PAEDIATRIC ANAESTHESIA Tutorial 396

Anaesthetic Management of
Craniopharyngioma Resection
Dr Maria Cristina Sanchez Jordan1†, Dr Faye Evans2, Dr Sulpicio
Soriano2
1
Pediatric Anesthesia Fellow, Boston Children’s Hospital, Boston, Massachusetts, USA
2
Senior Associates in Perioperative Anesthesia, Boston Children’s Hospital, Boston,
Massachusetts, USA

Edited by: Dr Kate Wilson, Consultant Paediatric Anaesthetist, Sheffield Children’s

Hospital, Sheffield, UK
†
Corresponding author e-mail: [email protected]

Published 22 January 2019

KEY POINTS
 Effective perioperative management of a patient with a craniopharyngioma entails a multidisciplinary team including
an endocrinologist, an oncologist, a neurosurgeon, an anaesthesiologist, and an intensivist.
 Hypothalamic damage and endocrine derangements are more common in the paediatric population due to slow
tumour growth. A patient’s perioperative course may be complicated by intracranial hypertension, growth failure,
hypothyroidism, diabetes insipidus, obesity, and seizures.
 Treatment options include medical management of clinical symptoms, surgical treatment with subtotal resection and
postoperative radiation therapy, or complete resection. Other less-invasive procedures include endoscopic cyst
fenestration or placement of an Ommaya reservoir into the tumour cyst for delivery of antineoplastic agents.
 Key anaesthetic considerations include surgical approach, patient positioning, perioperative steroid supplementation,
surgical damage to the optic chiasm and the circle of Willis, and management of diabetes insipidus and other
endocrine disorders.

INTRODUCTION
Craniopharyngiomas are rare, histologically benign brain tumours derived from pituitary gland embryonic tissue that are seen
most commonly in children between the ages of 5 and 10 years. They represent about 2% to 6% of all paediatric primary
intracranial tumours.1 Effective perioperative management entails a multidisciplinary team including an endocrinologist, an
oncologist, a neurosurgeon, an anaesthesiologist, and an intensivist. Preoperative evaluation should include determination of
tumour size and proximity to critical neural and vascular structures, presence of compressive symptoms, effect on intracranial
pressure (ICP), presence of any endocrine abnormalities, and any effects of radiotherapy already given. The anaesthesiologist
plays an important role in directing the perioperative care of these patients, which is critical to help improve overall outcome.

CLINICAL PRESENTATION
Clinical presentation will depend on the site and size of the tumour. Craniopharyngiomas are slow growing and symptoms often
are present for a year or more before the diagnosis is established.5 Hypothalamic damage and endocrine disturbances are
more common in children and typically ensue before the appearance of visual symptoms.

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ATOTW 396 — Anaesthetic Management of Craniopharyngioma Resection (22 January 2019) Page 1 of 5
Clinical presentation includes the following:
1. Intracranial hypertension: nausea, vomiting, headache, papilledema, altered level of consciousness
2. Visual pathway disturbances: bitemporal hemianopsia, homonymous anopsia, optic nerve atrophy in severe cases
3. Endocrine disturbances: hypothyroidism, growth failure, hypogonadism, syndrome of inappropriate antidiuretic hormone
secretion, diabetes insipidus (DI), precocious puberty
4. Thalamus, hypothalamus, and/or frontal lobe disturbances: aphagia, obesity, poor energy, somnolence, emotional lability,
hallucinations, autonomic disturbances
5. Brain parenchyma disturbances: seizures, cognitive dysfunction.

TREATMENT
Historically, optimal treatment has been controversial and included two basic approaches: aggressive surgery with an attempt
to achieve complete resection versus a more conservative partial surgical resection followed by radiation therapy to eradicate
residual tumour.5 Medical management will be aimed at hormonal replacement therapy for endocrine abnormalities that are
present.
Craniopharyngiomas are divided into two categories: adamantinomatous and papillary. Surgical resection in children is more
challenging due to the predominance of the adamantinomatous histological subtype. This subtype of tumour is more cystic and
calcified, larger at presentation, and more adherent to adjacent neural tissue as a result of fibrosis and inflammation.2
Surgical approach depends on both the location of the tumour and the age of the patient. A transsphenoidal endoscopic
approach is typically used for intrasellar tumours in children older than 10 years. Tumours with suprasellar extension require an
extended approach.
For the surgeon experienced in endoscopy, an endonasal approach, as compared to an open approach, has been shown to
provide higher rates for gross total resection, with lower recurrence, lower rates of permanent DI, and less visual deterioration.7
Nevertheless, the diminutive size of the sphenoid sinus in infants and young children necessitates a frontal craniotomy to
access the tumour.
One of the least invasive treatment options is the insertion of an Ommaya reservoir into the cystic aspect of the tumour followed
by drainage with or without subsequent instillation of antineoplastic agents.7 Radiation therapy is used to treat patients who
have undergone a partial surgical resection or to treat recurrence.

PREOPERATIVE EVALUATION
Preoperatively, diagnosis is marked by the presence of a mass on magnetic resonance imaging and/or computed tomography.
The tumour can vary from a small, solid, well-circumscribed mass to a huge multi-locular cyst that invades the sella turcica and
displaces neighbouring cerebral structures.5 A thorough history and physical examination looking for systemic manifestations
provides important information regarding clinical presentation and subsequent treatment. In addition to performing a standard
system-based preoperative anaesthetic evaluation, specific considerations in the paediatric patient with craniopharyngioma
include the following:
1. Endocrine evaluation to identify abnormalities including thyroid function tests, and determination of growth hormone, cortisol,
sex hormones, adrenocorticotropic hormone, and prolactin levels. Hypoadrenalism, DI, and hypothyroidism have shown to
have significant morbidity and mortality and should be corrected preoperatively.
2. Evaluation of current medications including antiepileptic agents for seizure management and/or hormonal therapy.
3. Ophthalmological assessment to provide information about visual acuity, field of vision, and possible papilledema.
4. Assessment and correction of volume status and electrolyte disturbances. This will be particularly important in children with
signs and symptoms of DI and increased intracranial pressure.
5. A thorough airway examination, taking into consideration the differences between the adult and paediatric airways. Short
stature and obesity in this patient population may pose a challenge in airway management. Obese children may have
obstructive sleep apnoea and/or respiratory issues and thus should be identified preoperatively and considered at a higher
risk for airway complications.

ANESTHETIC CONSIDERATIONS
Neurosurgery in the paediatric population is challenging to the anaesthesiologist due to the neurophysiological variations that
exist between the adult and paediatric population. Cerebral blood flow and cerebral metabolic rate of oxygen are increased in
infants and children as compared to adults and a tight coupling exists between the two. Although cerebral autoregulation exists
in the paediatric brain, any extremes in blood pressure beyond the autoregulatory limits place the child at risk of developing
ischemia or intracerebral hemorrhage.3
For nonemergent procedures, local fasting guidelines should be followed. Patients may present to surgery with hypovolemia
due to DI, vomiting, fluid restriction, contrast agents, or osmotic diuresis. Adequate volume replacement is an important

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ATOTW 396 — Anaesthetic Management of Craniopharyngioma Resection (22 January 2019) Page 2 of 5
consideration prior to anaesthesia as well as when deciding upon an induction agent. Patients who are on hormonal therapy
should receive their morning doses of medications as well as those on antiepileptic agents for seizure management.
Premedication will depend on the patient’s age, anxiety level, physical state, and comorbidities, including increased ICP or
alterations in mental status. Premedication with opioids may pose a risk of hypoventilation with subsequent hypercarbia and
hypoxemia in infants or children and should be avoided. Oral midazolam at a dose of 0.5 mg/kg may provide effective anxiolysis
without significantly impacting oxygen saturation. (See ATOTW 367: ‘‘Paediatric anaesthesia: challenges with induction.’’)

MONITORING
Standard American Society of Anesthesiologists’ monitors including pulse oximetry, electrocardiography, blood pressure
measurement, temperature monitoring, and end tidal CO2 measurement should be employed. Direct arterial blood pressure
measurement should be obtained as it aids in anaesthetic management during surgical resection. Serial arterial blood gas
sampling will allow for tight intraoperative control of the partial pressure of carbon dioxide (PaCO2), which may help improve
surgical visualization, especially during endoscopic procedures. In addition, the proportionate increase in dead space, high flow
rates, and small tidal volumes of infants and children may cause end tidal CO2 measurements to underestimate PaCO2. This
may result in hypoventilation and alterations in ICP, making direct PaCO2 measurements crucial in helping to maintain cerebral
perfusion pressure.4 Arterial line cannulation will also allow for direct blood pressure monitoring as well as the ability to closely
monitor blood gases, serum electrolytes, and osmolality and haemoglobin levels. Placement of a urinary catheter is
recommended as it will aid in monitoring urine output, which is crucial in the detection and management of DI.

VASCULAR ACCESS
Vascular access should include at least two large-bore peripheral intravenous lines for rapid resuscitation. There is a risk for
significant bleeding due to the close proximity of the internal carotid artery and other vessels of the circle of Willis. Central
venous cannulation is not necessarily required. Nevertheless, it should be considered depending on the size and extent of the
tumour, presence of DI, possible prolonged use of inotropes, and if central venous pressure (CVP) measurement is deemed
necessary.

INDUCTION AND MAINTENANCE OF ANAESTHESIA

Intravenous induction or inhalation induction with sevoflurane is considered appropriate for induction of anaesthesia. The
intravenous induction drug of choice will depend on the child’s volume and haemodynamic status as well as on patient
comorbidities. Irrespective of the agent used, the goal is to maintain cerebral perfusion pressure and avoid increases in ICP as
well as major variations in mean arterial pressure. The sympathetic response to laryngoscopy should be blunted and
preparations for a difficult airway undertaken if anticipated.
There are several options for maintenance of anaesthesia. The technique chosen should be tailored to ensure a rapid
awakening at the completion of surgery to allow for a complete neurological examination. Agents such as propofol, remifentanil,
sufentanil, and dexmedetomidine have all been used with techniques ranging from total intravenous anaesthesia to
combination techniques with inhalation agents. (See ATOTW 392: ‘‘A practical approach to propofol-based TIVA in children.’’)
Regardless of the agents chosen, their effects on cerebral nervous system physiology and haemodynamics should be
considered and closely monitored. Intermittent or continuous infusion of a neuromuscular blocking agent should be employed to
ensure a motionless surgical field. Mannitol and other ICP-lowering interventions may be needed for children presenting with
increased ICP. Normal saline is the most commonly used fluid for neurosurgical procedures as its hyperosmolarity protects
against cerebral oedema. Nevertheless, it is important to remember that large quantities may produce hyperchloremic
metabolic acidosis and hypernatraemia. Topical nasal vasoconstrictors such a cocaine or phenylephrine are routinely applied
to the nasal passages to minimize bleeding. However, these drugs can cause hypertension and tachycardia and these side
effects should be closely monitored for and treated appropriately. In the rare setting of acute presentation of tumours
obstructing the fourth ventricle and aqueduct of Sylvius, an emergent ventriculostomy may be required with all the anaesthetic
implications of an emergent surgery.1

POSITIONING
Patient positioning is of paramount importance to the anaesthesiologist. Surgical approach will ultimately dictate the position of
the patient. Supine position with head tilt is commonly employed for resection of a craniopharyngioma. The Mayfield frame is
often used to fix the head with paediatric pins or the head may be supported in a horseshoe frame. Pin application in the
paediatric patient carries the risk of intracranial haematoma, dural tear, and/or skull fracture, and should be done cautiously.1
During and after positioning it is of utmost importance that the endotracheal tube position be verified. It is not uncommon for
endobronchial tube migration or cuff herniation at the glottic opening with head and neck manipulation to occur. Adequate
venous drainage from the head and neck must be ensured as venous congestion may lead to increased ICP. Intravenous

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ATOTW 396 — Anaesthetic Management of Craniopharyngioma Resection (22 January 2019) Page 3 of 5
access lines, monitoring cables, and arterial line tubing should be free of kinking or risk of dislodgement. Padding of pressure
points cannot be underestimated as pressure ulcers after prolonged hours of surgery may occur, especially in the paediatric
patient.

INTRAOPERATIVE CONCERNS AND RISKS

The following table summarizes some of the intraoperative considerations and risks associated with craniopharyngioma resection.

DIABETES INSIPIDUS
Diabetes insipidus results form a deficiency of antidiuretic hormone, or arginine vasopressin. This hormone, produced in the
hypothalamus, stored and released from the posterior pituitary, acts on the distal tubules and collecting ducts of the kidneys to
promote reabsorption of water. A lack of antidiuretic hormone due to surgical resection therefore results in diuresis and
electrolyte imbalance. Approximately 75% of patients develop DI following extended surgical resection of a pituitary tumour and
DI occurs in 10% to 44% of patients after transsphenoidal pituitary surgery.6 Managing DI in children can be challenging, and
often physicians from different specialties have different protocols, which may further complicate treatment. It is for this reason
that a multidisciplinary discussion should be held during the preoperative evaluation to determine what management algorithm
will be followed.
Signs suggestive of DI include the following:
& Urine output  4 mL/kg/h
& Serum Na  145 mEq/L
& Serum osmolality . 300 mOsm/kg
& Urine osmolality , 300 mOsm/kg
& Polyuria  30 minutes
& Other causes of polyuria ruled out (eg, mannitol, saline, osmotic contrast agents, glucose, diuretics).
In the authors’ institution, the standard vasopressin concentration is 30 milliunits/mL. When there is intraoperative evidence of
DI, a vasopressin infusion is started at 1 milliunit/kg/h and the rate is slowly (every 5 to 10 minutes) increased (maximum 10
milliunits /kg/h) to avoid hypertension. The goal is to decrease the urine output to less than 2 mL/kg/h.
For fluid management, the authors’ institution algorithm includes the following:
& Replace fluid deficits with normal saline or lactated Ringer solution as needed to support blood pressure until antidiuresis is
established.
& After a reduction in the rate of diuresis, the intravenous fluids rate should be at two-thirds maintenance (plus fluids necessary
for blood replacement and blood pressure support).
& Replace blood loss with normal saline, lactated Ringer solution, 5% albumin, or blood products as appropriate.
& Check serum sodium every hour.
Vasopressin infusion is continued postoperatively for management in the intensive care unit.

Risk Consideration
Adrenal insufficiency Perioperative steroid replacement with IV hydrocortisone as per endocrinology
recommendations.
Significant blood loss Preoperatively, calculate patient blood volume and allowable blood loss.
Typed and cross-matched blood should be immediately available. Closely monitor
with intraoperative haematocrit and haemoglobin.
Diabetes insipidus Most commonly encountered postoperatively
See DI section below.
Hypothalamic disturbance May lead to temperature dysregulation.
Anteromedial hypothalamic simulation may induce ST segment changes which
can mimic cardiac ischemia.1
Brainstem injury Rare but can occur in tumours with widespread invasion and may result in
comatose state.
Seizures Common after frontal lobe retraction and manipulation with conventional
craniotomy.
Cerebrospinal fluid leak Common with transsphenoidal approach.
Avoid bucking and coughing on extubation of the trachea.
Table. Intraoperative Considerations and Risks Associated with Craniopharyngioma Resection

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POSTOPERATIVE CARE
Patients undergoing an uneventful intraoperative course can be extubated at the conclusion of surgery. This allows for rapid
neurological evaluation and avoids complications associated with postoperative mechanical ventilation. Postoperative care
should continue in the intensive care unit with input from a multidisciplinary team including a neurosurgeon, an
anaesthesiologist, an endocrinologist, and an intensivist. This team should be informed of any intraoperative concerns
encountered both from a surgical and anaesthetic standpoint; management and potential complications should also be
addressed. Plans for ongoing or potential endocrine derangements should be reviewed and most recent laboratory data
shared. Fluid balance and potential seizure control should be discussed and managed accordingly. A plan for analgesia should
be discussed by the multidisciplinary team, involving the pain management team if needed. Acute paediatric pain management
is increasingly characterized by a multimodal approach leading to smaller doses of both opioid and nonopioid analgesics.
Medications such as nonsteroidal anti-inflammatory drugs, a-2 adrenergic agonists, acetaminophen, and opioids can be used
in combination to maximize pain control while decreasing untoward drug-induced side effects. Haemodynamic parameters and
patient age and level of awareness as well as other comorbidities should be taken into consideration at the moment of drug
election and dosing.

REFERENCES
1. Moningi S. Anaesthetic management of children with craniopharyngioma. J Neuroanaesth Crit Care. 2017;4:30-37.
2. Alli S, Isik S, Rutka J. Microsurgical removal of craniopharyngioma: endoscopic and transcranial techniques for
complication avoidance. J Neurooncol. 2016;130:299-307.
3. Krass IS. Lippincott Williams & Wilkins. Physiology and metabolism of brain and spinal cord. In: Handbook of
Neuroanaesthesia. Philadelphia, PA: 2007.
4. Johnson J, Jimenez D, Tobias J. Anaesthetic care during minimally invasive neurosurgical procedures in infants and
children. Paediatr Anaesth. 2002;12:478-488.
5. Harsh G, Recht L, Marcus K. Craniopharyngioma. https://www.uptodate.com/contents/craniopharyngioma?search¼
craniopharyngioma%20children&source¼search_result&selectedTitle¼1~47&usage_type¼default&display_rank¼1#H19.
Accessed April 23, 2018.
6. Wise L, Sulpicio S, Ferrari L, et al. Perioperative management of diabetes insipidus in children. J Nerusosurg Anesthesiol.
2004;16:220-225.
7. Reddy G, Hansen D, Patel A, et al. Treatment options for pediatric craniopharyngioma. Surg Neurol Int. 2016;7(Suppl
6):S174-S178.

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