Esophageal motility disorders in Mexican patioents with Duchenne's muscular dystrophy

Objetive: Myopathies are entities tahat mainly involve strieted muscle. In Duchenne's muscular dystrophy (DMD) there have been reported smooth muscle alterations in the pre-oral phase of swallowing, in gastric emptying, and pseudoobstruction. Nevertheless, esophageal motility alterations are not concluding. The objetive of this work was to determine if there are motor esophageal alterations is this patients, and if this alterations are related to the clinical manifestations of disease. Study design: nine consecutive patients with DMD (mean age 8, range 6-11 years; males) were evaluated, comparing clinical and manometric findings. Results: esophageal manometry alterations were found in all patients, mainly simultaneous non-peristaltic waves (60.86 percent) of diminished amplitude, in both striated and smooth muscle. Seventy seven percent presented with upper and lower gastrointestinal symptoms (dysphagia, regurgitation, epigastric pain, constipation, and distention). No correlation was found between esophageal motility alterations and gastrointestinal symptoms, nor with the clinical stage of disease in accordance to Brook (r=0.27). Conclusion: these results show that patients with DMD present esophageal motor disorders in both striated and smooth muscle, as well as upper and lower gastrointestinal symptoms. Specialized motility studies, could yield a better understanding of disease, and, possibly with adequate treatment, provide for a better quality of life in children with DMD.

Esophageal motility disorders in Mexican patioents with Duchennes muscular dystrophy

Objetive: Myopathies are entities tahat mainly involve strieted muscle. In Duchennes muscular dystrophy (DMD) there have been reported smooth muscle alterations in the pre-oral phase of swallowing, in gastric emptying, and pseudoobstruction. Nevertheless, esophageal motility alterations are not concluding. The objetive of this work was to determine if there are motor esophageal alterations is this patients, and if this alterations are related to the clinical manifestations of disease. Study design: nine consecutive patients with DMD (mean age 8, range 6-11 years; males) were evaluated, comparing clinical and manometric findings. Results: esophageal manometry alterations were found in all patients, mainly simultaneous non-peristaltic waves (60.86 percent) of diminished amplitude, in both striated and smooth muscle. Seventy seven percent presented with upper and lower gastrointestinal symptoms (dysphagia, regurgitation, epigastric pain, constipation, and distention). No correlation was found between esophageal motility alterations and gastrointestinal symptoms, nor with the clinical stage of disease in accordance to Brook (r=0.27). Conclusion: these results show that patients with DMD present esophageal motor disorders in both striated and smooth muscle, as well as upper and lower gastrointestinal symptoms. Specialized motility studies, could yield a better understanding of disease, and, possibly with adequate treatment, provide for a better quality of life in children with DMD. (AU)

Retroalimentacion biologica y cirugia en el tratamiento de la incontinencia fecal con lesion del esfinter anal externo: seguimento a largo plazo / Biofeedback and surgery in the treatment of fecal incontinence and lesion of the external anal sphincter: long-term follow-up

INTRODUCCION: En niños, el traumatismo de la región anal que afecta tanto al esfínter anal externo como al esfínter anal interno es un grave problema. La retroalimentación biológica no es efectiva si hay destrucción del esfínter anal externo, y la restitución de la morfologia con cirugía tampoco cura la incontinencia. CASO CLINICO: Niña de 6 años 8 meses de edad, que desde los 2 años sufrió tricocefalosis masiva recurrente; condicionándole prolapso rectal, abscesos y fístulas perianales, fistulectomías, y finalmente destrucción de la región perianal, con incontinencia total. Previa manometría rectoanal, y determinación de receptores rectales, se efectuó retroalimentación biológica antes y después de la transposición del músculo gracilis y colostomía. a los 12 meses se obtuvo continencia total, que aún persiste a 7 años de seguimiento. CONCLUSIONES: En la incontinencia fecal con destrucción del esfínter anal externo, y alteración fisiológica del esfínter anal interno, se debe efectuar: manometria y determinación de receptores rectales, reconstrucción del esfínter anal externo con transposición del músculo gracilis, y retroalimentación biológica

Retroalimentacion biologica y cirugia en el tratamiento de la incontinencia fecal con lesion del esfinter anal externo: seguimento a largo plazo / Biofeedback and surgery in the treatment of fecal incontinence and lesion of the external anal sphincter: long-term follow-up

INTRODUCCION: En niños, el traumatismo de la región anal que afecta tanto al esfínter anal externo como al esfínter anal interno es un grave problema. La retroalimentación biológica no es efectiva si hay destrucción del esfínter anal externo, y la restitución de la morfologia con cirugía tampoco cura la incontinencia. CASO CLINICO: Niña de 6 años 8 meses de edad, que desde los 2 años sufrió tricocefalosis masiva recurrente; condicionándole prolapso rectal, abscesos y fístulas perianales, fistulectomías, y finalmente destrucción de la región perianal, con incontinencia total. Previa manometría rectoanal, y determinación de receptores rectales, se efectuó retroalimentación biológica antes y después de la transposición del músculo gracilis y colostomía. a los 12 meses se obtuvo continencia total, que aún persiste a 7 años de seguimiento. CONCLUSIONES: En la incontinencia fecal con destrucción del esfínter anal externo, y alteración fisiológica del esfínter anal interno, se debe efectuar: manometria y determinación de receptores rectales, reconstrucción del esfínter anal externo con transposición del músculo gracilis, y retroalimentación biológica (AU)