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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 21  |  Issue : 2  |  Page : 189-192

Anesthetic challenges in leaking meningomyelocele repair in a neonate


Department of Anesthesiology and Critical Care, INHS Asvini, Mumbai, Maharashtra, India

Date of Submission31-Oct-2018
Date of Acceptance08-Feb-2019
Date of Web Publication07-Oct-2019

Correspondence Address:
Surg Cdr (Dr) Vidhu Bhatnagar
Department of Anesthesiology and Critical Care, INHS Asvini, Near RC Church, Colaba, Mumbai - 400 005, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmms.jmms_71_18

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  Abstract 


Meningomyelocele is a neural tube defect, which comprises of a saccular protrusion containing a neural placode bathed in cerebral spinal fluid. Arachnoid covers the surface of the sac and dura or skin is missing. This is a congenital spinal anomaly, a type of spina bifida where there is a failure of fusion of spine. Early surgery is advisable because of increased risk of infection and further damage to nervous tissue. Anesthetic challenges for meningomyelocele surgical repair in a neonate include intubation with proper positioning to avoid rupture of sac, intraoperative prone position ventilation and its related complications maintenance of anesthesia, temperature control, and fluid management. We present a case report of anesthetic challenge of a 2-day-old neonate for detethering and repair of lumbosacral meningomyelocele.

Keywords: Arnold–Chiari malformation, hypothermia, intratracheal, intubation, meningomyelocele, neural tube defect, prone position, spinal dysraphism


How to cite this article:
Bhatnagar V, Kulkarni S N, Muzammil M D. Anesthetic challenges in leaking meningomyelocele repair in a neonate. J Mar Med Soc 2019;21:189-92

How to cite this URL:
Bhatnagar V, Kulkarni S N, Muzammil M D. Anesthetic challenges in leaking meningomyelocele repair in a neonate. J Mar Med Soc [serial online] 2019 [cited 2019 Dec 9];21:189-92. Available from: http://www.marinemedicalsociety.in/text.asp?2019/21/2/189/268630




  Introduction Top


Meningomyelocele is a congenital spinal anomaly resulting from the failure of neural tube to fuse in fetus which leads to herniation of neural elements and cerebrospinal fluid (CSF). The incidence of meningomyelocele is one in 1000 live births approximately. It is often a saccular protrusion which contains a neural placode which is enveloped by CSF.[1] The sac is covered by arachnoid while dura and skin are missing. Many fragile vessels are embedded in arachnoid, and the spinal cord remains tethered to the bony defect in spine. Different symptoms such as loss of bladder and bowel functions arise due to tethering of spinal cord.[2] Motor and sensory functions may also be impaired. Various other anomalies associated with meningomyelocele are Chiari II malformation, hydrocephalus, syringomyelia, brainstem malformations (cranial nerve), and orthopedic deformities. There is risk of meningitis because of exposed meninges.[2],[3]


  Case Report Top


A newborn male baby, weighing 3.90 kg born to a 29-year-old primigravida mother at 38 weeks 2 days of gestation by spontaneous vaginal delivery. The baby cried immediately after birth, Apgar score was seven and nine at 1–5 min, respectively. The baby noted to have spinal dysraphism (lumbosacral meningomyelocele) and shifted to the neonatal intensive care unit (NICU) for further management.

Maternal history revealed an immunized and regularly followed up antenatal case. No history of other illness or medication during antenatal period. Antenatal ultrasonography in first and second trimester did not detect any anomalies.

On examination, neonate was afebrile, heart rate (HR) of 150/min, respiratory rate (RR) was 46/min, room air saturation was 98%. No pallor, icterus, cyanosis, and no VACTERL malformation were noted. Cardiovascular system, abdomen, and respiratory system were essentially normal, and the neonate was moving all four limbs.

The spinal dysraphism on examination was a cystic, pedunculated swelling (4 cm × 5 cm) at lumbosacral region. Small excoriation was present on swelling; no neural elements could be visible on naked eye examination; however, moistness on the dysraphism was noted suggesting CSF leak [Figure 1] and [Figure 2]. Neonate also had element of Chiari II malformation.
Figure 1: Spinal dysraphism, cystic swelling pedunculated 4 cm × 5 cm at lumbosacral region. Small excoriation present on swelling, no neural element seen and no obvious cerebrospinal fluid leakage

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Figure 2: Spinal dysraphism, cystic swelling pedunculated 4 cm × 5 cm at lumbosacral region. Small excoriation present on swelling, no neural element seen

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Investigations revealed hemoglobin of 19 g%, a raised TLC-23910 cells/cumm; platelet count, renal and hepatic function tests were within normal limits. Magnetic resonance imaging lumbar spine revealed lumbar meningomyelocele, low lying conus/tethered cord, and suspicious intraventricular hemorrhage [Figure 3]. A two-dimensional echocardiography did not exhibit any structural abnormality in the heart. Neonate was taken up for emergency surgical repair in view of CSF leak. Neonate was kept nil orally for 4 h in the morning and maintenance fluid (10% dextrose) was started in NICU. The patient was brought in a prewarmed operating room (OR) and placed on doughnut padding to prevent any injury to meningomyelocele. The monitoring (electrocardiogram, HR, noninvasive blood pressure, pulse oximeter, and temperature) was applied. Neonate was induced with oxygen, air, and Sevoflurane. The neonate was endotracheally intubated with size 3.0, uncuffed endotracheal tube (ETT) using Fentanyl 3 microgram intravenously (iv), propofol 5 mg iv, and atracurium 2 mg. After confirming bilateral auscultation and capnogram, the ETT was fixed at 9.5 cm on the right angle of the mouth, and a throat pack was inserted for stabilizing the ETT. The bilateral air entry was again checked by flexing the neck. Neonate put in prone position with rolls under chest and pelvis and eye padding applied. Air entry checked again after prone position. All exposed parts of neonate were wrapped in cotton for the conservation of body heat. The head was wrapped with abdominal towels. All endeavors were carried out to maintain normothermia.
Figure 3: Magnetic resonance imaging lumbar spine revealed lumbar meningomyelocele, low lying conus/tethered cord, suspicious intraventricular hemorrhage

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Anesthesia was maintained with O2+ Air + Sevoflurane (minimum alveolar concentration of 0.7–0.9), injection atracurium 1.5 mg iv in divided doses, fentanyl 2 microgram in divided doses, and propofol 5 mg. Urine output was checked by weighing the soaked cotton at the conclusion of surgery which came to around 25 mL. The maintenance iv fluid was 1% lactated ringers and a volume of 82 mL was transfused intraoperatively in 200 min. Total blood loss was approximately 40 mL. Surgical duration was 185 min. After the completion of surgery, neonate was turned supine and reversed with 200 mg neostigmine and 40 mg glycopyrrolate and extubated once adequate respiratory efforts and recovery was present. The recovery was complete in OR and neonate was actively moving all four limbs. The neonate was monitored in NICU for neurological status for next 7 days intensively for apneic spells, ventilator difficulties, and brainstem herniation symptoms.


  Discussion Top


Neural tube defects (NTD) are common congenital anomaly which can involve any part of the vertebral column. NTDs arise from failed migration of neural crest cells. The cranial and caudal ends of the neural tube close in the last, thus, any interruptions in neural crest cell migration lead to variable defects. Cranial closure failure leads to exencephaly/anencephaly which is a fatal condition. Caudal neuropore closure failure results in open defects such as meningocele and meningomyelocele or spina bifida cystica.[4],[5] Meningomyelocele is the most common NTD which is compatible with life (an incidence of 0.44–1 per 1000 live births).[4],[5] After the failed closure, agenesis of fetal vertebral arches leads to spinal cord, nerve roots, and meninges protrusion in a sac that bulges in the lumbar region. Around 80% of meningomyelocele defects arise in lumbosacral area.[5],[6],[7],[8] Neurological deficits distal to the defect are more severe. Urologic symptoms (incontinence) and orthopedic symptoms (abnormalities of lower limbs and numbness in lower limbs) may occur in children who have uncorrected NTDs due to tethering of spinal cord by sacral nerve roots. Various other anomalies are also associated with meningomyelocele such as Chiari Type II malformation, hydrocephalus, syringomyelia, brainstem malformations, cerebral ventricle abnormalities, cerebellar heterotopias, and agenesis of the corpus callosum. The exposed sac greatly increases the risk of infection in the neonatal period.[2],[5],[6],[7],[8]

To reestablish normal alignment of spine an early surgery is advisable, and risk of infection also decreases.[3],[8],[9] Various challenges are anticipated during surgery such as rupture of sac if proper care not taken to pad, challenges encountered in positioning and airway management, estimation of blood loss, temperature regulation and avoidance of hypothermia, increased chances of delayed awakening, and apneic spells. Temperature regulation is very important in these neonates/infants because autonomic control below the level of the defect is abnormal.[6],[7] Surgical repair requires neurosurgical dural repair, dethethering of spinal cord, and then soft-tissue coverage.[9]

Our patient was a 2-day-old neonate, and thus, all the challenges of a neonate coming for emergency neurosurgery were omnipotent. The care for cautious positioning, eye care, and airway management, titrated fluid management, blood loss management and maintenance of normothermia, titration of opioid and pain relief and adequate recovery were all alertly anticipated and vigilantly managed. After the neurosurgical repair in our patient, the defect could be closed by primary repair and postoperative recovery exhibited by the neonate was remarkable. Anesthetic management of a 48-hour-old neonate for meningomyelocele repair is fraught with various complication which could be preventable and nonpreventable. While complications such as accidental extubation, dislodgement of venous access, periorbital edema, and hypothermia are preventable with adequate precautionary measures but certain complications such as bradycardia and cardiac arrest due to brainstem compression, inspiratory stridor and respiratory arrest or requirement of prolonged postoperative ventilation due to associated  Chiari malformation More Details cannot be prevented but a vigilant eye and expert care can help divert catastrophe.[10]

There are many studies regarding challenges and complications in pediatric neurosurgical procedures and meningomyelocele repairs, but none of the patients were as young as 48 h like ours. Although our patient did not have any complications perioperatively, we wanted to highlight the importance of expert planning, skilled execution, added vigilance, and proficient care in the management of a case which could turn into a nightmare.


  Conclusion Top


Anesthesia for a pediatric population undergoing neurosurgery requires not only the knowledge of cerebral pathophysiology and deep understanding of pharmacokinetics and pharmacodynamics of anesthetic agents but also an extra dose of vigilance, prempting complications, and intense readiness for the management of perioperative challenges for a successful outcome as was highlighted in our case.

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.

Acknowledgments

We would lie to acknowledge for our neurosurgeon, Gp Capt (Dr) SGS Datta for his expertise and cooperation when it comes to careful planning required for the little patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sadler TW. Embryology of neural tube development. Am J Med Genet C Semin Med Genet 2005;135C: 2-8.  Back to cited text no. 1
    
2.
Nogueira M, Greenfield SP, Wan J, Santana A, Li V. Tethered cord in children: A clinical classification with urodynamic correlation. J Urol 2004;172:1677-80.  Back to cited text no. 2
    
3.
Rinaldi F, Cioffi FA, Columbano L, Krasagakis G, Bernini FP. Tethered cord syndrome. J Neurosurg Sci 2005;49:131-5.  Back to cited text no. 3
    
4.
Wilde JJ, Petersen JR, Niswander L. Genetic, epigenetic, and environmental contributions to neural tube closure. Annu Rev Genet 2014;48:583-611.  Back to cited text no. 4
    
5.
Northrup H, Volcik KA. Spina bifida and other neural tube defects. Curr Probl Pediatr 2000;30:313-32.  Back to cited text no. 5
    
6.
Lerman J, Cote CJ, Steward DJ. Anesthesia for specific procedures-neurosurgery and invasive neuroradiology. In: Manual of Paediatric Anesthesia. 6th ed. Amsterdam: Elsevier Churchill Livingstone; 2010. p. 251.  Back to cited text no. 6
    
7.
Krane EJ, Philip BM, Yeh KK, Domino DB. Anesthesia for pediatric neurosurgery. In: Motoyamma EK, Davis PJ, editors. Smith's Anaesthesia for Infants and Children. 7th ed. Atlanta: Mosby; 2005.  Back to cited text no. 7
    
8.
Detrait ER, George TM, Etchevers HC, Gilbert JR, Vekemans M, Speer MC, et al. Human neural tube defects: Developmental biology, epidemiology, and genetics. Neurotoxicol Teratol 2005;27:515-24.  Back to cited text no. 8
    
9.
Kobraei EM, Ricci JA, Vasconez HC, Rinker BD. A comparison of techniques for myelomeningocele defect closure in the neonatal period. Childs Nerv Syst 2014;30:1535-41.  Back to cited text no. 9
    
10.
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.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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