Anesthesia for Patients with Congenital Insensitivity to Pain and Anhidrosis: A Questionnaire Study in Japan

Tomioka, Takashi; Awaya, Yutaka; Nihei, Kenji; Sekiyama, Hiroshi; Sawamura, Shigehito; Hanaoka, Kazuo

doi:10.1097/00000539-200202000-00008

Cited by 24 publications

(33 citation statements)

References 9 publications

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“…While Tamioka et al reported that all anesthetic agents could be safely used in these cases 12 , in another study which analyzed the anesthetic agents used in 358 procedures applied under general anesthesia to 35 CIPA syndrome patients, it was seen that thiopental was used in 4% of cases, ketamine in 27% and propofol in 71%, and in contrast, opioids were used in 8% and muscle relaxants in 27% 13 .…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, there is an absolute need for the administration of anesthesia during a surgical procedure and it has been reported that the type of anesthesia should be general anesthesia as the majority of these cases are mentally retarded pediatric cases 10,12 . In the case reported here, it was thought that ketamine and inhalation anesthesia would be sufficient.…”

Section: Discussionmentioning

confidence: 99%

“…Although the development of malignant hyperthermia and values >40°C have not been often observed, this subject is still noteworthy 12,13 . In the current case, body temperature was closely monitored and was seen to be within the normal value range.…”

Section: Discussionmentioning

confidence: 99%

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Anesthesia Procedure for Congenital Insensitivity to Pain in a Child with Anhidrosis Syndrome: A Rare Case

et al. 2017

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Congenital insensitivity to pain with anhidrosis (CIPA) syndrome is a neuropathy characterized by insensitivity to pain, impaired thermoregulation, anhidrosis, and mental retardation. A 9-year old boy with CIPA syndrome, underwent 2 operations for a calcaneal ulcer. During the first operation standard monitorization was performed. In the second operation, Bispectral Index (BIS) monitoring was added and temperature was monitored with an esophageal probe. In the first operation, in which anesthesia induction was applied with ketamine and midazolam, extremity movements with surgical stimuli were seen. Despite pain insensitivity, as extremity movements were seen with surgical stimuli, propofol was administered in the second operation. Throughout the operation, the BIS values varied from 19 58 and body temperature was measured as 36.1°C 36.9°C. In conclusion, despite the absence of pain sensitivity in CIPA syndrome cases, there is an absolute need for the administration of anesthesia in surgical procedures because of tactile hyperesthesia. (J Nippon Med Sch 2017; 84: 237 240)

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Anesthesia Procedure for Congenital Insensitivity to Pain in a Child with Anhidrosis Syndrome: A Rare Case

et al. 2017

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“…It has been reported that stress and anxiety may elevate body temperature and thus applying appropriate premedication is recommended. 4 Even though there may be a relationship between the development of hyperthermia and the use of anticholinergic drugs in patients with CIPA in the literature, there are case reports showing safe usage of these agents without inducing hyperthermia. 5 In our patient 0.2 mg of atropine was used safely during the perioperative period without inducing hyperthermia.…”

Section: Discussionmentioning

confidence: 99%

Congenital insensitivity to pain: How should anesthesia be managed?

et al. 2017

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Congenital insensitivity to pain syndrome is a rare, sensorial and autonomic neuropathy characterized by unexplained fever, insensitivity to pain and anhidrosis. Patients may require anesthesia even for minor surgical procedures due to mental retardation and trauma arising from self-mutilating behavior.A child diagnosed with congenital insensitivity to pain syndrome was scheduled for gastric endoscopy under sedation due to suspected ingestion of a household cleaning disinfectant. Deep sedation was achieved, and spontaneous respiration was maintained. We did not encounter any complications.There is limited data regarding the safe anesthesia management in these patients because of the rarity of the disease. Therefore, we think that more clinical experience, case reports and studies are needed to establish the appropriate anesthesia management.

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“…Generally, patients with CIPA do not require analgesics for the maintenance of anesthesia, and are prone to become hemodynamically unstable with high concentrations of either volatile or intravenous anesthetics. Small doses of sedatives could achieve appropriate anesthetic depth in those patients [6,7]. The anesthetic regimen without opioids could diminish the risk of respiratory depression.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic Management of Congenital Insensitivity to Pain with Anhidrosis in a Child: A Case Report

Huang¹,

Hu²,

Jiang³

et al. 2017

J Anesth Clin Res

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Anesthesia for Patients with Congenital Insensitivity to Pain and Anhidrosis: A Questionnaire Study in Japan

Cited by 24 publications

References 9 publications

Anesthesia Procedure for Congenital Insensitivity to Pain in a Child with Anhidrosis Syndrome: A Rare Case

Anesthesia Procedure for Congenital Insensitivity to Pain in a Child with Anhidrosis Syndrome: A Rare Case

Congenital insensitivity to pain: How should anesthesia be managed?

Anesthetic Management of Congenital Insensitivity to Pain with Anhidrosis in a Child: A Case Report

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