【Q024】十二指肠狭窄综合征（LADD综合征）

【双语病例】 10-day-old newborn with vomiting...

双语学影像；本期病例选自AuntMinnie.com

Our appreciation is extended to Dr. Ameya Nayate, University of Pennsylvania Department of Radiology, for contributing this case.

History and Radiograph

History

A 10-day-old newborn with vomiting.

An abdominal radiograph is shown below. Click image below to enlarge.

1.Which choice best characterizes the image?

Malrotation 旋转不良

Mass effect with displaced bowel loops 肠袢移位伴占位效应

Abnormal proximal bowel 近端肠管异常

Abnormal distal bowel 远端肠管异常

Abnormal bowel wall 肠壁异常

GI images

2.Images confirm abnormal dilation of the duodenum.

GI图像证实十二指肠异常扩张。

True

False

3.Images show no contrast advancing past the duodenum.

图像示没有造影剂通过十二指肠。

True

False

4.Overall picture is most consistent with acute midgut volvulus.

以上图像反映了急性中肠扭转.

True

False

Findings and diagnosis

Findings

There is gas within the stomach and duodenum. There is contrast in the bowels due to prior swallow study. There is dilation of the duodenum with no contrast advancing past the duodenum.

胃和十二指肠内均可见气体影。曾口服造影剂，肠道内可见造影剂影。十二指肠内扩张，造影剂不能顺利通过十二指肠。

Differential Diagnosis

• Duodenal atresia

  十二指肠先天性闭锁

• Malrotation

  扭转不良

• Annular pancreas

  环形胰腺

• Duodenal web

  十二指肠蹼

Diagnosis

Duodenal stenosis, cause unknown

十二指肠狭窄，原因不明

Additional Questions

5.Duodenal stenosis commonly occurs distally to the ampulla of Vater.

十二指肠狭窄通常发生在Vater壶腹远侧。

True

False

6.Duodenal stenosis is often associated with prematurity, cardiac abnormalities, and trisomy 21.

十二指肠狭窄常合并早熟、心脏异常、21三体综合征。

True

False

7.With timely surgical repair, survival rates are excellent.

及时行手术治疗，患者的生存率很高。

True

False

答案：

1. Abnormal proximal bowel

   There is a so-called double-bubble sign (gas within the stomach and duodenum).

   胃和十二指肠内可见气体，称为双泡征。

2. True

3. True

4. False

5. True

   Duodenal stenosis commonly occurs distal to the ampulla of Vater, which results in bilious emesis.

   十二指肠狭窄常发生在Vater壶腹远侧，引起胆汁性呕吐。

6. True

7. True

   With surgical repair, survival rate is greater than 90%

   手术修复后，生存率可达到90%以上。

Discussion

• Duodenal stenosis is a common cause of upper-bowel obstruction in neonates. The more common causes include duodenal atresia, malrotation, annular pancreas, and duodenal web. Though the causes are many, each presents a similar clinical picture with vomiting shortly after birth, dehydration, weight loss, and electrolyte imbalance. The stenosis commonly occurs distally to the ampulla of Vate, which results in bilious emesis. Duodenal stenosis is often associated with prematurity, cardiac abnormalities, and trisomy 21.

  十二指肠狭窄是婴儿常见的近段肠道梗阻性病变，常见的病因包括先天性闭锁、肠扭转、环形胰腺、十二指肠蹼。梗阻的病因很多，但临床一般表现为出生后不久发现呕吐、脱水、体重减轻、电解质紊乱。梗阻常发生于Vater壶腹远侧，可引起胆汁性呕吐。十二指肠狭窄常合并早熟、心脏异常、21三体综合征。

• If left untreated, duodenal stenosis can lead to death. With surgical repair, survival rate is greater than 90%. The type of repair depends on the cause of the stenosis.

  十二指肠狭窄发现后如果不加治疗，可导致死亡。手术修复后，存活率可达到90%以上。修复的方式根据狭窄原因不同而不同。

Radiologic overview of the diagnosis放射

The most helpful radiologic finding is the 'double-bubble' sign, which describes gas within the stomach and proximal duodenum. If this is clearly seen, duodenal stenosis can be confidently diagnosed and no other imaging study is needed. If the duodenal jejunal junction is not visualized, an upper GI should be performed to rule out midgut volvulus.

X线最主要的征象是“双泡征”，即胃和十二指肠近段可见气体影。如果能够发现这一征象，即可明确诊断，无需进行其他检查。如果十二指肠-空肠交界区无法清晰显示，则需要行上消化道造影排除中肠扭转。

In this case, the abdominal radiograph demonstrates gas within the stomach and duodenum. The duodenal jejunal junction is not clearly visualized and an upper GI was obtained to rule out malrotation. During the upper GI procedure, the proximal duodenum is dilated and contrast fails to advance past the second segment of the duodenum. Given the dilation of the proximal duodenum, the stenosis is chronic and an acute volvulus is an unlikely etiology. Possible causes of chronic stenosis leading to duodenal dilation include annular pancreas, in-utero volvulus, and duodenal atresia.

本例患者，胃和十二指肠内可见气体密度影，但十二指肠-空肠交界区显示不清，所以行上消化道造影以排除中肠扭转。造影显示十二指肠扩张，造影剂无法顺利通过十二指肠二段。由于十二指肠近端是扩张的，所以考虑病变为长期、慢性病变，急性肠扭转不是疾病的病因。慢性狭窄导致十二指肠扩张的常见病因包括：环形胰腺、宫内肠扭转、十二指肠先天闭锁。

Key Points

• Duodenal stenosis is the most common type of uppe- bowel obstruction in neonates.

  十二指肠狭窄是婴儿最常见的近段肠道梗阻性病变。

• The stenosis is usually distal to the ampulla of Vater.

  狭窄一般发生在Vater壶腹远侧。

• A 'double-bubble' sign with air in the stomach and duodenum is highly suggestive of duodenal stenosis.

  胃和十二指肠内均可见气体，即“双泡征”，高度提示十二指肠狭窄。

• Dilation of the proximal duodenum occurs when the stenosis is chronic in nature and does not occur in the acute setting, such as acute midgut volvulus.

  十二指肠近端扩张一般是长期慢性过程导致，所以病因不会是急性的病变，如急性中肠扭转。

——经典内容——