1/42. association of misoprostol, Moebius syndrome and congenital central alveolar hypoventilation. Case report.We report a case showing the association of Moebius syndrome, the use of misoprostol during pregnancy and the development of central congenital alveolar hypoventilation. Pathophysiological aspects of these three diseases are discussed and also the unfavorable prognosis of this association.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
2/42. Anesthetic care for the child with congenital central alveolar hypoventilation syndrome (Ondine's curse).Idiopathic congenital central alveolar hypoventilation syndrome, otherwise known as Ondine's curse, is a rare neuropathologic syndrome characterized by an inadequate respiratory drive with hypoventilation and periods of prolonged apnea resulting in hypercarbia and hypoxemia. Although no definite pathologic abnormality has been identified to account for the disorder, it is thought to represent a primary defect related to altered function of central chemoreceptors resulting in defective control of minute ventilation. Associated problems related to neural crest cell migration, including neuroblastoma formation and Hirschsprung's disease, suggest that the primary defect is defective neural crest cell migration and function. Problems that may impact on perioperative care include the defective central control of ventilation and defective control of upper respiratory musculature, which may lead to upper airway obstruction. Although many patients will have previously undergone tracheostomy and chronic mechanical ventilation, problems in other organ systems can impact on perioperative care. Cardiovascular issues include the possible presence of cor pulmonale and autonomic nervous system dysfunction. central nervous system issues include the frequent occurrence of seizures and mental retardation. The preoperative work-up, premedication, and the intraoperative/postoperative care and monitoring of these patients is reviewed.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
3/42. Upper airway function in Ondine's curse.Congenital central alveolar hypoventilation (Ondine's curse) is a rare disorder with absence of automatic control of ventilation but preservation of voluntary breathing. Phrenic pacing, used to treat this condition, is usually not successful without a tracheotomy. We performed fiberoptic videoendoscopy on an affected infant airway. During wakefulness and ventilation by a negative pressure ventilator, the airway was normal, but with sleep onset, passive inspiration produced phasic epiglottic collapse. This case demonstrates the importance of central control in maintaining upper airway patency.- - - - - - - - - - ranking = 0.2keywords = alveolar (Clic here for more details about this article) |
4/42. Congenital central alveolar hypoventilation syndrome (Ondine's Curse): effectiveness of early home ventilation for normal development.An 8 month old Caucasian girl, with congenital central alveolar hypoventilation syndrome (Ondine's Curse), was discharged with her home ventilation managed by her parents. Her subsequent neurophysical development assessed at 22 months of age was satisfactory.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
5/42. Successful use of nasal-CPAP for obstructive sleep apnea in Hunter syndrome with diffuse airway involvement.A patient with Hunter syndrome and diffuse airway obstruction had daytime hypersomnolence, snoring, and alveolar hypoventilation. polysomnography showed severe obstructive sleep apnea. In the past, all reported cases of sleep apnea in patients with mucopolysaccharidoses had been treated with tonsillectomy/adenoidectomy or tracheostomy. This patient, in whom tracheostomy would have been very difficult due to the diffuse nature of his airway involvement, was successfully treated with high pressure nasal CPAP and supplemental oxygen.- - - - - - - - - - ranking = 0.2keywords = alveolar (Clic here for more details about this article) |
6/42. Anaesthesia for a patient with central alveolar hypoventilation syndrome (Ondine's Curse).The perioperative anaesthetic management of an adult patient with central alveolar hypoventilation syndrome (CAHS), Ondine's Curse, is described for anterior resection of a carcinoma of the bowel. This rare syndrome results in alveolar hypoventilation, hypercarbia, hypoxaemia with secondary polycythaemia, pulmonary artery hypertension, and cor pulmonale. Epidural morphine was used for postoperative analgesia in an attempt to improve postoperative respiratory function. However, postoperative mechanical ventilation was required until recovery of the respiratory drive, which was ablated by anaesthetic drugs, epidural morphine and high inspired oxygen concentrations. The pathophysiology and treatment of this syndrome are reviewed.- - - - - - - - - - ranking = 1.2keywords = alveolar (Clic here for more details about this article) |
7/42. Changes in cerebrospinal fluid homovanillic acid in children with Ondine's curse.The cerebrospinal fluid (CSF) concentrations of three acid monoamine metabolites, two purines, and a group of amino acids were determined in two children with chronic central alveolar hypoventilation (Ondine's curse). The levels of all assayed neuroactive substances, metabolites, and amino acids, with one exception, were normal compared to an age-matched group of neurologically healthy children. The levels of the dopamine metabolite homovanillic acid in the children with Ondine's curse were approximately 2.4 times higher than expected for age range. The present findings may indicate a link between central nervous system dopamine activity and chronic central alveolar hypoventilation. Among other possible explanations, the changes seen might represent a primary alteration in dopamine activity or may reflect a change in dopamine turnover resulting from the chronic hypoventilation.- - - - - - - - - - ranking = 0.4keywords = alveolar (Clic here for more details about this article) |
8/42. Central alveolar hypoventilation and sleep. Treatment by intermittent positive-pressure ventilation through nasal mask in an adult.Idiopathic central alveolar hypoventilation, uncommonly seen in middle-aged adults, has often been treated by tracheostomy and assisted ventilation during sleep or by implantation of a diaphragmatic pacemaker with or without tracheostomy. We report the successful treatment of a middle-aged man by the easy application of intermittent positive-pressure ventilation through a nose mask.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
9/42. recurrence of sleep apnea syndrome following tracheostomy. A shift from obstructive to central apnea.This report describes an unusual case of severe obstructive sleep apnea and alveolar hypoventilation leading to hypersomnolence and cor pulmonale, which were corrected by tracheostomy. Four years later, after a 22.5-kg weight gain, nocturnal apneas of similar frequency, duration, and depth of desaturation reappeared but were totally central in origin. The central apneas were eliminated with home nocturnal positive-pressure ventilation via cuffed tracheostomy tube. Each time the patient's apneas were corrected (obstructive: tracheostomy; central: mechanical ventilation), daytime alveolar hypoventilation disappeared rapidly. Yearly right heart catheterizations and radionuclide ejection fractions documented pulmonary hypertension and right heart failure, with resolution following tracheostomy and recurrence after appearance of central apneas. The changes in hemodynamic status corresponded to the patient's weight, presence of apnea, daytime alveolar hypoventilation, and treatment of nocturnal oxyhemoglobin desaturation. This case illustrates the theory of a common etiology of both central and obstructive apnea through abnormal respiratory controller gain and points to several roles obesity may play in apnea.- - - - - - - - - - ranking = 0.6keywords = alveolar (Clic here for more details about this article) |
10/42. Pathophysiological findings in a patient with Shy-Drager and alveolar hypoventilation syndromes.This report describes the clinical, physiologic and pathologic findings in a patient with alveolar hypoventilation, acquired autonomic dysfunction, parkinsonism, and sensorimotor polyneuropathy. While there were pathologic findings in both central and peripheral nervous systems, there were no morphologic changes in the regions of the brainstem considered responsible for respiratory rhythmogenesis. A hypothesis is developed based on the assumption that the abnormalities in respiratory control which led to the patient's death are explained by abnormalities in the feedback control of respiration.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
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