Charles D. Vinocur

Isolated liver transplantation for liver failure in patients with short bowel syndrome

The ultimate prognosis for patients with short bowel syndrome (SBS) has become progressively more favorable over the past decade. Advances in long-term total parenteral nutrition (TPN) have allowed this group of patients to meet nutritional needs while the process of intestinal adaptation occurs. Unfortunately, a subgroup of patients with SBS have hepatic failure (HF), most often secondary to TPN-induced cholestasis. Combined small bowel and liver transplantation (LT) offers a sound anatomic solution for cases of HF with SGS, but it remains experimental at this time. We propose that an isolated LT is a viable alternative mode of therapy for the patient with HF and SBS. The following characteristics were reviewed for five patients with SBS and HF who underwent LT: age at transplantation, weight, liver function, survival, intestinal length, volume of feeding before surgery, and current feeding tolerance and liver function. Four boys and one girl, aged 5.5 to 15 months (average, 11.9), had LT. The total bilirubin level at the time of transplantation was 14.4 to 37 mg/dL (average, 24.7). The patients weighed between 3.8 and 12 kg (average, 8.0), and feeding tolerance ranged from no enteric to complete enteric feeding (average, < 33% of calories by enteric feeding). Bowel loss was attributed to necrotizing enterocolitis in two cases, volvulus in two, and birth hypoxia in one. Bowel length ranged from 60 to 120 cm (average, 88.6). Four children (80%) survived LT, and the average follow-up period was 9.3 months. Three (75%) are home; one is on combined hyperalimentation and enteral feeding, and two are on full enteric feeding. One remains in a chronic care facility, on combined enteral and intravenous feeding. The average daily enteral feeding now comprises more than 70% of caloric requirements. The total bilirubin level is .6 to .8 mg/dL (average, .71). Isolated LT for HF in the patient with SBS effectively restores liver function, allowing time for further intestinal adaptation.

Is age less than 1 year a high-risk category for orthotopic liver transplantation?

The aim of this study was to determine if age less than 1 year is a high risk group for orthotopic liver transplantation (OLT). Retrospective analysis was done of patients with liver failure who received OLT. Comparison was made between patients aged < 1 year and > 1 year with regard to survival, allograft survival, hepatic artery thrombosis, and medical status at OLT. Between January 1, 1987 and September 30, 1991, 46 children received OLT. Fifteen (35%) were < 1 year (average age, 7.93 months). Survival in children < 1 year was 80% and children > 1 year was 91%. Allograft survival in children < 1 year was 57% (21 allografts required for 12 survivors) and 78% in children > 1 year (37 allografts required for 29 survivors). Retransplantation was required in 5 of 15 children < 1 year (33%) and in 5 of 29 children (17%) > 1 year. Medical status in children < 1 year was similar to medical status in children > 1 year at the time of transplant. Children with chronic stable liver disease represented 60% of children < 1 year and 60.1% of children > 1 year. Children requiring hospitalization represented 26% of children < 1 year and 29% of children > 1 year. Children in intensive care represented 13% of children < 1 year and 11% of children > 1 year. Survival for all status groups was similar. Hepatic artery thrombosis occurred in one child < 1 year and in 2 children > 1 year. No statistical difference (chi 2 analysis) was found by age between the categories evaluated.(ABSTRACT TRUNCATED AT 250 WORDS)

Gastroesophageal reflux in the infant with cystic fibrosis

The association of gastroesophageal reflux and its sequelae in the infant with cystic fibrosis has gone virtually unnoticed. Eight of 40 newly diagnosed infants with cystic fibrosis seen over a 24 month period had significant gastroesophageal reflux, characterized by vomiting (7 infants), recurrent pneumonia (7 infants), and failure to thrive (4 infants). Gastroesophageal reflux was demonstrated by a combination of barium swallow, scintiscan, manometry, and esophagoscopy. Three infants had rapid and permanent alleviation of symptoms after standard medical therapy; in five infants, therapy failed and they required a Nissen fundoplication. Three infants required postoperative ventilatory support for 1, 2, and 5 days. No tracheostomies were required. Postoperative hospital stay averaged 12 days (range 5 to 30 days). There were no complications or perioperative deaths. All children had complete relief of their preoperative symptoms. The group that required surgery presented earlier (mean 7 weeks of age) to the cystic fibrosis center than either the medically treated group (mean 5 months of age) or the group free of gastroesophageal reflux symptoms (5 1/2 months of age). Also, postoperative hospitalization time markedly decreased from 50 percent of 577 combined patient days preoperatively to 4 percent of 1,639 days postoperatively; this 4 percent then paralleled what was seen in the group without gastroesophageal reflux (3 percent of 19,966 combined patient days) and the group controlled medically (5 percent of 1,897 days). The pulmonary manifestations of cystic fibrosis are extremely variable, and evaluation of the effect that any intervention has on the natural history of the disease is difficult. Nonetheless, we believe that even this small series suggests that gastroesophageal reflux and its complications can significantly alter the courses of some children with cystic fibrosis. Gastroesophageal reflux should be managed as aggressively as it is in any child with reflux, and a successful and safe reduction of symptoms can be expected with intensive management.

Gastrointestinal perforation after pediatric orthotopic liver transplantation.

PURPOSE The aim of this review was to determine the incidence of gastrointestinal perforation after pediatric liver transplantation and to identify risk factors and clinical indicators that may lead to an earlier diagnosis. METHODS A retrospective chart review of all children who presented with gastrointestinal perforation after liver transplantation at our institution between January 1, 1987 and August 1, 1996 was performed. RESULTS One hundred fifty-seven orthotopic liver transplants were performed in 128 children. Fifty-eight reexplorations, excluding those for retransplantation, were performed in 38 children. Ten perforations occurred in six children (incidence, 6.4%). Two children required multiple reexplorations because of several episodes of perforation. The sites of perforation were duodenum (n=1), jejunum (n=8), and ileum (n=1). A single-layer closure was used to repair five perforations, two-layer closures in four, and resection with primary anastomosis in another. The type of repair did not affect the occurrence of subsequent perforations. All the children were less than 18 months old. Four children had undergone prior laparotomy. All children had choledochoenteric anastomoses, but only one had a perforation associated with it. One child sustained bowel injury during the dissection for the liver transplant, but none of the perforations occurred at this site. Bowel function had returned before perforation in five children. Five children were receiving systemic antibiotics at the time of their perforation, and none had been dosed with pulse steroids for rejection. All of the children had significant changes in their temperature. Acute leukopenia developed in one child. A leukocytosis developed in the rest of the children. Abdominal radiographs demonstrated pneumoperitoneum in only one child. All children had positive culture findings from their abdominal drains. Cytomegalovirus developed in one child. Although the diagnosis of gastrointestinal perforation after pediatric liver transplant remains difficult, positive drain culture findings and significant alterations in temperature and leukocyte counts suggest its presence. Pneumoperitoneum is rarely present. CONCLUSION A high index of suspicion and timely laparotomy, especially in children less than 2 years of age, may be the only way to rapidly diagnose and treat this potentially devastating complication of liver transplant.

Transplantation for acute hepatic failure in children

BACKGROUND/PURPOSE Acute liver failure in the pediatric population is a rare but highly lethal health problem. Sometimes it is difficult to predict who will benefit from liver transplantation. The authors report on their experience in the past 8 years at a pediatric transplant center. METHODS A retrospective chart review was performed on all children referred to the liver transplant (TX) service with the diagnosis of acute hepatocellular dysfunction (AHD) from 1988 to 1996. Presentation, chemistries, and clinical course were evaluated. Statistical analysis was performed using analysis of variance. RESULTS Twenty-six children underwent evaluation. Seventeen patients fulfilled the criteria for fulminant hepatic failure (FHF). Eleven patients recovered without TX, 14 received a TX, and one died awaiting TX. Of those that received a TX, four died in the early postoperative period and 10 survived (mean follow-up of 4.2 years). There was a wide range in most laboratory values. Serum bilirubin levels, ammonia levels, and coagulation parameters, however, reached statistical significance in patients requiring transplant. The most consistent discriminators of need for transplantation and outcome were neurological findings and multisystem organ failure. Children who recovered without TX had no seizures and minimal encephalopathy. Of the 15 children who were recommended for TX, six had seizures and all had encephalopathy, 12 having grade III or IV. All five nonsurvivors had respiratory failure early in their clinical course, and four of five nonsurvivors also had renal failure. CONCLUSIONS There is significant overlap in the presentation and laboratory findings of children who present with AHD or FHF. Neurological status was an important discriminator of need for transplantation. Patients who presented with multisystem organ failure, including renal failure and respiratory failure, had 100% mortality rate despite liver transplantation.

Contrast enema findings of fibrosing colonopathy.

Purpose. Our objective was to describe characteristic contrast enema findings of fibrosing colonopathy. Materials and methods. We performed barium enemas on 18 children with cystic fibrosis who had received greater than 4500 U of lipase per kg body weight per meal and who had distal intestinal obstruction syndrome unresponsive to medical management. Results. Fifteen patients had abnormal enema results. The most common findings included stricture, loss of haustra, and colonic shortening. Ten patients required surgery, nine underwent colon resection, and one had lysis of adhesions. Pathologists confirmed fibrosing colonopathy in eight of nine speci- mens. Conclusion. Colonic stricture, abnormal haustra, and longitudinal shortening are characteristic barium enema findings of fibrosing colonopathy.

Gastrointestinal surgery in cystic fibrosis.

This review is an attempt to summarize relevant gastrointestinal surgical issues in the patient with cystic fibrosis. Many of the surgical treatments are standard and have remained unchanged for several years and are only briefly discussed. A few areas with new developments are meconium ileus and the implications of prenatal diagnosis of meconium peritonitis. In addition, new findings with hepatobiliary disease and gastroesophageal reflux disease associated with cystic fibrosis patients may change the manner in which these entities are approached because these patients are now living longer. Finally, we review the recent findings associated with fibrosing colonopathy.

Fungal splenic abscesses: management in childhood leukemia.

Fungal splenic abscesses are a potentially fatal complication of childhood leukemia, especially during hematologic relapse. Optimal treatment requires splenectomy combined with amphotericin B therapy. Previous reports have suggested that splenectomy should not be performed prior to complete control of the relapse. Three children with relapsed acute lymphocytic leukemia developed fever and splenomegaly, two during the re-induction phase of chemotherapy and the third within 1 month after a second remission. Imaging studies revealed non-homogeneity of the spleen. Uneventful splenectomy was performed for both diagnostic and therapeutic purposes before the return of normal hematopoiesis. Our results indicate that splenectomy for treatment of fungal abscesses can be successfully undertaken before complete hematologic remission is accomplished.