Venous access can be difficult. In the authors’ practice, an inhalational induction is often performed allowing careful selection of the optimal puncture site in a quiet and motionless patient [28]. In the authors’ opinion, this can even be justified in patients with a history of reflux [29].

Endotracheal intubation is strongly recommended for children with gastroesophageal reflux and prolonged surgery. On the other hand, for many children with cerebral palsy and no history of reflux, a laryngeal mask airway (LMA) with a gastric access, e.g., LMA Supreme^® or LMA ProSeal^®, can be a valuable airway tool. In case of scoliosis and severe deformity, optimal positioning for intubation can be challenging, even if the airway itself is normal. Often, the length of the trachea may not be as long as expected for age, and auscultation may not reliably allow identifying bronchial main stem intubation. It is the authors’ practice to use fiberoptic control liberally to verify the correct position of the tip of the endotracheal tube.

Intermittent airway obstruction may occur in some patients with CP – even without anesthesia – because of hypotonia of the pharyngeal muscles; the symptoms tend to become worse with increasing age [30] and ultimately may lead to a tracheostomy in some patients. Perioperatively, special attention must be paid to avoid anesthetic overhang or benzodiazepine medication. During mask induction, the skilled practitioner will generally be able to maintain an open airway with jaw thrust and positive airway pressure.

Hypothermia is the most common perioperative complication in children with CP [4]. The etiology is probably multifactorial: undoubtedly, the most important factor in its pathogenesis is a reduced metabolic rate (“vita reducta”) coupled with exaggerated losses due to exposure of large areas of the body necessary for many orthopedic interventions. In addition, these patients often have very little subcutaneous fat tissue to insulate against heat loss. Finally, it has been speculated that there may be abnormal thermoregulatory mechanisms in patients with a compromised central nervous system.

Forced air systems (e.g., Bair Hugger^®) are successfully used to prevent perioperative hypothermia. In addition to an elevated room temperature, the authors use two forced air systems in vulnerable patients: one on which the patient is placed and one that covers the body surface as much as possible. Continuous monitoring of the patient’s body temperature using rectal, esophageal, or bladder temperature probes is advisable. With the appropriate use of modern equipment, hypothermia can almost invariably be prevented.

Hypnotics: Patients with CP, as well as many other severely handicapped children, seem to be much more sensible to hypnotic agents. Frei et al. reported that the MAC values of halothane are reduced by around 1/3 in children with CP [31]. This is clearly a pharmacodynamic phenomenon: a lower concentration is needed for a certain effect. Similarly, less propofol is needed to reach a target BIS value of 40 [32]. This may also be caused by an altered pharmacodynamic response; however, different pharmacokinetics may also be responsible. Lower BIS values are recorded in children with cerebral palsy while awake, as well as after exposure to different sevoflurane concentrations [33]. Other authors, however, have found similar BIS values before induction among patients with CP when compared with healthy children [34]. The reduced requirement for hypnotic agents seems to be a common phenomenon for most, if not all, severely handicapped children [35]. It is the authors’ practice to monitor the hypnotic state with an EEG-based monitor, i.e., bispectral index (BIS^®), in all of these patients despite the fact that the validity of these monitors has not been tested extensively in this patient population, and an individual patient may well present with a very low BIS^® value before anesthesia induction.

Opioids: Only limited information is available about the sensitivity to opioids in patients with CP. It has been speculated that chronic hypoxia increases the sensitivity to opioids, especially to the respiratory depressant effects of opioids [36]. Children living in high altitude exposed to chronic hypoxic conditions seem to require less opioids after surgery for sufficient pain relief [37]. Typically, increased sensitivity to opioids is discussed in the context of obstructive sleep apnea [38] where it is associated with relevant morbidity and even mortality [39]. There is little doubt that similar phenomena can be found in children with CP; intermittent nocturnal desaturations up-regulate endorphin receptors and enhance sensitivity to opioids [40]. Therefore, careful dosing and adequate monitoring is very important in this patient population.

Neuromuscular blocking agents: Surprisingly, children with CP, despite the fact that they often have a very reduced muscle mass (Fig. 17.3), seem to be resistant to non-depolarizing neuromuscular blocking agents. Clinically, a given dose has a shorter duration of action [41]. This resistance does not appear to be related to concomitant use of antiepileptic medication [42]. Receptor up-regulation seems to be a suitable explanation. This also explains the increased sensitivity to succinylcholine [43]. Hyperkalemia does not seem to be a problem, likely due to a reduced muscular mass [44]. Despite the fact that the airway is usually normal, difficult intubation is reported to be more common in severely handicapped patients; insufficient doses of non-depolarizing neuromuscular blocking agents may be at least in part responsible in some cases. Given the availability of sugammadex, higher doses of rocuronium, which provide excellent intubation conditions, have become an attractive option in patients with CP or other neuromuscular disorders [45].

Prevention of difficult inductions by using an adequate premedication is of paramount importance. It is the authors’ practice to add ketamine to midazolam if relevant uncooperative behavior can be anticipated [46]. Often, a peaceful induction can be achieved by gentle conviction of the patient and parental support. In addition, success is highly dependent on the skill and empathy of the anesthesiologist. Inhalation of nitrous oxide followed by sevoflurane using a flavored mask while the child is in his/her preferred position is a good option. In the authors’ institution, an uncooperative child is usually left in his/her hospital bed for induction [47].

In patients with CP, pain is not restricted to the perioperative period but rather a predominant symptom [48]; up to 75 % of young people with CP experience some pain in a typical week [49]. Possibly as a consequence, pain appears to be more difficult to treat in this patient population. A first challenge is to recognize whether the patient is in pain. It is likely that pain is often undertreated in these patients [50]. On the other hand, because of the difficulties to distinguish pain and other causes of agitation, overdosing with oversedation can easily occur.

Whenever possible, regional blocks are probably the most elegant way to provide reliable analgesia in these vulnerable patients; continuous epidural analgesia may be considered after major surgery, especially in conditions where spasms of the muscles of the lower extremities can be expected [51].

Major orthopedic surgery is commonly undertaken in children with CP [52]. Scoliosis surgery is often extensive involving segments from the high thoracic region down to the sacrum, and pelvic stabilization will assist in sitting. Intraoperative monitoring has become commonplace [53]. Postoperative pain relief is challenging; intrathecal morphine can easily be administered [54], enhances perioperative stability, and thus may reduce blood loss [55]. Epidural catheters, positioned intraoperatively by the surgeons, have also been successfully used [56].

Hip reconstruction involving pelvic and femoral osteotomies is offered even to the most debilitated children with the aim to reduce pain, to facilitate positioning, and even occasionally to allow a sitting position. Palliative surgery, e.g., resection of the femoral head, is nowadays only rarely performed [57]. Postoperative pain relief can be provided through lumbar epidural catheters; in the authors’ practice, however, caudal morphine combined with peripheral nerve blocks, e.g., lumbar plexus [58] or femoral and/or sciatic nerve blocks, are often used.

Blood loss can be major. Antifibrinolytic agents, e.g., tranexamic acid, are recommended by most authorities to reduce blood loss [59]; its effectiveness has been specifically shown in cerebral palsy scoliosis too [60]. However, the pediatric data covering other types of surgery are still very limited [61].

Latex allergy seems to be a lesser problem nowadays; nevertheless, all children presenting for repeated surgery are at increased risk (Fig. 17.4) [62]; latex allergy is not caused by spina bifida per se [63].

Nissen fundoplication is considered as the treatment of choice for children with gastroesophageal reflux disease. Laparoscopic Nissen fundoplication is offered today as the standard procedure, although the evidence of its superiority over open surgery is limited [64]. The technique involves prolonged pneumoperitoneum with the patient in a head-up position [65]. Ventilation and PEEP have to be adapted to counteract the effects of the elevated intra-abdominal pressure. Pneumothorax can occur by transition of CO₂ from the abdominal cavity to the pleural space through lesions caused by dissection; generally, no specific treatment is required provided that a sufficiently high PEEP level is maintained [66]. Hemodynamic monitoring is crucial; the critical phases are the creation of the pneumoperitoneum as well as its release at the end of surgery. The patients usually develop a transient oliguric state during the period with elevated intra-abdominal pressure [67]; there is some consensus that this does not require aggressive treatment.