Anesthetic management of a ventilator-dependent parturient with the King-Denborough syndrome

Abstract

P Pu ur rp po os se e: : The King-Denborough syndrome (KDS) is a rare disorder that is associated with myopathy, susceptibility to malignant hyperthermia (MH) as well as congenital skeletal and facial anomalies. We report the anesthetic management of a parturient with KDS.C Cl li in ni ic ca al l f fe ea at tu ur re es s: : We describe the management of a 24-yr-old primiparous woman with a diagnosis of KDS and a history of previous MH reaction (age two). Her KDS resulted in chronic respiratory failure. She had a permanent tracheostomy and required overnight ventilatory support for the previous two years. She had three admissions during her pregnancy, one for pneumonia and two for preterm labour. Labour was induced at 37 weeks. Her labour was managed in the operating room where a "clean" anesthesia machine was ready. Cooling aids and a MH emergency kit were immediately available. Intravenous access, an arterial line and a lumbar epidural catheter were inserted before induction of labour.Ropivacaine 0.08% + fentanyl 2 µg·mL -1 were used for patientcontrolled epidural analgesia. After 6.5 hr of labour the patient required ventilation. An outlet forceps was performed for delivery. Postpartum, she was ventilated overnight in the intensive care unit.C Co on nc cl lu us si io on n: : The use of epidural analgesia, close monitoring and collaboration between the various disciplines were important in achieving a safe and uneventful labour in this high-risk parturient. King-Denborough (SKD) Objectif : Le syndrome de

Native American myopathy: Congenital myopathy with cleft palate, skeletal anomalies, and susceptibility to malignant hyperthermia

“…Two of the children developed malignant hyperthermia during cleft palate repair. Clinical features of this disorder described in the literature include short stature, cleft palate, high-arched palate, myopathic facies (malar hypoplasia, ptosis), scoliosis, congenital joint contractures, and talipes deformities [Bailey and Bloch, 1987;Stewart et al, 1988;Meluch et al, 1989;Abel and Grotegut, 2003;Habib et al, 2003].…”

“…Patient 13 is now 30 years old, has chronic respiratory failure with a permanent tracheostomy, and requires overnight continuous mechanical ventilatory support. Even though Individual 13 has respiratory compromise, she delivered a normal healthy baby with low forceps delivery in 2001 [Habib et al, 2003]. Individual 13 has short stature, cachexia with a BMI of 10.9, severe scoliosis and myopathic facies with short palpebral fissures and malar hypoplasia.…”

Glycolic acid modulates the mechanical property and degradation of poly(glycerol, sebacate, glycolic acid)

“…Patient 13 is now 30 years old, has chronic respiratory failure with a permanent tracheostomy, and requires overnight continuous mechanical ventilatory support. Even though Individual 13 has respiratory compromise, she delivered a normal healthy baby with low forceps delivery in 2001 [Habib et al, 2003]. Individual 13 has short stature, cachexia with a BMI of 10.9, severe scoliosis and myopathic facies with short palpebral fissures and malar hypoplasia.…”

“…Two of the children developed malignant hyperthermia during cleft palate repair. Clinical features of this disorder described in the literature include short stature, cleft palate, high‐arched palate, myopathic facies (malar hypoplasia, ptosis), scoliosis, congenital joint contractures, and talipes deformities [Bailey and Bloch, 1987; Stewart et al, 1988; Meluch et al, 1989; Abel and Grotegut, 2003; Habib et al, 2003].…”

Neuromuscular disorders in pregnancy

“…Women susceptible to malignant hyperthermia need special monitoring and care during delivery. 50,96 They must avoid halogenated inhalation anesthetics and succinylcholine. Malignant hyperthermia complicating caesarian section can be successfully treated with dantrolene 71 and some physicians advocate giving dantrolene prophylactically prior to delivery.…”