What are the risks and management strategies for kyphoscoliosis in pregnancy?

Kyphoscoliosis in Pregnancy: Risks and Management

Pregnant women with kyphoscoliosis require specialized multidisciplinary care at tertiary centers with experience in high-risk pregnancies to minimize maternal cardiopulmonary complications and optimize delivery outcomes. 1

Maternal Risks

Cardiopulmonary Complications

• Respiratory deterioration is the primary concern, particularly in the second and third trimesters when the growing uterus further restricts diaphragmatic movement 2
• Women with kyphoscoliosis characterized by short trunk are at higher risk for:
  • Cardiopulmonary complications
  • Maternal complications
  • Preterm delivery 1
• Increasing fundal height may adversely affect maternal cardiopulmonary and musculoskeletal status 1

Anesthetic Challenges

• Anatomical differences increase risk during both general and regional anesthesia 1
• Potential difficulties include:
  • Challenging airway access due to cervical instability or immobility
  • Difficulties with epidural placement due to spinal deformity
  • Increased risk of high spinal block

Management Strategies

Preconception and Early Pregnancy

• Establish correct diagnosis and provide individualized preconception genetic counseling 1
• Early referral to maternal-fetal medicine specialists and anesthesiology for planning
• Baseline pulmonary function testing to assess respiratory reserve

Respiratory Management

• Monitor for signs of respiratory deterioration:

  • Exercise intolerance
  • Fatigue
  • Hypersomnolence
  • Nocturnal oxygen desaturations

• Consider non-invasive positive pressure ventilation (NIPPV) for:

  • Symptomatic nocturnal hypoventilation
  • Declining pulmonary function
  • Exercise intolerance 2, 3

• NIPPV with bilevel positive airway pressure (BiPAP) has been shown to:

  • Correct exercise tolerance
  • Reduce fatigue
  • Improve nocturnal oxygen saturations 2

Antenatal Care

• Vigilant monitoring throughout pregnancy with more frequent visits
• Serial ultrasound assessments to monitor fetal growth
• Careful attention to fluid management to avoid fluid overload in peripartum period 1
• Early planning for delivery, including location, mode of delivery, and anesthetic options 1

Delivery Planning

• Cesarean delivery is often recommended due to pelvic anatomy constraints in most women with skeletal dysplasia 1
• Can typically be performed with Pfannenstiel skin incision and low transverse uterine incision
• Combined spinal-epidural anesthesia with availability of fiberoptic intubation equipment if general anesthesia becomes necessary 2
• Delivery should occur at a tertiary center with:
  • Experienced obstetric anesthesiologists
  • Access to intensive care facilities
  • Neonatal intensive care unit

Prognosis

Recent studies show improved outcomes compared to historical reports:

• No maternal or perinatal mortality in modern case series 4, 5
• The high maternal and perinatal risks reported in earlier literature are no longer valid with modern obstetric and orthopedic care 5
• Even patients with severe kyphoscoliosis and profound ventilatory limitation can carry successful pregnancies into the third trimester 6

Postpartum Considerations

• Continue respiratory monitoring postpartum as nocturnal episodic desaturations may persist 2
• Some patients may need to continue home ventilatory support temporarily
• Most patients return to their prepregnancy level of respiratory function after delivery 2, 3

Key Pitfalls to Avoid

1. Failing to plan for anesthesia challenges early in pregnancy
2. Overlooking progressive respiratory compromise during pregnancy
3. Delaying intervention for respiratory deterioration
4. Inadequate preparation for potential emergency cesarean delivery
5. Managing delivery at centers without appropriate expertise and resources