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Case Report
Congenital
Cystic
Adenomatoid
Malformation
彰濱秀傳紀念醫院
放射師 陳立珣 姚宜君
25歲女性,G1P0, 無特殊病史,
於妊娠23週來院接受中期
胎兒超音波檢查
超音波影像發現
超音波影像發現
Multiple large cysts > 2cm,
Smaller cysts can also be found
Alveolus-like structures
超音波影像發現
CCAM volume = length x width x height of the lesion x 0.52
Fetal CVR = CCAM volume / head circumference
this case = (5.05 x 4.45 x 4.20) x 0.52 / 21.54 = 2.28
超音波影像發現
Mediastinal shift
Pulmonary hypoplasia
Doppler影像發現
UA
DV
MCA
Discussion
Congenital Cystic
Adenomatoid Malformation
Definition
A pulmonary anomaly characterized by
overgrowth of terminal bronchioles.
Usually unilateral with variable size
and rate of growth giving rise to clinical
presentations, ranging from
intrauterine fetal demise with hydrops
to discovery in childhood after
recurrent pulmonary infections.
Type I , 50%Type II, 40%
Type III,
10%
Congenital Cystic
Adenomatoid Malformation
Cyst < 10mm Cyst in 2-7 cm
Larger in number Few in number (1-4)
Hydrops,
Pulmonary hypoplasia
Favorable prognosis
CAM Volume Ration
CVR< 1.6 = favorable prognosis
The average gestational age at the CCAM
growth is reached is 26weeks.
Hydrops?
Placental swelling?
Doppler finding?
CCAM volume = length x width x height of the lesion x 0.52
Fetal CVR = CCAM volume / head circumference
Associated anomalies:
The condition is usually isolated.
Unlike diaphragmatic hernia there is no association with
chromosomal defects.
In about 10% of cases there are additional malformations :
· renal abnormalities (bilateral renal agenesis)
· abdominal wall abnormalities
· central nervous system defects (hydrocephalus)
· spinal deformities (cervical spine/thoracic spine)
· gastrointestinal defects
(diaphragmatic hernia, jejunal atresia, tracheoesophageal fistula)
· cardiac anomalies and anomalies of the great vessels
(ventricular septal defect, tetralogy of Fallot, truncus arteriosus)
· sirenomelia (including agenesis of ureters, bladder, urethra, uterus, cervix,
vagina, gallbladder, descending colon, sigmoid colon and rectum, and imperforate
anus)
Differential diagnosis:
✓Diaphragmatic hernia
http://sonoworld.com/TheFetus/page.aspx?id=1248
✓Pulmonary sequestration
✓Mediastinal lesions:
enterogenous cyst, neurenteric cyst, or
cystic teratoma
✓Bronchogenic cyst
Fetal Intervention
Take Home Messege
Cyst大小
確認正常肺組織
計算CVR
是否合併其他異常或Hydrops
鑑別診斷
Thanks for your attentions 彰濱秀傳紀念醫
院

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CCAM Congenital Cystic Adenomatoid Malformation