ANSWERDIFFERENTIAL DIAGNOSIS OF CHYLOTHORAX
Primary Lymphedema, Idiopathic Effusion
Yellow Nail syndrome, Congenital Chylothorax
Lymphatic Injury Lymphangioma; acquired progressive
Lymphatic abnormalities & inherited disorders
Embryology: Lymphangiomas are likely embryologic remnants of lymphatic tissue that either failed to connect to efferent channels or arose from portions of lymph sac sequestration.
Clinical Features: Lymphangiomas usually present within the first 2 years of life as swelling in head, neck, axillae. Patients can complain of cough, dyspnea, stridor, hemoptysis, Horner's Syndrome, dysphagia, superior vena caval syndrome, constrictive pericarditis, or phrenic nerve palsy
Lymphangiectasia: Pathologic dilation of lymphatics
Two Forms: Primary (congenital): usually fatal
Secondary: Either impaired lymph drainage and/or increase lymph production
Embryology: Likely failure of pulmonary interstitial connective tissue regression that normally occurs in the fifth month of life that leads to dilation of pulmonary lymphatic capillaries
Clinical Features: Presents soon after birth. The dilated lymphatics with chylothorax lead to pulmonary hypoplasia and respiratory failure
Two Forms: Single organ system involvement e.g. diffuse pulmonary lymphangiomatosis Multiple organ system involvement (75%)
Embryology: Likely due to lymphatic developmental abnormality but presents at a later age because influence of hormonal factors more subtle
Clinical Features: Patients frequently presents in late childhood (no sex preference). Lesions most often occur in the neck and thorax. Up to 75% of patients can have bony involvement and chylous effusions are common.
Dysplasia: A heterogenous group of disorders
Primary Lymphedema (age of presentation)
Lymphedema Congenita (neonates)
Lymphedema Precox (<35 years old)
Lymphedema Tarda (> 35 years old)
Pleural, Pericardial, Peritoneal
Yellow Nail syndrome; impaired lymphatic drainage triad of idiopathic pleural effusions, lymphedema, dystrophic nails
Congenital Chylothorax (15% of all chylothorax cases)
Clinical Features: History of insidious onset dyspnea, fever, pleuritic chest pain. Chyloptosis is rare.
Prognosis: Variable and depends on gestational age, presence of other abnormalities, and the severity of pulmonary hypoplasia.
abnormalities and inherited disorders
Congenital anomaly syndromes
Congenital Chylothorax AR
Familial Milroy Lymphedema AD (variable penetrance)
Primary Chylous effusions
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