The role of aortopexy in severe tracheomalacia.

PURPOSE: Aortopexy is the accepted operative treatment for severe and localized tracheomalacia (TM). The standard surgical approach involves a left anterior thoracotomy, often under bronchoscopic control. We report the results of aortopexy in 28 children with severe and localized TM; 12 had a left lateral muscle-sparing approach and one had a thoracoscopic aortopexy. METHODS: Retrospective review of patient notes was performed to note the indications, investigation findings, and postoperative course after aortopexy. RESULTS: The median age at aortopexy was 5 months. The indications included acute life-threatening events in 22, failure to extubate in 5, and recurrent pneumonia in 1. Fifteen had associated esophageal atresia and 13 had primary TM. Symptoms of TM were abolished in 26 of the 28 patients after aortopexy. CONCLUSIONS: Aortopexy is a safe and reliable procedure to treat localized intrathoracic TM presenting with acute life-threatening events. It is important to exclude associated problems such as vascular rings and to ensure that the tracheomalacic portion is segmental and does not significantly involve the main bronchi. The lateral muscle-sparing thoracotomy provides good access and is more cosmetic than the standard anterior approach. We would attempt the thoracoscopic approach in older infants and children.

Successful resection of localized intestinal lymphangiectasia post-Fontan: role of (99m)technetium-dextran scintigraphy.

Intestinal lymphangiectasia is a well-recognized complication of the Fontan procedure, occurring in up to 24% of patients. Because of the loss of chylous fluid into the gut lumen, protein-losing enteropathy results as well as lymphopenia and hypogammaglobulinaemia. In some cases, dilated lymphatics in the intestinal serosa or mesentery also rupture, causing chylous ascites. Standard medical and cardiac surgical interventions are generally ineffective and the condition is frequently lethal. We report a case of intractable and life-threatening chylous ascites and chylothorax in a 14-year-old girl, associated with intestinal lymphangiectasia and protein-losing enteropathy after a Fontan procedure for tricuspid atresia. The condition was refractory to all standard medical therapies, including dietary modifications, diuretics, corticosteroid therapy, albumin infusions, octreotide, heparin, bowel rest, and parenteral nutrition. Cardiac surgery to optimize her hemodynamic status was also ineffective and large volume pleural and ascitic fluid losses continued. Having exhausted all other therapeutic modalities, (99m)technetium-dextran scintigraphy was performed to assess the extent of intestinal protein loss and the potential for surgical intervention. Scintigraphy suggested localized protein loss from the proximal jejunum and subsequent segmental resection was effective. Postoperatively, ascites and pleural effusions resolved, and there was no evidence of short bowel syndrome. Growth has accelerated and the patient has entered puberty. There is mild persistent intestinal protein loss requiring diuretic therapy. Ascites or pleural effusions are absent, and the patient remains well >2 years after surgery. Intestinal lymphangiectasia post-Fontan procedures has traditionally been ascribed to hemodynamic factors such as raised systemic venous pressure, which would predispose to a generalized intestinal lesion. However, in this case, scintigraphy demonstrated a localized, surgically correctible lesion. To our knowledge, this is the first reported case of the use of (99m)technetium-dextran scintigraphy for this indication and of successful partial small bowel resection in such a case.