结肠神经节缺乏症的临床和多层螺旋CT诊断

李晓阳; 田立斌

doi:10.15953/j.ctta.2022.161

结肠神经节缺乏症的临床和多层螺旋CT诊断

doi: 10.15953/j.ctta.2022.161

李晓阳^a, ,,
田立斌^b

1.
CT室；河北省唐山市人民医院
2.
病理科，河北唐山063001 河北省唐山市人民医院

详细信息

作者简介:
李晓阳：男，河北省唐山市人民医院主任医师、科主任，主要从事肿瘤影像诊断以及脏器功能影像学，E-mail：13315512960@163.com

通讯作者:
男，河北省唐山市人民医院主任医师、科主任，主要从事肿瘤影像诊断以及脏器功能影像学，E-mail：13315512960@163.com

中图分类号: R 814
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出版历程
- 收稿日期: 2022-08-07
- 修回日期: 2022-10-29
- 录用日期: 2022-11-08
- 网络出版日期: 2023-01-03
- 刊出日期: 2023-07-31

Clinical and Multilayer Spiral CT Diagnosis of Colon Innervation Defect

LI Xiaoyang^{a
, ,},
TIAN Libin^b

1.
Tangshan People's Hospital, Tangshan 063001, China CT Roathom
2.
Tangshan People's Hospital, Tangshan 063001, China Pology Department

摘要

摘要: 目的：分析结肠神经节缺乏症的多层螺旋CT（MSCT）的影像和临床表现，提高对结肠神经节缺乏症认识。方法：回顾性分析经手术病理证实的结肠神经节缺乏症患者临床以及MSCT影像资料。检查设备采用GE Discovery 750 HD宝石能谱CT和Philips Brilliance Ingenuity 128层多层螺旋，层厚1 mm，层间距1 mm，管电压120 kV，自动管电流，扫描范围自膈顶扫描至双侧耻骨联合下缘。在肠道自然状态下CT扫描（不做肠道准备，即不清洁灌肠和洗肠），扫描后在CT工作站进行MPR三维重建，在PACS系统存档分析。观察MSCT病变段肠管位置，分别测量扩张段和狭窄段肠壁厚度；对病理标本测量狭窄段（病变段）肠管长度；通过多期MSCT观察肠道蠕动情况；通过增强观察肠道血运情况；总结结肠神经节缺乏症患者的临床特征性表现。结果：结肠神经节缺乏症的临床特点表现为成年人长期的便秘和不完全性肠梗阻。本组5例成人结肠神经节缺乏症患者，病变部位分别位于结肠脾曲和降结肠，其中病变段为狭窄段肠管，扩张段结肠为次生继发性改变，病变段肠管位于降结肠3例，位于结肠脾曲2例；MSCT表现为病变段结肠相对狭窄和病变近端结肠扩张，影像特征表现为结肠扩张后狭窄；测量扩张段和狭窄段肠壁厚度：狭窄段病变区肠壁厚度正常，扩张段肠壁厚度正常或有所增厚，本组增厚的肠壁厚度小于0.9 cm；测量病变段肠管长度：本组病变段肠管长度介于4.3～8.6 cm之间；观察结肠血供和肠道功能情况：MSCT增强扫描肠系膜血管以及系膜密度均未见异常改变、病变段结肠肠壁无异常强化，提示扩张与狭窄段结肠血供正常；MSCT三期增强扫描显示病变段肠管僵直、无蠕动，提示病变段肠管蠕动功能丧失。结论：结肠神经节缺乏症具有结肠扩张后狭窄特征性的影像学表现和临床特点，MSCT结合临床资料能够在术前提示结肠神经节缺乏症的诊断。
- X线计算机体层成像 /
- 影像诊断 /
- 结肠神经节缺乏症
Abstract: Objective: To analyze the image performance of colon innervation defect with multilayer spiral CT (MSCT) and clinical manifestations, summarize its image characteristics and make correct diagnosis. Methods: The clinical features of colonic innervations deficiency present with prolonged constipation and incomplete ileus. MSCT imaging data using GE gem energy spectrum, CT 750 HD, and Philips MSCT. 1 mm layer thickness, 1 mm layer spacing, tube voltage, 120 kV, automatic tube current from the diaphragm to the bilateral pubic joint. Scan in the natural state of the intestine (No bowel preparation, no cleansing enema and bowel cleansing), after scanning, conduct MPR 3D reconstruction at the CT workstation, and the reconstructed data were archived and analyzed in the PACS system. Clarify the intestinal location of the diseased segment, measure intestinal wall thickness of dilated segment and narrow segment respectively; measure intestinal tube length of diseased segment (narrow segment); observe intestinal peristalsis with multiple-stage MSCT; and observe intestinal blood transport through enhancement. Results: The clinical features of colon innervations defect was constipation and incomplete ileus. In this study group, there were 5 adult patients with colon innervations defect, and the lesion site was located in the spleen and descending colon respectively, among which the diseased segment was located in 3 cases and the spleen was located in 2 cases of colon; MSCT shows relative narrowing of the colon and expansion of the proximal colon; The intestinal wall thickness was normal in the diseased area, and the intestinal wall thickness of the dilated colon section was normal or somewhat thickened, and the thickened intestinal wall in this group is less than 0.9 cm; The intestinal length of the diseased segment in this group was somewhere between 4.3~8.6 cm. The MSCT enhancement scan of mesangic vessels and mesangial density showed no abnormal changes, and no abnormal enhancement of the colon wall in the diseased section, suggesting normal blood supply; MSCT enhanced scan showed rigidity and no peristalsis in the diseased segment, suggesting loss of peristaltic function in the diseased segment. Conclusion: Colonic innervations defect has imaging findings of characteristic post dilating stenosis and clinical features of prolonged constipation and incomplete obstruction in adults, The MSCT combined with clinical data was able to indicate the diagnosis of colonic innervations defect before surgery.
- X-ray computer tomography /
- imaging diagnosis /
- colon innervations defect

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图 1 上腹CT平扫，横结肠脾曲肠腔扩张内径7 cm，肠壁均一弥漫性增厚，厚约0.5 cm，扩张肠腔内大量粪便集聚；结肠脾曲肠腔相对狭窄（空白箭），肠壁厚0.4～0.6 cm

Figure 1. CT image of the abdomen，The splenic flexure of colon intestinal lumen was dilated, with an internal diameter of 6 cm, and the intestinal wall was diffuse and thickened, about 0.5 cm thick, and a large number of feces were concentrated in the dilated intestinal lumen. Colonic spleen curved intestinal lumen is relatively narrow (blank arrow), and the intestinal wall is 0.4～0.6 cm thick

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图 2 上腹CT平扫，横结肠肠腔扩张，大部分肠壁厚度正常、局部肠壁轻微增厚，最厚约0.4 cm，扩张的肠腔内大量粪便；降结肠病变段肠管相对狭窄（空白箭），肠壁厚约0.8 cm

Figure 2. CT image of the abdomen，The transverse colon lumen is dilated, with uneven thickening, the thickest intestinal wall diameter is about 0.4 cm, a large number of fees were concentrated in the dilated intestinallumen. The diseased segment of the descending colon is relatively narrow (blank arrow), the local thickness is about 0.8 cm

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图 3 与图2同一患者，斜冠状位重建图像，降结肠相对狭窄（粗黑箭）、横结肠显著扩张（细黑箭）和小肠扩张（白箭）

Figure 3. In the same patient as in fig.2, the descending colon lumen was relatively narrow and the transverse colon lumen was significantly dilated

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图 4 图1患者术后大体标本，肉眼所见：全结肠切除标本：结肠长68 cm，周径5～12 cm，回肠长7 cm，周径3 cm。扩张段肠管长15 cm，周径12 cm，壁厚0.5 cm，粘膜灰红色，质软，皱襞较清晰；狭窄段肠管，长5 cm，周径5 cm，壁厚0.5 cm

Figure 4. The Patient of fig.1, postoperative gross specimen, as seen by the naked eye. Total colon resection specimen: The colon is 68 cm long, the peripheral diameter is 5~12 cm, The ileum was 7 cm long with a circumference diameter of 3 cm. The intestine of the dilated segment was 15 cm long, Peridiameter 12 cm, wall thickness 0.5 cm, Mucosal membrane is grey-red, soft, The wrinkles are clear; The arrow segment intestine, 5 cm long, The circumference diameter is 5 cm and the wall thickness is 0.5 cm

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图 5 与图1同一患者，HE染色10×10结合免疫组化S-100，显示狭窄段肠黏膜下层及肌间神经节细胞数量显著减少。病理诊断：结肠神经节缺乏症（结肠假性）

Figure 5. The same patient of fig.1, HE pathological staining, 10×10 combined with immunohistochemistry S-100, a narrow segment of the intestinal mucosa is shown the number of lower and myomuscular ganglion cells was significantly reduced. Pathological diagnosis: Colonic innervation defect (Hirschsprung' Disease)

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参考文献(11)

[1]	隋金珂, 张卫. 成人巨结肠病因分析及手术方式选择[J]. 中华胃肠外科杂志, 2021,24(12): 1054−1057. DOI: 10.3760/cma.j.cn441530-20211103-00445. SUI J K, ZHANG W. Etiological analysis and surgical method selection of adult megacolon[J]. Chinese Journal of Gastrointestinal Surgery, 2021, 24(12): 1054−1057. DOI: 10.3760/cma.j.cn441530-20211103-00445. (in Chinese).
[2]	STARLING J R, CROOM R D, THOMAS C G. Hirschsprung's disease in young adults[J]. American journal of Surgery, 1986, 151(1): 104−109. DOI: 10.1016/0002-9610(86)90019-x.
[3]	戴春娟, 叶祖萍. 先天性巨结肠遗传学基础研究进展[J]. 河北医药, 2010, 53(4), 89-92. DOI:10.3969/J.issn 1002-7386.2010.04.053. DAI C J, YE Z P. Research progress on genetic basis of Hirschsprung’s disease[J], Hebei Medicine, 2010, 53(4), 89-92. DOI:10.3969/J.issn1002-7386.2010.04.053(in chinese).
[4]	张文, 武海燕, 李惠, 等. 先天性巨结肠病理诊断规范[J]. 中华病理学杂志, 2016,45(3): 149−152. DOI: 10.3760/cma.j.issn.0529-5807.2016.03.002. ZHANG W, WU H Y, LI H, et al. The guideline of pathological diagnosis of Hirschsprung's disease[J]. Chinese Journal of Pathology, 2016, 45(3): 149−152. DOI: 10.3760/cma.j.issn.0529-5807.2016.03.002. (in Chinese).
[5]	Isolated hypoganglionosis: Systematic review of a rare intestinal innervation defect[J]. Pediatric Surgery Internation, 2010, 26(11): 1111-1115. DOI: 10.1007/s00383-010-2693-3.
[6]	DO M Y, MYUNG S J, PARK H J, et al. Novel classification and pathogenetic analysis of hypoganglionosis and adult-onset Hirschsprung's disease[J]. Digestive Diseases and Sciences, 2011, 56(6): 1818−1827. DOI: 10.1007/s10620-010-1522-9.
[7]	HUIZER V, WIJEKOON N, ROORDA D, et al. Generic and disease-specific henlth-ralated quality of life in pentints with Hirschspurung disease: A systematic review and Meta-angnlysis[J]. World Journal of Gastroenterology, 2022, 28(13): 1362−1376. doi: 10.3748/wjg.v28.i13.1362
[8]	FRIEDMACHER F, PURI P. Classification and diagnostic criteria of variants of Hirschsprung's disease[J]. Pediatric Surgery Internation, 2013, 29(9): 855−872. doi: 10.1007/s00383-013-3351-3
[9]	王维林. 关于肠神经元发育异常的再思考[J]. 中华小儿外科杂志, 2014,35(7): 481−483. DOI: 10.3760/cma.j.issn.0253-3006.2014.07.001. WANG W L. Rethinking about intestinal neuronal dysplasia[J]. The Chinese Journal of Pediatric Surgery, 2014, 35(7): 481−483. DOI: 10.3760/cma.j.issn.0253-3006.2014.07.001. (in Chinese).
[10]	孙晓毅. 巨结肠同源病诊断治疗难点解析[J]. 中华实用儿科临床杂志, 2014,29(23): 1763−1768. DOI: 10.3760/cma.j.issn.2095-428X.2014.23.002. SUN X Y. A comment on difficulties in diagnosis and treatment of Hirschsprung's disease allied disorders[J]. The Chinese Clinical Journal of Practical Pediatrics, 2014, 29(23): 1763−1768. DOI: 10.3760/cma.j.issn.2095-428X.2014.23.002. (in Chinese).
[11]	朱天琦, 冯杰雄, 张文, 等. 新生儿先天性巨结肠经脐腹腔镜根治术十例报告[J]. 中华外科杂志, 2011,49(11): 1049−1051. DOI: 10.3760/cma.j.issn.0529-5815.2011.11.025. ZHU T Q, FENG J X, ZHANG W, et al. 10 case reports of neonatal Hirschsprung disease by transumbilical laparoscopic colectomy[J]. Chinese Journal of Surgery, 2011, 49(11): 1049−1051. DOI: 10.3760/cma.j.issn.0529-5815.2011.11.025. (in Chinese).