PDMR0498

Emergency Department Management of Children with Cerebral Palsy

    Author: Melanie Swiersz Heniff, MD, Resident in Indiana University-Methodist Combined Emergency and Pediatrics Residency, Indianapolis, IN.

    Peer Reviewers: Kate Cronen, MD, Chief, Division of Emergency Medicine, A.I. duPont Hospital for Children, Wilmington, DE; Steven Bachrach, MD, Chief, Division of General Pediatrics, duPont Hospital for Children, Wilmington, DE; Clinical Professor of Pediatrics, Jefferson Medical College, Philadelphia, PA.

Children with cerebral palsy (CP) present a daily challenge to the emergency department (ED) physician. The increased prevalence of CP over the last 20 years and advances in technology have created a population of children with very complicated health care needs. In addition, multiple medical and surgical therapies have enhanced the patient's life but also created new complications that the ED physician may be asked to navigate.

This article reviews the disabilities of children with CP, associated complications, and suggested strategies for management in the ED. In addition, commonly associated side effects of currently used treatment regimens are reviewed.

-The Editor

Introduction

Cerebral palsy (CP) is a nonprogressive central motor deficit with multiple causes and manifestations. Currently, CP is the single most widespread permanent physical disability in the United States.1 Due to the prevalence of CP, as well as the coexistence of multiple medical problems present in patients with CP, the emergency physician is likely to encounter these patients on a regular basis. The prevalence of CP has increased 15-20% over the past 20 years and is thought to be coincident with the increased survival of very low birth weight infants.2 CP patients may have multiple medical problems including, but not limited to, motor deficits, seizures, recurrent aspiration, restrictive pulmonary disease, and nutritional problems. Patients with CP require a significantly higher use of health services than non-affected children. For example, in a survey of health services, children with CP had five times as many physician visits and seven times the number of episodes of hospitalization as children without CP.3

CP Defined

CP is not a single disease but actually a symptom complex with multiple causes and a variety of manifestations. Abnormal motion, posture, and difficulty in neuromuscular control are primary manifestations of CP. This condition results from a CNS injury early in brain development; evidence of this disorder usually presents in the first 3-5 years of life. Since the neurologic insult is central, motor involvement is commonly seen along with other CNS problems such as abnormalities of cognition and communication. Although the brain lesion responsible for CP is by definition nonprogressive, the evident neuromuscular impairment often worsens over time.4

Epidemiology

The prevalence of CP is 2.5 per 1000 live births.5 There has been minimal change in prevalence over the last 40 years despite drastic changes in obstetric and perinatal care (such as the use of ultrasound, fetal monitoring, etc.). The rate of CP per 1000 live births is markedly increased in very low-birth weight babies, increasing with each drop in birth weight, to an incidence of 10-11% in infants less than 1000 g.6

Risk Factors/Etiology

The brain injury responsible for CP may occur prenatally, perinatally, or postnatally.7 Althourgh may risk factors have been identified, an etiology may not be evident in half of the patients with CP.8 Despite the presence of risk factors for the development of cerebral palsy, most children born with risk factors do not develop CP. Finally, Even though prematurity is the mostcommon preceding history, most infants who develop CP were born at term. (See Table 1.)

Risk Factors Before Pregnancy

An unusually long or short interval between pregnancies and history of miscarriage or stillbirth are factors that have been identified as placing a woman at increased risk of having a child who develops CP.

Risk Factors During Pregnancy

The presence of growth retardation, congenital malformations, birth weight less than 2000 g, and twin gestation (risk is related to intrauterine growth retardation) are associated with increased risk. In addition, pregnant women who took estrogens or thyroid hormones or had preeclampsia have a higher incidence of children with CP.

Risk Factors During the Perinatal Period

An increased risk of CP occurs with the presence of chorionitis, asphyxia, non-vertex presentation, and congenital infections such as rubella, CMV, and toxoplasmosis.

Risk Factors During the Postnatal Period

Neonates who develop neonatal seizures, intracranial hemorrhage, acidosis, sepsis, or kernicterus are at increased risk of developing CP. Kernicterus, or encephalopathy due to bilirubin toxicity, is now rare due to effective treatment of Rh disease.

Older children may develop CP following bacterial meningitis, viral encephalitis, or traumatic brain injury.

Pathogenesis

The lack of consistently recognizable areas of brain injury or abnormality supports the theory that CP is due to abnormal brain development at the microscopic level, and, less likely, secondary to injury to a normal brain. Preterm infants who develop intraventricular hemorrhage with extension of hemorrhage into cerebral white matter are at greatest risk for developing CP.5 Periventricular white matter has long-tract pyramidal fibers that go to spinal cord segments that supply neuromotor control of lower extremities. Intraventricular hemorrhage can lead to `periventricular leukomalacia" (PVL), or areas of necrosis in the white matter near the lateral ventricles. PVL is strongly predictive of CP with resultant spastic diplegia. Patients with spastic diplegia have more significant involvement of the lower extremities than the upper extremities.

Clinical Approach to Diagnosis

In general, emergency medicine physicians will be concerned with management of medical complications that occur in patients already given the diagnosis of CP. Nevertheless, important elements pertaining to the diagnostic approach of CP should be reviewed. This disorder is difficult to diagnose before age 6 months. In 70% of cases, the diagnosis of CP is made by a child's first year.4 Presentations in early infancy include a variation in tone, delayed achievement of motor milestones, asymmetric use of extremities, and hyperreflexia. (See Table 2.) While no clinical findings are absolute predictors of the development of CP a number of red flags can be noted.

CP is a diagnosis of exclusion since there is no specific diagnostic test to differentiate CP from other disorders. The differential diagnosis includes neurodegenerative disorders, spinal cord lesions, neuromuscular disorders, inborn errors of metabolism, and mental retardation. Approximately 20-30 % of children that manifest signs suggestive of CP in infancy seem to "grow out" of it by their second year.5 Reasons for this are unknown.

Management of Associated Disabilities/Medical Problems

General Approach. In general, the ED practitioner evaluating the CP patient must consider a number of unique issues. When a physician performs the initial history and exam, he/she must coordinate and manage many associated medical complications. A patient with CP may have a wide variety of orthopedic, neurologic, gastrointestinal, genitourinary, and pulmonary problems. Parents may not report these as individual medical problems, especially if they are under control. If possible, within the time contstraints of one's emergency practice, it may be helpful to list all medical problems and to establish whether each problem is stable or unstable. Focus on the acute problem while taking into consideration coexisting medical issues. Inquire about the use of current therapy, changes in therapy, and escalation of care. This helps the ED physician determine the need for inpatient vs. outpatient care. For example, the reported increased frequency of use of albuterol nebulizations or chest physiotherapy (PT) at home may suggest the necessity for admission. Be sure to discuss any home health care resources the family may use and their impression of the problem.

Use a Multidisciplinary Team Approach. CP patients are often followed by a number of different specialists. A primary care physician, neurologist, gastroenterologist, orthopedist, physiatrist, dentist, physical therapist, and others are often involved in a CP patient's care. When appropriate, consultants should be involved as early as possible for problems related to their area of expertise.

Identify the Baseline Level of Function. Quite often, the baseline level of function for children with CP will not be known or obvious. The patient's "normal" health status must often be defined by the patient's caregivers. Also involve home health care nurses or resources. A parent's concern that the patient "seems uncomfortable" or "isn't acting like himself" should alert the physician to the possibility of an occult potentially serious medical condition.

Consider Communication Issues. While communication may be limited, one should be cognizant the the CP patient may have normal cognitive function.9 Comments made to the patient and caregivers must always be sensitive and appropriate. Additionally, one should not assume that a patient with CP who has difficulty articulating is cognitively impaired.

Be Familiar with Commonly Used Medications and Their Side Effects. CP patients are often on multiple medications including drugs to control seizures, reflux, and to help manage problems such as spasticity and drooling. (See Table 3.)

Management of Associated Disabilities/Medical Problems

Musculoskeletal Problems. Many orthopedic problems in children with CP are due to the effect of abnormally high muscle tone on growing bones. The primary mechanism involved in this is an initial brain lesion causing loss of supraspinal inhibition and thus abnormally increased reflex activity followed by spasticity and muscle contracture.10 Spasticity prevents the normal growth of muscles. Contractures limit the use of a muscle and bring it into a shortened position. This results in a loss of protein synthesis, loss of sarcomeres, and decreased extensibility. CP is nonprogressive, but the effects of muscle imbalance are progressive and cause growth dysfunction. Muscle contractures most often occur in the gastrocnemius, soleus, hamstrings, and hip flexors and extensors.

Hip Subluxation/Dislocation. The risk of hip subluxation/ dislocation is increased by spasticity of muscles surrounding hips and by delayed ambulation.11 The subluxation is caused by muscle imbalance; flexors and adductors of hips overpowering extensors and abductors because of spasticity or contracture. In growing children with CP, acetabular dysplasia and hip subluxation may occur over time. This subluxation may or may not be painful. Subluxation may present as a change in posture or difficulty sitting.

Osteopenia/Fractures. Osteopenia and/or fractures may develop secondary to decreased activity and nutritional deficiency.12 In older children with CP, there is an increased risk of fracture with seemingly insignificant trauma (such as moving the child from bed to a wheelchair). Consider the possibility of a fracture in an irritable CP patient who may not be able to communicate exactly where he or she has pain.

Management of Chronic Orthopedic Problems

Spasticity is a chronic problem that does not typically present with acute exacerbations. Physical therapy is done to maintain range of motion and to focus on posture, ambulation, and use of adaptive devices. Occupational therapy involvement focuses on the approach to activities of daily living. Anti-spasticity medications are often used; however, they are relatively ineffective in severe spasticity. Nevertheless, the emergency physician should be aware of the common uses and side effects of these medications. Oral administration of these drugs is relatively ineffective in reducing severe spasticity, partly due to a limited ability to penetrate the blood-brain barrier.10 Diazepam (Valium) is a centrally acting muscle relaxant that may be useful for treating dyskinetic CP.5 This drug increases pre-synaptic inhibition, probably by increasing the affinity of gamma-aminobutyric acid (GABA) for receptors on neuronal membranes. Side effects of diazepam include sedation, weakness, confusion, urinary retention, constipation and physical dependency. Lioresal (Baclofen) acts presynaptically and inhibits the release of excitatory neurotransmitters. This agent is more effective for spasticity of spinal origin but may be used as adjunctive therapy for CP with severe spasticity. Baclofen can be used intrathecally via a pump implanted in the abdominal wall. Side effects of Baclofen will include sedation that tends to disappear a few days after the first dose, weakness, confusion, constipation, hypotension, and ataxia.13 Side effects of abrupt withdrawal of Baclofen, if the pump malfunctions, will include a sudden and acute increase in muscle tone. Seizures and hallucinations are also possible. One should call the physician responsible for inserting/managing the pump. An overdose of Baclofen via the pump can result in respiratory depression. Dantrium (Dantrolene) inhibits the release of calcium from the sarcoplasmic reticulum resulting in decreased force of muscle contraction followed by muscle weakness. This drug is primarily useful in children with CP who are difficult to care for due to prolonged muscle contraction but who would not be severely affected by decreased strength of voluntary muscle use. Side effects include a risk of hepatotoxicity, constipation, weakness, and drowsiness.14 There has been recent increased interest in intramuscular botulinum toxin A for temporary muscle relaxation. This agent has a localized effect (no systemic toxicity) that lasts 2-6 months.15 Use of this toxin can be a problem if it is injected into a weight-bearing muscle, resulting in decreased sitting or walking ability.

Bracing/Casting. Bracing and, sometimes, temporary casting are done to delay the development of contractures, to enhance function, and to decrease involuntary movements and excessive energy consumption. The most common example of this is an ankle foot orthosis (AFO) that is used to stabilize lower extremity movement.16 A hip abduction orthosis brings the head of the femur and the acetabulum into alignment, therefore, helping to prevent hip dislocation/subluxation. Because of growth and wear, patients usually need new braces yearly. Pressure sores or skin breakdown underneath these braces should be considered as a possible cause of fever or irritability in a patient with CP.

Surgery. For CP patients, orthopedic surgery is done to preserve function, assist in ambulation, prevent or treat contractures, or to decrease pain. An example of this is lengthening or release of a contracted or tight heel cord, or of spastic hip and knee flexors. Another operation, a selective posterior rhizotomy (SPR), involves an L2-L5 laminectomy to localize posterior roots of the cauda equina. Some of the posterior roots from L1 to S1 are severed depending on the degree and pattern of abnormal response to intraoperative electrical stimulation. As a result, spasticity is reduced by decreasing stimuli from afferent fibers in dorsal roots to muscles in the lower extremities.4,17 This results in decreased tone while preserving sensation. Unfortunately, this procedure can lead to bladder dysfunction. A UTI should be strongly considered when these patients present with a fever without an obvious source.

ED Approach to Musculoskeletal Problems

The Painful Extremity. A CP patient may present with irritability and tachycardia due to musculoskeletal pain that may be very difficult to localize. The patient should be undressed and a thorough exam of skin and joints should be performed. Hip subluxation may or may not be painful and could present as a change in posture or inability to properly position the patient in a wheelchair. Fractures may present as localized tenderness, swelling, or difficulty with weight-bearing. Due to osteopenia, fractures may occur without a history of significant trauma. Skin should be examined for pressure sores, particularly in non-ambulatory patients or those who use orthoses. Always consider the possibility of a septic joint. On the other hand, what appears to be a painful right hip in a child with CP may be a deceptive presentation of appendicitis.

Seizures. A significant portion of children (35-50%) with CP develop seizures, usually by age 2.4,5,18 The highest incidence of seizures is in patients with spastic hemiparesis and quadriparesis. All types of seizures can be seen in patients with CP. The most common types of seizures in these patients are tonic-clonic and complex partial.4,19 Complex partial seizures are commonly preceded by an aura that may be a sense of fear or unpleasant feeling. Another hallmark of complex partial seizures is the presence of automatisms or repetitive, stereotypical movement or behavior, such as chewing, swallowing, or pulling at clothes. Complex partial seizures usually last 1-2 minutes and consciousness is impaired. Atypical psychomotor seizures may be difficult to recognize.20,21 Psychosensory symptoms like illusions or hallucinations can occur and should be regarded as possible seizures. Episodes of sudden immobility or unusual behavior patterns may indicate the presence of seizures.

Seizure Management in the ED

Seizures in a CP patient with a known history of seizures may be due to inadequate medication levels because of drug interactions or noncompliance with a drug regimen. Families should be questioned regarding the frequency and type of seizures the child has and the management program. Drug levels should be checked and an appropriate dose of antiepileptic should be given.The weight of the patient with CP should be carefully checked since it is often below average for age. Each 1 mg/kg of phenytoin or phenobarbital will increase the serum level by approximately 1 mcg/mL.

Management of Status Epilepticus

Status epilepticus in patients with CP should be approached as in any other patient. First priority should be given to management of airway, breathing, and circulation. A brief initial history should include questions about current medications, history of seizures, and recent or current illness. Exam should include evaluation for signs of airway or circulatory compromise, evidence of trauma or infection, and assessment for presence of a focal seizure.

Lorazepam (0.1 mg/kg IV, with a maximum dose of 4 mg) is a good first-line choice for treatment of status epilepticus. If IV access cannot be obtained immediately, diazepam can be given rectally at a dose of 0.5 mg/kg, maximum of 10 mg, of the IV solution.

Whenever diazepam or lorazepam is used, the patient should be carefully monitored for respiratory depression. Following administration of a rapidly acting agent to terminate the seizure activity, the patient must then receive a bolus of a medication he is on (subtherapeutic level) or be loaded with a longer acting agent. The most commonly used acute agents include phenytoin or phenobarbital.

Patients with CP may have multiple types of seizures and, therefore, may be on many different medications for seizure control. Be aware of the many different drugs used for seizure control, side effects, and drug interactions. (See Table 4.) Medication selection for long-term seizure management is done with consideration given to type of seizure as well as effects on the patients' behavior and alertness.

Phenobarbital is a barbiturate that can be used for treatment of generalized tonic-clonic seizures and partial seizures. Side effects include hyperactivity, irritability, Stevens-Johnson syndrome, decreased level of cognitive function, and drowsiness.22,23,24 Phenytoin (Dilantin) is effective for treatment of partial and secondarily generalized tonic-clonic seizures. Problems that can occur with phenytoin include ataxia (especially with levels over 20), drowsiness, diplopia, hirsutism, Stevens-Johnson syndrome, gingival hyperplasia, rickets, osteomalacia, and nystagmus.22,23,24 Ataxia may be difficult to assess if the patient is confined to a wheelchair; however, truncal ataxia may be noted. Phenytoin levels may be affected by a concomitant use of a variety of other medications. Serum levels of phenytoin may be decreased by concurrent use of carbamazepine and sucralfate. Phenobarbital, sodium valproate, and valproic acid may result in either increased or decreased levels of phenytoin. Drugs that may increase levels of phenytoin include diazepam, H2-antagonists, phenothiazines, and salicylates.25 Carbamazepine (Tegretol) is effective for control of partial and secondarily generalized tonic clonic seizures. Adverse effects include dizziness, drowsiness, ataxia, nausea/vomiting, anemia, and mild leukopenia. Levels of carbamazepine may be decreased by rifampin, phenobarbital, phenytoin, and theophylline. Erythromycin, clarithromycin, cimetidine, propoxyphene, ketoconazole, and itraconazole may inhibit metabolism of carbamazepine, resulting in increased carbamazepine levels and neurotoxicity.25 The ability of a patient with CP to function may be significantly reduced by even slight neurotoxicity from phenytoin or carbamazepine. 23

Valproate (Depakote, Depakene) is used for absence, generalized tonic-clonic, myoclonic, and atonic seizures.22 Potential problems include drowsiness, nausea/vomiting, tremor, and pancreatitis. Fatal liver failure has occurred in children younger than 2 years old. Additionally, valproate may displace phenytoin and phenobarbital from protein-binding sites. This may result in toxic serum levels of phenytoin and phenobarbital.5 Valproate and phenobarbital administered together can cause CNS depression even without significant elevations of serum levels of either drug.25 Clonazepam (Klonopin) is a benzodiazepine used for treatment of myoclonic, atonic, and absence seizures. Side effects include drooling, ataxia, drowsiness, and behavior problems.22,24

Three new anti-epileptic medications, felbamate, lamotrigene, and gabapentin have been released since 1994. Felbamate (Felbatol) is used for refractory generalized or focal seizures, although its use has fallen out of favor due to the occurrence of aplastic anemia and hepatic toxicity in multiple patients using this drug.22,26 Side effects include insomnia, weight loss, anorexia, nausea/vomiting, diplopia, and headache. Significant interactions with other anti-epileptic medications are possible. Felbamate taken concurrently with carbamazepine causes an increased level of a metabolite of carbamazepine followed by clinical toxicity. Valproate and phenytoin levels are increased by concurrent use of felbamate.26

Gabapentin (Neurontin) is used in patients 12 years and older for adjunctive treatment of complex partial seizures. Gabapentin does not affect the metabolism of other anti-epileptic drugs.22 Adverse effects include drowsiness, ataxia, nystagmus, and dizziness.

Lamotrigine (Lamictal) is approved for use in adults as adjunctive therapy for uncontrolled partial seizures. Common side effects include ataxia, somnolence, dizziness, headache, nausea, vomiting, and rash.22

Pulmonary Problems

In patients with CP, respiratory problems can frequently occur due to oropharyngeal incoordination and reflux, which can cause choking, gagging and aspiration.21 Aspiration can cause acute pneumonia as well as chronic lung fibrosis and decreased lung compliance.27,28 Chronic congestion, wheezing, and recurrent pneumonias are common. Wheezing is often due to underlying lung disease, especially in a young patient with CP with a history of prematurity and bronchopulmonary dysplasia (BPD). Aspiration of even small amounts of food, saliva, or gastric secretions can cause wheezing. In addition to problems with aspiration, multiple other reasons for pulmonary impairment exist in patient with CP. The cough reflex may be decreased or absent. Restrictive lung disease with decreased vital capacity is prevalent.1,21 Severe scoliosis may further aggravate restrictive lung disease and may contribute to worsening reflux.

ED Management of Pulmonary Problems

Airway. A maneuver as simple as proper positioning of a patient is important in a patient with CP and weakness or poor head control. Appropriate positioning may help to open the airway and decrease pooling of secretions in the posterior pharynx. Aggressive use of suctioning and chest physiotherapy is necessary. When intubation is needed for a patient with CP, remember the possibility that sensitivity to succinycholine may exist, resulting in prolonged duration of the effects of this drug.29 In patients on anticonvulsants, the presumed mechanism for this sensitivity is up-regulation of acetylcholine receptors.

Reactive Airways Disease and Pneumonia. Liberal use of bronchdilators and steroids should be provided to patients with CP and reactive airway disease. Due to the many underlying pulmonary problems in patients with CP, what appears to be a minor respiratory problem can quickly result in severe respiratory distress. Physicians should have a low threshold for consideration of admission of a patient with CP presenting with respiratory problems, particularly if a pneumonia is present. Pneumonia can be devastating in this patient population, especially in the presence of coexisting reasons for respiratory impairment. Pneumonia is a major cause of decreased life expectancy in CP patients.

Gastrointestinal Problems. Most GI problems in CP patients result from motor problems including hypotonia, weak sucking ability, poor swallowing coordination, hyperactive gag reflex, tonic bite reflex (causes the patient to bite down without being able to let go), and exaggerated tongue thrust.21,27 Pseudobulbar palsy, involving paresis of facial, lingual, and pharyngeal muscles may be present and may complicate processes of speech, feeding, and control of secretions.1 Failure to thrive can occur and may present in infancy.14 This is due to feeding problems and increased caloric needs. Energy expenditure for physical activity may be several times normal.5 Often CP patients have underlying malnutrition and resultant growth retardation. Malnutrition can result in weakness and behavioral apathy.28 Children with CP may be unable to feed themselves due to motor impairment and communication difficulties may prevent them from expressing hunger or thirst.30,31

Superior Mesenteric Artery Syndrome. Superior Mesenteric Artery syndrome (SMA) may occur in patients with CP and is due to intermittent or functional obstruction of the duodenum. It is named because one proposed etiology is compression of the third part of the duodenum between the superior mesenteric artery and the aorta.32 This problem is most likely due to loss of supporting fat to the second and third parts of the duodenum along with the presence of the normal or an exaggerated (such as with scoliosis) lordosis occluding the duodenum.34 Patients usually present with recurrent vomiting ,which may be bilious. SMA syndrome is most likely to occur after spinal surgery for scoliosis or after recent, rapid weight loss.33,34 Diagnosis of SMA syndrome can be done by a barium swallow, which would show megaduodenum with rapid back and forth peristaltic movements. Management includes improved nutrition (usually via hyperalimentation along with enteral, often jejunal, feeds), with the goal of weight gain to allow periduodenal fat to accumulate. In addition, patients are often positioned prone after meals. This lets the duodenum fall away from the retroperitoneal structures that may be causing obstruction. Metoclopramide (Reglan) or Cisapride (Propulsid) may be used to facilitate peristalsis. Surgery may be needed despite the above efforts, though this is rarely required.

Excessive Drooling. This is mainly a cosmetic problem that sometimes resolves spontaneously by age 5 to 10 years.5 More serious problems associated with drooling are maceration of the skin and decreased intake of fluids and nutrition.35 Drooling is due to poor oral and pharyngeal motor coordination resulting in pooling of secretions. Patients may be on an anti-cholinergic medication, such as glycopyrolate, to decrease secretions. Be aware of the atropinic effects of anti-cholinergic medications used for drooling, including urinary retention, blurry vision, flushing, constipation, and, in severe cases, pseudo-obstructive signs and symptoms. Additionally, unilateral mydriasis or anisocoria can occur when a patient inadvertently transfers scopolamine from the patch to the eye.36 Transdermal scopolamine patches used to manage drooling have been associated with toxic psychosis.37,38 In severe cases, surgery, such as transposition of the parotid and/or submandibular gland, may be required.39 A complication of surgery that may occur is xerostomia, or thick secretions that impair the ability to swallow food.5,40

ED Management of GI Problems

For all of the previously mentioned reasons, children with CP may develop rapid dehydration with what initially appears to be a mild gastroenteritis. Dehydration may result from decreased intake alone, without a history of nausea and vomiting. Patients with CP should be carefully evaluated and the ED physician should have a lower threshold for admission if the child has ongoing vomiting and diarrhea. In a chronically malnourished patient, assessment of dehydration may be difficult. Lab values may be more indicative of dehydration than physical exam.

Gastrointestinal reflux and esophagitis are common in patients with CP. These problems may present as discomfort/pain, arching, recurrent pneumonias, and, sometimes, failure to thrive. Reflux can cause loss of nutrients from vomiting. Patients with CP are often on Cisapride (Propulsid) to enhance GI motility. It is important to remember significant drug interactions that are possible with cisapride. Cisapride is metabolized by the hepatic P450 cytochrome. Drugs that inhibit this system result in increased cisapride levels that have been associated with the occurrence of ventricular tachycardia, ventricular fibrillation, torsades, and QT prolongation.25 Inhibitors of the P450 cytochrome include ketoconazole, itraconazole, miconazole, fluconazole, erythromycin, and clarithromycin; these medications are contraindicated in a patient taking cisapride.

ED Management of Gastrostomy Tube Problems

Patients severely affected by CP often require gastrostomy tubes for feeding and medications. Irritability or feeding intolerance may result from gastrostomy tube malfunctions.41 Additionally, the abdominal wall site should be checked for evidence of erosions or infection. Water-soluble contrast and x-rays should be used to check for fractured catheters, intraperitoneal leak, misplacement, or obstruction.

Replacement of a Dislodged Gastrostomy Tube. A dislodged gastrostomy tube may be reinserted into the gastrocutaneous tract as long as adhesions are formed between the gastric and abdominal walls (7 days to 3 months after initial insertion).42 Application of lubricating gel to the tract opening and distal tube may facilitate reinsertion of the tube. If a regular gastrostomy tube is not available, a foley catheter may be temporarily inserted to keep the tract patent and to permit continued feedings. Correct tube placement can be determined by easy passage, borborygmi with 20 mL of air insufflation, and rapid return of stomach juices with aspiration.43 The Foley balloon should then be inflated with saline.

Management of a Clogged Gastrostomy Tube. A frequent cause of gastrostomy tube blockage, kinking of the tube, most commonly occurs shortly after reinsertion of a tube. Simply withdrawing the tube a few centimeters should remove the kink. Irrigation of the tube with carbonated beverages is a simple but often ineffective technique for unclogging a gastrostomy tube. Enzymatic preparations can soften the consistency of materials (usually feedings and/or medications) clogging the tube. Two papain tablets or a crushed chymotrypsin tablet dissolved in 2 mL of distilled water may be irrigated into the tube. The tube should then be clamped for 1-4 hours.43

Constipation

Severe constipation is common due to increased tone and impaired anal sphincter relaxation. This can be worsened by medications used for spasticity or drooling and inadequate intake of fluid and fiber. Constipation can result in chronic abdominal pain, decreased absorption of medications, decreased appetite due to discomfort, and worsened gastroesophageal reflux.21,28 Rectal tears can occur and should be considered as a possible cause of pain/irritability in a CP patient. Avoid the routine use of mineral oil for constipation in CP patients with dysphagia or vomiting because of the risk of aspiration pneumonia.44 Hypertonic phosphate enemas should not be used in children since they can result in electrolyte abnormalities such as elevated phosphorus, hypocalcemia, hypernatremia, hypokalemia, and dehydration.44 Disimpaction can be attempted with a hyperosmolar milk and molasses enema (1:1 milk & molasses).

Bladder Problems

Children with CP have an increased incidence of bladder problems.45,46 The presence of a neurogenic bladder in patients with CP is due to an upper motor neuron lesion.10 Voiding dysfunction may present with incontinence, urgency, and frequency. Dysfunctional voiding and constipation, both common in patients with CP, are associated with recurrent urinary tract infections.46,47 These difficulties are worsened by hygiene problems (contractures make it difficult to clean the perineum). Urinary tract infections should always be considered as a possible source for a fever in patients with CP. Urinary retention should be considered as a possible cause of abdominal pain or irritability.

HEENT Problems

Chronic otitis media is a common problem in patients with CP and is associated with eustacian tube dysfunction.14 Children with CP are at high risk for dental disease for a number of reasons. Drooling may result in decreased clearance of retained food. Poor oral hygiene is common. Bruxism and reflux may contribute to erosion of teeth.21 Dental problems that are very common include defective enamel and malocclusion.48,49 Dental caries are more common after surgical procedures to control drooling.50 Always consider the possibility of more severe dental infections (apical abscess, deep space infection) in a patient with CP with pain, fever, and/or decreased oral intake.

Increased Risk of Child Abuse or Neglect

Having a child with any developmental disability invariably places additional financial, emotional, and physical stress on family members. Children with disabilities are thought to be at increased risk of abuse or neglect.51 In one study, 20% of children with CP had suffered abuse severe enough to warrant removal from the home. Forty-three percent were found to be at enough risk to have had some type of intervention.1 Remember the stress placed on patients and their families, and be patient when dealing with these individuals.

Other Medical Problems

Vision and hearing deficits are common in patients with CP. Visual abnormalities include amblyopia, field defects, strabismus, myopia, and blindness.4,52 Hearing deficits may occur after congenital infections or following recurrent otitis media. Mental retardation is present in 50% of patients with CP.5 Remember when addressing CP patients and families that the patient may have normal intelligence but an impaired ability to communicate. Of all types of CP, children with only mild hemiplegia have the best intellectual outcome, with more than 60% having normal intellect.4

Behavior/Emotional Problems

Behavior problems, in particular hyperactivity, are common in children with CP.4,53 Adolescents with CP may suffer from depression, dependency, and low self esteem. With severe mental retardation, stereotypical or self mutilation behavior may be present.

Life Expectancy

Life expectancy is normal for CP patients with little functional disability. Immobility and severe cognitive defects are the strongest predictors of mortality in children with cerebral palsy.54 For the most severely affected patients, the 20-year survival rate is 42-58%.55 In the event of a CP patient presenting with a potentially life-threatening illness, the issue of code status may need to be brought up by the ED physician. Primary care physicians often discuss this issue with families in advance, but unfortunately this is not always the case.

Summary

Even when the complaint is seemingly minor, children with CP require a meticulous and thorough evaluation. An understanding and awareness of this complex disease and its treatment complications is mandatory for the emergency medicine physician. While one must focus on the acute problem, an understanding of the patient's complete history and health baseline is a necessity. Importantly, the family and subspecialists must be inolved in order to successfully evaluate and manage these patients.

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CME Questions

Which of the following has been associated with an increased risk of the development of CP?

a. Birth weight < 2000g

b. Congenital rubella infection

c. Neonatal intracranial hemorrhage

d. Maternal use of thyroid hormones

e. all of the above

Medical problems often present in CP patients include:

a. osteopenia.

b. constipation.

c. urinary retention.

d. aspiration.

e. All of the above.

The most common types of seizures in CP patients are:

a. tonic clonic

b. partial

c. absence

d. all of the above

Mental retardation is present in what percent of CP patients?

a. < 10%

b. 25%

c. 50%

d. 75%

e. All

Aplastic anemia is a significant adverse effect of:

a. felbamate.

b. phenytoin.

c. gabapentin.

d. lamotrigine.

e. None of the above

Superior mesenteric artery syndrome:

a. is due to fecal impaction.

>b. is uncommon after rapid weight loss.

c. presents with recurrent vomiting.

d. All of the above

CP patients may have prolonged duration of effect of which medication?

a. Atropine

b. Phenytoin

c. Cisapride

d. Clonazepam

e. Succinylcholine

CP patients may have dental problems due to:

a. defective enamel.

b. bruxism.

c. gastroesophageal reflux.

d. all of the above