Planning anesthesia of a child with a rare disease: useful tools.

Up to 8,000 rare diseases are currently described in the scientific literature. The presence of a rare disease constitutes an additional challenge for the practitioner given its implications on the management of anesthesia. Moreover, it is not possible for an anesthesiologist to know them all especially as the sources of information are scattered. This narrative review aims at proposing pre-anesthetic evaluation clear tools to summarize the individual aspects of a known or suspected rare disease in a patient and to define an adapted anesthetic strategy. It also describes a decision-making process, called « the rare disease reflex ¼, to guide the practitioner to quickly initiate diagnostic investigations in the presence of unusual perioperative clinical signs suggestive of an undiagnosed rare disease.

Successful ventilation through a Rüsch intubation guide catheter in severe laryngotracheal stenosis.

BACKGROUND: Providing adequate ventilation may remain complex in patients with severe proximal laryngotracheal stenosis, especially when the airway is shared with the surgeon during tracheal resection surgery. We describe an effective alternative to standard endotracheal intubation using a Rüsch flexible intubation guide catheter. METHODS: In two patients undergoing tracheal repair surgery, we failed to insert a 5.0 inner diameter endotracheal tube (6.9 mm outer diameter) or a 6.0 mm outer diameter endoscope through the laryngotracheal stenosis. However, using indirect laryngoscopy, a 6.0 outer diameter Rüsch flexible intubation guide catheter was passed successfully through the vocal cords and then through the stenosis. Controlled ventilation was achieved by means of the Rüsch guide, provided with its two large Murphy's eyes. When the trachea was opened, the Rüsch guide was removed just enough for the surgeons to place a Montandon tracheal tube, at that point taking over ventilation. A 7.0 inner diameter endotracheal cuffed tube had been inserted onto the Rüsch guide and left pending upstream from the vocal cords. Once the posterior tracheal wall was sutured, this endotracheal cuffed tube was slid along the Rüsch guide through the vocal cords with the cuff placed beyond the tracheal sutures. RESULTS: Controlled ventilation through the Rüsch flexible intubation guide catheter showed satisfying and stable ventilatory parameters in both patients. Inspiratory pressures of 25-30 mmHg were enough to reach adequate tidal volumes around 450 ml. End tidal CO2 was kept between 35 and 40 mmHg (PaCO2 showed similar values). Standard endotracheal intubation at the end of the tracheal resection was easy and safe thanks to the Rüsch guide still in place between the vocal cords. CONCLUSIONS: We suggest an effective and reliable method using a Rüsch flexible intubation guide catheter for airway management in patients suffering from laryngotracheal stenosis in the setting of tracheal repair surgery.

Dynamic view of postoperative pain evolution after total knee arthroplasty: a prospective observational study.

BACKGROUND: Having a dynamic view of postoperative pain resolution allows a better understanding of the transition towards chronic pain. Sleep and quality of life are important determinants of satisfaction after total knee arthroplasty (TKA), besides functional recovery and pain. METHODS: For 114 patients undergoing TKA we recorded the presence of pain at rest, pain evoked at movement and pain located at the incision site in the acute (postoperative day 1, 2, 3, 8), subacute (30 days, 3 months) and chronic (6 months and 1 year) period. Analgesics consumption and need of medical assistance for pain were questioned. Quality of life measured by the impact on enjoyment of life, sleep and mood were monitored. RESULTS: Average incidence for subacute pain was 54% at rest, 66% at mobilization. For chronic pain, the incidence was 14% at rest, 22% during mobilization. Pain at rest peaked at day 30 while pain during mobilization displayed a plateau between day 8 and 3 months. Three per cent of the patients complained at 1 year of pain at the incision site. 11% of patients still took analgesics one year after the surgery. More than 40% of patients reported moderate to severe alterations of sleep and quality of life in the acute period, decreasing to less than a half at one year. CONCLUSIONS: The trajectories of the different types of pain after TKA show their non-linear evolution, highlighting the need of a better pain control at precise moments. Sleep disturbances and alterations of quality of life are still present one year after the surgery.

Pheochromocytoma - when acute medicine comes to the surgeon's rescue and vice versa. Case report of a patient presenting unmanageable haemodynamic instability during elective surgery for pheochromocytoma.

We report and discuss the case of a 51-year-old patient undergoing elective laparoscopic surgery for pheochromocytoma after 10 days of medical pre-treatment. After anaesthetic induction, a deep level of anaesthesia could not prevent the onset of repeated hypertensive peaks, followed by severe hypotensive periods. Once the surgical incision was made, the patient developed acute pulmonary oedema along with significant oxygen desaturation. The decision was made to stop the surgery and transfer the patient to the intensive care unit (ICU) for further support and management. Unfortunately, additional monitoring and symptomatic treatment did not help haemodynamic stabilisation. In the absence of any external stimulation or medical support, the oscillation of blood pressure (BP) continued with peaks every 20 minutes up to 300 mmHg systolic blood pressure (SBP) and falls down to 30 mmHg SBP. The patient also sustained two episodes of cardiac arrest from which he recovered. Facing this unmanageable situation, a decision was made after a multi-disciplinary discussion to go back to surgery in order to remove the source of adrenergic stimulation. Surgery by laparotomy was performed and catecholamine substitution was provided. Nevertheless, after tumour removal, BP dropped leading to a third cardiac arrest that was successfully managed. Following a 10-day stay in the ICU, the patient left with subsequent cardiac stabilisation and full recovery.

Myotonic dystrophies type 1 and 2: anesthetic care.

SUMMARY: Myotonic dystrophy is classified as one of the myotonic syndromes although myotonia is only a minor characteristic of it. It is, in fact, also a multisystem disease with cardiac, digestive, ocular, and endocrine abnormalities. Two subgroups are currently identified with many similarities: DM1 refers to classic dystrophia myotonica (Steinert disease), while DM2, formerly called proximal myotonic myopathy has a later onset. The congenital form is present only in DM1. The genetic causes of DM1 and 2 are different but end up in a similar way of altering RNAm processing and splicing of other genes. The anesthetic risk is increased in case of DM1 type. This review summarizes current knowledge concerning the pathophysiology and anesthetic management of this disease in children and adults.

Do intraoperative analgesics influence oncological outcomes after radical prostatectomy for prostate cancer?

BACKGROUND: The potential impact of intraoperative analgesics on oncological outcome after radical prostatectomy is debated. Some investigators have suggested that use of opioids favour relapse, whereas regional analgesia and NSAIDs improve oncological outcomes. OBJECTIVE: To evaluate the impact of intraoperative analgesia (epidural and intravenous) on the incidence of biochemical recurrence-free (BRF) survival. DESIGN, SETTING AND PARTICIPANTS: This retrospective study includes 1111 consecutive retropubic radical prostatectomies (RRPs) for localised prostate cancer, performed between 1993 and 2006. Median follow-up was 38 months (interquartile range 16-69). BRF survival probabilities were compared with log-rank tests and the Cox regression model. MAIN OUTCOME MEASURES AND RESULTS: Epidural analgesia was used in 52% of patients, intravenous ketorolac in 25%, sufentanil in 97%, clonidine in 25% and ketamine in 16%. Univariate and multivariate analyses showed that intravenous sufentanil significantly reduced BRF survival rate, hazard ratio 7.78 [95% confidence interval (CI) 5.79, 9.78), for extracapsular extension stage pT 2 or less, hazard ratio 0.44 (95% CI 0.12, 0.75), Gleason score at least 7, hazard ratio 1.96 (95% CI 1.65, 2.26), positive margin, hazard ratio 1.87 (95% CI 1.58, 2.02) and lymph node involvement, hazard ratio 1.77 (95% CI 1.27, 2.27, P > 0.05). In contrast, neither epidural analgesia nor other analgesics were associated with a statistically significant effect (P > 0.05). CONCLUSION: This retrospective analysis suggests that intraoperative sufentanil administration is associated with an increased risk of cancer relapse after RRP, whereas epidural analgesia, with local anaesthetic and opioid, was not associated with a significant effect.

Randomized clinical trial on Harmonic Focus shears versus clamp-and-tie technique for total thyroidectomy.

BACKGROUND: The Harmonic Focus is the last ultrasonic device designed for thyroid surgery. The aim is to assess its efficacy and safety compared with traditional dissection in a prospective randomized trial of total thyroidectomy procedures. METHODS: Total thyroidectomy was performed in 34 patients using the Harmonic Focus, and in 34 patients using the clamp-and-tie technique. RESULTS: In the Harmonic Focus group, relative reductions of 29% and 46% were observed in surgical time and blood loss, respectively. The number of intraoperative instrument exchanges also decreased by 70%, and use of specific materials required to achieve hemostasis decreased significantly. Safety was found to be similar in both patient groups. CONCLUSIONS: Our study showed beneficial effects of Harmonic Focus use in thyroid surgery. Further studies therefore are needed to evaluate cost in the light of savings made in surgical time, materials needed for hemostasis, and human resources.

Propofol infusion syndrome associated with short-term large-dose infusion during surgical anesthesia in an adult.

In this case report we describe a case of propofol infusion syndrome in an adult after a short-term infusion of large-dose propofol during a neurosurgical procedure. Large-dose propofol (9 mg.kg(-1).h(-1)) was given for only 3 h during surgery and was followed by a small-dose infusion (2.3 mg.kg(-1).h(-1)) for 20 h postoperatively. The patient had also received large doses of methylprednisolone. He developed a marked lactic acidosis with mild biological signs of renal impairment and rhabdomyolysis but no cardiocirculatory failure. There were no other evident causes of lactic acidosis as documented by laboratory data. We believe this is the first report of reversible lactic acidosis associated with a short duration of large-dose propofol anesthesia.

Surgical clipping may lead to better results than coil embolization: results from a series of 101 consecutive unruptured intracranial aneurysms.

OBJECTIVE: Recent reports in the literature have described a significant discrepancy in adverse outcomes between coil embolization (CE; 10%) and surgical clipping (SC; 25%) for the management of unruptured intracranial aneurysms (UIA). This discrepancy led us to analyze our experience. METHODS: In 1996, we designed a prospective study of patients with UIA in which CE was considered the treatment of choice and was performed if the interventional neuroradiologists deemed the aneurysm's fundus-to-neck ratio accessible for CE. SC was performed only if complete CE was unlikely to be achieved or in patients in whom CE already had failed. RESULTS: CE was performed in 38 patients with at least one UIA (41 UIAs, 83% in the anterior circulation). SC was performed in 39 patients with at least one UIA unsuitable for CE (59 UIAs, including 6 after failed CE, 96.5% in the anterior circulation). For CE, the total obliteration rate was 56.1%, the subtotal was 14.6%, and CE failed in 29.3%. There were transient complications in 10% of the cases and permanent complications in 7.5%. Of the 12 failed CE procedures, 7 (58%) were performed for middle cerebral artery aneurysms. For SC, the total obliteration rate was 93.2%, the subtotal was 1.7%, and SC failed (wrapping) in 5.1%. There were transient complications in 16.3% of the patients and permanent complications in 1.7%. The success rate for CE was similar to that for SC only when CE was used for aneurysms with a fundus-to-neck ratio of at least 2.5. CONCLUSION: SC can produce better results than CE in patients with UIA of the anterior circulation. CE as a first-line treatment should be reserved for patients with UIAs with a fundus-to-neck ratio of 2.5 or greater.