Ex utero intrapartum treatment of fetal micrognathia.

BACKGROUND: Ex utero intrapartum treatment (EXIT) procedures have emerged as a viable option for potentially life-saving procedures in fetuses with predicted airway compromise at birth. The ability to maintain maternal-fetal uteroplacental perfusion allows for prolonged procedures in a stable fetal hemodynamic environment thereby avoiding neonatal hypoxemia or sequelae of an emergent tracheostomy. CASE: A 26-year-old female presents with a 20-week ultrasound and subsequent magnetic resonance imaging demonstrating severe fetal micrognathia (jaw index below the 5th percentile), glossoptosis, polyhydramnios, absence of a gastric bubble, and suspected microtia concerning for Treacher Collins syndrome. An EXIT procedure was completed with successful intrapartum endotracheal intubation with a flexible fiber-optic bronchoscope through a laryngeal mask airway. CONCLUSION: This case represents the first EXIT procedure completed at Naval Medical Center San Diego. Although this case is unique, the clinical skills and coordination of care required to perform this procedure are exemplified in our daily practice of stabilizing, transporting, and definitively treating our wounded warriors. The ability to work in coordination across multiple armed services to provide the EXIT procedure to our military families, for potentially life-saving procedures, is a true testament to the current state of Military Medicine.

Anesthesia for pheochromocytoma resection in a child with Fontan circulation.

PURPOSE: To report the anesthetic management of a successful resection of a pheochromocytoma in a child with a completed Fontan circulation. CLINICAL FEATURES: The patient was an 11-yr-old boy with Ivemark syndrome who had undergone Fontan palliation at three years of age. Six weeks earlier, he had been diagnosed with a norepinephrine-producing pheochromocytoma, and he had been pretreated with oral propranolol and phenoxybenzamine. During surgery, intravenous administration of magnesium sulphate, esmolol, and phentolamine provided good hemodynamic control. Postoperatively, the patient tended to be hypotensive, and treatment with fluid administration resulted in prolonged intensive care. CONCLUSION: Although intraoperative management was not problematic, postoperative care of this 11-yr old child with pheochromocytoma was complicated by residual sympathetic blockade.