A Unique Association with Persistent Truncus Arteriosus and Partial

Midline Chest Wall Defect

I
n our first case, a male newborn delivered at 34 weeks of
gestation weighing 3.1 kg was admitted because of cyanosis
and respiratory distress. Echocardiogram showed persistent
truncus arteriosus and tricuspid atresia with mild truncal valve
insufficiency. He had the defects of subdermal tissue and the
lower sternum (Figure 1, A). He underwent bilateral
pulmonary arterial banding at 6 days of age and bidirectional
cavopulmonary shunt at 11 months of age.
In our second case, a female newborn delivered at 41 weeks
of gestation weighing 2.7 kg was admitted because of respira-
tory distress. Echocardiogram revealed persistent truncus
arteriosus with moderate truncal valve insufficiency and
supracardiac total anomalous pulmonary venous connec- Figure 2. Sagittal sections of computed tomography in the A,
tion. She also had the midline tissue defect similar to the first first case and B, the second case show the defect of sub-
case (Figure 1, B). She underwent primary correction at 12 dermal tissue and lower sternum (circled).
days of age, however, she died the next day because of
heart failure. Computed tomography in both cases showed
the similar defects of the subdermal tissues and the lower the lateral plate mesoderm and truncal septum is formed by
parts of sternum (Figure 2). Both the abdominal wall and migrated neural crest cells. We assume that this unique
diaphragm were intact. These findings suggested a variant association with conotruncal cardiac defect and the partial
of midline chest wall defect. midline chest wall defect can be responsible for the
The midline chest wall defect described is classified as part developmental disorder of lateral plate mesoderm in partial
of the spectrum of midline ventral wall defects such as cleft somite. n
sterunum, pectoris cordis, and the pentalogy of Cantrell.1 In
embryological development, the ventral wall is formed by Jun Muneuchi, MD
the meeting of parietal layer of lateral plate mesoderm and Yuichiro Hirata, MD
overlying ectoderm in the midline by the fourth week of post- Division of Pediatrics
fertilization.2 Truncus arteriosus is also differentiated from
Yoshie Ochiai, MD
Division of Cardiovascular Surgery

Kunitaka Joo, MD
Division of Pediatrics
Kyushu Koseinenkin Hospital
Kitakyushu, Fukuoka, Japan

References

1. Engum SA. Embryology, sternal clefts, ectopia cordis, and Cantrells pen-
talogy. Semin Pediatr Surg 2008;17:154-60.
2. Sadler TW. The embryologic origin of ventral body wall defects. Semin
Pediatr Surg 2010;19:209-14.

Figure 1. The appearances of the chest in the A, first case

and B, the second case. The midline tissue defect in the
length of 2 cm was noted in both cases.

J Pediatr 2012;161:564.
0022-3476/$ - see front matter. Copyright 2012 Mosby Inc.
All rights reserved. 10.1016/j.jpeds.2012.04.018

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