Lymphatic obstruction and protein-losing enteropathy (lymphangiectasia) in patients with congenital heart disease.

Lymphatic obstruction and protein-losing enteropathy (lymphangiectasia) in patients with congenital heart disease.

Congenit Heart Dis. 2008 Jul

Meadows J, Gauvreau K, Jenkins K.
Department of Cardiology, Children's Hospital, Boston, MA, USA. jeffery.meadows@ucsf.edu

OBJECTIVE: Protein-losing enteropathy (PLE) is a known complication of surgical procedures for congenital heart disease. The pathogenesis and pathophysiology of PLE remain poorly understood. However, lymphatic insufficiency appears central to the disease process. We sought to investigate the role of lymphatic obstruction and central venous catheter-related central venous thrombosis in patients with congenital heart disease and PLE.

DESIGN: A case-control study design was constructed consisting of patients with congenital heart disease and PLE and 2:1 matched controls having undergone the same definitive surgical procedure. Obstruction to lymphatic return was considered present if the thoracic duct was ligated, or if there was complete central venous obstruction at the usual site of thoracic duct drainage.

RESULTS: Obstruction to lymphatic return was identified in 4 of 16 cases (25%) and 1 of 32 controls (4%), P = .06. There was no association between PLE and central venous catheter use or duration, and no discriminating characteristics between cases and controls with respect to anatomy, pre-Fontan hemodynamic variables, operative or perioperative factors, or hemodynamic variables at the time of PLE diagnosis. Mortality for patients with PLE was 25% compared with 9% in controls (P = not significant). Long-term resolution of PLE was obtained in six patients (38%).

CONCLUSION: There is a high prevalence of apparent lymphatic obstruction in patients with congenital heart disease and PLE, suggesting that physical lymphatic obstruction may play an important, and previously unrecognized role in the development of PLE in patients with complex congenital heart disease.

PMID: 18715461 [PubMed - indexed for MEDLINE]

A case of Hennekam syndrome presenting with massive pericardial effusion.]

A case of Hennekam syndrome (lymphangiectasia) presenting with massive pericardial effusion.
Turk Kardiyol Dern Ars. 2008 Jul
Nişli K, Oner N, Kayserili H, Ertuğrul T.
Department of Pediatrics, Istanbul Medicine Faculty of Istanbul University, Istanbul, Turkey. kemalnisli@yahoo.com.

Hennekam syndrome is an autosomal recessive disease characterized by intestinal lymphangiectasia accompanied by severe lymphedema of the limbs, genitalia, and face, and learning difficulties. A 38-month-old boy was admitted with breathing difficulty. He had facial abnormalities and preputial hyperplasia consistent with Hennekam syndrome. Lymphangiography showed lymphedema in the left eye and right foot. Teleradiography showed cardiomegaly and echocardiography showed massive pericardial effusion. He first underwent pericardiocentesis for the removal of pericardial effusion, but pericardial tube drainage was required upon recurrence of effusion. On the fifth day, the drain was removed because of significant decrease in the drainage.

Full Text Article PDF

Computed tomographic scan in the diagnosis of bilateral renal lymphangiectasia.

Computed tomographic scan in the diagnosis of bilateral renal lymphangiectasia.
Saudi J Kidney Dis Transpl. 2008 Nov

Rastogi R, Rastogi V.
Yash Diagnostic Center, Yash Hospital and Research Center, Civil Lines, Kanth Road, Moradabad (UP), India. eesharastogi@gmail.com.

Renal lymphangiectasia is a rarely reported disorder of lymphatic malformation. Although benign, it may lead to hypertension and renal failure in undetected or undiagnosed cases. Adult polycystic kidney disease is a close mimic. We herewith present a rare case of bilateral renal lymphangiectasia that was strongly suspected on Computed tomographic (CT) scan and confirmed by aspiration cytology. Awareness about this condition will result in early diagnosis, early treatment and reduced morbidity.

PMID: 18974588 [PubMed - in process]