Gastroesophageal Reflux and Reflux Oesophageal Strictures in Children with Cornelia de Lange Syndrome

Vincent, M; Duncan, N. D.

doi:10.7727/wimj.2012.096

Cited by 2 publications

(2 citation statements)

References 33 publications

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“…CdLS is a rare genetic disorder characterized by mental retardation and distinctive physical anomalies; various systemic medical problems may coexist. Mutations in several genes, including NIPBL gene on chromosome 5, SMC1A on the X chromosome and SMC3 on chromosome 10 are thought to be responsible for this syndrome [5]. The estimated prevalence is 1 in 10,000 to 1 in 30,000, and two-thirds of children with CdLS die within the first year of life [6].…”

Section: Discussionmentioning

confidence: 99%

Successful difficult airway management using GlideScope video laryngoscope in a child with Cornelia de Lange Syndrome

Park

Choi

Park

et al. 2018

Yeungnam Univ J Med

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Management of airway in a child with Cornelia de Lange Syndrome (CdLS) should be given due consideration because most of them have the problems related to difficult airway. The GlideScope video laryngoscope can be attempted during routine intubation, however it is mostly used in case of difficulty. With adequate preoperative airway assessment, we used the pediatric video laryngoscope as useful alternative airway device in a child with CdLS and orotracheal intubation proceeded uneventfully.

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Section: Discussionmentioning

confidence: 99%

Successful difficult airway management using GlideScope video laryngoscope in a child with Cornelia de Lange Syndrome

Park

Choi

Park

et al. 2018

Yeungnam Univ J Med

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“…Upper airways obstruction may occur even during light anesthesia [Stavinoha et al, ]. CdLS patients often suffer from gastroesophageal reflux [Vincent and Duncan, ], which highly increase their risk for aspiration pneumonia [Schrier et al, ]. Unpredictable responses to commonly used anesthetic drugs have been documented [Stevic et al, ].…”

Section: Introductionmentioning

confidence: 99%

Sedation and general anesthesia for patients with Cornelia De Lange syndrome: A case series

Moretto¹,

Scaravilli

Ciceri

et al. 2016

American J of Med Genetics Pt C

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Cornelia De Lange syndrome (CdLS) is a rare congenital disease characterized by typical facial dysmorphism, developmental disability, and limb deficiency defects. Various congenital malformations and medical complications have been described with gastroesophageal reflux as the major one. CdLS patients often require multiple high-risk anesthetic procedures. At San Gerardo Hospital (Monza, Italy) the management of CdLS patients is routinely organized through a standard protocol and a dedicated pediatric anesthesia team has been implemented. We report on a retrospective descriptive analysis of the anesthetic records of the CdLS patients admitted to San Gerardo Hospital from January 2010 to December 2015. We retrieved: demographics, genetic profiles, type of procedures, anesthetic approaches, anesthetics usage and complications. Data are reported as median (interquartile range) values. Twenty-seven patients (11 female), with age 12 (7-15) years old, weight 24 (14-35) kg, and severity score of 25 (18-32) were included. NIBPL mutations were the most frequently represented. We analyzed 58 procedures (30 esophagogastroduodenoscopies, 8 evoked auditory potential tests, 5 radiodiagnostics, 5 catheters positioning, 4 bronchoscopies) managed by sedation (36) and general anesthesia (6). Each patient underwent one (1-2) anesthetic procedure. Propofol (59%), sevoflurane (31%), fentanyl (24%), and ketamine (10%) were used. Three out of six endotracheal intubations were difficult. The only documented intraoperative complications were three episodes of desaturation (oxygen saturation <90%) occurring during sedations and were managed without the need for an invasive control of the airways. Implementation of a specific management protocol and a dedicated allowed to provide anesthesia to CdLS patients without the occurrence of major complications. © 2016 Wiley Periodicals, Inc.

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Gastroesophageal Reflux and Reflux Oesophageal Strictures in Children with Cornelia de Lange Syndrome

Cited by 2 publications

References 33 publications

Successful difficult airway management using GlideScope video laryngoscope in a child with Cornelia de Lange Syndrome

Successful difficult airway management using GlideScope video laryngoscope in a child with Cornelia de Lange Syndrome

Sedation and general anesthesia for patients with Cornelia De Lange syndrome: A case series

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