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Contains fulltext : 170587.pdf (Publisher’s version ) (Closed access) BACKGROUND: Open lung biopsy can help differentiate between reversible and irreversible lung disease and may guide therapy. To assess the risk-benefit ratio of this procedure in pediatric extracorporeal membrane oxygenation (ECMO) patients, we reviewed data of all patients who underwent an open lung biopsy during ECMO in one of the two pediatric ECMO centers in a nationwide study in the Netherlands. RESULTS: In nineteen neonatal and six pediatric patients (0-15.5years), twenty-five open lung biopsies were performed during the study period. In 13 patients (52%), a classifying diagnosis of underlying lung disease could be made. In another nine patients (36%), specific pathological abnormalities were described. In three patients (12%), only nonspecific abnormalities were described. The histological results led to withdrawal of ECMO treatment in 6 neonates with alveolar capillary dysplasia/misalignment of pulmonary veins (24%) and in another 6 patients, corticosteroids were started (24%). All patients survived the biopsy procedure. Hemorrhagic complications were rare. CONCLUSION: An open lung biopsy during an ECMO run in neonates and children is a safe procedure with a minimum risk for blood loss and biopsy-related death. It can be very useful in diagnosing the underlying pathology and can guide cessation of ECMO treatment and thereby avoid continuation of futile treatment, especially in neonatal patients. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Diagnostic study.

Background/purpose The authors experienced 3 cases of persistent pulmonary hypertension of the neonate (PPHN) associated with duodenal atresia with paradoxical dilatation of the distal blind end of the duodenum. The aim of this study was to clarify the characteristics of this novel association. Methods The medical charts of the patients were reviewed retrospectively. Results Case 1 was a 2,862-g male infant with a prenatal diagnosis of duodenal atresia. The dilated intestine was identified as the distal blind end of the duodenum during duodeno- duodenostomy. PPHN developed after surgery, and the patient died despite treatment with inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). Lung biopsy results showed alveolar capillary dysplasia (ACD) with misalignment of pulmonary vessels. Case 2 was a 2,244-g female infant with duodenal atresia. Surgery could not be performed because of refractory PPHN, which resulted in death. Autopsy results showed ACD with misalignment of pulmonary vessels and duodenal atresia associated with dilatation of the distal blind end. Case 3 was a 2,462-g female infant with a prenatal diagnosis of duodenal atresia associated with dilatation of the distal blind end. Enlarged echogenic fetal lungs were noted antenatally. PPHN developed after surgery, and she was extubated successfully under combined therapy with iNO and intravenous prostacyclin. Unfortunately, she finally died of refractory PPHN. A retrospective review of the prenatal ultrasonographic findings showed enlarged echogenic fetal lungs as in the first 2 cases. Conclusions The characteristics of this novel association are enlarged echogenic fetal lungs, duodenal atresia associated with paradoxical dilatation of the distal blind end, and refractory PPHN resistant to iNO.

Treatment of congenital diaphragmatic hernia (CDH) has undergone a revolutionary change in philosophy, from previous urgent repair to the present practice of stabilization and delayed repair. However, when extracorporeal membrane oxygenation (ECMO) is required, many people believe that the risk of postoperative pulmonary hypertension (PPHN) mandates hernia repair while on ECMO. This report details the experience in two ECMO centers with stabilization, ECMO if required, and CDH repair post-ECMO. All CDH patients symptomatic in the first hour of life with a gestational age of at least 34 weeks during the period were reviewed retrospectively. Standard criteria were used to select patients for ECMO. High-frequency jet or oscillating ventilators and nitric oxide were not routinely available throughout the study period, but were used in some of the more recent patients. A total of 60 patients presented to the two centers; 24 cases were stabilized with conventional management, repair of the CDH was done elective, and survival was 100%. Eight patients were referred after having repair elsewhere; six survived (75%). The two deaths were attributable to associated lethal lesions--complex cyanotic heart disease and alveolar capillary dysplasia. Eight patients who required ECMO were managed with the intention of repairing the defect on ECMO. Four survived (50%). Two patients died before repair. Twenty patients were managed with ECMO, with the intention of repairing the defect after decannulation. Overall survival was 13 (65%), deaths were caused by pre-ECMO hypoxia, pulmonary insufficiency, and associated cardiac disease. No patient had recurrent pulmonary hypertension after late repair.(ABSTRACT TRUNCATED AT 250 WORDS)

Purpose: The aim of the study was to review the authors' experience with alveolar capillary dysplasia (ACD), a cause of persistent pulmonary hypertension of the neonate (PPHN) caused by decreased alveolar units, dilated anomalous pulmonary veins, thick-walled arterioles, and thickened interalveolar septa. Methods: The records of all neonates with ACD were reviewed from Children's Hospital, Columbus, Ohio, and Sophia's Children's Hospital, Rotterdam, The Netherlands. The clinical characteristics and pathological findings are discussed. Results: Eight neonates were diagnosed with ACD from 1994 to 2002. Twenty-five percent (2/8) experienced respiratory distress immediately after birth, whereas 75% (6/8) had normal Apgar scores but deteriorated 1.5 hours to 30 days after birth. All infants required conventional ventilation initially; 50% (4/8) were placed on high-frequency oscillating ventilation and 87% (7/8) on extracorporeal membrane oxygenation. A premorbid diagnosis was established in 3 patients by open lung biopsy. The diagnosis of ACD was confirmed at autopsy in all patients. Conclusions: ACD is a fatal disease that should be suspected in all neonates with respiratory failure and PPHN who fail conventional therapy. Prompt diagnosis helps to avoid prolongation of costly treatment modalities in a uniformly fatal disease. An algorithm is proposed in which neonates with PPHN who fail treatment with extracorporeal membrane oxygenation are managed by open lung biopsy.

Stabilization and delayed operation for patients with congenital diaphragmatic hernia (CDH) is now widely accepted. When preoperative extracorporeal membrane oxygenation (ECMO) is needed, most centers have CDH repaired on ECMO to minimize the risk of postoperative deterioration. The authors adopted a policy of weaning from ECMO before repair in an effort to avoid hemorrhagic risks. They reviewed their experience with CDH patients who required ECMO for stabilization before repair but for whom post-ECMO repair was planned. The records of all high-risk CDH patients with a gestational age of at least 34 weeks were reviewed. Eighteen patients were identified. None of the eight who were stabilized and operated on without ECMO required bypass postoperatively; all survived. Ten were placed on bypass, nine for stabilization before repair. Of the nine, seven (78%) were weaned from ECMO to conventional ventilation. Repair of the diaphragmatic defect was performed an average of 3.8 days later; none of these patients had severe pulmonary hypertension postoperatively, and all survived. Two could not be weaned before repair, one of whom had a complex congenital heart defect. This patient died. The other patient had repair on ECMO because of intrathoracic gastric volvulus. Severe blood loss prompted decannulation, and the patient died. One patient who was placed on bypass was transferred 10 days after having had repair elsewhere (at 4 hours of age). Pulmonary hypertension did not resolve, and the postmortem examination showed alveolar capillary dysplasia, with focal misalignment of the pulmonary vessels.(ABSTRACT TRUNCATED AT 250 WORDS)

Misalignment of pulmonary veins with alveolar capillary dysplasia (MPV) has been reported to be a rare cause of persistent pulmonary hypertension of the newborn (PPHN) and to be fatal despite extracorporeal membrane oxygenation (ECMO). A full-term female neonate with PPHN was brought to the hospital for ECMO therapy at 2 days of age. On the 14th day of life, she was extubated early after the second run of ECMO, and underwent nitric oxide (NO) inhalation therapy in the incubator. She died of catheter-related sepsis on the 61st day of life. After autopsy findings revealed MPV, the longest survival with this disease was documented. NO inhalation therapy in the incubator may provide time for lung transplantation.