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Published on: June 11, 2019
Case Report: Spleen-preserving Multivisceral Transplant for Peutz-Jeghers Syndrome
L Moulin1, N Pedraza1, J Padin2
1Instituto de Trasplante Multiorgánico, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
Insights
A multivisceral transplant successfully treated a patient with Peutz-Jeghers syndrome and extensive gastrointestinal polyposis. The patient achieved long-term survival with an excellent quality of life post-transplant.
Area of Science:
- Gastroenterology and Hepatology
- Transplant Surgery
Background:
- Peutz-Jeghers syndrome (PJS) is a rare genetic disorder characterized by hamartomatous polyps in the gastrointestinal (GI) tract, increasing cancer risk.
- Patients with PJS often require multiple surgeries for complications like intussusception, leading to significant morbidity.
Observation:
- A 24-year-old man with PJS presented with over 150 GI polyps, some with high-grade dysplasia, despite maintaining intestinal sufficiency.
- A modified multivisceral transplantation was deemed necessary due to the extensive polyposis and dysplasia.
Findings:
- The multivisceral transplant procedure involved complex reconstruction of vascular and biliary systems, including aortic and portal vein anastomoses, and duct-to-duct biliary reconstruction.
- Post-transplant, the patient experienced a short intensive care unit stay and initiated enteral feeds on day 7.
- At 23 months post-transplant, the patient remains alive with an excellent quality of life, indicating successful graft function and disease management.
Implications:
- This case highlights the potential of multivisceral transplantation as a viable option for managing severe, refractory gastrointestinal polyposis in PJS.
- Successful long-term outcomes underscore the importance of considering advanced surgical interventions for rare genetic disorders with significant GI manifestations.
- Further research into the long-term efficacy and management protocols for PJS patients undergoing transplantation is warranted.
Case Report:
A 24-year-old man diagnosed with Peutz-Jeghers syndrome as a child underwent multiple surgeries owing to intussusception. Pretransplant workup showed >150 polyps along the gastrointestinal (GI) tract, some of them with high-grade dysplasia. Despite having intestinal sufficiency, a modified multivisceral transplantation was offered.
Procedure:
An 18-year-old donor was procured using University of Wisconsin solution. The recipient's surgery started with a midline incision. Mobilization of the right colon and the root of the mesentery was done to isolate the superior mesenteric artery. The same maneuver was done with the left and sigmoid colon. The common bile duct was then isolated and transected at the cystic duct level. The abdominal portion of the esophagus and the proximal stomach were isolated and divided at the gastroesophageal junction. After that, the pancreas was mobilized, preserving the spleen with the splenic vessels. The distal GI tract was transacted at the level of the proximal rectum. For engraftment, an arterial conduit was placed in the infrarenal aorta and anastomosed to the graft's aortic patch. End-to-side portal reconstruction was made at the level of the portal vein, allowing performing a duct-to-duct biliary reconstruction over a 5-Fr T-tube. A hand-sewn gastrogastric anastomosis and piloroplasty were performed; the distal anastomosis was done with circular staplers. A gastrojejunostomy and a loop ileostomy were the final steps of the procedure.
Results:
The patient stayed in intensive care for 2 days and enteral feeds were started on day 7. Currently, 23 months after transplant he is alive with an excellent quality of life.
