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Myelomeningocele is a neural tube defect characterized by failure of the spinal cord to fuse posteriorly during primary neurulation. Commonly located in the thoracolumbar spine and rarely in the cervical spine. This congenital abnormality of the spinal cord results in a saccular protrusion near the base of the spine. The sac contains neural elements and CSF. Immediate repair of the sac and covering of the defect with skin is desirable to preserve neurological function and avoid infections.

{{Infobox surgical procedure
| anesthesia_type = General
| airway = ETT
| lines_access = PIV
| monitors = Standard
| considerations_preoperative = Assessment of other present congenital anomalies
| considerations_intraoperative = Latex Precautions
| considerations_postoperative = Prone positioning required for healing
NICU/PICU Bed
}}

== Overview ==

=== Indications ===
Presence of myelomeningocele upon birth. Can be detected before birth by high resolution ultrasound and/or elevated maternal serum alpha fetoprotein, as well as fetal MRI.

=== Surgical procedure ===
Fundamental goals of the procedure are to preserve neural tissue, reconstruct a normal intrathecal environment, and complete skin closure to prevent a spinal fluid leak and meningitis. Closure is recommended within 72 hours of birth. The defect is dissected so that the various anatomic layers can be separated. The incision will be surround the defect, preserving skin that can be utilized during the closure. Will require operating microscope.

== Preoperative management ==

=== Patient evaluation ===
Screen patient for presence of other congenital anomalies and should/can be completed 24 hours post birth.
{| class="wikitable"
|+
!System
!Considerations
|-
|Airway
|Craniofacial Abnormalities
|-
|Neurologic
|Hydrocephalus, Chiari Malformations, Motor/Sensory Defects
|-
|Cardiovascular
|ASD, VSD
|-
|Pulmonary
|
|-
|Gastrointestinal
|Malrotation of gut
|-
|Hematologic
|
|-
|Renal
|Hydronephrosis
|-
|Endocrine
|
|-
|Other
|Premature infant has independent risk factors
|}

=== Labs and studies ===
Echo, Renal Ultrasound

=== Operating room setup ===
Latex Precautions

=== Patient preparation and premedication ===
None indicated for procedure specifically

Age group of typical patient does not require premedication with Midazolam.

== Intraoperative management ==

=== Monitoring and access ===
1 PIV

Potentially require 2 on case by case basis

=== Induction and airway management ===
Positioning during induction: supine- protect defect with sterile donut or rolls to prevent pressure or rupture of the defect.

Typically patient will come to OR with IV access. If so- proceed with standard IV induction. If no IV placed, proceed with standard inhalation induction and IV placement. Sevo preferred for standard inhalation induction due to low blood-gas partition coefficient and absence of airway irritability.

Consider administration of atropine 0.02 mg/kg with induction to prevent bradycardia.

Neuromuscular blockade is indicated for this procedure. Consider use of rocuronium 0.6-1 mg/kg or vecuronium 0.1 mg/kg.

Antibiotics: Ceftriaxone 50 mg/kg IV or Vancomycin 15 mg/kg IV and infused over 60 minutes to reduce risk of rapid administration complications.

=== Positioning ===
Prone with rolls under chest and hips. Dependent on size of infant, consider use of head ring for positioning infant with no compression of myelomeningocele for induction and prone positioning.

=== Maintenance and surgical considerations ===
Warming measures and maintaining normothermia for infant. Warm room to 75-80 degrees F.

Sevoflurane 2-3% or Isoflurane 1-2%

Consider redosing of rocuronium (0.3 mg/kg) or vecuronium (0.05 mg/kg) as needed.

=== Emergence ===
Underwent simple repair- reverse and extubate patient.

Underwent complex repair with fascial releases and tight abdomen- remain intubated for 1st 24 hours.

== Postoperative management ==

=== Pain management ===
IV Opioids

== Procedure variants ==

{| class="wikitable wikitable-horizontal-scroll"
|+
!
!Variant 1
|-
|Unique considerations
|Commonly associated with Chiari malformations that require VP shunt placement post myelomeningocele repair
|-
|Position
|Prone
|-
|Surgical time
|1.5 -3 hours
|-
|EBL
|Negligible-25 ml
|-
|Postoperative disposition
|Neonatal Nursery
|-
|Pain management
|Pain Score 3-5
|-
|Potential complications
|Meningitis, Ventriculitis, Wound Infection, CSF Leak, Hydrocephalus, Renal Failure, Respiratory compromise from tight abdomen
|}

== References ==

[[Category:Surgical procedures]]
[[Category:Pediatrics]]
[[Category:Neurosurgery]]

Closure of Myelomeningocele - Revision history

Aioannou: Added article