Spinal Anesthesia in a Patient with Congenital Insensitivity to Pain with Anhidrosis

Oliveira, Carlos Rogério Degrandi; Santos, Fúlvio A dos; Nogueira, Celso Schmalfuss; Mainardes, Emerson J

doi:10.1213/01.ane.0000261247.53075.dd

Cited by 6 publications

(5 citation statements)

References 5 publications

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“…However, remarkable tachycardia and hypertension occurred during airway manipulation and when the surgical stimuli increased, suggesting a stress response. Patients with CIPA lack pain sensation, but they may have tactile hyperesthesia [ 7 ]. Surgical noxious stimuli may therefore produce a stress response and unpleasant sensations, leading to hemodynamic fluctuation.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic management during adenotonsillectomy for twins with congenital insensitivity to pain with anhidrosis: two case reports

2017

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BackgroundCongenital insensitivity to pain with anhidrosis is a rare autosomal recessive disorder characterized by hyperpyrexia, anhidrosis, pain insensitivity, self-inflicted injuries, and intellectual disability. The anesthetic management of these patients is challenging owing to the high risk of perioperative complications resulting from their autonomic dysfunction, such as hyperthermia, hypotension, and bradycardia, which result from autonomic nervous system dysfunction.Case presentationTwo 3-year-old Han Chinese identical male twins (weighing 13.5 kg and measuring 93 cm tall) were previously diagnosed as having congenital insensitivity to pain with anhidrosis based on clinical features and genetic screening. According to the presence of loud snoring and heavy breathing during sleep and neck radiograph findings, they were diagnosed as having tonsil and adenoid hypertrophy and needed adenotonsillectomy. Because of innate analgesia, some reports suggested that patients with congenital insensitivity to pain with anhidrosis do not require perioperative pain control. Accordingly, our patients did not receive opiates. We describe the general anesthetic management of these patients using sevoflurane and propofol, but without opiates, for adenotonsillectomy. Remarkable tachycardia and hypertension occurred during airway manipulation and when the surgical stimuli increased, and their temperatures increased from 36 °C and 36.8 °C to 37.8 °C and 38.5 °C, respectively. Patients with congenital insensitivity to pain with anhidrosis lack pain sensation, but they may have tactile hyperesthesia. Surgical noxious stimuli may therefore produce a stress response and unpleasant sensations, leading to hemodynamic fluctuation and temperature increase.ConclusionsOn the basis of these findings, we suggest that careful intraoperative opiate titration may be justified to blunt the surgical stress response and promote hemodynamic and temperature stability in similar patients; we also recommend the preparation of warming and cooling devices and continuous temperature monitoring in these patients. Since anesthetic management of these patients is not simple, careful attention is required.

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Section: Discussionmentioning

confidence: 99%

Anesthetic management during adenotonsillectomy for twins with congenital insensitivity to pain with anhidrosis: two case reports

2017

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“…7 Consequently, strict perioperative temperature control has been advocated to maintain a core body temperature <37°C (98.6°F). 7,8,11 Although hyperthermia was historically considered a primary concern in the anesthetic management of CIPA patients, study seems to suggest that this is an extremely rare complication. Only 1 child without prior fever had an elevation in body temperature from 37.4°C (99.3°F) to 38.2°C (100.7°F) without being actively warmed.…”

Section: Discussionmentioning

confidence: 99%

“…Autonomic nervous system abnormalities are common, making CIPA patients subject to numerous anesthetic complications, including an increased risk of regurgitation and aspiration, hyperthermia, hypotension, and bradycardia. 2,7,8 However, CIPA is rare, and there is insufficient data in the literature regarding the rate of complications. In the largest analysis of anesthetics in CIPA patients to date, the experience of 40 cases from our institution was described.…”

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confidence: 99%

Anesthetic Management of Patients with Congenital Insensitivity to Pain with Anhidrosis

Zlotnik

Natanel

Kutz

et al. 2015

Anesthesia &Amp; Analgesia

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BACKGROUND Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disorder characterized by recurrent episodic fevers, anhidrosis, absent reaction to noxious stimuli, self-mutilating behavior, and mental retardation. The anesthetic management of patients with CIPA is challenging. Autonomic nervous system abnormalities are common, and patients are at increased risk for perioperative complications. METHODS In this study, we describe our experience with 35 patients with CIPA who underwent 358 procedures requiring general anesthesia between 1990 and 2013. RESULTS During surgery, 3 patients developed hyperthermia intraoperatively (>37.5°C) without prior fever. There were no cases of intraoperative hyperpyrexia (>40°C). Aspiration was suspected in 2 patients, and in another patient aspiration was prevented by the use of endotracheal tube, early detection of regurgitation, and aggressive suctioning. One patient had cardiac arrest requiring cardiopulmonary resuscitation. Intraoperative bradycardia was observed in 10 cases, and postoperative bradycardia was observed in 11 cases. CONCLUSIONS Regurgitation, hyperthermia, and aspiration were uncommon, but the incidence of bradycardia was higher than has been reported in previous studies. CIPA remains a challenge for anesthesiologists. Because of the rare nature of this disorder, the risk of various complications is difficult to predict.

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“…2 It should not be confused with congenital insensitivity to pain with anhydrosis (CIPA), which is associated with anhydrosis, insensitivity to pain, episodes of hyperpyrexia and loss of nociceptive afferent neurons. 1,2,5 There is no literature regarding CIP and pregnancy.…”

Section: Discussionmentioning

confidence: 99%

Labour outcomes in siblings with channelopathy associated insensitivity to pain due to bi-alleleic SCN9A mutations

2012

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The gene SCN9A encodes for the voltage-gated sodium channel Nav1.7, which is highly expressed in pain sensing neurons. Bi-allelic ‘loss of function’ mutations result in a channelopathy associated with insensitivity to pain and anosmia. This is the first report of the labour and postpartum outcomes of two sisters who belong to a non-consanguineous Caucasian family with homozygous SCN9A mutations. Neither sister experienced pain during labour; this had major implications for the staff titrating the syntocinon for labour augmentation and contributed towards their ultimate delivery by caesarean section. During the postpartum period, one of the sisters developed lower limb sensory loss and investigations revealed a spinal haematoma and unrecognized bilateral pelvic fractures. The other sister had an uneventful recovery and both babies are well. These case histories underline the importance of pain in labour management and its function in alerting patients and staff to problems during the puerperium.

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Spinal Anesthesia in a Patient with Congenital Insensitivity to Pain with Anhidrosis

Cited by 6 publications

References 5 publications

Anesthetic management during adenotonsillectomy for twins with congenital insensitivity to pain with anhidrosis: two case reports

Anesthetic management during adenotonsillectomy for twins with congenital insensitivity to pain with anhidrosis: two case reports

Anesthetic Management of Patients with Congenital Insensitivity to Pain with Anhidrosis

Labour outcomes in siblings with channelopathy associated insensitivity to pain due to bi-alleleic SCN9A mutations

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