Case Report: Spleen-preserving Multivisceral Transplant for Peutz-Jeghers Syndrome.
METHODS
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.


METHODS
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.
Instituto de Trasplante Multiorgánico, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
L
L
Moulin
Instituto de Trasplante Multiorgánico, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
N
N
Pedraza
Servicio de Cirugía, Clínica "La Pequeña Familia", Ciudad de Junín, Argentina.
J
J
Padin
Servicio de Anestesiología, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
S
S
Niveyro
Unidad de Terapia Intensiva y Trasplante, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
G
G
Tuhay
Hepatología y Gastroenterología Pediátrica, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
C
C
Rumbo
Instituto de Trasplante Multiorgánico, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
P Barros
PB
Schelotto
Instituto de Trasplante Multiorgánico, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
A
A
Crivelli
Instituto de Trasplante Multiorgánico, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
H
H
Solar Muñiz
Instituto de Trasplante Multiorgánico, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina.
D
D
Ramisch
Instituto de Trasplante Multiorgánico, Hospital Universitario Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina. Electronic address: ggondolesi@ffavaloro.org.
G
G
Gondolesi
D000293:Adolescent; D006801:Humans; D008297:Male; D016377:Organ Transplantation / Q000379:methods*; D010580:Peutz-Jeghers Syndrome / Q000601:surgery*; D013154:Spleen / Q000601:surgery*; D055815:Young Adult