The choice whether intubation or tracheostomy would assure the best airway management requires a full agreement between the anaesthesiologist and the surgeon. Such a common decision should be based on the precise preoperative knowledge of the ‘syndromic’ pattern of a given malformation, that is, all the possible anatomical features limiting the manoeuvres of intubation and/or a thorough evaluation of the risk of performing the surgical procedure itself as well as postoperative complications, namely, postoperative upper airway oedema [5]. A further aspect to be taken into account is the choice of the anterior or posterior surgical approach [64].

Micrognathia and mandibular hypo-dysplasia as well as macroglossia constitute an absolute cause of difficult intubation due to reduced anatomical space between the basihyoid bone and the mandible which limits the displacement of the soft parts from the laryngoscopes (during laryngoscopy). Difficult intubation may be encountered also in Goldenhar syndrome and Klippel-Feil syndrome due to the reduced mobility between atlanto-occipital and the cervical vertebrae [52]. Anaesthesia and surgery were associated with worsening of previously unrecognized abnormalities in children with Down syndrome, abnormalities that probably reflected some degree of pre-existing cord compression. Therefore, if there is any suspicion of signs or symptoms consistent with cervical cord compression, a thorough history and physical examination should be conducted [36]. In mucopolysaccharidosis, mainly in type IV (i.e. Morquio syndrome), the macroglossia and the reduction of the upper airway volume play a role in favouring alternatives to classic orotracheal intubation. Airway management in this patients can be complicated by macroglossia, prognathism, and hypertrophy of pharyngeal and laryngeal tissues, which make mask fit, mask ventilation, laryngoscopy, and correct tracheal tube placement difficult [42]. In this and similar pathological conditions, no method of airway assessment is foolproof for predicting the difficult airway; nevertheless, commonly accepted preoperative assessment evaluation does exist, such as the Mallampati classification, which is based on the anatomical structures visualized when patients open their mouths (Table 2).

A Mallampati class of III or IV is predictive of airway management difficulty in acromegalics [59]. In older children, an alternate airway assessment modality is the upper lip bite test (ULBT) in which a patient is asked to cover the vermillion of the upper lip with the lower incisors (Table 3). A higher grade can be predictive of airway management difficulty [27]. The Cormack and Lehane grade, at the time of laryngoscopy, is used to define difficulty with laryngoscopy (Table 4). Patients with a Cormack and Lehane grade 3 or 4 are considered to pose difficulty with laryngoscopy.

In general, tracheal intubation is considered difficult if more than two failed attempts occur in requiring rescue anaesthesiological techniques, e.g. laryngoscope blade change, bougie, video laryngoscopy, and fibre-optic bronchoscope [55]. In this case awake fibre-optic intubation can be strongly advocated in case of surgical procedures on the CVJ but also tracheostomy must be taken into consideration.

Modern fibre-optic technology offers a safe and effective method of airway management. However, it requires great skills and acquired experience and is very difficult to perform with uncooperative patients, especially children [59]. Moreover, this technique may be particularly difficult in relation with retropharyngeal swelling and adenoidal hypertrophy in children.

It is worth to note that in the last 15 years, adequate instruments for fibre-optic bronchoscopy have been introduced in the anaesthesiological armamentarium, thus making easier the application of this technique in the paediatric population, including infants and small children [6], consequently overcoming the difficulties encountered in the past when only ‘adult’ fibre-optic apparatus, 6 mm in diameter, were available. Failure to intubate may necessitate alternative airway control devices such as video-assisted intubation devices or intubation through laryngeal mask airways (LMA). LMA may be particularly useful in this setting, and endotracheal tube placement can be achieved using the fibrescope through the LMA/iLMA as a guide [38]. However, till more evidence is available, there are not strong recommendations for standardized procedures in children with difficult or failed intubation. This is the reason why for a continuous search of better intubating devices such as, for example, the video laryngoscopes, are able to provide a better laryngeal view as compared to direct laryngoscopy [69].

The decision to intubate or perform a tracheostomy is essentially made by estimating the risk of upper airway oedema that may occur postoperatively. If excessive oedema is expected, a tracheostomy should be taken into consideration to assure a controlled and stable airway in the postoperative period. This would permit while allowing an early wake-up, useful to obtain an immediate neurological postoperative evaluation and prevent from harmful consequences of a prolonged tracheal intubation [58].

Although tracheostomy and tracheotomy are used interchangeably, it should be reminded that tracheostomy from the Greek word stomoun refers to the act of providing an opening, whereas tracheotomy, from the Greek word tome, to cut, means specifically the result of surgical cut. The need of cervical external orthosis, that is, a Halo Vest system, should suggest to proceed to the intervention of tracheostomy in order to prevent the necessity of performing an emergency procedure in the postoperative phase due to overwhelming respiratory distress.

Sedation is necessary to evaluate spontaneous breathing and ventilation. When the oxygenation and ventilation are adequate, it is possible to proceed to laryngoscopy after induction with administration of not irritating inhalational agents (sevoflurane) or intravenous drugs (propofol and remifentanil). In cases where difficult intubation is anticipated, it is useful to consider the use of a fibre-optic bronchoscope. Indeed, fibre-optic techniques have gained greater use in selected paediatric patients following a combination of topical anaesthesia, local airway blocks and IV sedation, or under general anaesthesia by mask with maintenance of spontaneous ventilation [44]. The ideal size of fibrescope depends on the diameter of the endotracheal tube to be passed through. Intubation requires an ETT size that is only slightly larger than the diameter of the fibrescope. The modern flexible fibrescopes arrive to a diameter of 2 Fr and allow the passage of endotracheal tube inside diameter of 2.5 Fr.

Providing moderate levels of sedation without causing respiratory distress or haemodynamic instability, dexmedetomidine could be successfully used in patients requiring awake fibre-optic intubation for cervical spine surgery [3, 33]. In all patients, electrocardiography pulse oximetry and blood pressure recordings must be monitored throughout the procedure. The maintenance of anaesthesia is obtained with Sevorane (MAC 1, 1.5) in O2 plus air with a paCO2 target level of 32–35 mmHg. Muscle relaxation is obtained with cisatracurium besylate.

Intraoperative monitoring of spinal cord function is considered a standard of care in spinal surgery [54]. The methods of monitoring the integrity of the spinal cord function intraoperatively include:

Intraoperative fluid therapy may start at the time of the surgical treatment as simply as replacing the deficits of the preoperative fasting status and maintenance of normal fluid condition or as complex therapy need at correcting preoperative abnormal deficits. During the surgery, care should be given to compensate intraoperative translocated fluids and variable blood loss.

Because of their high metabolic rate and water turnover, significant hypoglycaemia and dehydration may occur in infants who are required to fast preoperatively for prolonged periods of time. If not provided previously, the fluid deficit occurred during fasting should be replaced during anaesthesia. Assuming that a healthy child is in water and electrolyte balance at the time oral feedings are discontinued, the fluid deficit at the start of anaesthesia can be estimated by multiplying the child’s hourly maintenance fluid requirement by the number of hours that have elapsed since the last feeding. This deficit may be replaced by giving half of the calculated volume during the first hour of anaesthesia and the other half over the next 2 h, in addition to intraoperative maintenance fluids. Five percent dextrose in quarter-normal saline (5 % dextrose/0.25 NS) is frequently used for fluid maintenance.

Conflicting data do exist in the literature about the intraoperative use of glucose. The metabolic need for glucose in the infant age group is 5 g/kg/day. Infants under 4 years of age who are kept fasting for over 6 h may become hypoglycaemic and irritable. Since the metabolic rate and oxygen consumption of infants and small children is significantly greater than older children and adults, it has been recommended to provide 5 % dextrose for all infants at risk of hypoglycaemia (serum glucose <40 mg/dl) from preoperative starvation. It must be remembered, however, that preterm infants receiving glucose infusions should be monitored carefully for hyperglycaemia, since their kidneys are unable to hyperfiltrate in the presence of hyperglycaemia. Hyperglycaemia (glucose >200 mg/dl) has also been noted in older infants and children given 5 % dextrose in lactated Ringer’s as an intraoperative maintenance fluid. Elevated blood glucose concentrations may lead to an osmotic diuresis and may exacerbate neurological injury associated with a cardiac arrest or other cerebral insults (i.e. cerebral ischaemia and asphyxia). The clinical significance of postoperative hyperglycaemia in otherwise healthy children would seem to be minimal. However, in major surgical cases, such hyperglycaemia and osmotic diuresis may complicate or confuse ongoing fluid therapy. Therefore, we can consider blood glucose control as a reasonable goal for the majority of the cases and a critical goal in a few children. Blood glucose control can be achieved in several ways. Most children receiving glucose-free intravenous fluids during surgical procedures demonstrate an increase in their blood glucose concentration. Physiological factors accounting for the hyperglycaemic response to anaesthesia and surgery include increased sympathoadrenal activity leading to a decrease in glucose tolerance, decreased glucose utilization, and increased gluconeogenesis. However, about 12 % of patients who receive glucose-free solutions fail to increase their blood glucose concentrations. Since hypoglycaemia may be undetected during anaesthesia, it would seem prudent to measure intraoperative glucose concentrations if glucose-free solutions are used and to treat hypoglycaemia if diagnosed. It seems paradoxical, however, to be depending on the stress of surgery to increase glucose at a time when ‘stress-free’ anaesthetics are being advocated. Therefore, in general, we advocate the use of glucose-containing solutions. One way to avoid hyperglycaemia with a 5 % dextrose solution is to decrease the infusion rate, but this may contribute to hypotension in fluid-depleted children. A less concentrated glucose solution (e.g. 2.5 % dextrose in lactated Ringer’s) given at recommended infusion rates provides adequate glucose for healthy children (300 mg/kg/h) and adequate volume replacement while avoiding hyperglycaemia and hypoglycaemia. Since 2.5 % dextrose in lactated Ringer’s is not commercially available, it can easily be made by the individual practitioner with 50 % dextrose solution.

Surgical traumas are associated with the isotonic transfer of fluids from the extracellular fluid compartment (and to a lesser extent from the intracellular compartment) to a nonfunctional interstitial compartment. This acute sequestration of fluid to a nonfunctional compartment has been called third-space loss and should be replaced. In neurosurgery, it is negligible (1–2 ml/kg/h) [16]. Generally, lactated Ringer’s solution or normal saline is used to restore third-space losses.

Paediatric patients undergoing major procedures (e.g. tumour resection, reconstructive surgery, cardiac surgery, organ transplantation, trauma surgery, or surgery for burns) will invariably require some form of blood volume replacement. Indications for blood or blood component therapy are not always clear-cut; hence, the anaesthesiologist should formulate a preoperative plan of management utilizing scientific rationale for a decision on blood therapy. Factors on which this decision is based include the patient’s blood volume estimates, preoperative haematocrit, general medical condition, and ability to provide adequate oxygen transport to the tissues, as well as the nature of the surgical procedure and the risks vs. benefits of transfusion [49, 62]. All blood loss in neonates, infants, and children should somehow be replaced. An accurate measurement of blood loss and an accurate assessment of acceptable blood loss in the child are vital to any replacement regimen. Calculation of sponge weight, the use of calibrated miniaturized suction bottles, and a visual estimation (combined with a ‘guess factor’) make it possible to define the magnitude of the blood loss. The transfusion practice guidelines of the American Society of Anesthesiologists Task Force on Blood Component Therapy [3] are largely applicable to paediatric patients without cardiopulmonary disease. The concept of allowable red-cell loss or allowable blood loss, preferable as a guide to blood replacement normovolemic haemodilution to a predetermined haematocrit, can be achieved with crystalloid or colloid solutions. Blood volume is age-related: The neonatal blood volume has been reported to vary widely from 65 to 129 ml/kg, depending on the magnitude of the placental transfusion [17].

Fluid management is carefully monitored, avoiding fluid overload as well as hypovolaemia. It is advisable to give crystalloids (normal saline 0.9 % NaCl or Ringer’s lactate) in order to allow an adequate glycaemic level. It has been documented that hyperglycaemia is common in critically ill children and is associated with mortality and morbidity in PICU [1, 39]. However, the actual relevance of strict glycaemic control is particularly controversial in the context of neurocritical care. In the first 24 h after neurosurgery, hyperglycaemia is observed in many children, even in the absence of glucose infusion. After traumatic brain injury (TBI), hyperglycaemia has been associated with poor outcome, explained either by a more severe TBI or by the neurotoxicity of glucose [7, 60]. Hyperglycaemia increases oxidative stress and inflammation, augments excitotoxicity mediated by NMDA receptors, and alters microcirculatory perfusion. On the other hand, brain metabolism depends on a constant supply of glucose, and hypoglycaemia also favours excitotoxicity, apoptosis, and oxygen free-radical accumulation. Neuroglycopaenia and metabolic crisis can occur with even mild hypoglycaemia. It is therefore advisable to keep blood glucose levels less than 150 mg/dl through insulin infusion and, at the same time, prevent hypoglycaemia by adding glucose solution when serum glucose falls below 70 mg/dl [25].

Short course antibiotic prophylaxis is initiated just before the operation. Guidelines recommend that antibiotic prophylaxis should be given only at premedication, but in clinical practice, antibiotic prophylaxis is often unnecessarily prolonged through the next few days. Prophylaxis against stress ulcers should be recommended for the prevention of upper gastrointestinal bleeding in these patients. A systematic review and meta-analysis found limited high-quality data to recommend routine use of stress ulcer prophylaxis [47]. At the present time, stress ulcer prophylaxis using ranitidine, sucralfate, or proton-pump inhibitors (PPIs) is instituted in children with risk factors for development of stress ulcers.