Abstract:【Objective】To investigate the clinical features of patients with neonatal necrotizing enterocolitis (NEC) complicated with Hirschsprung's disease (HD). 【Methods】The clinical data of 289 children with NEC who were admitted to Zhongshan Bo'ai Hospital affiliated to Southern Medical University from January 2010 to December 2017 were retrospectively analyzed. Among them, there were 23 cases of NEC combined with HD. The gestational age, birth weight, feeding style, gender, age of onset, clinical manifestations, laboratory findings, and abdominal X-ray findings of children with NEC combined with HD were collected.【Results】A total of 23 NEC patients with HD accounted for 7.95% (23/289) , of the patients, including 17 males and 6 females, with a mean gestational age of 35.6± 3.7 weeks. Patients enrolled in this study ( n =23) were 14 predominantly term infants (60.9%, 14/23) and 9 premature infants. None of the 23 patients had history of asphyxia/hypoxia or delayed meconium excretion. There were 2 patients of Bell stageⅠ, 4 patients of Bell stage Ⅱ and 14 patients of Bell stage Ⅲ, respectively. There were 11 patients with fever and 2 patients with low temperature.All 23 patients had abdominal distention, of whom 21 patients had history of vomiting and 11 patients had gastrointestinal hemorrhage, accounting for 91.3% and 47.8% respectively. There were 9 cases (39.1%) with swollen redness of abdominal wall, 13 cases (56.3%) with abdominal wall tenderness and 11 cases (47.8%) with peristaltic sound disappearance. One patient was found with gas feces ejection during rectum examination. In 23 patients, there were 8 cases (34.8%) with pneumoperitoneum, 6 cases (26.1%)with intestinal wall pneumatosis, 18 cases (78.3%) with intestinal stiffness, 16 cases (69.6%) with intestinal wall thickening, 2 cases(8.7%) with colon dilatation, 16 cases(69.6%) with intestinal obstruction, and 4 cases (17.4%) with hepatic portal venous gas. Treatment and prognosis: Symptoms of 6 patients of Bell stage Ⅰ and stage Ⅱ patients were improved after conservative treatment; 17 children of Bell stage Ⅲ received surgery. In total, 21 cases were cured, 1 case quitted treatment, and 1 case died. 【Conclusion】It is not uncommon for children with NEC to have HD. The presentations and imaging manifestations are not specific. Rectal biopsy is an important means of diagnosis. Early diagnosis is useful for surgical options and prognosis.
苏嘉鸿, 杨六成, 胡小华, 莫优炼, 张平锋, 钟陈. 新生儿坏死性小肠结肠炎合并先天性巨结肠23例临床分析[J]. 医学临床研究, 2018, 35(7): 1314-1316.
SU Jia-hong, YANG Liu-cheng, HU Xiao-hua, et al. Clinical Analysis of 23 Patients with Neonatal Necrotizing Enterocolitis Complicated with Hirschsprung's Disease. JOURNAL OF CLINICAL RESEARCH, 2018, 35(7): 1314-1316.
[1] 罗洁, 姜敏, 林影. 足月儿与早产儿坏死性小肠结肠炎临床表现及危险因素分析[J].北京医学, 2009,31(11):669-671. [2] Heanue TA, Pachnis V.Enteric nervous system development and Hirschsprung's disease: advances in genetic and stem cell studies[J]. Nat Rev Neurosci, 2007,8(6):466-479. [3] 邵肖梅. 实用新生儿学[M].第4版,2011:477-482. [4] Bell MJ, Ternberg JL, Feigin RD, et al . Neonatal necrotizing enterocolitis. Therapeutic decisions based upon clinical staging[J]. Ann Surg, 1978,187(1):1-7. [5] Tepas JR, Sharma R, Leaphart CL, et al . Timing of surgical intervention in necrotizing enterocolitis can be determined by trajectory of metabolic derangement[J]. J Pediatr Surg, 2010,45(2):310-313. [6] 施诚仁. 小儿外科特色治疗技术[M].2003:510-518. [7] 周雅芮,张丙宏,严彩霞,等.肠三叶因子对新生鼠坏死性小肠结肠炎模型Caspase-9表达的影响[J].实用儿科临床杂志,2009,(19):1490-1491. [8] Singh R.Necrotizing enterocolitis and the role of anemia of prematurity[J]. Semin Perinatol, 2012,36(4):277-282. [9] 杜勇. 先天性巨结肠肠粘膜紧密连接蛋白分布表达方式的研究[J].临床儿科杂志, 2006,24(5):410-413. [10] Pratap A, Gupta DK, Tiwari A, et al . Application of a plain abdominal radiograph transition zone (PARTZ) in Hirschsprung's disease[J]. BMC Pediatr, 2007,7:5. [11] 冯杰雄, 高贺云.新生儿先天性巨结肠诊治进展[J].实用儿科临床杂志, 2008,23(11):808-810. [12] Thompson AM, Bizzarro MJ.Necrotizing enterocolitis in newborns: pathogenesis, prevention and management[J]. Drugs, 2008,68(9):1227-1238. [13] 王献良, 邵雷朋, 谢文雅, 等. 新生儿坏死性小肠结肠炎164例分析[J].中华小儿外科杂志, 2015,36(2):105-108. [14] 唐维兵. 新生儿坏死性小肠结肠炎的诊治现状[J].临床外科杂志, 2017,25(12):946-948. [15] 江逊. 先天性巨结肠的诊断与治疗现状[J].中华实用儿科临床杂志, 2013,28(7):559-560.