Department of Pediatric Surgery, Hospital Federal dos Servidores do Estado, Brazilian Ministry of Health, Rio de Janeiro, Brazil.
Department of Pediatric Surgery, Hospital Federal dos Servidores do Estado, Brazilian Ministry of Health, Rio de Janeiro, Brazil.
J Pediatr Surg. 2024 Dec;59(12):161935. doi: 10.1016/j.jpedsurg.2024.161935. Epub 2024 Sep 14.
Ileal obstruction caused by thick meconium associated with functional immaturity (IOMFI) is an uncommon disease associated with prematurity. IOMFI is not well known, and late or wrong diagnosis is a problem. In this research, we review the clinical characteristics and therapeutic methods of IOMFI.
Critical descriptive literature review.
Most patients eliminate meconium previously to IOMFI. More premature babies tend to become symptomatic in their second week of life, with progressive abdominal distension. The most frequent complication is perforation. Radiologically there is diffuse intestinal distention without air-fluid levels. In contrast enema a caliber transition zone is observed in the distal ileum with multiple filling defects in the ileum and colon. Neural ganglia are present in biopsies from the rectum, colon, and stoma, mostly with characteristics of immature ganglia. Most patients respond to treatment with water soluble contrast enemas. Surgery is needed for patients who do not respond to enemas and those presenting perforations.
IOMFI literature is limited to retrospective mainly small and heterogeneous cohorts. Patients usually respond promptly to water soluble enemas, but a favorable response is highly dependent in contrast reflux through the ileocecal valve and success is related to early treatment. Contrast inflow may be controlled by intermittent radiographies or real time ultrasound.
由不成熟功能相关的厚胎粪引起的回肠梗阻(IOMFI)是一种与早产相关的罕见疾病。IOMFI 并不广为人知,因此存在延误或误诊的问题。在这项研究中,我们回顾了 IOMFI 的临床特征和治疗方法。
关键描述性文献回顾。
大多数患者在发生 IOMFI 之前已经排出胎粪。更多的早产儿倾向于在生命的第二周出现症状,表现为进行性腹胀。最常见的并发症是穿孔。放射学上表现为弥漫性肠扩张,无气液平面。相比之下,灌肠显示远端回肠有口径过渡区,回肠和结肠有多个充盈缺损。直肠、结肠和造口的活检中存在神经节,这些神经节大多具有不成熟神经节的特征。大多数患者对水溶性对比灌肠治疗有反应。对于那些对灌肠治疗无反应和出现穿孔的患者,需要手术治疗。
IOMFI 的文献主要局限于回顾性的、小样本的、异质性的队列研究。患者通常对水溶性灌肠治疗迅速有反应,但良好的反应高度依赖于通过回盲瓣的对比反流,并且成功与早期治疗相关。对比剂流入可以通过间歇性放射学或实时超声来控制。