Epidural anesthesia for labor and delivery in a patient with May-Hegglin anomaly: a case report.

We report a case of May-Hegglin anomaly (MHA) in a woman who had a successful labor and delivery under epidural anesthesia. MHA is an inherited thrombocytopenia easily misdiagnosed as idiopathic (immune) thrombocytopenic purpura (ITP). Early and appropriate diagnosis of MHA during pregnancy is essential for optimal maternal and neonatal delivery outcome. Additionally, it can avoid unnecessary diagnostic studies, such as bone marrow aspiration and biopsy, and even harmful therapies with corticosteroids, immunosuppressive agents, and splenectomy. Consequently, the most serious impacts of this disease are iatrogenic managements due to misdiagnosis. It seems that in patients with MHA, adequate clinical coagulation is far more dependent on adequate platelet function than any particular platelet count. The diagnosis of MHA may pose a challenge for clinicians managing pregnant women with thrombocytopenia.

ANESTHETIC MANAGEMENT DURING COMBINED LIVER AND KIDNEY TRANSPLANTATION.

Combined liver and kidney transplantation is a highly demanding and challenging procedure for anesthesiologists due to the lengthy and complicated nature of the procedure, the critical patient condition and the need to balance the intravascular volume to maintain the venous outflow of the hepatic allograft and also the diuresis of the renal allograft. Intravascular volume management and coagulation control, seem to be the most important issues during combined liver and kidney transplantation. There is sparsity of data in the literature concerning the anesthetic and fluid management in CLKT. We present and discuss the anesthetic management in a case series in three patients, who underwent combined liver and kidney transplantation in our institution during the last two years.

A CASE OF GRANISETRON ASSOCIATED INTRAOPERATIVE CARDIAC ARREST.

We report a case of intraoperative severe bradycardia that resulted in asystole and cardiac arrest shortly after (<2 min) intravenous granisetron 1mg for postoperative nausea and vomiting prophylaxis, that occurred in a female patient who underwent an elective total thyroidectomy. After two cycles of cardiopulmonary resuscitation and defibrillation, spontaneous circulation and sinus rhythm returned successfully. Postoperatively, the patient was diagnosed with a drug-induced long QT syndrome. At the time of the event, granisetron was the only medication administered. Furthermore, there was no reason to suspect electrolyte abnormalities. We explore the association of the onset of severe sinus bradycardia with the intravenous administration of granisetron.

ANESTHETIC MANAGEMENT IN UNEXPECTED EXTRA- ADRENAL PHEOCROMOCYTOMA PRESENTING WITH THORACIC SPINAL CORD COMPRESSION.

A 52 yearold female presented with a thoracic paravertebral tumour causing spinal nerve root compression and lower limbs neurologic symptoms. The patient was scheduled to undergo thoracic decompression laminectomy and instrumentation. Markedly severe hemodynamic fluctuations happened during the manipulation of the tumor and continued after the tumor was removed. After multimodal antihypertensive therapy the vital signs were adequately managed and the surgery was successfully performed without complications. The patient was discharged without any sequelae ten days later. The pathology report indicated the diagnosis of extra-adrenal pheochromocytoma. Unexpected pheochromocytoma may lead to a fatal hypertensive crisis during surgery. For anesthesiologists and surgeons who encounter an unexpected hypertensive crisis during surgery, undiagnosed pheochromocytoma should always be considered.

ANESTHESIA MANAGEMENT IN AN INFANT WITH GLYCOGEN STORAGE DISEASE TYPE II (POMPE DISEASE).

Pompe or Glycogen Storage Disease type II (GSD-II) is a genetic disorder affecting both cardiac and skeletal muscle. Historically, patients with the infantile form usually die within the first year of life due to cardiac and respiratory failure. Recently a promising enzyme replacement therapy has resulted in improved clinical outcomes and a resurgence of elective anesthesia for these patients. Understanding the unique cardiac physiology in patients with GSD-II is essential to providing safe general anesthesia. Additional care in maximizing coronary perfusion pressure and minimizing arrhythmia risk must be given. For these reasons, it is recommended that anesthesia for infantile Pompe patients should specifically avoid propofol or high concentrations of sevoflurane and, instead, use an agent such as ketamine as the cornerstone for induction in order to better support coronary perfusion pressure and to avoid decreasing diastolic blood pressure (DBP) with vasodilatory agents. We present the anesthetic technique in a case of infantile type Pompe disease.

AIRTRAQ8 OPTICAL LARYNGOSCOPE FOR TRACHEAL INTUBATION IN A PATIENT WITH AN UNCOMMON GIANT LIPOMA ON THE POSTERIOR ASPECT OF NECK AND ADDITIONAL RISK FACTORS OF ANTICIPATED DIFFICULT AIRWAY: A CASE REPORT.

Patients with restricted neck movement present a difficult airway situation because of improper positioning and inadequate extension of the atlanto-occipital joint. The Airtraq optical laryngoscope is a new single use device that permits an indirect view of the glottis without the need to achieve a direct line of sight by conventional use of the 'sniffing position'. We present and discuss a case of uncommon giant lipoma (16 x 12 x 10 cm) in the posterior aspect of the neck in addition with other independent factors of anticipated difficult airway, intubated successfully in the semi-lateral position with the use of Airtraq.

Vaginal delivery in a patient with pheochromocytoma, medullary thyroid cancer, and primary hyperparathyroidism (multiple endocrine neoplasia type 2A, Sipple's syndrome).

Multiple endocrine neoplasia 2A (MEN 2A), or Sipple's syndrome is a rare inherited dominant syndrome, characterised by medullary thyroid carcinoma, adrenal pheochromocytoma and hyperparathyroidism, due to specific RET proto-oncogene mutations. The women with MEN 2A syndrome are at risk of complicated pregnancy because of unrecognised pheochromocytoma and transmission of RET mutation to the progeny. We report a case of a woman with MEN 2A diagnosed in early pregnancy. Alpha-blockade medical therapy was used effectively and time was given for fetal maturation. Uncomplicated vaginal delivery performed under epidural analgesia. Six weeks postpartum adrenalectomy, thyroidectomy and parathyroidectomy were performed uneventfully.

Impossible mask ventilation after an unusually low dose fentanyl-induced muscle rigidity in a patient with essential tremor: a case report and review of the literature.

Although opioid-induced muscle rigidity occurs more commonly with large doses and rapid administration of the drugs, there is a number of cases reported, where muscle rigidity was experienced with lower doses of opioids. We present and discuss a case of muscle rigidity induced by an unusually low dose of fentanyl as primary agent during induction of anesthesia. A 79 year old male patient, scheduled for hernia repair, and with a preoperative physical examination of slight hand tremor, received a bolus of 100 mcg (1.2 mcg/kg) fentanyl as primary agent for induction. About 40 sec later he stopped responding, lost consciousness and developed neck and masseter muscle spasm with jaw closure and thoracoabdominal rigidity. Blood pressure was increased significantly. Ventilation was impossible. Rapid oxygen desaturation led us to proceed with IV propofol 150 mg and suxamethonium 100 mg. Opioid-induced muscle rigidity may cause life-threatening respiratory compromise and should be readily recognized and treated by anesthesiologists.

Evaluation of an intraoperative algorithm based on near-infrared refracted spectroscopy monitoring, in the intraoperative decision for shunt placement, in patients undergoing carotid endarterectomy.

BACKGROUND: We evaluated whether the use of an intraoperative algorithm based on cerebral oximetry with near-infrared refracted spectroscopy (NIRS) monitoring, could aid in the intraoperative decision for shunt placement, in patients undergoing carotid endarterectomy (CEA). METHODS: In this prospective, randomized, controlled study were included 253 patients who underwent CEA under general anesthesia. They were randomly allocated in Group A (n=83) using NIRS monitoring and the suggested algorithm, Group B (n=84) using NIRS monitoring without the algorithm and Group C (n=86) who served as controls. Shunt placement criterion for Group A and B was 20% drop in ipsilateral regional saturation from the baseline value recorded before surgery. Primary endpoint of the study was to evaluate the use of the intraoperative algorithm based on NIRS monitoring, in the intraoperative decision for shunt placement, in patients undergoing carotid endarterectomy. Additionally, we examined whether this might affect the rate of postoperative neurologic deficits. RESULTS: When compared with Group A, Group B and Group C had 3.7 times (99% c.i. 1.5-9.5) and 70.6 times (99% c.i. 15-724.3) respectively, greater likelihood of having a shunt placed. When compared with Group B, Group C had 19.4 times (99% c.i. 4.3-191.2) greater likelihood of having a shunt placed. Regarding the rate of postoperative neurologic deficits no significant difference was found between the three groups. CONCLUSIONS: The use of a specific algorithm based on NIRS monitoring, in patients undergoing CEA, may aid in the intraoperative decision for shunt placement.

Comparison of standard polyvinyl chloride tracheal tubes and straight reinforced tracheal tubes for tracheal intubation through different sizes of the Airtraq laryngoscope in anesthetized and paralyzed patients: a randomized prospective study.

BACKGROUND: The authors compared the intubation success rate of straight reinforced tracheal tubes emerging from the Airtraq laryngoscope (Prodol Meditec S.A., Vizcaya, Spain) with standard preformed polyvinyl chloride tracheal tubes in anesthetized patients. METHODS: The authors randomly allocated 347 adult patients to be intubated with standard polyvinyl chloride tracheal tubes, standard straight wire-reinforced tracheal tubes, or silicone straight wire-reinforced tubes, through either the larger or the smaller adult laryngoscope sizes. The possible influence of laryngoscope size, tube size, and tube type on intubation failure was examined. RESULTS: Success rates were 100% for polyvinyl chloride tracheal tubes, 78.5% for standard wire-reinforced tracheal tubes, and 75.4% for silicone wire-reinforced tubes (P < 0.01). Compared with the former, patients in the straight standard and silicone wire-reinforced tube groups required more optimization maneuvers (4.1% vs. 42.1%; P < 0.01) and more attempts at successful intubation (0% vs. 7.3%; P < 0.01). The angle created by the tube emerging from the Airtraq guiding channel was inversely correlated to the ratio of the endotracheal tube OD to the width of the channel in the standard and silicone wire-reinforced tube groups (r = -0.95 and r = -0.82, respectively; P < 0.01). Finally, a decrease in 0.1 of the above ratio was associated with a 3.1 (95% confidence interval, 1.9-5.2; P < 0.01) times increase in the odds ratio of intubation failure. CONCLUSIONS: Standard polyvinyl chloride tracheal tubes were found to be superior compared with standard and silicone straight wire-reinforced tubes for intubation through the Airtraq laryngoscope. In the latter groups, a decrease of the ratio of their OD to the width of the Airtraq guiding channel resulted in increased intubation failure.

Upper cervicothoracic sympathetic block increases blood supply of unipedicled TRAM flap.

A prospective clinical study was conducted to evaluate the impact of upper cervicothoracic sympathetic block (CTGB) on blood supply of the unipedicled transverse rectus abdominis musculocutaneous (TRAM) flap. The use of the technique is first reported herein, as a manipulation improving arterial blood flow within the flap in high-risk patients, thus reducing postoperative morbidity. From March 2003 to September 2006, 28 heavy smokers, who underwent delayed breast reconstruction with unipedicled TRAM flap, were included in the study. Intraoperative upper cervicothoracic block (ganglia C5,6,7 and T1,2) was performed in 16 patients (group A), while 12 patients, who did not consent to have the blockade (group B), were the control. Clinical evaluation and thermographic monitoring of skin temperature, using the Thermacam A40 (FLIR systems, Wilsonville, OR), was used in all patients and determined the blood flow within the flap. All patients were monitored for early and late complications. In all group A patients, CTGB resulted in TRAM flap temperature increase within 9.5 to 16 min. Flap temperature elevation was found to be significantly higher (P < 0.001) and hospital stay was significantly shorter (P = 0.004) in group A patients. No CTGB or TRAM flap complications were recorded in group A patients. However, in group B, major fat necrosis occurred in 2 patients and partial (1/3) flap necrosis in 1 patient. Upper cervicothoracic sympathetic block is a reliable, safe, and useful technique for increasing blood flow within TRAM flaps in high-risk patients, like heavy smokers, and it minimizes postoperative morbidity.