PULMONARY AGENESIS WITH INTRATHORACIC UPPER-LIMB

A 10-week-old boy was referred because of respiratory distress, absenc e of right upper limb, and a mass in the right upper chest and shoulde r region. Investigations showed agenesis of the right lung; the right hemithorax was filled by a mass that was mainly cystic but also contai ned a deformed right upper limb. When surgery was performed at the age of 13 weeks, the mass was delivered out of the chest and removed toge ther with the deformed limb. The upper 5 cm of the shaft of the humeru s could be preserved, and the chest wall could be repaired satisfactor ily. Recovery was uneventful. The embryology of the malformation is br iefly discussed. Copyright (C) 1997 by W.B. Saunders Company.