Liver retransplantation: a single-center outcome and financial analysis.

Retransplantation of the liver (re-OLTx) accounts for approximately 10% of all liver transplants in the United States. The decision to offer a patient a second liver transplant has significant financial, ethical, and outcome implications. This large, single-center experience describes some outcome and financial data to consider when making this decision. One thousand three liver transplants were performed in 921 patients at our center. Patients were divided into adult and pediatric groups, and further by whether they received a single transplant or more than one. Overall survival, variation in survival by timing of re-OLTx, and survival in adults with hepatitis C were investigated, as were hospital charges and cost of re-OLTx. Adults, but not children, had a significant decrement in survival following a second transplant. Second transplants more than double the cost of the initial transplant, but there is a significantly higher cost associated with early retransplantation compared to the cost associated with late retransplantation (costs of first and second transplants included in both cases). This difference is due to a longer length of stay and associated cost in the ICU. Adult patients retransplanted early have the same overall survival compared to those done late. The sample size of the adult HCV re-OLTx population was too small to reach statistical significance despite their observed poorer outcome.

Resection of the inferior vena cava for hepatic malignancy.

Involvement of the inferior vena cava (IVC) by hepatic tumors, although uncommon, is considered to be unresectable by standard surgical techniques. Recent advances in hepatic surgery have made combined hepatic and vena caval resection possible. The purpose of this study is to describe the surgical techniques and early results of combined resection of the liver and IVC. From 1997 to 2000, 11 patients underwent resection of the IVC along with four to seven liver segments. Resections were carried out for hepatocellular carcinoma (four); colorectal metastases (four); and hepatoblastoma, gastrointestinal stromal tumor metastases, and squamous cell carcinoma in one patient each. Ex vivo procedures were performed twice, and total vascular isolation was used in the nine other cases. The IVC was reconstructed with ringed Gore-Tex tube graft (five), primarily (five), or with Gore-Tex patches (one). There were two early deaths: one from liver failure at 3 weeks and one from sepsis secondary to a perforated segment of small bowel 4 months postresection. One patient with a gastrointestinal stromal tumor died at 32 months of recurrent tumor and one patient with hepatocellular carcinoma is alive with recurrent tumor at 16 months. The remaining patients are alive and disease free with follow-up ranging from 3 to 40 months without evidence of IVC occlusion. Combined resection of the liver and IVC is a formidable undertaking with substantial surgical risk. However, this aggressive surgical approach offers a chance for cure in patients with tumors involving the IVC that would otherwise have a dismal prognosis.

Graft survival in pediatric liver transplantation.

BACKGROUND/PURPOSE: Liver transplantation is standard therapy for children with a variety of liver diseases. The current shortage of organ donors has led to aggressive use of reduced or split grafts and living-related donors to provide timely liver transplants to these children. The purpose of this study is to examine the impact of these techniques on graft survival in children currently treated with liver transplantation. METHODS: Data were obtained on all patients less than 21 years of age treated with isolated liver transplants performed after January 1, 1996 in an integrated statewide pediatric liver transplant program, which encompasses 2 high-volume centers. Nonparametric tests of association and life table analysis were used to analyze these data (SAS v 6.12). RESULTS: One hundred twenty-three children received 147 grafts (62 at the University of Florida, 85 at the University of Miami). Fifty-two (36%) children were less than 1 year of age at time of transplant, and 80 (55%) were less than 2 years of age. Patient survival rate was identical in the 2 centers (1-year actuarial survival rate, 88.4% and 87.1%). Twenty-five (17%) grafts were reduced, 28 (19%) were split, 6 were from living donors (4%), and 88 (60%) were whole organs. One-year graft survival rate was 80% for whole grafts, 71.6% for reduced grafts, and 64.3% for split grafts (P =.06). Children who received whole organs (mean age, 6.1 years) were older than those who received segmental grafts (mean age, 2.5 years; P <.01). Multifactorial analysis suggested that patient age, gender, and use of the graft for retransplant did not influence graft survival, nor did the type of graft used influence patient survival. CONCLUSIONS: The survival rate of children after liver transplantation is excellent independent of graft type. Use of current techniques to split grafts between 2 recipients is associated with an increased graft loss and need for retransplantation. Improvement in graft survival of these organs could reduce the morbidity and cost of liver transplantation significantly in children.

Intraoperative endoscopy in a child with Turner's syndrome and gastrointestinal hemorrhage: a case report.

Gastrointestinal bleeding in Turner's syndrome can represent vascular lesions that are frequently beyond standard endoscopic reach and often life threatening. This report describes the successful use of intraoperative endoscopy to identify the souce of bleeding in an adolescent with Turner's syndrome and significant intestinal hemorrhage. J Pediatr Surg 36:951-952.

Susceptibility of liver allografts to high or low concentrations of preformed antibodies as measured by flow cytometry.

Liver grafts are more resistant to damage by HLA antibodies than other organ allografts, but it is not clear if the antibodies are associated with graft rejection or graft loss, or if different antibody concentrations have different effects. To explore potential associations between antibody concentrations and outcome, preformed IgG antibodies against donor cells were quantified by flow cytometry in 465 consecutive liver transplant recipients. Antibody-positive patients were classified according to whether they had high or low antibody concentrations and analyzed for possible correlation with graft rejection or graft loss. The results showed that the incidence of rejection was not significantly different between antibody-positive and negative patients. However, patients with high antibody concentrations had a higher incidence of steroid-resistant rejections (31% at 1 year) than patients with low antibody (4%) or no antibody (8%, p < 0.0004). These effects were mainly due to T-cell (HLA class 1) antibodies. The overall incidence of rejection at 1 year was 69% for high antibody patients, 51% for patients with low antibodies and 53% for patients with no antibodies (p not significant). In an apparent paradox, antibody-positive patients underwent fewer early graft losses. Thus, the associations of preformed antibodies and outcome depend, on the one hand, on antibody concentrations, and on the other hand on whether the outcome measured is steroid-sensitive rejection, steroid-resistant rejection or graft survival. These complex interactions may explain the controversial results observed in previous studies.

Resolution of hypoxemia in a liver transplant recipient after ligation of a portosystemic shunt.

This report describes the unique development of pulmonary vascular dilatation and hypoxemia associated with a portosystemic shunt in a pediatric liver transplant recipient. Ligation of the shunt resulted in resolution of hypoxemia. The outcome suggests that hepatic venous return to the pulmonary circulation is important in maintaining normal pulmonary vascular caliber.

Detrimental effects of standard medical therapy in congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the impact of a nonstandard ventilation strategy on survival in congenital diaphragmatic hernia (CDH). BACKGROUND: Despite recent advances, including nitric oxide, CDH remains an unsolved problem with a mortality rate of 35% to 50%. Hyperventilation and alkalization remain common therapies. METHODS: In 1992, the authors prospectively abandoned hyperventilation and alkalization. Patients are lightly sedated and ventilated with the lowest pressure providing adequate chest movement, and the rate is set to patient comfort. Nitric oxide and extracorporeal membrane oxygenation (ECMO) are reserved for life-threatening instability. Surgical repair is delayed 1 to 5 days. Sixty consecutive patients are compared with 29 previous patients treated with hyperventilation and alkalization, 13 before and 16 after the availability of ECMO. RESULTS: Overall, 47 of 60 patients (78%) in study era 3 survived compared with 2 of 13 (15%) in the hyperventilation era and 7 of 16 (44%) in the hyperventilation/ECMO era (p < 0.0001). The disease severity and the incidence of associated anomalies did not differ between groups. To compare management strategies, patients who had treatment withheld because of lethal associated conditions were then removed from analysis. Peak inspiratory pressure and arterial pH were lower (p < 0.0001) and Paco2 was higher (p < 0.05) in era 3 than in the previous eras. The rate of pneumothorax (1.9%) decreased (p < 0.0001). In era 3, survival was 47 of 53 (89%) treated patients, and 23 of 25 inborn patients with isolated CDH survived (92%). CONCLUSIONS: Nonstandard ventilatory support of patients with CDH has led to significantly improved survival rates. This study sets a survival benchmark and strongly suggests the negative effects of hyperventilation and alkalization.

Primary intraocular posttransplantation lymphoproliferative disorder.

We report a case of posttransplantation lymphoproliferative disorder manifesting as an isolated, unilateral iris tumor. A 2-year-old boy who had undergone liver transplantation for biliary atresia at age 4 months was seen with a 2-month history of an enlarging iris nodule. Histopathologic examination of the iris lesion demonstrated a mixed population of lymphoid cells. To our knowledge, this is the youngest patient with posttransplantation lymphoproliferative disorder isolated to the eye.

Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group.

BACKGROUND: Repair of congenital diaphragmatic hernia (CDH) has changed from an emergent procedure to a delayed procedure in the last decade. Many other aspects of management have also evolved since the first successful repair. However, most reports are from single institutions. The lack of a large multicenter database has hampered progress in the management of congenital diaphragmatic hernia (CDH) and makes determination of the current standard difficult. METHODS: The CDH study group was formed in 1995 to collect data from multiple institutions in North America, Europe, and Australia. Participating centers completed a registry form on all live-born infants with CDH during 1995 and 1996. Demographic information, data about surgical management, and outcome were collected for all patients. RESULTS: Sixty-two centers participated, with 461 patients entered. Overall survival was 280 of 442 patients (63%) where survival was recorded. The defect was left-sided in 78%, right-sided in 21%, and bilateral in 1%. A subcostal approach was used in 91% of patients, with pleural drainage used in 76%. A patch of some kind was used in just over half (51%) of the patients, with polytetrafluoroethylene being the most commonly used material (81%) in those patients with a patch. The mean surgical time was 102 minutes, with an average blood loss of 14 mL (range, 0 to 500 mL). The overwhelming majority of patients underwent repair between 6:00 AM and 6:00 PM (289 of 329, 88%). Nineteen percent of patients had surgical repair on extracorporeal membrane oxygenation (ECMO) at a mean time of 170 hours into the ECMO course (range, 10 to 593 hours). The mean age at surgery in patients not treated with ECMO was 73 hours (range, 1 to 445 hours). CONCLUSIONS: The multicenter nature of this report makes it a snapshot of current management. The data would indicate that prosthetic patching of the defect has become common, that after-hours repair is infrequent, and that delayed surgical repair has become the preferred approach in many centers. Furthermore, the mean survival rate of 63% indicates that despite decades of individual effort, the CDH problem is far from solved. This highlights the need for a centralized database and cooperative multicenter studies in the future.

The arterioportal fistula syndrome: clinicopathologic features, diagnosis, and therapy.

BACKGROUND & AIMS: Arterioportal fistulas (APFs) are rare vascular disorders of the mesenteric circulation. The aim of this study was to determine the etiology, anatomical location, and main symptom at presentation of APFs, and analyze the various modes of treatment. METHODS: The etiology, clinical presentation, radiographs, and treatment of 12 patients with APFs are reported in detail, and another 76 cases published since 1980 are reviewed. RESULTS: APFs result from trauma (n = 25, 28%), iatrogenic procedures (n = 14, 16%), congenital vascular malformations (n = 13, 15%), tumor (n = 13, 15%), aneurysm (n = 12, 14%), and other causes (n = 11, 12%). The origin of APFs is the hepatic artery in the majority of patients (n = 56, 65%). The main symptoms at presentation are lower or upper gastrointestinal bleeding (n = 29, 33%), ascites (n = 23, 26%), heart failure (n = 4.5%), or diarrhea (n = 4.5%). Radiological intervention provides definitive treatment in 42% (n = 33) of patients, whereas the remainder are treated by surgery alone (n = 27, 31%) or a combination of radiological intervention and surgery (n = 8, 9%). CONCLUSIONS: APFs result in a protean syndrome variously combining portal hypertension and other hemodynamic imbalances (heart failure, intestinal ischemia). Single or multiple interventional radiological procedures using arterial and/or venous approaches allow definitive treatment of most APFs. With increasing technological advances, it is anticipated that surgery will only be indicated in rare instances after failure of radiological intervention(s).

Hepatic undifferentiated (embryonal) sarcoma arising in a mesenchymal hamartoma.

We report the case of a hepatic undifferentiated (embryonal) sarcoma (UES) arising within a mesenchymal hamartoma (MH) in a 15-year-old girl. Mapping of the tumor demonstrated a typical MH transforming gradually into a UES composed of anaplastic stromal cells. When evaluated by flow cytometry, the MH was diploid and the UES showed a prominent aneuploid peak. Karyotypic analysis of the UES showed structural alterations of chromosome 19, which have been implicated as a potential genetic marker of MH. The histogenesis of MH and UES is still debated, and reports of a relationship between them, although suggested on the basis of histomorphologic similarities, have never been convincing. The histologic, flow cytometric, and cytogenetic evidence reported herein suggests a link between these two hepatic tumors of the pediatric population.

Gastrointestinal myoelectric activity in a child with gastroschisis and ileal atresia.

Gastroschisis is frequently associated with intestinal atresia and alterations in gastrointestinal function. The authors studied gastric and small bowel myoelectric activity in a child who had a complex course and prolonged inability to tolerate oral intake after staged repair of gastroschisis and an associated ileal atresia. The child remained unable to tolerate oral intake after repair of the atresia and was reexplored 3 months later to rule out a partial small bowel obstruction, with simultaneous placement of serosal electrodes on the stomach and proximal small bowel. Persistent gastric dysrhythmias were observed postoperatively, and the child was unable to tolerate gastrostomy tube feedings. Abnormalities were also seen in small bowel motility, including retrograde propagation of activity fronts of the migrating myoelectric complex. However, the intestine converted to a fed myoelectric pattern with tube feedings, and the child was subsequently able to tolerate feedings via a tube placed directly into the small bowel. The authors conclude that myoelectric recordings via implanted electrodes are safe and feasible in children, and may give information regarding underlying motility alterations. The ultimate clinical role of myoelectric recordings in treating children with suspected motility disorders will require further study.

Significance of preformed anti-donor antibodies in liver transplantation.

The significance of a positive cross-match in liver transplantation remains controversial, as documented by a number of recent conflicting reports. In this study, we evaluated 195 consecutive orthotopic liver transplant recipients in whom the cross-match was either negative or positive for T or B cells. Special emphasis was placed on the outcome of patients with high levels of preformed IgG antibodies directed against donor T cells. IgG anti-donor antibodies were confirmed by flow cytometry in all cases. Of 10 patients with strong T-cell antibodies, there was one early death due to nonimmunological causes. Transplantation was successful in 9/10 patients followed for 3 months to 3 years. Graft survival, incidence of acute rejection, and number of liver biopsies in patients with a positive cross-match (strong T, weak T, or B cell) were not significantly different from those of patients with a negative cross-match. In the strong T cell antibody group, one patient had early graft dysfunction, with extensive hepatic necrosis and histological signs of antibody-induced damage. Two other patients also showed some evidence of possible antibody-mediated events, such as neutrophil infiltration and hepatocyte swelling. These lesions were reversible, and the patients had uneventful recoveries. Thus, in our experience, preformed antibodies did not preclude good graft survival.

Congenital diaphragmatic hernia. Epidemiology and outcome.

Congenital diaphragmatic hernia is a relatively common birth defect. It affects about 1114 babies a year in the United States. Reported survival averages 60% but may be significantly lower. We do not understand the etiology of CDH. Its association with other anomalies and several distinct patterns of presentation suggest that more than one cause may exist. There is a high degree of variability in both treatment and outcomes, but no data exist to allow a rigorous comparison of the efficacy of various treatment strategies. Stratification of patients into more homogeneous groups will be a necessary prerequisite for the design of meaningful comparative trials. The incidence of the lesion prevents any single institution from accruing sufficient patients to conduct such a trial. An ad hoc multicenter study group (the Congenital Diaphragmatic Hernia Study Group) has been formed for this purpose. This organization has begun collecting data with an initial goal of developing a stratification scheme. Prospective data collection should allow verification of several of the estimates made in this article. Current data make it clear that CDH represents a major cause of perinatal morbidity and mortality.

Ion transport properties of fetal sheep alveolar epithelial cells in monolayer culture.

Alveolar type II cells were isolated from late-term fetal sheep to investigate ion transport across fetal distal lung epithelium. In Ussing chambers, basal transepithelial potential difference (PD; reference apical side), equivalent short-circuit current (Ieq), and resistance were -0.10 +/- 0.05 mV, 0.10 +/- 0.08 microA/cm2, and 821.5 +/- 38.8 omega .cm2, respectively. Epinephrine (100 nM) increased PD from -0.13 +/- 0.19 to -1.37 +/- 0.20 mV and Ieq from 0.18 +/- 0.26 to 1.47 +/- 0.28 microA/cm2. Propranolol (100 nM) inhibited responses to epinephrine. Forskolin (10 microM) increased PD to -0.81 +/- 0.08 mV and Ieq to 1.02 +/- 0.12 microA/cm2. Mucosal amiloride (200 microM) and serosal bumetanide (10 microM) decreased the forskolin-stimulated PD by 23.42 +/- 4.73 and 25.57 +/- 3.9%, respectively. We conclude that in fetal sheep distal lung epithelium amiloride-inhibitable sodium absorption and bumetanide-sensitive chloride secretion are stimulated by forskolin and that epinephrine effects on ion transport are mediated by beta-adrenergic receptors.

The prenatal sonographic appearance of enteric duplication cysts.

Enteric duplication cysts are rare lesions, and relatively few cases have been diagnosed prenatally. We report two cases with a distinctive presentation. One, a duplication of the antrum of the stomach, was adjacent to an extralobar pulmonary sequestration. The other, a duodenal duplication, appeared as a thick-walled right lower quadrant cyst. We observed distinct peristalsis in this cyst, a finding that helped us make a firm diagnosis of enteric duplication.

In utero lung growth of fetal sheep with diaphragmatic hernia and tracheal stenosis.

The observation that tracheal ligation produces pulmonary hyperplasia even in animals with surgically induced diaphragmatic hernia (DH) has led to rapid application of the technique to human fetuses with DH. The aim of this study was to determine how rapidly fetal lung volume increases after creation of a high-grade tracheal stenosis in fetal sheep with surgically created DH. Twenty-three fetal sheep were prepared with a left thoracotomy at 90 days' gestational. Six had creation of a DH with tracheal stenosis (DHTS) over an 18-gauge cannula, which was then removed. Ten had DH alone, and seven control animals (CT) had a thoracotomy without DH. Thirty days later, vascular and tracheal loop catheters were inserted in all animals and tunneled out the ewes' flank. Between 125 and 140 days' gestation, lung volumes and lung liquid production were measured in awake, unanesthetized animals using a standard double-marker dilution technique. Average lung volumes (in milliliters) were 150.9 +/- 13.9 for CT, 29.3 +/- 4.4 for DH, and 414.5 +/- 88 for DHTS (p < 0.01). Mean lung liquid production varied from 6.00 +/- 2.23 mL/h in DH animals before 130 days to 16.69 +/- 8.29 mL/h in DHTS animals after 135 days' gestation. DH animals had lower lung liquid production (8.51 +/- 1.4 mL/h) than CT (12.4 +/- 0.8 mL/h) or DHTS animals (12.4 +/- 2.2 mL/h)(P < .01). The rate constant gamma (h-1) for lung liquid production was significantly higher in DH animals than in either CT or DHTS animals (P < .01). Tracheal stenosis in this model causes rapid lung growth before 130 days' gestation. The authors speculate that short periods of incomplete stenosis might reverse the pulmonary hypoplasia associated with DH. To achieve this goal, the timing and duration of treatment and the optimal degree of stenosis must be defined.

Physiologic response to two endotracheal suctioning techniques in newborn lambs with and without acute pulmonary hypertension.

BACKGROUND: Endotracheal suctioning may cause sudden increases in pulmonary arterial pressure, which can result in hypoxia secondary to right ventricular failure and/or increased right-to-left shunting. An adaptor that allows suctioning without disconnecting the ventilator has been proposed to prevent these problems; however, its efficacy has not been rigorously studied. OBJECTIVE: To examine the physiologic responses to two endotracheal suctioning techniques in newborn lambs with and without acute pulmonary hypertension. METHODS: A repeated-measures design was used to compare two endotracheal suctioning techniques in seven newborn lambs with and without acute pulmonary hypertension. An adaptor was used in the ventilator-controlled technique, making disconnection of the ventilator during suctioning unnecessary. In the bag-controlled technique, the ventilator was disconnected and ventilation was done with a manual resuscitation bag. Physiologic variables, pulmonary and mean arterial pressure, peak inspiratory pressure, mixed venous oxygen saturation, cardiac index, and arterial blood gas values were recorded before, during, and after endotracheal suctioning. RESULTS: Endotracheal suctioning caused a statistically significant systemic hypertensive response in lambs with and without acute pulmonary hypertension, regardless of which suctioning technique was used. No statistically significant changes occurred in pulmonary arterial pressure using either technique. CONCLUSIONS: Use of an adaptor resulted in no differences in the physiologic responses to endotracheal suctioning. However, endotracheal suctioning was easier to perform using an adaptor because no extra equipment or person was needed.

Unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula.

A small-for-gestational-age, premature infant with the combination of unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula was treated successfully by early gastrostomy and delayed fistula division with esophagoesophagostomy. Only 2 other successfully treated cases have been reported previously. Both were full-term infants treated with early division of the tracheoesophageal fistula and esophagoesophagostomy. Gestational age, size, and associated medical problems need to be considered when planning operative therapy for these babies.