Anesthesia for children with complete trisomy 18 (Edwards syndrome): A cohort review of 84 anesthesia encounters in nine patients

Birmingham, Erin; Stucke, Astrid G.; Diaz, Christina D.

doi:10.1111/pan.14131

Cited by 7 publications

(7 citation statements)

References 16 publications

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“…Higher risk procedures include scoliosis repair surgery and cardiac surgery. Notably, most observed procedures were performed during infancy [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…Nevertheless, the large cohort review encompassing 11 patients with trisomy 18 did include two patients, who were 16 and 11 years of age, respectively; combined, these two patients received a total of 31 anesthetics with complications related to difficult intubation, laryngospasm, and bradycardia. Other anesthetic complications cited in the review included difficult bag-valve-mask ventilation, difficult IV access and cardiac events necessitating resuscitation, which included cardiopulmonary reanimation (CPR) in younger patients [ 3 ]. Further, a recent retrospective review of airway management of trisomy 18 patients found that there was a decreased risk of difficult intubation in patients older than 6 years of age compared with their younger counterparts [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

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Anesthetic Management of a Patient With Trisomy 18 Undergoing a Multilevel Spinal Fusion

Frease,

Rico Mora

2024

J Med Cases

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Trisomy 18 is the second most common autosomal trisomy aside from trisomy 21. Anesthesiologists were unlikely to manage such patients in the past, specifically those surviving later into childhood due to the 90% mortality rate within the first year of life and the lack of procedural options that were available. However, a paucity of literature regarding the anesthetic management of such patients exists. Trisomy 18 patients present a unique anesthetic challenge, given the presence of associated dysmorphic facial features and the involvement of multiple organ systems, leading to difficult airway and hemodynamic disturbances. In this case report, we present the anesthetic management of a 9-year-old patient with trisomy 18 undergoing a multilevel spinal fusion. Despite significant intraoperative hemorrhage, the patient was able to tolerate the procedure without complications, likely owing to the meticulous preoperative preparation and the patient’s survival later into childhood. This case contributes to a small subset of literature which suggests that patients with trisomy 18 who survive later into childhood have an improved ability to tolerate general anesthesia.

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“…Higher risk procedures include scoliosis repair surgery and cardiac surgery. Notably, most observed procedures were performed during infancy [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Anesthetic Management of a Patient With Trisomy 18 Undergoing a Multilevel Spinal Fusion

Frease,

Rico Mora

2024

J Med Cases

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“…Trisomy 18 is a common autosomal chromosomal abnormality characterized by malformations throughout the body and occurs in approximately 1/3,600 to 1/10,000 live births [ 1 ]. Approximately 50% of newborns with trisomy 18 expire within the first week of life, with only 10% reaching their first birthday [ 2 ]. Children with trisomy 18 may require gastrointestinal, airway, or cardiac surgery during infancy.…”

Section: Introductionmentioning

confidence: 99%

“…Children with trisomy 18 may require gastrointestinal, airway, or cardiac surgery during infancy. The abnormal airway anatomy, including micrognathia, high-arched palate, and short neck makes mask ventilation, and tracheal intubation challenging [2]. Supraglottic airways (SGAs) play an important role in difficult airway management in both adults and children [3].…”

Section: Introductionmentioning

confidence: 99%

Anesthetic Management of Inguinal Hernia Surgery Using a Second-Generation Supraglottic Airway in a Patient With Trisomy 18: A Case Report

Akimaru,

Nakanishi,

Hasegawa

et al. 2023

Cureus

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Children with trisomy 18 have abnormal airway anatomy, making their airway management challenging. Only a few papers have comprehensively described and discussed the use of supraglottic airway devices in patients with trisomy 18. We present a case of a 20-month-old boy with trisomy 18 who was scheduled for open repair of the right inguinal hernia. He had micrognathia, a short neck, and an atrial septal defect but was in a clinically stable condition. A supraglottic airway device was inserted under general anesthesia. The patient’s respiration was maintained by pressure support ventilation with spontaneous breathing. A right ilioinguinal-iliohypogastric nerve block was performed for perioperative analgesia. The surgery ended without complications. After removing the supraglottic airway device and ensuring proper respiratory parameters, the patient was transferred to the post-anesthesia care unit. In our case, supraglottic airway devices could be effectively used as a primary airway for inguinal hernia repair. The concomitant ilioinguinal-iliohypogastric nerve block was helpful for anesthetic management with spontaneous breathing maintained using pressure support ventilation. A supraglottic airway device may be a potential alternative as a primary airway for superficial surgery in pediatric patients with trisomy 18. For pediatric patients with difficult airways, a second-generation supraglottic airway device with the insertion of a gastric tube to prevent gastric insufflation combining pressure support ventilation and positive end-expiratory pressure may be a beneficial choice for the maintenance of spontaneous breathing.

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“…2e4 Airway management is thought to be challenging because of micrognathia/retrognathia and restricted mouth opening, which can lead to difficulties with mask ventilation and tracheal intubation, as reported in a single case series. 5 Larger studies comprehensively examining airway management in children with trisomy 18, including incidence and risk factors of difficult airway, specific techniques used, and related complications, have not been published.…”

mentioning

confidence: 99%

Difficult airway management in children with trisomy 18: a retrospective single-centre study of incidence, outcomes, and complications

Bai

Klumpner

Zhao

et al. 2023

British Journal of Anaesthesia

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Anesthesia for children with complete trisomy 18 (Edwards syndrome): A cohort review of 84 anesthesia encounters in nine patients

Abstract: Background: Trisomy 18 or Edwards syndrome is the second most common ane

Cited by 7 publications

References 16 publications

Anesthetic Management of a Patient With Trisomy 18 Undergoing a Multilevel Spinal Fusion

Anesthetic Management of a Patient With Trisomy 18 Undergoing a Multilevel Spinal Fusion

Anesthetic Management of Inguinal Hernia Surgery Using a Second-Generation Supraglottic Airway in a Patient With Trisomy 18: A Case Report

Difficult airway management in children with trisomy 18: a retrospective single-centre study of incidence, outcomes, and complications

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