圣诞树型回肠闭锁伴肠道旋转不良的15日龄婴儿:儿科人群中罕见的双重先天性异常-一例报告

IF 0.7 Q4 SURGERY
Mathayo Shadrack , Mwajabu Rashidi Mbaga , Sylvanus Christopher , Linda Kalisti Tilisho , Ibrahim Mkoma , Victor Ngotta
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引用次数: 0

摘要

背景肠闭锁是新生儿肠梗阻的常见原因。IIIb型闭锁,也被称为“圣诞树”或“苹果皮”闭锁,是一种罕见而严重的变型,其特征是肠系膜缺失和远端小肠缠绕在单个动脉血管周围。发病率低,约占小肠闭锁的5 - 10%。由于胚胎期正常肠道旋转失败而发生的肠道旋转不良也很少见,可导致肠扭转和十二指肠梗阻。这两种异常的共存是非常罕见的。病例介绍:我们报告一个15天大的男性新生儿出现进行性腹胀、胆汁性呕吐和高钾血症。腹部x线片显示有小肠梗阻的特征,而超声则没有结论。剖腹探查显示IIIb型回肠闭锁伴典型的“圣诞树”构型,远端肠塌陷。此外,不完全的肠旋转被确定为自由漂浮的盲肠和右侧十二指肠空肠交界处。手术切除远端塌陷的回肠、盲肠和部分升结肠,然后造双口(回肠造口术和结肠造口术)。术后顺利,造口功能良好。本病例强调了罕见的双先天性异常所带来的诊断和手术挑战。圣诞树姿势增加了患短肠综合症的风险,而旋转不良则增加了肠扭转的可能性。早期手术干预至关重要,特别是在资源有限的情况下。结论临床医生在评估新生儿肠梗阻时应考虑并发异常。即使在复杂的病例中,及时的手术和个性化的治疗也能带来良好的结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Christmas tree pattern ileal atresia with intestinal malrotation in a 15-day-old infant: A rare dual congenital anomaly in the pediatric population – A case report

Background

Intestinal atresia is a common cause of neonatal intestinal obstruction. Type IIIb, also known as “Christmas Tree” or “Apple Peel” atresia, is a rare and severe variant characterized by the absence of the mesentery and a distal small bowel coiled around a single arterial vessel. Its incidence is low, accounting for 5–10 % of small bowel atresias. Intestinal malrotation, occurs due to failure of normal embryological gut rotation, is also rare and can predispose to volvulus and duodenal obstruction. The coexistence of both anomalies is extremely uncommon.

Case presentation

We report a 15-day-old-male neonate with progressive abdominal distension, bilious vomiting, and hyperkalemia. Abdominal radiographs showed features of small bowel obstruction, while ultrasound was inconclusive. Exploratory laparotomy revealed type IIIb ileal atresia with the classical “Christmas Tree” configuration and collapsed distal bowels. Additionally, incomplete intestinal rotation was identified with a free-floating cecum and right-sided duodenojejunal junction. Surgical resection of the distal collapsed ileum, cecum, and part of the ascending colon was performed, followed by creation of a double stoma (ileostomy and colostomy). The postoperative period was uneventful with good stoma function.

Discussion

This case highlights the diagnostic and surgical challenges posed by a rare dual congenital anomaly. The Christmas Tree pattern increases the risk of short bowel syndrome, while malrotation adds potential for volvulus. Early operative intervention is essential, especially in resource-limited settings.

Conclusion

Clinicians must consider coexisting anomalies when evaluating neonatal intestinal obstruction. Prompt surgery and individualized management can lead to favorable outcomes even in complex cases.
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来源期刊
CiteScore
1.10
自引率
0.00%
发文量
1116
审稿时长
46 days
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