Children with mucopolysaccharidosis: Perioperative care, morbidity, mortality, and new findings

Kumar G. Belani; William Krivit; Becky L.M. Carpenter; Elizabeth Braunlin; Joseph J. Buckley; Ji Chia Liao; Thomas Floyd; Arnold S. Leonard; C. Gail Summers; Samuel Levine; Chester B. Whitley

doi:10.1016/0022-3468(93)90240-L

Children with mucopolysaccharidosis: Perioperative care, morbidity, mortality, and new findings

Kumar G. Belani, William Krivit, Becky L.M. Carpenter, Elizabeth Braunlin, Joseph J. Buckley, Ji Chia Liao, Thomas Floyd, Arnold S. Leonard, C. Gail Summers, Samuel Levine, Chester B. Whitley

Research output: Contribution to journal › Article › peer-review

96 Scopus citations

Abstract

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 C₁C₂ 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.

Original language	English (US)
Pages (from-to)	403-410
Number of pages	8
Journal	Journal of Pediatric Surgery
Volume	28
Issue number	3
DOIs	https://doi.org/10.1016/0022-3468(93)90240-L
State	Published - Mar 1993

Bibliographical note

Funding Information:
From the Departments of Anesthesiology, Pediatrics, Radiology. Otolaryngologv, Ophthalmology, and Snrgev, Universiy of Minnesota, Minneapolis, MN. Presented at the 23rd Annual Meeting of the American Pediattic SurgicalAssociation, Colorado Springs7 May 13-16, 1992. Suppotted in part by NIHININDS grant numbers ROl NS 29099-01A and ROI DK-39891, and the Anesthesia Research Fund through the Minnesota Medical Foundation. Address reprint requests to Kumar G. Belani, MBBS. MS, UMHC Box 386, Department of Anesthesiology, University of Minnesota Medical School, 420 Delaware St SE, Minneapolis, MN 554.55. Copyright 0 1993 by W.B. Saunders Company 0022-346819312803-0022$03.00/O

Keywords

Hunter syndrome
Hurler syndrome
Maroteaux-Lamy syndrome
Morquio syndrome
Mucopolysaccharidosis
Sanfilippo syndrome
bone marrow transplantation
coronary artery disease
hydrocephalus
obstructive apnea
odontoid dysplasia

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Children with mucopolysaccharidosis : Perioperative care, morbidity, mortality, and new findings. / Belani, Kumar G.; Krivit, William; Carpenter, Becky L.M.; Braunlin, Elizabeth; Buckley, Joseph J.; Liao, Ji Chia; Floyd, Thomas; Leonard, Arnold S.; Gail Summers, C.; Levine, Samuel; Whitley, Chester B.

In: Journal of Pediatric Surgery, Vol. 28, No. 3, 03.1993, p. 403-410.

Research output: Contribution to journal › Article › peer-review

Belani, Kumar G. ; Krivit, William ; Carpenter, Becky L.M. ; Braunlin, Elizabeth ; Buckley, Joseph J. ; Liao, Ji Chia ; Floyd, Thomas ; Leonard, Arnold S. ; Gail Summers, C. ; Levine, Samuel ; Whitley, Chester B. / Children with mucopolysaccharidosis : Perioperative care, morbidity, mortality, and new findings. In: Journal of Pediatric Surgery. 1993 ; Vol. 28, No. 3. pp. 403-410.

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abstract = "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 C1C2 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.",

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AU - Belani, Kumar G.

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AU - Braunlin, Elizabeth

AU - Buckley, Joseph J.

AU - Liao, Ji Chia

AU - Floyd, Thomas

AU - Leonard, Arnold S.

AU - Gail Summers, C.

AU - Levine, Samuel

AU - Whitley, Chester B.

N1 - Funding Information: From the Departments of Anesthesiology, Pediatrics, Radiology. Otolaryngologv, Ophthalmology, and Snrgev, Universiy of Minnesota, Minneapolis, MN. Presented at the 23rd Annual Meeting of the American Pediattic SurgicalAssociation, Colorado Springs7 May 13-16, 1992. Suppotted in part by NIHININDS grant numbers ROl NS 29099-01A and ROI DK-39891, and the Anesthesia Research Fund through the Minnesota Medical Foundation. Address reprint requests to Kumar G. Belani, MBBS. MS, UMHC Box 386, Department of Anesthesiology, University of Minnesota Medical School, 420 Delaware St SE, Minneapolis, MN 554.55. Copyright 0 1993 by W.B. Saunders Company 0022-346819312803-0022$03.00/O

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N2 - 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 C1C2 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.

AB - 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 C1C2 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.

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KW - Hurler syndrome

KW - Maroteaux-Lamy syndrome

KW - Morquio syndrome

KW - Mucopolysaccharidosis

KW - Sanfilippo syndrome

KW - bone marrow transplantation

KW - coronary artery disease

KW - hydrocephalus

KW - obstructive apnea

KW - odontoid dysplasia

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EP - 410

JO - Journal of Pediatric Surgery

JF - Journal of Pediatric Surgery

SN - 0022-3468

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ER -

Children with mucopolysaccharidosis: Perioperative care, morbidity, mortality, and new findings

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Bibliographical note

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