Myelomeningocele Overview

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Questions and Answers

What is myelomeningocele classified as?

A defect that does not involve any neural tissue.

A congenital malformation that only affects the vertebral column.

A herniated meningeal sac containing neural tissue. (correct)

A type of spina bifida where the defect is covered with skin.

What characterizes spina bifida occulta?

It is associated with visible neural tissue at birth.

The defect is covered with intact skin but may have underlying deformities. (correct)

It always leads to immediate surgical intervention upon diagnosis.

The defect occurs without any skin covering.

How is myelomeningocele typically diagnosed?

Using ultrasonography in the prenatal period. (correct)

By analyzing genetic markers postnatally.

Through physical examination only after birth.

Through MRI scans performed at the age of 2.

What is the recommended surgical intervention timeframe after birth for myelomeningocele?

Within 24 hours. (A) Signup and view all the answers

What condition is commonly associated with hydrocephalus in myelomeningocele cases?

Type II Chiari malformation. (D) Signup and view all the answers

What is the prevalence rate of myelomeningocele?

Approximately 4 per 10,000 live births. (A) Signup and view all the answers

What is the definition of spina bifida cystic?

A defect lacking skin covering present at birth. (A) Signup and view all the answers

Which form of spina bifida contains a herniated meningeal sac without neural tissue?

Meningocele. (B) Signup and view all the answers

What should be considered for transfusion if the allowable blood loss is exceeded during surgery?

Packed red blood cells (D) Signup and view all the answers

What is required for neonates with myelomeningocele regarding their surgical environment?

Latex-free operating room (D) Signup and view all the answers

What is a common postoperative complication for patients with Arnold-Chiari malformation?

Ventilatory responses to hypoxia may be impaired (C) Signup and view all the answers

Children with which condition may require tracheostomy and gastrostomy?

Vocal cord paralysis (D) Signup and view all the answers

What may occur in the postoperative period after a tight skin closure?

Breathing difficulties (A) Signup and view all the answers

What should be replaced to prevent insensible fluid loss during surgery?

Warmed crystalloids (A) Signup and view all the answers

What potential effect does extreme head flexion have on children regarding health outcomes?

Brainstem compression (D) Signup and view all the answers

What dietary sensitivity might children with a latex allergy exhibit?

Certain fruits like kiwi and bananas (A) Signup and view all the answers

What is the initial surgical procedure often required for patients with Type II Chiari malformation?

Ventriculoperitoneal shunt placement (C) Signup and view all the answers

Why are neonates with myelodysplasia at high risk for developing latex allergy?

Frequent surgical procedures using latex gloves (D) Signup and view all the answers

What is a critical consideration for the operating room before the arrival of the patient?

Prewarming the operating room (D) Signup and view all the answers

Which position is generally preferred for tracheal intubation in infants with small defects?

Supine position (D) Signup and view all the answers

What type of muscle relaxant is generally contraindicated during maintenance of anesthesia?

Depolarizing muscle relaxant (D) Signup and view all the answers

In neonates with myelomeningocele, confirmation of endotracheal tube position is critical due to the potential for what anatomical issue?

Abnormally short trachea (C) Signup and view all the answers

What is indicated for patients with significant loss from a skin defect during surgical repair?

Intraoperative blood transfusion (C) Signup and view all the answers

How should infused fluids be managed during the surgical procedure?

Warm all infused fluids (C) Signup and view all the answers

What should be done prior to administering succinylcholine during intubation in neonates?

Administer a dose of atropine (A) Signup and view all the answers

Which anesthetic agents are acceptable for use during induction and maintenance?

Both intravenous and inhaled anesthetic agents (D) Signup and view all the answers

Study Notes

Introduction to Myelomeningocele

Myelodysplasia, commonly known as spina bifida, involves congenital malformations of midline back structures, affecting skin, vertebral bones, and neural elements.
Occurs primarily at the lumbar level, but may arise at any vertebral level.
Classified into two main types based on skin coverage:
- Spina Bifida Cystic: Defect without skin covering, evident at birth.
- Spina Bifida Occulta: Covered by intact skin, potentially masking the defect until later detection.

Types of Spina Bifida Cystic

Divided into:
- Meningocele: Meningeal sac herniates through the defect but contains no neural tissue.
- Myelomeningocele: Contains neural tissue, representing the most common congenital central nervous system defect, approximately 4 cases per 10,000 live births.

Diagnosis

Prenatal diagnosis typically performed via ultrasonography.

Anesthetic Considerations

Preoperative

Urgent surgical intervention necessary within 24 hours post-birth to prevent neural damage and infection due to exposed structures.
Hydrocephalus often linked with type II Chiari malformation; requires ventricular shunt placement.
Latex allergy risk must be acknowledged; avoidance of latex products starts at birth.
Preoperative evaluation includes monitoring neurological deficits and assessing defect size for surgical planning.
Routine preoperative tests: hemoglobin level and type & screen. Ensure availability of packed red blood cells (PRBCs) for surgery.

Intraoperative

High risk of hypothermia due to large exposed area; maintain a warm operating environment.
Anesthesia induction positioning challenges arise from defect size.
Standard monitoring and acceptable anesthetic agents used; succinylcholine should be preceded by atropine due to bradycardia risk.
Non-depolarizing muscle relaxants may be used, ensuring reversal before surgery for nerve monitoring.
Difficult airways should be anticipated given head and trachea anomalies; extubation strategy planned.

Postoperative Care

Close monitoring of respiratory function needed post-surgery due to tight closures potentially impacting breathing.
Schedule VP shunt placement for hydrocephalus treatment as necessary.

Long-Term Outcomes

Associated conditions include macrocephaly, vocal cord paralysis, and cranial nerve dysfunction manifesting in various complications.
Risk of chronic aspiration may necessitate tracheostomy and gastrostomy in severe cases.
Children may experience abnormal reactions to hypoxia and hypercarbia due to central nervous system issues.
Neurological deficits may persist, affecting bowel and bladder function and lower extremity mobility based on defect level.
Individuals with lesions above L4 commonly experience paraplegia; lower levels may allow ambulation.
Latex allergies can influence reactions to certain foods and medications.
Intrauterine surgery experimented to mitigate myelodysplasia damage is practiced in select U.S. centers.

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Description

This quiz explores the topic of myelomeningocele, a form of spina bifida that involves congenital malformations of the spinal column. It delves into its classifications, implications, and locations along the vertebral column. Gain insights into this condition and its effects on affected individuals.