Acute Illness in a Patient With a Chronic Cough Since Birth

Jason M. Richerson, MD, Benjamin Estrada, MD, Michael M. Fuenfer, MD, Andrew B. Walker, MD


December 06, 2002

Review Questions

What is a pulmonary sequestration?

  1. A pulmonary sequestration is a congenital anomaly of the lung characterized by cystic, nonfunctioning pulmonary tissue, usually nourished by systemic arteries more frequently from the thoracic and abdominal aorta. A pulmonary sequestration lacks communication with the tracheobronchial tree.

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Are there different types of pulmonary sequestration?

  1. There are 2 types of pulmonary sequestration: (1) intralobar or intrapulmonary sequestration (ILS), and (2) extralobar sequestration (ELS). ILS is contained within the lung; ELS is covered by its own pleura.

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What are the epidemiologic characteristics of pulmonary sequestration?

  1. In the United States, the frequency of pulmonary sequestration is approximately 6%. The gender preponderance and ratios are:

    • ILS: males:females = 1:1

    • ELS: males:females = 4:1

    • ILS:ELS = 4:1

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At what age does pulmonary sequestration usually present?

  1. The intralobar type is typically diagnosed after adolescence, although symptoms may occur early in childhood, presenting as multiple episodes of pneumonia and usually due to fistula formation between the sequestration and the airway. The extralobar type is typically diagnosed during infancy, secondary to other congenital anomalies that are usually present (eg, cardiac malformations, gastrointestinal malformations, cervical vertebral anomalies, and congenital diaphragmatic hernia). Infection may lead to detection of this type of sequestration, as was the case with the patient presented here. Patients with this type of sequestration may also experience chronic cough.

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What are the common clinical findings in a patient with a pulmonary sequestration?

  1. Pulmonary sequestration often presents with findings of consolidation on lung exam. If the lesion is superinfected, patients may also present with fever and crackles.

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What studies are most helpful in the diagnosis of pulmonary sequestration?

  1. Routine chest x-rays, CT scan, and MRI are helpful in the preoperative assessment of patients for whom pulmonary sequestration is suspected. Arteriograms are also helpful in the evaluation of the lesion's vascular supply.

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What are the therapeutic options for patients with a pulmonary sequestration?

  1. The treatment of choice for a pulmonary sequestration is surgical resection. If the lesion is intralobar, lobectomy is usually indicated. However, in cases of ELS, the lesion can be separated and lobectomy is not always required.

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