Anesthetic management of a patient with Smith-Lemli-Opitz syndrome complicated with thrombocytopenia

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive syndrome, characterized by severe growth failure and congenital anomalies (for example dysgenesis, mental retardation, renal and cardiac defects, and various malformation). SLOS results from error of a cholesterol enzyme and generalized cholesterol deficiency. This report describes our experience of a patient with SLOS and thrombocytopenia who underwent anesthesia twice for surgical procedures in a year. The patient received drip platelet transfusion for thrombocytopenia before operations. Anesthesia was induced with inhalation of oxygen, nitrous oxide and sevoflurane, and maintained with oxygen, propofol, fentanyl and low concentrations of sevoflurane. Airway was maintained with laryngeal mask airway. Complications were not seen in this case. One of the problems in anesthetic management of SLOS is difficult intubation because of the typical dysmorphic facial features such as micrognathia, cleft palate and abnormal tongue. We thought that laryngeal mask airway was useful and safe for SLOS patients. Two cases of malignant hyperthermia were reported in anesthetic management of SLOS by using halothane or suxamethonium. In this case, the anesthetic maintenance was mostly with propofol and fentanyl. Malignant hyperthermia did not occur but sevoflurane was used at low concentrations. SLOS presents various problems with anesthetic management and we have to administer general anesthesia carefully.     

Anesthetic considerations of an infant with Beckwith-Wiedemann syndrome

The case of a 3-day-old infant with Beckwith-Wiedemann syndrome who required anesthetic care during closure of an abdominal wall defect is presented. Beckwith-Wiedemann syndrome comprises a constellation of clinical features, including macroglossia, macrosomia, omphalocele, visceromegaly, mild microcephaly, facial nevus flammeus, horizontal earlobe creases, and renal medullary dysplasia. Due to the high rate of omphalocele in this syndrome, anesthetic care is frequently required during the neonatal period. Many of these infants (greater than 50%) are born prematurely. Therefore, their anesthetic care may be further complicated by associated diseases of prematurity, such as hyaline membrane disease. Additional anesthetic implications of this syndrome relate to the occurrence and management of hypoglycemia and polycythemia. Careful intraoperative management of glucose homeostasis is particularly important, since eventual neurologic outcome and intelligence will be normal provided prolonged neonatal hypoglycemia is avoided. Preoperative evaluation of the cardiac and genitourinary system, including echocardiography and renal ultrasound, are recommended because of the frequent occurrence of associated anomalies with omphalocele.     

Anesthetic and obstetric considerations in a parturient with klippel-trenaunay syndrome

PURPOSE: To explain the considerations governing the anesthetic management of pregnant patients with Klippel-Trenaunay syndrome (KTS). Klippel-Trenaunay syndrome is a congenital vascular disease characterized by cutaneous hemangiomas, venous varicosities, and limb hypertrophy; and is associated with both hemorrhagic and thrombotic complications. The importance of this diagnosis, including the presence of neuraxial vascular anomalies, is often under-appreciated by both obstetric and anesthesia providers. While regional anesthetic management of patients with KTS has been discussed by others, we present a case in which regional anesthesia presented an unwarranted risk to the patient. CLINICAL FEATURES: An obese, 18-yr-old parturient with a fetus in the breech position underwent Cesarean delivery at 35 weeks gestation secondary to evolving preeclampsia. Unfortunately, no neurovascular imaging of this patient's spine was available. The patient underwent an attempted external cephalic version, a failed obstetric induction, and, ultimately, a Cesarean delivery under general anesthesia. The resulting infant was without any stigmata of KTS. Both mother and infant did well during the course of their hospitalization, and were discharged home without incident. CONCLUSIONS: The posterior cutaneous hemangiomas of KTS may be associated with underlying epidural and subdural vascular malformations. Disruption of these vascular anomalies during regional anesthesia may lead to neuraxial hematoma formation, which may be further compounded by a consumptive coagulopathy observed in some cases of KTS. If neuraxial vascular anomalies cannot be ruled out radiographically, regional anesthesia should be avoided. Additionally, regardless of the anesthetic technique chosen, the coagulation profile of these patients should be verified for signs of coagulopathy.     

Anesthetic considerations of two sisters with Beckwith-Wiedemann syndrome.

Anesthetic considerations of 21-mo-old and 4-yr-old sisters with Beckwith-Wiedemann syndrome during surgical repair of cleft palate and reduction of macroglossia are presented and discussed. This syndrome is characterized by exomphalos, macroglossia, gigantism, hypoglycemia in infancy, and many other clinical features. This syndrome is also known as exomphalos, macroglossia, and gigantism (EMG) syndrome. Principal problems associated with anesthetic management in this syndrome are hypoglycemia and macroglossia. Careful intraoperative plasma glucose monitoring is particularly important to prevent the neurologic sequelae of unrecognized hypoglycemia. It is expected that airway management would be complicated by the macroglossia, which might cause difficult bag/mask ventilation and endotracheal intubation following the induction of anesthesia and muscle paralysis, so preparations for airway difficulty (e.g., awake vocal cord inspection) should be considered before induction. A nasopharyngeal airway is useful in relieving postoperative airway obstruction.     

Anesthetic considerations in premature birth

Premature birth still accounts for about 75% of perinatal mortality. Although great strides have been made in the care of premature babies over the past two decades, markedly decreasing mortality, the prevention of premature birth has not been greatly improved. Although tocolysis, particularly with the beta-2 agonists and magnesium sulfate, may delay birth and allow fetal maturation, it poses several risks which, if not recognized, can cause serious morbidity and even mortality. The use of these drugs and other less widely used tocolytics has important implications for the anesthesiologist. The premature infant itself is subjected to such risks as RDS, IVH, NEC, asphyxia, hypothermia, increased incidence of breech presentation, metabolic disturbances, and predisposition for trauma. To ensure safe delivery, premature babies should be delivered in a tertiary care center equipped and ready to attend to their needs. Major conduction block, particularly continuous lumbar epidural analgesia, is an ideal form of analgesia for the delivery of most premature neonates. Properly administered, it maintains maternal physiology, is not associated with drug depression in the newborn, enables a controlled, atraumatic vaginal delivery, and has little interaction with tocolytics (and indeed may protect against some of their side effects). It is ideal for a trial of labor and, if initiated early, allows for an emergency cesarean section. Continuous lumbar epidural block and subarachnoid block are both superb for elective or urgent cesarean section. However, when their use is contraindicated, inhalation analgesia for vaginal delivery or general anesthesia for cesarean section can be safely administered from the standpoint of both mother and child. Expertly administered anesthesia is not a luxury but is indeed indispensable for successful premature delivery.     

Anesthetic considerations in steroid-induced mediastinal lipomatosis

Mediastinal lipomatosis (ML) is a benign condition characterized by circumscribed overgrowth of adipose tissue producing mediastinal widening that can cause errors in diagnosis on chest roentgenogram. We describe a case of steroid-induced ML leading to difficulty in central venous catheterization during surgery and its other implications for anesthesiologists. Because many patients receive long-term steroid administration and present for surgical intervention, it is essential that they undergo detailed preoperative evaluation to exclude Cushing's syndrome and various pressure effects. IMPLICATIONS: This case report highlights problems during right internal jugular vein cannulation resulting from high back pressure and flow from superior vena cava obstruction in steroid-induced mediastinal lipomatosis. Other anesthetic considerations in mediastinal lipomatosis are also discussed.     

Anesthetic considerations in intracranial aneurysm surgery

Rupture of an intracranial aneurysm generally has a poor outcome, though perioperative treatments have improved. At the present time, the important factors in the management of intracranial aneurysm surgery appear to be the maintenance of adequate cerebral perfusion pressure and the avoidance of hyperglycemia. Relevant features of the anesthetic management of this surgery are discussed.