• BMC anesthesiology · Apr 2021

    Review Case Reports

    Case report and review of literature of a rare congenital disorder: Adams-Oliver syndrome.

    • Edwin Suarez, Mia J Bertoli, Jean Daniel Eloy, and ShahShridevi PandyaSPDepartment of Anesthesiology, Rutgers New Jersey Medical School, Newark, New Jersey, USA. pandyas1@njms.rutgers.edu..
    • Department of Internal Medicine, White River Medical Center, Batesville, Arkansas, USA.
    • BMC Anesthesiol. 2021 Apr 15; 21 (1): 117.

    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.

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