Simultaneous Bilateral Lung and Pancreas Transplantation in
Recipient With Cystic Fibrosis
J.A. Fridell, T.C. Wozniak, J.A. Powelson, and J.M. Reynolds
ABSTRACT
Introduction. Cystic fibrosis (CF) is an inherited disorder that presents in childhood as a
multisystem disease. Pulmonary failure and pancreatic insufficiency, including CF-related
diabetes (CFRD) and exocrine insufficiency, are significant causes of morbidity and
mortality in these patients. In this report we have reviewed our experience with a
simultaneous lung and pancreas transplantation in a patient with CF.
Methods. The recipient was a 25-year-old man with CF complicated by bronchiectasis
with recurrent episodes of pneumonia, pancreatic exocrine insufficiency, and CFRD. He
had normal hepatic and renal function.
Surgical Technique. The lung and pancreas allografts were procured from a single
cadaveric donor. The double lung transplantation was performed through separate
thoracic incisions. The pancreas transplantation was performed through a midline incision
with systemic venous drainage and proximal enteric exocrine drainage.
Results. The recipient recovered well from his transplantation with early extubation. The
pancreas allograft functioned well with normal blood glucose independent of insulin. As a
result of the enteric drainage of the pancreas allograft, the patient no longer required
supplemental pancreatic enzymes. His postoperative course was complicated by distal
intestinal obstruction, a complex wound infection, and reversible leukoencephalopathy. At
1-year posttransplantation he remains free of supplemental oxygen, insulin, and pancreatic
enzyme replacement.
Conclusion. Simultaneous lung and pancreas transplantation in a patient with CF was
performed safely, providing the advantages of normalization of glucose and improved
nutrition for a patient requiring lung transplantation.
C
YSTIC FIBROSIS (CF) is an inherited disorder that
affects epithelial chloride transport presenting in
early childhood as a multisystem disease. This autosomal
recessive disorder has an incidence of 1:2500 among the
white population. It is the most commonly inherited condi-
tion leading to premature death. Due to improvements in
pulmonary therapy, the predicted median survival for pa-
tients with CF in the year 2000 was 32.2 years.
1
As survival
improves, more patients are living long enough to develop
extrapulmonary complications.
2
Despite optimized therapy,
however, more than 95% of affected individuals ultimately
die of respiratory failure. Lung transplantation is the only
available therapy that deals definitively with the end-stage
pulmonary disease. It has become the treatment of choice
for these patients. Recently quoted survivals rates for
bilateral sequential lung transplantation in recipients with
CF are 70%– 80% at 1 year, 55%– 60% at 5 years, and
32%–38% at 10 years.
3–8
Pancreatic failure, manifesting as pancreatic exocrine
insufficiency and CF-related diabetes (CFRD), is a common
comorbidity in CF patients. Pancreatic islet cell transplan-
tation at the time of lung transplantation has been previ-
ously attempted with modest improvement in insulin re-
From the Indiana University School of Medicine, and Method-
ist Hospital, Indianapolis, Indiana, USA.
Address reprint requests to Jonathan A. Fridell, MD, Assistant
Professor of Surgery, Director of Pancreas Transplantation,
Indiana University School of Medicine, 550 N University BLVD,
#4258, Indianapolis, IN 46202. E-mail: jfridell@iupui.edu
0041-1345/08/$–see front matter © 2008 by Elsevier Inc. All rights reserved.
doi:10.1016/j.transproceed.2008.01.010 360 Park Avenue South, New York, NY 10010-1710
494 Transplantation Proceedings, 40, 494 – 497 (2008)