Anesthetic considerations in a child with Sotos syndrome -A case report-

Chung, Joo-Young; Kim, Gahyun; Park, Ju-Hun; Choi, Hyen Kyu; Park, Byoung Hark; Choi, Mi Young; Kim, Jong-Sool

doi:10.17085/apm.2017.12.3.240

Cited by 3 publications

(4 citation statements)

References 12 publications

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“…Despite preexisting hypotonia, we also did not observe any excessive sensitivity to nondepolarizing neuromuscular blockade or delay in return of neuromuscular function following administration of sugammadex. This is also consistent with published reports in the pediatric population [ 4 , 7 , 8 ], wherein there were no issues with neuromuscular blockade despite pronounced hypotonia.…”

Section: Discussionsupporting

confidence: 92%

“…However, the patient had an easy mask airway and easy intubation with direct laryngoscopy. This is consistent with the experience of other authors in the management of airways in children with Sotos syndrome [ 4 – 10 ], wherein there was no difficulty with intubation. Despite preexisting hypotonia, we also did not observe any excessive sensitivity to nondepolarizing neuromuscular blockade or delay in return of neuromuscular function following administration of sugammadex.…”

Section: Discussionsupporting

confidence: 92%

“…We present a case of an 18-year-old male with Sotos syndrome who had multiple dental extractions under general anesthesia and summarize previously published cases of Sotos syndrome anesthetics. In searching the literature, we found seven case reports describing anesthesia in children with Sotos syndrome [ 4 – 10 ]. However, we were not able to find any reports on the anesthetic management of adults with Sotos syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Successful Anesthetic Management of an Adult with Sotos Syndrome

Winegarner

Kendall

Lecamwasam

2022

Case Reports in Anesthesiology

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Sotos syndrome is a rare genetic disorder presenting with craniofacial abnormalities, profound hypotonia, and cardiac abnormalities, giving rise to several potential challenges and concerns for an anesthesiologist. When preparing for a Sotos syndrome patient’s case, we consulted the literature for precedents on how to plan the anesthetic, to which we were only able to find a few reports and nothing in the age group our patient fell within. We present our case of an adult in addition to examining the previous cases so as to document a precedent when encountering patients with this syndrome in the operating room. We describe a unique case of a nonverbal adult with hypotonia and severe craniofacial abnormalities who successfully underwent multiple dental extractions under general anesthesia, with no complications other than a delay of emergence attenuated by naloxone. Our case and the seven previous documented cases over the past several decades demonstrate anesthesia, including paralytics and intubation itself safe despite obvious concerns given the common features of the syndrome for both pediatric patients and the one adult we described in this report.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 92%

Section: Introductionmentioning

confidence: 99%

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Successful Anesthetic Management of an Adult with Sotos Syndrome

Winegarner

Kendall

Lecamwasam

2022

Case Reports in Anesthesiology

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“…In mitochondrial myopathies and myotonic syndromes, the use of propofol is considered contraindicated due to the serious complications, but it showed safe in the case report described above. Volatile anesthetics can lead to rhabdomyolysis, hyperkalemia and asystole in patients with muscular dystrophies (55,56). There is no available literature about non-depolarizing muscle relaxants effect in this disorder.…”

Section: Ullrich Congenital Muscular Dystrophy (Ucmd)mentioning

confidence: 99%

Anesthesia and rare neuromuscular diseases

Radeka

Stojanović

Vasilijic

et al. 2023

Front. Anesthesiol.

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Neuromuscular diseases, although rare in the general population, can be present in people who need to undergo surgery and anesthesia. Fortunately, the possibility of early diagnosis, new therapeutic approaches and the application of a multidisciplinary approach have significantly extended the life expectancy of such patients. The presence of such a disorder represents a challenge in anesthesiology practice, both during the preparation for surgery itself, the operative procedure and postoperative recovery in intensive care units. A basic understanding of the main disorders of the disease and their potential interactions with the anesthetic itself and the necessary agents in anesthesiology is necessary in order to reduce the risk of perioperative morbidity and mortality, with the aim of reducing the possibility of complications. Rare muscle diseases are very difficult to classify in a standard way because of the variability of their etiology and pathology. This work focuses on a number of muscle diseases such as Dubowitz syndrome, rhabdomyosarcoma in children, spinal muscular atrophy, Kennedy's disease, Congenital muscular dystrophy of the Ullrich type (UCMD), Sotos syndrome, Polymyositis and Setleis syndrome, Nemaline rod myopathy, Pompe disease, Emery-Dreifuss muscular dystrophy (EDMD). The medical literature on anesthetic techniques and perioperative complications was reviewed. Given that they can have a very unfavorable effect on the course of general anesthesia in particular and that these diseases can cause very serious, even life-threatening complications, an adequate anesthesiological approach is very important to reduce the possibility of the mentioned complications. A multidisciplinary approach is imperative for such patients before and after surgery, whether it is performed under analgosedation, some type of regional or general anesthesia. The operative procedure itself must be performed in institutions that are adequately equipped to treat such patients and by personnel who have experience in treating them.

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