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Year : 2022  |  Volume : 11  |  Issue : 5  |  Page : 34-37

Role of anaesthesiologist in the management of an infant with hydrocephalus for shunt surgery

Department of Anaesthesiology, Sri Venkateswara Institute of Medical Sciences, Tirupati 517 507, Andhra Pradesh, India

Date of Submission05-Jan-2021
Date of Decision25-Mar-2021
Date of Acceptance29-May-2021
Date of Web Publication30-Aug-2022

Correspondence Address:
Janaki Subhadra Peyyety
Professor, Department of Anaesthesiology, Sri Venkateswara Institute of Medical Sciences, Tirupati 517 507, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcsr.jcsr_6_21

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Hydrocephalus is a disorder of abnormal accumulation of cerebrospinal fluid (CSF) in the ventricular system. Accumulation of CSF occurs due to an imbalance between CSF production and absorption. In the new-born and infants, hydrocephalus almost entirely presents as an enlarged head resulting from the separation of cranial bone plates, so any new-born or infant with an enlarged head should undergo evaluation. We report the case of a gross hydrocephalus in a 9-month-old infant (head circumference 55 cm) posted for ventriculoperitoneal shunt surgery with anaesthetic consideration of difficult intubation because of such a large head size, positioning, age-related pathophysiology and temperature regulation. The case was managed successfully with an uneventful hospital stay, and a significant decrease in head size was noted.

Keywords: Difficult airway, gross hydrocephalus, ventriculoperitoneal shunt

How to cite this article:
Radhika B, Jasmitha K, Peyyety JS, Ray AS, Rao MH. Role of anaesthesiologist in the management of an infant with hydrocephalus for shunt surgery. J Clin Sci Res 2022;11, Suppl S1:34-7

How to cite this URL:
Radhika B, Jasmitha K, Peyyety JS, Ray AS, Rao MH. Role of anaesthesiologist in the management of an infant with hydrocephalus for shunt surgery. J Clin Sci Res [serial online] 2022 [cited 2022 Oct 6];11, Suppl S1:34-7. Available from: https://www.jcsr.co.in/text.asp?2022/11/5/34/355063

  Introduction Top

Hydrocephalus is a Greek word hydro means water and cephalus means head, a condition in which there is an excessive accumulation of cerebrospinal fluid (CSF), resulting from increased production or decreased absorption of CSF. Hydrocephalus may be of two types, namely communicating or non-communicating (obstructive) hydrocephalus. Hydrocephalus cases require thorough preoperative anaesthetic assessment to rule out the congenital defects, delayed milestones and difficult airway assessment. Here, we present a case of a 9-month-old male patient with gross hydrocephalus and discuss the anaesthestic consideration.[1],[2],[3],[4]

  Case Report Top

A 9-month-old male infant presented to the emergency room with chief complaints of excessive cry for the last 10 days, decreased alertness for the last 1 week, decreased activity for the last 1 week, developed regurgitation of feeds and failure to thrive with progressive hydrocephalus since birth. The patient was delivered via caesarean section at 8 months 2 week of age; the indication for early caesarean section was oligohydramnios. The patient had one episode of seizure at 1 month of age and admitted in the neonatal intensive care unit for 21 days; the baby was diagnosed meningoencephalitis. Intravenous (IV) antibiotics were administered and ventriculoperitoneal (VP) shunt surgery was advised in view of hydrocephalus. VP shunt could not be done immediately due to poor general condition.

On examination, the infant weighed 14 Kg and was drowsy. AUPN scale baby stand for P à Crying, moving, withdrawing to painful stimuli/touch. Tone was appropriate with good muscle bulk. Pupils were bilaterally reacting to light. He had head circumference of 55 cm and both anterior and posterior fontanelles were enlarged; bulging was present in the anterior fontanelle and positive sunset sign present. The cardiac, respiratory and abdominal examinations were normal. Of particular note, there were no murmur, palpable heart sounds and thrills. Computed tomography (CT) of the brain showed dilated lateral and third ventricle with tinning of cortex with normal fourth ventricle. The infant was posted for emergency VP shunt.

A thorough pre-anaesthetic management was performed. Routine laboratory investigations were within normal limits. A difficult airway anticipated because of massively enlarged head size. Hence, the “difficult airway cart”, sheets and pillows were kept ready for adequate positioning as the risk of hypothermia was anticipated. To prevent the risk of hypothermia, use warm cleaning solutions, IV fluids and AC in the operating room (OR) switched off before shifting the patient inside. Written informed consent was obtained from the mother as the patient was a nine months old infant. Fasting for 4 h after breast milk was ensured. The baby came to the operating room with 22G IV cannula in situ from the ward. Standard monitoring with pulse oximeter, electrocardiogram and non-invasive blood pressure (Ni-BP) were done. The baby was covered in thick cotton wool. The child was pre-oxygenated with 100% oxygen for 3 min with the help of facemask and Jackson-Rees circuit at 6 L/min and induced with IV thiopentone sodium 100 mg, IV vecuronium 1.2 mg followed by IV fentanyl 20 μg according to he weight of the baby (14 kg). Layers of folded sheets were kept below the body of the body to bring the body in level with head to facilitate the intubation. After checking ability to ventilate, the lungs was intubated with orotracheal endotracheal tube with 4.0 mm(ID) cuffed tube, fixed at 14 cm mark at lips and position confirmed by end-tidal carbon dioxide (EtCO2), bilateral air entry into the chest, chest lifting, and adequacy of tidal volume. The patient was connected to ventilator with (VCV) mode with fraction of inspired oxygen (FIO2) 0.5, rate 25/min, tidal volume 110 mL. Anaesthesia was maintained with sevoflurane in O2 plus air, vecuronium. Intraoperative vital parameters were stable throughout the procedure. IV fluid Isolyte P was given according 4-2-1 rule at 48 mL/h and hourly urine output monitored and shunt was placed uneventfully. At end of the procedure, residual neuromusular blocker was reversed with neostigmine and glycopyrrolate. Suction was done and the child was extubated when protective airway reflexes were adequate and the child was awake. The child was shifted to post-operative recovery room for observation.

  Discussion Top

Congenital hydrocephalus is capable of producing brain atrophy hence poor prognosis and may also be associated with severe mental retardation. Management of patients presenting for shunt surgery can be challenging, especially in children with congenital anomalies and neurological deficits. Anaesthetic management can be challenging: Given the fact that many procedures are done as emergency surgeries difficulty in securing the airway in view of large head and risk of hypothermia, it requires careful planning and preparation – we raised the body of the child in level with hydrocephalic head by placing folded sheets under the body and also taken precaution to prevent the heat loss, hypothermia.[1],[2]

CSF is primarily produced by choroid plexus with partial contribution of ependymal capillaries and brain interstitial fluid. Production occurs at the rate of 0.3–0.6 mL/min with about 500–600 mL/day.[5] Production rate is related to cerebral blood flow that is higher in infants and children in comparison to adults (90–100 mL/100g/min Vs 50 mL/100g/min).[6],[7] CSF circulates from site of secretion to site of absorption (arachnoid villi), and any resistance in CSF circulation pathway and absorption or increased rate of production leads to the development of hydrocephalus. An increasing volume of CSF results in ventricular enlargement and later on rise in intracranial pressure (ICP).[5],[6],[7]

Causes of hydrocephalus may be classified into congenital and acquired ones, and the lesion may be of communicating or non-communicating type.[8],[9] Congenital hydrocephalus can be genetic or may be commonly associated with neural tube defects such as spina bifida, myelomeningocele and Arnold-Chiari or Dandy-Walker malformation, vascular malformations, arachnoid cysts, syndromes such as X-linked hydrocephalus and trisomies 13, 18, 9 and 9p and other central nervous system structural abnormalities.[8],[9] In our case, it was the congenital hydrocephalus and VP shunt was not done due to poor general condition at 1 month of age. Later at 9 months of age, the baby presented with gross hydrocephalus with symptoms and signs and posted for emergency VP shunt. Hence, preoperative history and perioperative anaesthetic plan should be formulated by keeping these possibilities of associated neurologic abnormalities and comorbidities in mind.

Intracranial hypertension almost always accompanies hydrocephalus in infants and children which is the most important anaesthetic consideration.[3] Normal range of ICP in infant is 1.5-6 mmHg, while in adult, it is higher (10-15 mmHg).[10] Autoregulatory reserve is decreased in infants due to lower values of mean arterial pressure, cerebral blood flow, and cerebral perfusion pressure (40–60 mmHg for infants, 50–70 mmHg for adults).[11],[12] Flexibility of infant's skull leads to enlargement of cranial compartment if hydrocephalus progresses slowly; once the upper limit of enlargement reaches, ICP begins to rise, and acute increase in cranial volume may cause herniation.[11] Increased ICP leads to non-specific symptoms in infants such as irritability, poor feeding, vomiting, lethargy and bulging fontanelle and downward gazing eyes.[11] Repeated episodes of vomiting, signs of intravascular volume contraction and infant's altered mental status call for arterial blood gas analysis before surgery.[13] Measures should be taken to avoid rise in ICP, i.e., slight head-up tilt, avoidance of hypercarbia and hypoxia, correction of hypotension and hypertension, administration of osmotic agents and diuretics and avoidance of positive end-expiratory pressure to prevent venous engorgement.[3]

Positional changes (head flexion, head down), behavioural change (crying) and physiologic change (hypercarbia) can affect ICP, so the child should be kept in head-up position with minimal possible agitating manoeuvres.[3] Paediatric airway is often considered more challenging than in adults because of anatomical differences (larger tongue, cephalad larynx, angulated vocal cord, omega-shaped epiglottis, and narrowed subglottic region) and physiological differences (rapid desaturation during periods of apnoea, more chances of upper airway obstruction under anaesthesia).[14] In addition, in congenital hydrocephalus, macrocephaly may distort the normal anatomy of the skull, making airway management even more difficult. Perioperative plan must be framed by anaesthesiologist based on difficult airway management as enlarged occiput forces neck in the extreme flexion and large forehead may obscure view of laryngoscopy, so elevating the body with folded sheet or pillow is necessary to facilitate intubation.[15] Rolling of head, especially after induction of anaesthesia, is to be avoided by keeping head rest. Our patient's head circumference was 55 cm; this resulted in a challenging combination for intubation. Prop-up positioning of the patient with the towel sheets allowed us to get optimised view of laryngoscopy. Difficult airway cart and alternative management plan were kept ready. Balance must be maintained between promoting sedation by pharmacological means and minimising risk of hypoventilation as increased partial pressure of arterial carbon dioxide (PaCO2) causes further rise in ICP.[3] Method of induction should be determined by circumstances of case and effect of anaesthetic agents on neurophysiology.[3] iv induction should be preferred if child has signs of raised ICP, stupor or delayed gastric emptying, as it reduces cerebral blood flow and ICP.[3],[16] Inhalational induction can be an acceptable alternative with non-irritant volatile anaesthetics such as sevoflurane in children with no intravenous access.[3] Maintenance of anaesthesia can be done by a combination of volatile anaesthetics, muscle relaxants and opioids intra-operatively.

Temperature regulation and maintenance of normoglycaemia should be kept in mind as infants are more prone for hypothermia and hypoglycaemia and surgical procedure of VP shunt requires exposure from head to abdomen, which further adds in issues of hypothermia.[17] Core temperature monitoring should be done along with measures such as maintenance of operating room temperature, covering of infant's body with cotton pads or warm air blankets and use of warm iv fluids. Initial skin incision and tunnelling of the shunt are the most stimulating parts of surgery; increase in heart rate and ICP during this period can be managed by either increasing depth of anaesthesia or using short-acting opioids such as fentanyl or remifentanil.[18],[19] On initial cannulation of ventricle, BP may drop abruptly as brainstem pressure is relieved suddenly. During ventriculostomies, serious dysrhythmias, especially bradycardias, may occur, apart from using atropine, simply alerting the surgeon of the bradycardia and pausing may resolve problem many times.[17] Look for the signs of pneumothorax as trocar is placed subcutaneously to allow tunnelling. Removal of large quantity of CSF may cause hemodynamic and electrolyte disturbances that needs to be corrected perioperatively and rapid drainage of CSF may lead to serious arrhythmias or even cardiac arrest; precautions should be taken to avoid this.[20]

Postoperatively, the infant can be at risk of respiratory problems and vomiting aspiration because of poor airway control due to neurological deficit, so these infants should be kept under close observation.[8] Risk of chronic neurological deficit exists postoperatively. This is attributed not only to failure rate of shunts but also to known fact that hydrocephalus causes ischemia and hypoxia by stretching and compressing the periventricular tissue, and chronically, it may result in altered neuronal activity, axonal degeneration, neurogliosis, altered transport, metabolic impairment and at last cellular death.[8]

The anaesthetic management in a gross hydrocephalic child can be particularly challenging due to complex issues such as difficulties related to airway management, cardiovascular dysfunction, age-related needs of positioning and congenital anomalies. Proper preparation and positioning of children are mandatory for successful intubation.[1],[2]

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Conflicts of interest

The authors are faculty members/Postgraduate students/ residents of Sri Venkateswara Institute of Medical Sciences, Tirupati, of which Journal of Clinical and Scientific Research is the official Publication. The article was subject to the journal's standard procedures, with peer review handled independently of these faculty and their research groups.

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