Anesthetic management of an infant with Joubert syndrome for cardiac surgery

Bhaskar, P.; John, Jiju; Sivamurthy, Suresh K.; Lone, Reyaz Ahmad; Tysarowski, Pawel A.; Riyas, Mohammed; Syed, Syed A.; Bhat, Akhlaque N.; Sallehuddin, Ahmed

doi:10.1016/j.jclinane.2013.02.013

Cited by 10 publications

(11 citation statements)

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“…Dexmedetomidine is currently the most widely used α2 adrenergic receptor agonist in the clinical treatment ( 10 ). Dexmedetomidine can induce physiological sleep, achieve certain synergistic analgesic and sympathetic inhibitory effects ( 11 ). Previous studies ( 12 ) have shown that dexmedetomidine can reduce body stress, inflammation, and inhibit oxidation reaction.…”

Section: Discussionmentioning

confidence: 99%

Effects of dexmedetomidine on postoperative cognitive function in patients undergoing coronary artery bypass grafting

Gong

Zhong

et al. 2018

Exp Ther Med

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Effects of dexmedetomidine on postoperative cognitive function in patients undergoing coronary artery bypass grafting were investigated. Eighty patients undergoing systemic anesthesia with extracorporeal coronary artery bypass grafting in The People's Hospital of Guangxi Zhuang Autonomous Region from January 2015 to August 2017 were selected and randomly divided into the observation group (n=40) and control group (n=40). The two groups were treated with dexmedetomidine and equal volume of normal saline, respectively. Moreover, safety indexes including EEG bispectral index (BIS) at 30 min before induction of anesthesia (T0), immediately after intubation (T1), when incision was made (T2), when chest was closed (T3), when operation was completed (T4) and at 6 h after operation (T5), intraoperative circulatory system-related complications, cortisol, epinephrine and norepinephrine levels at the end of surgery as well as anesthesia recovery time and postoperative mechanical ventilation time were recorded and compared. All the patients were followed up for 1 week. Mini-mental state examination (MMSE) and Montreal cognitive assessment (MoCA) were administered at 1, 3 and 7 days after operation, and the incidence of intraoperative awareness and postoperative cognitive dysfunction was recorded. BIS value in the observation group was lower than that in the control group (P<0.05) at T1-T4 time points, and the BIS value in the observation group was higher than that in the control group (P<0.05) at T5. Incidence rates of intraoperative arrhythmia, hypertension and hypotension in the observation group was significantly lower than those in the control group (P<0.05). At the end of operation, levels of cortisol, epinephrine and norepinephrine in the observation group were significantly lower than those in the control group (P<0.05). Anesthesia recovery time and postoperative mechanical ventilation time in the observation group was significantly shorter than the time in the control group (P<0.05). MMSE and MoCA scores of the observation group were better than those of the control group (P<0.05). The incidence of cognitive impairment and postoperative cognitive impairment in the observation group was significantly lower than those in the control group (P<0.05). Therefore, it is concluded that dexmedetomidine can effectively reduce the incidence of postoperative cognitive impairment in patients undergoing coronary artery bypass grafting, and it is of high safety for circulatory function.

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Section: Discussionmentioning

confidence: 99%

Effects of dexmedetomidine on postoperative cognitive function in patients undergoing coronary artery bypass grafting

Gong

Zhong

et al. 2018

Exp Ther Med

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“…Given their known structural abnormalities of the brainstem, central respiratory control problems, airway hypotonia and swallow dysfunction, they should be considered high risk for anesthesia. In at least 20 individuals reported in the literature, apnea is a common complication, sometimes requiring airway intervention such as intubation or mask airway (Atalay, Soylu, & Tekcan, ; Bhaskar et al, ; Buntenbroich & Dullenkopf, , ; Galante, Meola, Cinnella, & Dambrosio, ; Habre, Sims, & D'Souza, ; Matthews, ; Platis et al, ; Sriganesh et al, ; Sriganesh, Smita, & Aravind, ; Vodopich & Gordon, ). Some authors suggest avoiding sedatives known to be associated with central respiratory depression, and avoiding unnecessary conscious sedation for brief, uncomplicated procedures such as MRI when possible; however, insufficient evidence exists to provide specific guidelines for individuals with JS.…”

Section: Methodsmentioning

confidence: 99%

“…of the brainstem, central respiratory control problems, airway hypotonia and swallow dysfunction, they should be considered high risk for anesthesia. In at least 20 individuals reported in the literature, apnea is a common complication, sometimes requiring airway intervention such as intubation or mask airway (Atalay, Soylu, & Tekcan, 2016;Bhaskar et al, 2013;Buntenbroich & Dullenkopf, 2013a, 2013bGalante, Meola, Cinnella, & Dambrosio, 2009;Habre, Sims, & D'Souza, 1997;Matthews, 1989;Platis et al, 2006;Sriganesh et al, 2014;Sriganesh, Smita, & Aravind, 2010;Vodopich & Gordon, 2004 the absence of abnormalities is not a guarantee that the fetus is unaffected. The decision to pursue or decline alternative reproductive options is very personal, and discussion with a geneticist or genetic counselor may provide an opportunity to explore all available options.…”

Section: Sedation and Anesthesiamentioning

confidence: 99%

Healthcare recommendations for Joubert syndrome

Bachmann‐Gagescu

Dempsey

Bulgheroni

et al. 2019

American J of Med Genetics Pt A

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Joubert syndrome (JS) is a recessive neurodevelopmental disorder defined by a characteristic cerebellar and brainstem malformation recognizable on axial brain magnetic resonance imaging as the "Molar Tooth Sign". Although defined by the neurological features, JS is associated with clinical features affecting many other organ systems, particularly progressive involvement of the retina, kidney, and liver. JS is a rare condition; therefore, many affected individuals may not have easy access to subspecialty providers familiar with JS (e.g., geneticists, neurologists, developmental pediatricians, ophthalmologists, nephrologists, hepatologists, psychiatrists, therapists, and educators). Expert recommendations can enable practitioners of all types to provide quality care to individuals with JS and know when to refer for subspecialty care. This need will only increase as precision treatments targeting specific genetic causes of JS emerge. The goal of these recommendations is to provide a resource for general practitioners, subspecialists, and families to maximize the health of individuals with JS throughout the lifespan.

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“…Previous reports have described total intravenous anesthesia with propofol–remifentanil for dental extraction , inhalational anesthesia for dental treatment and suturing and meningocoele repair with frequent perioperative apnea and oxygen desaturation , hernia repair under inhalational anesthesia , combined caudal and general anesthesia for inguinal hernia repair , and dexmedetomidine–remifentanil combination for cardiac surgery . None of the patients described were breathing spontaneously during sedation like in our series.…”

Section: Discussionmentioning

confidence: 50%