rocuronium and Abnormalities--Multiple

Arthrogryposis is characterized by multiple, nonprogressive joint contractures which may be caused by maternal disorders such as oligohydramnios as well as fetal akinesia resulting from primary disorders of muscle, connective tissue, or neurologic tissue. Its prevalence is about 1 : 3000. Distal arthrogryposis (DA) is a heterogenous group of genetic disorders with a characteristic flexion of the joints of the hands and feet divided into different types with additional features. Sheldon-Hall Syndrome (SHS), also known as distal arthrogryposis type 2A (DA2A), has some nonorthopedic features of specific importance to anesthetic care.

Topics: Abnormalities, Multiple; Androstanols; Anesthetics, Intravenous; Arthrogryposis; Child, Preschool; Clubfoot; Fentanyl; Humans; Intubation, Intratracheal; Male; Methohexital; Neuromuscular Nondepolarizing Agents; Rocuronium; Tooth Abnormalities

Topics: Abnormalities, Multiple; Androstanols; Anesthesia; Anesthetics, Inhalation; Anesthetics, Intravenous; Desflurane; Facies; Female; Humans; Infant, Newborn; Intubation, Gastrointestinal; Intubation, Intratracheal; Isoflurane; Neuromuscular Nondepolarizing Agents; Piperidines; Prenatal Diagnosis; Propofol; Remifentanil; Rocuronium; Stomach; Syndrome

Topics: Abnormalities, Multiple; Alfentanil; Androstanols; Anesthesia; Anesthesia, Epidural; Anesthetics, Combined; Anesthetics, Inhalation; Anesthetics, Intravenous; Anesthetics, Local; Bupivacaine; Child, Preschool; Desflurane; Female; Femur; Fractures, Bone; Hip Dislocation, Congenital; Humans; Intellectual Disability; Isoflurane; Monitoring, Intraoperative; Muscular Diseases; Neuromuscular Nondepolarizing Agents; Orthopedic Procedures; Propofol; Rocuronium; Sufentanil; Syndrome

A case is described of a difficult intubation in an infant presenting for cleft palate closure, due to an unsuspected sublingual mass. The report stresses the fact that multiple anomalies can coexist in an infant and contribute to difficult intubation through different mechanisms. Various airway management strategies are explored.

Topics: Abnormalities, Multiple; Androstanols; Anesthetics, Inhalation; Choristoma; Cleft Palate; Female; Fiber Optic Technology; Humans; Infant; Intubation, Intratracheal; Methyl Ethers; Mouth Floor; Mouth Neoplasms; Neuromuscular Nondepolarizing Agents; Rocuronium; Sevoflurane

Robinow (fetal face) syndrome is a rare inherited multisystem disorder featuring mesomelic or acromesomelic limb shortening, facial and spinal deformities, hypoplastic genitalia, kidney disease and congenital heart defects. We report the anaesthetic management of a patient with Robinow syndrome presenting for elective surgery and review specific issues of interest in the perioperative workup.

Topics: Abnormalities, Multiple; Androstanols; Anesthesia, General; Anesthetics, Inhalation; Anesthetics, Intravenous; Child, Preschool; Dwarfism; Face; Female; Fentanyl; Heart Defects, Congenital; Humans; Intubation, Intratracheal; Kidney Function Tests; Limb Deformities, Congenital; Liver Function Tests; Methyl Ethers; Neuromuscular Nondepolarizing Agents; Propofol; Rhinoplasty; Rocuronium; Sevoflurane; Spine; Syndrome