The reason I became interested in this is due to having exposure to patients who have undergone combined liver/intestine transplants at not-to-be named children's hospital, where I am currently doing my inpatient pediatrics rotation on the gastroenterology service.
Before, I had really only ever thought about isolated liver transplants, most commonly seen in adult medicine due to liver failure or cancer. The need for a combined transplant is something that I did not really appreciate before seeing kids with short gut syndrome in the hospital. Liver transplant can occur by itself, with intestine, and also possibly other organs such as pancreas or stomach (when this happens it is called a 'multi-visceral' transplant).
The children who require a combined liver and intestine transplantation usually have a history of short gut syndrome. Short gut syndrome is essentially when the small intestine is not functioning well or is shorter due to surgical resection. There are many reasons a child might have short gut syndrome. It can be secondary to a variety of etiologies such as necrotizing enterocolitis associated with prematurity (premature infants are particularly vulnerable, because their lungs are not fully developed, and this can cause poor oxygenation of the bowel which allows existing gut bacteria to infiltrate and cause necrosis of the bowel), any sort of atresia (congenital blockage/obstruction of bowel) requiring surgical removal and anastomosis, or complicated gastroschisis (which is essentially when bowel herniates through a full thickness defect in the abdominal wall; in some cases, when the opening is small it can pinch the herniated bowel which loses blood supply and becomes necrotic, necessitating resection). Other reasons have to do with the functioning of the small intestine. There are some kids who have innervation issues (Hirshprung's disease), food allergies (i.e. milk protein), or recurrent infections that make their gut less functional.
Some patients with short gut syndrome develop liver failure associated with receiving nutrition parenterally (a.k.a. via TPN). The reason for this is complex, however the current hypothesis has to do with bile stasis in the liver since the gut is not working to digest as much. For this reason, children with failure to thrive are slowly weaned off TPN and while their tube feeds are increased to avoid this issue. Additionally, the lipid component of the nutrition is carefully controlled as to not overwhelm the liver.
Recently, two of the patients on my service were children who had received combined intestine/liver transplants, and both had complications following transplantation. One of them was transplanted at an early age and subsequently developed hepatocellular carcinoma. He was terminally ill and died in the hospital. The other had acute-on-chronic rejection of the transplanted liver.
A phrase doctors like to say is "it's always about the patient". Although pathologists have minimal to no contact with patients on a day-to-day basis, the information pathologists supply to the clinical team is crucial. Another adage is that a physician who treats himself/herself has a fool for a patient.
Can you think of any other instances in which multiple transplants would be indicated? Share!
