Case report and review of literature of a rare congenital disorder: Adams-Oliver syndrome
Abstract Background Adams-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...
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doaj-3ac62259aaf74d3b822a32b9e1aa0e832021-04-18T11:31:18ZengBMCBMC Anesthesiology1471-22532021-04-012111510.1186/s12871-021-01339-0Case report and review of literature of a rare congenital disorder: Adams-Oliver syndromeEdwin Suarez0Mia J. Bertoli1Jean Daniel Eloy2Shridevi Pandya Shah3Department of Internal Medicine, White River Medical CenterRutgers New Jersey Medical SchoolDepartment of Anesthesiology, Rutgers New Jersey Medical SchoolDepartment of Anesthesiology, Rutgers New Jersey Medical SchoolAbstract Background Adams-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 presentation A 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. Conclusions Due 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.https://doi.org/10.1186/s12871-021-01339-0Difficult airwayPediatric airway managementSeizure disordersAdams‐oliver syndrome |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Edwin Suarez Mia J. Bertoli Jean Daniel Eloy Shridevi Pandya Shah |
spellingShingle |
Edwin Suarez Mia J. Bertoli Jean Daniel Eloy Shridevi Pandya Shah Case report and review of literature of a rare congenital disorder: Adams-Oliver syndrome BMC Anesthesiology Difficult airway Pediatric airway management Seizure disorders Adams‐oliver syndrome |
author_facet |
Edwin Suarez Mia J. Bertoli Jean Daniel Eloy Shridevi Pandya Shah |
author_sort |
Edwin Suarez |
title |
Case report and review of literature of a rare congenital disorder: Adams-Oliver syndrome |
title_short |
Case report and review of literature of a rare congenital disorder: Adams-Oliver syndrome |
title_full |
Case report and review of literature of a rare congenital disorder: Adams-Oliver syndrome |
title_fullStr |
Case report and review of literature of a rare congenital disorder: Adams-Oliver syndrome |
title_full_unstemmed |
Case report and review of literature of a rare congenital disorder: Adams-Oliver syndrome |
title_sort |
case report and review of literature of a rare congenital disorder: adams-oliver syndrome |
publisher |
BMC |
series |
BMC Anesthesiology |
issn |
1471-2253 |
publishDate |
2021-04-01 |
description |
Abstract Background Adams-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 presentation A 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. Conclusions Due 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. |
topic |
Difficult airway Pediatric airway management Seizure disorders Adams‐oliver syndrome |
url |
https://doi.org/10.1186/s12871-021-01339-0 |
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