Abstract: Holt-Oram syndrome is a rare genetic disorder affecting the heart and upper limbs (atriodigital dysplasia). The manifestations of the limb defects may vary in severity from subtle carpal bone defects and triphalangeal thumb to digit aplasia and upper extremity phocomelia. Cardiac abnormalities include atrial and/or ventricular septal defects, anomalies in pulmonary venous return and various dysrhythmias. We present the anesthetic management of a parturient with this syndrome who underwent elective cesarean section and tubal ligation, conducted under combined spinal-epidural anesthesia with a low dose of intrathecal bupivacaine. Our goal was to avoid an excessively high sympathetic block or excessive sympathetic stimulation accompanied by potential deleterious effects on cardiac rhythm. Cardiac monitoring was continued in the postoperative period for 6 h because of the possibility of dysrhythmia.
Abstract: Acute lung disease may originate in pregnancy because of the pregnancy itself or because of an intercurrent etiology. The purpose of this study was to describe the effect of prolonged antepartum mechanical ventilatory support on the mother and the neonate when the strategy was to prolong the pregnancy rather than deliver preterm. Among 72 312 parturients over eight years, three gravidae required mechanical ventilation 12-48 h after admission for different conditions, 45-91 days before delivery. Gestational age at intubation was 21-28 weeks. Appropriate analgesia, broad-spectrum antibiotics, vasopressors and betamethsone for fetal lung maturity were used in all cases. None received tocolysis. Despite uterine distension, respiratory support provided adequate oxygenation and FiO(2) could be maintained below critical levels, obviating the need for early delivery. All women survived, were weaned from ventilatory support, discharged, and delivered healthy neonates at term. Mode of delivery was dictated by obstetrical indicators only. All five infants (two sets of twins) are healthy at 12-36 months with appropriate developmental milestones. We conclude that when the maternal condition is amenable to therapy, and given the risks of labor induction and of prematurity, there is only limited benefit of delivery while on mechanical ventilation.
Abstract: Management of parturients with a history of Fontan procedure requires careful monitoring of cardiovascular parameters and anticipation of potential complications. We describe potential pitfalls in a parturient with atriopulmonary Fontan circulation, who received epidural analgesia for labor and who later required emergency cesarean section. Low-dose local anesthetic in combination with meperidine provided excellent perioperative epidural analgesia and cardiovascular stability. Epidural analgesia during labor offered optimal pain relief and facilitated conversion to surgical anesthesia for an emergency cesarean section.
Abstract: PURPOSE: The purpose of this retrospective review was to highlight clinical issues relating to anesthetic management in children who present with Gaucher disease-specific features that may impact on anesthetic management and surgical outcome. Previous reports have dealt primarily with neuronopathic forms where neurological dysfunction determined the mode of anesthesia. To date, no series of routine surgeries in pediatric patients with non-neuronopathic Gaucher disease has been published. METHODS: All surgeries performed in children with Gaucher retrospectively analyzed. RESULTS: There were 31 procedures under anesthesia in 15 pediatric patients. Twenty-seven of these (87%) involved either insertion or removal of a central venous catheter. There was no correlation between disease severity and the need for blood transfusion postoperatively [required in only eight cases (25.8%), including a total hip replacement]. No difficult intubations or other airway problems were recorded. Positioning of two patients, because of gibbus and prior to hip replacement, respectively, required special attention. CONCLUSIONS: We record our experience in surgeries in children with mild, non-neuronopathic type I and severe neuronopathic type III Gaucher disease, who had relatively short surgeries under general anesthesia. Attention to hematological parameters in particular can minimize postoperative bleeding, the most serious complication.
Abstract: Pregnancy and delivery in patients with non-neuronopathic Gaucher disease, whether treated with enzyme replacement or untreated, are usually uncomplicated. Various factors may influence mode of delivery, vaginal or cesarean section, as well as type of anesthesia, general or regional, used during delivery. This retrospective review was intended to highlight some of the practical issues relating to obstetric anesthetic management, based on a review of the literature and experiences from a large referral clinic for Gaucher disease. In the past decade, there were 16 deliveries in 11 women in our institution. There were five normal vaginal deliveries, two vacuum extractions, one placental extraction, and eight cesarean sections. Platelet counts were 27-215 x 10(9)/L. Two spontaneous deliveries and one vacuum extraction were performed under epidural anesthesia; two other women having vaginal deliveries and one vacuum extraction were given i.v. analgesia; the fifth was given i.v. patient-controlled analgesia. The placental extraction was performed under general anesthesia. Seven of the women having cesarean deliveries received spinal anesthesia; the breech presentation required general anesthesia. There were no anesthesia-related side effects or complications, although there were some instances of post-partum bleeding irrespective of enzyme therapy. Gaucher disease affects multiple organs and can be a challenge to the anesthesiologist. Based on this survey we suggest that anesthetic management requires particular attention to hematological parameters before delivery. A multidisciplinary approach and extensive communication among obstetrician, hematologist and anesthesiologist is required to anticipate the possibility of post-partum hemorrhage, and preclude skeletal damage.
