Perioperative outcomes and the effects of anesthesia in congenital central hypoventilation patients

Chang, Gloria Y; Salazar, Tate; Karnwal, Abhishek; Kun, Sheila S.; Ellashek, J.; Shin, Cathy E.; McComb, J. Gordon; Keens, Thomas G.; Perez, Iris A.

doi:10.1007/s11325-022-02632-z

Cited by 4 publications

(7 citation statements)

References 27 publications

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“…Congenital cases are caused by a gene mutation in PHOX2B , leading to a polyalanine repeat expansion mutation, affecting neural crest cell migration and autonomic nervous system development. 8 CHS presents when the descending anterolateral medullocervical pathway ( Fig. 3 ), which is responsible for autonomic breathing, is affected, and therefore may include congenital or acquired etiologies.…”

Section: Discussionmentioning

confidence: 99%

Ondine’s curse: clinical presentation with diaphragmatic pacing and spontaneous respiratory recovery. Illustrative case

Schüpper

Devarajan

Lee

et al. 2023

Journal of Neurosurgery: Case Lessons

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BACKGROUND The complexity of posterior fossa surgery can often lead to rare complications due to the anatomy involved. Vestibular schwannoma resection is a common pathology in the posterior fossa, often requiring surgical intervention. Given the proximity of this space to the brainstem, cranial nerve VII/VIII complex, and posterior inferior cerebellar artery (PICA), neurovascular complications are not infrequent. A rare vascular complication from this surgical approach is a lateral medullary infarction from injury to the lateral medullary segment of the proximal PICA, leading to central hypoventilation syndrome (CHS). OBSERVATIONS This report presents a unique case of a 51-year-old man who underwent a retrosigmoid craniectomy for resection of a vestibular schwannoma. Following surgery, the patient was unable to be weaned off the ventilator and was noted to become apneic while he slept, a clinical picture consistent with Ondine’s curse. LESSONS This report discusses the anatomical considerations of this surgical corridor leading to this complication and the management of a patient with acquired Ondine’s curse and reviews the scarce literature on this uncommon cause of acquired CHS.

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Section: Discussionmentioning

confidence: 99%

Ondine’s curse: clinical presentation with diaphragmatic pacing and spontaneous respiratory recovery. Illustrative case

Schüpper

Devarajan

Lee

et al. 2023

Journal of Neurosurgery: Case Lessons

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“…Additionally, DP for SCI is associated with a reduced risk of respiratory infections ( 47 ) and improved quality of life ( 32 ). Beyond SCI, reports in congenital central hypoventilation syndrome ( 48 ), acute flaccid myelitis ( 49 ), and other neurological conditions ( 50 ) indicate off-label clinical use of DP can be implemented safely and associated with relatively few adverse effects ( 31 ).…”

Section: Discussionmentioning

confidence: 99%

Diaphragm pacing and independent breathing in individuals with severe Pompe disease

Liberati

Byrne

Fuller

et al. 2023

Front. Rehabil. Sci.

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IntroductionPompe disease is an inherited disease characterized by a deficit in acid-α-glucosidase (GAA), an enzyme which degrades lysosomal glycogen. The phrenic-diaphragm motor system is affected preferentially, and respiratory failure often occurs despite GAA enzyme replacement therapy. We hypothesized that the continued use of diaphragm pacing (DP) might improve ventilator-dependent subjects' respiratory outcomes and increase ventilator-free time tolerance.MethodsSix patients (3 pediatric) underwent clinical DP implantation and started diaphragm conditioning, which involved progressively longer periods of daily, low intensity stimulation. Longitudinal respiratory breathing pattern, diaphragm electromyography, and pulmonary function tests were completed when possible, to assess feasibility of use, as well as diaphragm and ventilatory responses to conditioning.ResultsAll subjects were eventually able to undergo full-time conditioning via DP and increase their maximal tolerated time off-ventilator, when compared to pre-implant function. Over time, 3 of 6 subjects also demonstrated increased or stable minute ventilation throughout the day, without positive-pressure ventilation assistance.DiscussionRespiratory insufficiency is one of the main causes of death in patients with Pompe disease. Our results indicate that DP in Pompe disease was feasible, led to few adverse events and stabilized breathing for up to 7 years.

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“…Complications can occur after neuromuscular block. [60][61][62] Patients with CCHS are sensitive to sedatives and narcotics. Significant respiratory depression can occur after non-oral opioid administration 60,63,64 ; short-acting sedatives are recommended.…”

Section: Anaesthesiamentioning

confidence: 99%

“…This difficulty may be the first manifestation of late-onset CCHS. 62 Pain relief should consist of non-opioid analgesic agents. Patients may require increased and prolonged ventilatory support after surgery.…”

Section: Anaesthesiamentioning

confidence: 99%

Congenital central hypoventilation syndrome in children: a Hong Kong perspective

Hon,

Fung,

Leung

et al. 2023

Hong Kong Med J

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Perioperative outcomes and the effects of anesthesia in congenital central hypoventilation patients

Cited by 4 publications

References 27 publications

Ondine’s curse: clinical presentation with diaphragmatic pacing and spontaneous respiratory recovery. Illustrative case

Ondine’s curse: clinical presentation with diaphragmatic pacing and spontaneous respiratory recovery. Illustrative case

Diaphragm pacing and independent breathing in individuals with severe Pompe disease

Congenital central hypoventilation syndrome in children: a Hong Kong perspective

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