Anaesthetic considerations in the Prader-Willi syndrome: Report of four cases

Yamashita, Masao; Koishi, Keiko; Yamaya, R; Tsubo, Toshihito; Matsuki, Akitomo; Oyama, Tatsuo

doi:10.1007/bf03009349

Cited by 20 publications

(11 citation statements)

References 14 publications

(1 reference statement)

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“…4 There have been reports in the literature of cardiovascular abnormalities, such as hypertension and arrhythmias (premature ventricular contractions), in patients with this syndrome. 5 However, we could find no reports of recalcitrant bradycardia occurring in association with the Prader-Willi Syndrome upon prone positioning.…”

Section: Discussionmentioning

confidence: 95%

Anaesthesia for scoliosis correction surgery complicated by severe recalcitrant bradycardia upon prone positioning in an adolescent with Prader-Willi syndrome

Conrick-Martin

Walshe

Carson

et al. 2013

Southern African Journal of Anaesthesia and Analgesia

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Section: Discussionmentioning

confidence: 95%

Anaesthesia for scoliosis correction surgery complicated by severe recalcitrant bradycardia upon prone positioning in an adolescent with Prader-Willi syndrome

Conrick-Martin

Walshe

Carson

et al. 2013

Southern African Journal of Anaesthesia and Analgesia

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“…Both succinylcholine and non-depolarizing relaxants can be safely used in patients with PWS [23]. Additionally, in PWS patients, morbid obesity increases the abdominal pressure, which increases the occurrence rate of hiatal hernia along with the risk of aspiration pneumonia [24].…”

Section: Discussionmentioning

confidence: 99%

Anaesthesia Related Death in Prader Willi Syndrome: Appropriate Management and Medical Liability

Snenghi¹

2017

Austin J Anesthesia and Analgesia

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Prader -Willi syndrome (PWS) is a complex multisystem genetic disorder associated with a constant chromosomal abnormality. The anesthesiological care of patients with PWS include a careful approach to the potential difficulties with airway, an intraoperative strict control of ventilatory modality, proper management of metabolic disturbances and adequate cardio circulatory support.The PWS requires various heterogeneous surgical interventions such as squint correction, spinal surgery for scoliosis, palatal and orthopedic surgery, dental rehabilitation for common tooth decay, cholecystectomy, pacemaker placement and orchidopexy for cryptorchidism For these reasons patients with PWS may frequently undergo local, regional and general anesthesia. Some Authors describe data related to patients with medical complex disease to have a higher rate of mortality and morbidity and poor outcome which reported for procedures performed in and outside the operative room.We present a fatal case of severe bronchospasm with serious hypoxia during general anesthesia in a young patient affected by PWS with chronic pancreatitis and biliary calculosis.

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“…Hypotonia has prompted some authors to caution against the use of neuromuscular blocking agents in patients with PWS, especially neonates and infants in phase 1 of the syndrome. However, several reports have demonstrated the safe use of various nondepolarizing neuromuscular blocking agents including pancuronium, atracurium, vecuronium, and rocuronium without evidence of prolonged effects (17,26–28,31). Although we would suggest caution with the use of succinylcholine in the presence of hypotonia given the theoretical risk of an exaggerated hyperkalemic response, several of the reports have also demonstrated the safe use of succinylcholine in patients with PWS (17,25,26,30).…”

Section: Discussionmentioning

confidence: 99%

“…However, several reports have demonstrated the safe use of various nondepolarizing neuromuscular blocking agents including pancuronium, atracurium, vecuronium, and rocuronium without evidence of prolonged effects (17,26–28,31). Although we would suggest caution with the use of succinylcholine in the presence of hypotonia given the theoretical risk of an exaggerated hyperkalemic response, several of the reports have also demonstrated the safe use of succinylcholine in patients with PWS (17,25,26,30). Although Mayhew and Taylor (26) reported a malignant hyperthermia‐like picture in one of their patients following the administration of succinylcholine and halothane, the postoperative creatinine phosphokinase level was not elevated and other than a rapid elevation of body temperature and acidosis, no other manifestations of MH were noted.…”

Section: Discussionmentioning

confidence: 99%

Anesthesia and Prader–Willi syndrome: preliminary experience with regional anesthesia

Legrand

Tobias

2006

Pediatric Anesthesia

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The constellation of neonatal hypotonia, developmental delay, hypogonadism and obesity caused by hyperphagia was first reported in 1956 and subsequently termed Prader-Willi syndrome (PWS). Genetic analysis has demonstrated abnormalities of chromosome 15. Anesthesia concerns of PWS include morbid obesity, the potential for difficulties with airway management, risk for perioperative respiratory failure, abnormalities in the central control of ventilation and temperature, rare reports of primary myocardial involvement, aggressive and at times violent behavior and glucose intolerance. For the first time, we report the use of regional anesthesia in four patients with PWS. A lumbar plexus catheter was used to provide postoperative analgesia in one patient while regional anesthesia (fasica iliaca block, spinal anesthesia, and lateral vertical infraclavicular block) was used to provide primary intraoperative anesthesia in three other patients while avoiding the need for general anesthesia. Previous reports of the anesthesia care of patients with PWS are reviewed and the potential perioperative implications of the sequelae of PWS are discussed.

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Anaesthetic considerations in the Prader-Willi syndrome: Report of four cases

Cited by 20 publications

References 14 publications

Anaesthesia for scoliosis correction surgery complicated by severe recalcitrant bradycardia upon prone positioning in an adolescent with Prader-Willi syndrome

Anaesthesia for scoliosis correction surgery complicated by severe recalcitrant bradycardia upon prone positioning in an adolescent with Prader-Willi syndrome

Anaesthesia Related Death in Prader Willi Syndrome: Appropriate Management and Medical Liability

Anesthesia and Prader–Willi syndrome: preliminary experience with regional anesthesia

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