Is This Complex Regional Pain Syndrome?

Patient Presentation
A 13-year-old female came to clinic with a 1 day history of a painful burning sensation in both feet. The previous day she and friends had a dance party and after returning home the feet began to be painful beginning on the right and then on the left. She and her parents noted some reddening and mild swelling. She took ibuprofen with some relief and she was able to sleep. The pain continued in the morning but the skin changes had subsided. She denied any trauma. She was somewhat hesitant to walk but said that she could despite the irritating burning/pain. The past medical history was positive for a broken right foot 4 months previous. After the cast was removed the patient developed pain, swelling and minor vascular changes and was diagnosed with complex regional pain syndrome that responded to physical therapy. The family history was positive for rheumatoid arthritis, Ehler-Danlos, and early myocardial infarction. The review of systems was otherwise negative.

The pertinent physical exam showed a well-appearing female who was cooperative and not in significant distress. Her right foot showed mild dorsal swelling and mottled discoloration of the sole. She complained of burning/pain from her toes to just above her ankle. The left foot had no swelling or skin changes but she complained of burning/pain on the dorsum of the foot. Lower extremities had full range of motion, normal strength and reflexes. There was no bruising or point tenderness. Femoral pulses were normal. She had no other skin changes and the rest of her examination was normal. As the physician was talking, she brought up that this could be trauma but most likely was a recurrence of the diagnosis of complex regional pain syndrome. The patient and mother both agreed that her current problems were exactly like her previous problems. They were confused though why both legs would be affected and the physician noted that this extension to other body parts does occur. The patient was to restart her previous physical therapy routine and to call if symptoms worsened or persisted more than 1-2 weeks. She had crutches at home if she needed them to help with mobility for brief time periods such as the end of the school day. Radiographs of the feet for possible fracture were also negative. At a health supervision visit 1 month later, she reported that the symptoms slowly improved over 2 weeks and that they hadn’t limited her activities very much. Physical examination at that time was normal.

Discussion
Musculoskeletal problems are a significant percentage of primary care visits (~20%), with pain being a common feature. Pain is a normal noxious sensation that is protective, but which has complex neurophysiological underpinnings and may have psychosocial overlays. Chronic musculoskeletal pain in children often begins orthopedic, rheumatologic, and oncologic evaluations. Chronic pain without an obvious biological cause can be frustrating for patients and clinicians alike to evaluate and treat.

Learning Point
Complex regional pain syndrome (CRPS) was previously known as reflex sympathetic dystrophy. It is usually seen in adults, with women more commonly than men experiencing the problem. It is seen in children usually starting in the tween and teenage years. There are often family histories of other chronic pain, neurological or psychiatric problems.

The exact cause is unknown but it is due to a disorder of pain neurophysiology and is not due to specific tissue damage. Although there often is a known precipitating event, such as acute obvious trauma, immobilization, repeated use, etc., the noxious sensations are out of proportion to this acute tissue damage and extend beyond the normal tissue healing time (i.e. the tissue at the time the patient is experiencing the noxious sensations is normal). Classic symptoms include: edema, vascular changes and increased pain in a body part. Other sensations include numbness, tingling, burning, and temperature changes. The body part affected is usually lower extremities but it may affect other areas. Trophic changes can also be seen.

Since it is a diagnosis of exclusion, appropriate evaluation for other possible causes should be made. Patients should be evaluated and treated with a multi-modal approach. Consultants may include orthopaedics, rheumatology, neurology, anesthesia, rehabilitation medicine, physical therapy and psychiatry/psychology. Getting the patient up and moving is critical to the treatment. Immobility makes patients worse. Physical therapy is key and may involve a number of treatments including mobilization, desensitization, hydrotherapy, massage, and other treatments. At the same time, pain control should be focused to allow physical therapy. Pain control can include oral analgesics, psychiatric/neurological medication, electrical stimulation, local or regional blocks, etc. Psychiatric/psychological evaluation and therapy for a primary or co-morbid mental health problem (i.e. conversion disorder, factitious disorder, anxiety, etc.) or a secondary problem in response to chronic pain and concomitant social withdrawal is vital to the overall patient management. Tricyclic antidepressants are often used as an adjunctive therapy. Most patients (80%) have resolution of symptoms by 6 months.

Questions for Further Discussion
1. What are the criteria for diagnosis of fibromyalgia?
2. How is fibromyalgia related to CRPS?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for theses topic: Peripheral Nerve Disorders and Pain

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Dangel T. Chronic pain management in children. Part II: Reflex sympathetic dystrophy. Paediatr Anaesth. 1998;8(2):105-12.

Small E. Chronic musculoskeletal pain in young athletes. Pediatr Clin North Am. 2002 Jun;49(3):655-62, vii.

Littlejohn GO. Reflex sympathetic dystrophy in adolescents: lessons for adults. Arthritis Rheum. 2004 Apr 15;51(2):151-3.

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    6. Information technology to support patient care decisions and patient education is used.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.

  • Systems Based Practice
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What are the Laws Regarding Teenage Contraception?

    Patient Presentation
    A 14-year-old female came to clinic for a health supervision visit. She stated that she recently had become sexually active with a single male partner and they were using condoms consistently. She denied any vaginal or abdominal problems. The past medical history and family history were non-contributory. The social history found that she was a high school freshmen who played in the band and got above average grades. She had a good relationship with her parents but had not disclosed the sexual activity.

    The pertinent physical exam showed a well-appearing female with growth parameters in 10-25%. Her examination was normal including a pelvic examination. The laboratory evaluation for pregnancy and sexually transmitted infections were negative. The diagnosis of a healthy adolescent with recent initiation of sexual intercourse was made. During staffing, the resident said that he didn’t know the state laws regarding contraception for teenagers in the state. The faculty physician reviewed them with him and noted that each state had slightly different laws. The resident physician talked to the patient and discussed the risks of early sexual activity. He also discussed the proper use of condoms and recommending adding a spermicide if no other contraception was used, and he discussed the pros and cons of other contraceptive alternatives including abstinence, Depo-provera® and oral contraceptives. They also discussed what to do for emergency contraception. He also offered to talk with her parents or to be in the room while she told her parents about her sexual activity. The patient’s clinical course 3 months later revealed her returning to initiate Depo-provera contraception after she had told her parents. Her mother said, “I was really surprised and I’m not happy about it and she knows that. We talked about the risks. But I also don’t want her to get pregnant or have an infection. So that’s why we’re here.”

    Discussion
    Teenage pregnancy has decreased in recent years but the United States still has one of the highest rates in the world. Education is imperative so children, teens and adults understand the risks of early initiation of sexual intercourse, contraception, sexually transmitted infection prevention and treatment and adolescent gender issues. Confidentiality for seeking care and treatment is imperative for teens.

    While family relationships, health care providers and programs that promote and support abstinence and later initiation of sexual activity are extremely important, a high number of pre-teens and teens initiate sexual activity in the pre-teen and teen years. Therefore, confidentiality to seek sexual health care is important. “Sixty percent of teens younger than 18 who use a clinic for sexual health services say their parents know they are there. Among those whose parents do not know, 70% would not use the clinic to obtain prescription contraceptives if the law required that their parents be notified.” (See To Learn More Below). Supports to help teens make good decisions about their current and future health are needed.

    Other cases about adolescent sexual health include:
    What Are Some of the Complications of Teenage Pregnancy? and How Can I Make My Sexual Interview More Gender Neutral?.

    Learning Point
    In the United States, some contraceptives are easily available such as male and female condoms and sperimicides, and minors may legally purchase them without a prescription. Other contraceptives require a prescription or are supplied (i.e. Depo-provera injection) by a health care provider. In most states, contraceptive counseling and distribution is confidentially available to minors (i.e. there is no parental notification requirement). Some states do have restrictions which may or may not be enforced.

    Abortion services may be more restricted and are highly variable from state to state in the U.S.. Some states have no parental/family notification, some require one or both parents to be notified, some allow other adult family members other than parents to be notified, and some states allow the notification to be waived by a judge. Planned Parenthood has a listing of parental/family notification requirements, here.

    Questions for Further Discussion
    1. What are the laws regarding confidentiality and treatment for teen sexual health issues in your state or country?
    2. How do you counsel teens regarding all contraceptive choices including abstinence?
    3. What services and programs are available in the local schools and community to support teen sexual health?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Teen Sexual Health and Birth Control.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Maradiegue A. Minor’s Rights Versus Parental Rights: Review of Legal Issues in Adolescent Health Care. MedScape.
    Available from the Internet at http://www.medscape.com/viewarticle/456472 (rev. 2003, cited 12/16/2010).

    Eisenberg ME, Swain C, Bearinger LH, Sieving RE, Resnick MD. Parental notification laws for minors’ access to contraception: what do parents say? Arch Pediatr Adolesc Med. 2005 Feb;159(2):120-5.

    Planned Parenthood. Parental Consent and Notification Laws.
    Available from the Internet at http://www.plannedparenthood.org/health-topics/abortion/parental-consent-notification-laws-25268.htm (rev. 8/3/09, cited 12/16/10).

    Guttmacher Institute. Facts on American Teens’ Sexual and Reproductive Health
    Available from the Internet at http://www.guttmacher.org/pubs/FB-ATSRH.html (rev. 1/2010, cited 12/16/10).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    16. Learning of students and other health care professionals is facilitated.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.
    18. Using effective nonverbal, explanatory, questioning, and writing skills, the healthcare professional uses effective listening skills and elicits and provides information.

  • Professionalism
    20. Respect, compassion, and integrity; a responsiveness to the needs of patients and society that supercedes self-interest; accountability to patients, society, and the profession; and a commitment to excellence and on-going professional development are demonstrated.
    21. A commitment to ethical principles pertaining to provision or withholding of clinical care, confidentiality of patient information, informed consent, and business practices are demonstrated.
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What is the Dose of Light Therapy for Seasonal Affective Disorder?

    Patient Presentation
    A 21-year-old female came to clinic during winter college break to refill her acne medications. She said she was doing well in a college located 3 hours away and noted that in addition to her antidepressant medication she was also using her light box daily since the late fall. The health care provider expressed surprise as he was unaware that she was diagnosed with seasonal affective disorder. The student said that she had been diagnosed at her college and had instituted the light therapy the previous winter. She noted a difference in her mood last winter, and therefore she re-instituted the light therapy this season. She said she used the light box during the early evenings when she was studying. The past medical history revealed major depression and seasonal affective disorder. The family history was positive for depression.

    The pertinent physical exam showed a happy appearing female with mild comedomal acne primarily on her forehead and some on her back. The physician added the diagnosis of seasonal affective disorder to her medical record problem list. He refilled her medications and reiterated that she could call the office if she ever had questions or concerns even though she was away at college. Later he reviewed some guidelines since he wasn’t sure how the light was dosed.

    Discussion
    Seasonal affective disorder was first systematically described in 1984. It can occur at different times of the year but predominantly winter and less commonly summer.
    The DSM IV criteria includes:

    • There is a temporal relationship between the onset of major depressive episodes and a particular time of year.
    • Full remissions (or a change from depression to mania or hypomania) also occur at a characteristic time of year.
    • In the last two years, two major depressive episodes have occurred that demonstrate the temporal seasonal relationships defined in the two criteria above and no nonseasonal major depressive episodes have occurred during the same period.
    • Seasonal major depressive episodes substantially outnumber the nonseasonal major depressive episodes that may have occurred over the individual’s lifetime.

    Patients with seasonal affective disorder usually have typical depressive symptoms including decreased interest or pleasure, psychomotor retardation or agitation, energy loss, feelings of worthlessness, excessive or inappropriate guilt, decreased concentration, indecisiveness, and recurrent thoughts of death. Most patients with seasonal affective disorder also have “atypical” symptoms including hyposomnia, fatigue, increased appetite and weight gain (often for carbohydrates and sugars that is almost uncontrollable).

    Prevalence depends on the population but US rates are around 0.4% and in Canada 1.7-2.9%. The cause is unknown but appears to studies appear to support genetic, neurotransmitter and circadian rhythms as possible causes. Light therapy is effective treatment with response rates from 60-90% in controlled studies.

    Learning Point
    Lux is an illumination unit. A sunny day is 50,000-100,000 lux or more, and a cloudy winter day is about 4,000 lux. A bright office light is about 100 lux, and indoor light is < 500 lux. The standard dose for treatment is 10,000 lux of white, fluorescent light for 30 minutes per day. Light boxes are usually used to delivery the therapy. A 10,000 lux light box is the usual standard light box but each light box is different (including the intensity and wavelengths). All should have an ultraviolet filter to protect eyes and skin. More recent data appears to support wavelengths nearer to the blue spectrum. The light boxes need to be positioned at the proper distance from the patient and used for the proper amount of time. For example, a light box may deliver 2500 lux at a distance of 15-18 inches for 30 minutes. Therefore the patient should have the light box 15-18 inches away and would need to use it for 2 hours to achieve the 10,000 lux dose. Receiving the light therapy in the morning is superior to later in the day, but light therapy other times of day may be helpful to some patients. Light therapy generally will improve symptoms in a few days with studies often evaluating symptom changes at 1-3 weeks.

    Questions for Further Discussion
    1. What study instruments can be used to diagnosed seasonal affective disorder?
    2. What is the differential diagnosis of seasonal affective disorder?
    3. What is the efficacy of using antidepressants and light therapy for seasonal affective disorder?
    4. What are the side effects of light therapy?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Seasonal Affective Disorder

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Lam RW, Levitt AJ, eds. Canadian Consensus Guidelines for the Treatment of Seasonal Affective Disorder. Clinical and Academic Publishing. 1999.

    Westrin A, Lam RW. Seasonal affective disorder: a clinical update. Ann Clin Psychiatry. 2007 Oct-Dec;19(4):239-46.

    Shirani A, St. Louis EK. Illuminating Rationale and Uses for Light Therapy. J Clin Sleep Med. 2009;5:155-163.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    5. Patients and their families are counseled and educated.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What Methods Can Be Used to Reduce Radial Head Subluxation?

    Patient Presentation
    An 11-month-old male came to clinic with a 4 hour history of not bending his right elbow. He was playing with his father on a bed and was near the edge. The father pulled the child back toward the center of the bed by the arm. After that the child refused to crawl, but did not cry or appear distressed. After a nap, his parents noted that he refused to eat or take toys with his right hand, but was otherwise well. The pertinent physical exam showed a normal male in no distress sitting on his mother’s lap. His growth parameters were 10-25%. His right elbow was flexed and pronated. He refused to extend and supinate the elbow. There was no palpable abnormalities, tenderness, swelling or bruising from the shoulder to the finger tips. There was normal range of motion in the shoulder, wrist and fingers. The rest of his examination including neurological and skin were normal.

    The diagnosis of radial head subluxation was made and because there was no history or physical evidence of probable fracture the decision to treat without a radiograph was made. The elbow was reduced by supination of the forearm and flexion of the elbow. A palpable “pop” was felt over the proximal radius. Within a minute the infant was flexing and supinating the elbow on his own, and repeat examination revealed full range of motion in the elbow. The family was counseled that the child may still not use his elbow normally for a couple of hours but he already seemed to be doing better. The family was also told that this could occur again because of the infant’s age.

    Discussion
    Radial head subluxation commonly occurs in infants and toddlers because of the anatomy and child development. The radius is connected to the ulna just distal to the radial head by an annular ligament that encircles the radius “neck” (i.e. radial diaphysis) and inserts into the ulnar tuberosity. However the annular ligament is relatively small and also not as fibrous in young children compared to older children and adults. Young children often have their arm extended upward to hold hands with an adult especially when they are new walkers. If the child stumbles or for some other reason has the adult place traction (i.e. pull the extended arm) on the arm, the radial head can be subluxed distally and become entrapped in the ligament. This gives rise to the common name of nursemaid’s elbow. The child may cry in pain or not, but refuses to use the arm, especially the elbow, properly. The adult also may be unaware of what has happened since the incident doesn’t appear to have any trauma associated to it.

    Learning Point
    There are basically two methods for radial head reduction:

    • Elbow supination and flexion

      • With the elbow in ~90 degrees of flexion, support the elbow while placing pressure on the radial head.
      • Grasp the forearm/wrist and supinate the forearm fully.
      • Flex the elbow completely.
      • Usually the “pop” of the reduced radial head can be appreciated during elbow flexion.
    • Hyperpronation
      • With the elbow in ~90 flexion, support the elbow.
      • Grasp the forearm/wrist and firmly hyperpronate the elbow.
      • Usually the “pop” of the reduced radial head can be appreciated during hyperpronation.

    Illustrations and videos of the procedures can be found here.

    The hyperpronation technique in some studies has a higher rate of success and may be less painful. One technique may be tried and if it appears unsuccessful the other technique can also be tried. Audible or palpable clicks are often appreciated with proper repositioning, but not always.

    In general the shorter amount of time the radial head is subluxed the better, as there is little time for edema to occur. Most children will move the elbow within 30 minutes post reductin. Subluxation that has been present for several to many hours can cause edema, and when the radial head is repositioned, may cause the patient to still have pain and refuse to use the elbow. Children who do not move the arm after an appropriate amount of time may not have the radial head properly repositioned, or the radial head may be entrapped in a partial tear of the annular ligament. Other diagnoses are also possible such as fracture. If a couple of attempts are unsuccessful, then positioning the child in a posterior splint and referral to orthopaedics is indicated.

    Radiographs may be considered prior to reduction especially if the history is consistent with greater force (i.e. fell from a height, hit by an object, etc.), history is not consistent with the diagnosis, or there is physical evidence of possible fracture such as swelling, tenderness, bruising or deformity of the elbow or contiguous structures. Positioning the elbow for radiographs may cause a radial head subluxation to be reduced. Therefore a child may return from the radiology suite moving the elbow.

    Questions for Further Discussion
    1. How often does radial head subluxation recur?
    2. What is the treatment for recurrent radial head subluxation?
    3. What radiographic findings may indicate an elbow fracture?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Elbow Injuries and Disorders.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Macias C, Bothner J, Wiebe R. A Comparison of Supination/Flexion to Hyperpronation in the Reduction of Radial Head Subluxations. Pediatrics. 1998;102:e10.

    Kaplan RE, Lillis KA, Recurrent Nursemaid”s Elbow (Annular Ligament Displacement) Treatment Via Telephone. Pediatrics 2002;110;171-174.

    Lamb RP. Joint Reduction, Radial Head Subluxation. eMedicine.
    Available from the Internet at http://emedicine.medscape.com/article/104158-overview (rev. 11/192009, cited 11/29/2010).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital