The disorder may affect the head (cloverleaf skull,
dolichocephaly or scaphocephaly, marked deformation of the face, cervical cystic
hygroma or lymphocele, redundant subcutaneous tissue, severe cervical edema,
microphthalmos, cleft palate), the limbs (tetramelic camptomelia with bowed
diaphyses, micromelia, and brachydactyly talipes equinovarus), the heart
(dextrocardia, total anomalous pulmonary venous drainage, left-sided superior vena
cava, aortic arch anomalies), the lungs (hypoplasia and/or abnormal lobation in a
short, bell-shaped chest), the kidneys (polycystic kidney disease), the liver
(hepatomegaly, multiple liver cysts), and other organs (multiple pancreatic cysts
and anatomical anomalies, polysplenia, short bowel, ectopic or undescended testes,
absent uterus and Fallopion tubes). Lymph edema may be generalized. Some
researchers consider heterotaxia to be part of this syndrome.