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Research Notes: Surgery, Anesthesia and Prader-Willi Syndrome (PWS)Related topics: The following from Medscape Pediatrics, Treatment of Mitochondrial Cytopathies, is included here because muscle biopsies of those with PWS and reports in the literature suggest that mitochondrial impairment is probably more frequent in PWS than is commonly recognized. Most anesthetic and surgical procedures are well tolerated in patients with documented or suspected mitochondrial cytopathies. As part of a diagnostic evaluation, many patients undergo a muscle biopsy, in which general anesthesia has not been reported to cause problems. However, anesthesia probably does pose a small additional risk to those with mitochondrial diseases.
General anesthesia consists of induction with intravenous agents (i.e., thiopental, propofol, etomidate) followed by inhalation agents (i.e., nitrous oxide, halothane, enflurane, isoflurane, sevoflurane, and desflurane) for maintenance of anesthesia. Finally, muscle relaxants are occasionally used and include depolarizing (succinylcholine) and nondepolarizing agents.
There is intrinsically a greater risk of experiencing medication side effects in the setting of mitochondrial dysfunction. Despite the fact that some agents may interfere directly with mitochondrial function, complications associated with anesthesia are more likely to be related to the patient's clinical status prior to surgery. Reported adverse events include significant deterioration of baseline neurologic status, seizures, stroke, cardiac rhythm disturbances, respiratory failure, coma, and death. Increased sensitivity to several agents has been described, though reports are limited and usually anecdotal. Furthermore, a single anesthetic agent can rarely be implicated as the etiology of the decompensation. The absolute risk of an adverse anesthetic outcome in those with mitochondrial dysfunction is not known, although there is expanding literature on anesthetic-associated problems. Despite this, the majority of patients with mitochondrial cytopathies tolerate surgery and anesthesia without complications.
The anesthesiologist should be informed about the underlying pathophysiology of these disorders and potential complications related to general anesthesia. Additionally, preoperative assessment of patients should encompass a wide scope of clinical considerations given the multiple organ involvement frequently observed. Overall, the goal during anesthesia and surgery should be to maintain metabolic balance, which may require monitoring biochemical parameters throughout the procedure and sometimes for hours to days following surgery. This monitoring should include blood glucose, body temperature, and acid-base balance.
There are reports that document tolerance to many anesthetic medications. A 12-year-old boy with Kearns-Sayre syndrome tolerated anesthesia with opioids and isoflurane.[2] A 6-week-old infant girl with mitochondrial encephalomyopathy due to fumarase deficiency tolerated induction with intravenous thiopentone followed by isoflurane and nitrous oxide in oxygen. There were no intra- or postoperative complications or deterioration from her baseline status.[3] A 23-year-old with Kearns-Sayre syndrome was studied for her response to succinylcholine (1 mg per kg) and pancuronium (three divided doses of 0.02 mg per kg per dose). There was no change in her neurologic examination, and her response to succinylcholine and pancuronium was normal.[4]
Complications consisting of severe, diffuse white matter degeneration and death following anesthesia with thiopental, fentanyl, isoflurane, and pancuronium are described in a 13-month-old girl with mitochondrial myopathy.[5] A 51-year-old patient with Kearns-Sayre syndrome underwent emergent exploratory laparotomy for possible appendicitis. Anesthesia included thiopental (200 mg), vecuronium (0.1 mg per kg), nitrous oxide, oxygen, isoflurane, and supplemental fentanyl and vecuronium. Intraoperatively, the patient received lactated Ringer's solution. Postoperatively, he developed cyanosis and dyspnea, and subsequently required reintubation. EKG revealed left bundle-branch block and subsequent atrial fibrillation with ST-segment depression. Following appropriate treatment, the EKG reverted to his preoperative baseline. It is likely that the volatile anesthetics contributed directly to myocardial depression. The respiratory muscle weakness itself could have been due to the effects of premedication or as residual of inhaled anesthetics and/or muscle relaxants.[6]
The lack of uniformity from case reports make it impossible to draw conclusions regarding the hazards of a specific anesthetic agent, and the safest regimen of anesthesia for patients with mitochondrial cytopathies remains unknown. Review of the literature and personal experience, however, does allow for the application of some general rules and deductions, and global management considerations can be inferred.
An increased risk of perioperative pneumonia exists in the setting of hypotonia, bulbar dysfunction, and diminished respiratory capacity, a scenario common in patients with mitochondrial disease. Therefore, respiratory function should be strictly attended to during the perioperative period, as should heightened awareness for the possibility of infection. Chest physiotherapy should be included as a standard postoperative measure for those patients with premorbid pulmonary dysfunction. Moreover, patients may not have adequate responses to hypoxemia and/or hypercarbia [note that several studies have found that a significantly reduced or missing response to hypercapnia is almost universal in PWS]. If inhalation agents that are known to depress the ventilatory response to CO2 are to be administered (isoflurane, desflurane), patients must be adequately monitored in the perioperative and recuperative periods for hypoventilation and impending respiratory failure.[7]
Patients with an underlying seizure disorder may experience an increase in seizures immediately following surgery, and these should be appropriately managed. Dextrose-containing intravenous fluids should be provided if patients are required to fast preoperatively. Lactated Ringer's solution contains lactic acid and should probably be avoided.
Risk for malignant hyperthermia (MH) may be a consideration in those with mitochondrial dysfunction especially if myopathy is present. MH is triggered by inhalation anesthetics (i.e., halothane, enthrane) and/or depolarizing muscle relaxants (i.e., succinylcholine). Those agents known to trigger MH should be avoided if there has been a prior adverse reaction involving either the patient or a family member, but inhalation agents are routinely used safely in patients known to have a mitochondrial cytopathy. Regardless, dantrolene should be available and used at the first signs of malignant hyperthermia.
In association with infectious illnesses and other stressors, it is frequently noted that patients with mitochondrial cytopathies are at risk for respiratory failure and/or worsening of their underlying neurologic status. This deterioration is seen outside the setting of surgery and anesthesia, but can also occur with the stress of an illness requiring surgery and the necessary anesthesia. This worsening is believed to be in part related to the increase of cytokine production and subsequent formation of nitric oxide, which, in high amounts, may adversely affect energy production. In response to surgery, cytokines, including tumor necrosis factor (TNF), are also released. Consequently, these patients are at increased risk of worsened neurologic status, infections, and potential respiratory failure during the perioperative period. Elective surgery for patients with concurrent infection or other stressors should be delayed in an effort to avoid an exacerbation or clinical worsening of the underlying disease process.
For those patients at risk for cardiac conduction block (i.e., Kearns-Sayre syndrome), isoflurane is preferred over halothane because of the reduced risk of causing heart rhythm disturbance. Precautionary measures (i.e., readily available external cardiac pacemaker) and cardiac monitoring must be undertaken, given the risk for cardiac conduction abnormalities.
Spinal anesthesia should be used with caution in those patients with neuropathy or myopathy as part of their disease manifestations. However, it may permit monitoring of the patient's neurologic status intraoperatively and will allow for airway patency, and agents that may potentially trigger malignant hyperthermia can be avoided. For these reasons, spinal anesthesia (tetracaine) was administered to a 40-year-old man with mitochondrial encephalopathy who underwent open reduction and internal fixation of an ankle fracture. There were no immediate or delayed postoperative problems.[8]
It has been demonstrated in animal studies that propofol impairs mitochondrial function. Nonetheless, propofol has been safely utilized during anesthesia for many patients with mitochondrial dysfunction. Observations have been made, however, that extended and high-dose use over a period of days in treatment of refractory seizures leads to a syndrome analogous to mitochondrial failure.[9] Sodium nipride acts as an inhibitor of the respiratory chain and should be avoided as well.
Despite precautions, clinical worsening may occur following otherwise successful surgery and may be due to either the natural course of the mitochondrial disease process or the exacerbation due to the stress of surgery and consequent cytokine-mediated changes.
Ann Fr Anesth Reanim. 2007 Apr. OBJECTIVE: Mitochondria play a key role in energy metabolism within the cell through the oxidative phosphorylation. They are also involved in many cellular processes like apoptosis, calcium signaling or reactive oxygen species production. The objectives of this review are to understand the interactions between mitochondrial metabolism and anaesthetics or different stress situations observed in ICU and to know the clinical implications. DATA SOURCES: References were obtained from PubMed data bank (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi) using the following keywords: mitochondria, anaesthesia, anaesthetics, sepsis, preconditioning, ischaemia, hypoxia. DATA SYNTHESIS: Mitochondria act as a pharmacological target for the anaesthetic agents. The effects can be toxic like in the case of the local anaesthetics-induced myotoxicity. On the other hand, beneficial effects are observed in the anaesthetic-induced myocardial preconditioning. Mitochondrial metabolism could be disturbed in many critical situations (sepsis, chronic hypoxia, ischaemia-reperfusion injury). The study of the underlying mechanisms should allow to propose in the future new specific therapeutics. Anesthesiology. 2006 Oct. Mitochondria produce metabolic energy, serve as biosensors for oxidative stress, and eventually become effector organelles for cell death through apoptosis. The extent to which these manifold mitochondrial functions are altered by previously unrecognized actions of anesthetic agents seems to explain and link a wide variety of perioperative phenomena that are currently of interest to anesthesiologists from both a clinical and a scientific perspective. In addition, many surgical patients may be at increased perioperative risk because of inherited or acquired mitochondrial dysfunction leading to increased oxidative stress. This review summarizes the essential aspects of the bioenergetic process, presents current knowledge regarding the effects of anesthetics on mitochondrial function and the extent to which mitochondrial state determines anesthetic requirement and potential anesthetic toxicity, and considers some of the many implications that our knowledge of mitochondrial dysfunction poses for anesthetic management and perioperative medicine. Paediatr Anaesth. 2006 Jul. 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. Paediatr Anaesth. 2006 Jul. The case of a morbidly obese 3.5-year-old boy, with Prader-Willi syndrome (PWS), who experienced a life-threatening episode of pulmonary edema soon after induction of general anesthesia with sevoflurane and intubation for orchidopexy is presented. The patient, who had history of sleep apnea and who had an uneventful laparoscopy under general anesthesia 6 months previously, was supported with mechanical ventilation with positive end expiratory pressure but developed hyperthermia, pneumonia, sepsis, and Acute Respiratory Distress Syndrome in the intensive care unit. He recovered fully 11 days after surgery. The possible contributing factors for the development of pulmonary edema are discussed. Arrangements for monitoring in an intensive care setting after surgery are highly recommended for patients with PWS. Pediatr Pulmonol. 2006 Jan. The aim of our study was to evaluate the efficacy of adenotonsillectomy for the treatment of obstructive sleep apnea syndrome (OSA) in pediatric patients with Prader-Willi syndrome (PWS), and to describe the postoperative complications. Five patients (4 males; median age, 4.4 years; range, 1.6-14.2 years) were studied. All patients underwent an overnight cardiorespiratory sleep study. All patients had adenotonsillar hypertrophy (ATH), and two were also obese. The preoperative obstructive apnea/hypopnea index (AHI; median and range) was 12.2 (9.0-19.9) events/hr; the mean oxygen saturation was 95 (79-96)%; the nadir oxygen saturation was 71 (58-78)%; and the oxygen desaturation index (ODI) was 15.8 (11.4-35.9) events/hr. Preoperatively, patients were classified as having moderate to severe OSA. A second sleep study, performed 16 (3-43) months after adenotonsillectomy, showed a significant decrease in AHI (P = 0.009) and ODI (P = 0.009). Mean and nadir oxygen saturation did not differ significantly postsurgery (P = 0.188, P = 0.073, respectively). Four out of five children showed at least one postoperative complication. Difficult awakening from anesthesia, hemorrhages, and respiratory complications requiring reintubation and/or supplemental oxygen administration were observed. In conclusion, patients with PWS and OSA who underwent adenotonsillectomy showed a significant decrease in AHI and number of oxygen desaturations. Rev Esp Anestesiol Reanim. 2005 Aug-Sep. A 12-month-old boy diagnosed with propionic acidemia underwent gastrostomy. The patient's general state was good and he was alert, but with reduced muscular tone (unstable when seated with support, floppy head) and with dystonic movements in all extremities. An electroencephalogram showed slightly slowed brain activity. The patient was being treated with a low protein diet, phenobarbital, L-carnitine, L-isoleucine, and biotin. Surgery was carried out in satisfactory conditions with general anesthesia without opioids combined with infiltration of the surgical wound with local anesthetic. Recovery from anesthesia was rapid and free of complications. Propionic acidemia is caused by mitochondrial propionyl coenzyme carboxylase deficiency. Most patients have episodes of severe metabolic ketoacidosis as a result of excessive protein intake, delayed development, vomiting, gastroesophageal reflux, lethargy, hypotonia, and convulsions. The anesthetic approach involves avoiding triggers of metabolic acidosis (such as fasting, dehydration, hypoxemia, and hypotension) and preventing airway complications. Agents that metabolize propionic acid (such as succinylcholine, benzylisoquinoline neuromuscular blocking agents, and propofol) are not used, as they can exacerbate acidemia. We also believe that using local or regional anesthesia in combination with general anesthesia without opiates is safe and effective for controlling pain during surgery and postoperative recovery, as that combination avoids respiratory depression in these patients, who are highly sensitive to opiates. Eur J Anaesthesiol. 2004 Oct. No abstract available. Chang Gung Med J. 2003 Jun. Prader-Willi syndrome (PWS) is a sporadic disorder of chromosome abnormalities with an estimated prevalence of 1 in 15,000. It mainly affects the central nervous system, and often involves the hypothalamus. Both general and regional anesthesia for these patients is difficult mainly due to morbid obesity. Other common problems include hypotonia, disturbance in thermoregulation, arrhythmia, cor pulmonale, diabetes mellitus, behavior problems, and convulsions. We report on 2 pediatric patients with PWS receiving general anesthesia. The first patient experienced life-threatening episodes of severe hypoxemia in the postanesthesia care unit (PACU) as well as in the pediatric intensive care unit (PICU). Nasal continuous positive airway pressure (CPAP) was suggested by the pediatric pulmonary medicine specialist, and thereafter the patient's condition improved. The clinical course of the second patient was uneventful except for transient intermittent episodes of bronchospasms during emergence. In addition, we discuss differences between these 2 cases and our strategy for the prevention of perioperative complications for PWS patients in the future. Minerva Anestesiol. 2002 Oct. Prader-Willi syndrome (PWS) is a genetic disease caused by a loss of paternal genes located in chromosome 15. Children affected by this syndrome often have preterm delivery; during childhood the hallmarks are: severe infantile hypotonia and feeding problems. Afterward, neurologic manifestations, endocrine signs and dysmetabolic abnormalities are usually seen together with craniofacial manifestations and musculoskeletal abnormalities. Obesity causes sleep abnormalities including sleep apnea. The case we present is of a 5 year old child (CA) scheduled for strabismus surgery. The child has a lot of typical (PWS) signs. A number of anaesthesiologic problems are associated with (PWS). Some of them relate to obesity, others to facial dysmorphism. Moreover, the syndrome may give a prolonged and exaggerated response to every sedative drug. P.W.S. is also characterized by thermoregulatory disorders. Sleep apnea occurs often. Considering all these problems, we planned a monopharmacologic anaesthesiologic procedure using sevoflurane. Brain Pathol. 2002 Oct. Physiological cell death (PCD), a process by which redundant or unsuccessful neurons are deleted by apoptosis (cell suicide) from the developing central nervous system, has been recognized as a natural phenomenon for many years. Whether environmental factors can interact with PCD mechanisms to increase the number of neurons undergoing PCD, thereby converting this natural phenomenon into a pathological process, is an interesting question for which new answers are just now becoming available. In a series of recent studies we have shown that 2 major classes of drugs (those that block NMDA glutamate receptors and those that promote GABAA receptor activation), when administered to immature rodents during the period of synaptogenesis, trigger widespread apoptotic neurodegeneration throughout the developing brain. In addition, we have found that ethanol, which has both NMDA antagonist and GABAmimetic properties, triggers a robust pattern of apoptotic neurodegeneration, thereby deleting large numbers of neurons from many different regions of the developing brain. These findings provide a more likely explanation than has heretofore been available for the reduced brain mass and lifelong neurobehavioral disturbances associated with the human fetal alcohol syndrome (FAS). The period of synaptogenesis, also known as the brain growth spurt period, occurs in different species at different times relative to birth. In rats and mice it is a postnatal event, but in humans it extends from the sixth month of gestation to several years after birth. Thus, there is a period in pre- and postnatal human development, lasting for several years, during which immature CNS neurons are prone to commit suicide if exposed to intoxicating concentrations of drugs with NMDA antagonist or GABAmimetic properties. These findings are important, not only because of their relevance to the FAS, but because there are many agents in the human environment, other than ethanol, that have NMDA antagonist or GABAmimetic properties. Such agents include drugs that may be abused by pregnant mothers (ethanol, phencyclidine [angel dust], ketamine [Special K], nitrous oxide [laughing gas], barbiturates, benzodiazepines), and many medicinals used in obstetric and pediatric neurology (anticonvulsants), and anesthesiology (all general anesthetics are either NMDA antagonists or GABAmimetics). Rev Esp Anestesiol Reanim. 2001 Aug-Sep. No abstract available. Paediatr Anaesth. 2001 Jul. We report the anaesthetic management of a child with Prader-Willi syndrome and mitochondrial myopathy for open heart surgery. We used ketamine, fentanyl, rocuronium and caudal morphine together with a propofol infusion with no untoward effects. The implications of both conditions for anaesthesia are discussed. Rev Esp Anestesiol Reanim. 2001 Apr. No abstract available. Anesth Analg. 2000 Aug. Propionic acidemia is a rare genetic disease associated with significant medical problems. When patients with this disease present for surgery, their anesthetic must be tailored to meet their special needs. This case report provides information regarding propionic acidemia and its anesthetic management. World J Surg. 2000 Jun. Under normal circumstances there is a reciprocal relation between the availability of free fatty acids (FFAs) and glucose in plasma. In the fasted state, FFAs predominate in both availability and the relative contribution to energy production, whereas the same is true for glucose in the fed state. The extent of glucose oxidation is directly determined by its availability, whereas FFAs are normally available well in excess of their rate of oxidation. The rate of FFA oxidation is determined by the rate of transfer into the mitochondria via the carnitine palmitoyltransferase (CPT) enzyme system, which in turn is regulated by the metabolism of glucose. With critical illness the stress response involves mobilization of both plasma glucose and FFAs simultaneously in both the fed and fasted states. In the situation of excess availability of substrates, the metabolism of glucose limits the oxidation of FFAs, thereby channeling those fatty acids into triglyceride (TG) stores in the muscle and the liver. The high FFA concentrations and increased tissue TG stores can limit glucose clearance from the blood, thereby contributing to the development of hyperglycemia. Also, the excessive metabolism of glucose can result in lactic acidemia and can contribute to the depletion of muscle glutamine. The nutritional treatment of such patients must account for these underlying metabolic responses to avoid amplifying potentially detrimental responses to the excess availability of substrates already present in the fasting state. Environ Health Perspect. 2000 Jun. We review recent findings pertaining to several environmental agents (ethanol, phencyclidine, ketamine, nitrous oxide, barbiturates, benzodiazepines, halothane, isoflurane, and propofol) that have the potential to delete large numbers of neurons from the developing brain by a newly discovered mechanism involving interference in the action of neurotransmitters [glutamate and gamma-amino butyric acid (GABA) at (italic)N(/italic)-methyl-d-aspartate (NMDA)] and GABA(subscript)A(/subscript) receptors during the synaptogenesis period, also known as the brain growth-spurt period. Transient interference (lasting >= 4 hr) in the activity of these transmitters during the synaptogenesis period (the last trimester of pregnancy and the first several years after birth in humans) causes millions of developing neurons to commit suicide (die by apoptosis). Many of these agents are drugs of abuse (ethanol is a prime example) to which the human fetal brain may be exposed during the third trimester by drug-abusing mothers. Ethanol triggers massive apoptotic neurodegeneration in the developing brain by interfering with both the NMDA and GABA(subscript)A(/subscript) receptor systems, and this can explain the reduced brain mass and lifelong neurobehavioral disturbances associated with intrauterine exposure of the human fetus to ethanol (fetal alcohol syndrome). Exposure of the immature brain in a medical treatment context is also of concern because many of these agents are drugs used frequently as sedatives, tranquilizers, anticonvulsants, or anesthetics in pediatric and/or obstetrical medicine. Because this is a newly discovered mechanism, further research will be required to fully ascertain the nature and degree of risk posed by exposure of the developing human brain to environmental agents that act by this mechanism. Paediatr Anaesth. 1998. A review of a case series of sixteen anaesthetics in eight cases was undertaken to determine whether children with Prader-Willi syndrome present particular problems to the anaesthetist. Children in an early stage of the condition who are below their centile for weight present no specific problems. Children who are heavier than 97th centile weight have problems associated with their obesity: difficult intravenous access and sleep apnoea. Scoliosis was noted in both groups and was not associated with problems after minor surgery. Rev Esp Anestesiol Reanim. 1997 Oct. No abstract available. Minerva Anestesiol. 1996 Oct. The case of a woman of 27 affected by the Prader-Willi syndrome who underwent general anaesthesia for dental surgery is reported. The patient presented severe mental retardation, small stature, moderate muscular hypotonia, hyperphagia, obesity, and diabetes mellitus. Premedication consisted of diazepam and atropine; anaesthesia was induced with propofol and maintained with propofol, fentanyl and N2O; muscle paralysis was obtained with atracurium. A small glottis was observed at laryngoscopy so that a 6 mm cuffed tube was inserted. Surgery lasted 75 minutes; the patient recovered promptly a few minutes following the end of propofol infusion; no postoperative complication was recorded. As hypoglycemia can occur during and after surgery in the Prader-Willi syndrome, plasma samples for glucose, NEFA, insulin, cortisol, and growth hormone (GH) were collected prior to the induction of anaesthesia (A), 20 minutes after starting surgery (B), at the end of surgery (C), and 3 hours later (D). In spite of the infusion of glucose, hyperglycemia was observed just in C and D samples (A:77; B:88; C:245; D:279 mg/dl). Stable NEFA values, within the normal range, were observed (A:77; B:88; C:245; D:279 mg/dl) suggesting poor or absent lipolysis. Insulin decreased progressively during surgery (A:10.5; B:8.8; C:5.4; D:7.0 mU/L). Cortisol peaked in B (A:9.5; B:20.9; C:13.4; D:4.8 micrograms/dl), suggesting normal hypothalamic reactivity to the surgical stimulus. Finally very low GH levels were observed (A:0.04; B:0.07; C:0.06; D:0.09 ng/ml) suggesting GH deficiency, which had possibly affected the size of patient's glottis. Our data support the hypothesis that hypoglycemia in the Prader-Willi syndrome originates from inadequate lipolysis during starvation. Masui. 1995 Dec. A patient with Prader-Willi syndrome developed bronchospasm during anesthesia. The patient was a 9-year-old boy and was scheduled for orchiopexy. His psychomotor development was delayed, and at 12 months of age he was diagnosed as Prader-Willi syndrome by chromosomal examination. The patient weighed 17 kg, was 111 cm tall, and had no symptom of upper respiratory infection preoperatively. Preoperative examinations were normal except supraventricular extrasystole in electrocardiogram. Following administration of scopolamine 0.15 mg intramuscularly as preanesthetic medication, anesthesia was induced smoothly by slow induction using N2O-O2-sevoflurane. However, right after endotracheal intubation with vecuronium 2 mg, remarkable stridor was noticed. Despite hyperventilation, the patient exhibited hypercapnia, and the diagnosis of bronchospasm was made. Aminophylline and steroid were administered intravenously and halothane was inhaled instead of sevoflurane. The bronchospasm was improved gradually and surgery was finished. Prader-Willi syndrome is an uncommon disease first reported by Prader in 1956 and characterized by hypotonia, hypomentia, hypogonadism and obesity. In the perioperative management for a patient with Prader-Willi syndrome, special attention must be paid to the abnormalities in the upper and lower respiratory systems. J R Soc Med. 1991 Apr. J Bone Joint Surg Br. 1989 Aug. There is a high incidence of spinal deformity in children with the Prader-Willi syndrome. We have encountered major complications following spinal surgery in this condition. We report our experience and conclude that spinal surgery is a formidable undertaking and the risks should be appreciated by the surgeon and the parents. Rev Esp Anestesiol Reanim. 1989 May-Jun. No abstract available. Minerva Anestesiol. 1988 Dec. No abstract available. Rev Esp Anestesiol Reanim. 1988 Jul-Aug. No abstract available. Can Anaesth Soc J. 1983 Sep. No abstract available. Can Anaesth Soc J. 1983 Mar. The anaesthetic management of four paediatric patients with the Prader-Willi syndrome is reported. The syndrome is characterized by obesity, mental retardation, genital hypoplasia, hypotonia, and diabetes mellitus. All patients were anaesthetized with halothane. Succinylcholine or pancuronium were used for muscle relaxation, without evidence of abnormal response. Common anaesthetic difficulties in this syndrome are obesity, hypotonia, disturbance in thermoregulation, arrhythmias, diabetes mellitus and convulsions. Masui. 1981 Jul. No abstract available. Masui. 1979 May. No abstract available. Anesthesiology. 1976 Feb. No abstract available. Anesthesiology. 1975 Nov. No abstract available. |