|Year : 2020 | Volume
| Issue : 1 | Page : 50-53
An infant with hydrocephalus and lumbar meningomyelocele for ventriculoperitoneal shunt insertion: Concerns of an anesthesiologist
Amoolya Kamalnath, Ramyavel Thangavelu, Mamie Zachariah, Ranjan R Venkatesh
Department of Anaesthesiology, Pondicherry Institute of Medical Sciences, Puducherry, India
|Date of Submission||29-Aug-2019|
|Date of Acceptance||29-Nov-2019|
|Date of Web Publication||23-Jan-2020|
Dr. Ramyavel Thangavelu
Department of Anaesthesiology, Pondicherry Institute of Medical Sciences, Kalathumettupathai, Ganapathichettikulam, Village 20, Kalapet, Puducherry
Source of Support: None, Conflict of Interest: None
The anatomical and physiological differences in children make the administration of anesthesia difficult compared to the adults which is made more challenging when the child presents with a congenital anomaly. In this case, we report the anesthetic challenges presented by an 8-month-old baby born of full gestation with a lumbar swelling and hydrocephalus posted for ventriculoperitoneal shunt insertion. Magnetic resonance imaging scan revealed the presence of a large meningomyelocele with tethered sac and Arnold–Chiari malformation (Type 2). Anesthetic management included a thorough preoperative assessment of associated comorbidity, proper positioning, management of a difficult airway, prevention of any rise in intracranial pressure, and prevention of hypothermia. The perioperative period was uneventful, and the child recovered well.
Keywords: Arnold–Chiari malformation, congenital anomaly, hypothermia, ventriculoperitoneal shunt
|How to cite this article:|
Kamalnath A, Thangavelu R, Zachariah M, Venkatesh RR. An infant with hydrocephalus and lumbar meningomyelocele for ventriculoperitoneal shunt insertion: Concerns of an anesthesiologist. Indian J Health Sci Biomed Res 2020;13:50-3
|How to cite this URL:|
Kamalnath A, Thangavelu R, Zachariah M, Venkatesh RR. An infant with hydrocephalus and lumbar meningomyelocele for ventriculoperitoneal shunt insertion: Concerns of an anesthesiologist. Indian J Health Sci Biomed Res [serial online] 2020 [cited 2020 Jun 1];13:50-3. Available from: http://www.ijournalhs.org/text.asp?2020/13/1/50/276419
| Introduction|| |
Meningomyelocele, one of the neural tube defect (NTD) variants, is a condition in which the meninges and neural tissue protrude through a defect in vertebral arch and form a cyst-like sac with an incidence of 0.5–11/1000 live births. Hydrocephalus is a major problem for majority of patients with meningomyelocele due to the abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles of the brain. Meticulous preoperative evaluation with planning of the anesthetic technique, especially in regard to managing the airway, plays a crucial role in successful surgical management of these cases. Here, we report a case of lumbar meningomyelocele with hydrocephalus posted for an elective ventriculoperitoneal (VP) shunt procedure.
| Case Report|| |
A 9-month-old male baby born out of elective lower segment cesarean section at term gestation weighed 2 kg at birth. The child was born with macrocephaly with a skin-covered swelling at the lumbosacral region. Antenatal history did not reveal any history of maternal smoking, herpes simplex virus infection, or substance abuse. Prenatal ultrasound revealed a spina bifida.
The child was brought to the casualty at 9 months of age with a progressive increase in head size. There was no history of vomiting or weakness of limbs. The patient was scheduled for elective VP shunt insertion for hydrocephalus. On preanesthetic evaluation, the child had macrocephaly with a head circumference of 54 cm, and a 4 cm × 3 cm skin covered swelling in the lumbosacral region [Figure 1] with a grossly delayed milestones. The child was lethargic with presence of pallor, a tense anterior fontanelle with frontal and parietal bossing, a positive sunset sign, and dilated scalp veins. There were no obvious neurological deficits. Investigations revealed hemoglobin (Hb) of 6 g/dl (microcytic hypochromic anemia). Hundred milliliters of packed cells were transfused preoperatively in accordance with the guidelines recommended for transfusion of red blood cells in the pediatrics and the Hb improved to 9 g/dl. Magnetic resonance imaging (MRI) scan of the brain showed enlargement of the third and lateral ventricles with a large lumbar lipomeningomyelocele with tethered sac and cerebellar tonsils herniating the foramen magnum suggestive of Arnold–Chiari malformation (type 2) [Figure 2].
|Figure 1: Preoperative photo showing hydrocephalus with lumbar meningomyelocele|
Click here to view
|Figure 2: Magnetic resonance imaging showing a lumbar meningomyelocele with hydrocephalus|
Click here to view
In preparation for induction, the lumbar meningomyelocele warranted careful attention to positioning. The difficult airway cart and alternative management plan were kept ready in anticipation of difficult intubation. No sedative premedication was administered to the child in the preoperative period. On shifting the patient to Operation theatre (OT) table, the child was placed supine and the body elevated with a ramp of folded sheets leaving space for the meningomyelocele at the back to hang freely without any external pressure underneath. The head was placed on a padded gel ring to obtain an optimal sniffing position [Figure 3]. An inhalation induction with graded concentration of sevoflurane with oxygen was performed maintaining spontaneous respiration and an intravenous (IV) cannula secured. The patient was premedicated with injection midazolam 0.05 mg/kg IV and injection fentanyl 2 mcg/kg IV and induced with injection thiopentone 4 mg/kg IV. After checking for effective bag-mask ventilation, injection atracurium 5 mg/kg was given IV. Direct laryngoscopy was done using a Size 1 Macintosh blade (Grade 1 view) with minimal external laryngeal manipulation, and a 3.5-cuffed endotracheal tube was placed. Anesthesia was maintained with oxygen, nitrous oxide, sevoflurane mixture, and atracurium boluses with a controlled ventilation. Forced air warmer was used along with cotton pads to cover up the limbs of the baby to prevent hypothermia. During the process of tunneling during VP shunt procedure, an additional fentanyl bolus was administered. Throughout the intraoperative period, the vitals remained stable, and adequate care was taken to prevent any increase in intracranial pressure (ICP). At the end of the procedure, the patient was reversed and extubated uneventfully after the child was awake and showed adequate signs of respiration. Postoperatively, the child was shifted to the intensive care unit for observation, and the stay was uneventful.
|Figure 3: The child being induced supine with body elevated with a ramp of folded sheets and head in optimal sniffing position|
Click here to view
| Discussion|| |
Congenital hydrocephalus can be genetic or may be commonly associated with NTD such as spina bifida, meningomyelocele, and Arnold–Chiari malformation and syndromes such as trisomies 3, 18, 9, 9p. Congenital hydrocephalus is common in patients with open NTD, association with closed NTD are rarely reported. Our patient presented with a skin covered midline lumbar swelling at the level of L5-S1 (closed NTD). MRI scan demonstrated a lipomyelomeningocele. The hydrocephalus was tense with bulging fontanelles, and hence, the patient was posted for VP shunt procedure. When obtaining a preoperative history, associated comorbidities, neurological deficits, and signs of raised ICP (persistent vomiting, frontal bossing, dilated scalp veins, and cranial nerve palsies) should be looked for and assessed appropriately. Frequent vomiting episodes may lead to dehydration, electrolyte imbalance, and increased risk of aspiration. Furthermore, since these babies are often born prematurely and have low birth weight, there can be associated jaundice, anemia, coagulopathy, and cardiorespiratory disease. The infant in our case had severe anemia with Hb of 6 g/dl on preoperative evaluation. Hb improved on transfusion of packed cells preoperatively.
A solemn issue that often concerns the anesthesiologist, especially in a child with hydrocephalus is managing the airway. Pediatric airway is considered more difficult than adults because of large head and tongue, omega-shaped epiglottis, anterior larynx, and the subglottis being the narrowest part. In congenital hydrocephalus, macrocephaly places the neck in extreme flexion, and the large forehead may impede the line of sight with laryngoscopy. Difficult airway cart and alternative management plan were kept ready in anticipation of difficult intubation in our case. We achieved an optimal position for intubation by placing the child supine and elevating the body with folded towels leaving space for the meningomyelocele. The positioning facilitated a neutral position free of flexion and the attempted laryngoscopy yielded a Grade 1 view. Excessive flexion or extension of head might cause brain stem compression in Chiari malformation, and hence, a gentle laryngoscopy is required. An IV induction with thiopentone or propofol and neuromuscular blockade to facilitate intubation is ideal in children with raised ICP. Since our patient had no IV access preoperatively, we went ahead with an inhalational induction with sevoflurane and maintained with low end-tidal volatile agents, opioids, N2O, and controlled ventilation. Preparation for elevated ICP should be undertaken, including avoidance of hypercarbia, hypoxia, treatment of hypotension, hyperventilation, and avoidance of positive end-expiratory pressure to prevent venous congestion.
Forced-air warming, warm IV fluids, and cotton pads were used to cover up the limbs of the infant as shunt surgery requires exposure of the body from the head to abdomen, thus predisposing neonates and small infants to hypothermia. During the shunt procedure, hemodynamic changes during tunneling can be managed by increasing the anesthetic depth or administering a small dose of narcotic. On initial cannulation of the ventricle, bradycardia and abrupt fall in blood pressure may be encountered. Altering the surgeon and pausing for a while or injection atropine might be needed in some cases for its resolution. Electrolyte imbalances, accidental pneumothorax, and arrhythmias may be seen in the postoperative period due to the removal of large quantity of CSF. The infant in our case was kept under careful vigilance, and the recovery was uneventful with a significant decrease in head circumference postoperatively.
| Conclusion|| |
NTDs with congenital hydrocephalus present multiple challenges to the anesthesiologist. With meticulous planning and adequate preparation, one can optimize these issues and deliver a safe anesthesia with a reduced perioperative morbidity and mortality.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We would like to thank Dr. Manuj Kumar, junior resident, Department of Anaesthesiology, for his contribution.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Rath GP, Dash HH. Anaesthesia for neurosurgical procedures in paediatric patients. Indian J Anaesth 2012;56:502-10.
] [Full text]
Vinchon M, Rekate H, Kulkarni AV. Pediatric hydrocephalus outcomes: A review. Fluids Barriers CNS 2012;9:18.
Roseff SD, Luban NL, Manno CS. Guidelines for assessing appropriateness of pediatric transfusion. Transfusion 2002;42:1398-413.
McAllister JP 2nd
. Pathophysiology of congenital and neonatal hydrocephalus. Semin Fetal Neonatal Med 2012;17:285-94.
Chance A, Sandberg DI. Hydrocephalus in patients with closed neural tube defects. Childs Nerv Syst 2015;31:329-32.
Singh D, Rath GP, Dash HH, Bithal PK. Anesthetic concerns and perioperative complications in repair of myelomeningocele: A retrospective review of 135 cases. J Neurosurg Anesthesiol 2010;22:11-5.
Hamid RK, Newfield P. Pediatric neuroanesthesia. Hydrocephalus. Anesthesiol Clin North Am 2001;19:207-18.
Vagyannavar R, Bharti V, Hashim M. Difficult airway in a case of gross hydrocephalus for shunt surgery. Anesth Essays Res 2017;11:1109-11.
] [Full text]
Hooper VD, Chard R, Clifford T, Fetzer S, Fossum S, Godden B, et al
. ASPAN's evidence-based clinical practice guideline for the promotion of perioperative normothermia: Second edition. J Perianesth Nurs 2010;25:346-65.
Alfery DD, Shapiro HM, Gagnon RL. Cardiac arrest following rapid drainage of cerebrospinal fluid in a patient with hydrocephalus. Anesthesiology 1980;52:443-4.
[Figure 1], [Figure 2], [Figure 3]