The perioperative management of pediatric patients with psycho-physical disorders with related relational and cognitive problems must be carefully planned, in order to make the entire hospitalization process as comfortable and as less traumatic as possible.
1. Introduction
International scientific literature defines pediatric patients with “special needs” (SN) as children suffering from psycho-physical disorders with related relational and cognitive problems
[1][2].
The perioperative management of these pediatric patients must be carefully planned, in order to make the entire hospitalization process as comfortable and as least traumatic as possible
[3][4].
For pediatric patients with special needs, the standard perioperative path is inapplicable, mainly due to the surgical and anesthetic criticalities
[5]. These patients have a much-increased perioperative risk due to their anatomical features, underlying disease and the coexistence of complex comorbidities
[6].
2. Perioperative Anesthetic Phases
Table 1 summarizes the anesthetic phases in the management of the pediatric patient with Special Needs (SN).
Table 1. Anesthetic phases.
2.1. Preoperative Anesthetic Visit
During this stage, it is essential to identify the caregiver figure (CG) or the leading parent (LP). The first contact with the CG or LP can be by phone or at the pre-operative assessment service.
The non-cooperative patient with SN (difficult access to hospital, difficulty or inability to perform electrocardiogram and/or blood tests, inability to perform physical examination and evaluation of predictive indices of difficulty in managing airways) must be examined, after parents’ agreement, with the mediation of the surgeon
[7][8].
In the absence of important comorbidities of the anesthetic interest, preoperative examinations can be performed after the induction of general anesthesia. If possible, in case of comorbidities, a recent anamnestic documentation, not exceeding two years, is required, including the documentation relating to routine blood chemistry tests, the list and dosage of drugs in use (e.g., lithium, anticonvulsants, antipsychotics), instrumental examinations (chest X-ray, brain MRI, EEG) in possession of the parents/guardians
[9].
It is important to underline that prolonged administration of antiepileptic drugs is associated with several drug interactions, due to multiple factors operating alone or in combination. Specifically for anesthesia and critical care, sensitivity and resistance to non-depolarizing neuromuscular blockers (NDNMBs) after acute and long-term administration of antiepileptic drugs, have been well described. This can result from hepatic drug metabolism induction, increased protein binding of the NDNMBs and/or upregulation of acetylcholine receptors. A neuromuscular blocking enhancement due to pre- and post-junctional direct effects can occur after an acute administration of antiepileptic drugs
[10].
Patient documentation must be requested at the time of the first telephone contact with the CG or LP. It is often difficult to access the hospital, perform the electrocardiogram, blood tests and a physical examination, the evaluation of predictive indices of difficulty in ventilation/intubation which facilitate a safe and provident management of the airways. The anesthetist, therefore, is at a potential risk of difficult intubation and/or ventilation due to anatomical maxillofacial and upper airway malformations which are often present. The preoperative evaluation must be limited to the collection of the pathological anamnesis with the help of the LP or the CG. The pathological history allows for the identification of comorbidities (cardiological, respiratory, metabolic, neurological) and anesthetic risk factors (home therapy, airway management, myopathies, epilepsy, severe cervical and dorsal-lumbar spine deformities); the vision of any previously requested documentation and the home therapy (drug interactions with anesthesia drugs)
[11]. A form with an accurate drug history must be included in the anesthetic record chart. It allows for the avoidance of drug interactions that could interfere with all stages of anesthesia and in particular, with the patient’s awakening. Difficulty in finding a venous access should be noted. It is essential that the surgeon and the anesthesiologist have already discussed each individual case at this stage in order to plan the surgery and prepare special measures. The LP or the CG may also, at this stage, indicate habits, special needs and any alternative communication channels used by the patient (specifically relating to the communication of discomfort, agitation, fear, pain or a state of well-being mostly useful in the awakening phase). The LP or CG play an important role in mediating relationships with the patient and they help to build an empathic relationship between patient and anesthetist. Familiarization with the healthcare environment is another important element, however, it requires more than just one meeting and this is not always possible. If possible, it is advisable to fill in, with the help of the LP or CG, an additional preoperative evaluation form that helps to understand the particular needs and habits of each patient.
2.2. Establishment of a Dedicated Surgical Session and Hospitalization
It is essential to establish a fixed day for the operating session in order to organize the procedure
[12]. In the same way, any change in the day dedicated to the operating session must be communicated at least one month before. The nursing, auxiliary and medical staff must be fully dedicated to the activity carried out in the ward and in the operating room and they should be adequately trained
[13]. For each session, an operating list that respects the time assigned to that session must be prepared, compiled by evaluating the surgical and anesthetic times required for each procedure and based on the complexity of the patient
[14].
2.3. Preparation for Admission
All patients should be hospitalized on the same day of the surgery, in a reserved room in the surgical department, in order to facilitate treatment and assistance by the medical and the nursing staff dedicated to them. Admissions should be according to the order of the list and to the times provided for each procedure to avoid excessive hospitalization time, which leads to lack of care and stress for the patient
[15].
2.4. The Anesthetic Premedication
Hospitalization and surgery can cause significant stress and anxiety in children. Induction of anesthesia may be the most exhausting procedure that a child experiences during the entire perioperative period
[16]. Premedication is recommended. The premedication should be chosen on the basis of personal preferences because there is no sufficient literature to be able to choose based on evidence. Adequate preoperative sedation allows these patients and their families a more serene hospitalization. The main purpose of premedication in the pediatric patient, especially in the patient with SN, is anxiolysis which can facilitate separation from the LP or CG and make the induction of anesthesia easier. In addition, adequate premedication includes: amnesia, the prevention of physiological stress, the reduction in the demand for anesthetics, the antiemetic effect, vagolysis, reduction in secretions and analgesia. It is advisable during the anesthetic visit to establish with the LP or the CG the methods of administration of the pre-anesthetic drug: the type, the dosage, the route of administration, the time of administration, and the titration of the drug, in order to achieve a degree of sedation that allows for venous cannulation and intravenous or inhalation induction. In these patients, the induction of intravenous anesthesia is not always possible. It’s often administered the “steal inhalational induction”. This induction involves the arrival of the patient in the operating room moderately sedated thanks to anesthetic premedication
[17]. The drugs currently used are listed in
Table 8. The following are considered first choice drugs: midazolam, clonidine and dexmedetomidine; ketamine is a second-choice drug.
Table 8. Commonly used medications for premedication in children.
All drugs used may produce sedation and respiratory depression and should always be administered under supervision and monitoring. Devices for supplemental oxygen administration and support for ventilation and resuscitation should be readily available. Inhalation of the nebulized drug is an alternative method of administration that is relatively easy to set up, does not require venous access and is associated with a high bioavailability of the drug employed
[20]. Abdel Ghaffar observed that children premedicated with nebulized inhaled dexmedetomidine (2 μg/kg) have more satisfactory sedation scores, greater mask acceptance and shorter recovery times than who received nebulized ketamine (2 mg/kg)
[21] or midazolam (0.2 mg/kg)
[22]. Premedication with dexmedetomidine reduces the incidence of postoperative psychomotor agitation.
2.5. Awakening and Postoperative Management
Anti-epileptic drugs cause a prolonged awakening time from anesthesia, but these drugs should not be discontinued during the preoperative in order to reduce perioperative seizures
[7][23][24][25].
In Higuchi’s study, the awakening time in patients with intellectual disabilities is significantly longer than in patients without intellectual disabilities. Furthermore, the BIS Spectral Index is lower and the dose of Propofol and Remifentanil is significantly lower
[26].
Prolonged awakening time from anesthesia appears to be correlated with frequent cholinergic dysfunction in patients with mental disabilities
[26][27].
Therefore, it is important to meticulously monitor the patient during awakening because prolonged awakening time is associated with greater difficulty in maintaining patent airways due to the presence of craniofacial abnormalities and drug interactions.
Postoperative Complications
The risk of postoperative anesthetic complications in patients with SN depends on the patient’s ASA classification, clinical condition, type of anesthetic used and type of surgical procedure performed
[28]. About 4.2% of cases have moderate complications, such as hypotension. Airway obstruction is the most common complication, followed by nausea and vomiting
[28][29]. Yumura and Coll. reported an incidence of postoperative nausea and vomiting in patients with intellectual disabilities of 5.6%, a percentage higher than the general population
[28][30]. Lim and Coll. found that 44.4% of patients with cerebral palsy have complications secondary to difficult airway management. In particular, there was an incidence of respiratory problems of 30.4% in patients with ASD, 29.2% in patients with DS and 17.1% in patients with intellectual disabilities.