Children with mucopolysaccharidosis: Perioperative care, morbidity, mortality, and new findings
| Autor: | Joseph J. Buckley, Kumar G. Belani, Elizabeth A. Braunlin, C. Gail Summers, Becky L.M. Carpenter, William Krivit, Thomas F. Floyd, Chester B. Whitley, Samuel C. Levine, Arnold S. Leonard, Ji Chia Liao |
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| Rok vydání: | 1993 |
| Předmět: |
Adult
Anesthesia Endotracheal medicine.medical_specialty Adolescent Mucopolysaccharidosis Laryngoscopy Coronary artery disease Tracheostomy Preoperative Care Humans Medicine Child Intraoperative Complications Hurler syndrome Stroke Bone Marrow Transplantation Retrospective Studies Subluxation medicine.diagnostic_test business.industry Infant General Medicine Mucopolysaccharidoses Airway obstruction medicine.disease Surgery Airway Obstruction Maroteaux–Lamy syndrome Child Preschool Anesthesia Pediatrics Perinatology and Child Health Anesthesia Intravenous Morbidity business |
| Zdroj: | Journal of Pediatric Surgery. 28:403-410 |
| ISSN: | 0022-3468 |
| DOI: | 10.1016/0022-3468(93)90240-l |
| Popis: | The perioperative care, morbidity, and mortality in 30 patients with mucopolysaccharidosis (MPS) are presented. They underwent a detailed preoperative assessment and were anesthetized 141 times. An intravenous induction technique was used in most patients. It was easier to see the vocal cords, during laryngoscopy, in children with Hurler syndrome (HS) when they were younger (23 v 41 months, P ≤ .01) and smaller (12 v 15 kg, P ≤ .05). Preoperative obstructive breathing was associated with a significantly higher incidence of postextubation obstruction ( P ≤ .05). A total of 28 children underwent bone marrow transplantation (BMT); this reversed upper airway obstruction and also reversed intracranial hypertension. In children with HS, the incidence of odontoid dysplasia was 94%; 38% demonstrated anterior C 1 C 2 subluxation. Head and neck manipulation was limited in children with cervical spine defects. None of the 30 patients experienced spinal cord morbidity. One child suffered an intraoperative stroke; another, pulmonary edema. Severe and extensive coronary obstruction was responsible for 2 intraoperative deaths. Coronary angiography underestimated coronary artery disease. |
| Databáze: | OpenAIRE |
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