Anaesthetic management of two patients with pompe disease for caesarean section

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.

Patients with hypertrophic cardiomyopathy (HCM) and left ventricular outflow tract (LVOT) obstruction who remain symptomatic despite optimal medical therapy may undergo a septal myectomy (Morrow procedure). This procedure involves excising a portion of the hypertrophied septum below the aortic valve using a heart-lung machine to improve blood flow through the LVOT. Effective preoperative and postoperative nursing management ensures successful patient outcomes, minimizes complications, and...

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...

Skeletal muscle relaxants are used to relax muscle tone and alleviate painful muscle contractions. However, the choice of skeletal muscle relaxants depends on the duration of the surgical procedure in order to minimize potential side effects. Skeletal muscle relaxants like neuromuscular blocking agents [NMBAs] are commonly employed as adjuvants alongside general anesthetics in clinical settings. NMBAs are also used to maintain controlled ventilation during surgery of the larynx or pharynx...

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...

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...