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A 33-year-old female with first episode of sudden onset massive hemoptysis.

CLINICAL PRESENTATION

A 33-year-old, previously healthy African-American female was at work when she suddenly had a coughing spell with expectoration of approximately 150 ml of fresh blood without clots. She denied having chest pain, shortness of breath, weight loss, fever, chills, or any previous episode of hemoptysis. Her previous medical problems included newly diagnosed hypertension treated with dietary salt restriction, weight reduction, and lisinopril generic 20 mg once daily. She reported an upper respiratory tract infection one week prior and was given a single injection of steroid, which improved her symptoms. Her physical examination was normal, and laboratory investigations were unremarkable. A chest X-ray and contrast enhanced computed tomography (CT) scan of the chest was obtained at an outside facility (Figure 1), and she was later transferred to our center for further management.

QUESTIONS

1. What is the diagnosis?

2. What tests would be most helpful in confirming the diagnosis?

3. What is the management for this condition?

4. What are the possible complications?

Answers on p. 210

ANSWERS

Pulmonary sequestration is an embryonic malformation forming a nonfunctional cystic lung tissue which is not connected to the normal tracheobronchial tree and receives its blood supply aberrently from systemic circulation. (1) It is caused by failure of obliteration of the embryonic splanchnic vessels supplying the lung tissues. (1) Although it is usually asymptomatic, it can become symptopmatic at any age, i.e. presenting with hemoptysis, back pain, respiratory distress, and heart failure (secondary to the shunt), mostly during adolesence. (1)

As the most common congenital abnormality of the lung, it comprises 0.15-6.4% of all congenital pulmonary malformations and most commonly involves the basal segments (left > right). (2) The abberant artery in intralobar sequestration (located within the normal visceral pleura) usually (75% of cases) originates from the thoracic aorta, while the venous return is almost always (95.7%) via the pulmonary veins. (2) Multiple anomalous arteries are seen in 15% of the cases. (2,3)

Extralobar pulmonary sequestration, on the other hand, is less common (comprises 25% of the pulmonary sequestrations), has its own visceral pleura, and drains through an anomalous venous drainage into systemic veins routing to the right atrium. (2) Computed tomography, selective angiography, and magnetic resonance angiography (4) can reveal the anomalous blood supply and confirm the diagnosis.

Management includes surgical resection with thora cotomy or video-assisted thoracoscopic surgery (VATS) aiming to avoid complications such as massive hemoptysis and infection. Sequestrectomy (resection of the sequestered lobe) for extralobar type and lobectomy versus segmentectomy (as performed in this case) for the intralobar varients are the possible surgical options. (2) In the current case, the diagnosis was intralobar pulmonary sequestration located in the right lower lobe.

A right posterolateral thoracotomy was performed, and the sequestered tissue was found at the base of the right lower lobe (Figure 2A). The large aberrent feeding artery branching off from the right side of the thoracic aorta was identified and stapled (Figure 2B, 2C). Subsequently, the wedge resection of the sequestered tissue in the lower parts of the right lower lobe was performed (Figure 2D, 2E). Pathological examination revealed a prominent, thick-walled anomalous arterial vasculature. The pulmonary arteries in the resected specimen (Figure 2F) showed prominent medial hypertrophy with narrowed lumens, plexiform vessels, and thrombus formation with focal recanalization occurring in a background of extensive pulmonary hemorrhage andinfarction. The changes were consistent with pulmonary hypertension, with extensive hemorrhagic infarction confirming the diagnosis of bronchopulmonary sequestration with anomalous arterial supply. Postoperatively, the patient did not have any complications and was discharged home five days after surgery.

REFERENCES

(1.) Kayhan S, Celik B, Belet U, Aydin O. Intralobar pulmonary sequestration as an unusual cause of recurrent hemoptysis. J Clin Imaging Sci 2012; 2:71.

(2.) Savic B, Birtel FJ, Tholen W, et al. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979; 34(1):96-101.

(3.) Theodoropoulos I, Schwartz MZ. Intralobar pulmonary sequestration: an uncommon case with triple arterial supply and systemic venous drainage. Pediatr Surg Int 2012; 28(7):741-4.

(4.) Muller NL. Computed tomography and magnetic resonance imaging: Past, present and future. Eur Respir J Suppl 2002; 35:3-12s.

Drs. Hamidian Jahromi and Rao are in the Department of Surgery at Louisiana State University Health Sciences Center in Shreveport. Dr. Skweres is in the Department of Radiology at LSUHSC-Shreveport.

Alireza Hamidian Jahromi, MD; Justin Skweres, MD; Vyas R. Rao, MD
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Author:Jahromi, Alireza Hamidian; Skweres, Justin; Rao, Vyas R.
Publication:The Journal of the Louisiana State Medical Society
Article Type:Case study
Date:Jul 1, 2013
Words:732
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