Caesarean section in a complicated case of central core disease

Foster, Ryann; Boothroyd, K. P.

doi:10.1111/j.1365-2044.2007.05411.x

Cited by 23 publications

(18 citation statements)

References 23 publications

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“…The baby was initially apnoeic and required two doses naloxone at 3 and 5 min. His apgar scores were 3, 7, and 9 at 1, 5, 10 min respectively (15).…”

Section: Discussionmentioning

confidence: 93%

Epidural Anesthesia for Caesarean Section in a Patient with Risk of Malignant Hyperthermia

Vukotic

2018

Serbian Journal of Experimental and Clinical Research

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Malignant hyperthermia is a hypermetabolic disorder of skeletal muscle that occurs in genetically susceptible individuals after exposure to anesthetic. Basic disorder is an increase of calcium ions inside the skeletal muscle, increasing metabolism and reducing cell energy supplies leading to development of acidosis, cell membrane destruction and cell death. Due to the increased metabolism occurs hypercarbia and strong stimulation of the sympathetic nervous system (tachycardia, hypertension, ventricular arrhythmia, tachypnea dropped for the neuromuscular blockade). Sweating, cyanosis, muscle rigidity and hyperthermia are also present.is work presents the case of a female patient aged 32 who was heterozygous for the mutation RYR1 gene and therefore has an increased risk of malignant hyperthermia. Per anamnesis we got data that patient's brother suff ers from central core disease (myopathy). Patient has no muscle disease, 41 st week of pregnancy and was admitted to the hospital for childbirth. Vaginal delivery in epidural analgesia was planned. Epidural catheter is placed in the space L3 -L4, through which she received 0.25% levobupivacaine 10 ml. Due to adverse obstetric fi ndings cesarean section underwent after two hours. Given the increased risk of malignant hyperthermia, the safest type of anesthesia for cesarean is epidural anesthesia. Over the epidural catheter has received 0.5% levobupivacaine 18 ml. Anesthesia machine was verifi ed, hoses were replaced with new ones, CO 2 absorber system was replaced, and whole system is fl ushed with pure oxygen, before surgery started. During the operation the patient had stable vital parameters that are monitored. She got a male child Apgar score of 9/10 and saw her child at birth. After the operation was transferred to the intensive care unit where we monitored the vital parameters, laboratory analysis, the amount and color of urine. Since all parameters were satisfactory, following day she was transferred to the ward, and she was discharged with a child on the fourth day after the surgery.

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“…The baby was initially apnoeic and required two doses naloxone at 3 and 5 min. His apgar scores were 3, 7, and 9 at 1, 5, 10 min respectively (15).…”

Section: Discussionmentioning

confidence: 93%

Epidural Anesthesia for Caesarean Section in a Patient with Risk of Malignant Hyperthermia

Vukotic

2018

Serbian Journal of Experimental and Clinical Research

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“…The cause of MH may be associated with mutation of RYR1, a calcium release channel in the sarcoplasmic reticulum [23][24][25]. Central core disease may also be related to RYR1 mutation and is likely to be complicated with MH [26][27][28]. An RYR1 mutation has also been suggested to be involved in CNMDU1 [3].…”

Section: Discussionmentioning

confidence: 99%

Kyphoscoliosis associated with congenital neuromuscular disease with uniform type 1 fibers

et al. 2011

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Objective To report the first case of surgical treatment for severe kyphoscoliosis associated with respiratory disorder in a patient with congenital neuromuscular disease with uniform type 1 fibers (CNMDU1), including management of the possible onset of malignant hyperthermia (MH) in general anesthesia. Summary of background data CNMDU1 is rare among congenital neuromuscular diseases, and surgery for spinal deformity in CNMDU1 has not been described. Onset of MH in general anesthesia is a concern in this disease. Methods A 13-year-old female with motor retardation, suspected myopathy, and severe spinal deformity was followed at another pediatric hospital before referral to Meijo Hospital. Symptoms at the initial consultation were mild general muscular weakness and muscular atrophy. The rib hump was 60°and trunk balance was poor. The tendon reflex showed hyporeflexia, and blood tests were normal. Vital capacity was 0.69 L and forced expiratory volume percentage in 1 s was 75.5%, showing a restrictive and obstructive ventilatory defect. A plain radiograph showed severe kyphoscoliosis with thoracic scoliosis of 130°(T5-L1) and thoracic kyphosis of 110°(T2-T12) with almost no flexibility in bending or traction film.

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“…Although the cause of this serious disease is unknown, involvement of ryanodine receptor gene mutation has been reported [16,17], and in light of reports on anesthesia in central core disease, which is a similar nonprogressive congenital muscular disease that is often complicated by ryanodine receptor gene mutation [18,19], we focused on preventing MH during anesthetic management.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic case in a child with congenital neuromuscular disease with uniform type 1 fibers (CNMDU1)

2010

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Congenital neuromuscular disease with uniform type 1 fibers (CNMDU1) is an extremely rare, non-progressive, congenital neuromuscular disorder. Although the etiology is unknown, ryanodine receptor gene mutation is reportedly involved. No descriptions of anesthetic practice in patients with this disease have been reported around the world. We report a case of safe perioperative management with general anesthesia, using total intravenous anesthesia, propofol, fentanyl and a non-depolarizing muscle relaxant but avoiding the use of any inhaled anesthetics or depolarizing muscle relaxants to prevent malignant hyperthermia and postoperative respiratory failure, during anesthetic management for cranioplasty for premature synostosis of the cranial sutures in a pediatric patient of CNMDU1.

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Caesarean section in a complicated case of central core disease

Cited by 23 publications

References 23 publications

Epidural Anesthesia for Caesarean Section in a Patient with Risk of Malignant Hyperthermia

Epidural Anesthesia for Caesarean Section in a Patient with Risk of Malignant Hyperthermia

Kyphoscoliosis associated with congenital neuromuscular disease with uniform type 1 fibers

Anesthetic case in a child with congenital neuromuscular disease with uniform type 1 fibers (CNMDU1)

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