Linda S. Nield, MD

Pneumonia is one of the most common conditions seen by primary care physicians who treat children. Respiratory symptoms are among the most common reasons why outpatients seek medical care. The distinction between upper respiratory tract disease and pneumonia should be made so that pneumonia is managed appropriately.

Here we summarize the evaluation of pediatric pneumonia and offer our management recommendations-based on a literature review and on clinical experience-in the outpatient setting.


According to World Health Organization, about 150 million cases of pneumonia occur annually in children younger than age 5 years. About 20 million cases are severe enough to require hospital admission, and 2 million children die worldwide. The majority of these deaths occur in Africa and Southeast Asia. Mortality is much lower in the United States, but morbidity is substantial.

Due to person-to-person spread because of overcrowding and also because of decreased  mucociliary clearance from dry air, pneumonia is more common during winter in temperate climates. The incidence of pneumonia is higher among boys, children from lower socioeconomic levels, and those exposed to smoke. 

The exact cause of pneumonia is not determined in most pediatric cases because of the difficulty in obtaining sputum specimens and the low incidence of bacteremia in children. Respiratory viruses are a major cause of pneumonia and occur frequently in all age groups-especially in infants and young children. The most common causes of pneumonia include influenza A and B viruses; respiratory syncytial virus (RSV); and 

types 1, 2, and 3. Adenovirus and rhinovirus can also cause pneumonia. The importance of metapneumovirus as a causative agent of childhood pneumonia is still being clarified, as is its role in producing more severe symptoms if coinfection with RSV is present.


Patient age, likely bacterial pathogens, and empiric outpatient antibiotic treatment

Certain organisms cause pneumonia at particular ages (

). This makes it possible to select antimicrobials based on a patient's age. Neonates can become infected with maternal vaginal and rectal flora; therefore, group B streptococci (

are the causes of pneumonia in this age group. In the developing world, 

From 1 month to 3 to 4 months of age, viruses are the most common pathogens. In infants between 1 and 3 months viruses are the most common cause of pneumonia. However, group B streptococci and organisms encountered by older children (

) occasionally cause pneumonia, along with 

. This atypical pathogen is of particular concern in children between 2 and 19 weeks old. Infection with 

may also present as afebrile pneumonia, but its role in causing disease in young, healthy infants is controversial.

In older infants and preschoolers (those younger than 5 years), viruses-especially RSV and parainfluenza-continue to be leading causes of pneumonia. In children older than 5 years, 

play the most important roles. In at-risk populations, 

are, respectively, the second and third most commonly identified bacterial causes of community-acquired pneumonia and are especially prevalent in the 5- to 10-year-old age group. However, preschool-aged children are also at risk. Approximately one-quarter of patients may have a mixed bacterial and viral infection.

However, even after extensive evaluation. the causative organism for pneumonia can be identified in fewer than half the patients.


The type of pneumonia and age of the patient affect the clinical presentation. In general, bacterial
pneumonia presents abruptly with high fever and may be accompanied by a productive cough. Viral or atypical pneumonia can present insidiously with upper respiratory tract symptoms, wheezing, and minimal fever.

Neonates with pneumonia display respiratory distress, temperature instability, and lethargy. Early onset of group B streptococcal disease usually occurs within the first 24 hours of life, but it can occur at any time between 0 and 6 days of age.
 
The infant with pneumonia in the first several months of life may be febrile, tachypneic or apneic, and irritable. Fever and tachypnea may be the only findings in a young infant or toddler with pneumonia. Atypical pathogens (ie, 

) can lead to an afebrile illness accompanied by cough, tachypnea, or wheezing.

The older child with bacterial pneumonia will experience abrupt onset of fever, cough, and malaise. Minimal fever, malaise, and lingering cough typify atypical pneumonia. Poor oral intake, nausea, vomiting, and abdominal pain may manifest in any person with pneumonia.


The patient's history of present illness and past medical history are essential in determining the possible cause of the pneumonia. Note the patient's immune status: an immunocompromised child may have an opportunistic infection (with 

for example) in addition to infection with the typical and atypical organisms that an immunocompetent child may encounter. A history of international travel and the time of year that the symptoms are occurring expand the list of possible pathogens.

During the physical examination, determine the respiratory rate for a full minute-especially in younger patients-and compare the rate with normal values for that child's age group because tachypnea is the most sensitive and specific sign of pneumonia. Observe the patient for any signs of respiratory distress (retractions, flaring, grunting, cyanosis) and auscultate the chest for wheezing, rhonchi, or decreased breath sounds. Wheezing is usually associated with viral infection-not bacterial infection. In older patients, it may be possible to check for signs of lower respiratory tract disease, such as dullness to percussion, increased fremitus, and egophony.

When you suspect viral pneumonia or if influenza and/or RSV infection are prevalent in your
community, perform a rapid antigen test (if readily available) on nasopharyngeal secretions, which can lead to timely diagnosis. Serologic tests may be performed to diagnose 

infections. A urinary antigen test may also be performed to diagnose 

The recently published guidelines for the management of community acquired pneumonia  include a recommdation against the use of a urinary antigen test for the diagnosis of pneumoncoccal pneumonia.

The difficulty of obtaining an adequate sputum sample in a young child precludes sputum analysis from being a routine component of the outpatient evaluation of uncomplicated pediatric pneumonia. A total serum white blood cell count and differential and measurement of sedimentation rate and C-reactive protein level are nonspecific aids that may provide evidence for a viral or bacterial cause. Blood cultures are of limited value in the diagnosis of pneumonia because results are positive in fewer than 10% of patients with pneumonia; however, cultures should be obtained in the young febrile infant-especially if that infant is not fully immunized against pneumococci.

  Blood cultures should also be obtained in any child who does not improve or who worsens despite treatment with antibiotics.

Anteroposterior and lateral chest radiographs help reveal areas of consolidation or
interstitial disease in the ill patient (

). The pattern of abnormal findings on
x-ray films provides a clue to the possible causative agent. Bacteria (

)
are more likely to produce a lobar pattern and viral or atypical pathogens are more
likely to produce an interstitial pattern. These radiographic descriptions are not
absolute: bacterial and viral pathogens can produce various patterns of radiographic
findings.

During outpatient treatment, order follow-up chest radiographs when you suspect a complication such as pleural effusion or empyema, evidenced by chest pain, failure to respond to antibiotics, recurrence of fever, and worsening respiratory tract signs. Upright and lateral decubitus radiographs also may be necessary in this setting. After a patient completes therapy for uncomplicated pneumonia, repeated chest radiographs are not necessary; if they are obtained, however, be aware that initial radiographic abnormalities may take 6 or more weeks to resolve.
 

The chest radiograph is not capable of detecting all pneumonias. High-resolution CT scans can detect infiltrates that are not evident on plain chest radiographs. A chest CT scan may be required in a pediatric patient with normal chest films and serious or unexplained respiratory symptoms-especially a febrile immunocompromised patient. More invasive testing is not routine in the general pediatric clinic. In complicated cases, referral for bronchoscopy; bronchoalveolar lavage; or collection of a pleural fluid sample for culture, Gram stain, and analysis (cell count; levels of glucose, protein, and lactate dehydrogenase; pH; and specific gravity) may be needed.

A tuberculin skin test may be performed in patients who are at high risk for contracting tuberculosis.


The patient’s age, history, clinical presentation, and radiographic findings guide therapy. Should the child be hospitalized or treated as an outpatient? Generally those who require hospitalization are younger than 3 to 6 months; have persistent hypoxemia and require supplemental oxygen; appear toxic; require IV hydration; or who have underlying chronic conditions, such as cystic fibrosis. If outpatient compliance with prescribed treatment is a concern due to social circumstances, hospitalization is advised.

If you suspect viral infection, supportive care includes adequate hydration, oxygen, and analgesics for general discomfort. If influenza is diagnosed, the use of anti-influenza medications should be considered on a case-by-case basis and ideally administered within 48 hours of illness. When you suspect bacterial or atypical pneumonia, base your choice of empiric antibiotic therapy on the most likely causative organisms.

Patients who are up to 6 months old need to be hospitalized and treated with IV antibiotics effective against the typical causative agents in this age group. Obtain blood samples for culture before you start therapy. If afebrile pneumonitis is likely, outpatient treatment with a macrolide is appropriate. Although there is an association between orally administered erythromycin and the development of pyloric stenosis in infants younger than 6 weeks, erythromycin is recommended in this age group for suspected 

 infections. Be sure to warn parents of this possible association.  Data are scant concerning the safety and efficacy of the use of other macrolides (ie, azithromycin) in the young infant.

The older infant, toddler, and school-aged child can be treated safely as an outpatient if he or she is immunocompetent, appears to be well, is able to stay adequately hydrated, and is not hypoxic. When viral pneumonia is likely, provide supportive care for the child. For the young child with suspected bacterial pneumonia, we recommend initial therapy with amoxicillin, amoxicillin/clavulanate, or cefuroxime. If resistant 

is a major factor in your community, high-dose amoxicillin may be necessary.

Clindamycin is an alternative if a child with suspected pneumococcal infection is beta-lactam–allergic. A macrolide is another option; however, standard doses of azithromycin, for example, provide marginal beta-lactamase coverage. Although 

is the most likely bacterial cause of pneumonia, antibiotics such as amoxicillin/clavulanate and cefuroxime allow for treatment of beta-lactamase–producing organisms (such as nontypeable

), which may be an increasing threat in this era of conjugate pneumococcal vaccine use.

Treat the older child and adolescent with a macrolide initially because atypical pathogens have a greater significance in this age group. Doxycycline may be used for children older than 8 years of age. If the child fails to respond, it is reasonable to add or switch to one of the previously mentioned antibiotics.
 
In certain situations, it may be necessary to treat for both bacterial and atypical pneumonia, with 2 agents-a beta-lactam and a macrolide or doxycycline. Fluoroquinolones are active against both 

and organisms that cause atypical pneumonia; therefore, they may be used in adolescents with pneumonia. Antibiotics are usually prescribed for 7 to 10 days. If there is no response or if the patient’s health deteriorates, consider other organisms and change antibiotics accordingly.

The management of a complication of pneumonia depends on its severity; patients typically require hospitalization for close monitoring. Pneumonia with a small effusion can be successfully treated with antibiotics alone, but large parapneumonic effusions and empyemas often require drainage. Seek surgical consultation to determine the best course for each circumstance. Because the treatment of pneumonia is empiric, outpatients need to be reevaluated frequently to ensure adequate response. Hospitalization is necessary for the child whose health worsens despite optimal outpatient management.


Complications of pneumonia can be local and may occur secondary to the spread of pulmonary infections to contiguous structures (eg, pleural effusion and empyema, pericarditis, and worsening of pulmonary infection leading to formation of a pulmonary abscess) (

). Complications can also be systemic; they may arise secondary to bacteremia, which can cause meningitis, osteomyelitis, or septic arthritis.

Bacterial pneumonia is the most common cause of pleural effusion or empyema. Pleural effusion can be a part of the clinical spectrum of pneumonia as a synpneumonic effusion, or it may be a complication that leads to an accumulation of pus in the pleural cavity, resulting in an empyema. Patients often have signs and symptoms of pneumonia along with a characteristic pleural rub (especially in small pleural collections), worsening of fever, respiratory distress, decreased air entry on the affected side, dullness to percussion, and mediastinal shift to the opposite side if the fluid accumulation is rapid and significant. These complications are more common in patients with staphylococcal or streptococcal pneumonias and in those who use antibiotics inappropriately or incompletely.

Treatment of children with a complication includes ultrasonograms or chest CT scans for diagnosis, antibiotics based on sensitivity patterns, and thoracotomy with tube drainage. Most patients respond to these interventions and rarely require additional interventions such as injection of fibrinolytic agents into the pleural space.


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2011;53:e25-e76.
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http://www.cdc.gov/std/treatment/2010/chlamydial-infections.htm

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2011;12:52-59. Epub 2010 Oct 16.http://www.ncbi.nlm.nih.gov/pubmed/21172676

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Articles

Update on Pediatric Pneumonia: Causes-Treatment Options

Figure. MRI reveals a posterior fossa mass in the 5-month-oldpatient.


A 5-month-old girl was brought to her local emergency department because she displayed increasing fussiness and back arching for 3 days. She vomited once and was febrile on the day of presentation. Meningitis was suspected. A lumbar puncture was performed. It revealed a cerebrospinal fluid (CSF) protein level of 120 mg/dL and a CSF white blood cell count of 10/µL, with 50% polymorphonuclear cells. Antibiotics were empirically administered, and the infant was transferred to a tertiary care facility for further treatment.


On arrival at the tertiary care facility, the child had normal vital signs and was alert, mildly irritable, and consolable. Findings from her physical examination were significant for a large head, a bulging fontanelle, and a downward gaze preference.


The CSF cultures were pending from the referring facility, and radiological studies were then obtained. A CT scan of the brain revealed obstructive hydrocephalus and a posterior fossa mass. MRI (Figure) was obtained to better define the abnormalities; the findings revealed an enlarged right cerebellar hemisphere with no focal dominant mass but with diffuse nodular enlargement of cerebellar folia and widening of the white matter in a striated pattern with obstructive hydrocephalus and associated tonsillar herniation.


Pediatric neurosurgery was consulted for placementof a ventriculoperitoneal shunt, and the patientwas subsequently discharged to home withthe agreed-on diagnosis of Lhermitte-Duclos disease(LDD) based on the distinctive radiologicalfindings. No further treatment was administered,but close monitoring by the pediatric neurologist,neurosurgical team, and primary care pediatricianwas instituted.

One month later, the child was rehospitalized becausesymptoms recurred. On repeated MRI, themass was noted to have increased significantly insize, and the decision was made to excise it. Tissueanalysis revealed a tumor consistent with medulloblastoma.Despite aggressive treatment with 7 cyclesof chemotherapy, the patient succumbed todisease at approximately 18 months after initialpresentation.


LDD, or dysplastic cerebellar gangliocytoma, is a rare benign brain lesion described in approximately 200 cases in the medical literature,

 with the majority occurring in adult patients presenting with occipital head pain and other typical signs of a mass in the posterior fossa. In contrast, medulloblastoma is the most common, poorly differentiated CNS malignancy, accounting for 20% of all pediatric brain tumors.

 Both LDD and medulloblastoma arise in the posterior fossa, but the distinctive appearance of LDD on MRI typically allows for a straightforward diagnosis based on the MRI findings alone.

 This scenario of a 5-month-old girl in whom medulloblastoma was initially misdiagnosed as LDD based on her MRI findings is similar to one other case reported by Chen and colleagues

concerning an 18-month-old patient with medulloblastoma whose MRI findings mimicked LDD.


Accurate and prompt determination of the cause of a CNS mass in a child is crucial to administering appropriate therapy in a timely manner. Histological examination of any tumor is the definitive method of determining the exact type of lesion present, but obtaining a tissue specimen is fraught with serious risks. Although establishing a diagnosis based on radiological findings alone is attractive and noninvasive, it also carries the risk of misdiagnosis.	

The presence of a lesion rare for a patient's age should have raised doubts in the image-based diagnosis. LDD is a rare entity. Our case highlights the importance of questioning its diagnosis in any child. This case illustrates the pitfall of diagnosis based on morphological imaging alone, since our patient's tumor displayed the features (Figure) of enlarged cerebellar folia, enlargement of the white matter between adjacent cerebellar folia, and absence of a well-defined focal dominant mass that are so typically present with LDD.

The Table highlights the imaging features typically associated with LDD, medulloblastoma, and medulloblastoma with extensive nodularity (MBEN) in comparison with the features present in the case. Note that the features of the case, LDD, and the MBEN variant of medulloblastoma are highly concordant. This concordance of imaging findings contributed to the misdiagnosis. Although the case did have the finding of hyperintense signal on diffusion-weighted imaging, this was not considered highly in determining the diagnosis, since there is great variability and inconsistency in the presence of this finding in neoplastic brain lesions.

On MRI, LDD appears as a non-enhancing striated mass in the cerebellar hemisphere, hyperintense on T2-weighted images, and hypointense on the corresponding T1-weighted images. The folia are thickened because of an excess of dysplastic cortical neurons that results in a loss of Purkinje cells and thinning of the medullary white matter.

 This finding is unique to LDD because most other cerebellar masses destroy the folial pattern and produce enhancement.

In contrast to LDD, the typical medulloblastoma in a young child is a midline, spherical mass that fills the fourth ventricle, arising from the superior medullary velum of the fourth ventricle. It appears to be a discrete mass that displaces, rather than invades, adjacent normal cerebellar tissue. Typically, medulloblastoma will be hyperdense to normal brain tissue on a noncontrasted CT scan, and it may calcify. Medulloblastoma enhances intensely on either CT or MRI. T2-weighted images of typical medulloblastoma are near isointense to gray matter.

MBEN, which affects children younger than 3 years, is a rare variant of medulloblastoma, which has alternating layers, or zones, of differing tumor histology.

 These alternating layers mimic the striated pattern seen in LDD.

Differentiating medulloblastoma from LDD is essential in order to counsel families about further therapy and prognosis. The long-term prognosis of medulloblastoma can be poor and is affected by age (poorer in the patient younger than 3 years), extent of postoperative residual disease (poorer if residual disease is greater than 1.5 cm

), and the presence of metastatic disease.

 In contrast, the few reported cases of pediatric LDD describe a fairly benign course.

Since the management of LDD and medulloblastoma differ markedly, differentiating these 2 entities is paramount. The optimum management of either disease is yet to be decided, but the diagnosis must be definitively determined before a treatment pathway can even be established in a particular patient.

In general, the treatment of both tumors includes surgical resection. Although LDD is "benign," it is managed by surgical decompression or tumor excision if the patient is symptomatic.

 If surgery is not needed, the patient is monitored closely for neurological sequelae. Children with this entity have survived by using a wait and see approach.

Whereas surgery or close observation alone will be effective treatments for LDD,

 management of medulloblastoma also includes combined modalities (radiotherapy and chemotherapy) for improved survival rates.

 Pediatric survivors of medulloblastoma are at particular risk for serious adverse effects secondary to their treatments.

Our case of misdiagnosed medulloblastoma reiterates the pitfalls associated with applying radiological findings alone to diagnosis of a tumor. Included is the serious psychological distress that misdiagnosis   causes patients' parents and older patients.

Each case of posterior tumor fossa must be evaluated individually, but the clinician should give strong consideration to obtaining a histological diagnosis in all cases despite the great risks that accompany tissue sampling, even if MRI findings are very highly suggestive of the nonmalignant entity of LDD.    

 Robinson S, Cohen AR. Cowden disease and Lhermitte-Duclos disease: an update. Case report and review of the literature. 

 Frosch MP, Anthony DC, De Girolami U. The central nervous system. In: Kumar V, Abbas AK, Fausto N, eds. 

Robbins and Cotran's Pathologic Basis of Disease. 

7th ed. Philadelphia: Saunders; 2005:1401-1420.


 Carlson JJ, Milburn JM, Barre GM. Lhermitte-Duclos disease: case report. 

 Kulkantrakorn K, Awwad EE, Levy B, et al. MRI in Lhermitte-Duclos disease. 

 Meltzer CC, Smirniotopoulos JG, Jones RV. The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dysplastic gangliocytoma). 

 Chen KS, Hung PC, Wang HS, et al. Medulloblastoma or cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease)? 

 Suresh TN, Santosh V, Yasha TC, et al. Medulloblastoma with extensive nodularity: a variant occurring in the very young-clinicopathological and immunohistochemical study of four cases. 

 Gilbertson RJ. Medulloblastoma: signaling a change in treatment. 

 Capone Mori A, Hoeltzenbein M, Poetsch M, et al. Lhermitte-Duclos disease in 3 children: a clinical long-term observation. 

 Walker DA, Wilne S. Treatment of medulloblastoma in young children. 

A 5-month-old girl was brought to her local emergency department because she displayed increasing fussiness and back arching for 3 days. She vomited once and was febrile on the day of presentation. Meningitis was suspected. A lumbar puncture was performed. It revealed a cerebrospinal fluid (CSF) protein level of 120 mg/dL and a CSF white blood cell count of 10/µL, with 50% polymorphonuclear cells. Antibiotics were empirically administered, and the infant was transferred to a tertiary care facility for further treatment.

Lhermitte-Duclos Disease or Neoplasm?

Cerebral palsy (CP) refers to a group of nonprogressiveneurologic conditions defined byspecific motor deficits or movement disorders.It is often associated with other systemicsequelae. Because 65% to 90% of childrenwith CP survive into adulthood,1 it is importantfor clinicians-whether they have a pediatricoradult-based practice-to understand themultisystemic nature of CP.

 Prevalence in surviving neonates is about 0.2%, and CP has been reported to account for 2% to 8% of office visits to the pediatric neurologist.

 followed by the ataxic and athetoid types. Visits to the doctor are often precipitated by comorbidities involving organ systems other than the nervous system.

Historically, CP was thought to be associated with birth trauma. Although the percentage of CP cases attributable to birth asphyxia is debatable, Nelson and Ellenberg

 estimated that 6% to 7% of cases result from asphyxia at birth and that the cause of 80% of cases has to do with prenatal complications. Congenital, genetic, inflammatory, anoxic, traumatic, toxic, and metabolic causes all have been implicated as causes of CP. Prenatal risk factors include intrauterine infections; chorioamnionitis; fetal thrombophilia; exposure to teratogens; placental complications; multiple births; and maternal conditions such as mental retardation, history of seizures, and hyperthyroidism. New models that look at the inflammatory-mediated model of CP and its developmental sequelae are being developed.

Perinatal events such as preterm birth, low birth weight, intracranial hemorrhage, infection, seizure, hypoglycemia, and hyperbilirubinemia are well-known risks for neurologic sequelae. Careful developmental and neurologic screening is warranted in pediatric patients who have been affected by these conditions in the perinatal period. Furthermore, accurate determination of the cause of CP has specific implications for prevention in future pregnancies, treatment, prognosis, and medicolegal issues.

 Parents' and pediatrician's concerns about abnormal motor development provide the first clue to a possible diagnosis of CP and may initiate referral to a pediatric neurologist. The history may reveal risk factors; findings from the physical examination may identify the type of CP. For guidance on the diagnostic assessment of the child with CP see "Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society," which was authored by Ashwal and colleagues and appeared in 

The traditional diagnostic approach consists of early assessment of impairments in muscle tone, strength, and control; assessment of involuntary movements; asymmetry; persistence of primitive reflexes; and late development of postural responses. Note that only about one quarter of children aged 7 years who have CP have a history of an abnormal neurologic examination at birth.

In reviewing the differential diagnosis, conditions such as metabolic disorders should not be overlooked. Be aware that genetic syndromes such as Lesch-Nyhan syndrome can mimic CP. Consider them in the differential diagnosis if the pregnancy and birth history are unremarkable.

CP also can be detected based on functional limitations.  Estimating functional limitations using the motor quotient (motor age divided by chronologic age multiplied by 100) is one diagnostic tool.  A motor quotient below 50 is predictive of gross motor delay.

 Neuroimaging is recommended in the evaluation of a child with CP if the cause has not been previously established by perinatal imaging. MRI is preferred to CT because this form of imaging is more likely to identify a cause.

 Cranial ultrasound is recommended for infants between 7 and 14 days old and near term-corrected age to identify intraventricular hemorrhage, periventricular leukomalacia, and low-pres- sure ventriculomegaly.

 When an asphyxial episode during gestation is suspected, cord and serum laboratory studies (ie, hemoglobin/hematocrit, platelet count, pH, liver, and renal function tests) are available to help make a determination of timing and severity of the insult. Metabolic and genetic studies should not be routinely ordered but should be considered when clinical history or findings and neuroimaging do not identify a specific structural abnormality.

 Coagulation studies should be obtained in hemiplegic CP.

 A genetics consultation may be useful to further evaluate specific conditions and dysmorphologies in which CP is one characteristic. An electroencephalogram should be obtained only when a child with CP has a history suggestive of epilepsy.

 CP is associated with motor and mental disabilities of variable severity in a growing child. The motor abnormalities include spasticity (spastic quadriplegia, hemiplegia, and diplegia) and movement disorders that may coexist in the same patient. Because bone growth exceeds muscle growth in the presence of spasticity in a growing child, contractures and bone deformities develop.

Dystonia, athetosis, or both are present in 10% to 20% of patients, and children with dystonia may have eye movement and oromotor abnormalities.

 The ataxic form of CP is uncommon and presents with gait abnormalities and fine motor dysfunction.

Although not a defining feature of CP, cognitive impairment occurs in many patients and ranges from learning disabilities to attention deficit disorders to severe mental retardation. Motor development is not a reliable predictor of cognitive development.

 For example, children with athetotic CP may have normal intelligence.

Hearing loss of the sensorineural or conductive type and visual impairment (refractory errors, visual field defects, cortical visual impairment, faulty accommodation, strabismus, nystagmus, and optic atrophy) are common.

 Other sensory defects may include impairment of touch and pain perception, proprioception, and ste- reoagnosis in spastic forms of CP.

Seizures--typically grand mal, which often are difficult to treat

--occur in many children with CP. They usually manifest in the first year of life, more so in children with spastic hemiplegia or quadriplegia.

 Dislocations and bony deformities are not uncommon in the CP population. Spinal deformities such as kyphoscoliosis and lordosis develop in children with CP, particularly those who cannot ambulate. Incidence is as high as 60%.

 The spinal deformities progress despite skeletal maturity and may interfere with good seating posture and cause pain, pelvic misalignment, and pressure sores. Cervical and lumbar spondylosis and myelopathy may occur in athetoid CP secondary to stresses incurred by repetitive complex trunk movements.

Nonambulators also are at high risk for osteopenia and fractures. Reduced bone mineral density is attributed mainly to immobility, poor nutrition, and use of anticonvulsant medication.

 Fractures usually occur at the metaphysis of the distal femur and proximal tibia and may be caused by contractures, falls, seizures, and overzealous physical therapy.

Contractures at the hip and knee in particular are a source of pain and disruption to daily activities such as sitting, standing, and perineal hygiene.

 Equinus deformity occurs in almost all patients with spastic CP secondary to gastronemius and soleus muscle spasticity, which results in toe walking.

Upper limb deformities are seen in patients with spastic hemiplegia and quadriplegia. The shoulders are internally rotated and flexion contractures occur at the elbow and wrist. Forearm pronations, fisted hand with thumb in palm, and swan finger deformities also are common.

 Oromotor muscle incoordination and spasticity may interfere with speech, suck, and swallow mechanisms and may cause temporomandibular joint contractures.

 Speech delay and dysarthria are potential sequelae. Also, dental hygiene can be difficult to maintain. Malocclusion, enamel dysplasia, and dental caries occur frequently.

 Gastroesophageal reflux disease (GERD) has been associated with dental erosion in CP, and degree of GERD severity has been correlated with degree of tooth erosion.

Drooling is one of the most troublesome oromotor problems for patients with CP. Poor head control and posture, impaired lip closure, abnormal tongue movements, and oral sensory impairment may contribute.

Swallowing difficulties, GERD, and constipation also contribute to feeding problems, which may occur very early in life. In a population survey of children with CP aged 12 to 72 months, more than 90% had clinically significant oromotor dysfunction. Severe feeding problems preceded the CP diagnosis in 60% of these children.

 The undernourishment that results from feeding problems leads to growth failure.

 In those children who do not have major feeding disturbances, however, excessive weight gain can occur because of the child's inability to participate in physical activities.

 Congential heart disease may be a component of genetically mediated CP. Pneumonia may occur frequently in the severely affected patient secondary to immobility, weak cough, contorted posture (scoliosis or chest wall deformities), GERD, and risk of aspiration.

 Over 90% of children with CP have associated GI symptoms, including constipation, swallowing problems, regurgitation and/or vomiting and abdominal pain.

 GERD, which is often difficult to control, has been found in up to 75% of patients

 and increases the risk of hematemesis, rumination, and dental erosion. Constipation has been associated with transit time delay in at least one segment of the colon and to ambulatory function.

 Bladder difficulties often occur concurrently with bowel difficulties in children with CP. Dysfunctional voiding symptoms, such as incontinence and urinary urgency, have been noted in more than half of all patients with CP.

Reproductive issues are often overlooked in the adolescent patient with CP. According to a recent cross-sectional survey, puberty tends to begin earlier in children with CP compared with the general population.

 However, menarche occurs later, with the median age being 14 years, as opposed to 12.8 years in the general population.

It is important to note that children with developmental disabilities are vulnerable to sexual abuse. Findings based on data from the National Center on Child Abuse and Neglect suggest that children with disabilities are twice as likely to be sexually abused as are children in the general population.

 Furthermore, the US Department of Justice reports that up to 83% of women with developmental disabilities are sexually abused at some point in their lives.

 Behavioral disorders, including attention-deficit/hyperactivity disorder, are more prevalent in children with CP.

 Frustration, aggression, and mood disturbances may be part of the picture. A change in behavior may signal that the child is in pain, especially in a child with communication difficulties. The degree of pain is difficult to assess and may also manifest as sleep disturbance, decreased appetite, or intolerance of movement. Several potential sources of pain include dental caries, GERD and esophagitis, constipation, infections, and musculoskeletal abnormalities.

 Management of the child with CP begins with a comprehensive medical evaluation by a multidisciplinary team that includes a pediatrician; a pediatric neurologist; physical, occupational, and speech therapists; an audiologist; an ophthalmologist; a social worker; and possibly a neurosurgeon and orthopedic surgeon. Family members are the most important part of the treatment team. Hearing tests and early referrals to audiologists and speech therapists are necessary to prevent delays in language and learning skills. Use of electronic communication devices may help alleviate frustration caused by communication difficulties. Screening for visual defects and ophthalmologic referrals should take place within the first year of life. Cognitive delays warrant careful assessment, initiation into early intervention programs, and the development of an individualized education plan in the face of severe motor and communication difficulties.

The goals of overall therapy should be aimed at maximizing the patient's level of function. Important areas of focus include ambulation, cognitive skills, activities of daily living, personal hygiene, and rehabilitation into society.

 The primary care physician will likely play the central role in coordinating care, but the pediatric neurologist will be called upon for expert guidance.

 Pharmacotherapeutic resources are available to manage symptoms such as spasticity and sleep disturbances and to manage seizures and movement disorders in general. Oral antispasticity medications include benzodiazepines, baclofen, a

-adrenergic agonists, and dantrolene sodium (Dantrium).

 Injectable medications include botulinum toxin A (Botox).

Invasive procedures include placement of an intrathecal infusion pump for continuous delivery of baclofen to the CNS for the treatment of severe spasticity. Several studies have shown that intrathecal baclofen therapy is safe and effective in children with CP.

Another neurosurgical procedure for spasticity is highly selective dorsal root rhizotomy. This procedure attempts to cut fewer than 50% of the sensory fascicles from each cauda equine nerve root, guided by intraoperative neuromuscular testing to identify the most abnormal nerve fascicles that should undergo ablation.

Few studies have directly compared the safety, efficacy, and long-term outcomes or relative costs of dorsal root rhizotomy and the baclofen pump. Therefore, the decision to use either of these interventions must be made on a case-by- case basis.

 Seizure control with antiepileptic drugs or use of a ketogenic diet or surgery also should be considered on a case-by-case basis, depending on patient presentation and seizure type.

Therapies for dystonia include anticholinergics and dopamine agonists. Sleep problems, which can be attributed to pain and motor dysfunction, can be managed with melatonin and other sleep aids and by simply establishing an appropriate bedtime routine.

 Management of the musculoskeletal sequelae of CP should include the participation of the pediatric orthopedic surgeon and physical therapist. Treatment should be tailor-made for the specific orthopedic abnormality present. Consultation with the specialists will determine optimum timing and type of treatment.

 Of various strategies studied to reduce plaque and gingivitis in patients with CP, an electric toothbrush was found most effective in optimizing oral hygiene.

 The use of cryotherapy to enhance mouth opening by providing a temporary reduction in masseter spasticity has been shown to improve oral access and thus facilitate preventative dental care and other dental procedures.

Occupational and behavioral therapy using oral stimulation techniques, such as stroking, tapping, and blowing exercises, has been shown to improve drooling.

 The centrally acting antispastic agent modafinil (Provigil)

 and anticholinergic medications such as scopolamine, glycopyrrolate (Robinul), and benzotropine improve drooling but are associat- ed with significant systemic adverse effects.

 Ultrasound-guided intraglandular injection of botulinum toxin A can successfully reduce drooling without major adverse effects.

 Surgical options include submandibular gland excision, parotid duct ligation, duct rerouting, and transtympanic neurectomy.

Placement of gastrostomy tubes (G-tubes) for non-oral feeding should be considered in patients at high risk for chronic undernourishment because of severe impairment of oromotor function. Oral feeding interventions for such patients have not been shown to be effective in promoting feeding efficiency or weight gain.

 Although concerns have been raised, no correlation between respiratory problems and G-tube placement has been found.

The placement of a G-tube has been shown to improve the caregiver's quality of life as measured by significantly reduced length of feeding times, increased ease of drug administration, and reduced parental concern regarding the child's nutritional status.

 Consultation with a nutritionist may be of benefit for the child with CP who experiences either poor weight gain or obesity.

 Any evidence of heart or lung disease should be aggressively evaluated. Pediatric cardiology consultation should be sought for definitive diagnosis and treatment of the cardiac abnormality. Effective pulmonary toileting and administration of antibiotics, including anaerobic coverage, are essential for the treatment of pneumonia in the patient with CP.

 In patients with GERD in whom medical management with proton pump inhibitors fails, Nissen fundoplication may be undertaken. A study

 of gastrojejunal tube placement suggested that the surgical procedure may be equally helpful in improving GERD. Administering baclofen orally or via a nasogastric tube to reduce symptoms of GERD also has been shown to reduce the frequency of emesis and the total number of events of acid reflux.

Treatment of chronic constipation demands a multifaceted approach that includes a regular toileting schedule, proper toilet positioning, dietary modifications, and medication. In patients with impacted stool, it is important to begin with a "clean-out" regimen using either oral osmotic agents or serial enemas. After the impacted stool is removed, a maintenance program using titrated agents such as polyethylene glycol to achieve daily soft stool should be continued on a long-term basis. Oral mineral oil should not be used because of risk of aspiration.

 Highly selective dorsal root rhizotomy may improve spasticity and bladder storage capacity in appropriate patients. Neurogenic detrusor overactivity--the most common finding in patients with lower urinary tract symptoms--can most often be controlled with a clean intermittent catheterization protocol and concomitant anticholinergic medication.

In regard to precocious puberty, a pediatric endocrinologist may be consulted. Gonadotropin-releasing hormone agonists remain the mainstay of treatment.

Older female patients in particular have unique medical needs, including contraceptive care and breast cancer screening. Mammography is underutilized by women with CP.

 Barriers to care include communication problems, transport, and physical limitations requiring adaptation of mammographic technique. Similar barriers also have been associated with access to contraceptive care in this patient population.

 Those patients who are taking antiepileptic agents for management of concomitant seizure disorders and who request contraceptive care should be counseled about the decreased effectiveness of oral contraceptives when used with certain antiepileptic drugs. The increased risk of thrombosis in women who have limited mobility also should be considered before initiating oral contraceptives.

 An integrative team approach, including monthly meetings between the rehabilitation therapist, child psychiatrist, developmental pediatrician, psychologist, and a preventative medicine specialist, has been shown to improve standardized measures of family stress associated with the parents' attitude toward the child with a disability. It also has shown significant improvements in the subscales of the Vineland Adaptive Behavior Scales assessment of the child.

Mood and behavioral disturbances are treated on a case-by-case basis, with behavior modifications and medication that depend upon the individual patient's circumstances. If pain is the possible trigger for the behavior change, for example, management should focus on uncovering and treating the underlying cause.   

 Zaffuto-Sforza CD. Aging with cerebral palsy. 

 Curless RG. Diagnostic problems in three pediatric neurology practice plans. 

 Odding E, Roebroeck ME, Stam HJ. The epidemiology of cerebral palsy: incidence, impairments and risk factors. 

 Nelson KB, Ellenberg JH. Antecedents of cerebral palsy: multivariate analysis of risk. 

 Toso L, Poggi S, Park J, et al. Inflammatory- mediated model of cerebral palsy with developmental sequelae. 

 Ashwal S, Russman BS, Blasco PA, et al; Quality Standards Subcommittee of the American Academy of Neurology; Practice Committee of the Child Neurology Society. Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. 

 Palmer FB. Strategies for early diagnosis of cerebral palsy. 

 Khan A, Vasudevan A. Lesch-Nyhan Syndrome. 

 Hadders-Algra M. General movements: A window for early identification of children at high risk for developmental disorders. 

Author | Linda S. Nield, MD

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