Role of Anesthesiologist in the Management of a Child with Cerebral Palsy.

Cerebral palsy (CP) refers to a spectrum of nonprogressive neurological disorders with disturbances in posture and movement, resulting from perinatal intrauterine insult to developing infant brain. Many conditions associated with CP require surgery. Such cases pose important gastrointestinal, respiratory, and other perioperative considerations. Anesthetic management in these cases is delicate. Intraoperative complications including hypovolemia, hypothermia, muscle spasms, seizures, and delayed recovery might complicate the anesthetic management. A thorough preanesthetic evaluation allows for a better intra- and post-operative care. Postoperative analgesia is important, particularly in orthopedic surgeries one for pain relief. This review highlights the clinical manifestations in CP and anesthetic considerations in such child presenting for various surgeries.

INTRODUCTION

Cerebral palsy (CP) is a common cause of morbidity and disability in childhood. CP is a heterogeneous group of chronic nonprogressive disorders of motor development and posture in children that are associated with cognitive and neurosensory disturbances.[12]

The prevalence in the general population in developed world is about 1:500 live births.[23]

Most cases of CP arise from intrauterine – antepartum causes. Birth asphyxia, perinatal ischemia and hypoxia, congenital malformations, intraventricular hemorrhages, intrauterine infections – all contribute to the development of CP.[456789101112]

A child with CP can present for surgery, and anesthetic management is challenging because of the involvement of various organ systems and associated complications. Central nervous system is often involved, and they can present with varying degrees of movement disorders as described below.

CLASSIFICATION

Swedish classification shown below is the most widely used as it addresses both movement disorder as well as its distribution.[4891013]

Spastic CP (70%):

Quadriplegia

Diplegia

Hemiplegia.

Dyskinetic CP (10%):

Dystonia

Athetosis

Chorea.

Ataxic CP (10%):

Intention tremor and head tremor (titubation).

Mixed (10%):

For example, spastic athetosis.

PATHOPHYSIOLOGY AND CLINICAL MANIFESTATIONS[91314]

Central nervous system

The degree of disability in CP depends on the area of the brain affected. Involvement of motor cortex leads to spasticity; abnormalities of basal ganglia cause athetosis and cerebellar defects result in ataxia. Spasticity can lead to contractures, spine deformities, and chronic pain. Congenital talipes equinovarus is often associated with CP

Seizures are seen in almost a third of CP cases, most common in spastic CP. Visual and hearing impairments are often seen

Visual impairments include:

Retinopathy of prematurity

Strabismus

Visual field defects

Myopia

Amblyopia

Cortical blindness.

Psychological

Intellectual disability is observed in two-thirds of patients with CP

Attention deficit hyperactivity disorder, self-injurious behavior, depression, and emotional lability are commonly observed.

Gastrointestinal system

In CP, along with neurological symptoms, involvement of gastrointestinal and respiratory systems is common

Gastroesophageal reflux (GOR) is common in these children and they often present for Nissen fundoplication. Nasogastric feeding or gastrostomy may be needed[15]

The ability to handle pharyngeal secretions and pooling of secretions in oropharyngeal area is common. The etiology of increased salivation is hyperactive salivary glands, tongue thrusting, or impaired cranial nerve function due to pseudobulbar palsy causing impaired swallowing[16]

Reflux is worsened by poor head control.[17]

Respiratory system

Chronic aspiration and lack of an effective cough reflex are often encountered

Recurrent pneumonia and reactive airway disease are results of such chronic insults; chronic aspiration can even cause death in some cases

Eliciting the history of allergies, especially latex allergy is important during preoperative workup. Latex allergy has been earlier reported in patients with CP.

Cardiovascular system

Chronic respiratory system involvement can lead to pulmonary hypertension, right ventricular hypertrophy, or cardiac failure.

MEDICAL MANAGEMENT OF CEREBRAL PALSY[48]

Children with CP are often on treatment with multiple medications for associated conditions. In the preoperative history, all current medications should be recorded, including anticonvulsants, antispasticity, and antireflux medications[13]

Medical therapy for controlling seizures, spasticity, reflux, constipation, and depression should be well understood, in particular with respect to side effects and potential interactions

Carbamazepine and sodium valproate are prescribed for seizure management. While carbamazepine can cause induction of hepatic enzymes, leading to reduced duration of action of vecuronium, sodium valproate is known to cause impaired platelet function, increased risk of bleeding, hepatotoxicity, and bone marrow suppression

Medications commonly used for spasticity include - diazepam, baclofen, tizanidine, dantrolene sodium, and botulinum toxin

Benzodiazepines cause sedation, respiratory depression, and dependence. Common problems reported with baclofen are muscle weakness, malaise, nausea, diarrhea, skin rash, and abnormal liver function tests (LFTs). Both baclofen and dantrolene are known to complicate the resumption of adequate respiratory effort during emergence from anesthesia. Tizanidine can cause dry mouth, muscle weakness, reversible abnormalities in LFTs, and hypotension. Drug-induced hepatitis and hallucinations are rare, serious side-effects[18]

Botulinum toxin causes dose-dependent, reversible muscle denervation, muscle atrophy if given long-term. It also leads to fatigue, transient weakness of the injected muscle. Generalized weakness or systemic toxicity is rare. May potentiate the action of muscle relaxants (not proven clinically).

COMMON SURGERIES IN CEREBRAL PALSY[489]

Children with CP can present for many interventional diagnostic and surgical procedures. A detailed knowledge of these procedures is essential for the treating anesthesiologist. Commonly performed surgical interventions in these children include:

Tracheostomy

Dental surgeries

Orthopedic surgeries – rhizotomy-contracture release, tendon lengthening, and scoliosis correction

Diagnostic endoscopy

Nissen fundoplication

Feeding gastrostomy.

ANESTHETIC IMPLICATIONS AND SURGICAL MANAGEMENT

Perioperative anesthetic implications in child with CP vary with the severity of the disease as well as associated medical conditions

Spasticity is a crippling feature of CP. Children are often on antispastic mediations preoperatively. Baclofen should not be withheld abruptly. Seizures are common. It is important to keep a detailed account of ongoing anticonvulsant medications during preanesthetic work-up and to continue these anticonvulsants perioperatively

Antireflux medications for GOR must be continued perioperatively. Increased oropharyngeal secretions can contribute to increased the risk of aspiration, recurrent pneumonia, and reactive airway disease in these children. To minimize this problem, anticholinergics must be given intravenously once this access is secured. It is necessary to suction the oropharynx immediately following anesthesia. Some patients may have already been started on anti-sialogogue such as glycopyrrolate preoperatively itself

Airway assessment must focus on possible difficult laryngoscopy due to abnormal dentition, temperomandibular joint dysfunction, and positioning difficulties. The previous anesthetic records can be of help in airway management

Care must be taken while positioning to prevent pressure sores, especially during prolonged orthopedic surgeries

Preoperative anxiety needs to be well managed as many of these children may not communicate effectively or may have undergone multiple surgeries. Premedication with oral or intravenous midazolam is appropriate in such cases

Minimum alveolar concentration (MAC) is reduced for inhalational agents. MAC of halothane has been shown to be 20% lower in children with CP and a further 10% lower in those currently taking anticonvulsants. There is resistance to nondepolarizing muscle relaxants, attributable due to drug interaction with anticonvulsants, and chronic immobilization[1920212223]

Children with CP are more prone to develop hypothermia perioperatively. Hypothermia can be precipitated by decreased circulatory volume, increased blood loss during surgery

Perioperative chest physiotherapy will help those with poor cough, poor secretion clearance, and recurrent chest infections

Judicious postoperative suctioning helps in clearing the pooled secretions

Patients with poor respiratory function might require postoperative ventilation and even tracheostomy during weaning

Meticulous care must be taken in positioning the patient during regional anesthesia, to overcome difficulties caused by contractures, and spine deformities such as scoliosis

Important anesthetic implications and management strategies are summarized in Table 1.

Table 1

Important anesthetic considerations and management strategies in a pediatric patient with cerebral palsy for surgery

ANESTHESIA FOR ORTHOPEDIC PROCEDURES INCLUDING SCOLIOSIS

Most of the orthopedic surgeries performed in children with CP are to treat the chronic effects of increased tone on muscles and bones of extremities and spine. Commonly performed surgeries include soft-tissue release surgeries, tendon transfers, osteotomies, tenotomies, and scoliosis correction

Usually, the major surgical procedures are done under combined general anesthesia (GA) and regional analgesia[24]

In pediatric patients, it is common practice to administer caudal epidural analgesia along with GA to decrease intraoperative inhalational anesthetic requirements and postoperative pain[41325]

Epidural analgesia, in particular, continued into the postoperative period will diminish both postoperative pain and muscle spasm

GA

Standard monitoring along with temperature monitoring and neuromuscular monitoring are important. Monitoring the depth of anesthesia is helpful as the MAC value is varied

Induction of anesthesia may be challenging. This is mainly attributable to intellectual disability causing lack of cooperation, communication difficulties, and difficult intravenous access. This is particularly so in those children who have previously been in special care baby units or neonatal intensive care; who may have previously used and scarred veins. The presence of an assistant and topical application of EMLA cream is often helpful to establish vascular access. If intravenous induction of anesthesia is planned, all commonly used agents are suitable, although propofol appears to be more painful in these patients

Airway management in the presence of increased incidence of GOR in this group of patients varies

Many anesthesiologists choose a laryngeal mask airway when clinically appropriate in conjunction with a nasal or oral gastric tube aspirated and left on free drainage; others chose a tracheal tube routinely

GA is indicated for scoliosis correction. Surgery for scoliosis is associated with deterioration in the respiratory function. Preoperative respiratory function tests are unreliable and not predictive of outcome. Postoperative nasal continuous positive airway pressure may help to prevent respiratory complications. Occasionally, the patient may require long-term ventilation; tracheostomy can be considered during weaning[4]

Reduced MAC and hypothermia contribute to emergence-related problems.

Regional anesthesia

Regional techniques are preferred for intra- and post-operative analgesia in most major procedures

Epidural analgesia is preferred neuraxial technique for lower limb surgery

Epidural catheter insertion requires an experienced anesthesiologist as associated scoliosis and prior back surgery may make insertion challenging

Any regional infusion must be carefully monitored by an experienced pain management team and well-trained nursing staff

Postoperative pain management[14]

Postoperative pain following lower limb orthopedic surgery is managed in these children with continuous epidural infusions (caudal or lumbar):

Lidocaine (1.5–2 mg/kg/h) plus fentanyl (0.5 mcg/kg/h)

Bupivacaine (0.625–1 mg/ml/h) with or without fentanyl (0.5 mcg/kg/h)

Hydromorphone (2–4 mcg/kg/h).

Muscle spasms are virtually universal and are treated prophylactically with intravenous diazepam 0.1–0.2 mg/kg or addition of clonidine to the postoperative epidural infusion at a dose of 0.08–0.12 mcg/kg/h.

ANESTHESIA FOR ANTIREFLUX SURGERY

Children with CP often present for antireflux surgeries, mainly Nissen fundoplication

Compared to laparoscopic technique, the open technique is associated with significant morbidity; hence, laparoscopic methods are preferred over open techniques in CP

GA is the choice of anesthesia for these surgeries. Anesthetic management here is same as described earlier

The studies have shown that in laparoscopic Nissen fundoplication, children are well managed with oral and rectal analgesia postoperatively. The need for postoperative high dependency and intensive care appears to be minimal.[26] Epidural analgesia may help in the prevention of painful spasms postoperatively.

ANESTHESIA FOR DENTAL PROCEDURES[2728]

GA is preferred for young patients who require extensive dental treatment and who do not achieve desired results with the use of local anesthesia and behavior management

Patients with a physical/psychological disability, characterized by poor oral hygiene, soft and cariogenic diets, periodontal disease, and polypharmacy will require GA

Medically compromised patients with an underlying disease, must undergo surgery under GA so they may be monitored better

Intraoperative management remains same as that for other cases under GA

Special attention must be provided to tackle problems that may arise due to defective dentition, intraoperative bleeding as well as pooled secretions

Effort should be made to motivate CP patients and their parents to visit the dentist and receive primary prevention. There should be special programs that enhance communication between pediatric dentists and pediatricians to prevent these dental problems

Oral health problems can be prevented by programs designed to enhance communication between the pediatrician and pediatric dentist.

CONCLUSION

The management of CP is multimodal one. The treatment in these cases demands a team therapy by psychotherapy, physiotherapy, medical, and surgical approaches. Considering the ethical aspects, the primary goal of such specialized care is mainly to improve the quality of life and the quality of care. Anesthesiologist comes into picture during the perioperative management, in high-dependency care and intensive care as well as pain management. Recent advances in treatment modalities and surgical techniques along with improved postoperative care have significantly reduced the morbidity. A successful management in CP is done by a team of experienced specialists as well as psychological support by caregivers.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.