Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

General anesthesia in patients with mediastinal masses can lead to life-threatening cardiorespiratory complications. We report the cases of 2 pediatric patients with mediastinal masses who developed serious complications during general anesthesia. The first was a 13-year-old boy with a suspected diagnosis of high-grade T-cell non-Hodgkin lymphoma, lymph node disease, and an anterior mediastinal mass. He developed negative pressure pulmonary edema secondary to severe upper airway obstruction in spontaneous ventilation. The second was a 14-year-old boy with Rosai-Dorfman disease and paratracheal lymph node involvement. He developed severe airway obstruction in the early postoperative period. The anesthetic difficulties that arise in these cases include acute airway occlusion, superior vena cava syndrome, pulmonary artery or cardiac compression, acute pulmonary edema, and cardiopulmonary collapse. The technique of choice is induction with inhaled anesthetics and maintenance of spontaneous ventilation. Neuromuscular relaxants are avoided.
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PMID:[Perioperative respiratory complications in pediatric patients with mediastinal masses: a report of 2 cases]. 1730 81

A 19-year-old woman presented with a history of severe lower backache and asymmetric proximal lower limb weakness during the past 3 months. In addition, she also suffered from lower motor neuron-type bladder and bowel symptoms. On examination, paraparesis was noted. Further, sensory examination suggested patchy asymmetric sensory loss in both lower limbs with saddle anaesthesia and areflexia. A clinical diagnosis of Conus-Cauda syndrome was made and contrast-enhanced MRI of the lumbar and sacral spine was done, which confirmed the presence of a mass lesion within the spinal canal involving the cauda equina extending up to the sacral level. She underwent partial resection of the lesion following which the neurological deficits and lower backache resolved. Histopathological evaluation and immunohistochemical analyses uncovered Rosai-Dorfman disease. There was no evidence of disease elsewhere in the body. Since the patient improved significantly following surgery and exhibited no further neurological worsening, she remains under close follow-up.
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PMID:A quintessential syndrome with a rare marvelling aetiology: Rosai-Dorfman disease presenting as Conus-Cauda syndrome. 2945 79