Sharmila Nanda, MD

A 9-year-old asymptomatic boy was referred to our tertiary care facility with a blood lead level (BLL) of 59 ?g/dL. A diagnosis of attention deficit hyperactivity disorder, which was managed with amphetamine/dextroamphetamine, had been made when the patient was 6 years old. 

Other than the patient's abnormal eating habits, a review of systems was negative. According to his mother, the child ate "anything and everything- including refrigerator magnets!" Results of a physical examination were unremarkable. The complete blood cell count was normal, as was the zinc protoporphyrin level.

) revealed multiple radiopaque specks throughout the colon and moderate stool retention. The patient was hospitalized for bowel clean-out and initiation of chelation therapy with succimer (meso-2,3-dimercaptosuccinic acid [DMSA]). Inspection of his stool revealed parts of refrigerator magnets, string, paper, labels, pigments of various colors, pencil points, and other unidentifiable particles (

). The exact source of lead was not determined- even after an extensive environmental inspection.

Figure 1 - This abdominal radiograph reveals multiple radiopaque foci in the colon that could represent lead-containing or other radiopaque material.

Figure 2 - These items were recovered from the stool of a child with pica 

 Pica is the unusual phenomenon of compulsive ingestion of nonfood substances. Normally, children in the first 2 to 3 years of life explore objects in the environment with their mouths. When older persons ingest non-food substances, the habit is called pica.

Paper, clay, and dirt are a few of the more common non-food substances ingested, along with the chewing of ice (pagophagia). Pica may signal the presence of an underlying condition, including developmental and cognitive disorders (mental retardation, autism, dementia, delirium), psychiatric disorders (depression, schizophrenia, obsessive- compulsive disorder), and nutritional deficiencies (iron or zinc deficiency). It may be symptomatic of celiac disease, child neglect or inadequate supervision, or pregnancy. In some of these conditions, it is unknown whether pica is the cause or the effect.

The known potential consequences of pica are formation of a gastric bezoar,

 parasitic infection, and an elevated blood lead level.

 The normal mouthing of objects in early toddlerhood and pica are risk factors for elevated BLLs (in excess of 10 ?g/dL).

 In the United States, the main sources of lead are paint (mainly in homes built before the late 1970s), dust, and soil.

 Although adverse effects may result from a BLL lower than 10 ?g/dL, the American Academy of Pediatrics (AAP) and the CDC consider a BLL of 10 ?g/dL or greater as the threshold for concern.

An elevated BLL can be confirmed with a single venous blood specimen or with any combination of 2 capillary and/or unknown blood specimens drawn within 12 weeks of each other.

US surveillance data from 2005 revealed that BLLs were elevated in fewer than 2% of children who were tested.

 In approximately 500 of those children, BLLs exceeded 44 ?g/dL. BLLs typically peak at 2 years of age and do not generally persist into older childhood, even when housing improvements have not been made.

 In addition to undetermined reasons, this is probably because, at this age, children stop putting objects into their mouths. Populations of children at particular risk for a high BLL include medicaid- eligible youngsters, immigrants, and international adoptees.

. Cognitive deficits and behavioral abnormalities are the main sequelae of lead poisoning. High lead levels can cause encephalopathy and seizures, but the true impact of lead exposure on the development of behavioral disorders (such as attentional problems) is unknown.

 The most recent AAP policy statement on the management of lead poisoning recommends chelation with succimer for patients with a BLL in excess of 45 ?g/dL.

 The statement emphasizes that there are no data to support chelation therapy at lower BLLs .

 Treatment must begin after the environmental source of lead exposure has been eliminated. Underlying iron deficiency, which increases GI absorption of lead, must also be corrected.

The reader is referred to the AAP's policy statement

 and also to a recent publication by the CDC that outlines management for children with BLLs below 10 ?g/dL, environmental interventions, prevention, and screening recommendations.

The treatment of pica may be the more difficult task-especially in the cognitively or developmentally delayed patient. Consistent attention and careful adult supervision, behavioral modifications (verbal reprimands, physical restraints), and aggressive treatment of any potential underlying associated conditions may require the expertise of other specialists.

Conyers R, Efron D. Agitation and weight loss    in an autistic boy. J Paediatr Child Health. 2007;    43:186-187. 

Kim S, Qureshi F. Images in emergency medicine.    Small-bowel obstruction as a result of sand    pica. Ann Emerg Med. 2007;49:378, 386.    

de Freitas CU, De Capitani EM, Gouveia N, et al.    Lead exposure in an urban community: investigation    of risk factors and assessment of the impact of    lead abatement measures. Environ Res. 2007;103:    338-344.    

American Academy of Pediatrics Committee on    Environmental Health. Lead exposure in children:    prevention, detection and management. Pediatrics.    2005;116:1036-1046.    

Department of Health and Human Services    Centers for Disease Control. CDC Surveillance    Data, 1997-2006. Available at: http://www.cdc.gov/    nceh/lead/surv/stats.htm. Accessed January 29,    2008.    

Department of Health and Human Services    Centers for Disease Control. Number of children    tested and confirmed EBLLs by state, year, and BLL    group, children < 72 months old. Available at:    http://www.cdc.gov/nceh/lead/surv/database/    State_Confirmed_byYear_1997_to_2005.xls.    Accessed January 29, 2008.    

Hussain J, Woolf AD, Sandel M, Shannon MW.    Environmental evaluation of a child with developmental    disability. Pediatr Clin North Am. 2007;54:    47-62.    

Centers for Disease Control and Prevention    (CDC) Advisory Committee on Childhood Lead    Poisoning Prevention. Interpreting and managing    lead levels < 10 microg/dL in children and reducing    childhood exposures to lead: recommendations of    the Advisory Committee on Childhood Lead    Poisoning Prevention [published correction appears    in MMWR. 2007;56:1241]. MMWR. 2007;56(RR-8):    1-16. Available at: http://www.cdc.gov/MMWR/    preview/mmwrhtml/rr5608a1.htm. Accessed    January 29, 2008.    

Articles

Boy With Extraordinarily High Blood Lead Levels

Cerebral palsy (CP) refers to a group of nonprogressive neurological conditions defined by specific motor deficits or movement disorders. It is often associated with other systemic sequelae. Because 65% to 90% of children with CP survive into adulthood,

 it is important for clinicians to understand the multisystemic nature of CP. Here we present an overview.

 Prevalence in surviving neonates is about 0.2%. CP accounts 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 resulted from asphyxia at birth--and that 80% of cases were associated with prenatal complications. Congenital, genetic, inflammatory, anoxic, traumatic, toxic, and metabolic conditions 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 neurological sequelae. Careful developmental and neurological 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.

The 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 diagnostic assessment, 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."

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. Only about one quarter of children with CP aged 7 years had an abnormal neurological examination at birth.

In reviewing the differential diagnosis, conditions such as metabolic disorders should not be overlooked. Also be aware that genetic syndromes such as Lesch-Nyhan syndrome can mimic CP and should be considered 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 mo- tor quotient (motor age divided by chronological age times 100) is one diagnostic tool. A motor quotient below 50 predicts 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 it is more likely to identify a cause.

 Cranial ultrasonography is recommended for infants between 7 and 14 days old and near term-corrected age to identify intraventricular hemorrhage, periventricular leukomalacia, and low-pressure 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) help determine the 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 that suggests 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 often 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 be of 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 stereognosis 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. The incidence is as high as 60%.

 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 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 gastrocnemius 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, the ability to 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 their 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 1 segment of the colon and with 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 CP patients.

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 than in the general population.

 However, menarche occurs later: the median age is 14 years, as compared with 12.8 years in the general population.

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 than 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 to 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 an 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 ophthalmological referrals should take place within the first year of life. Cognitivedelays warrantcareful 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 probably play the central role in coordinating care, but the pediatric neurologist will be called on 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 use of either of these interventions must be decided 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 needs to 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 preventive 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 diminish drooling.

 The centrally acting antispastic agent modafinil (Provigil)

 and anticholinergic medications, such as scopolamine, glycopyrrolate (Robinul), and benzotropine reduce drooling but are associated 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 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 improves the caregiver's quality of life as measured by a significant reduction in feeding times, increased ease of drug administration, and reduced parental concern regarding the child's nutritional status.

 Consultation with a nutritionist may benefit 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 who do not respond to medical management with proton pump inhibitors, Nissen fundoplication may be undertaken. A study of gastrojejunal tube placement suggested that this surgical procedure may be equally helpful in improving GERD.

 Oral administration of baclofen 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.

For precocious puberty, a pediatric endocrinologist may be consulted. Gonadotropin-releasing hormone agonists remain the mainstay treatment.

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

Mood and behavioral disturbances are treated on a case-by-case basis, with behavior modifications and medication that depend on 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. 

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 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

 Accordo J, Kammann H, Hoon A. Neuroimaging in cerebral palsy. 

 Flett PJ. Rehabilitation of spasticity and related problems in childhood cerebral palsy. 

 Sanger TD, Delgado MR, Gaebler-Spira D, et al. Classification and definition of disorders causing hypertonia in childhood. 

 Walker WD, Johnson CP. Mental retardation: overview and diagnosis. 

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 Krigger KW. Cerebral palsy: an overview. 

 Deluca PA. The musculoskeletal management of children with cerebral palsy. 

 Sakai T, Yamada H, Nakamura T, et al. Lumbar spinal disorders in patients with athetoid cerebral palsy: a clinical and biomechanical study. 

 Henderson RC, Kairalla JA, Barrington JW, et al. Longitudinal changes in bone density in children and adolescents with moderate to severe cerebral palsy. 

 Dabney KW, Lipton GE, Miller F. Cerebral palsy. 

 Cooley WC. Providing a primary care medical home for children and youth with cerebral palsy. 

 Su JM, Tsamtsouris A, Laskou M. Gastroesophageal reflux in children with cerebral palsy and its relationship to erosion of primary and permanent teeth. 

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 Chong SK. Gastrointestinal problems in the handicapped child. 

 Del Guidice E, Staiano A, Capano G, et al. Gastrointestinal manifestations in children with cerebral palsy. 

 Bohmer CJ, Klinkenberg-Knol EC, Niezen-de Boer MC, Meuwissen SG. Gastroesophageal reflux disease in intellectually disabled individuals: how often, how serious, how manageable? 

 Park ES, Park CI, Cho SR, et al. Colonic transit time and constipation in children with spastic cerebral palsy. 

 Karaman MI, Kaya C, Caskurlu T, et al. Urodynamic findings in children with cerebral palsy. 

 Worley G, Houlihan CM, Herman-Giddens ME, et al. Secondary sexual characteristics in children with cerebral palsy and moderate to severe motor impairment: a cross-sectional survey.

 Murphy N, Elias ER. Sexuality of children and adolescents with developmental disabilities. 

Working With Victims of Crime With Disabilities.

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Verrotti A, Greco R, Spalice A, et al. Pharmacotherapy of spasticity in children with cerebral palsy. 

 Albright AL, Gilmartin R, Swift D, et al. Long-term intrathecal baclofen therapy for severe spasticity of cerebral origin.

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 Gerszten PC, Albright AL, Johnstone GF. Intrathecal baclofen infusion and subsequent ortho- pedic surgery in patients with spastic cerebral palsy. 

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 Hurst D, Cedrone N. Modafinil for drooling in cerebral palsy. 

 Jongerius PH, van den Hoogen FJ, van Limbeek J, et al. Effect of botulinum toxin in the treatment of drooling: a controlled clinical trial. 

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 Sullivan PB, Juszczak E, Bachlet AM, et al. Gastrostomy tube feeding in children with cerebral palsy: a prospective, longitudinal study. 

ABSTRACT: Most cases of cerebral palsy (CP) are the result of congenital, genetic, inflammatory, anoxic, traumatic, toxic, and metabolic disorders. A minority of cases result from asphyxia at birth. Nearly three-quarters of children with CP aged 7 years had a normal neurological evaluation at birth. Abnormal motor development usually provides the first diagnostic clue. Neuroimaging is recommended if the cause of CP has not been established with perinatal imaging. MRI is preferred to CT. Management of the multisystemic manifestations begins with a comprehensive medical evaluation by a multidisciplinary team that includes family members. Therapy is aimed at maximizing the patient's level of function. Key areas include ambulation, cognitive skills, activities of daily living, hygiene, and rehabilitation into society.

Cerebral Palsy: A Multisystem  Review

Even in the winter, children should be encouraged to play outdoors. Such exercise has significant cardiovascular and musculoskeletal benefits. However, those benefits must be weighed against the risk of potential localized or systemic consequences of excessive exposure to cold. Hypothermia is life-threatening. Localized injuries are typically less severe but may be accompanied by generalized hypothermia and are associated with great morbidity and even death (

By understanding the epidemiology and pathophysiology of hypothermia, cold-related deaths can be prevented.

 Here we describe effective preventive measures. We also review various types of cold injuries and discuss their treatment.

Hypothermia is defined as a core temperature at or below 35ºC (95ºF).

 Mild: 34ºC to 35ºC (93ºF to 95ºF).

 Moderate: 30ºC to 34ºC (86ºF to 93ºF).

Certain factors--such as age or preexisting medical conditions--increase the risk of hypothermia (

). Hypothermia is not limited to the northern states: other areas of the country with milder climates (the Carolinas and Virginia, for example) have also reported deaths from hypothermia.

 The majority of these deaths occur in November through February.

 Each year, approximately 500 deaths are attributed to hypothermia; about half the victims are 65 years or older and two thirds are male.

 Fortunately, fewer than 20 children younger than 20 years die every year of overexposure to the cold: the vast majority of these deaths are preventable.

 Children at highest risk for death secondary to hypothermia include infants younger than 1 year, boys in early adolescence, and inadequately dressed older adolescents who abuse alcohol or illicit drugs.

The most clinically significant type of localized cold injury in the pediatric population is frostbite, which implies actual freezing of tissue. Like burns, frostbite is rated by degrees (see 

). First degree is the mildest and most superficial type. Fourth-degree frostbite involves the freezing of subcutaneous tissue, muscle, and bone.

The number of cases of localized cold injuries that occur per year nationally is unknown. The American Red Cross predicts that thousands of people can expect to experience frostbite in the winter months.

 The burden of this cold- related problem outside the United States is revealed in a study from Finland: the authors reported that the lifetime and annual incidence of frostbite among military servicemen (some as young as 17) was 44% and 2.2%, respectively.

Human core temperature is normally maintained within 0.6ºC (1ºF). Hypothermia occurs when the body's ability to conserve adequate heat is overwhelmed. Heat is lost via radiation, conduction, convection, and evaporation. The reaction of the hypothalamus to neural feedback from environmental sensations is the key component of temperature regulation. Changes in cutaneous circulation help maintain core body temperature. Radiation or heat transfer from a relatively warm body into a colder environment is responsible for the majority of heat lost. This phenomenon is particularly important in infants and young children because of their greater body surface area-to-mass ratio. When a child is in cold and wet clothing or submerged in cold water (such as in drowning), heat loss is increased through conduction. Through convection (which accounts for the wind chill index), cold wind also increases heat loss. Evaporation is a source of heat loss, particularly for a newborn immediately after birth in the delivery room.

Normally, peripheral vasoconstriction occurs when the ambient temperature is about 15ºC (59ºF).

 Other generalized bodily responses include sweating cessation, shivering, and release of such chemicals as epinephrine and thyroxine to increase heat production. The body's intense response to persistent cold leads to significant adverse effects on the major organ systems (

Peripheral vasoconstriction di- verts blood to the kidneys, which causes an initial diuresis with resultant decreased intravascular volume. Blood flow to the brain is eventually decreased, resulting in CNS depression of various degrees. CNS abnormalities are progressive. Each drop in temperature of 1ºC produces a 6% to 7% decline in cerebral blood flow.

The heart responds with initial tachycardia; eventually, bradycardia and arrhythmias ensue. Apnea, adult respiratory distress syndrome, and aspiration pneumonia are serious pulmonary consequences. The metabolic acidosis, respiratory acidosis, hypokalemia, and hypoglycemia that accompany severe hypothermia wreak havoc on all bodily functions. Coma, cardiac and renal failure, and respiratory arrest are the end points if the hypothermia is untreated.

Localized cold injuries in children result mainly from inadequate protection against the environment. The most peripheral body parts (fingers, nose, ears, toes), which contain abundant arteriovenous anastomoses that shunt blood to the body's core, are the most vulnerable to injury. Cold objects (eg, a popsicle or ice pack) placed against the skin for prolonged periods can also injure the skin (

 Local damage occurs when tissue temperature drops to 0ºC (32ºF) (

A freezing cascade that leads to frostbite has been described

 The first, or prefreeze, phase involves the loss of skin sensation, vascular constriction, and endothelial plasma leakage.

 The development of ice crystals in the extracellular fluid causes water to move out of cells: the result is cell shrinkage and collapse during the second, or freeze-thaw, phase.

 The third, or vascular stasis and progression of ischemia, phase is characterized by endothelial cell damage, necrosis, and sloughing of dead tissue in the affected area.

The stasis and thrombosis that occur in the affected body part provide a conducive environment for gangrene. Sequelae of frostbite depend on the depth of damage sustained. First-degree frostbite can lead to telangiectasias,

 for example, while the most severe types lead to permanent disability (arthritis, premature epiphyseal closure), disfigurement (deformed digits), or amputation of the involved body part.

 A detailed account of the child's symptoms and accompanying environmental conditions usually suggests the diagnosis of hypothermia. Accidental hypothermia may occur indoors in poorly heated homes or outdoors. Those affected may include scantily dressed toddlers who wander outdoors, lost mountain climbers, hikers, backpackers, skiers, and near-drowning victims.

 In the hospital setting, iatrogenic hypothermia may occur during neonatal resuscitations.

The medical history should focus on any underlying medical conditions. When evaluating a cold-injured child, 2 initial steps are crucial:

 Measure vital signs, including the core body temperature with a low-reading thermometer.

Examine the child for clinical features of hypothermia or frostbite. In mild hypothermia, the patient may appear fatigued and display persistent shivering, ataxia, clumsiness, and confusion. Tachypnea and tachycardia are usually present.

ABSTRACT: Hypothermia is not limited to the northern states: people also die of hypothermia in other areas with milder climates. Infants, young adolescent boys, and inadequately dressed teens who abuse alcohol or illicit drugs are at highest risk for death secondary to hypothermia. The mildly hypothermic patient may appear fatigued and display persistent shivering, ataxia, clumsiness, confusion, tachypnea, and tachycardia. The child with moderate hypothermia will not be shivering; declining mental status may cause the freezing patient to remove clothing. An irregular heartbeat is likely at this stage. Severe hypothermia is marked by apnea, stupor, and coma. In a frostbitten patient, rapid rewarming of the affected area in warm water for 15 to 30 minutes is the first step. Potent analgesia is often necessary. After thawing, the frostbitten part is kept dry, warm, and loosely covered. With an adequate dose of common sense, the vast majority of deaths from cold injury can be prevented.

Author | Sharmila Nanda, MD

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