@entity9676 is a well-recognized complication of the Fontan procedure, occurring in up to 24% of @entity1 . Because of the loss of chylous fluid into the gut lumen, @entity6518 results as well as @entity2905 and hypogammaglobulinaemia. In some cases, dilated lymphatics in the intestinal serosa or mesentery also rupture, causing @entity3417 . Standard medical and cardiac surgical interventions are generally ineffective and the condition is frequently lethal. We report a case of intractable and life-threatening @entity3417 and chylothorax in a 14-year-old @entity1 , associated with @entity9676 and @entity6518 after a Fontan procedure for @entity1145 . The condition was refractory to all standard medical therapies, including dietary modifications, diuretics, corticosteroid therapy, albumin infusions, @entity2390 , 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, @entity1136 -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, @entity843 and @entity1143 resolved, and there was no evidence of @entity5277 . Growth has accelerated and the @entity1 has entered puberty. There is mild persistent intestinal protein loss requiring diuretic therapy. Ascites or @entity1143 are absent, and the @entity1 remains well >2 years after surgery. @entity9676 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 @entity1136 -dextran scintigraphy for this indication and of successful partial small bowel resection in such a case.

Successful resection of localized XXXX post-Fontan: role of @entity1136 -dextran scintigraphy.

@entity1 :: ('9606', 'Species') :: ['girl', 'patients', 'patient'] @entity1136 :: ('MESH:D013667', 'Chemical') :: ['(99m)technetium'] @entity1143 :: ('MESH:D010996', 'Disease') :: ['pleural effusions'] @entity2905 :: ('MESH:D008231', 'Disease') :: ['lymphopenia'] @entity1145 :: ('MESH:D018785', 'Disease') :: ['tricuspid atresia'] @entity843 :: ('MESH:D001201', 'Disease') :: ['ascites'] @entity6518 :: ('MESH:D011504', 'Disease') :: ['protein-losing enteropathy'] @entity3417 :: ('MESH:D002915', 'Disease') :: ['chylous ascites'] @entity2390 :: ('MESH:D015282', 'Chemical') :: ['octreotide'] @entity5277 :: ('MESH:D012778', 'Disease') :: ['short bowel syndrome'] @entity9676 :: ('MESH:D008201', 'Disease') :: ['Intestinal lymphangiectasia', 'intestinal lymphangiectasia']
@entity9676 :: (MESH:D008201,Disease) :: ['intestinal lymphangiectasia']
