Prevalence of unsuspected myopathy in infants presenting for clubfoot surgery

Zanette, Gastone; Manani, G; Pittoni, G; Angelini, C.; Trevisan, Carlo P.; Turra, S

doi:10.1111/j.1460-9592.1995.tb00272.x

Cited by 7 publications

(4 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The anesthesiologist's concern for MHS may have a straightforward solution: avoidance of trigger substances in particular high‐risk patients such as the orthopedic patient suspected of having CCD. The unexpected high rate of myopathy found in muscle biopsies of orthopedic pediatric patients, supports our belief that precautions should always be taken in pediatric patients presenting for clubfoot surgery, to prevent MH related problems (8). We believe that regional anesthesia and complete monitoring should be the first choice anesthesia technique in such cases.…”

Section: Discussionmentioning

confidence: 70%

“…General anesthesia or deep sedation is standard practice before the administration of regional anesthesia in children. Therefore avoidance of triggering agents and adequate monitoring are of paramount importance in preventing MH related problems in children presenting for orthopedic surgery and suspected of having CCD (7,8,18). Induction of general anesthesia or deep sedation with ketamine seem to offer some advantages in pediatric patients (19) specially in a multimodal and preemptive strategy (10); moreover the pharmacological association of propofol and ketamine is reported to be safe in malignant hyperthermia susceptible patients (20).…”

Section: Discussionmentioning

confidence: 99%

“…These patients are considered at risk for development of MH (6) and related perioperative complications (7). Moreover, a high prevalence of myopathic changes in children undergoing clubfoot surgery is reported (8), thus anesthesiologists must take precautions including having a high index of suspicion and using trigger free anesthesia techniques in children suspected of having CCD. We present a paradigmatic example of this situation: a child diagnosed with CCD between two orthopedic interventions for bilateral clubfoot correction.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Undetected central core disease myopathy in an infant presenting for clubfoot surgery

Zanette

Robb

Zadra

et al. 2006

Pediatric Anesthesia

View full text Add to dashboard Cite

A 1-year-old child was scheduled for two stage bilateral clubfoot surgery. Preoperative evaluation was normal and total intravenous anesthesia with a continuous sciatic nerve block was performed. Two months later, before the second clubfoot correction, a hip subluxation was evident suggesting a provisional diagnosis of neuromuscular disease. Anesthesia was identical, except that a femoral nerve block, necessary to permit a diagnostic muscle biopsy was performed. The perioperative course was uneventful but result of the muscular biopsy was surprising in that central core disease was diagnosed. Although congenital myopathies of all grades and severity exist, they are often mild and underestimated. Patients affected by central core disease are considered susceptible to malignant hyperthermia. Because a high prevalence of myopathic changes is reported in children undergoing clubfoot surgery, anesthesiologists must take precautions including a hightened awareness of these events and a high index of suspicion.

show abstract

Section: Discussionmentioning

confidence: 70%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Undetected central core disease myopathy in an infant presenting for clubfoot surgery

Zanette

Robb

Zadra

et al. 2006

Pediatric Anesthesia

View full text Add to dashboard Cite

show abstract

“…A high rate of myopathic change on muscle biopsy is found, suggesting possible underlying myopathy. 6 Studies on twins, male predominance, and frequent CTEV-affected relatives suggest a genetic factor. 7 DM1 is caused by an unstable CTG repeat expansion in the DMPK gene, and DM2 is caused by a CCTG expansion in the ZNF9 gene.…”

Section: Congenital Talipes Equinovarus Associated With Myotonic Dystmentioning

confidence: 99%

Self‐reported follow‐up post‐intervention adherence to resistance exercise training in Charcot–Marie–Tooth disease patients

2010

View full text Add to dashboard Cite

A previously published study investigating 3 months of home-based resistance exercise showed improved strength and performance of activities of daily living (ADLs) in 20 Charcot-Marie-Tooth (CMT) patients. 1 We attempted to follow up these patients 20-34 months after completion of the intervention to see if the improvement was sustained. Only 9 (6 women, 3 men; mean age 48.2 6 7.8 years) of the original 20 patients (<50%) agreed to undergo follow-up assessment. Of these 9, only 3 (33%; 2 women, 1 man) reported continuing regular exercise. Subjects who continued strength training utilized the resistance exercise program described in the study by Chetlin and co-workers, 1 including elbow and knee flexion and extension movements performed three times per week with wrist and ankle weights. Hand-grip exercises were also performed. Of the 11 subjects who did not participate in the follow-up study, 8 could not be contacted and 3 declined participation, without specifying a reason. Isometric muscle force and timed ADLs were measured. Independent t-tests determined demographic differences between continued trainers (CTs) and discontinued trainers (DTs). Dependent t-tests compared follow-up to original data. Pearson's product correlations determined relationships between strength, ADLs, and demographics. CTs produced less force versus original posttest measures in right elbow extension (7.9 6 3.1 kg vs. 9.2 6 2.6 kg, P < 0.05) and left elbow flexion (10.8 6 3.5 kg vs. 15.3 6 4.0 kg, P < 0.05), whereas DTs produced less force in right elbow extension only (8.9 6 1.7 kg vs. 10.1 6 2.0 kg, P < 0.05). DTs were slower in lift and reach (19.6 6 3.5 s vs. 17.3 6 1.9 s, P < 0.05), supine rise (1.6 6 0.3 s vs. 1.3 6 0.3 s, P < 0.01), and stair climb (11.0 6 8.1 s vs. 9.4 6 7.6 s, P < 0.01), whereas CTs were not different versus original posttest ADL performance.Our findings show that both those who continued training and those who discontinued training lost strength versus the original study posttest measures, but functional ability was lost only in those who discontinued training. We conclude that patients with CMT benefited from a supervised exercise program and had improved strength and functional abilities, but, despite some loss in strength, functional gains may be maintained by continued unsupervised exercise. Although the number of patients retested is small, these results, in conjunction with our previous study, are encouraging in showing that CMT patients can achieve sustained improvement in function with exercise education and supervision if regular exercise is continued. This adds to the pool of information that helps dispel concerns that exercise is harmful to patients with CMT. The recent work by Burns and colleagues appears to support our contention that an appropriate resistance exercise prescription for CMT patients is not harmful and may provide additional benefit if such training is continued long term.2 The moderate-intensity strength training program that our CMT patients continued included those exercise varia...

show abstract