Reduced size liver transplantation from a donor supported by a Berlin Heart.

Patients on cardiac assist devices are often considered to be high-risk solid organ donors. We report the first case of a reduced size liver transplant performed using the left lateral segment of a pediatric donor whose cardiac function was supported by a Berlin Heart. The recipient was a 22-day-old boy with neonatal hemochromatosis who developed fulminant liver failure shortly after birth. The transplant was complicated by mild delayed graft function, which required delayed biliary reconstruction and abdominal wall closure, as well as a bile leak. However, the graft function improved quickly over the first week and the patient was discharged home with normal liver function 8 weeks after transplant. The presence of a cardiac assist device should not be considered an absolute contraindication for abdominal organ donation. Normal organ procurement procedures may require alteration due to the unusual technical obstacles that are encountered when the donor has a cardiac assist device.

Open-lung biopsy guides therapy in children.

BACKGROUND: Open-lung biopsy is uncommon in children. Modern indications and outcomes are unknown. METHODS: This is a retrospective review of 64 open-lung biopsies (58 patients) from 1976 to 1996. Open-lung biopsies were used to grade vasculopathy in 8 patients (12% of 64) with pulmonary hypertension and in 10 patients (16% of 64) with combined pulmonary hypertension and lung parenchymal disease. Forty-six biopsies (72%) were obtained to diagnose parenchymal disease. Comparisons were made between biopsies performed from 1976 to 1989 and from 1990 to 1996. RESULTS: In the period 1990 to 1996, there were significantly more infants (p = 0.03), comorbid disease (p = 0.009), extracorporeal membrane oxygenation support (p < 10(-4)), and ventilator dependence (p = 0.05) and significantly less immunocompromise (p = 0.04). A definitive diagnosis was made in 43 of 64 cases (67%) and altered workup in 63 of 64 cases (98%). No correlation existed between Heath-Edwards grade of microangiopathy and catheterization data. Definitive diagnosis was most strongly associated with a nonimmunocompromised patient (p < 10(-4)). Although only one death (1.5%) was related to open-lung biopsy, the procedure was associated with a 30% inhospital mortality rate and an 11% morbidity rate. Of the 19 deaths, 1 patient died from the procedure, 13 died from their diseases, and 5 had support withdrawn. Death was associated with preoperative ventilator dependence (p < 10(-4)) and extracorporeal membrane oxygenation (p = 0.007). CONCLUSIONS: Pediatric open-lung biopsy commonly alters the diagnostic workup (98%). It is recommended for children who have been supported for 2 weeks by extracorporeal membrane oxygenation and for those with combined pulmonary hypertension and parenchymal lung disease. It is less useful in immunocompromised children.

Development of a true primary repair for the full spectrum of esophageal atresia.

OBJECTIVE: To determine whether or not a true primary repair, without myotomies and with the gastroesophageal junction below the diaphragm, can be accomplished across the esophageal atresia (EA) spectrum. Our hypothesis is that the esophageal anastomosis can withstand significant tension. The consequences, particularly for those patients with a very long gap atresia, were assessed. SUMMARY OF BACKGROUND DATA: Difficulties arise roughly in proportion to the size of the gap between esophageal segments. Reported surgical complications remain frequent, and particularly at the far end of the EA spectrum, not all children are left with a satisfactorily functioning esophagus or esophageal substitute. METHODS: The outcomes of all infants who had a true primary repair of EA from 1976-1997 were determined. Surgically, the methods used to achieve a reliable true primary repair were expanded to accomplish this, even for a very long gap EA. RESULTS: From 1976-97, 70 infants with or without associated tracheoesophageal fistula (TEF) had primary repairs performed with no surgery-related deaths and 11% later deaths. No interpositions were performed since 1983. There were no discernible anastomotic leaks and one late recurrent TEF related to the early use of balloon dilation. Ten infants had gaps of 5.0-6.8 cm and, among these, four had gaps of 5.5-6.8 cm that could not be pulled together initially. Traction sutures in the esophageal ends, however, produced sufficient lengthening within 6-10 days for a true primary repair. Very long gap repairs produced more reflux (10 of 10 required a fundoplication versus 24 of 70 overall) and more dilations to relieve strictures. Two infants underwent stricture resection with no recurrence. On follow-up, all patients over 2 years of age were eating well or satisfactorily, and none had a gastrostomy tube. CONCLUSIONS: (1) The esophageal anastomosis can withstand considerable tension and allows a reliable true primary repair for the full EA spectrum. (2) Growth is rapid and traction sutures will produce significant esophageal lengthening within days. (3) With increasing tension, gastroesophageal reflux (GER) and strictures are more common; however, both are treatable. Follow-up reveals the benefits of true primary repair over other solutions.