Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

[Anesthesia and intensive therapy in autonomic dysfunction]

K Stubbig1, H Schmidt, R Schreckenberger

  • 1Abteilung für Anästhesie und operative Intensivmedizin, Klinikum Aschaffenburg.

Der Anaesthesist
|May 1, 1993
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

[Hans Killian - surgical protagonist of German anesthesia].

Der Anaesthesist·2017
Same author

Der Anaesthesist·2016
Same author

[Is the preoperative removal of dentures reasonable in every case?].

Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie : AINS·2004
Same author

[Spinal anaesthesia in 50 patients with previous lumbar spinal surgery].

Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie : AINS·2004
Same author

[Repeated improper intravenous injection of fentanyl from a transdermal system].

Deutsche medizinische Wochenschrift (1946)·2004
Same author

[A child with a full stomach has to be anaesthesized...Anasthesiol Intensivmed Notfallmed Schmerzther; 37; 514-519. Reply].

Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie : AINS·2004
Same journal

[Promoting young academics in anesthesiology: factors for an attractive internship].

Der Anaesthesist·2022
Same journal

[Respiratory support in COVID-19: all in due time!]

Der Anaesthesist·2022
Same journal

[Noninvasive respiratory support and invasive ventilation in COVID‑19 : Where do we stand today?]

Der Anaesthesist·2022
Same journal

[Management of acutely decompensated liver cirrhosis in emergency and critical care medicine].

Der Anaesthesist·2022
Same journal

[Anesthesia in patients with acute porphyria].

Der Anaesthesist·2022
Same journal

[Quality and safe anesthesia for all children : That is their right!]

Der Anaesthesist·2022
See all related articles

Familial dysautonomia, a rare genetic disorder, presents significant perioperative risks. This case study highlights successful anesthetic management in a child with this condition, emphasizing optimized therapeutic strategies for improved outcomes.

Area of Science:

  • Genetics
  • Neurology
  • Anesthesiology

Background:

  • Familial dysautonomia (Riley-Day syndrome) is an autosomal recessive genetic disorder affecting the central and peripheral nervous systems.
  • Characterized by autonomic and sensory neuron deficits, it manifests in early childhood with symptoms like poor pain/temperature perception, muscle coordination issues, and autonomic crises.

Observation:

  • A 1.5-year-old child with familial dysautonomia underwent general anesthesia for gastrostomy and fundoplication due to chronic aspiration.
  • The procedure involved balanced anesthesia with comprehensive invasive monitoring.
  • Significant pulmonary complications, including hypoxemia during intubation, excessive bronchial secretions, and pneumonia, were observed.

Findings:

  • Hemodynamic parameters remained stable during anesthesia.

Related Experiment Videos

  • Postoperatively, the patient experienced severe pneumonia, bronchoconstriction, atelectasis, and required prolonged mechanical ventilation.
  • Cardiovascular instability and autonomic crises occurred, managed with diazepam.

    • Implications:

    • Patients with familial dysautonomia face high perioperative risks due to autonomic dysfunction.
    • This case demonstrates that with optimized therapeutic efforts and vigilant monitoring, safe perioperative management is achievable.
    • Highlights the importance of tailored anesthetic and critical care strategies for rare genetic disorders.