[Anesthetic Management of a Patient with Central Core Disease for Scoliosis Surgery].

Central core disease (CCD) is a dominantly inherited congenital myopathy. CCD is also associated with muscular and skeletal abnormalities such as abnormal curvature of the spine (scoliosis), hip dislocation, and joint deformities. CCD and malignant hyperthermia (MH) are both associated with mutations in the ryanodine receptor on chromosome 19q13.1. An 11-year-old boy with CCD complicated with severe scoliosis was scheduled for spinal fusion surgery under general anesthesia. Furthermore, he had trismus caused by temporomandibular contracture. He was considered as MH susceptible. Anesthesia was managed with remifentanil and propofol without using muscle relaxtants and volatile anesthetics. We could intubate the trachea with Airtraq laryngoscope without any complications. The perioperative course was uneventful.

[Superior Mesenteric Artery Syndrome following Scoliosis Surgery during Intravenous Patient Controlled Analgesia (IV-PCA) with Fentanyl: A Case Report].

Compression and obstruction of the duodenum can occur after surgical correction of spinal scoliosis. We report a case of 15-year-old girl who developed superior mesenteric artery syndrome (SMAS) following scoliosis surgery. On the 4th postoperative day, the patient complained of nausea and vomiting, which was considered as side effects of opioids as she was treated with intravenous fentanyl infusion with patient-controlled analgesia (PCA) device. Nasogastric tube was placed and background infusion rate of the PCA was tapered. On the 5th postoperative day, fentanyl infusion was stopped, but she complained of persistent nausea and vomiting. Barium upper gastrointestinal series and abdominal echography revealed compression in the third portion of the duodenum between the superior mesenteric artery and aorta on the 7th postoperative day. She responded to conservative treatment (nutritional and fluid supplementation), which lasted about two weeks. She was discharged home on the 51st postoperative day. SMAS is rare but sometimes carries serious complications. Vomiting following scoliosis surgery should be examined thoroughly including the possibility of SMAS, especially during postoperative pain management with opioids (i. e., IV-PCA with fentanyl). Early diagnosis and institution of appropriate conservative therapy is essential to reduce the likelihood of future complications and avoid the need for surgery.

[Evaluation by ultrasonography of the size of bilateral internal jugular vein in infants and young children with congenital heart disease].

UNLABELLED: Background: A great difference in size often exists between the right and left internal jugular vein (IJV) in neonates and infants with congenital heart disease. We examined laterality of size in bilateral IJV. METHODS: We surveyed 31 cases with ventricular septal defect under 6 years of age. Using ultrasound, the transverse and longitudinal diameters and cross-sectional areas (CSA) of bilateral IJV were measured. RESULTS: There were significant differences in the transverse diameter (right 0.73 ± 0.21 cm vs. left 0.62 ± 0.20 cm, P = 0012), the longitudinal diameter(right 0.51 ± 0.14 cm vs. left 0.42 ± 0.11 cm, P = 0.010) and the CSA (right 0.31 ± 0.16 cm2 vs. left 0.22 ± 0.13 cm2 P = 0.012). However, in some cases the right IJV was smaller than the left IJV. CONCLUSIONS: It is necessary to select the cannulation site after measuring the size of bilateral IJV for IJV cannulation in neonates and infants with congenital heart disease.

[Preoperative fasting and fluid management in pediatric patients].

Preoperative fasting is principally intended to minimize the risk of pulmonary aspiration of gastric contents and facilitate the safe and efficient conduct of anesthesia. Liberalization of fasting guidelines has been implemented in most countries. In general, clear fluids are allowed up to 2h before anesthesia, and light meals up to 6h. In infants, most recommendations now allow breast milk feeding up to 4h and other kinds of milk up to 6h. Recently, the concept of preoperative oral rehydration using a carbohydrate-rich beverage up to 2h has also gained support. Drinking carbohydrate-rich fluids before elective surgery may reduce dehydration, improve hemodynamic stability under anesthesia, facilitate intravenous access, maintain glucose homeostasis, reduce patient irritability, and improve child and parent satisfaction. These guidelines apply to healthy children only. Exclusion criteria included obesity, diabetes, gastroesophageal reflux, ileus, bowel obstruction and emergency care. In particular, trauma and other emergency cases are at higher risk for aspiration regardless of fasting interval and should be managed appropriately.

[Comparison of the effects of phosphodiesterase III inhibitors, milrinone and olprinone, in infant corrective cardiac surgery].

BACKGROUND: Clinical characteristics of phosphodiesterase (PDE) III inhibitors, milrinone and olprinone, is not fully understood in infants. We therefore retrospectively examined the hemodynamics, metabolism, and oxygenation of two different PDE III inhibitors in infants undergoing radical correction of ventricular septal defect with pulmonary hypertension. METHODS: Twenty-six infants with pulmonary hypertension undergoing ventricular septum defect repair were retrospectively allocated to milrinone group (n= 13)and olprinone group(n=13). Hemodynamic parameters, acid-base balance, oxygenation and postoperative mechanical ventilation period were compared between the two groups at induction of anesthesia, weaning from cardiopulmonary bypass and the end of the surgery. RESULTS: The patients' mean age was 4.4 +/- 2.5 months. Demographic data were almost similar between the two groups. Milrinone and olprinone were administered at the rates of 0.5 and 0.3 microg x kg-1 x min-1 at the end of surgery, respectively. Hemodynamic variables, acid-base balance, Pao2 /FIo2 ratio and mechanical ventilation period were not significantly different between the two groups. No adverse side effects were observed during the study period. CONCLUSIONS: The effects of the PDE III inhibitors, milrinone and olprinone, on hemodynamic parameters, acid-base balance and oxygenation were similar in these infants. Both milrinone and olprinone could be used safely in infant cardiac surgery.

[Case of trachea agenesis managed with esophageal intubation].

A male neonate was born after 39 weeks of gestation by normal vaginal delivery. The pregnancy was uneventful. He developed immediate respiratory distress at birth with an Apgar score of 4 and 5 at 1 and 5 minutes, respectively. He was initially resuscitated with bag-mask ventilation, and subsequently transferred to the neonatal intensive care unit. Oral tracheal intubation was attempted in the NICU with no success and finally he was barely ventilated after esophageal intubation. Neonatologists consulted with an otolaryngologist for emergency tracheostomy. After our preanesthesia evaluation, we pointed out the possibility of trachea agenesis and requested surgeon for distal esophageal banding with gastrostomy to stabilize mechanical ventilation. General anesthesia was induced and maintained with sevoflurane in oxygen-air mixture supplemented with continuous infusion of fentanyl. His respiratory condition improved when distal esophageal banding and gastrostomy were completed. Esophageal endoscopy and CT scan were performed postoperatively and their findings revealed Floyd's type II tracheal agenesis in which tracheostomy was impossible. In this case, not enforcing tracheostomy proved to be a turning point in respiratory management.

[Prophylactic effect of diphenhydramine on postoperative vomiting in children after laparoscopic surgery].

BACKGROUND: Laparoscopic percutaneous extraperitoneal closure (LPEC), introduced as an alternative to a conventional open inguinal hernia repair in children, has shown a higher incidence of postoperative vomiting (POV). The aim of this study was to examine whether a prophylactic use of diphenhydramine can decrease the incidence of POV in children undergoing LPEC. METHODS: We studied 60 girls between 1 and 6 years of age with ASA physical status I or II undergoing LPEC. Patients were allocated to receive either diphenhydramine 1 mg x kg(-1) intravenously (n = 30) or placebo (n = 30) during the operation. Anesthesia was performed with air-oxygen-sevoflurane in combination with epidural anesthesia. Opioids were avoided throughout the perioperative period. The incidence of POV was recorded postoperatively. RESULTS: Demographic data were similar between the groups. The overall incidence of POV during the first 24 postoperative hours was significantly higher in the placebo group (56.7%) than in the diphenhydramine (6.7%) group (P < 0.01). The wake-up in the ward was significantly prolonged in the diphenhydramine group than control group. CONCLUSIONS: Prophylactic use of diphenhydramine substantially reduced the risk of postoperative vomiting, but was associated with prolonged sedation in pediatric patients undergoing LPEC.

Anchoring geometry is a significant factor in determining the direction of kinesin-14 motility on microtubules.

Kinesin-14 microtubule-based motors have an N-terminal tail attaching the catalytic core to its load and usually move towards microtubule minus ends, whilst most other kinesins have a C-terminal tail and move towards plus ends. Loss of conserved sequences external to the motor domain causes kinesin-14 to switch to plus-end motility, showing that an N-terminal attachment is compatible with plus-end motility. However, there has been no systematic study on the role of attachment position in minus-end motility. We therefore examined the motility of monomeric kinesin-14s differing only in their attachment point. We find that a C-terminal attachment point causes kinesin-14s to become plus-end-directed, with microtubule corkscrewing rotation direction and pitch in motility assays similar to that of kinesin-1, suggesting that both C-kinesin kinesins-14 and N-kinesin kinesin-1 share a highly conserved catalytic core function with an intrinsic plus-end bias. Thus, an N-terminal attachment is one of the requirements for minus-end motility in kinesin-14.