Current Selection Criteria and Perioperative Therapy Used for Fetal Myelomeningocele Surgery.

OBJECTIVE: To determine the current maternal and fetal selection criteria and operative approaches used at centers performing fetal myelomeningocele surgery. METHODS: The 17 principal investigators participating in the Fetal Myelomeningocele Consortium were asked to participate in an anonymous online survey regarding the current practice of maternal-fetal surgery for neural tube defect repair and results were tabulated. The 35-question survey related to diagnostic testing, inclusion and exclusion criteria, and clinical management. RESULTS: Sixty-five percent (11/17) of principal investigators responded to the survey and not all centers responded to all 35 questions. All centers continue to use magnetic resonance imaging in their preoperative evaluation. Diagnostic testing from amniocentesis is varied: 5 of 11 (45%) require amniotic fluid α-fetoprotein, 4 of 10 (40%) amniotic fluid acetylcholinesterase, and 8 of 11 (73%) DNA microarray. There is also variation from the Management of Myelomeningocele Study with regard to body mass index (BMI) (1/11; 9% would offer surgery with BMIs higher than 35), maternal medical risk factors (surgery would be offered for controlled pregestational diabetes [3/10 (30%)]), hepatitis C with negative viral load (4/11 [36%]), and human immunodeficiency virus with an undetectable viral load (1/10 [10%] or an obstetric history [3/11 (27%)] would offer surgery with a history of preterm delivery on progesterone). Ten of 11 (91%) centers did not consider ventriculomegaly of 18 mm and 9 of 11 (82%) centers did not consider lack of leg movement as an exclusion criteria. Nuances in the perioperative and intraoperative management were also reported, including 5 of 11 (45%) use intraoperative echocardiography and alterations in postoperative tocolytics. CONCLUSION: Variation in practice patterns for offering and performing maternal-fetal surgery for myelomeningocele repair exists among centers. Ongoing evaluation of inclusion and exclusion criteria as well as operative techniques is warranted to ensure continued safety, effectiveness, and beneficence.

Anesthetic Management of a Parturient with PHACE Syndrome for Cesarean Delivery.

PHACE syndrome is a disorder that features posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. PHACE syndrome includes abnormalities in several organ systems that may influence anesthetic management. We discuss the anesthetic management of a 26-year-old woman with PHACE syndrome presenting for cesarean delivery. Management included careful airway examination, slowly dosed epidural anesthesia, close hemodynamic monitoring aided by a radial arterial line, and continuous intraoperative neurologic assessment.

Fetal surgery: the ochsner experience with in utero spina bifida repair.

BACKGROUND: Myelomeningocele is the most common form of congenital central nervous system defect that is compatible with life. Most patients with myelomeningocele have significant functional impairment of ambulation and bowel and bladder function, require permanent cerebrospinal fluid diversion with shunting, and have significant morbidity and mortality from hindbrain herniation (Chiari II malformation). The advent of intrauterine surgery has provided new opportunities to better address this lifelong debilitating disease. CASE REPORT: The patient was a 19-year-old gravida 2 para 1 at 22-6/7 weeks whose fetus was diagnosed with an open neural tube defect and further demonstrated to have ventriculomegaly and hindbrain herniation. Amniocentesis confirmed normal karyotype and the presence of acetylcholinesterase. After an intrauterine procedure, the patient underwent cesarean section at 35-5/7 weeks and delivered a male infant. His spinal incision was well healed at birth without any evidence of cerebrospinal fluid leakage, and his extremities were normal in appearance, range of motion, and movement. The infant also has maintained relatively normal, age-appropriate bowel and bladder function and has no obvious neurologic deficit. CONCLUSION: As the benefit of fetal surgery becomes more widely accepted, quality of care and patient safety must be at the forefront of any institution's effort to offer fetal surgery. Given the current prevalence of spina bifida and the amount of resources required to treat this disease effectively either in utero or postnatally, it is our opinion that the treatment of spina bifida should be regionalized to tertiary referral centers with the interdisciplinary expertise to offer comprehensive treatment for all aspects of the disease and all phases of care for the patients.

Shoulder dystocia with and without brachial plexus injury: experience from three centers.

This article compares the maneuvers used to relieve shoulder dystocia (SD) at three centers and discerns risk factors for brachial plexus injury (BPI) following SD. Retrospectively SD managed at three tertiary centers was identified and charts reviewed. Unconditional logistic regression was used to identify risk factors for BPI. SD was encountered in 2% of vaginal deliveries (624/29,591), and BPI followed impacted shoulders in 6% (38/624). The rate of SD among the three institutes varied significantly (1.5%, 2%, 0.8% of vaginal births; P < 0.0001). The use of the McRoberts' maneuver to relieve SD differed significantly by center (98%, 80%, 90%; P < 0.0001) as did the use of suprapubic pressure (83%, 66%, 54%; P < 0.0001). The rate of BPI per case of SD (10%, 3%, 5%) was significantly different at the three centers ( P = 0.009). A multivariate predictive model indicates that among those with and without concomitant fractures, there is a significantly increased risk of BPI if three or more maneuvers are used rather than two or fewer. In conclusion, not only does the rate of SD and BPI following it occur at significantly different rates, the management differs too. Compared with two maneuvers or fewer, there is an increased risk of BPI if three or more maneuvers are used to relieve SD.