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SPECIAL ARTICLE
Year : 2008  |  Volume : 52  |  Issue : 4  |  Page : 397 Table of Contents     

The Child with Cerebral Palsy and Anaesthesia


1 Hon. Consultant Anaesthesiologist, Apollo Gleneagles Hospital, Kolkata., India
2 Assistant Professor of Anaesthesiology, Medical College & Hospital, Kolkata., India
3 Consultant Anaesthesiologist, Apollo Gleneagles Hospital, Kolkata., India
4 P.G. Student, Apollo Gleneagles Hospital, Kolkata., India

Date of Acceptance30-Jun-2008
Date of Web Publication19-Mar-2010

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A Rudra
1, Shibnarayan Das Lane, Kolkata - 700006
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Cerebral palsy (CP) is the result of an injury to the developing brain during the antenatal, perinatal or postnatal period. Clinical manifestation relate to the areas affected. Patients with CP often present for elective surgical proce­dures to correct various deformities. Anaesthetic concerns of anaesthesia are intraoperative hypothermia , and slow emergence. Suxamethonium does not cause hyperkalaemia in these patients, and a rapid sequence induction may be indicated. Temperature should be monitored and an effort made to keep the patient warm. Cerebral abnormalities may lead to slow awakening; the patient should remain intubated until fully awake and airway reflexes have returned. Pulmonary infection can complicate the postoperative course. Postoperative pain management and the prevention of muscle spasms are important and drugs as baclofen and botulinum toxin are discussed. Epidural analgesia is particu­larly valuable when major orthopaedic procedures are performed.

Keywords: Anaesthesia, Analgesia, Cerebral palsy.


How to cite this article:
Rudra A, Chatterjee S, Sengupta S, Iqbal A, Pal S, Wankhede R. The Child with Cerebral Palsy and Anaesthesia. Indian J Anaesth 2008;52:397

How to cite this URL:
Rudra A, Chatterjee S, Sengupta S, Iqbal A, Pal S, Wankhede R. The Child with Cerebral Palsy and Anaesthesia. Indian J Anaesth [serial online] 2008 [cited 2014 Aug 2];52:397. Available from: http://www.ijaweb.org/text.asp?2008/52/4/397/60653

Cerebral palsy (CP) is a common cause of child­hood disability. It is defined as "a group of nonpro­gressive, but often changing motor impairment syn­dromes secondary to lesions or anomalies of brain aris­ing in early stages of its development" [1] .

It is a static encephalopathy and excludes all pro­gressive neurobiological disorders. Other neurological deficits are frequently associated and they add to the disability caused by the motor deficit.

An understanding of the aetiology, clinical prob­lems and their management, including knowledge of the drugs used in this condition will help the anaesthe­siologist to manage these patients better during anaes­thesia and the perioperative period.


   Etiology Top


Although genetic abnormalities, perinatal an­oxia, infection, and trauma have been proposed as etio­logic factors in CP, no clear simple cause has been iden­tified. However, a notable feature of CP is that infants generally have very low birth wright [2] ; especially birth weight is < 1500 gm [3] . Cerebral palsy in the premature infant is commonly due to periventricular leucomalacia as a result of periventricular haemorrhages. Other hy­potheses in the term infant include antenatal infection, thyroid disease, and neuronal migration disorders. Post­natal causes include meningitis, viral encephalitis, hydrocephalus, and trauma [4] .


   Epidemiology Top


It is the leading cause of childhood motor dis­ability and the prevalence varies from 1.5 to 2.5 per 1000 live births in developed countries. Despite im­proved antenatal and postnatal care, this incidence has not changed in recent years, partly due to increased survival in premature neonates and partly because neo­natal asphyxia is not as common a cause as previously thought. Exact incidence and prevalence figures from our country are not available.


   Classifications Top


Cerebral palsy can be subdivided into several types based on predominant motor pattern into four groups according to symptoms [5] shown in [Table 1].


   Associated developmental disabilities Top


Almost all children with CP will have at least one additional developmental disabilities. Such disabilities include mental retardation, epilepsy, and visual, hear­ing, speech, cognitive, and behavioural abnormalities, as well as the chronic systemic problems resulting from their disease (gastrointestinal, respiratory and ortho­paedic). Generally, the likelihood of such deficits is increased in those with a greater degree of spasticity in­volving a greater number of limbs.


   Diagnosis Top


The diagnosis of CP is essentially clinical. In se­vere and long-standing cases, the diagnosis of CP is not difficult. Diagnosis of mild cases requires experi­ence. Some observations and signs are useful in pre­diction and early identification of CP[Table 2].


   Anaesthesia Top


Patients with CP require special consideration because of their various disabilities. It must not be for­gotten that they have feelings and emotions which are sensitive to the way they are handled and their under­standing may be greater than seems apparent on first meeting. These children often require multiple surgical procedures. Orthopaedic operations to improve func­tion of the extremities are common, and some patients require surgical correction of progressive spinal defor­mities. Common procedures include surgical soft tis­sue procedures that reduce muscle spasm around the hip girdle, including an adductor tenotomy or psoas transfer and release.


   Preoperative assessment Top


Thorough preoperative preparation is important in ensuring an uneventful perioperative course. These children are ideal for assessment by a multidisciplinary team which include anaesthesiologists. A complete his­tory and physical examination is essential before an­aesthetizing these children, including a discussion of postoperative pain management with the parents. Cognitive and communication problems may make preop­erative assessment more difficult. Parents and caretak­ers are best to be involved during this period not only to gain information but also allay the fears of the child. The following systems may be involved and should be considered preoperatively:


   Gastrointestinal Top


Gastroesophageal reflux is common and may cause chronic respiratory problems. Esophagitis is not uncommon and night wakening may indicate reflux or spasm. Patients may present for Nissen fundoplication, gastrostomy, or esophageal dilatation for strictures. Children who have CP have an impaired ability to handle pharyngeal secretions, and pooling of secretions in the oropharyngeal area is common. The etiology of in­creased salivation is caused by hyperactive salivary glands and impaired cranial nerve function [6] . Some patients may have already been placed on antisialogogue; the most common agent used is the anticholinergic, glycopyrrolate. It is necessary to suction the orophar­ynx immediately following induction of anaesthesia (or even before). Anticholinergics given intravenously, once this access is secured, will aid in minimizing this prob­lem.


   Respiratory Top


Respiratory disorders are usually a common cause of death. Recurrent pneumonias, pulmonary aspiration and chronic lung disease are aggravated by the inability to cough and poor nutritional state which decreases immune response. Respiratory pathology of some sig­nificance may go unnoticed in case where communica­tion is poor and exercise tolerance test cannot be prop­erly assessed because of neurological impairment.

Scoliosis may compound the problem with a re­strictive lung pattern. Noisy breathing may be indica­tive of upper airway obstruction which may worsen on induction or postoperatively.

The airway should be assessed for potential difficult laryngoscopy because of dental caries, loose teeth, and an increased incidence of temporomandibu­lar joint dysfunction [7] .

Following intubation of the trachea, suctioning via an endotracheal tube is a good practice in children with increased secretions, especially if rhonchi or expi­ratory wheeze is present. Often breath sounds will clear using this simple maneuver.


   Epilepsy Top


Epilepsy occurs in 30% of patients with CP. It is most common in spastic hemiplegia and least common in ataxic and choreoathetoid forms [8] . Anticonvulsant therapy should be continued including day of surgery, and also postoperatively and should be administered per schedule.


   Visual and hearing deficits Top


Visual and hearing defects are present in 40% of CP patients including strabismus, myopia, retinopathy of prematurity and cortical blindness.


   Intellectual disability Top


Intellectual disability occurs in two-third of pa­tients with CP and is severe in 50% of those affected. Sixty percent of children with hemiplegia will have nor­mal intelligence but it is present in less than 30% with spastic quadriplegia. Depression and emotional liabil­ity are common problems in adolescence.


   Contracture Top


Contractures may make positioning and intrave­nous access difficult.


   Medical therapy Top


Baclofen, is a GABA receptor agonist with ac­tion in the dorsal horn of the spinal cord. It is com­monly given orally to reduce muscle spasms and the associated pain and may delay the development of contractures. It can also be administered intrathecally via an implantable pump, allowing a lower dose and fewer side effects [9] . Baclofen is rarely implicated in de­layed awakening, bradycardia or hypotension during general anaesthesia. Abrupt withdrawal can result in seizures, hallucinations, itching, and disorientation last­ing up to 72 hours [9] . Side effects such as urinary reten­tion and hypotension may respond to reducing the dose. Pump malfunction must be considered if overdose is suspected and intravenous flumazenil and physostig­mine along with respiratory support and removal of cerebrospinal fluid via lumbar puncture may be needed.

Botulinum neurotoxin (Botox), is indicated when spasticity is interfering with function. It produce revers­ible muscle denervation and temporary reduction in muscle tone. Generalized weakness from systemic tox­icity is rare. Lower extremity injections have been given in hamstrings or gastrocnemius muscle [10] . The onset of effect is 12 hours to 7 days with effect for 2 to 6 months. Botox injections are performed under general anaes­thesia or in the older child with EMLA. It can also de­crease pain scores and muscle spasm postoperatively when given prior to adductor tendon release surgery of the hip. Potentiation of muscle relaxants has not been substantiated clinically [11] .


   Preoperative sedation and preparation Top


Sedation may be valuable to decrease anxiety but children with poor pharyngeal coordination may need special precautions. Loss of airway tone and increased risk of aspiration is greater in hypotonic children. Be­fore preoperative sedation is ordered, parents should be consulted to learn how well the child previously tol­erated. However, most patient will handle premedica­tion without incident, and reducing the dose may be more appropriate than omitting premedication alto­gether. If obtaining intravenous access is necessary before induction, then use of EMLA cream (lidocaine 2.5% and prilocaine 2.5%) can be helpful. Often, a combination of EMLA cream, nitrous oxide (50%­70%) with oxygen, and preoperative sedation will help in facilitating intravenous access. If premedication is chosen, oral midazolam 0.25-1 mg.kg -1 or ketamine 3-6 mg.kg -1 with glycopyrrolate 0.02 mg.kg -1 may be considered. Antacids, antiemetics and drugs to reduce secretions may also be indicated. The extremities of these children are often cold and vasoconstricted. Use of a warming device, such as warm towels, will aid in vasodilatation and make it easier and quicker to ac­cess a peripheral vein. Latex allergy is more frequent in these children [12] especially if they have had multiple op­erations. A history of such sensitivity should be sought in all patients with CP.


   Intraoperative management Top


Careful positioning is paramount in the child with spastic CP to avoid nerve and muscle damage. Fixed contracture may add to difficulty in positioning.

Propofol for intravenous induction is a good choice since many children with CP have reactive air­way disease and propofol unlike thiopentone decreases the airway tone [13] .

Tracheal intubation should be performed if this is a concern or if there is a history of gastroesophageal reflux. Children with CP are often small for their age. Tracheal tube size selection should be based on their age as this usually provides the most appropriate fit [4] . If a rapid sequence induction is considered necessary, poor cooperation may allow only a 'modified' tech­nique is best. The risk between securing an airway rap­idly and a slower inhalational induction in an uncoop­erative child must be balanced.

Drug responses may vary from the normal re­sponse in the child with CP. There may be resistance to non depolarizing muscle relaxants [14] , although this is of doubtful clinical significance. Sensitivity to suxam­ethonium probably does not occur [15] . Train-of-four re­sponses in a severely affected limb may not represent the actual state of neuromuscular blockade [16] and the degree of block can be underestimated.

Mean alveolar concentration (MAC) of halothane are lower in children with CP when compared with normal children [17] . The MAC of halothane is 0.9 in healthy children, in children who have CP, it is 0.71. It is interesting to note that children who have CP and who were receiving anticonvulsants had an even lower MAC; 0.63. Similarly, anaesthetic agents lead to a greater hypnotic state in these children as evidenced by lower bispectral index score (BIS) when exposed to similar concentrations of sevoflurane and compared with patients who are otherwise normal [18] .

Hypothermia is a problem due to hypothalamic dysfunction and the malnourished child. Often, these children will come to the operating room with border­line hypothermia (<35.0 0 C). Measures to conserve tem­perature should include warming of intravenous and ir­rigating fluids plus warming blankets or forced air warm­ers. Otherwise, it is likely that 1-2 0 C could be lost before the patient is prepared and draped.

Combined general and regional anaesthetic tech­niques are highly recommended especially in lower limb surgery or dorsal rhizotomy where patients are sus­ceptible to severe postoperative muscle spasms. In patients undergoing infraumbilical orthopaedic proce­dures or Nissen fundoplication, a continuous epidural infusion of 0.2-0.5 ml.kg -1 .hr -1 of 0.1% bupivacaine with fentanyl 2 mcg.ml -1 for 72 hours provided satisfactory pain control [19] . However, due to high incidence of nau­sea and vomiting with opioid, use of bupivacaine 0.125% with clonidine 2.5 mcg.ml -1 at 0.2-0.3 ml.kg­1 .hr -1 provide better analgesia, less muscle spasm and vomiting [4] . Clonidine may also decrease spasticity. In clinical practice, narcotics appear to have greater potency in children who have CP. Doses need to be re­duced, and greater vigilance is necessary to ensure maintenance of a patent airway post extubation.

Patients undergoing scoliosis surgery may have a higher volume of blood loss than idiopathic scoliotic patients, especially if anterior and posterior procedures are performed [20] . More segments are usually involved with the intramuscular scoliotic fusion procedures [21] . A recent report shows that cerebral palsy patients may have abnormalities of coagulation factor levels. Plate­lets may be borderline low because of prolonged use of anticonvulsants and their function may also be af­fected by anticonvulsants [22],[23] .


   Postoperative care Top


The immediate postoperative period can present specific problems in the recovery room for chil­dren suffering from CP.

  • Emergence from anaesthesia may be delayed due to hypothermia. Delayed emergence may also be due to residual volatile anaesthetic agents. MAC is reduced relative to normal controls and it is conceivable that MAC awake is also reduced.
  • Drooling, if present preoperatively, require frequent suctioning for maintenance of airway. Moreover, care­ful attention is needed to protecting the airway in chil­dren with pseudobulbar palsy from regurgitation of gas­tric fluids and vomiting.
  • Irritability on emergence from anaesthesia is not uncommon. This may be due to pain, urinary retention or being in an unfamiliar environment. Parental pres­ence in the recovery room can help with the latter.
  • Children, who suffer from epilepsy may be unable to have orally administered anticonvulsants if they are nil by mouth or experiencing postoperative nausea and vomiting. Most anticonvulsants, however, have long elimination half-lives (24-36 h) and, if their levels are within the therapeutic range, a 24 hour period can elapse without significantly increasing the risk of seizures.
  • Those with poor cough, recurrent respiratory in­fections, and impaired clearance of secretions will ben­efit most from postoperative chest physiotherapy.
  • Contractures can cause difficulties with position­ing. If epidural analgesia is used for postoperative pain management, prolonged pressure over bony promi­nences may lead to troublesome skin breakdown, es­pecially in malnourished children.
  • Children with CP are prone to constipation. Oral, intravenous, epidural opioids may compound this prob­lem. Therefore, attention to normal bowel habits for that child and the prescription of laxatives, supplemented by enemas, may be necessary.



   Postoperative pain management Top


The surgical procedures, particularly those for relieving spasticity, are extremely painful. Therefore, adequate pain relief is important in the postoperative care of cerebral palsy patients. Moreover, assessment of pain in these children is very difficult because of con­ceptual problems associated with intellectual disability or poor verbal communication skills. Behavioural indi­cators such as facial grimacing, groaning, moaning or altered sleep patterns may be present in the non-pain state and can be inconsistent and difficult to interpret. Moreover, postsurgical pain must be differentiated from hunger, anxiety, discomfort due to positioning and other benign causes, e.g., headache toothache, otitis media, and menstrual pain in females.

Postoperative pain following extensive lower limb orthopedic surgery is treated in these children with con­tinuous epidural (caudal or lumber) infusions with [16] :

  • Lidocaine (1.5 to 2 mg.kg -1 .hr -1 ) plus fentanyl (0.5 mcg.kg-1.hr-1).
  • Bupivacaine (0.625 to 1 mg.ml -1 .hr -1 ) with or with­out fentanyl (0.5 mcg.kg-1.hr-1).
  • Hydromorphone (2 to 4 mcg.kg -1 .hr -1 )


Muscle spasms are virtually universal and are treated prophylactically with intravenous diazepam 0.1- 0.2 mg.kg -1 or addition of clonidine to the postopera­tive epidural infusion at a dose of 0.08 to 0.12 mcg.kg­1. hr -1 . [16]

Patients with CP who undergo extensive surgical procedures that necessitate analgesic infusions should be monitored as far any child receiving analgesia by the same route.


   Conclusion Top


Cerebral palsy may be due to antenatal, perinatal or postnatal causes. Knowledge of pathology associ­ated with different types of CP, related conditions and their drug treatment will enable the anaesthesiologist to predict likely problems which might impinge on anaes­thesia and the perioperative period. The choice of an­aesthetic drugs should be tailored to the individual need and surgical regiment. Severely affected children may have gastroesophageal reflux and problems with pha­ryngeal coordination. This may make them vulnerable to aspiration during induction and extubation, and should mandate a cautious approach to these potentially anaemic, malnourished children. Suxamethonium does not cause hyperkalaemia in the children with CP. The response to nondepolarizing muscle relaxants appears to be normal. Careful postoperative care should be provided to adequately maintain the vitals.

Postoperative pain management and the preven­tion of muscle spasms are important with drugs such as baclofen and botulinum toxins. Epidural analgesia is particularly valuable when major orthopaedic proce­dures are performed.

 
   References Top

1.Salem MR, Klowden AJ. Anesthesia for orthopedic surgery. In : Pediatric Anesthesia. Gregory GA (editor) 4th edition, Churchill Liviingstone 2002 ; p. 65A.  Back to cited text no. 1      
2.Kuban KC, Leviton A. Cerebral palsy. N Engl J Med 1994 ; 330 : 188-95.  Back to cited text no. 2      
3.Inder TE, Warfield SK, Wang H, et al. Abnormal cerebral structure is present at term in premature infants. Pediat­rics 2005; 115 : 286-94.  Back to cited text no. 3      
4.Nolan J, Chalkiodis GA, Low J, Olesch CA, Brown TCK. Anaesthesia and pain management in cerebral palsy. Anaesthesia 2000; 55 : 32-41.  Back to cited text no. 4      
5.Mutch L, Alberman E, Hagberg B, Kodama K, Perat M. Cerebral palsy epidemiology where are we now and where are we going ? Developmental Medicine and child Neurology 1992 ; 34 : 547-51.  Back to cited text no. 5      
6.Reddihough D, Johnson H, Staples H, Hudson I, Exarchos H. Use of benzhexol hydrochloride to control drooling of children with cerebral palsy. Developmental Medicine and child Neurology 1990 ; 32 : 985-9.  Back to cited text no. 6      
7.Pelegano JP, Nowysz S, Geopferd S. Temperomandibular joint contracture, in spastic quadriplegia : effect on oral - motor skills. Developmental Medicine and child Neu­rology 1994;36:487-94.  Back to cited text no. 7      
8.Eicher PS. Batshaw ML. Cerebral palsy. Pediatr Clin N Am 1993;40:537-51.  Back to cited text no. 8      
9.Albright AL. Intrathecal baclofen in cerebral palsy move­ment disorders. J Child Neurol 1996;11:S 29-35.  Back to cited text no. 9      
10.Forssberg H, Tendorff KB, Botulinum toxin treatment in cerebral palsy : intervention with poor evaluatio? De­velopmental Medicine Child Neurology 1997;39:635-­40.  Back to cited text no. 10      
11.Fiacchino F, Grandi L, Soliveri P, Carella F, Bricchi M. Sensitivity to vecuronium after botulinum toxin admin­istration. J. Neurosurg Anesthesiol 1997;9:149-53.  Back to cited text no. 11      
12.Delfico AJ, Dormans JP, Craythorne CB, Templeton JJ. Intraoperative anaphylaxis due to allergy to latex in children who have cerebral palsy : a report of six cases. Developmental Medicine and Child Neurology 1996; 39 : 194-7.  Back to cited text no. 12      
13.Shapiro JM, Management of respiratory failure in sta­tus asthmaticus. Am J Resp Med 2002;1:409-16.  Back to cited text no. 13      
14.Moorthy SS, Krishna G, Dierdorf SF. Resistance to vecuronium in patients with cerebral palsy. Anesth Analg 1991;73:275-7.  Back to cited text no. 14      
15.Dierdorf SF, McNiece WL, Rao CC, et al. Effect of succi­nylcholine on plasma potassium in children with cere­bral palsy. Anesthesiology 1985;62:88-90.  Back to cited text no. 15      
16.Zuckerberg AL, Yaster M. Anesthesia for orthopaedic surgery. In : Motoyama EK, Davis PJ,(editors). Smith's Anesthesia for Infants and Children. St. Louis, MO : CV Mosby, 2006 ; pp. 756-8.  Back to cited text no. 16      
17.Frei FJ, Haemmerle MH, Brunner R, Kern C. Minimum alveolar concentration for halothane in children with cerebral palsy and severe mental retardation. Anaes­thesia 1997 ; 52 : 1056-60.  Back to cited text no. 17      
18.Choudhury DK, Brenn BR. Bispectral index monitoring : a comparison between normal children with quadriple­gic cerebral palsy. Anesth Analg 2002; 95 :1582-5.  Back to cited text no. 18      
19.Bren BR, Rose JB. Epidural analgesia in children with cerebral palsy. Can J Anaesth 1998; 45 : 1156-61.  Back to cited text no. 19      
20.Bobechko WP. Blood loss in scoliosis surgery. Spine 1991 ; 5 : 75-82.  Back to cited text no. 20      
21.Brenn BR, Theroux MC, Dabney KW, et al. Clotting parmeters and thromboelastography in children with neuromuscular and idiopathic scoliosis undergoing pos­terior spinal fusion. Spine 2004;29:310-4.  Back to cited text no. 21      
22.Sherard Jr ES, Couri D. Treatment of childhood epilepsy with valproic acid results of the first 100 patients in a six-month trial. Neurology 1980; 30 : 31-5.  Back to cited text no. 22      
23.Kreuz W, Funk M, Meyer-Schrod R, et al. Valproate therapy induces von Willebrand disease type 1. Epilepsia 1992; 33 : 178-84.  Back to cited text no. 23      



 
 
    Tables

  [Table 1], [Table 2]



 

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  In this article
    Abstract
    Etiology
    Epidemiology
    Classifications
    Associated devel...
    Diagnosis
    Anaesthesia
    Preoperative ass...
    Gastrointestinal
    Respiratory
    Epilepsy
    Visual and heari...
    Intellectual dis...
    Contracture
    Medical therapy
    Preoperative sed...
    Intraoperative m...
    Postoperative care
    Postoperative pa...
    Conclusion
    References
    Article Tables

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