General anesthesia for dental treatment in a Williams syndrome patient with severe aortic and pulmonary valve stenosis: suspected episode of postoperatively malignant hyperthermia

Kohase, Hikaru; Wakita, Ryou; Doi, Syozaburoh; Umino, Masahiro

doi:10.1016/j.tripleo.2007.04.013

Cited by 7 publications

(6 citation statements)

References 16 publications

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“…General anesthesia is usually recommended for dental treatment in an attempt to avoid anxiety caused by the procedure and for behavioral control in patients with extensive dental needs. Also, this procedure is considered to be safer for the patient and may prevent cardiovascular exacerbation, since conventional dental therapy could be very difficult due to the patient's mental retardation [4,10]. In the present case, it was possible to treat the patient under local anesthesia using conventional behavior management since the patient was cooperative and did not present any cardiac abnormality.…”

Section: Discussionmentioning

confidence: 86%

“…Williams syndrome (WS) is a rare multisystem disorder, first described by Williams et al, [1] that is caused by the deletion of 26 contiguous genes, including elastin (ELN) on chromosome 7q11.23 [2]. This genomic disorder with an incidence of 1: 7,500 [3] is mainly characterized by: connective tissue abnormalities affecting the cardiovascular organs, dysmorphic facial features known as "elfin face", a unique cognitive profile, short stature, behavior characteristics and neurological abnormalities [4].…”

Section: Introductionmentioning

confidence: 99%

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Orofacial Findings and Dental Management of a Child with Williams Syndrome

Pereira

Soares

Pedro

et al. 2016

Rev. Odonto Ciênc. (Online)

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Williams syndrome (WS) is a multisystem, congenital and panethnic disorder characterized by developmental and physical abnormalities. Developmental abnormalities in dentition are frequently observed in various patients with craniofacial syndromes, however there are a few reports regarding dental management in patients with WS. The aim of the present report is to describe the case of a 6-year-old child with characteristic facial features of WS with remarkable orofacial findings and the ensuing dental management. An intraoral examination revealed a mixed dentition period, with tooth agenesis and caries requiring treatment such as extraction, restoration and fluoride application. The dental treatment was carried out under local anesthesia and was completed successfully. Since then clinical and radiographic evaluations have been conducted periodically.

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Section: Discussionmentioning

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Orofacial Findings and Dental Management of a Child with Williams Syndrome

Pereira

Soares

Pedro

et al. 2016

Rev. Odonto Ciênc. (Online)

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“…Curative dental treatment was also necessary because dental caries could pose a risk to endocarditis due to focal infections. 13 A medical evaluation to rule out cardiovascular abnormalities which could affect dental treatment is mandatory. Cases of sudden death have been reported with the administration of anesthetic in these individuals.…”

Section: Discussionmentioning

confidence: 99%

“…12 In this case, general anesthesia was performed for dental treatment because somatic and emotional stress, such as pain, fear, excitement, and anxiety caused by the conventional dental treatment can lead to cardiovascular exacerbation. 13 The treatment should be performed comprehensively by involving pediatric dentists, oral surgeons, cardiologists, anesthesiologists, and pediatricians to determine treatment procedures, prevent infective endocarditis, and improve dental treatment quality. 12…”

Section: Introductionmentioning

confidence: 99%

William-Beuren Syndrome: Comprehensive Dental Care in Child Patient

Marzuqi¹,

Ainayah²,

Mufida³

et al. 2021

Acta Med Philipp

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The most common symptoms of Williams-Beuren syndrome (WBS) are heart defects and unusual facial features. The objective of this case report was to highlight the preparation of comprehensive dental care for this syndrome. A seven-year-old boy came with multiple dental caries as a chief complaint. Parents reported a history of corrected tetralogy of Fallot (ToF) three years ago. The routine medical check-up was carried out annually. Based on the typical signs and symptoms, a diagnosis of William-Beuren Syndrome was made. Comprehensive dental care under general anesthesia on a patient with a history of heart defect and multiple dental caries was the best choice and exhibits a high success rate. Extraction treatment was carried out to avoid the risk of endocarditis infection, restoration to prevent the progressivity of caries, and space maintainers to restore function and aesthetics.

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“…Several options exist for intravenous induction with a drug that maintains contractility and coronary perfusion while it ensures intravenous access to give vasopressors rapidly. Etomidate has been used with success at our institution and elsewhere in such patients . High‐dose opiate inductions have long been used safely in patients at risk for myocardial ischemia .…”

Section: Management Of Anesthesiamentioning

confidence: 99%

Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review

Matisoff

Olivieri

Schwartz

et al. 2015

Pediatric Anesthesia

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Since the first description in 1961, several case reports have documented an increased incidence of anesthesia-related cardiac arrest in patients with Williams-Beuren syndrome, commonly known as Williams syndrome (WS). Widespread arteriopathy secondary to an elastin gene defect results in various cardiac defects, including supravalvar aortic stenosis (SVAS) and coronary artery anomalies, which can increase the risk of myocardial ischemia. Even though patients with WS are known to have increased risk of adverse events during anesthesia and sedation, they often undergo several procedures that require anesthesia during their lifetimes, and cases of perianesthetic cardiac arrest continue to be reported. To date, no prospective studies have been reported that quantify anesthetic risk in individual patients with WS. In this article, we review the clinical manifestations of WS, propose a consensus, expert-informed method to estimate anesthetic risk based on the current literature, and provide recommendations for periprocedural management of this patient population.

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General anesthesia for dental treatment in a Williams syndrome patient with severe aortic and pulmonary valve stenosis: suspected episode of postoperatively malignant hyperthermia

Cited by 7 publications

References 16 publications

Orofacial Findings and Dental Management of a Child with Williams Syndrome

Orofacial Findings and Dental Management of a Child with Williams Syndrome

William-Beuren Syndrome: Comprehensive Dental Care in Child Patient

Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review

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