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Children with mucopolysaccharidosis: perioperative care, morbidity, mortality, and new findings
K G Belani1, W Krivit, B L Carpenter
1Department of Anesthesiology, University of Minnesota, Minneapolis.
Insights
Perioperative care for mucopolysaccharidosis (MPS) patients requires careful assessment. Bone marrow transplantation (BMT) can improve airway obstruction and intracranial hypertension in Hurler syndrome (HS).
Area of Science:
- Anesthesiology
- Pediatric Medicine
- Genetics
Background:
- Mucopolysaccharidosis (MPS) encompasses a group of rare genetic disorders.
- Patients with MPS often present complex anesthetic challenges due to multisystem involvement.
- Limited data exists on perioperative outcomes and specific anesthetic considerations for MPS patients.
Purpose of the Study:
- To describe the perioperative care, morbidity, and mortality in a cohort of 30 patients with mucopolysaccharidosis (MPS).
- To evaluate anesthetic techniques and outcomes in MPS patients undergoing various procedures.
- To assess the impact of bone marrow transplantation (BMT) on airway obstruction and intracranial hypertension in Hurler syndrome (HS).
Main Methods:
- Retrospective review of 30 MPS patients undergoing 141 anesthetics.
- Detailed preoperative assessment including airway evaluation.
- Analysis of anesthetic techniques, intraoperative events, and postoperative outcomes.
- Assessment of BMT effects on airway and intracranial pressure.
Main Results:
- Intravenous induction was the predominant anesthetic technique.
- Easier laryngoscopy in younger and smaller children with Hurler syndrome (HS).
- Preoperative obstructive breathing correlated with postextubation obstruction.
- BMT reversed upper airway obstruction and intracranial hypertension in 28 children.
- High incidence of odontoid dysplasia (94%) and C1-C2 subluxation (38%) in HS patients.
- Limited head/neck manipulation due to cervical spine defects.
- No spinal cord morbidity observed.
- Two intraoperative deaths due to severe coronary obstruction; angiography underestimated disease.
Conclusions:
- Perioperative management of MPS patients is complex and requires individualized care.
- Airway management is a critical concern, with specific considerations for Hurler syndrome.
- Bone marrow transplantation offers significant benefits for airway and intracranial hypertension in HS.
- Cervical spine instability is common and impacts anesthetic procedures.
- Coronary artery disease is a significant risk factor for mortality, potentially underestimated by angiography.
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 < or = .01) and smaller (12 v 15 kg, P < or = .05). Preoperative obstructive breathing was associated with a significantly higher incidence of postextubation obstruction (P < or = .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 C1-C2 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.
