Outcome of children with pulmonary lymphangiectasis.

Pulmonary lymphangiectasis (PL) is a very rare developmental defect of the lungs, which has previously been reported to have a very poor prognosis. However, recent reports have suggested improved outcomes, possibly as a result of advances in neonatal and pediatric intensive care medicine. We performed a retrospective study on the outcome of children with PL between 1990 and 2008 referred to our tertiary center. Seven patients with histologically proven PL were identified over the 18-year period. Six patients presented in the neonatal period and one patient at 7 months of age, all of them requiring intensive care treatment. Three neonatal patients required extracorporeal membrane oxygenation (ECMO). Six of the seven patients did not survive including all those who received ECMO. Two of the six non-survivors died of other causes than their underlying disease. The only survivor had an antenatal diagnosis of hydrops and required in utero chest drain insertion. Postnatally he was managed with maximal medical treatment for bilateral pleural effusions and persistent pulmonary hypertension. A 7-month follow-up showed this infant to be doing well. In conclusion overall the prognosis of congenital PL remains poor. The one survivor demonstrates that this condition is survivable with aggressive intervention and as current evidence suggests gradual improvement of symptoms may occur over time, and that maximal medical treatment remains warranted.

Portopulmonary hypertension in childhood presenting as sudden death.

We present the case of a 9-year-old boy with portal hypertension who died suddenly and unexpectedly due to pulmonary hypertensive crisis during a routine endoscopic procedure. He had known portal hypertension with esophageal varices but had no preceding clinical symptoms suggestive of significant pulmonary hypertensive disease despite postmortem histological evidence of advanced pulmonary vascular changes. Portopulmonary hypertension is a well-described and distinct clinical syndrome that is rare in childhood and is associated with a relatively poor prognosis. Occasional patients with histologically advanced disease may remain asymptomatic but present with pulmonary hypertensive crisis. Children with portopulmonary hypertension should be considered at high risk for surgical procedures, and pulmonary hypertensive complications should be excluded as a cause of death in all children dying suddenly in the setting of portal hypertension.