Anesthetic Management of Two Pediatric Patients With Concurrent Diagnoses of Mitochondrial Disease and Malignant Hyperthermia Susceptibility: A Case Report.

We report the case of 2 pediatric patients with coexisting diagnoses of malignant hyperthermia susceptibility and mitochondrial disease in 2 different surgical settings. Due to the rare occurrence of each disorder, and even more so together, we reviewed evidence-based anesthetic concerns and described our perioperative management, with the goal of aiding future practitioners in safely caring for these patients. Consent was obtained for both patients, as well as IRB approval before publication.

Normovolemic hemodilution using hydroxyethyl starch 130/0.4 (Voluven) in a Jehovah's Witness child requiring cardiopulmonary bypass for ventricular septal defect repair.

Surgical repair of congenital heart disease during cardiopulmonary bypass is common, and performing these complicated procedures in the absence of blood transfusions is especially challenging. A case of a Jehovah's Witness child who underwent surgical repair of a ventricular septal defect utilizing a new tetrastarch for autologous normovolemic hemodilution is reported. A successful operative repair was achieved without the need for non-autologous blood transfusion.

Postoperative malignant hyperthermia: an analysis of cases from the North American Malignant Hyperthermia Registry.

BACKGROUND: The initial presentation of malignant hyperthermia (MH) may begin in the postoperative period. However, the maximal latency period between the end of anesthesia care and the onset of postoperative MH is unknown. The authors hypothesized that this latency period is short and is not manifested by hyperthermia as the initial presenting sign. The authors sought to test this hypothesis and to describe the clinical characteristics of postoperative MH by analysis of suspected cases in the North American Malignant Hyperthermia Registry. METHODS: Of 528 possible or suspected cases of MH in the North American Malignant Hyperthermia Registry, the authors identified 64 possible reports of postoperative MH. The records were reviewed in detail by the authors, each of whom assigned a qualitative score of "likely," "not likely," "not enough information available," or "not applicable" (where MH was not the final definitive diagnosis). Postoperative MH was confirmed after a consensus meeting of the three senior authors who reviewed in detail all possible "likely" cases. RESULTS: The authors identified postoperative MH in 10 subjects. All received volatile agents and 5 also received succinylcholine. All demonstrated signs characteristic of acute MH, including generalized rigidity, hypercapnia and/or tachypnea, tachycardia, and hyperthermia. No subject demonstrated hyperthermia as the presenting sign. The latency period between the anesthesia finish time and the onset of a sign indicative of acute MH ranged from 0 to 40 min. CONCLUSIONS: Postoperative MH is uncommon, occurring in 10 of 528 suspected MH cases (1.9%) reported to the North American Malignant Hyperthermia Registry. Postoperative MH began shortly after completion of the anesthetic care. Hyperthermia was not a presenting sign of MH.

The effect of 3-year treatment with 0.25 mg/day of micronized 17beta-estradiol on cognitive function in older postmenopausal women.

OBJECTIVES: To evaluate the effect of ultra-low-dose (0.25 mg/d) micronized 17beta-estradiol on cognitive function in older postmenopausal women. DESIGN: Randomized, placebo-controlled trial conducted for 3 years. SETTING: Academic health center in greater Hartford, Connecticut. PARTICIPANTS: Fifty-seven healthy, community-dwelling, older postmenopausal women. INTERVENTION: Women received 0.25 mg/d of micronized 17beta-estradiol (estrogen therapy (ET), n=32) or placebo (n=25); all women who had not had a hysterectomy received 100 mg/d of oral micronized progesterone for 2-week periods every 6 months. MEASUREMENTS: Neuropsychological measures of memory, language, mood, and executive function were collected at baseline, 3 months, and 36 months. Measures of executive function included the Controlled Oral Word Association Test, the Trail Making Test, and the Wisconsin Card Sorting Test. The Boston Naming Test was used to measure language skills. The Symbol Digit Modalities Test was used as a measure of sustained attention. Measures of memory included the Complex Figure Test, Fuld Object Memory Test, and a selected subtest from the Wechsler Memory Scale. Scores from the Geriatric Depression Scale and the Beck Anxiety Inventory were used to assess symptoms of depression. RESULTS: No differences were found between ET and placebo on any of the neurocognitive measures or depression instruments, nor were there any differences when the groups were stratified according to age. CONCLUSION: This small study, which had adequate power to detect change in some but not all domains of cognition tested, revealed that low-dose estrogen neither benefits nor harms cognitive function in older women after 3 years of treatment, but confirmation is needed from larger trials.

The effect of short-term estradiol therapy on cognitive function in older men receiving hormonal suppression therapy for prostate cancer.

OBJECTIVES: To determine the effect of estrogen (E) alone (without the influence of testosterone (T)) on cognitive function in older men, using 17-beta micronized estradiol versus placebo in older men rendered hypogonadal (low T and E) by treatment for prostate cancer. DESIGN: Short-term double-blind, randomized, controlled trial. SETTING: An outpatient General Clinical Research Center. PARTICIPANTS: Twenty-seven community-dwelling men aged 65 and older receiving neoadjuvant or established therapy with luteinizing-hormone releasing-hormone agonists for treatment of prostate cancer enrolled in a short-term randomized, controlled trial of 17-beta micronized estradiol versus placebo on the effect on biochemical markers of bone turnover. MEASUREMENTS: Hormone levels, including E, T, and sex hormone-binding globulin; standardized neurocognitive tests, including measures of sustained attention, executive function, and memory; and questionnaires to assess subjects' perception of cognitive deficits and symptoms of depression. RESULTS: There were no significant differences between patients receiving E or placebo on 15 of 17 neurocognitive measures and no significant differences in self-reported cognitive deficits or number of depressive symptoms. CONCLUSION: Although studies have suggested that E replacement therapy may improve cognitive function, most notably memory performance in postmenopausal woman, there was no evidence in the present study that the addition of short-term E therapy was more beneficial than placebo in tests of cognitive performance in hypogonadal men.

Testing anal sphincter tone predicts the effectiveness of caudal analgesia in children.

UNLABELLED: In this study, we examined the effectiveness of caudal blocks and correlated it with the laxity of the patients' anal sphincter before emergence from anesthesia in 178 children undergoing inguinal and/or penile surgery. Bupivacaine 0.25% in a volume of 0.6-1.25 mL/kg was used in all patients. The presence of a lax anal sphincter at the end of surgery correlated significantly with the reduced administration of narcotics intraoperatively and in the postanesthesia care unit (P < 0.001). The sensitivity of the sphincter tone test was 98.1% with a 95% confidence interval (CI) ranging from 94.3% to 99.6%. The specificity of the test was 94.4% with a 95% CI of 72.0%-100%. The positive predictive value of this test in predicting adequate caudal block was excellent (99.4%) with a 95% CI of 96.1%-100%. The negative predictive value was better than average (85%) with a 95% CI of 62.9%-95.4%. We conclude that a lax anal sphincter can predict the effectiveness of analgesia after pediatric caudal blockade. A tight sphincter may suggest the need to repeat the block before the child awakens, or consider alternate methods of postoperative analgesia. IMPLICATIONS: A lax anal sphincter in children undergoing inguinal and/or penile surgery can predict the effectiveness of analgesia after pediatric caudal blockade.

Sedation and analgesia in pediatric patients for procedures outside the operating room.

Sedation and analgesia in pediatric patients for procedures outside the operating room are becoming more frequent as health care is being driven to be more cost effective and "efficient." Although anesthesiologists may not be directly involved in sedation or analgesia outside of the operating room, there is a high likelihood that they will be asked by their institutions to be integrally involved in creating and supervising sedation policy given that the American Society of Anesthesiologists and the Joint Commission on Accreditation of Healthcare Organizations consider sedation and analgesia as part of a continuum ranging from minimal sedation to moderate sedation and analgesia, deep sedation and analgesia, and, finally, general anesthesia. Further, anesthesiologists will be asked to define, teach, and credential nonanesthesiology practitioners who perform deep sedation because these practitioners are now required to be qualified to "rescue from general anesthesia."