Spontaneous Chylothorax in Noonan Syndrome

Goens, M. Beth; Campbell, David I.; Wiggins, James W.

doi:10.1001/archpedi.1992.02160240063021

Cited by 32 publications

(15 citation statements)

References 26 publications

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“…Associations with congenital heart disease, chromosomal disorders (eg, Down, Turner or Noonan syndrome7), prenatal infections, birth trauma, thrombosis of the superior vena cava or other malformations (eg, primary congenital pulmonary lymphangiectasis) have been reported 4 8–10. The male/female ratio is said to be 2:1 9.…”

Section: Introductionmentioning

confidence: 99%

Congenital chylothorax: a prospective nationwide epidemiological study in Germany

Bialkowski

Poets

Franz

2014

Arch Dis Child Fetal Neonatal Ed

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Section: Introductionmentioning

confidence: 99%

Congenital chylothorax: a prospective nationwide epidemiological study in Germany

Bialkowski

Poets

Franz

2014

Arch Dis Child Fetal Neonatal Ed

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“…Secondary disorders present later in life and are associated with other congenital and genetic diseases such as Noonan and Down's syndrome. 3 The most common defect is pulmonary stenosis, which can be accompanied by atrial or ventricular septal defects, patent ductus arteriosus, and hypertrophic cardiomyopathy. It is an autosomal dominant disease with variable expression and it affects both men and women.…”

Section: Discussionmentioning

confidence: 99%

Chylothorax Associated with Noonan Syndrome

Wohlrab¹,

Read²

2002

Journal of Bronchology

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Treatment of chylous pleural effusions with chest tube thoracostomy and pleurodesis has had limited success despite the sclerosing agent used. It also usually requires prolonged drainage, which could lead to nutritional and immune compromise. We report a case of a 41-year-old man with a recurrent chylothorax secondary to Noonan syndrome that was treated successfully with medical thoracoscopic talc pleurodesis. He presented initially with symptoms suggestive of pneumonia, and a chest radiograph showed a right effusion. Pleural studies revealed the effusion was a chylous exudate with negative cytology and cultures. He was treated initially with antibiotics, but the effusion recurred and he required five subsequent thoracenteses for symptomatic relief. He eventually un-derwent medical thoracoscopy with talc pleurodesis for definitive management and is without recurrence 4 months postoperatively. Spontaneous chylothorax associated with Noonan syndrome is a rare complication that typically affects children and can be managed easily with enteric rest. This option is less feasible in an active adult patient, and therapy should include medical thoracoscopic talc pleurodesis because it is more likely to be successful than chest tube thoracostomy pleurodesis.

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“…Such occurrences can occasionally be associated with pulmonary lymphangiectasia or appear postoperatively after cardiac surgery. Goens et al [1992] summarized the 11 reported cases of congenital (n ‫ס‬ 1), spontaneous (n ‫ס‬ 5), and post-operative (n ‫ס‬ 5) chylothorax and noted that all but two eventually resolved. Histologically, the distribution of dilated lymphatic channels can be localized, leading to isolated chylothorax, or diffuse, accounting for accompanying pulmonary lymphangiectasia.…”

Section: Discussionmentioning

confidence: 99%

“…Histologically, the distribution of dilated lymphatic channels can be localized, leading to isolated chylothorax, or diffuse, accounting for accompanying pulmonary lymphangiectasia. Steroid therapy has been reported on an anecdotal basis as being successful [Goens et al, 1992] if chemical or surgical pleurodesis and restriction of dietary triglyceride fails.…”

Section: Discussionmentioning

confidence: 99%