He was diagnosed with jejunoileal atresia at birth. He underwent multiple intestinal resections, after which 20 cm of small intestine remained. As a consequence, he required total parenteral nutrition, and had been known to have elevated liver function tests and hepatitis in the past. He had a history of renal insufficiency, which had been attributed to multiple courses of antibiotics administered for central line sepsis and pneumonia. He also had undergone a cholecystectomy for cholelithiasis.
The patient received a small intestine and kidney transplant at Children's Hospital of Pittsburgh (CHP) in June. The 26-hour surgery was complicated by intermittent poor venous drainage of the graft, which was eventually corrected, and significant graft edema and swelling, which necessitated leaving the abdomen open. Postoperatively, he spent approximately one month in the Intensive Care Unit (ICU) with prolonged periods of intubation complicated by asthma and pleural effusions. He had multiple endoscopies and biopsies which showed cellular rejection as early as four days post-transplant. He received Solumedrol and FK 506 immunosuppression. During his stay, he had transient episodes of hypotension which required fluids and pressors. He had several episodes of central line sepsis and infection of his abdominal wound, which were treated with antibiotics. Surveillance biopsies of the allograft at 2 months, 26 days post-transplant showed Epstein-Barr virus infected cells as well as mild rejection. The last series of biopsies prior to his discharge at 3 months, 8 days post-transplant showed cellular rejection and mild glandular distortion. Throughout his hospital stay, he required large volumes of fluid replacement for ascites and stomal output. At the time of discharge in September, he was managed with tube feedings, and gradually developed a good appetite and followed a regular diet. Granulation tissue formed which covered the open abdominal wound.
The patient was readmitted for fever and increased stomal and rectal output with bleeding. Blood cultures grew Enterococcus and Pseudomonas, which required antibiotic therapy. He had persistent intestinal bleeding which was attributed clinically to either or thrombocytopenia. He required blood products and fluids. OKT3 was started for persistent rejection of the small intestinal graft. He also became hyponatremic, which was attributed to large stomal losses, and which required supplementation. Episodes of hypotension from his intestinal bleeding required stays in the ICU. In addition to OKT3, he was receiving Imuran, mycophenolic acid, and Prednisone. The patient stabilized and was relatively well for the remainder of this hospitalization.
In October, the patient and his parents were dining at a restaurant when he became ill and complained of chest pain. He was described as having a seizure-like episode, and then hunched over the table. Paramedics found that he had atrioventricular dissociation (heart block) and low blood pressure. He arrested in the CHP emergency room where he required chest compressions, intubation, and high-dose epinephrine. He was immediately admitted to the ICU where he experienced four additional episodes of complete heart block. Cardiac function became normal by echocardiogram. His cardiac rhythm stabilized after placement of a pacemaker. Endoscopy of the allograft revealed exfoliation of the mucosa. Severe rejection with sepsis was suspected. In view of his worsening clinical status and appearance of the intestinal graft, the patient was taken to the operating room for graft enterectomy. While the abdomen was being opened, there was excessive bleeding, and he became increasingly hemodynamically unstable with progression to complete cardiovascular collapse. High dose pressors, fluids, and external chest compressions were started. Bilateral chest tubes were placed. Blood gases demonstrated increasing acidosis and decreasing pO2. Resuscitation efforts were halted after 45 minutes and the patient was pronounced dead. Autopsy permission was granted for thoracic and abdominal contents only.