A rare medical condition characterized by the clinical
presentation of discolored, hypoplastic nails, recurring pleural effusions,
lymphedema, recurrent pneumonia and lymphedema.
Lymphedema and Yellow Nails.
Unknown (between 1927 and 1960, 10 cases
reported in the literature), more
frequent in the presence of severe rhinosinusitis symptoms and immunological
Can be caused by mutation in the forkhead family
transcription factor gene MFH1 located on 16q24.3.
Two of the following criteria must be present:
slow-growing nails (89% of cases), lymphedema (80% of cases),
pleuropulmonary symptoms (63% of cases) (pleural effusion, recurrent
pneumonitis, bronchiectasis, rhinosinusitis).
The characteristics of the nails include
thickening, diminished growth, and onycholysis. The color may vary from a
pale yellow to green. The edema is the initial symptom in one-third of
cases. Although it mainly occurs in the lower limb, in time edema also
affects the genitalia, hands, face, and vocal cords. Respiratory tract is
involved with pleural effusion, restrictive or obstructive defects that are
poorly responsive to bronchodilatators. Bronchiectasis, severe
rhinosinusitis, and laryngeal edema can also be present. These patients may
present chylous ascites and pericardial effusion. Some authors report a lack
of IgG2. This illness is well known in adults, often occurring with a late
onset. One case report described a nonimmune fetal hydrops and recurrent
left chylothorax at 4 weeks of age in an infant with maternal Yellow Nail
In the presence of yellow nails, ask
about recurrent respiratory infections and watch for the consequences of
lymphedema (previous pleural, pericardial or ascitic effusion). Obtain an
echocardiography for the exclusion of pericardial effusion.
Pulmonary tests are indicated to assess the
severity of the respiratory tract involvement. A chest radiograph should be
obtained to eliminate the presence of bronchiectasia. Ask about any changes
in the voice. If a voice change is present, an orolaryngotracheal examination is useful to assess
the extent of the laryngeal edema.
If an alternative to tracheal intubation
is available, it must be considered in the evaluation of the case because of
the lymphedematous involvement of each part of the respiratory system. The venous access
should prefer large veins and should be maintained for a duration as short as possible
because of generalized, congenitally hypoplastic lymphatics.
Postoperative chest physiotherapy should be considered. Consider patients as
suffering from recurrent airway infection with high respiratory reactivity.
No interactions are known with
anesthetic medications. If tracheal intubation is needed, the
prophylactic administration of antibiotic is recommended. The use of
muscle relaxants should be considered once the airway is secured and
lung ventilation confirmed. Anticholinergic agents may make pulmonary secretions
more tenacious and difficult to clear.
Govaert P, Leroy JG, Pauwels R, et al: Perinatal manifestations of
maternal yellow nail syndrome. Pediatrics
Riedel M: Multiple effusions and lymphedema in the yellow nail syndrome.