Abstract
BackgroundAdams-Oliver syndrome is characterized by the combination of congenital scalp defects and terminal transverse limb defects. In some instances, cardiovascular malformations and orofacial malformations have been observed. Little is written with regards to the anesthetic management and airway concerns of patients with Adams-Oliver syndrome.Case presentationA five-year-old female with Adams-Oliver syndrome presented for repeat lower extremity surgery. Airway exam was significant for dysmorphic features, such as hypertelorism, deviated jaw, and retrognathia. Video laryngoscope was utilized for intubation due to the patients retrognathic jaw, cranial deformities, and facial dysmorphism. A vein finder with ultrasound guidance was needed to place the peripheral intravenous line due to her history of difficult intravenous access. The patient was successfully intubated with slight cricoid pressure applied to direct the endotracheal tube smoothly. Surgery and recovery were both unremarkable.ConclusionsDue to varying presentations of Adams-Oliver syndrome, anesthetic and airway management considerations should be carefully assessed prior to surgery. Anesthesiologists must take into consideration possible orofacial abnormalities that may make intubation difficult. Amniotic band syndrome and other limb defects could potentially impact intravenous access as well.
Highlights
Adams-Oliver syndrome is characterized by the combination of congenital scalp defects and terminal transverse limb defects
Anesthesiologists must take into consideration possible orofacial abnormalities that may make intubation difficult
We present a case illustrating the anesthetic management of a 5-year-old child with Adams-Oliver syndrome (AOS) with a perceived potentially challenging airway
Summary
AOS is a complex disorder presenting with phenotypic variability. Possible defects include congenital scalp defects, limb defects, cardiovascular malformations, orofacial malformations, retrognathia, and many others. Our patient had a retrognathic jaw and amniotic band syndrome, with a history of difficult intravenous access. Since proper precautions were taken, the anesthetic management was without complications and the patient successfully recovered. Since there is little written about AOS and anesthesia, anesthesiologists must be aware of the possible challenges and prepare for difficult airway maintenance and intravenous access. Due to the heterogeneity in disease symptoms and multisystem implications, it is imperative for anesthesiologists to collaborate with multiple different specialties prior to anesthetic management to ensure a safe perioperative experience
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