PEDIATRICS Vol. 89 No. 6 June 1992, pp. 1016-1018
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Perinatal Manifestations of Maternal Yellow Nail Syndrome

Paul Govaert MD1, Jules G. Leroy MD1, Romain Pauwels MD2, Piet Vanhaesebrouck MD1, Claudine De Praeter MD1, Henri Van Kets MD3, and Marleen Goeteyn MD4

1 From the Department of Pediatrics, University Hospital Gent, Belgium
2 From the Department of Respiratory Diseases, University Hospital Gent, Belgium.
3 From the Department of Obstetrics, University Hospital Gent, Belgium
4 From the Department of Dermatology, University Hospital Gent, Belgium

A term female firstborn infant had unexplained nonimmune fetal hydrops and recurrent left chylothorax at 4 weeks of age. A few months before conception, her mother had had acute dystrophic nail changes and is being treated for recurrent sinusitis, bronchiectasis, and a deficiency of serum IgG2. We suggest that they both suffer from a dominantly inherited congenital lymphedema syndrome known as `yellow nail dystrophy.' Prenatal manifestation of this disorder has not been reported previously. The child's anthropometric and neurological development was normal at 1 year of age, whereas mild ankle edema and marbling of the skin of the limbs were salient clinical findings. Inherited lymphedema leading to nonimmune fetal hydrops also has been recognized in chromosomal disorders, Noonan's syndrome, multiple pterygium syndrome, pulmonary lymphangiectasis, and mixed-vessel lymphatic dysfunction. Indicators of parental lymphedema are not on record in those instances.

Key Words: hydrops fetalis • chylothorax • lymphedema • yellow nails

Submitted on June 12, 1991
Accepted on September 9, 1991


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