Chapter 1 of 4 · Case-Based Learning
Case presentation
Case presentation: 6-year-old with heterotaxy, situs inversus, and suspected malrotation
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Host summaries · secondary, not cited in answers
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
In heterotaxy syndrome with volvulus, operative intervention is mandatory.
In right atrial isomerism, there is a higher incidence of malrotation compared to left atrial isomerism.
In the speaker's institutional data, children with right atrial isomerism were the ones that developed volvulus.
Only one patient with left atrial isomerism had any real issues, and it was not a true volvulus.
Failure to thrive should be considered a GI symptom warranting laparoscopic exploration to divide Ladd bands and perform appendectomy in heterotaxy patients.
Appendectomy is not without its long-term obstruction rate from adhesions.
In situs inversus with malrotation, the appendix is in the correct location (a double negative), so appendectomy may not be indicated.
In heterotaxy, the cecum will be floppy, warranting inversion appendectomy if laparoscopy is performed.
Malrotation is defined by low-lying ligament of Treitz, not by structures crossing the midline, as structures can be floppy and cross midline without excluding malrotation.
The speaker has seen 3-4 cases (in addition to half a dozen during fellowship) where a good C-loop crossed midline but the patient had low-lying ligament of Treitz and was malrotated on exploration.
A baby with low-lying ligament of Treitz and reassuring imaging was observed and subsequently returned with volvulus.
In a Texas Children's series of 95 consecutive heterotaxy patients with malrotation, three-quarters underwent Ladd procedure with no post-operative volvulus but 11% small bowel obstruction rate requiring admission and often surgery.
In the Texas Children's series, 25% of heterotaxy patients were observed with no small bowel obstruction and no volvulus over 10-15 year follow-up.