### At a Glance

A rare medical condition characterized by the clinical presentation of discolored, hypoplastic nails, recurring pleural effusions, lymphedema, recurrent pneumonia and lymphedema.

### Synonym

Lymphedema and Yellow Nails.

### Incidence

Unknown (between 1927 and 1960, 10 cases reported in the literature), more frequent in the presence of severe rhinosinusitis symptoms and immunological disorders.

### Genetic Inheritance

Autosomal dominant.

### Pathophysiology

Can be caused by mutation in the forkhead family transcription factor gene MFH1 located on 16q24.3.

### Diagnosis

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).

### Clinical Aspects

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 Syndrome.

### Precautions before Anesthesia

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.

### Anesthetic Considerations

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.

### Pharmacological Implications

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 ...

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

## Subscription Options

### AccessAnesthesiology Full Site: One-Year Subscription

Connect to the full suite of AccessAnesthesiology content and resources including procedural videos, interactive self-assessment, real-life cases, 20+ textbooks, and more