Keywords:
Volvulus, Congenital, Barium meal, CT, Conventional radiography, Veins / Vena cava, Anatomy
Authors:
C. Vila1, A. Villanueva Marcos2, O. Persiva Morenza1, R. Vidal1, R. Ahmed3; 1Barcelona/ES, 2Huntingdon/UK, 3Redhill/UK
DOI:
10.26044/esti2019/P-0086
Imaging findings OR procedure details
Five patients with HLAI were discovered between 2014 and 2018 (Figues Fig. 1 ,
Fig. 2 ,
Fig. 3 ,
Fig. 4 and Fig. 5 ).
All of them had a left-sided heart and a right stomach bubble.
Most of them have a prominent azygos vein.
Out of five three had enhanced CT and one had a barium small-bowel study.
The remaining patient had only chest and abdominal radiographs.
All patients were asymptomatic.
Two of them were discovered in preoperative chest radiographs and another two in routine check-ups.
The fifth one was an 11 y.o.
child with chickenpox.
Key features for suspecting the diagnosis were the enlarged azygos in the chest radiograph and the gastric bubble on the right side in all five patients.
The three patients with CT had inversion of the abdominal viscera,
as well as polysplenia and two of them have azygos continuation of the IVC.
The patient with barium study showed a right-sided stomach and intestinal malrotation.
The last patient showed inversion of the abdominal viscera in the plain film of the abdomen.
Comparison with a chest radiograph taken ten years earlier did not show any change.
Table 1.
Summary of the features in 5 patients with Heterotaxy syndrome with laevocardia and abdominal inversion.
|
Prominent Azygos |
Levocardia |
Right Stomach |
Midline liver |
Polysplenia |
Case 1 |
Yes |
Yes |
Yes |
? |
? |
Case 2 |
Yes |
Yes |
Yes |
? |
? |
Case 3 |
Yes |
Yes |
Yes |
Yes |
Yes |
Case 4 |
No |
Yes |
Yes |
Yes |
Yes |
Case 5 |
Yes |
Yes |
Yes |
Yes |
Yes |
There are very few cases published of HLAI (1-3).
All of them emphasize the rarity of this condition and that 90% of them have severe cardiac disease.
We don’t believe the condition is so unusual as we discovered five cases in a relatively short period of time,
all of them symptomless.