# Acute Primary Small Bowel Volvulus in a Male With Loeys-Dietz Syndrome

## TL;DR

A young man with Loeys-Dietz syndrome, a connective tissue disorder, developed a rare gastrointestinal condition called primary small bowel volvulus.

## Contribution

This case report highlights a potential new gastrointestinal manifestation of Loeys-Dietz syndrome.

## Key findings

- A male with Loeys-Dietz syndrome experienced acute primary small bowel volvulus.
- The volvulus occurred independently, suggesting a possible link to the connective tissue disorder.
- This case expands the known clinical spectrum of gastrointestinal involvement in Loeys-Dietz syndrome.

## Abstract

Loeys-Dietz syndrome (LDS) is a connective tissue disorder with features including, but not limited to, aortic dissections, skeletal abnormalities, and craniofacial defects. However, considering its relatively recent discovery, there are still many unknowns about LDS. The extent of a connective tissue disorder like LDS is yet to be defined throughout the various organ systems, including the gastrointestinal system. Connective tissue disorders have been found to have higher associations with certain conditions, like constipation. In a similar manner, LDS may increase the propensity for developing uncommon gastrointestinal manifestations, like primary small bowel volvulus. A volvulus is defined as an abnormal rotation of the small bowel segment along the axis of its mesentery. Primary small bowel volvulus is differentiated from secondary small bowel volvulus by its nature of origin: primary small bowel volvulus occurs as an independent spontaneous occurrence, whereas secondary small bowel volvulus is secondary to the presence of adhesions, diverticular disease, or abdominal masses. In this case report, we highlight a potential gastrointestinal manifestation of LDS with the occurrence of a primary small bowel volvulus in a young adult male diagnosed with LDS. The patient experienced acute primary small bowel volvulus 14 days into his stay, which may have been influenced by this newfound connective tissue disorder.