Use of airway pressure release ventilation in a child with refractory hepatopulmonary syndrome after liver transplantation.

HPS is a life-threatening condition in patients with end-stage liver disease, in which intrapulmonary vascular dilatations result in intrapulmonary shunts and hypoxemia. The only successful treatment is liver transplantation. Hypoxemia may be severe prior to transplantation; however, it can worsen or become refractory after liver transplantation and result in increased post-operative mortality. Here, we present the case of a 10-month-old female infant with progressive end-stage liver disease and severe HPS, who developed refractory hypoxemia after a successful liver transplantation. After 19 days of unsuccessful attempts to reverse the hypoxemia using conventional mechanical ventilation and HFOV, the patient responded dramatically to APRV, with rapid improvement in her PaO2 and sharp decline in her OI. She was able to begin weaning from APRV two days later and was extubated within seven days. APRV was successful in treating refractory hypoxemia in this patient with severe HPS after liver transplantation, possibly by modifying distribution of pulmonary blood flow. Although we cannot rule out coincidental natural resolution of the HPS, APRV could be a useful rescue therapy in patients with HPS and refractory hypoxemia.

Hepatoadrenal syndrome in pediatric patients with end-stage liver disease.

OBJECTIVE: Adrenal insufficiency in patients with liver failure, referred to as hepatoadrenal syndrome, is well characterized in adult patients but has not yet been described in children. We present 22 pediatric subjects with end-stage liver disease and adrenal insufficiency, diagnosed using the cosyntropin stimulation test. DESIGN AND SETTING: A retrospective chart review using inpatient records from a pediatric intensive care unit in an academic medical center with a busy pediatric transplant program. PATIENTS: Most were infants with anatomical short gut and severe, total parenteral nutrition-induced liver failure awaiting liver transplantation. Many were critically ill; 68% required mechanical ventilation and 59% required vasopressors. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: All patients had low baseline cortisol levels and ten also had an abnormal cosyntropin stimulation test. Cortisol levels at baseline and increments of serum cortisol at 30 and 60 mins post cosyntropin were 9.3 ± 5 µg/dL, 9.3 ± 4 µg/dL, and 10.7 ± 6 µg/dL, respectively, compared to these values in five patients with liver failure and normal adrenal function (21.3 ± 3 µg/dL, 10.5 ± 5 µg/dL, and 12.7 ± 3 µg/dL, respectively). Baseline cortisol levels were higher in patients who required vasopressors (11.1 ± 5 µg/dL) compared to those who did not (6.6 ± 4.3 µg/dL, p = .04), and 60-min increment cortisol levels were lower in nonsurvivors compared to survivors (8.6 ± 4.8 µg/dL vs. 15.1 ± 5.1 µg/dL, p = .002). The severity of adrenal insufficiency did not correlate with the degree of hepatic decompensation. Clinical characteristics, including serum electrolytes and vasopressor requirements, were similar in patients with hepatoadrenal syndrome and patients with liver failure and normal adrenal function. Twelve (55%) of the patients died in the hospital, 11 without receiving a transplant. Hydrocortisone therapy permitted rapid weaning of vasopressor therapy but did not affect survival. CONCLUSIONS: Children with end-stage liver disease are at risk for hepatoadrenal syndrome and should have their cortisol levels monitored since clinical manifestations may not be diagnostic.

Pediatric intestinal and multivisceral transplantation: a new challenge for the pediatric intensivist.

INTRODUCTION: With increasing survival rates, intestinal transplantation (ITx) and multivisceral transplantation have reached the mainstream of medical care. Pediatric candidates for ITx often suffer from severe multisystem impairments that pose challenges to the medical team. These patients frequently require intensive care preoperatively and have unique intensive care needs postoperatively. METHODS: We reviewed the literature on intensive care of pediatric intestinal transplantation as well as our own experience. This review is not aimed only at pediatric intensivists from ITx centers; these patients frequently require ICU care at other institutions. RESULTS: Preoperative management focuses on optimization of organ function, minimizing ventilator-induced lung injury, preventing excessive edema yet maintaining adequate organ perfusion, preventing and controlling sepsis and bleeding from varices at enterocutaneous interfaces, and optimizing nutritional support. The goal is to extend life in stable condition to the point of transplantation. Postoperative care focuses on optimizing perfusion of the mesenteric circulation by maintaining intravascular volume, minimizing hypercoagulability, and providing adequate oxygen delivery. Careful monitoring of the stoma and its output and correction of electrolyte imbalances that may require renal replacement therapy is critical, as are monitoring for and aggressively treating infections, which often present with only subtle clinical clues. Signs of intestinal rejection may be non-specific, and early differentiation from other causes of intestinal dysfunction is important. Understanding of the expanding armamentarium of immunosuppressive agents and their side-effects is required. CONCLUSIONS: As outcomes of ITx improve, transplant teams accept patients with higher pre-operative morbidity and at higher risk for complications. Many ITx patients would benefit from earlier referral for transplant evaluation before severe liver disease, recurrent central venous catheter-related sepsis and venous thromboses develop.

Comparison of intermittent versus continuous infusion of propofol for elective oncology procedures in children.

OBJECTIVE: To compare the effects of administering propofol as a continuous infusion vs. bolus dosing in children undergoing ambulatory oncologic procedures in the pediatric intensive care unit (PICU). DESIGN: Prospective, randomized study. SETTING: Tertiary PICU in a university hospital. PATIENTS: Ambulatory oncology patients scheduled for diagnostic or therapeutic procedures with propofol anesthesia in the PICU were eligible for enrollment. INTERVENTIONS: Patients were randomly assigned to receive either continuous infusion or bolus administration of propofol in a protocol-driven manner. All patients received an initial bolus of 1.5 mg/kg, with additional 0.5 mg/kg doses until complete induction. Continuous infusions were started at 0.1 mg/kg/min and, if needed, increased 20% after a bolus of 0.5 mg/kg. Bolus group patients were given doses of 0.5 mg/kg if needed. Ramsay scores of < 5 were used as criteria for additional dosing. MEASUREMENTS AND MAIN RESULTS: Eighteen patients undergoing 40 separate procedures were enrolled during the study period. Twenty procedures each were performed with continuous or bolus administration of propofol. No differences were present between groups in demographic characteristics, induction dose and time, procedure and recovery times, or adverse events. All patients had adequate anesthesia and favorable satisfaction scores. More boluses were needed in the bolus group (8.5 +/- 4.6 vs. 5.4 +/- 2.9; p < .05). Average systolic blood pressure decreased more in the continuous infusion group (26.4% +/- 12 vs. 19.3% +/- 10; p < .05). Total propofol dose was higher in the continuous infusion group (8.0 mg/kg +/- 3.8 vs. 5.7 mg/kg +/- 2.4; p < .05). CONCLUSION: Both continuous and bolus administration of propofol provided conditions for conducting oncologic procedures that were satisfying to patients, their families, and physicians. Continuous infusions were associated with a larger total dose and greater decreases in systolic blood pressure. Physician preference is likely to dictate which method is used.

Discordance in interpretation of chest radiographs between pediatric intensivists and a radiologist: impact on patient management.

PURPOSE: When radiologists are not available, chest radiographs (CXRs) of pediatric intensive care unit (PICU) patients are commonly interpreted by pediatric intensivists. We prospectively investigated the frequency of errors in CXR interpretation by pediatric intensivists and their impact on patient management. MATERIALS AND METHODS: Chest radiographs of PICU patients were evaluated by 5 pediatric intensivists then by a pediatric radiologist (the "gold standard"). If the interpretation of the radiologist and intensivist differed, an independent intensivist determined whether a management change took place. A pediatric pulmonologist determined how many intensivist interpretations were different from the radiologist's interpretations. RESULTS: Seven hundred twenty-eight radiographic findings were identified by the radiologist in 460 CXRs. There were 33 interpretation errors by the intensivists (4.5% of the findings in 7.1% of the CXRs). Only 3/33 error corrections (0.45% of the findings in 0.7% of the CXRs) resulted in change in patient management. CONCLUSIONS: Errors in interpretation of CXRs by pediatric intensivists were common but less than that in other series, probably because of education of the pediatric intensivists through daily rounds with the radiologist. Although interpretation errors that affected patient management were rare, their clinical importance supports the growing practice of 24/7 remote radiograph reading by radiologists.