ANSWER
DIFFERENTIAL DIAGNOSIS OF CHYLOTHORAX– Lymphangioma
– Lymphangiectasia
– Lymphangiomatosis
– Lymphatic Dysplasias
– Primary Lymphedema, Idiopathic Effusion
– Yellow Nail syndrome, Congenital Chylothorax
– Lymphatic Injury Lymphangioma; acquired progressive
– Lymphangiosarcoma
– Lymphangioleiomyomatosis (LAM)
– Hemangiolymphangiomas
– Lymphangiolipoma
– Lymphatic abnormalities & inherited disorders
Lymphangiomas
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
Lymphangiomatosis:
Multiple Lymphangiomas
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.
Lymphatic
Dysplasia: A heterogenous group of disorders
Classification:
–
Primary
Lymphedema (age of presentation)
–
Lymphedema
Congenita (neonates)
–
Lymphedema
Precox (<35 years old)
–
Lymphedema
Tarda (> 35 years old)
–
Idiopathic
Effusion(s)
–
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.
Lymphatic
abnormalities and inherited disorders
–
Congenital
anomaly syndromes
–
Achondroplasia
I
–
Aneuploidy
syndromes
–
Turners
Syndrome
–
Congenital
Chylothorax –
AR
–
Familial
Milroy Lymphedema –
AD (variable penetrance)
–
Gorham's
Syndrome
–
Primary
Chylous effusions
BASED
ON THE ABOVE INFORMATION, WHAT IS THE DIFFERENTIAL DIAGNOSIS FOR OUR
PATIENT?
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