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儿童肠套叠:从罕见的致套叠点——肠道淋巴瘤中吸取的教训

Intussusception in children: lessons learned from intestinal lymphoma as a rare lead-point.

作者信息

Bussell H R, Kroiss S, Tharakan S J, Meuli M, Moehrlen U

机构信息

Department of Pediatric Surgery, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland.

Division of Oncology, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland.

出版信息

Pediatr Surg Int. 2019 Aug;35(8):879-885. doi: 10.1007/s00383-019-04488-z. Epub 2019 May 28.

Abstract

BACKGROUND

Most cases of intussusception in children are idiopathic. Rarely, a malignant disease such as intestinal lymphoma may cause intussusception. Due to dramatic changes of expected outcome with chemotherapy regime alone, the surgical management of patients with intestinal lymphoma presenting with intussusception has to be reevaluated.

METHODS

Retrospective chart review from May 2011 to February 2017. We included all patients with intestinal lymphoma presenting with intussusception.

RESULTS

We found five patients with a mean age of 6.4 years (range 3-16). The most common presenting symptom was abdominal pain for several weeks which had acutely worsened. In all but one patient an ultrasound before pneumatic or hydrostatic reduction showed a finding suspicious of a pathological lead-point. Pneumatic or hydrostatic reduction was attempted in all patients, no complications were noted. In one patient reduction was not successful. Recurrence after reduction occurred in two patients. Two patients needed surgery, three had a percutaneous ultrasound-guided biopsy for diagnostic purposes. All patients had aggressive mature B cell non-Hodgkin lymphoma.

CONCLUSION

A high index of suspicion for the presence of a pathological lead-point in children older than 4 years and children with recurrent intussusception is necessary in patients presenting with intussusception. Malignant, highly aggressive B cell non-Hodgkin lymphoma, although rare, must actively be searched for. Pneumatic or hydrostatic reduction should remain the first line treatment in most cases.

摘要

背景

儿童肠套叠大多为特发性。极少情况下,肠道淋巴瘤等恶性疾病可导致肠套叠。由于单纯化疗方案会使预期结果发生显著变化,因此必须重新评估肠套叠型肠道淋巴瘤患者的手术治疗方法。

方法

对2011年5月至2017年2月期间的病历进行回顾性分析。纳入所有肠套叠型肠道淋巴瘤患者。

结果

我们发现5例患者,平均年龄6.4岁(范围3 - 16岁)。最常见的症状是数周的腹痛且急性加重。除1例患者外,所有患者在空气灌肠或水压灌肠复位前的超声检查均发现可疑病理性套入点。所有患者均尝试进行空气灌肠或水压灌肠复位,未发现并发症。1例患者复位未成功。2例患者复位后复发。2例患者需要手术,3例患者进行了经皮超声引导下活检以明确诊断。所有患者均为侵袭性成熟B细胞非霍奇金淋巴瘤。

结论

对于出现肠套叠的4岁以上儿童及复发性肠套叠患儿,必须高度怀疑存在病理性套入点。恶性、高度侵袭性的B细胞非霍奇金淋巴瘤虽罕见,但必须积极排查。在大多数情况下空气灌肠或水压灌肠复位仍应作为一线治疗方法。

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