The problematic Soave cuff in Hirschsprung disease: Manifestations and treatment

Purpose

Following a Soave pull-through for Hirschsprung disease (HD), some children struggle with obstructive symptoms. We hypothesized that these symptoms could result from a functional obstruction of the pull through caused by the Soave cuff, and that cuff resection might improve bowel emptying.

Methods

We reviewed patients referred to our center from 2008 to 2012 with obstructive problems following a Soave pull-through for HD (CCHMC IRB # 2011–2019). Only patients with an obstructing Soave cuff were analyzed. Patients with other reasons for obstruction (anastomotic stricture, transition zone, aganglionic segment) were excluded.

Results

Thirty-six patients underwent reoperation at our center for obstructive symptoms after an initial Soave pull-through. Seventeen of these patients had a Soave cuff only as the potential source of obstruction. Pre-operative symptoms included enterocolitis (10), constipation (6), and failure to thrive (1). Nine patients (53%) required irrigations to manage distension or enterocolitis pre-operatively. 14/17 patients (82%) had a palpable cuff on rectal exam. Eight patients (47%) had radiographic evidence of a cuff demonstrated by distal narrowing (4) or a prominent presacral space (4). Four children (23%) underwent excision of the cuff only. Thirteen (76%) had removal of the cuff and proximally dilated colon [(average length 7.2 cm) (12 performed transanally, and five needed laparotomy as well.)] Post-operatively, episodes of enterocolitis were reduced to zero, and need for irrigation to treat distension was reduced by 50%. Nine patients have voluntary bowel movements, and five are clean on enemas. 3/6 patients with pre-operative constipation or impaction now empty without enemas. (Follow up 1–17 months, mean 7 months.)

Conclusions

Recurrent enterocolitis, constipation, or failure to thrive can indicate a functional obstruction due to a Soave cuff when no other pathologic cause exists. Physical exam or contrast enema can identify a problematic cuff. Reoperation with cuff resection can dramatically improve bowel emptying.     

坏死性小肠结肠炎的影像学表现

目的探讨坏死性小肠结肠炎X线表现,以提高诊断正确率。方法分析66例坏死性小肠结肠炎患儿的X线表现,66例均行腹部平片检查,其中4例行腹部CT平扫,并结合临床表现和有关文献,对其X线特征进行描述。结果坏死性小肠结肠炎早期X线表现为胃泡影增大30例,肠曲充气多、肠腔扩张32例,肠曲充气不良14例,肠间隙增厚模26例,部分肠曲狭窄变细、形态僵直40例,进展期肠壁积气29例,门静脉积气者6例,腹腔积液20例,气腹6例。结论肠壁积气、肝门静脉积气为坏死性小肠结肠炎特征性X线征象。X线腹部平片检查对坏死性小肠结肠炎早期诊断,病变进展的估计及提供手术适应症起着重要作用。     

新生儿坏死性小肠结肠炎并发神经发育障碍的危险因素研究

背景 新生儿坏死性小肠结肠炎（NEC）会影响患儿远期神经发育情况,目前关于NEC并发不良神经结局的危险因素的研究较少。 目的 探讨新生儿NEC并发神经发育障碍的危险因素。 方法 选取2019年1月至2020年6月于郑州大学第三附属医院新生儿科就诊并确诊为Ⅱ~Ⅲ期NEC的早产儿为研究对象。记录患儿的新生儿一般情况、母亲孕期合并症、新生儿期并发症。所有患儿随访至纠正年龄1岁,并采用贝利婴幼儿发展量表Ⅲ（BSID-Ⅲ）进行神经发育评估,依据评估结果将患儿分为神经发育障碍组和神经发育正常组。采用多因素Logistic回归分析探究NEC患儿并发神经发育障碍的危险因素。 结果 本研究共纳入Ⅱ~Ⅲ期NEC患儿236例,其中死亡23例、失访13例,完成随访200例。200例患儿中神经发育障碍21例（10.5%,神经发育障碍组）,神经发育正常179例（89.5%,神经发育正常组）。神经发育障碍组患儿出生体质量低于神经发育正常组,败血症、肠穿孔、短肠综合征发生率高于神经发育正常组（P<0.05）。多因素Logistic回归分析结果显示,出生体质量<1 000 g〔OR=4.603,95%CI（1.220,17.365）,P=0.024〕、败血症〔OR=4.401,95%CI（1.191,16.262）,P=0.026〕、肠穿孔〔OR=5.239,95%CI（1.522,18.029）,P=0.009〕是NEC患儿并发神经发育障碍的独立危险因素。 结论 出生体质量<1 000 g、败血症、肠穿孔是NEC患儿并发神经发育障碍的危险因素,新生儿科医生应高度重视,注意早期识别和预防,以降低NEC伤残率。     

Campylobacter colitis: a common infectious form of acute colitis

A case of acute ulcerating colitis due toCampylobacter fetus is presented. The radiographic and endoscopic findings are indistinguishable from those of idiopathic acute ulcerative colitis. The clinical and bacteriological data relating to Campylobacter are discussed.     

抗结核药物致伪膜性肠炎八例诊治分析

伪膜性肠炎（Pseudomembraneous colitis,PMC）是一种主要发生于结肠,亦可累及小肠的急性黏膜坏死和纤维素渗出性炎症,黏膜表面覆有黄白或黄绿色伪膜,多为应用抗生素后导致肠道菌群失调,难辨梭状芽胞杆菌大量繁殖,产生毒素而致病[1-3].以往报道该病多因细菌感染使用抗生素所致,鲜见服用抗结核药物出现伪膜性肠炎的报道.2010年6月至2013年10月,我院共收治抗结核药物导致伪膜性肠炎患者8例,现将其临床特征及诊治过程分析如下.     

Redo pull-through in Hirschsprung's [corrected] disease for obstructive symptoms due to residual aganglionosis and transition zone bowel

Background

Reoperations in Hirschsprung disease may be required for residual aganglionosis or transition-zone bowel found at the distal pull-through. We aimed to review the management of patients who had this complication and offer suggestions on how to avoid it.

Methods

Ninety-three patients with Hirschsprung disease were referred to our institution with recurrent problems after a pull-through done elsewhere. All required reoperations with a variety of indications, and of these, 25 had residual aganglionosis/transition-zone histology. This was the only indication for redo in 16 children.

Results

Children (range, 2-17 years) presented 6 to 66 months after the initial pull-through. The predominant symptoms were enterocolitis (n = 9 [56%]), constipation (n = 7 [44%]), failure to thrive (n = 5 [31%]), and impaction (n = 4 [25%]). The rectal biopsy performed as part of their post pull-through work up showed hypertrophic nerves (n = 16), absent ganglion cells (n = 6), and normal ganglion cells (n = 10). The original frozen-section biopsy, determining the level of the pull-through, only sampled the seromuscular layer in 3 children, leading to misdiagnosis. Reoperations involved a transanal resection (n = 15) and a posterior sagittal approach (n = 1). In all cases, obstructive symptoms were resolved, and no patient has had recurrent enterocolitis.

Conclusion

Patients' post pull-through with recurrent obstructive symptoms may have residual aganglionosis or transition-zone bowel. Reoperation can result in the resolution of these symptoms. A full-thickness biopsy at the time of the initial pull-through to include the mucosa and submucosa may increase the possibility of identifying hypertrophic nerves.     

Prevalence of head deformities in preterm infants at term equivalent age

Introduction

Due to a rising number of head deformities in healthy newborns, there has been an increasing interest in nonsynostotic head deformities in children over recent years. Although preterm infants are more likely to have anomalous head shapes than term newborns, there is limited data available on early prevalence of head deformities in preterm infants.

Aims

The purposes of the present study were to acquire quantitative data on head shape of preterm infants at Term Equivalent Age (TEA), to determine the prevalence of symmetrical and asymmetrical head deformities and to identify possible risk factors.

Methods

In a cross-sectional study design, Cranial Vault Asymmetry Index (CVAI) and Cranial Index (CI) calculated from routine head-scans with a non-invasive laser shape digitizer were recorded and categorized in type and severity of deformation for three different groups of gestational age. Perinatal and postnatal patient data was tested for possible associations.

Results

Scans of 195 infants were included in the study. CVAI at TEA was higher in very preterm (4.1%) compared to term and late preterm infants. Prevalence of deformational plagiocephaly was 38% in very preterm infants. CI was lower in very (71.4%) and late (77.2%) preterm infants compared to term infants (80.0%). Compared to term babies (11%), a large number of very (73%) and late (28%) preterm infants exhibited dolichocephaly at TEA.

Discussion

Prevalence of symmetrical and asymmetrical head deformities in preterm infants is high at TEA. Interventions are required to prevent head deformities in preterm infants during the initial hospital stay.     

肠三叶因子在新生鼠坏死性小肠结肠炎模型中对Bax和Bcl-2及Caspase-3表达的影响及意义

目的通过研究肠三叶因子(ITF)对新生大鼠坏死性小肠结肠炎(NEC)模型肠组织病理学改变及肠道组织中蛋白酶Caspase-3、蛋白Bax和Bcl-2的含量变化,探讨ITF对NEC保护作用的可能机制。方法30只新生1日龄Wistar大鼠随机分为3组,正常对照组、实验组、干预组,每组10只。实验组为NEC模型后加生理盐水0.2ml腹腔注射;干预组为NEC模型后予以腹腔注射ITF 0.2 mg(0.2 ml);正常对照组未予处理。第4天处死所有大鼠,取肠组织待检,取近回盲部1～2 cm肠道组织,采用分光光度法检查Caspase-3的表达、采用免疫组化法检测肠道组织中Bax及Bcl-2的含量变化并做病理学检查。结果实验组Caspase-3表达高于正常对照组和干预组(P均<0.05),干预组与正常对照组比较差异无统计学意义(P>0.05);实验组Bax表达高于正常对照组和干预组(P均<0.05),干预组与正常对照组相近(P>0.05);干预组Bcl-2表达高于正常对照组和干预组(P均<0.05),实验组高于正常对照组(P<0.05)。正常对照组的肠组织病理学未见异常,病理评分为0分;实验组HE染色切片见肠壁损伤轻重不一...