文/馬偕兒童醫院 田智瑋醫師
Maternal history:
- G1P1
- 40-year-old mother
- Hepatitis B virus carrier
- Pre-natal diagnosis: suspect incomplete Cantrell syndrome
Birth History:
- Cesarean section due to suspected incomplete Cantrell syndrome
- Gestational age:38 weeks
- female, birth weight 2853 g
- Immediately admission to NICU after birth
Figure 1. Gross appearance of the protruding mass
Image Findings
Echocardiography: Elongated diverticulum from left ventricle apex, protruding outside of the chest wall. (Figure 2). The diverticulum showed presence of endocardium, myocardium, and epicardium on echocardiography. Its blind end attached to the umbilical cord. (Figure 3) （超音波下可見源自於左心室心尖的憩室，自胸壁下半突出胸腔外。此突出點下方有完整之肝臟及橫膈膜。憩室包含心內膜、心肌以及心外膜，血液於其中自由流通，且其盲端與臍帶相連。）
Figure 2. LV: Left ventricle.
Arrow: protruding level above liver with intact diaphragm.
（箭頭：從胸骨下方、肝臟上方突出並可見完整之橫膈膜）
Asterisk: The diverticulum connected directly to LV.
（米字：此憩室一端直接和左心室相連，且血液可於其中自由流通）
Figure3: Asterisk: blind end attached to the umbilical cord. Arrow: Presence of endocardium, myocardium, and epicardium
（盲端直接連結臍帶，超音波可見心內膜、心肌以及心外膜）
CT with 3D reconstruction
Apex toward right side (Dextrocardia). Elongated diverticulum from left ventricle apex, protruding outside of the chest wall, with attachment to the umbilical cord.
（心尖朝右且與憩室相通，此憩室遠端連接至臍帶）
Figure4. CT with 3D reconstruction.
Operation
- Dissect out the skin-covered extracardiac LV diverticulum（切開左心憩室外圍之皮膚及皮下組織）
- Dissect out the pericardium （切開心包膜）
- Apply pledgetted 5-0 prolene*5 over the extracardiac LV diverticulum without pericardium Excision of the extra-thoracic LV diverticulum（使用縫線將左心憩室根部綁住並切除）
- Pericardium repair with handmade patch (trimmed 6mm Gore-Tex graft)
- Ligation of umbilicus and excision of the left umbilical cord（使用Gore-Tex補片修補部分心包膜，分離臍帶及切除之左心憩室。）
Figure 4A: Excision of the extra-thoracic LV diverticulum.
Figure 4B: Midline incision of the diverticulum, which showed presence of the 3 layers of heart (endocardium, myocardium, and epicardium)
（切下之左心憩室擁有完整之心臟結構）
Outcome:
- Recovered well. Discharged 19 days after birth
- Follow-up regularly in outpatient departments.
