- Email: [email protected]
Neck Spasms in Children
EM:RAP COMMENTARY
Neck Spasms in Children Andrew Grock, MD*; David Ebenezer, MD; Paul Jhun, MD; Mel Herbert, MD *Corresponding Author. E-mail: [email protected], Twitter: @andygrock. 0196-0644/$-see front matter Copyright © 2016 by the American College of Emergency Physicians. http://dx.doi.org/10.1016/j.annemergmed.2016.05.028
SEE RELATED ARTICLE, P. e5. [Ann Emerg Med. 2016;68:126-128.] Editor’s note: Annals has partnered with Hippo Education and EM:RAP, enabling our readers without subscriptions to Hippo EM Board Review or EM:RAP to enjoy their commentary on Annals publications. This article did not undergo peer review and may not reflect the view and opinions of the editorial board of Annals of Emergency Medicine. There are no financial relationships or other consideration between Annals and Hippo Education, EM:RAP or its authors.
ANNALS CASE A 9-year-old boy with a history of asthma presents with acute, right-sided neck pain after a headstand. His vital signs are within normal limits and his physical examination demonstrates mild torticollis with an inability to range his neck. Radiographs and computed tomography (CT) scans reveal atlantoaxial rotatory fixation, also called atlantoaxial rotatory subluxation (AARS).1 HEY, MOM, I ALMOST DECAPITATED MYSELF! Honestly, the cat got our tongues on this one. As if there weren’t enough things to worry about in kids, add nearinternal decapitation to the list. We’ll talk about our approach to torticollis in a minute, but first a few words on the awkward elephant in the room. In digging through the literature, we found that AARS is uncommon enough that there are no good articles documenting its incidence (go figure). But, from what we do know, it almost always presents in children with torticollis after minor trauma, minor surgery, or an upper respiratory tract infection.2 But seriously, what child anywhere hasn’t had a recent minor trauma or upper respiratory tract infection? Turns out that the inflammation from the trauma or infection leads to laxity in the cervical alar or transverse ligaments, which destabilizes the atlantoaxial joint and allows subluxation of C1 on C2.3 This totally brings new meaning to the phrase “kids are pliable.” Yikes! Traditionally, the child’s head is described as being in the “cock robin” 126 Annals of Emergency Medicine
position, named for the look a robin has while listening for a worm: head laterally flexed to one side, rotated to the opposite side, and with slight flexion.4 With AARS, patients are often unable to return their heads to a neutral position.4 And yet, despite kids’ best attempts at internal decapitation, neurologic findings in AARS are rare, except for irritation to the greater occipital nerve as it emerges between C1 and C2.4 Diagnosis can be made by radiograph or CT, but CT is the recommended imaging modality because management depends on the degree of subluxation (Figures 1 and 2). Treatment depends on duration of symptoms, as well as classification. The consultant you’ll want to call is a pediatric orthopedic surgeon. Class 1 AARS with a symptom duration of less than 1 week often self-reduces with observation and can be treated with soft collar immobilization and supportive care.5 Symptom duration for more than 1 week, failure of usual therapies, and classes 2, 3, and 4 may require more aggressive interventions, like non-operative (Halter) traction, manual reduction in the operating room with cervical collar or Halo vest application, or even open reduction with posterior spinal fusion.2,3,6,7 One case series of 20 patients reported that 4 injuries reduced with a collar alone, 10 reduced successfully with traction, and 6 required posterior fusion because of traction failure or recurrence.8 Prolonged time to reduction was associated with an increased risk of requiring surgery.8 A BOARD REVIEW MOMENT Do you remember those conditions that increase atlantoaxial instability? Juvenile rheumatoid arthritis, Down’s syndrome, Marfan’s syndrome, osteogenesis imperfecta, and rickets. No surprise here, but these conditions increase the risk for AARS.2 In adults, although rare, AARS has been documented to occur in rheumatoid arthritis patients, in up to 18% (yikes!), because of chronic inflammation of the alar and transverse ligaments.9 APPROACH TO TORTICOLLIS IN KIDS Now that we’ve probably caused you some consternation about how many kids with torticollis you sent home with Volume 68, no. 1 : July 2016
Grock et al
EM:RAP Commentary
and—let’s be honest—what do we all want it to be? Muscle strain. And, although the direct problem involves abnormal sternocleidomastoid contraction, there are a few critical underlying diagnoses we can’t miss that present with torticollis.
Figure 1. Radiographic classification of AARS.5
potential AARS, let’s review a simplified approach to torticollis in the acute care setting. Torticollis, or involuntary twisting with the head tilted in one direction while the chin rotates oppositely, can seem like a benign chief complaint,
DRUGS, INFECTION, TRAUMA, NEUROLOGIC EXAMINATION As with all things in medicine, torticollis has a laundry list of causes, but you’ll cover the majority of bad stuff by doing a good history and physical examination. Ask about drugs that cause torticollis, look for deep neck infections, ask about any history of trauma, and do a good neurologic examination. Many of us are all too familiar with drug-induced dystonic reactions (ever call a code stroke on that?). Neuroleptics are the classic offenders and intravenous diphenhydramine
Figure 2. Anatomic depiction of AARS.11 Volume 68, no. 1 : July 2016
Annals of Emergency Medicine 127
Grock et al
EM:RAP Commentary
can work miracles. Moving on to deep neck infections, think about infections deep to or involving the sternocleidomastoid: retropharyngeal and peritonsillar abscesses, cervical adenitis, or sternocleidomastoid myositis, to name a few. Dysphagia, drooling, trismus, or “hot potato voice” are all classic red flags. Trauma is, well, trauma (and now you can add AARS to your differential.lucky you). Heck, even when we walked down memory lane and looked at our commentary in the May 2016 issue of Annals of Emergency Medicine we saw that apparently golf-pencil-induced penetrating neck injuries cause torticollis in children.10 And if any of you have ever had the pleasure of being involved in a medicolegal case, you should know by now to always document a good neurologic examination. The important items you don’t want to miss include increased intracranial pressure, posterior fossa tumors, or cervical spinal cord pathology. As you can imagine, checking for papilledema and performing cranial nerve and cerebellar examinations are useful screening tools (eg, diplopia, cranial nerve palsies, gait ataxias), as well as identifying red flag symptoms such as headache and vomiting. BACK TO THE CASE The patient’s injury was reduced manually by orthopedics and immobilized externally with a Philadelphia collar, and he was discharged with orthopedic follow-up.1 Seriously, we’ve all heard that kids are pliable, but we’re pretty sure that after reading this, you’re not going to be looking at kids with torticollis the same. Author affiliations: From the Department of Emergency Medicine, University of Southern California, Los Angeles, CA (Grock, Herbert); the Department of Pediatric Orthopedics, Valley Children’s Hospital, Madera, CA (Ebenezer); and the Department of
Emergency Medicine, University of California San Francisco, San Francisco, CA (Jhun). Funding and support: By Annals policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article as per ICMJE conflict of interest guidelines (see www.icmje.org). The authors have stated that no such relationships exist and provided the following details: Dr. Jhun reports other from Hippo Education, Inc., outside of the submitted work. Dr. Herbert reports other from EM:RAP and Hippo Education, Inc., outside of the submitted work.
REFERENCES 1. Sonoo T, Sato T, Shota D, et al. Child with acute neck pain. Ann Emerg Med. 2016;68:e5-e6. 2. Tomczak KK, Rosman NP. Torticollis. J Child Neurol. 2013;28: 365-378. 3. Crook TB, Eynon CA. Traumatic atlantoaxial rotatory subluxation. Emerg Med J. 2005;22:671-672. 4. Van Holsbeeck EM, MacKay NN. Diagnosis of acute atlanto-axial rotatory fixation. J Bone Joint Surg. 1989;71:90-91. 5. Copley LA. Disorders of the neck. In: Herring JA, ed. Tachdjian’s Pediatric Orthopaedics. 5th ed. Philadelphia, PA: Saunders-Elsevier; 2014:167-205. 6. Fielding JW, Hawkins RJ. Atlanto-axial rotatory fixation. (Fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg. 1977;59: 37-44. 7. Gomez J. Acute atlantoaxial rotatory subluxation (AARS). Pediatric Society of North America. Available at: https://posna.org/PhysicianEducation/Study-Guide/Acute-Atlantoaxial-Rotary-Subluxation(AARS). Accessed April 29, 2016. 8. Subach BR, McLaughlin MR, Albright AL, et al. Current management of pediatric atlantoaxial rotatory subluxation. Spine. 1998;23:2174-2179. 9. Neva MH, Häkkinen A, Mäkinen H, et al. High prevalence of asymptomatic cervical spine subluxation in patients with rheumatoid arthritis waiting for orthopaedic surgery. Ann Rheum Dis. 2006;65: 884-888. 10. Roepke C, Benjamin E, Jhun P, et al. Penetrating neck injuries: what’s in and what’s out. Ann Emerg Med. 2016;67:578-580. 11. Available at: https://posna.org/Physician-Education/Study-Guide/ Acute-Atlantoaxial-Rotary-Subluxation%28AARS%29. Accessed June 1, 2016.
Annals of Emergency Medicine is seeking up to two persons with interest in traffic injury prevention to assist with editing of the NHTSA Notes section. Responsibilities include recruitment of guest writers, selection of NHTSA reports for commentaries, authoring 1-2 commentaries per year, and editing of submitted commentaries. Please contact NHTSA Notes Section Editor Christopher Kahn, MD at [email protected] with any queries or interest.
128 Annals of Emergency Medicine
Volume 68, no. 1 : July 2016
Neck Spasms in Children Andrew Grock, MD*; David Ebenezer, MD; Paul Jhun, MD; Mel Herbert, MD *Corresponding Author. E-mail: [email protected], Twitter: @andygrock. 0196-0644/$-see front matter Copyright © 2016 by the American College of Emergency Physicians. http://dx.doi.org/10.1016/j.annemergmed.2016.05.028
SEE RELATED ARTICLE, P. e5. [Ann Emerg Med. 2016;68:126-128.] Editor’s note: Annals has partnered with Hippo Education and EM:RAP, enabling our readers without subscriptions to Hippo EM Board Review or EM:RAP to enjoy their commentary on Annals publications. This article did not undergo peer review and may not reflect the view and opinions of the editorial board of Annals of Emergency Medicine. There are no financial relationships or other consideration between Annals and Hippo Education, EM:RAP or its authors.
ANNALS CASE A 9-year-old boy with a history of asthma presents with acute, right-sided neck pain after a headstand. His vital signs are within normal limits and his physical examination demonstrates mild torticollis with an inability to range his neck. Radiographs and computed tomography (CT) scans reveal atlantoaxial rotatory fixation, also called atlantoaxial rotatory subluxation (AARS).1 HEY, MOM, I ALMOST DECAPITATED MYSELF! Honestly, the cat got our tongues on this one. As if there weren’t enough things to worry about in kids, add nearinternal decapitation to the list. We’ll talk about our approach to torticollis in a minute, but first a few words on the awkward elephant in the room. In digging through the literature, we found that AARS is uncommon enough that there are no good articles documenting its incidence (go figure). But, from what we do know, it almost always presents in children with torticollis after minor trauma, minor surgery, or an upper respiratory tract infection.2 But seriously, what child anywhere hasn’t had a recent minor trauma or upper respiratory tract infection? Turns out that the inflammation from the trauma or infection leads to laxity in the cervical alar or transverse ligaments, which destabilizes the atlantoaxial joint and allows subluxation of C1 on C2.3 This totally brings new meaning to the phrase “kids are pliable.” Yikes! Traditionally, the child’s head is described as being in the “cock robin” 126 Annals of Emergency Medicine
position, named for the look a robin has while listening for a worm: head laterally flexed to one side, rotated to the opposite side, and with slight flexion.4 With AARS, patients are often unable to return their heads to a neutral position.4 And yet, despite kids’ best attempts at internal decapitation, neurologic findings in AARS are rare, except for irritation to the greater occipital nerve as it emerges between C1 and C2.4 Diagnosis can be made by radiograph or CT, but CT is the recommended imaging modality because management depends on the degree of subluxation (Figures 1 and 2). Treatment depends on duration of symptoms, as well as classification. The consultant you’ll want to call is a pediatric orthopedic surgeon. Class 1 AARS with a symptom duration of less than 1 week often self-reduces with observation and can be treated with soft collar immobilization and supportive care.5 Symptom duration for more than 1 week, failure of usual therapies, and classes 2, 3, and 4 may require more aggressive interventions, like non-operative (Halter) traction, manual reduction in the operating room with cervical collar or Halo vest application, or even open reduction with posterior spinal fusion.2,3,6,7 One case series of 20 patients reported that 4 injuries reduced with a collar alone, 10 reduced successfully with traction, and 6 required posterior fusion because of traction failure or recurrence.8 Prolonged time to reduction was associated with an increased risk of requiring surgery.8 A BOARD REVIEW MOMENT Do you remember those conditions that increase atlantoaxial instability? Juvenile rheumatoid arthritis, Down’s syndrome, Marfan’s syndrome, osteogenesis imperfecta, and rickets. No surprise here, but these conditions increase the risk for AARS.2 In adults, although rare, AARS has been documented to occur in rheumatoid arthritis patients, in up to 18% (yikes!), because of chronic inflammation of the alar and transverse ligaments.9 APPROACH TO TORTICOLLIS IN KIDS Now that we’ve probably caused you some consternation about how many kids with torticollis you sent home with Volume 68, no. 1 : July 2016
Grock et al
EM:RAP Commentary
and—let’s be honest—what do we all want it to be? Muscle strain. And, although the direct problem involves abnormal sternocleidomastoid contraction, there are a few critical underlying diagnoses we can’t miss that present with torticollis.
Figure 1. Radiographic classification of AARS.5
potential AARS, let’s review a simplified approach to torticollis in the acute care setting. Torticollis, or involuntary twisting with the head tilted in one direction while the chin rotates oppositely, can seem like a benign chief complaint,
DRUGS, INFECTION, TRAUMA, NEUROLOGIC EXAMINATION As with all things in medicine, torticollis has a laundry list of causes, but you’ll cover the majority of bad stuff by doing a good history and physical examination. Ask about drugs that cause torticollis, look for deep neck infections, ask about any history of trauma, and do a good neurologic examination. Many of us are all too familiar with drug-induced dystonic reactions (ever call a code stroke on that?). Neuroleptics are the classic offenders and intravenous diphenhydramine
Figure 2. Anatomic depiction of AARS.11 Volume 68, no. 1 : July 2016
Annals of Emergency Medicine 127
Grock et al
EM:RAP Commentary
can work miracles. Moving on to deep neck infections, think about infections deep to or involving the sternocleidomastoid: retropharyngeal and peritonsillar abscesses, cervical adenitis, or sternocleidomastoid myositis, to name a few. Dysphagia, drooling, trismus, or “hot potato voice” are all classic red flags. Trauma is, well, trauma (and now you can add AARS to your differential.lucky you). Heck, even when we walked down memory lane and looked at our commentary in the May 2016 issue of Annals of Emergency Medicine we saw that apparently golf-pencil-induced penetrating neck injuries cause torticollis in children.10 And if any of you have ever had the pleasure of being involved in a medicolegal case, you should know by now to always document a good neurologic examination. The important items you don’t want to miss include increased intracranial pressure, posterior fossa tumors, or cervical spinal cord pathology. As you can imagine, checking for papilledema and performing cranial nerve and cerebellar examinations are useful screening tools (eg, diplopia, cranial nerve palsies, gait ataxias), as well as identifying red flag symptoms such as headache and vomiting. BACK TO THE CASE The patient’s injury was reduced manually by orthopedics and immobilized externally with a Philadelphia collar, and he was discharged with orthopedic follow-up.1 Seriously, we’ve all heard that kids are pliable, but we’re pretty sure that after reading this, you’re not going to be looking at kids with torticollis the same. Author affiliations: From the Department of Emergency Medicine, University of Southern California, Los Angeles, CA (Grock, Herbert); the Department of Pediatric Orthopedics, Valley Children’s Hospital, Madera, CA (Ebenezer); and the Department of
Emergency Medicine, University of California San Francisco, San Francisco, CA (Jhun). Funding and support: By Annals policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article as per ICMJE conflict of interest guidelines (see www.icmje.org). The authors have stated that no such relationships exist and provided the following details: Dr. Jhun reports other from Hippo Education, Inc., outside of the submitted work. Dr. Herbert reports other from EM:RAP and Hippo Education, Inc., outside of the submitted work.
REFERENCES 1. Sonoo T, Sato T, Shota D, et al. Child with acute neck pain. Ann Emerg Med. 2016;68:e5-e6. 2. Tomczak KK, Rosman NP. Torticollis. J Child Neurol. 2013;28: 365-378. 3. Crook TB, Eynon CA. Traumatic atlantoaxial rotatory subluxation. Emerg Med J. 2005;22:671-672. 4. Van Holsbeeck EM, MacKay NN. Diagnosis of acute atlanto-axial rotatory fixation. J Bone Joint Surg. 1989;71:90-91. 5. Copley LA. Disorders of the neck. In: Herring JA, ed. Tachdjian’s Pediatric Orthopaedics. 5th ed. Philadelphia, PA: Saunders-Elsevier; 2014:167-205. 6. Fielding JW, Hawkins RJ. Atlanto-axial rotatory fixation. (Fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg. 1977;59: 37-44. 7. Gomez J. Acute atlantoaxial rotatory subluxation (AARS). Pediatric Society of North America. Available at: https://posna.org/PhysicianEducation/Study-Guide/Acute-Atlantoaxial-Rotary-Subluxation(AARS). Accessed April 29, 2016. 8. Subach BR, McLaughlin MR, Albright AL, et al. Current management of pediatric atlantoaxial rotatory subluxation. Spine. 1998;23:2174-2179. 9. Neva MH, Häkkinen A, Mäkinen H, et al. High prevalence of asymptomatic cervical spine subluxation in patients with rheumatoid arthritis waiting for orthopaedic surgery. Ann Rheum Dis. 2006;65: 884-888. 10. Roepke C, Benjamin E, Jhun P, et al. Penetrating neck injuries: what’s in and what’s out. Ann Emerg Med. 2016;67:578-580. 11. Available at: https://posna.org/Physician-Education/Study-Guide/ Acute-Atlantoaxial-Rotary-Subluxation%28AARS%29. Accessed June 1, 2016.
Annals of Emergency Medicine is seeking up to two persons with interest in traffic injury prevention to assist with editing of the NHTSA Notes section. Responsibilities include recruitment of guest writers, selection of NHTSA reports for commentaries, authoring 1-2 commentaries per year, and editing of submitted commentaries. Please contact NHTSA Notes Section Editor Christopher Kahn, MD at [email protected] with any queries or interest.
128 Annals of Emergency Medicine
Volume 68, no. 1 : July 2016