Abstract
BACKGROUND: Ehlers-Danlos syndrome (EDS) is a rare genetic disorder that causes abnormal collagen structure and production, seriously impacting the quality of connective tissues. Reconstructive surgery can be challenging in affected patients, and additional precautions should be taken for microsurgical transfers. CASE DESCRIPTION: This case aimed to describe the management of a 27-year-old man with vascular EDS and a history of heavy smoking who developed a voluminous enterocutaneous fistula after multiple abdominal surgeries. Due to the high surgical risk of flap failure resulting from the patient's condition, the large full-thickness abdominal defect, and the lack of locoregional reconstructive options, a two-stage free latissimus dorsi flap reconstruction was performed. A left myocutaneous free latissimus dorsi flap (sized 10 cm × 25 cm) was transferred and anastomosed to the left superficial femoral artery and the proximal part of the rerouted greater saphenous vein. The flap was folded, sutured to itself, and left in place for 8 days. Once the flap's viability was confirmed, complete small bowel liberation with resection of the enterocutaneous fistula and end-to-end primary anastomosis were performed by the visceral surgeons. The latissimus dorsi flap was unfolded and moved cephalically to cover the defect. No complications were reported on the flap. A fistula recurrence occurred on postoperative day 9 but was successfully addressed within 6 weeks using a combination of nasogastric tube aspiration, somatostatin, antibiotics, and negative pressure therapy. Follow-up at 6 months showed complete wound healing with no further complications. CONCLUSIONS: This report suggests the two-stage free flap transfer strategy to manage a voluminous full-thickness abdominal wall defect in a patient with vascular EDS. This approach allowed for optimal tissue coverage and full abdominal restoration while minimizing the risk of complications.