Pulmonary agenesis with intrathoracic upper limb
1Park Children's Centre for Treatment & Research, Calcutta, India.
Abstract:
A 10-week-old boy was referred because of respiratory distress, absence of right upper limb, and a mass in the right upper chest and shoulder region. Investigations showed agenesis of the right lung; the right hemithorax was filled by a mass that was mainly cystic but also contained 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 together with the deformed limb. The upper 5 cm of the shaft of the humerus could be preserved, and the chest wall could be repaired satisfactorily. Recovery was uneventful. The embryology of the malformation is briefly discussed.
Related Concept Videos
Changes in the Appendicular Skeleton with Age
Initially, the limb buds consist of a core of mesenchyme covered by a layer of ectoderm. The ectoderm at the end of the limb bud thickens to form a narrow crest called the apical ectodermal ridge. This ridge stimulates the underlying...
Arteries of the Upper Limbs
Atelectasis II: Pathophysiology
Pneumothorax II: Pathophysiology
Respiratory System Abnormal Finding II: Palpation and Auscultation
Palpation Findings
During a respiratory assessment, palpation can reveal several vital abnormalities:
Gross Anatomy of the Lungs

