloma of the cervical spine., Objective: To present the clinical features, radiographic findings, management, and results of 7 pediatric patients with eosinophilic granuloma of the cervical spine., Summary of Background Data: Management of the eosinophilic granuloma of the pediatric cervical spine is challenging, and must be adapted according to the patient, as no level I evidences have been produced. The therapeutic goals in these children are spinal stability, preservation of neurologic function, and relief of pain, always keeping in mind that patients are still growing. The localization in the cervical spine of children is extremely rare, with less than 50 patients reported in literature between 1966 and 2008., Methods: We reviewed the 7 pediatric patients with a diagnosis of eosinophilic granuloma of the cervical spine who were managed between 1970 and 1990 by the senior author. All patients presented with isolated cervical spine involvement, and all of them had a histologic diagnosis of eosinophilic granuloma (EG). All had open physes., Results: There were 5 boys and 2 girls (mean age: 10 years; range 4-16). We followed-up patients for 19 years (range 8-29). The symptoms at presentation varied according to the localization of the tumor in the cervical spine., Conclusion: Management of pediatric patients with EG of the cervical spine is challenging, especially when there is neurologic involvement. Observation alone, prolonged immobilization, systemic chemotherapy, curettage with or without bone grafting, corticosteroid injection, and low dose radiation therapy have been proposed for the management of patients with EG. Surgery is required when the child presents neurologic involvement. Children who received a vertebral interbody fusion can show, at long period follow-up, normal shape of the neck, with maintenance of the normal motion of the adjacent segments.

Grisel syndrome is the subluxation of atlantoaxial joint as a result of infectious or inflammatory processes of the head and neck region. The etiopathogenesis of this clinical entity is not clear yet. Early interventions (antibiotherapy, cervical traction, and immobilization) are critical to avoid catastrophic outcome. Early detection of pediatric atlantoaxial subluxation is essential. It requires a combination of clinical assessment and appropriate radiographic imaging. In this report, we present a clinical and radiologic follow-up of a Grisel syndrome; by this way, we discuss the role of radiology on the diagnosis of this rare entity and preview the relevant literature.

A 34-year-old woman developed a bilateral horizontal gaze palsy, left gaze deviation, and right face turn consequent to a pontine hemorrhage. A bilateral horizontal recession and resection of extraocular muscles in both eyes (Parks procedure) eliminated the gaze deviation and face turn. This is the first reported case in which this procedure was used to correct a face turn in a patient with bilateral horizontal gaze palsy but without ocular misalignment.

The purpose of this paper is to present a case of severe atlantoaxial rotatory fixation owing to a previously unreported etiology, and to discuss its pathogenesis and management. Conservative measures were unable to prevent progression, thus requiring surgical intervention.

Study Design: A case report., Objective: To describe an interesting patient who underwent open reduction and C1-C2 transpedicular screw fixation with interspinous wiring due to high-riding vertebral artery., Summary of Background Data: Atlantoaxial rotatory fixation (AARF) is a rare complication found most frequently after trauma in children and young adults; the clinical diagnosis is difficult and often made late., Methods: We report a case of post-traumatic AARF associated with a facet fracture in an adult. Reduction proved difficult to obtain by skull traction and gentle manipulation. Therefore, after open reduction under general anesthesia, we performed C1-C2 transpedicular screw fixation with posterior wiring to avoid vertebral artery injury due to high-riding transverse foramen at the C2 vertebra., Results: The normal atlantoaxial relation was restored and the torticollis resolved. The patient remains neurologically intact and has radiographic documentation of fusion., Conclusion: Although technically demanding, C1-C2 transpedicular screw fixation combined with interspinous bone graft wiring after open reduction may be an alternative treatment option if conservative treatment fails to reduce AARF.

Background: There is uncertainty as to whether spasmus nutans (SN) is an isolated idiopathic entity or whether there are underlying conditions that could cause or be associated with the nystagmus. We undertook this study to determine the frequency of ocular, intracranial, and systemic conditions in patients with nystagmus having characteristics of SN., Methods: We performed a chart review of 22 consecutive patients examined from 2000 through 2005 at the Dean McGee Eye Institute and Children' Hospital of Oklahoma with nystagmus consistent with SN. We collected information related to gender, age at presentation and age at final visit, visual acuity, refractive error, laterality of nystagmus, presence of head nodding and torticollis, pattern of strabismus, neuroimaging and electroretinography results, and other associated clinical findings., Results: Visual acuity was reduced in 75% of eyes at presentation and 58% of eyes at last visit. Eight patients had significant refractive error. Seven patients had strabismus. Two patients had chiasmal gliomas. Four patients had cone or rod/cone dystrophy. Only three patients had no associated ocular, intracranial, or systemic conditions., Conclusions: A substantial proportion of patients presenting with SN-like nystagmus have important underlying ocular, intracranial, or systemic abnormalities that may require evaluation and management.

Congenital muscular torticollis may present in late childhood or adulthood with cervical contractures, limited range of motion, pain, and muscular hypertrophy. Seven of eight pediatric and adult patients with congenital muscular torticollis were injected with botulinum toxin type A. Only two patients had a modest benefit with improved range of motion. Early recognition and treatment of congenital muscular torticollis is crucial to prevent subsequent contractures and other complications.

In this report we discuss the case of a child who was initially diagnosed at 1 month of age with congenital muscular torticollis. After falling off a slide at 22 months of age, the patient had onset of pain and an abrupt worsening of his torticollis. After a full workup, it was found that the patient had a C1 fracture and a disproportionately large ipsilateral occipital "coconut" condyle. We believe this congenital anomaly to be the cause of his original head tilt and also predisposed him to C1 fracture and worsening head tilt.

Study Design: A family study to reach the diagnosis of a multiple malformation syndrome., Objective: To determine the cause of torticollis, in a patient with the VATER association., Summary of Background Data: The VATER association is a combination of vertebral anomalies, anal stenosis, tracheo-esophageal fistula, and radial anomalies. It needs a multidisciplinary approach with a major input from orthopedic surgeons. Torticollis in this condition has not been reported before., Methods: Detailed family history and radiologic study using plain radiographs and three-dimensional-reconstruction., Results: Bony abnormalities at the base of the skull and upper cervical vertebrae were found., Conclusions: It is postulated that the bony abnormalities were the underlying cause of the neurologic problem.

Study Design: A case report., Objectives: To describe an interesting patient who underwent a three-level corpectomy of the cervical spine complicated by cervical dystonia and eventually treated successfully with botulinum toxin injections., Summary of Background Data: Cervical dystonia is a relatively rare disease and unfamiliar to many clinicians. Various types of peripheral trauma or peripheral lesion have been reported to induce cervical dystonia. However, to the best of our knowledge, there have been no reports about cervical dystonia following cervical spine surgery., Methods: We present a case of a 45-year-old man who developed severe axial neck pain after cervical anterior corpectomy and fibula strut grafting due to cervical myelopathy. His neck pain gradually worsened, and involuntary spasmodic neck movement developed 6 weeks after operation when his halo-vest was removed. Initially, we considered his complaint to be transient or psychogenic, and diagnosis of cervical dystonia was delayed until 14 weeks after operation., Results: Pharmacologic treatment was unsuccessful, but he was successfully treated with local intramuscular injections of botulinum toxin., Conclusions: It must be kept in mind that cervical spine surgery is not an exceptional precipitator of cervical dystonia, despite the fact that it is extremely rare.

Intrauterine crowding has been implicated as a risk factor in several orthopaedic conditions, such as developmental dysplasia of the hip (DDH), metatarsus adductus, and torticollis. The goal of this study was to see whether orthopaedic conditions associated with intrauterine crowding were more frequent in multiple gestation pregnancies, specifically in triplets. The authors reviewed their experience over a 10-year period with 261 children who were products of triplet pregnancies. They surveyed 13 orthopaedic conditions and found only one condition, torticollis, that had a greater incidence than that reported in single gestation pregnancies. A 0% incidence of DDH was found in these patients. Routine ultrasound screening cannot be recommended in these patients based on these results.

Traumatic spinal epidural hematoma is rare in children. The presentation could be nonspecific, leading to a delay in diagnosis. We present an infant who sustained an epidural hematoma after a "trivial" injury. The delay in diagnosis led to minor neurological deficit at 6-week follow-up. Irritability and torticollis after a neck trauma in an afebrile child should alert to the possibility of spinal cord compression. Early imaging and early decompression will minimize morbidity.

Study Design: We describe our surgical experience to remove osteoid osteomas and osteoblastomas of the occipitocervical junction. In this location, vertebral artery vicinity requires special consideration., Objectives: We illustrate our surgical approaches to remove lesions confidently, while minimizing bone resection to preserve stability., Summary of Background Data: Up until now, osteoid osteomas and osteoblastomas of the occipitocervical junction are reported as case reports. Several treatment methods have been described to treat osteoid osteomas. Nevertheless, surgery is the treatment of choice for lesions located in the C0-C2 region., Methods: A retrospective review of 7 patients, including 5 men and 2 women, with a mean age of 21.0 years (range 3.0-38.0) was conducted. Clinical outcomes were evaluated immediately and after a mean follow-up of 27.6 months., Results: There were 6 and 1 patients who underwent surgery with the anterolateral and posterolateral approaches, respectively. In osteoid osteomas, the nidus was removed, and the peripheral condensation was drilled up to normal bone (n = 4) or partially resected (n = 2). One osteoblastoma was removed extensively up to soft tissues. No osteo-arthrodesis was performed. Before surgery, all patients complained of pain, 3 presented with neck stiffness, and 2 with a torticollis. Immediately after surgery, all complaints disappeared. One patient underwent repeat surgery 15 months later for a recurrence. At the end of the follow-up, all patients were symptom-free, and partially resected peripheral condensations were stable on computerized tomography., Conclusions: Removal of osteoid osteomas and osteoblastomas of the occipitocervical junction is safe and efficient. Stability is preserved if more than half the joints are preserved with a proper surgical approach that minimizes bone resection.

The primary study aim was to identify characteristics of torticollis associated with plagiocephaly without synostosis (PWS) and to differentiate this from other forms of torticollis. The three commonest causes of infantile torticollis are idiopathic muscular causes such as sternocleidomastoid fibrosis, structural anomalies in the cervical vertebrae, and neurologic or ocular causes, such as certain types of strabismus. Recently, several units have reported increasing numbers of babies presenting with head tilt and reduced range of cervical motion, a form of torticollis apparently associated with PWS and unrelated to the etiologies enumerated above. A secondary aim was to identify local trends in referral patterns over the preceding 3 years. This was a retrospective review of 159 children attending a secondary referral center for management of positional plagiocephaly or torticollis. The data was collected in a university-affiliated child development clinic, and the records of one of three similar, community-based outpatient clinics, staffed by physical therapists and working in conjunction with our tertiary craniofacial surgery center were analyzed by medical staff. There was a persistent escalation in referrals for both PWS and associated torticollis (ie, not attributable to 1 of the 3 causes mentioned) over the study period. Anatomic and clinical features of PWS-associated torticollis were characterised and contrasted with the classic forms of torticollis. Current treatment strategies are outlined and discussed. Torticollis can predispose to PWS, but in a large proportion of our cases of PWS, torticollis appears to develop secondary to plagiocephaly, and the number of presentations is escalating.

Study Design: Report of an 11-year-old girl with a left atlantoaxial rotatory subluxation and ankylosis found 20 months after she sustained a cervical injury., Objective: To describe the radiographic characteristics of this rare deformity assessed with a combination of spiral CT scan with multiplanar three-dimensional reformations and functional CT scan., Summary of Background Data: Atlantoaxial rotatory subluxation is a well-known condition, but its association with lateral C1-C2 ankylosis has not been reported to our knowledge., Methods: For a complete assessment of the dislocation, a combined morphologic volumetric and functional CT study was performed., Results: Spiral CT showed an atlantoaxial rotatory subluxation with lateral C1-C2 ankylosis. CT study also demonstrated a lateral C1-C2 subluxation and an ipsilateral occipitoatlantal subluxation. Cervical MRI showed no spinal cord compression despite the seriousness of the dislocation process., Conclusions: Whereas "classic" spiral study with multiplanar and three-dimensional reformations allows precise assessment of relationships between the upper cervical vertebrae, as well as bony changes, a functional CT study is essential for cervical biomechanic assessment of rotational instabilities of the craniovertebral junction and upper cervical spine.

Study Design: This report describes a case of successful surgical treatment of eosinophilic granuloma of the atlas in a 3.5-year-old boy who presented with torticollis., Objective: The purpose of this report was to illustrate the rare clinical presentation of eosinophilic granuloma in the atlas., Summary of Background Data: Eosinophilic granuloma, a benign solitary lesion that commonly affects children, has a variable clinical course. Although eosinophilic granuloma has been reported to occur in the cervical spine, there have been only five reported cases of eosinophilic granuloma affecting the atlas. The management of eosinophilic granuloma ranges from observation and immobilization of the cervical spine to surgical excision., Methods: The tumor was located on the left lateral mass of the atlas. Biopsy and curettage were performed through an oblique incision through the posterior border of the sternocleidomastoid muscle. A histopathologic evaluation confirmed the diagnosis of eosinophilic granuloma., Results: The patient tolerated the procedure with no complications, and his torticollis has completely resolved., Conclusion: Eosinophilic granuloma of the atlas initially presented in this patient as torticollis. Biopsy for confirmation of diagnosis and curettage for treatment provided a successful outcome in this patient. We suggest biopsy of the lesion and histopathologic evaluation to confirm the diagnosis in atypical cases.

Significant neck burns may lead to deforming lateral flexion and rotation contractures. A two-device splinting regimen has been designed to prevent such contractures. In the acute phase, the Dynamic Antitorticollis Strap is applied while the patient is in bed to gently rotate the head and neck toward the neutral position. This dynamic strap includes a Velfoam headband attached to Thera-Band secured to the patient's bed. The antitorticollis neck splint is used in the rehabilitation phase and can be serially adjusted to correct lateral flexion contractures of the neck. Thermoplastic material is cut from a modified neck splint pattern and draped over the temporaloccipital region and anterior/posterior shoulder ipsilateral to the contracture and the anterior and contralateral aspect of the neck. The combined use of these devices during the scar maturation phase provides therapists with alternatives in preventing burn scar torticollis.

Two cases of clavicle fractures associated with atlantoaxial rotatory displacement as well as 11 additional cases from the literature are presented. Most cases occur in girls 6 to 10 years old. The head is most often laterally bent toward and rotated away from the fractured clavicle. There is often a delay in making the diagnosis, which is best demonstrated by dynamic CT. Treatment of the clavicle fracture is straightforward, but failure to recognize and treat the C1-2 rotatory displacement promptly can lead to fixed C1-2 deformity. Displacement recognized within the first 3 weeks of injury can be successfully treated nonoperatively. A delay of 6 weeks or greater in the diagnosis leads to fixed deformity at C1-2 that can be addressed with a limited cervical fusion.

Study Design: A case of nontraumatic rotatory fixation of the atlantoaxial joint associated with tuberculosis of the occipital bone in an adult., Objectives: To report a rare case of atlantoaxial rotatory subluxation associated with tuberculosis of the occipital bone in an adult and to discuss the mechanism of fixation., Summary of the Background Data: Atlantoaxial rotatory fixation in adults is rare and has been reported due to variety of causes. To the authors' best knowledge no case has been reported secondary to tuberculosis of the skull bone., Methods: A 20-year-old male presented with resistant torticollis with a duration of 5 months., Results: The patient had type 1 atlantoaxial rotatory fixation secondary to tuberculosis of the occipital bone. The subluxation was partially reduced by conservative means, and healing of the occiput lesion was achieved. Thereafter, the patient had no restriction of cervical spine motion and had no reoccurrence of subluxation at a follow-up of one and a half years., Conclusions: Effusion in the atlantoaxial joint secondary to infection in the occiput due to close proximity with the joint led to the laxity of ligaments and contributed to the subluxation.

For children with torticollis, dynamic computed tomography scanning (DCTS) is the imaging modality of choice in diagnosing atlantoaxial rotatory subluxation (AARS). At present, there is no grouping system based on DCTS to determine severity and direct treatment. Fifty children with torticollis underwent DCTS in the workup for AARS. The relative rotation of C1 versus C2 was compared for left and right rotation views. Each DCTS was classified: stage 0, torticollis but normal DCTS; stage 1, limitation of motion (<15 degrees difference between C1 and C2, but C1 crosses midline of C2); and stage 2, fixed (C1 does not cross midline of C2). Duration of symptoms and treatment were compared. There were 27 girls and 23 boys with a mean age of 8.2 years. There were 8 stage 0, 30 stage 1, and 12 stage 2 DCTS. Average onset of symptoms to diagnosis was 6.7 days for stage 0, 8.6 days for stage 1, and 20 days for stage 2. A significant trend was found between increasing intensity of treatment and stage. Using this grouping system, the authors found that patients with a higher stage had an increase in the mean duration of symptoms and intensity of treatment.

Dynamic computerized tomography (DCT) has been accepted to be the standard diagnostic method of atlantoaxial rotatory subluxation (AARS) although its reliability and reproducibility has not been shown yet. The purpose of this study was to evaluate the intraobserver reproducibility and interobserver reliability of DCT. Standard DCT scans of 18 patients with acute torticollis and 12 normal subjects were examined two times in between a time interval of 1 month by three specialists and a last-year resident to define any existing AARS. The interobserver reliability kappa coefficient was -0.015 (poor) for the first examination and 0.327(fair) for the second one. The intraobserver reproducibility kappa coefficients were 0.135 (slight), -0.204 (poor), 1.00 (almost perfect), and 0.474 (moderate) respectively. It was found that DCT has a poor reliability and reproducibility in diagnosing AARS in patients with acute torticollis. Therefore, its routine use is not cost effective in patients with acute torticollis where the deformity usually resolves by a simple cervical mobilization.

Rotatory displacement of the atlantoaxial joint is one of the causes of torticollis in children. Some of these cases show persistent symptoms and may lead to rotatory fixation; however, only a few studies have been directed to the prognosis of atlantoaxial rotatory displacement after conservative treatment. Clinical and radiographic reviews were performed in 35 patients (16 male and 19 female) with rotatory displacement of the atlantoaxial joint. The mean age at admission was 6.5 (range: 2-12) years old, and the mean follow-up period was 3.4 (1.4-5.8) years. All patients successfully achieved reduction after 2 to 3 weeks of continuous halter traction; however, 9 patients (25.7%) experienced recurrence, and 7 patients (20.0%) had a slight torticollis at follow-up. The duration of symptoms before treatment affected the recurrence rate, and the torticollis was apt to remain in cases with severe displacement at admission.