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At a glance

A very rare medical condition characterized by the clinical presentation of discolored, hypoplastic nails, recurring pleural effusions, lower limb lymphedema, and recurrent pneumonia. Onset usually after 50 years of age but juvenile and familial forms observed.

Synonym

Lymphedema and Yellow Nails.

Incidence

Fewer than 400 cases reported. Estimated prevalence of less than 1/1,000,000. Pediatric forms are rarely described and may be congenital or develop before 10 years of age. More frequent in the presence of severe rhinosinusitis symptoms and immunological disorders.

Genetic inheritance

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

Pathophysiology

Impaired lymph transport leads to lymp­hedema, pleural effusion (chylothorax), and nail discoloration. Hypothetical role of tissue titanium accumulation.

Diagnosis

Two of the following criteria must be present: slow-growing discolored nails, lower limb lymphedema, pleuropulmonary symptoms (pleural effusion, recurrent pneumonitis, bronchiectasis, rhinosinusitis). Children with Yellow Nail Syndrome have lung manifestations in more than 75% and lymphedema 56 to 80%.

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 bronchodilators. Bronchiectasis, severe rhinosinusitis, and laryngeal edema can also be present. These patients may present chylous ascites and pericardial effusion. This syndrome has a predominantly a late onset in adult life. Lymphedema is the usually the first symptom in children.

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). Echocardiography may be required to exclude pericardial effusion. Pulmonary function tests are indicated to assess the severity of the respiratory tract involvement. A chest radiograph should be obtained to eliminate the presence of bronchiectasia. If a voice changes are present, an orolaryngotracheal examination is useful to assess the extent of a laryngeal edema.

Anesthetic considerations

Respiratory symptoms dictate anesthetic management. If an alternative to tracheal intubation is available, it should be considered. Use large veins for intravenous access and use minimal duration because of generalized, congenitally hypoplastic lymphatics. Postoperative chest physiotherapy should be considered. Consider patients as suffering from recurrent airway infection with highly reactive airways.

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 ...

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