Meningomyelocele

Meningomyelocele

Meningomyelocele (myelomeningocele) This is a very rare, severe form of spina bifida in which the spinal cord, nerves and meninges are literally hanging out of a hole in the spine, usually at birth. A surgical correction should be performed sufficiently early to prevent infection and further nerve injury. 

• Comes as a result of not closing the neural tube in the 3rd - 4th week of pregnancy.
• Skin does not cover the bare part of the neural tissue.
• Causes irreversible foci of neurological lesions.

Meningomyelocele symptoms

Signs and symptoms of meningomyelocele (myelomeningocele) vary based upon the position and size of the spinal defect, though most present at birth. 

Visible Symptoms at Birth

• On the back (in the majority of cases at the lower spine), there is sac-like swelling.
• Sac can either be open or have a thin membrane covering it.
• Lying nervous system or nerves.
• There may not be skin covering the lesion.

Neurological Symptoms

• Burnout or lack of the legs
• Below the level of the defect, loss of sensation.
• Absent or reduced reflexes
• Delayed motor development
• In extreme situations, the inability to walk.

Symptoms of bowel and bladder dysfunction

• Neurogenic bladder 
• Urinary incontinence
• Urinary retention
• Repeat urinary tract infections.
• Bowel incontinence
• Chronic constipation

Orthopedic Symptoms

• Clubfoot
• Hip dislocation
• Limb deformities
• Scoliosis or kyphosis
• Joint contractures

Symptoms Of Brain And Nervous System Disruption

• Hydrocephalus (enlargement of the head, bulging of fontanelle) 
• Arnold Chiari II malformation.
• Seizures (occasionally)
• Difficulty breathing (high lesions) or swallowing.

Skin and Sensory Symptoms

• Paralysis of pain and temperature below lesion.
• Reduced sensation pressure ulcers.

Correlative Developmental Symptoms

• Learning problems (typically caused by hydrocephalus)
• Delayed milestones

Symptoms by Level of Lesion

• Thoracic: No walking, severe paralysis.
• Lumbar: Partial mobility of the legs, can walk with the help.
• Sacral: Mild weakness, improved bladder/bowel control.

Meningomyelocele treatment

Surgical intervention is necessary in male meningocele patients, and such patients must be managed on a lifelong basis by a multidisciplinary team to prevent infections, protect neural tissue, and address chronic complications. 

Emergency postnatal care 

• Keep the baby prone
• Apply sterile saline-dipped dressing over the sac.
• Maintain body temperature
• Preventive antibiotics of wide spectrum.
• Sacral rupture or pressure should be avoided.
• Close neurological examination.

Unconditional Operative Care

Postnatal Surgical Repair

• Timing: Within 24–72 hours of birth

Procedure includes:

• Excision of the sac
• Laminectomy if required
• Dissociation of neural tissue.
• Watertight dural closure
• Skin closure

Goals:

• Prevent meningitis
• Conserve residual nerve activity.
• Prevent CSF leakage
• Prenatal (Fetal) Surgery (Chosen Cases)
• Conducted 19-26 weeks of gestation.

Benefits:

• Reduced need for VP shunt
• Improved motor outcomes

Cure of Co-morbidities 

• Hydrocephalus
• Ventriculoperitoneal shanty.
• Endoscopic third ventriculostomy (in cases of choice)

Bladder Dysfunction

• Clean intermittent catheterization (CIC).
• Anticholinergic medications
• Bladder augmentation (afterward as required)

Bowel Management

• Bowel training programs
• Laxatives or enemas
• Severe cases Antegrade continence enema (ACE).

Orthopedic Problems

• Physiotherapy
• Bracing
• Corrective orthopedic treatment.

Rehabilitation and Aftercare for the Long Term

• Physical and occupational therapy.
• Mobility aids 
• Regular urology checks to save kidneys. 
• Prevention of pressure sores in skin care.
• Neurodevelopmental monitoring

Meningomyelocele surgery

Meningomyelocele surgery is a life-saving and protective surgery that seeks to close the spinal defect, avoid infection, and preserve any remaining nerve action. It cannot undo the damage to the nervous system that is already present, but it can help to avoid further damage.

Indications for Surgery

• All infants containing meningomyelocele (incompatible with life)
• Leaking or ruptured sac
• Meningitis and CSF leak prevention.

Timing of Surgery

• Postnatal repair: 24-72 hours (ideal) postnatally.
• Emergency operation: In case sac is ruptured or infected.
• Prenatal (fetal) repair: Indications between 19 and 26 weeks gestation. 

Preparation before Surgery

• Baby kept prone
• Sac swapped with sterile saline-wet gauze.
• Biotics of a broad spectrum, IV.
• Keep warm and keep hydrated.
• Examination of the nervous system and of the urine apparatus.
• Cranial ultrasound/MRI for assessment of hydrocephalus. 

Procedure

Positioning

• General anaesthetic prone positioning.

Exposure

• Sacral incision.
• Neural tissue preservation by careful dissection.

Neural Tissue Management

• Spinal cord and nerve roots identification.
• Neural placade uncusation.
• Without alienation, nonfunctional tissue.

Laminectomy

• Laminectomy as minimal as necessary to liberate neural tissue.

Dural Closure

• Native dura watertight dural repair or graft.
• Prevents CSF leakage

Soft Tissue & Skin Closure

• Fascial layers and muscle closed.
• Skin closure (can have rotational flaps in big defects)

Associated Procedures

• Ventriculoperitoneal hydrocephalus (same sitting or later) shunt.
• Endoscopic third ventriculostomy (chosen cases)

Postoperative Care

• Continue IV antibiotics
• Prone or lateral positioning.
• Monitoring and wound care of CSF leak.
• Follow-up head circumference (hydrocephalus)
• Bladder capability measurement.
• Early physiotherapy

Best hospital for meningomyelocele India

• Artemis Hospital, Gurgaon
• Medanta-The Medicity, Gurgaon
• Fortis Memorial Research Institute, Gurgaon
• Max Hospital, Saket

Conclusion

Meningomyelocele is a critical, birth defect of the neural tube, and it requires immediate operation and lifelong care. Operation before 24-72 hours has been considered essential to shield the exposed neural tissue from trauma, contamination, and secondary problems such as meningitis and CSF leakage. Although surgery cannot undo neurological impairment, it is important in ensuring that there is no more progressive impairment and chance of survival. The outcome of the spinal lesion level, the presence of hydrocephalus and the quality of the postoperative rehabilitation and the follow-up are the determinants of the long-term results. Through early surgery, the proper treatment of related disorders (hydrocephalus, bladder and bowel dysfunction, orthopedic issues), and extensive rehabilitation, most children with meningomyelocele will be able to attain a higher quality of life. Periconceptional folic acid is the most effective prevention intervention in order to lower the incidence of this condition.

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FAQ

1. Does spina bifida include meningomyelocele?

• The meningomyelocele is the most severe form of spina bifida. The others are spina bifida occulta and meningocele and are less serious. 

2. Is meningomyelocele diagnosable in pregnancy?

• Yes. It is identified by the high maternal serum AFP, prenatal ultrasound and checked by MRI of the fetus.

3. Should every infant with meningomyelocele undergo operation?

• Yes. Surgical closure is indicated Early closure is indicated to prevent infection, preserve neural tissue and minimize complications.

4. Does surgery needed for meningomyelocoele? 

• No. Surgery does not restore nerve loss but allows further damage to be prevented.

5. Will the child be able to walk?

• The ability of walking is determined by the amount of lesion of the spinal region. Better chances are offered to children with low (sacral) lesions as compared to high lesions.

6. Will there be problems with bladder and bowels? 

• Yes. Neurogenic bladder and bowel are seen in many children and the functional needs for heart kidney and enable continence. Long-term management is required to protect the kidneys and to enable the child to become continent.

7. What is the life-expectancy of a child born with meningomyelocele? 

• In the era of medical surgery and follow-up most people will live to adulthood and be well taken care of.

8. Is meningocele prenatal?

• Yes, many cases could be prevented with periconceptional folic acid supplementation (prior to and in early pregnancy).