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Anesthetic Considerations for a Parturient with Noonan Syndrome, Severe Mitral Stenosis, and TIUP Presenting for Unplanned Cesarean Section
Abstract Number: RF3AC-385
Abstract Type: Case Report Case Series
Noonan Syndrome is an autosomal dominant disorder (incidence 1:1000) that includes a variety of congenital malformations (1). These patients are a unique challenge to the anesthesiologist due to their dysmorphic facial features, difficult airway, spine abnormalities, coagulation disorders, and congenital heart disease. We present a woman with Noonan Syndrome, TIUP, rheumatic mitral stenosis (MS), and preterm labor requiring C/S.
A 35 yr old G1P0 female with no prior anesthetic evaluation presented at 33 2/7 weeks’ gestation with malpositioned di/di twins (baby A transverse) in preterm labor. PMH included Noonan Syndrome, bicornuate uterus, rheumatic fever, VSD (closed spontaneously), and severe MS. PSH included pectus excavatum repair, knee arthroscopy, appendectomy, and bowel malrotation repair. Her airway was Mallampati III with micrognathia, high arched palate, and severe gingival hyperplasia. She c/o worsening edema and dyspnea; TTE showed severely elevated mitral valve gradient (peak 39 mmHg) and tachycardia (120 bpm). She received nifedipine and betamethasone but labor progressed quickly and C/S became necessary. Anesthetic plan was a preoperative arterial catheter, HR control using esmolol, and titrated epidural anesthesia (coags nl). She recovered in the ICU and was discharged POD 4.
Noonan Syndrome patients may present with short stature, webbed neck, pectus carinatum /excavatum, and cardiac abnormalities including pulmonic stenosis (2). Facial features include hypertelorism, epicanthal folds, down slanting palpebral fissures, high arched palate, micrognathia, low-set, posteriorly rotated ears, malar hypoplasia, ptosis, and short neck (3). They may have cervical spine fusion and scoliosis making regional anesthesia (RA) challenging (1) and edema of the hands and feet, making IV access difficult (2). They bruise easily, having multiple vasculitides, thrombocypenia (4) or Von Willebrand’s disease. In addition, clotting factor defects are seen in up to 50% of patients (prolonged APTT, Factor VIII:C, XI:C, XII:C, XI deficiency), so a coagulation profile is necessary prior to RA. Patients with severe MS may decompensate during labor and delivery (tachycardia, fluctuations in SVR) and they may not tolerate spinal anesthesia for C/S. We chose epidural anesthesia since we were able to titrate the anesthetic level and support maternal hemodynamics, while avoiding a potentially difficult airway. Esmolol is used with caution in pregnancy as it may cause prolonged FHR decelerations (5), however our patient was delivered expediently and the FHR remained stable. Early pre-anesthetic evaluation, would have been extremely valuable to optimize the management of this complicated patient.
1. J Bone & Joint Surg. 2001;83:1495-1502
2. J Rare Diseases 2007;2:4
3. Journal of Oral and Maxillofacial Surgery 1994;52:421-425
4. Archive of Disease in Childhood 1992;67:178-183
5. Anesthesiology 1989;71:718-22