Anesthesia in a Patient With Congenital Insensitivity to Pain and Anhidrosis

Oliveira, Carlos Rogério Degrandi; Paris, Valter César; Pereira, Renato Augusto; Lara, Felipe Souza Thyrso de

doi:10.1016/s0034-7094(09)70085-6

Cited by 11 publications

(10 citation statements)

References 20 publications

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“…Due to the insensitivity to pain in these patients, several issues are made difficult for anesthetists in surgical procedures, such as whether or not to administer anesthesia, and if so, the choice of anesthesia type and drugs, providing sufficient depth of anesthesia, the necessity of monitoring body temperature because of impaired ther- can occur in the hemodynamic parameters 10,11 . 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 .…”

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%

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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%

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

et al. 2017

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“…About the anesthetic considerations [ 39 , 40 ], although pain stimuli are absent, anxiety associated with surgical procedures may generate stress and consequent hemodynamic instability. It is necessary to minimize preoperative apprehension and anxiety with the use of sedatives.…”

Section: Discussionmentioning

confidence: 99%

Update Review and Clinical Presentation in Congenital Insensitivity to Pain and Anhidrosis

Perez-Lopez

Cabrera-González

Iglesia

et al. 2015

Case Reports in Pediatrics

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Introduction. Congenital insensitivity to pain and anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy type IV is an extremely rare syndrome. Three clinical findings define the syndrome: insensitivity to pain, impossibility to sweat, and mental retardation. This pathology is caused by a genetic mutation in the NTRK1 gene, which encodes a tyrosine receptor (TrkA) for nerve growth factor (NGF). Methods. The consultation of a child female in our center with CIPA and a tibia fracture in pseudoarthrosis encouraged us to carefully review literature and examine the therapeutic possibilities. A thorough review of literature published in Pubmed was done about CIPA and other connected medical issues mentioned in the paper. Conclusions. The therapeutic approach of CIPA remains unclear. The preventive approach remains the only possible treatment of CIPA. We propose two new important concepts in the therapeutic approach for these patients: (1) early surgical treatment for long bone fractures to prevent pseudoarthrosis and to allow early weight bearing, decreasing the risk of further osteopenia, and (2) bisphosphonates to avoid the progression of osteopenia and to reduce the number of consecutive fractures.

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“…[8] Despite patients are insensitive to pain, general anesthesia or sedation may be necessary because of disturbing conditions such as tactile hyperesthesia during the surgical procedure. [9] Experience on anesthesia management of these patients is limited due to few number of cases. We aimed to present our experience about the anesthesia management of two siblings with CIPA syndrome.…”

Section: Introductionmentioning

confidence: 99%

Anaesthetic Management of Two Siblings with Congenital Pain Insensitivity Syndrome with Anhydrosis

Destegül¹

2019

Agri

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Konjenital ağrıya duyarsızlık sendromu, konjenital duysal ve otonomik nöropatiye bağlı gelişen ağrıya duyarsızlık, anhidrozis, epizodik ateş, gelişme geriliği, farklı düzeylerde mental retardasyon ve kendine zarar verme ile karakterize nadir görülen bir sendromdur. Nörotrofik tirozin kinazın kodlandığı nörtrofik tirozin kinaz-1 genindeki mutasyon sonucu meydana gelen otozomal resesif bir sendromdur. Çoğu hasta hastaneye ağrısız iyileşmeyen yaralar ve farkedilmeyen travmatik kırıklar ile başvurmaktadır. Bu yazımızda on yedi ve on dört yaşında ağrıyı hissetmeme, anhidrosis, mental retardasyon ve septik artriti olan iki kardeşi sunmaktayız. Konjenital ağrıya duyarsızlık sendromu olan hastalarda, tek başına midazolom ile sedasyon, tatminkar bir cerrahi konforu, herhangi bir hemodinamik bir instabilite yaratmadan sağlamaktadır. Anahtar sözcükler: Ağrıya duyarsızlık; CIPA; konjenital ağrıya duyarsızlık sendromu.

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Anesthesia in a Patient With Congenital Insensitivity to Pain and Anhidrosis

Cited by 11 publications

References 20 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

Update Review and Clinical Presentation in Congenital Insensitivity to Pain and Anhidrosis

Anaesthetic Management of Two Siblings with Congenital Pain Insensitivity Syndrome with Anhydrosis

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