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Related Concept Videos

General Anesthesia: Overview01:24

General Anesthesia: Overview

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Anesthesia is a medical procedure that uses drugs for CNS suppression to enable painless surgeries and procedures. The selection of anesthetics is influenced by their pharmacokinetic properties, side effects, and patient characteristics. Various types of anesthesia include general, local, regional, spinal, and inhalational.
General anesthesia induces unconsciousness in the whole body, while the others target specific areas or sensations. It is administered to minimize adverse effects, maintain...
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Local Anesthetics: Clinical Application as Spinal Anesthesia01:11

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Spinal anesthetics are given during lower abdomen and limb surgeries to block sensory and motor neurons. They are administered in the mid to low lumbar regions, primarily acting on the cauda equina's nerve roots. The blockade level depends on the local anesthetic (LA) concentration. Usually, low LA concentrations are sufficient to block sensory fibers, while only high LA concentrations block motor fibers. Other factors like injection volume and speed, the patient's posture, and the drug...
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Local Anesthetics: Clinical Application as Epidural Anesthesia01:29

Local Anesthetics: Clinical Application as Epidural Anesthesia

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Epidural anesthetics are administered in the fat-filled epidural space, the outermost part of the spinal canal. This technique is commonly employed for pain management and anesthesia during lower abdomen and pelvis surgeries or labor and delivery.
Since epidural anesthetics can be infused through an epidural catheter, all types of drugs, including short-acting ones, can be administered. Chloroprocaine and lidocaine are examples of short and long-duration anesthetics, respectively. Bupivacaine...
631
Depolarizing Blockers: Pharmocokinetics01:19

Depolarizing Blockers: Pharmocokinetics

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Depolarizing blockers are administered through intravenous injection. Succinylcholine is the most common choice of depolarizing blockers in emergency clinical practices. Although they have a rapid onset, they readily diffuse away from the motor end plate into the extracellular fluid. They are metabolized by enzymes such as liver butyrylcholinesterase and plasma pseudocholinesterases. This produces a short duration of action, typically 5-10 minutes long, unlike nondepolarizing blockers, which...
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Parenteral Anesthetics: Overview01:24

Parenteral Anesthetics: Overview

498
Intravenous anesthetics are drugs administered parenterally to induce anesthesia or sedation. Propofol is a widely used agent formulated as a 1% emulsion in soybean oil, glycerol, and egg phosphatide. It induces rapid anesthesia primarily due to its rapid distribution from the bloodstream to target tissues and is metabolized in the liver. However, it can cause significant pain on injection and hypertriglyceridemia. Fospropofol, a water-based prodrug of propofol, lacks these adverse effects.
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Local Anesthetics: Clinical Application as Intravenous Regional Anesthesia01:16

Local Anesthetics: Clinical Application as Intravenous Regional Anesthesia

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Intravenous regional anesthesia or the Bier block technique is used to anesthetize a specific limb or extremity. It uses exsanguinated or blood-drained vessels to transport local anesthetics or LAs to the peripheral nerve trunks. Lidocaine without vasoconstrictors like epinephrine is most commonly used for this technique. Other drugs used are prilocaine, ropivacaine, and chloroprocaine. Bupivacaine is not recommended for this technique due to its high cardiac toxicity.
One of the advantages of...
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[General anesthesia for Crisponi syndrome: case report].

Chloé Allary1, Marco Caruselli1, Alexandre Fabre2

  • 1Aix Marseille University, APHM, La Timone Teaching Hospital, Department of Pediatric Anesthesia and Intensive Care Unit, Marseille, France.

Brazilian Journal of Anesthesiology (Elsevier)
|June 5, 2020
PubMed
Summary

Crisponi syndrome, a severe genetic disorder, requires careful perioperative management. This case study details successful endoscopic gastrostomy in a 17-month-old patient, highlighting temperature control and anesthetic strategies.

Keywords:
Anestesia pediátricaBody temperature regulationCrisponi syndromeHipertermia malignaMalignant hyperthermiaMorte súbitaPediatric anesthesiaRegulação da temperatura corpóreaSudden deathSíndrome de Crisponi

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Area of Science:

  • Genetics
  • Pediatric Anesthesiology
  • Rare Diseases

Background:

  • Crisponi syndrome is a severe, inherited disorder causing muscle contractions, apnea, and fever.
  • The condition is linked to mutations in the CRLF1 gene.
  • Management requires specialized care, particularly during surgical procedures.

Observation:

  • A 17-month-old female with Crisponi syndrome underwent endoscopic gastrostomy.
  • Perioperative care focused on strict temperature monitoring, maintaining the OR at 19°C.
  • The patient received both inhaled and intravenous anesthetics.

Findings:

  • The surgical and perioperative periods were uneventful.
  • Effective temperature management and anesthetic protocols were crucial.
  • Crisponi syndrome fever is distinct from malignant hyperthermia, originating from CRLF1 mutations.

Implications:

  • This case demonstrates the feasibility of safe endoscopic gastrostomy in Crisponi syndrome patients.
  • Highlights the importance of tailored perioperative strategies for rare genetic disorders.
  • Contributes to understanding the distinct pathophysiology of fever in Crisponi syndrome.