BackgroundPatients suffering from mucopolysaccharidosis are among the most complex from the anesthesiological point of view, especially regarding the management of the airway. The evidence base for anesthesia management is often limited to case reports and small case series.AimsTo identify useful information about experience with each subtype of mucopolysaccharidosis reported in the literature and propose a guide on the best options for airway management to the anesthesiologists who take care of these patients.MethodsA query of the PubMed database specific for “anesthesia” and “mucopolysaccharidosis” and a further query specific for “mucopolysaccharidosis and difficult airway management” was conducted. We looked for those items that offered practical guidance to anesthesiological management. We did not exclude case reports, especially those that reported a specific technique, because of their practical suggestions.ResultsWe identified 15 reviews, 17 retrospective case series, 5 prospective studies, and 28 case reports that focused on airway managements in anesthesia or had practical suggestions for preoperative evaluation and risk assessment. An accurate preoperative evaluation and the need for an experienced team are emphasized in all the reviewed articles and for each type of mucopolysaccharidosis. Many suggestions on how to plan the perioperative period have been highlighted. Insertion of a laryngeal mask airway generally improves ventilation and facilitates intubation with a fiberoptic bronchoscope. Furthermore, the videolaryngoscope is very useful in making intubation easier and facilitating bronchoscope passage.ConclusionsPatients with mucopolysaccharidosis are at high risk for anesthesia-related complications and require a high level of attention. However, a multidisciplinary approach, combined with expertise in the use of new techniques and new devices for airway management, makes anesthesiological management safer. Further research with prospective studies would be useful.Electronic supplementary materialThe online version of this article (10.1186/s13052-018-0554-1) contains supplementary material, which is available to authorized users.

Clonidine is effective in treating sevoflurane-induced postanesthesia agitation in children. We conducted a study on 169 children to quantify the risk reduction of clonidine agitation in patients admitted to our day-surgery pediatric clinic. Children were randomly allocated to receive clonidine 2 mug/kg or placebo before general anesthesia with sevoflurane that was also supplemented with a regional or central block. An observer blinded to the anesthetic technique assessed recovery variables and the presence of agitation. Pain and discomfort scores were significantly decreased in the clonidine group; the incidence of agitation was reduced by 57% (P = 0.029) and the incidence of severe agitation by 67% (P = 0.064). Relative risks for developing agitation and severe agitation were 0.43 (95% confidence interval, 0.24-0.78) and 0.32 (0.09-1.17), respectively. Clonidine produces a substantial reduction in the risk of postsevoflurane agitation in children.

Background: We report a case of mucormycosis in a healthy 17-year-old accident victim with multiple abdominal injuries which was caused by infection with Absidia Corymbifera, a ubiquitous saphrophyte in the ground.

The authors report their experience in laparoscopic repair of inguinal hernias in children. From May 2010 to November 2013, 122 patients with inguinal hernia underwent laparoscopic herniorrhaphy (92 males and 30 females). Telescope used was 5 mm, while trocars for the operative instruments were 3 or 2 mm. After introducing the camera at the umbilical level and trocars in triangulation, a 4-0 nonabsorbable monofilament suture was inserted directly through the abdominal wall. The internal inguinal ring was then closed by N or double N suture. All operations were performed in one-day surgery setting. In the case of association of inguinal and umbilical hernia an original technique was performed for positioning and fixing the umbilical trocar and for the primary closure of the abdominal wall defect. The postoperative follow-up consisted of outpatient visits at 1 week and 1, 3, and 6 months. The mean age of patients was 38.5 months. Of all patients, 26 were also suffering from umbilical hernia (19 males and 7 females). A total of 160 herniorrhaphies were performed; 84 were unilateral (66 inguinal hernia, 18 inguinal hernia associated with umbilical hernia), 38 bilateral (30 inguinal hernia, 8 inguinal hernia associated with umbilical hernia). Nine of 122 patients (6 males and 3 females) were operated in emergency for incarcerated hernia. A pre-operative diagnosis of unilateral inguinal hernia was performed in 106 cases. Of these patients, laparoscopy revealed a controlateral open internal inguinal ring in 22 cases (20.7%). The mean operative time was 29.9±15.9 min for the monolateral herniorrhaphies, while in case of bilateral repair the mean operative time was 41.5±10.4 min. The mean operative time for the repair of unilateral inguinal hernia associated with umbilical hernia was 30.1±7.4 while for the correction of bilateral inguinal hernia associated with umbilical hernia 39.5±10.6 min. There were 3 recurrences (1.8%): 2 cases in unilateral repair and 1 case a unilateral recurrence in a bilateral repair. No other complications were seen. Laparoscopic repair of inguinal hernia in children performed in this experience resulted a safe and effective procedure.

In this pilot study, we did not detect a difference between the postoperative analgesic requirements of the patients who received a single intraoperative dose of dexmedetomidine 0.5 lgAEkg )1 or morphine 50 lgAEkg )1 . Under the conditions of this study the use of dexmedetomidine was not associated with adverse effects. This pilot study can serve as the basis for a larger study to compare the effectiveness and side effect profile of single intraoperative doses of dexmedetomidine and morphine for postoperative pain in pediatric T&A patients. References1 Tobin JR, Shafer SL, Davis PJ. Pediatric research and scholarship: another Gordian knot. Anesth Analg 2006; 103: 43-48. 2 Hall JE, Ulrich TD, Barney JA et al. Sedative, amnestic and analgesic properties of small-dose dexmedetomidine infusions. Anesth Analg 2000; 90: 699-705. 3 McGrath PJ, Johnson G, Schillinger J et al. CHEOPS: a behavioral scale for rating postoperative pain in children. Adv Pain Research Therapy. 1985; 9: 395-402. 4 Petroz GC, Sikich N, James M et al. A phase 1, two-center study of the pharmacokinetics and pharmacodynamics of dexmedetomidine in children. Anesthesiology 2006; 105: 1098-1100.