What Treatment is Recommended for Common Headaches?

Patient Presentation
A 16-year-old female came to clinic for her health maintenance examination. She was overall healthy but had a past medical history of migraine without aura since 11 years of age. Her headaches had increased in severity and frequency around the time of puberty and she was started on topiramate. Since then her migraines only occurred approximately once every 2 months. The family history was strongly positive in the maternal side with her mother and maternal aunt both taking prophylactic migraine medication and migraines in her maternal grandmother. The pertinent physical exam showed a thin female in no distress. Her weight was 10%, height 25% and BMI was 18.5 and were appropriate with previous measurements. Her examination was normal.

The diagnosis of a healthy female with migraine without aura was made. She had seen something about a medical study that showed that there was a problem with topiramate for treating migraine. In the office, the pediatrician quickly found the study online and noted that the study didn’t find any benefit from the medication compared to placebo. “But,” he said, “It seems to help you. You have many fewer migraines. You’ve taken the medicine for a long time and it seems like this is consistent over time, plus you aren’t having any side effects.” He went on, “You could go off the medicine and see what happens. If the migraines stay the same, then obviously you may not need the medicine, but if they come back more often then you probably do. Unfortunately you have a big family history of migraine, so my guess is that you are going to need the medicine. Still it’s your choice.” The adolescent decided to continue the topiramate for the time being and was going to think about the options and talk it over with her mother. At a sick appointment 3 months later, the adolescent said that she continued the topiramate.

Headache is pain in the scalp, forehead, orbits and temple. Facial and neck pain are usually excluded from this definition. It is a common problem with more than 6 million pediatric patients having migraine. Headaches can also have co-morbidities and more than 1 primary headache type can co-exist. The costs are high both economically and in the quality of life for the patients and families. A review of common headache types and indications for neuroimaging can be found here.

Treatment is necessarily multi-pronged. Patients should understand their diagnosis so they can understand what reasonably can be expected from treatment, how they can prevent headaches and how they can help to treat themselves once the headache occurs. When patients and families think about treatment for headache they usually think about medications, but the first treatment should be behavior and lifestyle changes. A balanced, consistent lifestyle is recommended for anyone but especially individuals with headache. Discussing the patient’s current daily schedule can help identify problem areas to work on. Often sleep does not receive enough priority. Making a new plan that prioritizes self-care first especially around sleep, eating, and exercise and then filling in the day with school, work and recreational activities should help prevent headaches.

Patients should:

  • Participate in normal school, work and recreational activities as much as possible. Keeping involved also helps to mitigate stress from being behind on work and encourages social support by family, friends and co-workers.
  • Regular, consistent sleep
  • Regular, consistent exercise
  • Regular meal and snack times
  • Drinking adequate fluids (non-caffeinated) – a review of caffeine can be found here
  • Taking care of other health problems. Illness can also often make headaches worse, so illness prevention measures should be used. Chronic health problems should be managed optimally and if medications are prescribed, they should be taken as directed.
  • Avoiding triggers – these may be difficult to identify. Triggers for some people include alcohol, aspartame, caffeine, chocolate, monosodium glutamate, nitrites, or tyramine. Strict elimination diets or restriction of activities is usually not appropriate.
    Reasonableness should reign. For example, most patients can eat some foods with caffeine or chocolate. All patients should carry backpacks of reasonable weight, but those who know neck or shoulder pain triggers their headaches should certainly avoid heavy backpacks or use some type of roller bag.

Detailed symptom diaries can help with the diagnosis and management of headaches.
They can help to characterize the headache, timing, medication efficacy (both acute and prophylactic) or potential overuse. Lifestyle changes can also sometimes become evident.

Red flags that more evaluation and/or neuroimaging may be needed includes:

  • First headache that is severe
  • First headache is markedly different from previous headaches
  • An increasing amount, severity, or symptoms of the headache over time
  • Headache is worse in recumbent positions (e.g. lying down, sleeping or bending over) or with increased intracranial pressure (e.g. valsalva, coughing)
  • Abnormal puberty or growth
  • New onset seizures or change in seizure pattern
  • New neurological signs (e.g. “…ataxia, cranial nerve deficit, head-tilt, papilloedema, visual impairment.”)

Learning Point
Treatment options for headaches:

  • Healthy lifestyle for all patients (see above)
  • Non-medication treatment
    • Compresses – cold or warm
    • Distraction
    • Feverfew for migraine prophylaxis
    • Massage – head, neck or other areas of the body
    • Relaxation techniques
    • Acupressure/acupuncture
    • Cognitive behavioral therapy
  • Medication
    • Mild, intermittent headache or tension headache
      • No treatment
      • Simple analgesics – acetaminophen, ibuprofen
    • Medication overuse headache
      • Withdrawal of the offending medication – patients should be warned that headaches will often worsen for next 1-2 weeks
      • Other lifestyle changes or distraction methods
      • Prophylactic medication sometimes used during this period
    • Migraine with or without aura
      • Acute treatment
        • Triptans with ibuprofen or naproxan (in adolescents) have been shown to be effective.
          • Triptans can be repeated after 2 hours if needed, but no more than 2 doses in 24 hours and no more than 2 days/week to prevent overuse
          • One paper offers, if a triptan is not tolerated because of dizziness, unpleasant feeling or bad taste, to try an alternative triptan or the same triptan but in a different formulation (i.e. nasal, oral, subcutaneous).
            They go on to say, “If tolerated, a triptan should be tried for three different attacks before giving up for lack of efficacy. Three different triptans should be tried before accepting that this class of medicine is ineffective for a particular patient.”

          • Triptans that have been studied in the pediatric population include almotriptan, rizatriptan, sumatriptan and zolmitriptan.
        • Antiemetics may also be necessary
      • Prophylactic treatment – usually for patients with 3-4 episodes/month of headache that are significantly impacting their lifestyle or are incapacitating
        • For women with migraine without aura who have menstrual cycle triggers, suppressive oral contraception may be considered. Use of a triptan (zolomitripan or frovatriptan) 2-3x/day on days migraines are expected can also be helpful.
        • Topiramate has been one of the first line medications used (in lower dosing than for epilepsy) but a study that was stopped early for futility and published in the New England Journal of Medicine in 2017 found that topiramate and amitriptyline had no significant improvement over placebo for pediatric migraine prophylaxis.
          All had high rates of placebo effect (50-60%) consistent with previous studies.

        • Other medications include propanolol, gabapentin, riboflavin and pizotifen

Questions for Further Discussion
1. What are cluster headaches and how are they treated?
2. What are indications for referral to neurology for headache management?

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: Headache and Migraine.

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

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Nappi RE, Kaunitz AM, Bitzer J. Extended regimen combined oral contraception: A review of evolving concepts and acceptance by women and clinicians. Eur J Contracept Reprod Health Care. 2016;21(2):106-15.

Patniyot IR, Gelfand AA. Acute Treatment Therapies for Pediatric Migraine: A Qualitative Systematic Review. Headache. 2016 Jan;56(1):49-70.

Richer L, Billinghurst L, Linsdell MA, Russell K, Vandermeer B, Crumley ET, Durec T, Klassen TP, Hartling L. Drugs for the acute treatment of migraine in children and adolescents. Cochrane Database Syst Rev. 2016 Apr 19;4:CD005220.

Powers SW, Coffey CS, Chamberlin LA, Ecklund DJ, Klingner EA, Yankey JW, Korbee LL, Porter LL, Hershey AD; CHAMP Investigators. Trial of Amitriptyline, Topiramate, and Placebo for Pediatric Migraine. N Engl J Med. 2017 Jan 12;376(2):115-124.

Ng QX, Venkatanarayanan N, Kumar L. A Systematic Review and Meta-Analysis of the Efficacy of Cognitive Behavioral Therapy for the Management of Pediatric Migraine. Headache. 2017 Mar;57(3):349-362.
Whitehouse WP, Agrawal S. Management of children and young people with headache. Arch Dis Child Educ Pract Ed. 2017 Apr;102(2):58-65.

National Center for Complimentary and Integrative Health. Headaches. Available from the Internet at https://nccih.nih.gov/health/pain/headaches.htm (rev. 9/24/17, cited 10/31/17).

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

What Are Cutaneous Lesions Associated with Occult Spinal Dysraphism?

Patient Presentation
A 30-minute old, full-term, female infant was born by normal spontaneous vaginal delivery after an uncomplicated pregnancy and delivery. The family history was notable for heart disease and obesity.

The pertinent physical exam showed a normal appearing female with normal vital signs, weight of 3368 grams (25-50%), length of 51 cm (50%), and head circumference of 37 cm (90%). Ballard examination was consistent with a 39 week female. Her examination was normal except that the intern noted a small sacral dimple within the midline of the gluteal cleft. The base could be seen with mild retraction. It was estimated to be 3 mm deep and in diameter. It was approximately 2 cm from the anus. There were no other cutaneous abnormalities noted.

The diagnosis of of a healthy full-term female with a simple sacral dimple was made. The intern said that she knew that if you could see the bottom of the dimple, then it was okay but wanted to know if there were other abnormalities she should worry about. The attending said that generally if the dimple is small, within the gluteal cleft near the anus and the bottom can be seen and there are no other abnormalities then it should be fine. She said, “If the dimple seems too big, seems far away from the anus or is outside the gluteal cleft then I start to get concerned. Also if it isn’t midline, or has other skin lesions or spine problems, or the baby has other malformations then you need to measure and evaluate the dimple more. It may not be something that is simple and could be a spinal defect.” She went on, “If you aren’t really sure you can always ask someone else to look at the baby too. That is why you are still learning and we all still learn as we continue to practice over the years.”

Neural tube defects are a group of disorders that arise during embryogenesis. They include anenephaly, exencephaly, meningmyelocoeles and encephalocoeles and other malformations including occult spinal dysraphism. Occult spinal dysraphism (OSD) has incomplete fusion of the midline elements of the spine including the bony, neural, and mesenchymal tissues but the abnormalities are covered by skin (ectodermal tissues) and therefore are not obvious. OSD has a higher risk of tethered spinal cord syndrome or other neurological/neurosurgical problems.

Normally the caudal end of the spinal cord, the conus medullaris, hangs freely within the spinal column but is stabilized by the filum terminale. As the spinal column grows faster than the spinal cord, there is relative ascension of the conus medullaris during embryogenesis, reaching the adult level from birth to 2 months of age. The adult level is L2 to L3 for 98% of the population. Tethering occurs when a thickened filum terminale or mass does not allow normal ascension and damage to the spinal cord can occur because of ischemia or the mechanical traction. Tethered cord can produce a number of symptoms including bowel or bladder problems, back or leg pain, numbness or tingling, changes in leg strength or gait, muscle spasms, leg deformities, scoliosis, etc. Developmental regression of milestones previously obtained also can occur in children as a sign of tethered cord syndrome. Treatment is by freeing the conus medullaris. Unfortunately retethering can occur after treatment.

Learning Point
In OSD, the cutaneous lesions are usually midline (~70%) but if there is a laterality it is usually left-sided. Obviously it is possible to have OSD and not have any cutaneous abnormalities or have subtle abnormalities which potentially can lead to delayed diagnosis.

There is an increased risk of having OSD with more than 1 cutaneous finding. Also if anogenital malformations, lower extremity deformities, or problems consistent with tethered cord syndrome are present, OSD should be considered.

Cutaneous signs of potential OSD include:

  • Cutis aplasia
  • Dermal sinus
  • Deviation of the gluteal furrow (DGF)
  • Fibroma penculum
  • Hamartoma
  • Hyperpigmentation
    • Hemangioma
    • Port wine stain
  • Hypertrichosis
  • Subcutaneous lipomas
  • Tails

One paper says that in “.. patients with isolated lesions that are not usually associated with OSD: [port wine stain], hemangioma, hypertrichosis, simple dimple, pigmentary nevus, and mongolian spot[congenital dermal melanocytosis]…. a radiologic investigation does not seem to be appropriate…. Nevus flameus [also] has no pathologic significance.”

Simple dimple is not a sign of OSD. “…[S]imple dimple, or coccygeal pit, as an insolated small dimple (≤ 5 mm in diameter) 2.5 cm or closer to the anus and localized just above the gluteal furrow.” Atypical dimples are potential signs of OSD. “…Atypical dimples seemed to be large (≥ 5 mm), high on the back (≥ 2.5 cm from the anus, and appears in combination with other lesions.” DGF is classically taught that it is not a sign of OSD, but in some studies it can be a sign of lipoma. These studies are small, and larger studies are needed to better define the potential problem.

Test of choice for OSD is magnetic resonance imaging to detect the underlying lesion and also the conus medullaris. Spinal ultrasound can also be appropriate for newborns to age 5-6 months before the spinal bony elements are ossified.

Questions for Further Discussion
1. How is a suspected fetal meningomyelocoele treated during birth and in the immediate neonatal period?
2. Why are latex precautions indicated for patients with meningomyelocoele?

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: Neural Tube Defects and Birth Defects.

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

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Guggisberg D, Hadj-Rabia S, Viney C, Bodemer C, Brunelle F, Zerah M, Pierre-Kahn A, de Prost Y, Hamel-Teillac D.
Skin markers of occult spinal dysraphism in children: a review of 54 cases.
Arch Dermatol. 2004 Sep;140(9):1109-15.

Hertzler DA 2nd, DePowell JJ, Stevenson CB, Mangano FT. Tethered cord syndrome: a review of the literature from embryology to adult presentation.
Neurosurg Focus. 2010 Jul;29(1):E1.

Zerah M, Kulkarni AV. Spinal cord malformations. Handb Clin Neurol. 2013;112:975-91.

Bellet JS. Developmental anomalies of the skin. Semin Perinatol. 2013 Feb;37(1):20-5.

Sewell MJ, Chiu YE, Drolet BA. Neural tube dysraphism: review of cutaneous markers and imaging.
Pediatr Dermatol. 2015 Mar-Apr;32(2):161-70.

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

January 8, 2018

Holiday Break

PediatricEducation.org will be taking a holiday break and wishes our patrons a happy and healthy New Year.

The next case will be published on January 8th. In the meantime, please take a look at the Archives and Curriculum Maps listed on the right side of the page.

We appreciate your patronage,
Donna D’Alessandro and Michael D’Alessandro, curators.

What is DIOS?

Patient Presentation
A 17-year-old male with cystic fibrosis came to the emergency room with acute onset of abdominal pain. The patient had started 2 hours previously in the periumbilical area. It was described as a constant pain and generally didn’t radiate. He had nausea and emesis of food and fluid but it was not bilious or bloody. He denied any bad tastes in his mouth or other reflux symptoms. His last bowel movement was 2 days previously and was described as hard stools. He said he generally was eating and drinking normally.

The past medical history showed that he was diagnosed with cystic fibrosis with his newborn screening and had meconium ileus. He generally took his medications but over the past couple of years “forgot sometimes.” He had a history of constipation and had distal intestinal obstruction syndrome 4 months previously. His appendix was in place. His lung function was monitored closely and he had needed hospitalization for aggressive pulmonary therapy a few times in his lifetime. The family history was non-contributory. The review of systems was negative for fever, chills, and urinary frequency/urgency. He denied recent sexual activity.

The pertinent physical exam showed a thin male in distress from abdominal pain. His respiratory rate was 36 with a saturation of 93%, heart rate was 96, blood pressure was 132/76, and temperature of 99°F. HEENT was normal. Heart was slightly tachycardic without murmur. He was slightly barrel chested with coarse breath sounds. His abdomen was soft with pain in the periumbilical to right lower quadrant area. He had no guarding. There was a mass in the right lower quadrant, but no hepatosplenomegaly. He did not have specific guarding at McBurney’s point, nor peritoneal signs. His genitourinary examination showed no testicular pain or masses and no hernias. Rectal examination revealed no masses and guaiac negative stool.

The work-up included a normal complete blood count, urinalysis, and complete metabolic panel. A surgeon was consulted who felt this was most likely constipation or distal intestinal obstruction syndrome and not appendicitis but imaging was necessary.
The radiologic evaluation on plain film showed dilated loops of bowel and a heavy stool burden. A computed tomographic study of the abdomen showed no appendicitis.

The diagnosis of distal intestinal obstruction syndrome was made. The patient’s clinical course was given intravenous fluids, and non-opiate pain medication. Two hyperosmotic enemas administered by radiology along with laxatives eventually improved his symptoms and he was discharged 3 days later. He was re-educated regarding the importance of taking all of his medication daily and maintaining good hydration.

Case Image

Figure 121 – Supine view of the abdomen (above) reveals multiple dilated loops of distal small bowel and a colon packed with stool. The findings were compatible with a distal small bowel obstruction due to distal intestinal obstruction syndrome. A lateral view of the abdomen (below) taken during a hyperosmolar contrast enema, shows in the center of the image multiple filling defects in the terminal ileum due to it being packed with stool, which is a classic appearance of distal intestinal obstruction syndrome. The enema flushed this stool out of the terminal ileum and the obstruction was relieved.

Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane receptor (CFTR). It is found in the epithelium of the bronchi, intestine, pancreatic duct and biliary tree. It regulates chloride, bicarbonate and water secretion. The heterozygous state helps prevent against secretory diarrhea, but the homozygous state causes thickened secretions in the hollow tubes of the lungs and digestive tract. There are multiple mutations (> 2000) which have been currently classified into classes depending on their protein production and activity. CF patients generally are living longer with improved morbidity because of new treatments including lung transplantation but they also are at higher risk of having gastrointestinal problems over their lifespan. Gastrointestinal problems in patients with CF include:

  • Pancreas
    • Second most frequent organ affected after the lung
    • Concentrated pancreatic juice and thickened secretions can increase obstruction leading to acute or chronic injury and inflammation
      • Pancreatic insufficiency
        • Most (~90%) patients have exocrine pancreatic insufficiency
        • Greasy stools, abdominal bloating, flatulence, diarrhea and poor weight gain are symptoms
        • Treatment is pancreatic enzyme replacement therapy (PERT), proton pump inhibitors are used sometimes also
        • Fat soluble vitamins (A, D, E, and K) can occur and usually are also supplemented
      • Poor nutrition
        • Patients have increased nutrition needs because of the exocrine pancreatic insufficiency, potential vitamin deficiencies, increased caloric needs (additional 20-50%)
        • Patients who cannot consume the necessary nutrition orally are often treated with feeding tubes
      • Acute pancreatitis
        • Severe epigastric pain along with increased serum lipase and amylase levels
        • Affects up to 20% of CF patients
        • Occurs more often in patients with exocrine pancreatic sufficiency than those with insufficiency
      • Chronic pancreatitis
        • Pancreatic atrophy, fatty replacement, calcification and fibrosis on imaging
      • Pancreatic cysts
        • Can be single or multiple, large or small
      • Diabetes mellitis
        • Diffuse scaring causes endocrine pancreatic insufficiency and diabetes
        • Risk increases with age
  • Hepatobiliary tract
    • Hepatobiliary disease complications are some of the most serious after lung complications
      • Fatty liver disease
        • More common in children and adolescents than adults
        • Usually transient
      • Focal biliary cirrhosis
        • Thickened bile and slow flow causes inflammation and fibrosis
        • 11-70% of adults will have it but often it is not clinically discernable
        • Primary treatment is ursodoxycholic acid to increase bile flow. Liver transplantation is also a potential option
      • Large bile duct disease
        • Stasis in large bile duct causes a variety of problems including bile stones, inflammation and strictures
        • Treatment is stone removal and stricture dilatation
      • Cholelithiasis
        • The stones can be cholesterol stones or pigment stones
      • Microgallbladder
        • Found in 30% of CF patients
  • Gastrointestinal lumen
    • Appendicitis
      • The appendix itself can have an increased luminal volume because of increased mucous in asymptomatic CF patients and it is thought that this may be protective against appendicitis
      • Can be confused with Distal Intestinal Obstruction Syndrome (DIOS – see below)
    • Constipation
      • Common in CF patients (40-50%)
      • On radiographs, fecal mass throughout the colon (N.B. note location) without air fluid levels are more common with constipation in comparison to DIOS
    • Fibrosing colonopathy
      • Associated with increased amounts of PERT. It is less common today than previously with changes in PERT dosing
      • Occurs 12-15 months after starting increased amounts of PERT
      • Symptoms similar to colitis, inflammatory bowel disease or DIOS
      • Fibrosis can be in concentric rings (especially ascending colon) but can involve the entire colon
    • Gastroesophageal reflux disease (GERD)
      • Increased risk because of prolonged gastric emptying time, coughing increasing abdominal pressure, lung percussion therapy increasing abdominal pressure and various medications
      • Patients are treated aggressively because even with gastroesophageal reflux patients are at risk of refluxing into the lung and compromising respiratory function
      • GERD can cause bronchiolitis obliterans syndrome
      • In addition to usual GERD treatment, along with positioning for pulmonary toilet and judicious choices of medications, fundoplication may be necessary
    • Ulcer disease
      • Includes peptic and duodenal ulcers
    • Intussception
      • More common in CF patients than the general population
      • Ileocolic intussception is the most common with thickened secretions being the proposed lead point
      • Presents as acute abdominal pain and obstruction and can also resolve spontaneously
      • Can be confused with DIOS
      • Treatment is by enema
    • Gastrointestinal malignancy
      • There is an increased risk of gastrointestinal malignancies overall
      • There is an increased risk for those CF patients who have had a lung transplant
    • Meconium ileus
      • Thickened secretions occlude the gastrointestinal lumen in the terminal ileum (N.B. note location)
      • Affects 10-20% of newborn CF patients. If a full term infant has meconium ileus there is an 80-90% chance they have CF. Premature infants can also have meconium ileus though
      • Treatment is aggressive and can be fatal if untreated. Intravenous hydration, nasogastric decompression, antibiotics and hyperosmolar enemas
      • Classified as simple or complex
        • Simple (50% of patients)
          • Failure to pass meconium by 48 hours without complications
        • Complex (50% of patients)
          • Has one or more complications including intestinal atresia or microcolon, bowel necrosis, perforation, meconium peritonitis, pseudocyst formation, or volvulus
    • Meconium plug
      • Thickened secretions occlude the gastrointestinal lumen in the colon (N.B. note location)
      • Treatment by hyperosmolar enemas
    • Rectal prolapse
      • Occurs in toddlers (1-2.5 years)
      • Used to be a common presentation before universal newborn screening
      • Treatment is usually conservative with treatment of constipation or other ways to decrease abdominal pressure
    • Pneumotosis intestinalis
      • Usually asymptomatic with submucosal or subserosal air that appears linearly in the bowel wall
      • Usually not treated
    • Small intestine bacterial overgrowth
      • Patients can have discomfort, bloating and flatuance
      • The small intestine has decreased motility in many CF patients (but not other areas of the gastrointestinal tract) and therefore the bacterial overgrowth is seen primarily in the small intestine.
        Treatment includes antibiotics. Probiotics are also used sometimes as are prokinetic agents in children.

    Learning Point

  • Distal Intestinal Obstruction Syndrome (DIOS)
      • DIOS has thickened secretions that occlude the gastrointestinal lumen in the ileocecum (N.B. note location)
      • Has also been called meconium ileus equivalent
      • Occurs in any age after newborn period, more common in teens and adults
      • Affects 16-21% of CF patients but up to 50% of those who had meconium ileus as an infant. Risk increases with lung transplant and increased 10x with previous episode of DIOS
      • Patients have acute colicky abdominal pain that is located periumbilically to right lower quadrant with the pain usually being progressive
      • Patients also have bilious emesis or air fluid levels on radiographs
      • Palpation of a mass in the right lower abdominal occurs but this can also be palpated without ileus or obstruction
      • Plain radiographs show dilated loops of bowel with or without air fluid levels with “soap bubbles” in the intraluminal fecal material in the small intestine
      • Differential diagnosis mainly is constipation, intussception and appendicitis
      • Treatment is intravenous or oral hydration, osmotic laxatives, stool softened. Potentially hyperosmotic enemas administered radiographically or surgical intervention may be needed

    Questions for Further Discussion
    1. What are indications for lung transplantation in patients with cystic fibrosis?
    2. How is pulmonary toilet performed for patients with cystic fibrosis?
    3. What are some of the physical examination findings in patients with cystic fibrosis?

    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: Cystic Fibrosis

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

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Demeyer S, De Boeck K, Witters P, Cosaert K.Beyond pancreatic insufficiency and liver disease in cystic fibrosis. Eur J Pediatr. 2016 Jul;175(7):881-94.

    Assis DN, Freedman SD. Gastrointestinal Disorders in Cystic Fibrosis. Clin Chest Med. 2016 Mar;37(1):109-18.

    Kelly T, Buxbaum J. Gastrointestinal Manifestations of Cystic Fibrosis. Dig Dis Sci. 2015 Jul;60(7):1903-13.

    Classification of CFTR Mutations. CFTR.info. Available from the internet at:https://http://www.cftr.info/about-cf/cftr-mutations/the-six-classes-of-cftr-defects/ (rev. 2017, cited 10/17/18)

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