The anaesthetic management of an infant with frontometaphyseal dysplasia (Gorlin–Cohen syndrome)

Mehta, Y.; Schou, Henning

doi:10.1111/j.1399-6576.1988.tb02775.x

Cited by 11 publications

(4 citation statements)

References 9 publications

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“…Congenital subglottic stenosis is a rare but well-known cause of stridor in infancy. Cases of subglottic stenosis have been reported to be associated with such clinical syndromes as the Fraser-like (1), frontometaphyseal (2)(3), ulnar-mammary (4) and Opitz BBBG syndromes (5)(6), as well as sponastrime dysplasia (7), but it usually occurs sporadically in otherwise healthy children, and familial occurrence has not been reported previously. This report describes siblings from two families with inspiratory stridor in infancy who had congenital subglottic stenosis not accompanied by other major anomalies.…”

mentioning

confidence: 99%

Congenital subglottic stenosis in two unrelated pairs of siblings

et al. 2004

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mentioning

confidence: 99%

Congenital subglottic stenosis in two unrelated pairs of siblings

et al. 2004

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“…When patients are scheduled for surgical procedures, routine preoperative medication may be given without fear of respiratory compromise. This statement is supported by case reports (Masuda, Harada, Honma, Ichimiya, & Namiki, 1990;Mehta & Schou, 1988;Monedero et al, 1997) and the opinion of the panel. Preoperative sedation given orally is commonly practiced in pediatric anesthesia.…”

Section: Preoperative Sedationmentioning

confidence: 63%

Best practices in peri‐operative management of patients with skeletal dysplasias

White

Bompadre

Goldberg

et al. 2017

American J of Med Genetics Pt A

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Patients with skeletal dysplasia frequently require surgery. This patient population has an increased risk for peri-operative complications related to the anatomy of their upper airway, abnormalities of tracheal-bronchial morphology and function; deformity of their chest wall; abnormal mobility of their upper cervical spine; and associated issues with general health and body habitus. Utilizing evidence analysis and expert opinion, this study aims to describe best practices regarding the peri-operative management of patients with skeletal dysplasia. A panel of 13 multidisciplinary international experts participated in a Delphi process that included a thorough literature review; a list of 22 possible care recommendations; two rounds of anonymous voting; and a face to face meeting. Those recommendations with more than 80% agreement were considered as consensual. Consensus was reached to support 19 recommendations for best pre-operative management of patients with skeletal dysplasia. These recommendations include pre-operative pulmonary, polysomnography; cardiac, and neurological evaluations; imaging of the cervical spine; and anesthetic management of patients with a difficult airway for intubation and extubation. The goals of this consensus based best practice guideline are to provide a minimum of standardized care, reduce perioperative complications, and improve clinical outcomes for patients with skeletal dysplasia.

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“…Congenital heart disease, subglottic tracheal narrowing and genitourinary anomalies, muscular hypotonia. [34]…”

Section: Discussionmentioning

confidence: 99%

Airway management of a child with frontometaphyseal dysplasia (Gorlin Cohen syndrome)

Ganigara

Nishtala

Chandrika

et al. 2014

J Anaesthesiol Clin Pharmacol

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Frontometaphyseal dysplasia (FMD), also called Gorlin-Cohen syndrome, is a rare hereditary X-linked dominant craniotubular bone disorder. The presentation describes the airway management of a 2-year-old child suffering from FMD with significant retrognathia, posted for major long bone corrective osteotomy. Induction with a combination of dexmedetomidine and ketamine preceded a successful endotracheal intubation under spontaneous ventilation.

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The anaesthetic management of an infant with frontometaphyseal dysplasia (Gorlin–Cohen syndrome)

Cited by 11 publications

References 9 publications

Congenital subglottic stenosis in two unrelated pairs of siblings

Congenital subglottic stenosis in two unrelated pairs of siblings

Best practices in peri‐operative management of patients with skeletal dysplasias

Airway management of a child with frontometaphyseal dysplasia (Gorlin Cohen syndrome)

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