Perioperative airway management of a patient with Beckwith-Wiedemann syndrome

Tsukamoto, Masanori; Hitosugi, Takashi; Yokoyama, Takeshi

doi:10.17245/jdapm.2016.16.4.313

Cited by 8 publications

(5 citation statements)

References 13 publications

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“…6,8,9 Nonetheless, much of the literature describing the perioperative management of children with BWS comes from case reports and small case series. 8,10,11 Determining the prevalence and risk factors for difficult airway is important to guide clinicians in preparing to anesthetize these patients.…”

mentioning

confidence: 99%

The Prevalence of Difficult Airway in Children With Beckwith-Wiedemann Syndrome: A Retrospective Cohort Study

Sequera‐Ramos¹,

Duffy²,

Fiadjoe³

et al. 2021

Anesthesia &Amp; Analgesia

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BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is the most common congenital overgrowth disorder with an incidence of approximately 1 in 10,000 live births. The condition is characterized by lateralized overgrowth, abdominal wall defects, macroglossia, and predisposition to malignancy. Historically, children with BWS have been presumed to have difficult airways; however, most of the evidence to support this has been anecdotal and derived from case reports. Our study aimed to determine the prevalence of difficult airway in patients with BWS. We hypothesized that most patients with BWS would not have difficult airways. METHODS: We retrospectively reviewed the electronic medical records of patients enrolled in our institution’s BWS registry. Patients with a molecular diagnosis of BWS who were anesthetized between January 2012 and July 2019 were included for analysis. The primary outcome was the presence of difficult airway, defined as difficult facemask ventilation, difficult intubation, or both. We defined difficult intubation as the need for 3 or more tracheal intubation attempts and the need for advanced airway techniques (nondirect laryngoscopy) to perform tracheal intubation or a Cormack and Lehane grade ≥3 during direct laryngoscopy. Secondary objectives were to define predictors of difficult intubation and difficult facemask ventilation, and the prevalence of adverse airway events. Generalized linear mixed-effect models were used to account for multiple anesthesia events per patient. RESULTS: Of 201 BWS patients enrolled in the registry, 60% (n = 122) had one or more documented anesthetics, for a total of 310 anesthetics. A preexisting airway was present in 22 anesthetics. The prevalence of difficult airway was 5.3% (95% confidence interval [CI], 3.0-9.3; 18 of 288) of the cases. The prevalence of difficult intubation was 5.2% (95% CI, 2.9-9.4; 12 of 226). The prevalence of difficult facemask ventilation was 2.9% (95% CI, 1.4-6.2; 12 of 277), and facemask ventilation was not attempted in 42 anesthetics. Age <1 year, macroglossia, lower weight, endocrine comorbidities, plastics/craniofacial surgery, tongue reduction surgery, and obstructive sleep apnea were associated with difficult airways in cases without a preexisting airway. About 83.8% (95% CI, 77.6-88.5) of the cases were intubated with a single attempt. Hypoxemia was the most common adverse event. CONCLUSIONS: The prevalence of difficult tracheal intubation and difficult facemask ventilation in children with BWS was 5.2% and 2.9%, respectively. We identified factors associated with difficult airway, which included age <1 year, macroglossia, endocrine abnormalities, plastics/craniofacial surgery, tongue reduction surgery, and obstructive sleep apnea. Clinicians should anticipate difficult airways in patients with these factors.

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mentioning

confidence: 99%

The Prevalence of Difficult Airway in Children With Beckwith-Wiedemann Syndrome: A Retrospective Cohort Study

Sequera‐Ramos¹,

Duffy²,

Fiadjoe³

et al. 2021

Anesthesia &Amp; Analgesia

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“…Nevertheless, the surgical procedure should be performed in an interdisciplinary centre with experienced specialists. Postoperative oedema and swelling may lead to severe airway obstruction, requiring prolonged postoperative intubation and mechanical ventilation 13 . In many cases, extubation was possible immediately after or within the first 24h after surgery.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, tongue-reduction procedures can be of major importance in early childhood. In cases without immediate vital risk, tongue reduction should be delayed until after the first year of life because of potential perioperative complications 13 . Additional symptoms and disabilities associated with macroglossia can be an indication for surgical tongue reduction.…”

mentioning

confidence: 99%

Tongue reduction in Beckwith–Wiedemann syndrome: outcome and treatment algorithm

Naujokat

Möller

Terheyden

et al. 2019

International Journal of Oral and Maxillofacial Surgery

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“…These children frequently require corrective surgical Figure 7. Macroglossia in the patient with Beckwith-Wiedemann syndrome after intubation [38].…”

Section: Beckwith-wiedemann Syndromementioning

confidence: 99%

An Approach to the Airway Management in Children with Craniofacial Anomalies

Boudjenah¹,

Adham²,

Chinnappa³

et al. 2021

Special Considerations in Human Airway Management

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Managing the airways during anesthesia in pediatric patients with craniofacial abnormalities is a challenging and stressful situation for even experienced anesthesiologists. The prerequisites for a good management are a thorough understanding of the normal anatomy of the upper airway, its normal changes with growth, and the key features of congenital craniofacial abnormalities and their impact on the airways resulting in management difficulties. This chapter aims to provide an overview of various craniofacial anomalies and their airway management specificities. These include cleft lip and palate with or without Pierre Robin syndrome, craniofacial dysostosis (including Crouzon, Pfeiffer, and Apert syndromes), mandibulofacial dysostosis/Treacher Collins syndrome, hemifacial microsomia, Down's syndrome, and other anomalies.

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Perioperative airway management of a patient with Beckwith-Wiedemann syndrome

Cited by 8 publications

References 13 publications

The Prevalence of Difficult Airway in Children With Beckwith-Wiedemann Syndrome: A Retrospective Cohort Study

The Prevalence of Difficult Airway in Children With Beckwith-Wiedemann Syndrome: A Retrospective Cohort Study

Tongue reduction in Beckwith–Wiedemann syndrome: outcome and treatment algorithm

An Approach to the Airway Management in Children with Craniofacial Anomalies

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