Cases reported "Mucopolysaccharidosis II"

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1/15. Failure of the laryngeal mask to secure the airway in a patient with Hunter's syndrome (mucopolysaccharidosis type II).

    We present a case-study of a boy with Hunter's syndrome (mucopolysaccharidosis type II) and stridor in which a laryngeal mask airway (LMA) failed to secure airway control. A rigid bronchoscopy was performed and a polypoid formation discovered. We believe that the use of the LMA could explain the laryngeal obstruction in this child.
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ranking = 1
keywords = airway, obstruction
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2/15. Children with mucopolysaccharidoses--three cases report.

    The mucopolysaccharidoses (MPS) are a group of inherited disorders of metabolism, with widespread, progressive involvement and derangement of many organs and tissues. Because of their disabling nature, frequent surgical intervention for the abnormality entailed is common, and is associated with a high degree of anesthetic risks perioperatively. One of the major hazards which we find clinically is airway difficulty. Multiple factors are present in the mucopolysaccharidoses to make airway management and trachael intubation potentially hazardous. Aside from generalized infiltration and thickening of the soft tissues, the oropharynx may be obstructed by a large tongue with tonsillar hypertrophy. Also, the friable mucosa covering the nasal and oral pharynx renders these structures easily to bleed and edematous. The neck is typically short and immobile, and the cervical spine and tempromandibular joint may have a limited range of movement. From our experience, we have learned not to overlook the propensity of airway difficulty. The uniqueness of their anatomy and extremely sensitive airway often result in failed intubation and bronchospasm even after successful intubation. Recently, in Mackay Memorial Hospital we have encountered in series three pediatric cases with mucopolysaccharidoses (one Hurler and two Hunter syndromes). In this report we would like to share our experiences and to discuss the anesthetic risks and management of the MPS patients.
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ranking = 0.65122513788692
keywords = airway
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3/15. Hunter's syndrome and associated sleep apnoea cured by CPAP and surgery.

    A 42-yr-old male with Hunter's syndrome presented with severe obstructive sleep apnoea syndrome (OSAS) and daytime respiratory failure. continuous positive airway pressure (CPAP) therapy was initially ineffective and produced acute respiratory distress. Extensive Hunter's disease infiltration of the upper airway with a myxoma was confirmed. Following surgery to remove the myxoma at the level of the vocal cords, CPAP therapy was highly effective and well tolerated. This report demonstrates the necessity of evaluating fully the upper airway in patients with unusual variants of OSAS, particularly where the disease is not adequately controlled by CPAP.
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ranking = 0.48841885341519
keywords = airway
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4/15. Bladder obstruction in Hunter's syndrome.

    We report a case of bladder obstruction in a patient with Hunter's syndrome, presenting with acute painful symptomatology, due to the impossibility of voiding, which was diagnosed with ultrasonography and cystometrography. Intermittent catheterization with intravesical oxybutynin chloride lead to successful functional resolution of the obstruction.
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ranking = 0.13897375901775
keywords = obstruction
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5/15. anesthesia of CO2 laser surgery in a patient with Hunter syndrome: case report.

    Hunter syndrome (mucopolysaccharidosis, type II; MPS II) is one of a heterogeneous group of recessively inherited mucopolysaccharide storage diseases. patients with mucopolysaccharidosis show progressive involvement and derangement of many organs, especially upper airway anomalies, which are the major cause of perioperative death. In recent years, a CO2 laser is often applied to upper airway lesions. A 16-year-old patient suffering from Hunter syndrome was scheduled for CO2 laser surgery because of sleep apnea and respiratory stridor. Otolaryngological examination revealed bulging of the bilateral false cord with stenosis of the glottis. We adopted sevoflurane mask induction and high-frequency jet ventilation to overcome the perioperative airway problems. The anesthetic course was uneventful, and the patient was discharged 2 days after the operation.
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ranking = 0.48841885341519
keywords = airway
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6/15. Tracheobronchial stent insertions in the management of major airway obstruction in a patient with Hunter syndrome (type-II mucopolysaccharidosis).

    We report a case of a 22-year-old male with Hunter syndrome who developed progressive major airway obstruction and was treated with insertion of plastic and metallic stents, with dramatic improvement in the patient's symptomatic and functional status. To the best of our knowledge, this is the first reported case of endoluminal stents being used in the management of major airway obstruction in a patient with Hunter syndrome.
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ranking = 7.9577762126232
keywords = airway obstruction, airway, obstruction
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7/15. 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.
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ranking = 2.1403274577959
keywords = airway obstruction, airway, obstruction
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8/15. Upper airway obstruction in Hunter syndrome.

    patients with Hunter syndrome may have symptoms of hoarseness, stridor and breathing difficulties as a result of laryngeal and tracheal involvement. In planning their evaluation, we must prefer non-invasive methods as X-ray or CT scan, and avoid doing endotracheal intubation or bronchoscopies. review of adult cases in the literature and description of the only case of a child with Hunter syndrome having life-threatening complications of his upper airways is discussed in this report. In this case and in the literature we cannot exclude intubation or bronchoscopy as a serious aggravating factor, causing further narrowing of the larynx.
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ranking = 5.4679904262206
keywords = airway obstruction, airway, obstruction
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9/15. Hunter's syndrome: a study in airway obstruction.

    Hunter's disease is a genetically transmitted defect known to produce mucopolysaccharide infiltration of multiple organ systems. Upper airway obstruction is caused by an enlarged tongue, deformed pharynx, and short, thick neck. Its eventual lethal outcome by the second decade of life is known to result from an infiltrative cardiomyopathy leading to irreversible heart failure. Instead, our recent experience in the care of five patients with this disorder suggests the lethal event is related to progressive obstruction sequentially involving the upper, mid, and lower airway characterized by gradual deformation and collapse of the trachea. autopsy and histopathologic whole organ sections demonstrate anteroposterior flattening of the trachea and bronchi with submucosal thickening producing structural alterations known only to this disease.
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ranking = 6.8174487548274
keywords = airway obstruction, airway, obstruction
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10/15. airway obstruction and sleep apnea in Hurler and Hunter syndromes.

    The Hurler and Hunter syndromes are two forms of mucopolysaccharidosis. Although the diseases are rare, those afflicted commonly require otolaryngologic consultation. Upper airway obstruction is often severe, progressive, and not infrequently the suspected cause of death in these patients. Four patients with these problems are presented. In all of the children, obstructive sleep apnea was a major management problem. This and other upper airway difficulties are detailed with clinical and pathological correlates.
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ranking = 1.5817514925874
keywords = airway obstruction, airway, obstruction
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