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At a glance

Obstructive azoospermia, chronic sinopulmonary infections, and bronchiectasis without ciliary dysfunction. There are significant respiratory implications that will impact the conduct of anesthesia and postoperative care.

Synonyms

Sinusitis Infertility Syndrome; Barry Perkins Young Syndrome; Obstructive Azoospermia and Chronic Sinopulmonary Infections.

Incidence

Unknown, possibly declining in general population.

Genetic inheritance

Autosomal recessive. High rate of spontaneous mutation. Possible mercury exposure during childhood.

Pathophysiology

Prolonged mucociliary clearance time (in the presence of normal structure) resulting in chronic sinopulmonary infections. The incidence and severity of infections usually improve after adolescence (unlike with cystic fibrosis). Congenital or acquired obstruction to sperm outflow (between the caput and body of the epididymis) in the presence of normal spermatogenesis results in azoospermia and infertility.

Diagnosis

Combination of azoospermia and chronic sinopulmonary infection in the presence of normal spermatogenesis and hormone function with surgical findings of obstruction to sperm flow are suggestive of the diagnosis. Diagnosis of Young Syndrome is by exclusion of two similar syndromes—cystic fibrosis (screen test for CFTR mutations) and ☞Immotile Cilia Syndrome.

Clinical aspects

History of recurrent cough and sputum production in childhood. The respiratory symptoms improve after adolescence and only mild residual impairment in pulmonary function, as evidenced by mild decreases in residual volume and peak expiratory flow rate, remains. Chest and sinus radiographic abnormalities consistent with chronic sinopulmonary infections are common. The respiratory tract is not colonized with organisms unlike in cystic fibrosis. These patients most frequently present to infertility clinics later in life.

Precautions before anesthesia

Thorough assessment of the respiratory system is required with clinical evaluation alone often adequate. If indicated by clinical examination, chest radi­ography and pulmonary function tests may be required. It is important to exclude cystic fibrosis or ☞Immotile Cilia Syndrome.

Anesthetic considerations

In view of chronic sinopulmonary infections and mild decreases in pulmonary function tests, patients may be at greater risk for postoperative atelectasis. Regional anesthesia may be appropriate for intra- and postoperative pain management. If pulmonary disease is significant, inhalational induction may be prolonged secondary to slow uptake resulting from an abnormal ventilation/perfusion (V/Q) ratio. Laryngospasm and cough must be expected in Young Syndrome patients. Similar to other patients with chronic airway inflammation, bronchial reactivity is potentially increased. Nasal intubation and nasogastric tubes should probably be avoided because of the presence of sinus infection.

Pharmacological implications

Anticholinergic agents may make pulmonary secretions more tenacious and difficult to clear. Muscle relaxants should be used only with peripheral nerve stimulators because in association with high-dose antibiotics, especially aminoglycosides, the action of nondepolarizing muscle relaxants can be significantly prolonged.

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