Background
Methods
Results
Preoperative clinical characteristics
Clinical presentation | ||
---|---|---|
Pt | Neurologic/Muscular | Other |
GSD type 0 | ||
1 | Muscle weakness | OSA; hypertrophic cardiomyopathy; hypertension |
GSD type I (von Gierke) | ||
2 | Muscle weakness; gait abnormalities | Lactic acidosis; hypoglycemia; hepatic adenoma |
3 | None reported | Hypoglycemia |
4 | None reported | Hypertension; hypoglycemia; lactic acidosis |
5 | Seizures | Hyperuricemia; lactic acidosis; hypoglycemia |
6 | Developmental delay; velopharyngeal insufficiency | Hypoglycemia |
7 | Fatigue, muscle weakness | Hypoglycemia |
GSD type II (Pompe) | ||
8 | Severe scoliosis; muscle weakness; wheelchair-bound | Respiratory failure (BiPAP); restricted lung disease |
9 | Muscle weakness; hypotonia | None reported |
10 | Muscle weakness | Respiratory failure (BiPAP); pulmonary hypertension; CHF; dilated cardiomyopathy; atrial fibrillation |
11 | Muscle weakness | None reported |
12 | Muscle weakness | CHF; dilated cardiomyopathy |
13 | Muscle weakness | Respiratory failure (BiPAP) |
14 | Muscle weakness | Dyspnea; hypertension |
15 | None reported | Hypertrophic cardiomyopathy; ventricular tachycardia |
GSD type IV (Andersen) | ||
16 | Developmental delay | Hepatosplenomegaly |
GSD type V (McArdle) | ||
17 | Muscle weakness; cervical radiculopathy | None reported |
18 | Muscle stiffness; increased serum creatine kinase | None reported |
19 | Muscle weakness | None reported |
20 | Muscle weakness | None reported |
21 | Muscle cramps; exercise intolerance | None reported |
22 | Exercise intolerance | None reported |
23 | Muscle weakness | None reported |
24 | Muscle weakness and stiffness | None reported |
25 | Muscle weakness | None reported |
26 | Episodes of rhabdomyolysis; exercise intolerance | None reported |
27 | Muscle weakness | Hypertension |
GSD type VI (Hers) | ||
28 | None reported | Lactic acidosis |
GSD type VII (Tarui) | ||
29 | Muscle weakness | None reported |
Unidentified, likely glycolytic defect | ||
30 | Proximal muscle myopathy | Hypoglycemia |
Anesthesia course and Intraoperative fluid administration
Pt | Agea at Surgery, y | Surgery/Anesthesia duration, min/Airway/Anesthetic Agents | Fluid used | Disposition |
---|---|---|---|---|
GSD type 0 | ||||
1 | 51–60 | Muscle biopsy/120,P | LR | Outpatient |
GSD type I (Von Gierke) | ||||
2 | 19–30 | Liver transplant/360/P, C, S, Iso | NS; D5W; PRBC; Cryo; Albumin | ICU |
3 | 11–18 | Liver transplant/300/P, C, Iso | NS; D5W; Albumin | ICU |
4 | 6–10 | Liver transplant/330/P, V, Iso | NS; D5NS; PRBC; Albumin | ICU |
5 | 1–2 | Muscle biopsy/120/Sev, N2O | D5NS | Outpatient |
1–2 | Gastric tube placement/60/P, At, Iso | D10NS | Ward | |
6 | <1 | Gastric tube placement/100/P, At, Sev | LR; D10NS | Ward |
3–5 | Tonsillectomy/110/P, V | LR; D10NS | Ward | |
3–5 | Myringotomy/ 100/P | LR; D10NS | Ward | |
7 | 11–18 | Rectal abscess I&D/70/At, STP, N2O | D10W | Outpatient |
11–18 | Abscess I&D/120/P, At, N2O | LR; D10W | Outpatient | |
GSD type II (Pompe) | ||||
8 | 11–18 | Anterior spinal fusion/300/STP, Iso, Sev, P | LR; PRBC; FFP; Platelets; Hetastarch; Albumin | ICU |
11–18 | Posterior spinal fusion/730/P, Sev, N2O | LR | ICU | |
9 | <1 | Muscle biopsy/75/P, N2O | LR | Outpatient |
10 | 41–50 | Muscle biopsy/MAC/60, P | LR; NS | Ward |
41–50 | Tracheostomy/80/E, S, Sev | LR | ICU | |
11 | 51–60 | Muscle biopsy/MAC/90 mask, P | NS | Outpatient |
12 | 31–40 | Myocardial biopsy/MAC/90, Mz, AL | LR | Outpatient |
31–40 | Parathyroidectomy/120/E, V, Iso | LR | Ward | |
13 | 41–50 | Muscle biopsy/MAC/60,P | LR | ICU |
14 | 41–50 | Muscle biopsy/MAC/120/ Mask, P | NS; LR; Albumin | Outpatient |
15 | 3–5 | Myectomy (ICD)/ CPB/280,Sev, V, Iso | LR | ICU |
3–5 | Sternal wire removal/180/E, RO, Sev | Ward | ||
Glycogen storage disease type IV (Andersen) | ||||
16 | 1–2 | Liver biopsy/MAC/60/ Mask, Sev | LR | Outpatient |
1–2 | Myringotomy/GA/60/Sev, N2O | None | Outpatient | |
17 | 61–70 | Cervical discectomy/200, P, RO | Ward | |
Glycogen storage disease type V (McArdle) | ||||
18 | 11–18 | Muscle biopsy/60)/ LMA/Sev, N2O | LR | Outpatient |
19 | 6–10 | Muscle biopsy/60/P, Sev, N2O | LR | Outpatient |
20 | 41–50 | Whipple /GA /360/P, S, V, Iso | LR | Ward |
21 | 41–50 | Muscle biopsy/MAC/60/ Mask/P | LR | Outpatient |
22 | 11–18 | Muscle biopsy/MAC/90/ Mask/P, Sev | LR | Outpatient |
23 | 19–30 | Muscle biopsy/ MAC/105/ Mask, P | LR | Outpatient |
24 | 19–30 | Muscle biopsy/ MAC/60/ Mask, P | LR | Outpatient |
25 | 6–10 | Muscle biopsy/70/ LMA, P | LR | Outpatient |
26 | 19–30 | Muscle biopsy/ MAC/120/ Mask, P | LR | Outpatient |
27 | >70 | Muscle biopsy/MAC/60/ Mask, P | LR | Outpatient |
GSD type VI (Hers) | ||||
28 | 1–2 | Liver biopsy/105/Sev, RO | D5NS | Outpatient |
GSD type VII (Tarui) | ||||
29 | 61–70 | Muscle biopsy/MAC/210/Mask, P | LR | Outpatient |
Unidentified, likely glycolytic defect | ||||
30 | 31–40 | Dilation & curettage/60/LMA/P, N2O | LR | Outpatient |
31–40 | Mammoplasty/240/P, V, N2O | LR | Ward | |
31–40 | Breast hematoma evacuation/90/LMA/P, N2O | LR | Ward |