When a headache never goes away - Chronic daily headache in a teenager

Transcription

1 PBLD Table #6 When a headache never goes away - Chronic daily headache in a teenager Moderators: Taran Sangari M.D., Shailesh Shah M.D. Institution: Valley Children s Hospital, Madera, CA Objectives: Discuss the presentation and examination of a patient with chronic headaches Review the differential diagnosis of chronic headache in pediatric patient population Understand the characteristics of Ehlers-Danlos Syndrome when evaluating a teenager with chronic daily headache Develop pain management strategies in pediatric patients with chronic headache Understand the importance of adopting multidisciplinary, multimodal pain management approach for treatment of chronic daily headache. Case history: A 14 year old female presented with 2 year history of intractable headache, which had become daily over the past 3 months. The pain was located in frontal area but would radiate to parietal-occipital area and her neck. Questions: What is a chronic daily headache? What is the most common cause of chronic daily headache? What is the prevalence of headache in adolescent population? What is the difference between primary and secondary headaches? There was a strong family history of migraine and depression for which the patient was referred to a psychiatrist. She was an avid long distance runner and softball player in past but for last two years since her pain worsened she quit sports. Per her mother she has gained about 30 pounds since last year and quit school about 2 months ago. At present she was getting home schooled by her mother. Questions: What is the differential diagnosis for this adolescent? What does your usual work-up for these kinds of patients include? List the goals of chronic pain management in children What are the effects of chronic pain in children? Outline the commonly employed management strategies for chronic pain in children Discuss the benefits of adopting a multidisciplinary approach when managing chronic pediatric pain

2 What is the typical management strategy for headache patients? She had characteristics of a migraine with photophobia and phono phobia associated with nausea and vomiting. She denied any visual changes or focal neurological deficits. She also reported history of syncope; fatigue with generalized body ache progressively getting worse with history of hypermobility of joints. She described night time awakening due to her pain with day time drowsiness. Questions: What is Ehlers Danlos syndrome (EDS)? Describe the physical examination findings in a patient with EDS How is EDS diagnosed? Discuss the complex presenting features of EDS. Discuss pain management options in a child with EDS. Discussion: Classic Ehlers-Danlos syndrome (EDS) is an autosomal dominant inherited connective tissue disorder, which is typically characterized by skin hyper elasticity, tissue fragility (manifested by atrophic scarring), and joint hypermobility. Collagen supports the connective tissue (skin, ligaments, tendons, internal organ walls, cartilage, blood vessels) and defects in the collagen subsequently result in weakness leading to the above characteristics. Mutations in COL5A1 and COL5A2 genes that code for collagens, collagen modifying enzymes, and possibly other extracellular matrix components lead to defective collagen V protein.(1) It is estimated that the prevalence of classic EDS is 1 in 20,000. Diagnosis can be challenging since EDS cases can be ambiguous and do not fit in any of the well described subtypes. The classic EDS signs of joint hypermobility, hyper elastic skin, and atrophic scarring are well published. Joint hypermobility can be assessed using the Beighton scale in which a score of 5/9 or greater defines hypermobility. The total score is obtained by the following scale (2) Beighton Scale Characteristic Passive dorsiflexion of the little fingers beyond 90 degrees Passive apposition of the thumbs to the flexor aspect of the forearm Hyper extension of elbow beyond 10 degrees Hyper extension of knee beyond 10 degrees Forward flexion of the trunk with knees fully extended so that the palms of the hand rest flat on the floor Score 1 point for each hand 1 point for each hand 1 point for each elbow 1 point for each knee 1 point

3 Beighton Criteria: Beighton P. Am J Genet 1998; 77: Hypermobile Syndrome (The 1998 Revised Brighton Criteria) Major criteria Beighton score of 4 (either currently or historically) Arthralgia for 3 months in 4 joints Minor criteria Beighton score of 1, 2, or 3 (0 3 if age 50 years or older) Arthralgia (3 months) in 1 3 joints or back pain (3 months), Spondylosis, spondylolisthesis Dislocation/subluxation in 1 joint, or in 1 joint on 1 occasion Soft tissue rheumatism 3 lesions (e.g., epicondylitis, tensosynovitis, bursitis) Marfanoid habitus (tall, slim, span:height ratio 1.03, upper: lower segment 0.89, arachnodactyly) Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring Eye signs: drooping eyelids or myopia or antimongoloid slant

4 Varicose veins or hernia or uterine/rectal prolapse * The diagnosis of benign joint hypermobility syndrome (BJHS) requires the presence of 2 major criteria, or 1 major with 2 minor criteria, or 4 minor criteria, or 2 minor criteria and an unequivocally affected first-degree relative. Clinical Manifestations: System Cardiovascular Hematologic Musculoskeletal Gastrointestinal Neurological Gynecological/Obstetrical Abnormalities Aortic Root Dilatation Easy Bruising Arthralgia, Joint Dislocation, Back & Neck Pain Inguinal/Umbilical Hernia Headache, Autonomic Dysfunction & Chronic Pain Uterine Prolapse, Premature Labor & Post-Partum Bleeding Chronic Daily Headache (CDH) refers to at least 15 headaches days per month, for at least 3 months. True (Primary) Daily Headaches are not caused by the result of another medical condition and tension type headaches are most common type of primary headaches. There are short lasting and long lasting chronic daily headaches. The long lasting chronic daily headaches, last over four hours compared to short lasting chronic headaches. Primary Headache: Migraine Tension Type Headache Cluster Headache New Daily or Other Type of Headaches Secondary Headaches caused by: Head and Neck Trauma Cranial or Vascular Disorder Non Vascular Intracranial Disorder (Neoplasm) Intracranial Infection Psychiatric Disorder Facial Pain secondary to disorder of eyes, neck, cranium, ears, nose, teeth or mouth. Headache is a common chronic pain syndrome in pediatric and adolescent patients. A systematic review of population based studies revealed a prevalence of pediatric headache of 58%. (3) CDH has been reported by school age children and approximately 54% of children have reported a headache for 4 consecutive weeks, 30% for 8 consecutive weeks and 22% for 12 consecutive weeks. (4) The association of EDS with adolescent headache is sparse, however, studies in the adult population have described headache as a characteristic finding in EDS. Reports of EDS also demonstrate correlations with psychiatric disorders, fatigue, dizziness, musculoskeletal pain, and stomach pain. This is important to understand since children with CDH also report musculoskeletal pain, stomachache, fatigue,

5 dizziness/vertigo, and many have clinically significant mental health issues including depression and anxiety. (5) Specific treatment plans should be tailored to the patient. This patient presented with headache reported symptoms that met the criteria for mild classic EDS (hyper extensibility of the skin, joint hypermobility, easy bruising, tendency to bleeding, and motor delay). Headache type in adults with EDS have been reported as migraine with aura, migraine without aura, tension type or a combination of migraine and tension type headache. (6) The actual pathophysiology of headache in EDS is poorly understood. However, cervical spine instability, temporomandibular joint dysfunction, and intracranial hypotension due to spontaneous cerebrospinal fluid leak.have been noted as possible causes of headache in patients with EDS. (7) Headache may also be a manifestation of fatigue (due to poor sleep from chronic pain), poorly controlled or undiagnosed depression/anxiety or from analgesic overuse (used to treat chronic joint pain). In the absence of acute treatable causes for headache, clinicians treating headache patients with EDS need to develop a treatment plan that addresses sleep issues and fatigue, depression/anxiety, and pain management. Pain in EDS has been described as both nociceptive and neuropathic, thus, treatment plans in EDS patients with chronic headache may be tailored to use of anti-epileptics or antidepressants as opposed to nonsteroidal anti-inflammatory drugs (NSAIDs; to avoid analgesic overuse headache). (8) These patients require referral for genetic counseling. A comprehensive clinical evaluation by a geneticist is important since diagnosis is typically based on clinical features. Initial management should include a baseline echocardiogram (aortic diameter measurement for patients <10 years of age) and evaluation of clotting factors in the presence of easy bruising. Medication interventions that affect platelet function and prolong bleeding should be avoided including aspirin, dipyridamole, clopidogrel and NSAIDs such as ibuprofen and diclofenac; however, acetaminophen and celecoxib are considered safe since they do not affect hemostasis. Exercise regimens with little strain on the joint (hydrotherapy) should be initiated to promote muscle development but should exclude contact sports that involve heavy strain on the joints.(9) Medication intervention, maintaining good daily habits as well as biopsychological interventions are often a part of treatment plans for children with CDH. However, despite these interventions, some children continue to complain of CDH. Clinicians should not only be aware of the major manifestations but should also recognize the other characteristics for EDS in order to appropriately diagnose and treat children with both EDS and CDH. Management of Comorbid Disorders with Headaches Secondary to EDS: Cervical disc disorders/radiculopathy POTS Depression/Anxiety Intracranial hypotension Medication Overuse Restless Leg Syndrome PT, epidural steroids, surgery Increase salt intake, support stocking, fludrocortisone, midodrine SSRIs, NSRI Epidural blood patch, Fibrin glue (intrathecal), surgery Withdraw of offending medications Avoid caffeine, nicotine, alcohol, Dopamine agonists (worsen orthostasis)

6 References: 1. Malfait F, Wenstrup RJ, De Paepe A. Clinical and genetic aspects of Ehlers Danlos syndrome, classic type. Genet Med 2010;12: Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ. Ehlers Danlos syndromes: Revised nosology, Villefranche, Ehlers Danlos National Foundation (USA) and Ehlers Danlos Support Group (UK). Am J Med Genet 1998;77: Abu Arafeh I, Razak S, Sivaraman B, Graham C. Prevalence of headache and migraine in children and adolescents: A systematic review of population based studies. Dev Med Child Neurol 2010;52: Nyame YA, Ambrosy AP, Saps M, Adams PN, Dhroove GN, Suresh S. Recurrent headaches in children: An epidemiological survey of two middle schools in inner city Chicago. Pain Pract 2010;10: Nyame YA, Ambrosy AP, Saps M, Adams PN, Dhroove GN, Suresh S. Recurrent headaches in children: An epidemiological survey of two middle schools in inner city Chicago. Pain Pract 2010;10: Jacome DE. Headache in Ehlers Danlos syndrome. Cephalalgia 1999;19: Castori M, Morlino S, Celletti C, Celli M, Morrone A, Colombi M, et al. Management of pain and fatigue in the joint hypermobility syndrome (a.k.a. Ehlers Danlos syndrome, hypermobility type): Principles and proposal for a multidisciplinary approach. Am J Med Genet A 2012;158A: Camerota F, Celletti C, Castori M, Grammatico P, Padua L. Neuropathic pain is a common feature in Ehlers Danlos Syndrome. J Pain Symptom Manage 2010;41: e2-e4 9. Malfait F, De Paepe A. The Ehlers Danlos syndrome. Adv Exp Med Biol 2014;802: Walter SM. Case Report: Ehlers-Danlos syndrome in an adolescent presenting with Chronic Daily Headache. Surg Neurol Int 2014;5:S Joint Hypermobility Syndrome and Headache Disorders: Power point by Vincent T. Martin, MD, Professor; University of Cincinnati College of Medicine