The First Case of Anesthesia for Emergency Cesarean Section in a Patient with Meier-Gorlin Syndrome
Abstract Number: S-54
Abstract Type: Case Report/Case Series
Meier-Gorlin syndrome (MGS), also known as ear-patella short stature syndrome, is a clinical diagnosis consisting of the triad of intrauterine and postnatal growth retardation, bilateral microtia, patellar aplasia or hypoplasia1. Here we present the first reported case of a parturient with MGS to receive anesthesia for cesarean section.
A 23 yo G2P0 at 36 weeks gestation presented to labor and delivery complaining of abdominal pain and one episode of vaginal bleeding. The patient’s past medical history was significant for scoliosis, moderate-persistent asthma, and restrictive lung disease. Her height was 127 cm, weight-36.7 kg, BP 150/85, category II FHR tracing. Physical exam revealed a Mallampati class IV airway and a tender abdomen. Ultrasound demonstrated signs of placental abruption. The decision was made to proceed with urgent c-section.
Epidural anesthesia was planned however multiple attempts of identifying the epidural space were not successful. The patient developed a second episode of vaginal bleeding and severe abdominal pain, the FHR decreased to 90 beats/minute. The obstetrician declared that an emergent cesarean delivery was necessary. Endotracheal intubation was attempted but proved to be unsuccessful. The patient developed laryngospasm which could not be overcome with positive pressure ventilation. General anesthesia was induced with ketamine and succinylcholine cesarean delivery ensued. While the surgery was in progress the airway was secured with a size 2.5 LMA. The surgery was otherwise uneventful the newborn was discharged from the ICU within 24 hours and the mother successfully.
To the best of our knowledge this is the first reported case of anesthesia provided for a patient with Meier-Gorlin syndrome undergoing c-section. From an anesthesia perspective this patient was challenging due to her airway and skeletal anomalies. Spinal anesthesia was not attempted due to the difficulty to predict an adequate dose of spinal anesthetic. LMA can be used successfully in pregnant patients in case of failed endotracheal intubation.
As more female MGS patients mature to adulthood, and thus potentially pregnancy, anesthesiologists may be involved in their labor and delivery care. From our experience with a parturient with MGS, we believe these patients should be considered difficult, if not impossible, to intubate. It should also be noted that neuraxial anesthesia may be a challenge in this population.
Boles RG, Teebi AS, Schwartz D, Harper JF. Further delineation of the ear, patella, short stature syndrome (Meier-Gorlin syndrome). Clin Dysmorphol 1994;3(3):207-14.