1/27. Late-onset central hypoventilation with hypothalamic dysfunction: a distinct clinical syndrome.Idiopathic central hypoventilation has occasionally been reported in previously well children after infancy. The relationship between this late-onset central hypoventilation syndrome (LO-CHS) and congenital central hypoventilation syndrome (CCHS) has not been established. Both CCHS and LO-CHS have been associated with neural crest tumors, such as ganglioneuroblastoma and ganglioneuroma, and they generally occur in the presence of a histologically normal central nervous system. At least 10 case reports of idiopathic LO-CHS featured evidence of hypothalamic dysfunction (HD), including hyperphagia, hypersomnolence, thermal dysregulation, emotional lability, and endocrinopathies. We report on a case of LO-CHS/HD successfully treated by nasal intermittent positive pressure ventilation (NIPPV). Despite the commonalties with CCHS, we propose that LO-CHS/HD is a distinct clinical syndrome. In addition to the markedly different age at presentation, features of hypothalamic dysfunction are not seen in CCHS. review of the literature was undertaken to further clarify the full spectrum of the disease.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/27. Unexpectedly severe sleep and respiratory pathology in patients with amyotrophic lateral sclerosis.Daytime fatigue and sleep disturbance are frequent complaints in patients with amyotrophic lateral sclerosis (ALS). However, polysomnographic data are sparse. Nocturnal respiratory insufficiency may occur despite nearly normal daytime pulmonary function. We describe the clinical presentation and polysomnographic findings in two patients with clinically and electrophysiologically confirmed ALS with minimal weakness but excessive daytime sleepiness. polysomnography in the first patient showed a respiratory disturbance index of 43.5, and profound oxygen desaturations to 62%. The second patient had prolonged periods of hypoventilation, with oxygen saturations oscillating between 86 and 83%. Both patients showed severe sleep maintenance insomnia with a sleep efficiency < 40% and frequent arousals while asleep. Application of continuous positive airway pressure (CPAP) restored normal nocturnal ventilation, blood oxygenation and sleep parameters in the first patient; compliance, however, was poor. The second patient was unable to tolerate CPAP. We conclude that ALS patients with excessive daytime sleepiness or insomnia should undergo polysomnography to adequately diagnose nocturnal respiratory insufficiency and sleep disturbance. compliance with treatment, however, may be poor.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/27. Malignant hyperinflation of the nondependent lung during chest surgery.Unilateral malignant hyperinflation of the lungs during positive pressure mechanical ventilation was described during aggressive respiratory therapy of unilateral lung disease or in situations of significant difference in compliance between the two lungs. We report a case of malignant hyperinflation of the nondependent lung during chest surgery. The differential diagnosis and treatment with differential lung ventilation are described.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
4/27. tetany following resuscitation after abruptio placentae.BACKGROUND: serum ionized calcium and magnesium are normally decreased during later stages of pregnancy. A further rapid decline may be caused by the rapid infusion of blood bank products in which citrate is used as an anticoagulant/preservative. tetany, as reported here, may be precipitated by such infusions.CASE: A gravid woman presented in hemorrhagic shock due to abruptio placentae. Rapid infusion of packed red blood cells and fresh frozen plasma precipitated signs of tetany, muscle rigidity, posturing, high airway pressure during mechanical ventilation, etc. Ionized calcium and magnesium blood levels were very low (0.58 mmol/L and 1.0 mg/dL, respectively), but responded to rapid electrolyte administration.CONCLUSION: Binding of calcium and magnesium by citrate may lead to hypo-ionized calcemic and hypomagnesemic tetany after rapid replacement of blood products in the pregnant patient. This consequence is worsened when extreme alkalemia due to respiratory or metabolic causes is also present.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/27. Periodic apnea, exercise hypoventilation, and hypothalamic dysfunction.Periodic apnea and exercise hypoventilation were observed in a 14-year-old boy. hyperphagia, obesity, serum hyperosmolality without diabetes insipidus or appropriate thirst, and retardation of growth and sexual development indicated a hypothalamic disorder. Neurologic evaluation was normal except for electroencephalographic changes induced by apnea. Pulmonary function tests, resting arterial blood gases in the wakeful state, and ventilatory response to inhaled CO2 were also normal. Acute hypoxemia and respiratory acidosis occurred with apnea during sleep and with insufficient ventilation during exercise. The central origin of sleep apneas was shown by esophageal pressure monitoring. The hypothalamic dysfunction and exercise hypoventilation distinguish this patient from others with obesity and periodic apnea.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/27. Noninvasive ventilatory strategies in the management of a newborn infant and three children with congenital central hypoventilation syndrome.Four children with congenital central hypoventilation syndrome (CCHS) treated with noninvasive techniques of ventilation are presented. Two infants (one in the newborn period) were treated with nasal mask bilevel positive airway pressure (BiPAP), and then both were transitioned to negative pressure chamber ventilation at several years of age because of possible midface hypoplasia. Tracheostomies were not performed. Two older children were transitioned from mechanical ventilation via tracheostomy to nasal mask BiPAP, and then in one case to negative pressure chamber ventilation, and in the other to phrenic nerve pacing. Their tracheostomies were removed.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
7/27. Hindbrain-hernia-related syringomyelia without syringobulbia, complicated by permanent nocturnal central hypoventilation requiring non-invasive ventilation.INTRODUCTION: In the absence of syringobulbia, hindbrain hernia does not commonly cause permanent respiratory complications. We present two patients who developed permanent central nocturnal hypoventilation following acute deterioration of hindbrain-hernia-related syringomyelia despite successful surgery. patients: Two children (one boy and one girl, aged 7 and 13 years, respectively) presented with acute neurological deterioration. The boy presented with a 6-week history of progressive tiredness and weakness and a short history of respiratory depression. The girl presented with a 2-year history of left hemiparesis and a short history of left hemiplegia and respiratory depression. On magnetic resonance scan, both had hindbrain hernia, hydrocephalus and cervical syringomyelia. Following cranio-vertebral decompression and, later, a ventriculoperitoneal shunt, the syringomyelia remained well controlled in both patients. In contrast, their respiratory depression improved minimally. Repeated multichannel respiratory monitoring revealed a persistently slow sleeping respiratory rate, with nocturnal hypercapnia and hypoxemia, managed successfully by night-time non-invasive ventilation. Neither the boy nor the girl, after 7 and 4 years, respectively, has developed any complications of nocturnal hypoventilation. When awake, both breathe normally. RESULT: Both children appear to have suffered a permanent impairment of respiratory control, resulting in nocturnal hypoventilation. This is usually seen with brain stem lesions and is unexpected when syringomyelia does not extend above C2, in the absence of syringobulbia. DISCUSSION: Central nocturnal hypoventilation is postulated to be due to permanent damage of central brain stem pathways controlling the respiratory muscles, due to local pressure at the cranio-cervical junction associated with acute deterioration of the hindbrain herniation. CONCLUSION: Sudden deterioration of hindbrain hernia and cervical syringomyelia can rarely impair the central mechanisms of respiratory control, with long-term implications on quality of life.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/27. Non-invasive ventilation corrects alveolar hypoventilation during spinal anesthesia.PURPOSE: To document and explain the beneficial effects of non-invasive ventilation in correcting hypoxemia and hypoventilation in severe chronic obstructive pulmonary disease, during spinal anesthesia in the lithotomy position. CLINICAL FEATURES: A morbidly obese patient with severe chronic obstructive pulmonary disease underwent prostate surgery in the lithotomy position under spinal anesthesia. Hypoxemia was encountered during surgery, and a profound decrease of forced vital capacity associated with alveolar hypoventilation and ventilation/perfusion mismatching were observed. In the operating room, an M-mode sonographic study of the right diaphragm was performed, which confirmed that after spinal anesthesia and assuming the lithotomy position, there was a large decrease (-30%) in diaphragmatic excursion. Hypoxemia and alveolar hypoventilation were successfully treated with non-invasive positive pressure ventilation. CONCLUSIONS: Intraoperative application of non-invasive positive pressure ventilation improved diaphragmatic excursion and overall respiratory function, and reduced clinical discomfort in this patient.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
9/27. Chronic hypoventilation syndrome: treatment with non-invasive mechanical ventilation.Chronic hypoventilation syndrome can be caused by many disease states, although it is more commonly seen in neuromuscular disorders. Assessment of hypoventilation includes measurement of carbon dioxide level, respiratory muscle strength, pulmonary function testing, and any other system involved, such as cardiac dysfunction or sleep abnormalities. Often, chronic hypoventilation is initially diagnosed during an episode of acute respiratory failure. The use of noninvasive ventilation with positive pressure, negative pressure, or gravitational devices can be an effective treatment option for some patients, thus obviating the need for a tracheostomy. Noninvasive ventilatory equipment such as the nasal or oral masks, mouthpieces, bi-level positive airway pressure, chest cuirasses, ponchos, or the iron lung, and the rocking bed or pneumobelt can each ventilate a certain type of patient adequately. Each has specific indications, advantages, and disadvantages and must be individualized to the patient's needs.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
10/27. Blunted respiratory drive in congenital myopathy.Two patients with clinically mild congenital myopathies presented with chronic respiratory failure. muscle weakness alone could not account for the respiratory insufficiency since static respiratory pressures were not markedly impaired, ventilation during exercise was normal, and daytime ventilation was normal if ventilatory assistance was provided at night. The ventilatory responses to inhaled carbon dioxide were very low, suggesting that impairment of the central nervous respiratory chemoreceptor contributed to hypoventilation. These patients and others described in the literature suggest that central depression of ventilation may occur more frequently than previously recognized in patients with muscular disorders. patients with chronic respiratory failure due to central depression of respiratory drive can be effectively managed by assisted ventilation at night.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
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