Watanabe Yoshio, Kanamori Yutaka, Uchida Keiichi, Taguchi Tomoaki
Department of Pediatric Surgery, Aichi Children's Health and Medical Center, 1-2 Osakada, Morioka-cho, Obu-city, Aichi, 474-8710, Japan,
Pediatr Surg Int. 2013 Nov;29(11):1127-30. doi: 10.1007/s00383-013-3378-5.
This study analyzes how isolated hypoganglionosis (IH) is diagnosed and treated in Japanese pediatric surgical departments.
A second questionnaire was sent to 90 pediatric surgical departments that took part in an initial survey on variant Hirschsprung's disease.
Of 109 cases registered as having a certain diagnosis of IH, we targeted 90 patients and excluded the remaining 19. Symptom onset was neonatal ileus with a male:female ratio of 34:56. In most cases, results from radio-contrast enemas and suction rectal biopsies were normal. Anorectal manometry demonstrated that there was no relaxation in 73 % of the 37 patients examined. Furthermore, out of 55 patients who were examined intraoperatively, IH was diagnosed or suspected in 29 cases. Another 12 cases were eventually diagnosed with IH by other institutions using paraffin-embedded specimens. Stomas were initially created in 89 out of 90 cases and the mortality rate of the group that initially received jejunostomies was significantly lower than that of the ileostomy group (p < 0.05).
Preoperative diagnosis of IH is often difficult and intraoperative biopsies were only able to provide a correct diagnosis in 52 % of cases. More accurate diagnosis and the initial creation of jejunostomies rather than ileostomies may assist in improving patient management and outcomes.
本研究分析日本小儿外科科室如何诊断和治疗孤立性神经节减少症（IH）。
向90个参与先天性巨结肠变异型初步调查的小儿外科科室发送了第二份调查问卷。
在登记为确诊IH的109例病例中，我们选取了90例患者，排除了其余19例。症状表现为新生儿肠梗阻，男女比例为34:56。在大多数情况下，钡剂灌肠造影和直肠吸引活检结果正常。直肠肛管测压显示，在接受检查的37例患者中，73%没有松弛现象。此外，在55例接受术中检查的患者中，29例被诊断或怀疑为IH。另外12例最终由其他机构使用石蜡包埋标本确诊为IH。90例患者中有89例最初进行了造口术，最初接受空肠造口术的组死亡率显著低于回肠造口术组（p<0.05）。
IH的术前诊断通常很困难，术中活检仅在52%的病例中能够提供正确诊断。更准确的诊断以及最初进行空肠造口术而非回肠造口术可能有助于改善患者管理和治疗结果。
