Late onset congenital central hypoventilation syndrome after exposure to general anesthesia

Mahfouz, Abdul Kader; Rashid, Mohammed; Khan, Mohammed S.; Reddy, Prabhakar

doi:10.1007/s12630-011-9590-7

Cited by 30 publications

(18 citation statements)

References 17 publications

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“…Although this is a plausible explanation, it is less likely because our patient had two previous anesthetics for MRI without complications of respiratory depression. Another possible explanation is that the general anesthesia may have triggered an apneic event associated with late‐onset central hypoventilation syndrome occurring beyond the neonatal period owing to variable penetrance of the PHOX2B mutation . This scenario is unlikely because this syndrome usually results in the need for permanent nocturnal ventilator support, which was not the case.…”

Section: Discussionmentioning

confidence: 99%

“Saved by the Bell”: Near SUDEP during intracranial EEG monitoring

Baarmah¹,

Donner

Ochi

et al. 2018

Epilepsia Open

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Summary SUDEP is the sudden unexpected death of a person with epilepsy, when no structural or toxicological cause of death can be found. The majority of witnessed cases are reported to be preceded by a convulsive seizure and postictal hypoventilation. Here, we report an 8‐year‐old girl with drug‐resistant focal seizures secondary to a focal cortical dysplasia type IIb. While undergoing invasive intracranial monitoring with subdural and depth electrodes, she had a clinical apnea event recorded on video, followed by bradycardia, which required resuscitation. Her intracranial electroencephalogram (EEG) during the event showed diffuse slowing and attenuation of cortical activity, with bradycardia that responded to positive pressure ventilation with oxygen. This near SUDEP event was not preceded by either an electroclinical or electrographic seizure. This is the first report of a witnessed, near‐SUDEP event during intracranial monitoring. It emphasizes the fact that near‐SUDEP can occur without a preceding seizure.

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Section: Discussionmentioning

confidence: 99%

“Saved by the Bell”: Near SUDEP during intracranial EEG monitoring

Baarmah¹,

Donner

Ochi

et al. 2018

Epilepsia Open

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“…At present, the cardiorespiratory effects of pediatric CCHS and ROHHAD patients undergoing lung isolation on both lungs during the same anesthetic has not been well described. There are individual and compilations of case reports describing the anesthetic management of CCHS and ROHHAD patients . Data are further limited on the perioperative complications associated with thoracoscopic diaphragm pacemaker placement .…”

Section: Introductionmentioning

confidence: 99%

“…There are individual and compilations of case reports describing the anesthetic management of CCHS and ROHHAD patients. [13][14][15][16][17][18][19] Data are further limited on the perioperative complications associated with thoracoscopic diaphragm pacemaker placement. [8][9][10][11][12] Despite limited perioperative complications documented in the literature in patients with known CCHS and ROHHAD, 13 we hypothesized that these patients undergoing thoracoscopic phrenic nerve-diaphragmatic pacemaker insertion would be at high risk for cardiovascular instability and pulmonary issues under anesthesia due to their preoperative comorbidities, inability to mount adequate stress responses due to the innate autonomic dysregulation, and the need for sequential bilateral lung isolation.…”

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confidence: 99%

“…T A B L E 2 (Continued) Although the surgical literature describes the perioperative complications and operative success of these patients, there is limited literature on the anesthetic management of CCHS and ROHHAD patients undergoing thoracoscopic diaphragmatic pacemaker placement. Both intravenous and volatile anesthetics have been used successfully without postoperative complications, though authors recommend the use of short-acting sedatives in CCHS patients to decrease postoperative respiratory depression 13,15,16. Respiratory depression from narcotics was not deemed to be an issue because these patients are ventilated continuously postoperatively in the intensive care unit; the diaphragmatic pacemakers are not turned on until several weeks postoperatively to allow the surgical incisions time to heal.…”

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confidence: 99%

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Perioperative anesthetic management of children with congenital central hypoventilation syndrome and rapid‐onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation undergoing thoracoscopic phrenic nerve‐diaphragm pacemaker implantation

Ballard

Leavitt

Chin

et al. 2018

Pediatric Anesthesia

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Summary Background Congenital Central Hypoventilation Syndrome and Rapid‐Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation are rare neurocristopathies characterized by autonomic dysregulation including bradyarrhythmias, abnormal temperature control, and most significantly, abnormal control of breathing leading to tracheostomy and ventilator dependence as life support. Surgical advancements have made phrenic nerve‐diaphragm pacemakers available, to eliminate the tether to a mechanical ventilator for 12‐15 hours each day. The thoracoscopic approach to implantation has allowed for a less invasive approach which may have implications for pain control and recovery time. However, thoracoscopic implantation of these devices presents several challenges to the anesthesiologist in these complex ventilator‐dependent patients, including, but not limited to, sequential lung isolation, prevention of hypothermia, and management of arrhythmias. Postoperative challenges may also include strategies to treat hemodynamic instability, managing the ventilator following lung derecruitment, and providing adequate pain control. Aims We aimed to describe the anesthetic management of Congenital Central Hypoventilation Syndrome and Rapid‐Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation patients undergoing thoracoscopic phrenic nerve‐diaphragm pacemaker implantation and the nature and incidence of perioperative complications. Methods A retrospective chart review was performed of 14 children with Congenital Central Hypoventilation Syndrome and Rapid‐Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation undergoing phrenic nerve‐diaphragm pacemaker implantation at a single academic pediatric hospital between 2009 and 2017. Demographic information, intraoperative management, and perioperative complications were analyzed from patient records. Results Twelve of 14 patients (86%) underwent an inhalational induction via tracheostomy. Lung isolation was achieved via fiberoptic guidance of a single lumen endotracheal tube sequentially into the right or left mainstem bronchi for 12 patients (86%). Double lumen endotracheal tubes were utilized in two patients (7%) and bronchial blockers in two patients (7%) for lung isolation. Anesthesia was maintained using a balanced technique of volatile agents (sevoflurane/isoflurane) and opioids (fentanyl). Bradyarrhythmias developed in six patients (43%) during surgery, 5 (36%) responded to anticholinergics and one patient (7%) required backup cardiac pacing using a previously implanted bipolar cardiac pacemaker. Intraoperative hypothermia (<35.5°C) was present in five patients (36%) despite the use of warming devices. Hypercarbia (>50 mm Hg) during lung isolation was present in eight patients (57%) and hemoglobin desaturation (<90%) in four patients (29%). Postoperatively, oxygen desaturation was a common complication with nine patients (64%) requiring supplemental oxygen administration via mechani...

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“… 4 , 5 Older children and young adults have been identified where the presentation is severe respiratory compromise following general anesthesia or respiratory infections. 6 , 7 With increased awareness of CCHS and early intervention, many CCHS children are now surviving into adulthood. In this review, we will discuss the clinical features, diagnostic and management challenges of patients with CCHS.…”

Section: Introductionmentioning

confidence: 99%

Congenital central hypoventilation syndrome: Diagnostic and management challenges

Kasi¹,

Perez²,

Kun³

et al. 2016

PHMT

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Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder with failure of central control of breathing and of the autonomic nervous system function due to a mutation in the paired-like homeobox 2B (PHOX2B) gene. Affected patients have absent or negligible ventilatory sensitivity to hypercapnia and hypoxemia, and they do not exhibit signs of respiratory distress when challenged with hypercarbia or hypoxia. The diagnosis of CCHS must be confirmed with PHOX2B gene mutation. Generally, the PHOX2B mutation genotype can aid in anticipating the severity of the phenotype. They require ventilatory support for life. Home assisted ventilation options include positive pressure ventilation via tracheostomy, noninvasive positive pressure ventilation, and diaphragm pacing via phrenic nerve stimulation, but each strategy has its associated limitations and challenges. Since all the clinical manifestations of CCHS may not manifest at birth, periodic monitoring and early intervention are necessary to prevent complications and improve outcome. Life-threatening arrhythmias can manifest at different ages and a normal cardiac monitoring study does not exclude future occurrences leading to the dilemma of timing and frequency of cardiac rhythm monitoring and treatment. Given the rare incidence of CCHS, most health care professionals are not experienced with managing CCHS patients, particularly those with diaphragm pacers. With early diagnosis and advances in home mechanical ventilation and monitoring strategies, many CCHS children are surviving into adulthood presenting new challenges in their care.

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Late onset congenital central hypoventilation syndrome after exposure to general anesthesia

Cited by 30 publications

References 17 publications

“Saved by the Bell”: Near SUDEP during intracranial EEG monitoring

“Saved by the Bell”: Near SUDEP during intracranial EEG monitoring

Perioperative anesthetic management of children with congenital central hypoventilation syndrome and rapid‐onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation undergoing thoracoscopic phrenic nerve‐diaphragm pacemaker implantation

Congenital central hypoventilation syndrome: Diagnostic and management challenges

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