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2026 SOAP Annual Meeting

April 29 - May 3, 2026 | Montreal, Quebec Canada

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2322245

Prepared Before the Push: Peripartum Management in a Parturient with Gorlin and Sotos Syndromes and a Known Difficult Airway

Part of Topic

Triple A: Airway, Angioedema, Anaphylaxis

BACKGROUND

Traditionally, pregnancy has been deemed a cause of "difficultairway" due to increased airway edema, reduced functionalresidual capacity, and full-stomach precautions. Furthermore,the physical and hormonal changes during pregnancy lead tohigher risk of aspiration. Gorlin and Sotos Syndromes eachpose challenges to airway and neuraxial management, addingto the changes with pregnancy. 

Gorlin Syndrome:

 

  • Facial dysmorphism, odontogenic keratocysts,macrocephaly, meningioma, spina bifida, fused vertebrae

 

Sotos Syndrome:

 

  • Intellectual disability, hypotonia, pre-eclampsia,macroglossia, macrocephaly/frontal bossing, mandibularhypoplasia, high-arched palate, pointy chin, scoliosis

CLINICAL FINDINGS
  • 40-year-old G6P3 at 36+5 weeks gestation 

  • Induction of labor for history of pre-eclampsia with severe features

  • Prominent facial features from Gorlin and Sotos Syndromes

  • History of meningioma resection and ventricular drain forhydrocephalus prior to pregnancy

  • Unanticipated difficult airway during meningioma resection two yearsprior (failed direct laryngoscopy, required bougie and indirectlaryngoscopy for intubation)

  • Two prior vaginal deliveries at outside hospitals, epidural recordsunknown

  • Stable intracranial pressures throughout pregnancy

Key Considerations:

  • Concern for contraindications to neuraxial with increased intracranialpressure, possible mass effect, vertebral anomalies

  • Complex PMH and advanced maternal age increase risk of cesarian section

  • Known difficult airway and concern for fiberoptic intubation in STAT cesarean section

CLINICAL HISTORY
  • Meningioma resection two years prior, stable intracranial pressure

  • Two prior NSVD's at outside hospitals, epidural records unknown

  • Documented difficult airway

PRESENTATION
  • Induction at 36+5 weeks GA for pre-eclampsia with severe features

  • No symptoms of elevated intracranial pressure or mass effect

  • Mallampati III, large tongue, small mouth opening, mandibular hypoplasia

WORK-UP AND CONSULTS
  • Neurosurgery: MRI lumbar spine to rule out tethered cord/spina bifida, cleared for neuraxial anesthesia with vaginal delivery

  • ENT: consulted for flexible laryngoscopy confirming hypertrophied tongue

  • Trauma surgery: on standby for possible emergent surgical airway

DELIVERY PLAN
  • Multidisciplinary discussion advocating for early labor epidural

  • Indirect laryngoscopy and flexible bronchoscopy available for awake intubation

DELIVERY AND POSTPARTUM CARE
  • Successful placement of dural puncture epidural

  • Induction of labor with uncomplicated vaginal delivery

  • Postpartum follow-up with neurosurgery and ENT

DISCUSSION
  • This case highlights the need for meticulous planning in parturients with known difficult airways and complex comorbidities

  • Early multidisciplinary planning is essential with thorough work-up if time permits. Image based risk assessment allowedfor safe neuraxial anesthesia 

  • Without an early functioning neuraxial, an emergent cesarean delivery may have required an awake fiberoptic intubation,possibly delaying delivery of the fetus and increasing maternal and fetal risk 

TAKE AWAY POINTS
A functioning labor epidural reduced risk of intubation in case of an emergent cesarean section in a patient with a knowndifficult airway and complex medical history
 
 
 

 

 

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