背景：Megacystis微结肠肠蠕动减退（MMIHS）是一种罕见的疾病，其特征是膀胱扩张而不阻塞，微结肠,肠蠕动减少。MMIHS的预后特别差；然而，如果管理得当，生存可以延长。
方法：对关键词“巨结肠小结肠肠蠕动综合征”进行系统评价（1996-2016年）。\"此外,介绍了4例患者的病例系列以及MMIHS的诊断和治疗算法.
结果：文献中发现了135例MMIHS患者。73%（88/121）的患者为女性，65%接受诊断活检(64/99)，63%(66/106)的产前影像学检查结果。大多数患者接受TPN以及胃造口术或回肠造口术和CIC，然而，15%(18/116)接受了多内脏或肠道移植，30％（22/73）进行了膀胱造口术。成活率为57%(68/121)。
结论：MMIHS患者的适当管理至关重要。一个放大的，有肠蠕动问题的儿童的膀胱收缩应被视为诊断。如果存在频繁的尿路感染，除了预防性抗生素外，还可以通过CIC或膀胱造口术来控制膀胱扩张。这些患者通常需要胃造口术或回肠造口术以及全胃肠外营养。这种管理导致存活率的显著提高。
BACKGROUND: Megacystis microcolon intestinal hypoperistalsis (
MMIHS) is a rare disorder characterized by distended nonobstructed bladder, microcolon, and decreased intestinal peristalsis.
MMIHS has a particularly poor prognosis; however, when appropriately managed, survival can be prolonged.
METHODS: A systematic
review (1996-2016) was performed with the key words \"megacystis microcolon intestinal hypoperistalsis syndrome.\" In addition, a case series of four patients is presented as well as algorithms for the diagnosis and treatment of
MMIHS.
RESULTS: 135 patients with MMIHS were identified in the literature. 73% (88/121) of the patients were female, 65% underwent diagnostic biopsy (64/99), and 63% (66/106) were identified with prenatal imaging. The majority of patients were treated with TPN as well as gastrostomy or ileostomy and CIC, however 15% (18/116) received multivisceral or intestinal transplant, and 30% (22/73) had a vesicostomy. The survival rate was 57% (68/121).
CONCLUSIONS: Appropriate management of
MMIHS patients is crucial. An enlarged, acontractile bladder in a child with bowel motility problems should be considered diagnostic. Bladder distension can be managed with CIC or vesicostomy in addition to prophylactic antibiotics if frequent urinary tract infections are present. These patients often require gastrostomy or ileostomy as well as total parenteral nutrition. This management has led to significant improvement in survival rates.
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