Stepwise Fenestration via Natural Orifice Transluminal Endoscopic Surgery for Symptomatic Polycystic Liver Disease

A 60-year-old woman was referred to our hospital with a 2-year history of progressive abdominal distention. Physical examination showed a palpable enlarged liver and mild tenderness in the right upper quadrant. Computed tomography showed countless fluid-filled cysts of various sizes throughout the entire liver (Figure A), consistent with a diagnosis of Gigot type III polycystic liver disease (PLD). After informed consent, fenestration by means of a transrectal natural orifice transluminal endoscopic surgery (NOTES) procedure was proposed (Video). Almost all of the visible cysts on the surface of the liver were incised adequately with a HOOK (Olympus, Tokyo, Japan) or IT knife (Figure B), and they collapsed after releasing large amounts of clear liquid. The endoscope was advanced into the cavity of large cysts to aspirate residual fluid, and all visible additional cysts located in large cysts were fenestrated repeatedly (Figure C). The fenestration procedure continued until all cysts on the hepatic surface had diminished in size (Figure D), with a total aspirated liquid volume of approximately 2000 mL. After careful hemostasis and irrigation of the peritoneal cavity, the intestinal incision was closed with endoclips. Postoperative computed tomography showed a significant reduction in liver cyst burden. The patient experienced no adverse events and had complete symptom resolution, and has been without symptom recurrence after 3 months of follow-up evaluation. Liver transplantation usually is indicated for patients with Gigot type III PLD. NOTES technique-based fenestration and deroofing for symptomatic PLD aims to relieve symptoms resulting from an enlarged liver volume and improve quality of life. Moreover, compared with laparoscopic and transgastric NOTES liver cyst fenestration, our use of the intestinal approach provides better endoscopic surgical space and allows for full fenestration of left and right liver cysts. This could be a less-invasive treatment option for symptomatic PLD. Written informed consent was obtained from the patient for the publication of their information and imaging. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiI1MjcwYjEzNWUzMzk5ZjIwZTVlYTZlYWFkYmFkNzliNiIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNzA1MzcxNDM3fQ.TteGVqwNRnKU_Nb21LJJBmGemIaUZxhYvSAW5LdQOL-S3Ob1dYAz3fUo_sVAhIdFyvhiNBtKX0JpaCGWu4ErnOv1OIgKWcTBkbcOhXpA4brNQyiv9uTFjCRweIqOgpNp-1M6y8c-yhP_I3bjawq4L-16BXIOHVk_TwjkJ9yR4f_vyNhLcC6OTBU5otRsSPmQeaCmeWUcNq4NGmlPmHvKxTJbae9ou73A_-T4hB4avJqnzTMV2ofSgFXGCEyGcH3lOGxTJurvaoLceim9Lpw8TQFthRpl9JukmOJ-HIGAPK1tPuP445EAyv_CF6k2bXtHjoB2lluXj4QOvjI_Xrbk6w Download .mp4 (50.54 MB) Help with .mp4 files Video