Anesthetic considerations in children with Hutchinson‐Gilford progeria syndrome: A narrative review

Abstract

Background Hutchinson‐Gilford progeria syndrome is a rare disease in childhood that results in premature aging. The presence of multisystem derangements including skin, bone, and joint diseases and possibly a difficult airway makes the anesthetic management challenging. Because of the extremely low prevalence, experience is limited even for experienced pediatric anesthesiologists. Objective To review the available literature on anesthesia for patients with Hutchinson‐Gilford progeria syndrome and to give recommendations for establishing the best practice for patients with Hutchinson‐Gilford progeria syndrome. Design A narrative review of the rare existing literature. Date sources CENTRAL (Cochrane), EMBASE, Google Scholar, MEDLINE and PubMed. Eligibility criteria Articles addressing anesthesia in patients with Hutchinson‐Gilford progeria syndrome were included. Results An overview of the current literature was made on anesthesia care for patients with Hutchinson‐Gilford progeria syndrome. After screening the literature, only ten articles were found to be of interest and include some case reports and a correspondence. The focus points on how to perform anesthesia care in patients with Hutchinson‐Gilford progeria syndrome and the entire perioperative care are suggested. The available data are limited and results need to be interpreted with caution. Conclusion The patients with Hutchinson‐Gilford progeria syndrome are not just “frail” patients. Awareness concerning intubation difficulties is mandatory, and airway strategies must be addressed in advance. Although these patients present with the physiology of an elderly with accompanying comorbidities, emotionally they are only children and should be approached as such.

Large epidural hematoma in a child with progeria syndrome complicated by posterior cerebral artery stroke: A case report

“…Progeria, also known as Hutchinson-Gilford progeria Syndrome (HGPS), is a rare genetic disorder with a global incidence of 1 in 8 million live births (1). Patients usually demonstrate a prematurely aged appearance.…”

“…There are many physical consequences of HGPS that can interfere with an anaesthetic act and neurosurgical procedure (1). Although these patients present as elderly persons with accompanying comorbidities, it is important to interact with them at the appropriate developmental mental level.…”

Anaesthetic management of paediatric patient with Hutchinson–Gilford progeria syndrome: A case report

“…Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder, with an estimated incidence of 1:8,000,000 live births. [ 1 ] The clinical presentation involves characteristic changes in the skin, musculoskeletal and cardiovascular systems, resulting in a premature ageing phenotype. [ 2 ] These patients are at higher risk of complications during anaesthesia, related to abnormal airway anatomy, increased risk of ischaemic events, positioning-related injuries and greater propensity to hypothermia and hypoglycaemia.…”

“…[ 3 ] Difficult airway is a main concern: previous reports describe inappropriate ventilation under face mask and difficult intubation. [ 1 , 4 ] This is a consequence of multiple craniofacial abnormalities: limited mouth opening and neck mobility, micro- and retrognathia, mandibular and maxillary hypoplasia and abnormal dentition. [ 1 , 3 ] In this case, supraglottic devices, a videolaryngoscope and an optical fibrescope were available, and we carried out a strategy that preserved spontaneous ventilation.…”

“…[ 1 , 4 ] This is a consequence of multiple craniofacial abnormalities: limited mouth opening and neck mobility, micro- and retrognathia, mandibular and maxillary hypoplasia and abnormal dentition. [ 1 , 3 ] In this case, supraglottic devices, a videolaryngoscope and an optical fibrescope were available, and we carried out a strategy that preserved spontaneous ventilation. This is the first case report describing the successful use of ketamine and dexmedetomidine as induction agents for patients with HGPS.…”