Mechanical ventilation in Coffin-Lowry syndrome: a case report

Moura, Edmilson Bastos de; Moura, Érica Leal Teixeira de; Amorim, Fábio Ferreira; Oliveira, Vânia Maria

doi:10.5935/0103-507x.20160081

Cited by 1 publication

(1 citation statement)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Although the airway management strategy varied among cases, spontaneous breathing was often maintained. Moreover, Cormack grades III–IV [ 5 , 7 , 8 ] and Mallampati scale 3–4, [ 4 , 14 ] indicating difficult intubation, were reported in many cases.…”

Section: Discussionmentioning

confidence: 99%

Perioperative management of a patient with Coffin–Lowry syndrome complicated by severe obesity

et al. 2017

View full text Add to dashboard Cite

Rationale:Coffin–Lowry syndrome (CLS) is a rare inherited disease with specific clinical features, such as mental retardation, facial dysmorphism, and cardiac abnormality. In particular, the characteristic facial features of CLS, including retrognathia and large tongue, are associated with difficult ventilation and/or intubation, which is a serious problem of anesthesia management. However, case reports on anesthesia management of CLS are very limited as there are only two published English reports till date. In this case report, we discuss anesthetic and postoperative considerations in patients with CLS, focusing on difficult airway management, and summarize past reports including some Japanese articles.Patient concerns:A 25-year-old man with CLS was planning to undergo laminectomy because of progressive quadriplegia caused by calcification of the yellow ligament. We suspected difficulty in airway management because of several factors in his facial features, short thyromental and sternomental distances in computed tomography, severe obesity, and sleep apnea syndrome.Diagnoses:Difficult airway was suspected. However, because of mental retardation, awake intubation was considered difficult.Interventions:We selected bronchofiberscope-guided nasotracheal intubation, maintaining spontaneous breathing under moderate sedation with a propofol target-controlled infusion.Outcomes:Airway management was safely performed during anesthesia induction.Lessons:In many patients with CLS, difficult intubation was reported, and sedation or slow induction maintaining spontaneous breathing was mainly selected for anesthesia induction. Spontaneous breathing should be maintained during anesthesia induction in case of CLS patients.

show abstract

Section: Discussionmentioning

confidence: 99%