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Title: Paroxysmal extreme pain disorder (PEPD) is an autosomal dominant painful neuropathy with many, but not all, cases linked to gain-of-function mutations in SCN9A which encodes voltage-gated sodium channel Na. 1.7. It is a very rare condition featured by flushing of the lower half of the body and excruciating burning pain caused by any stimulus below the waist or in the perianal region. PEPD may be associated with cardiovascular instability, especially prolonged sinus pauses, and thus has anesthetic implications. Pacemaker implantation is the alternative therapeutic option, but its indications have not been clarified yet.Background: This condition is well described in neurological literature, but to our knowledge, this is the first case report of a patient with paroxysmal extreme pain disorder with prolonged sinus pauses requiring anesthesia for an epicardial pacemaker even with the perioperative risk of the pathology. This clinical observation can help for a better management and understanding of the cardiac risk complications of PEPD especially for an infant whose diagnostic is frequently made at the stage of complication This clinical observation can put the item on the necessity of establishing recommendations for management of cardiac complications during PEPD. Case report:We extensively searched the literature on cardiac pacing in patients with PEPD and we described a new case of a 9 month old infant who was admitted in the emergency department for an episode of malaise apnea and hemifacial cyanosis relevant to PEPD. The neurologic exploration was normal. The diagnostic was confirmed by genetic study. The 24 hours recording demonstrated long pauses of 15 seconds during the crisis justifying the implantation of epicardial pacemaker without peri-operatory complications due to the high anesthetic risk of this pathology. IntroductionParoxysmal extreme pain disorder (PEPD) is an autosomal dominant painful neuropathy with many, but not all, cases linked to gain-of-function mutations in SCN9A which encodes voltage-gated sodium channel Na. 1.7. The paper should be of interest because this clinical observation can put the item on the severity of the cardiac risk complications of this disease especially for an infant whose diagnostic is frequently made at the stage of complication. This clinical observation can help for a better understanding of the management of anesthetic process Because of the potential for cardiovascular instability ObservationA 9-month-old infant was brought to the emergency department after an episode of malaise and hemifacial cyanosis. Her parent reported that the crisis was triggered by crying. The infant had vegetative manifestations like hemifacial redness, snorkeling and sweats with foam at the lips, motor manifestations like rubbing feet with reduced consciousness. So the infant turned blue around the lips and began gasping for air. During this time, her eyes "rolled back", and she did not interact with her parents. There were no clonic movements, and she did not respond to ...

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