| Abstract|| |
Chiari 1 malformations (CIM) in children can develop into a debilitating disease, however, they usually present with apparently benign symptoms, such as moderate headache and neck pain. Early identification and treatment of this condition can lead to an overall decrease in morbidity and mortality associated with this disease. We are reporting on a child that initially presented with neck pain and no neurological findings.
Keywords: Chairi 1 malformation, children, neck pain
|How to cite this article:|
Haran JP, Pezzella L. A case of neck pain: The presentation of Chairi 1 malformations in children. J Emerg Trauma Shock 2014;7:32-4
| Introduction|| |
Chiari malformations are defects of the central nervous system (CNS) in which a portion of the cerebellum is located below the foramen magnum. Four types of malformations have been described, which are defined on the basis of extent of cerebellar herniation. Type one is the most prevalent, with estimates ranging from 1 in 1,000 to 5,000 individuals.  While these malformations can be both congenital and acquired, Chiari 1 malformations (CIM) are most commonly due to congenital underdevelopment of the posterior cranial fossa, resulting in downward displacement of one or both cerebellar tonsils below the foramen magnum. This herniation causes impaction of the tonsils and blocks the flow of cerebrospinal fluid at the level of the foramen; causing cerebellar, brainstem, and cervical cord symptoms.  Syringomyelia or hydrosyringomyelia is the most commonly associated finding, occurring in 65-80% of patients with CIM, and is often considered an indication for surgery.
CIM in children can develop into a debilitating disease, however, they usually present with apparently benign symptoms. Early identification and treatment of this condition will lead to an overall decrease in morbidity and mortality associated with this disease. We are reporting on a case that illustrates the importance of considering magnetic resonance imaging (MIR) for children with chronic neck pain with no identifiable cause and have subtle or even no neurological findings at all.
| Case Report|| |
A 16-year-old child was brought to the pediatric emergency department (ED) with headache and neck pain lasting 1 week. She had stiffness and a throbbing occipital headache, occurring suddenly after her gymnastics practice. Both the headache and neck pain were worsened with movement, laughing, and coughing; and neither had changed since onset. She did not recall a specific injury, but her routines included tumbling movements with both flexion and extension of her neck. A complete review of systems and medical history was notable only for 3 days of cough and mild sore throat. There was no personal or family history of headaches.
In the ED, she was alert and oriented with normal vital signs. On examination, her neck was stiff with pain to passive and active flexion/extension or rotation, and severely limited range of motion. No neck mass or adenopathy were noted. Neurological examination was nonfocal with normal cranial nerve exam, strength, reflexes, sensation, gait, and cerebellar testing. A complete physical examination was conducted, and was otherwise normal.
An MRI of the brain, cervical, and thoracic spine was obtained which revealed a 1.1 cm downward displacement of the cerebellar tonsils through the foramen magnum consistent with a CIM [Figure 1]. There was a syrinx seen at the level of T7-T8 measuring 3 mm × 2.5 cm, and left thoracolumbar convex scoliosis. The patient was admitted to the hospital and scheduled for urgent Chiari decompression surgery.
|Figure 1: (a) An MRI of brain performed during the initial ED visit showing a 1.1 cm downward displacement of the tonsils through the foramen magnum (arrow), (b) An MRI of spine demonstrated a 3 mm × 2.5 cm syrinx at the level of T7-T8 (arrow)|
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| Discussion|| |
Neck pain in children is a common presenting complaint in the emergency department (ED), accounting for about 2% of all pediatric ED visits.  The most common causes are trauma and infection, however there are many other underlying causes. 
Children with Chiari malformations can have unusual presentations; however the most common presenting symptom is suboccipital headache or neck pain (60-70%) which classically is aggravated by coughing, neck extension, exertion and other Valsalva maneuvers. Other common symptoms include arm pain, leg pain, numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. 
Upon examination, patients with CIM may have severe neurological findings or no findings at all. Up to 30% of pediatric patients with 5-10 mm herniation are completely asymptomatic, 12% have symptoms without neurological signs, and the remainder of patients had varying degrees of neurological abnormalities.  Any history of scoliosis and a convexity of the thoracic spine towards the left may be an important clue, as it is seen frequently in CIM patients.  Scoliosis is seen frequently in cases of CIM especially those associated with syringomyelia.  Progressive scoliosis is a common manifestation in CIM (30%) when there is a coexisting syringomyelia, and the diagnosis should be entertained in patients with scoliosis, particularly with a convexity towards the left.  Scoliosis in any pediatric patient with neck pain should raise suspicion of CIM, and is an important historical clue when considering this diagnosis.
MRI of both the brain and spine is essential to the diagnosis. Until the advent of MRI, the diagnosis of CIM was typically made in adolescents or adults, and required invasive investigations.  Since the noninvasive MRI has come into use, the diagnosis of CIM is being made in younger patients earlier in the course of their disease, when more subtle symptoms are present.  There are many benefits to early detection of CIM. The most important of which is the benefit of early surgical treatment, which has been shown to prevent disease progression.  There is no effective nonoperative alternative for the treatment of CIM, and furthermore the benefit of surgery has been well-documented in multiple retrospective studies over the past several decades.  Surgical intervention before adulthood is crucial, with dramatically better neurological outcomes seen in children compared to adults. More then 90-95% of children under the age of 16 years experience complete resolution of symptoms 1 year post-operation.  Many patients who go undiagnosed into adulthood develop debilitating neurological manifestations, which could have been avoided if their condition was diagnosed earlier.
| Conclusion|| |
Pediatric patients with a CIM can pose a diagnostic challenge for the ED physician, with unusual presentations that lack neurological findings, leading to neurological complications from both wrong diagnosis and delay in treatment. Children presenting with chronic head or neck pain, pain exacerbated by valsalva maneuvers, or children with pain and any neurological abnormalities could benefit from MRI evaluation for CIM. Consistent headache without a clear cause has become a relative indication for brain imaging by MRI.  Early diagnosis and definitive treatment of patients with subtle clinical presentations of CIM can lead to improved neurological outcomes in a population otherwise at risk for serious debilitating disease.
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John P Haran
Department of Emergency Medicine, University of Massachusetts Medical School, Worcester, Massachusetts
Source of Support: None, Conflict of Interest: None