RSNA2018读片会病例挑战（5）：66岁，慢性腹痛急性加重，既往有痛风、高血压及“先天性肺气肿”病史

2018 RSNA

Image Interpretation Session

Abdomen: GI

History

• 66 year-old male with chronic abdominal pain

• Acute exacerbation

• Chronic medical history, includes

– Gout

– Hypertension

– “Congenital emphysema”: Rx inhalers and prn steroids

• Recurrent pneumothoraces

• Recurrent infections

• Chronically short of breath

History

• Relevant Surgical history

– Bilateral carpal tunnel releases; right side 1970’s

– Bilateral finger amputations: etiology?

– Hx of intussusception: small bowel resection

• Relevant Surgical history

• Bilateral carpal tunnel releases; right side 1970’s

• Bilateral finger amputations: etiology?

Findings: X-ray

• Third finger amputation

• Macrodactyly (disproportionate overgrowth)

• Course trabeculation of enlarged phalanxes

• Soft tissue hypertrophy (metacarpus and around second digit)

• Abnormally calcified connective tissue

Findings: MR

• Soft-tissue fibrotic lesion (low signal on T1/T2), i.e. fibrous hamartoma

• Bone cystic lesions

Findings

•Overgrowth of connective tissues (bone, fat)

• Progressive disease

•Onset at young age

Findings

• “Congenital emphysema”: Rx inhalers and prn steroids

• Recurrent pneumothoraces

• Recurrent infections

• Chronically short of breath

Findings

• Chest X-ray

• Asymmetric hemithoraces; hyperinflation of left

lung

• Redistribution of blood flow to the upper lung

zones with abnormal enlargement of the upper

lobe vessels

• Prominent hila with reticular and streaky

densities in both perihilar areas

Findings

• Lung CT

• Hyperinflation of the left lung

• Emphysematous changes

• Areas of scarring and cystic changes in the left lower lobe

History

• Hx of intussusception: small bowel resection

Findings

• Multiple encapsulated lipomas in the stomach, duodenum and jejunum

• Fatty overgrowth in the omentum

• Lipohypoplasia of subcutaneous fat

• Dysregulated adipose tissue

• Splenomegaly

• ?Thick wall of the right ventricle

DIFFERENTIAL DIAGNOSIS

• Macrodystrophia lipomatosa

• Multiple symmetric lipomatosis (Madelung disease)

• CLOVE (Congenital Lipomatous Overgrowth, Vascular malformations, and Epidermal nevi, Skeletal) syndrome

• Proteus syndrome

---

与往年一样，医学影像学英语公众号将继续组织RSNA中稿及参会者微信群，只要中稿或参会的朋友均可加小编好友入群。另外，今年的中稿者还可免费参加《每天朗读一段医学影像学英语》小打卡活动。欲入群及加入小打卡活动者，请加小编（微信号：524861255）好友，加好友请注明RSNA2018中稿（或参会）+姓名+单位（或学校）。入群请修改群昵称为姓名+单位（或学校）。拒不实名者将被请出。

FROM：http://archive.rsna.org/2018/Final%20Unknown%20Cases%2011.12.18.pdf