Tatekawa Yukihiro, Muraji Toshihiro, Takamizawa Shigeru
Department of Surgery, Nara Medical University, Kashihara, Nara 634-8522, Japan.
J Pediatr Surg. 2007 Apr;42(4):E9-12. doi: 10.1016/j.jpedsurg.2007.01.058.
We report our experience with a patient with near-total intestinal aganglionosis who underwent extended myectomy-myotomy of the proximal jejunum. At 45 days of age, the patient underwent rejejunostomy at 34 cm from the ligament of Treiz (LOT) because of enteritis and the bowel rehabilitation therapy was started. Because the patient established the swelling and eating enough at 8 months of age, an extended myectomy-myotomy was performed when she was 245 days old. A 12-cm myectomy from the jejunostomy located at 27 cm from the LOT was coupled with a 30-cm antemesenteric border myotomy. This ultimately left the patient with 65 to 70 cm of small bowel from the LOT. Synbiotics therapy was performed to prevent bacterial translocation, and then central line infection or enteritis was not observed at all. The patient was doing well, weighing 9.65 kg, at 2 years of age. In conclusion, extended myectomy-myotomy has extended the patient's survival period, but it is important and necessary to prevent bacterial translocation and to improve intestinal adaptation by synbiotics therapy.
我们报告了一名患有近全肠无神经节症的患者接受近端空肠扩大肌切除术-肌切开术的经验。该患者45日龄时，因肠炎在距屈氏韧带（LOT）34 cm处进行了空肠造口术，并开始了肠道康复治疗。由于患者在8月龄时出现腹胀且进食充足，故在其245日龄时进行了扩大肌切除术-肌切开术。从距LOT 27 cm处的空肠造口处切除12 cm的肌肉，并在系膜对侧缘进行30 cm的肌切开术。这最终使患者从LOT处保留了65至70 cm的小肠。进行了合生元疗法以预防细菌移位，此后未观察到中心静脉感染或肠炎。该患者在2岁时情况良好，体重9.65 kg。总之，扩大肌切除术-肌切开术延长了患者的生存期，但通过合生元疗法预防细菌移位和改善肠道适应性很重要且必要。
