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Congenital cystic adenomatoid malformation and arteriovenous malformation: An unusual pulmonary association

Jean-François Lecompte, Daniel Pop, Nicolas Venissac, Jérôme Mouroux

Department of Thoracic Surgery, Pasteur Hospital, Nice, France


Abstract

Pulmonary malformations are usualy an isolated congenital anomaly. They are suspected in pediatric period in most of case, with prenatal ultrasound or pulmonary infection. We report a case of young man who presented congenital cystic adenomatoid malformation with arterio venous malformation diagnosed after spontaneous pneumothorax.

 

Correspondence

Jean-François Lecompte
Department of Thoracic Surgery
Pasteur Hospital, 30 Avenue de la Voie Romaine, 06002 Nice, France
Tel.: 0033492030316
Fax: þ33 4 92030486
E-mail : This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Introduction

Congenital cystic adenomatoid malformation (CCAM) is a rare developpemental anomaly of the lower respiratory tract. It is usualy isolated, and diagnosed in prenatal period or childhood period. Presentation in adulthood is uncommon. Affected patients may be completely asymptomatic or present with severe respiratory distress. We report here the case of a young adult with congenital cystic adenomatoid malformation associated with arteriovenous malformation in the same lobe.

Case report

A 23-year-old man was admited in our hospital for a left spontaneous pneumothorax. In his medical history, we noted only 2 years before a bleeding gastric lipoma requiring a partial gastrectomy. In front of persistent air-leak after seven days, surgery was planed.

The preoperative check-up showed on chest CT scan: a large cystic adenomatoid malformation of the left upper lobe (fig 1) associated with an arteriovenous malformation (fig. 2) and basal body fat left chest. In spirometry we had FEV1 = 40.12% and FVC= 76.26%. Ventilation-perfustion scintigraphy showed obvious impairment on lung fonction, left lung being responsible for 10% of ventilation and 13 % of vascular fonction.

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Figure 1: CT scan - a large cystic adenomatoid malformation of the left upper lobe

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Figure 2: CT scan - cystic adenomatoid malformation of the left upper lobe associated with an arteriovenous malformation

We have done a postero-lateral left thoracotomy. The CCAM was developed in fact from the lower lobe. The upper lobe was pushed forward and down. The arteriovenous malformation was seen in the apex of this lower lobe subpleural. It arises from the inferior lobar pulmonary artery. Lower lobectomy was performed.

The histological analysis revealed a cystic adenomatoid malformation type 1 of Stocker’s classification measuring 22 x 15 cm, with cysts measuring from 1 to 11cm, and 2 lipomas 8 cm x 6 x 3 and 8 x 6 x 2 cm, and confirm the AVM.

The postoperative period was uneventful. The patient was discharged in the ninth postoperative day.

Discussion

Congenital cystic adenomatoid malformation is a rare lower respiratory tract malformation. There are hamartomatous lesions composed of both cystic and adenomatous overgrowths of terminal bronchioles.

Stocker’s classification proposed 5 types based on the size of the cyst and cellular characteristics [1].

The most commun mode of presentation for congenital cystic adenomatoid malformation in europ and United States has now an abnormal antenatal ultrasound scan (79%) [2]. Symptoms presented in neonatal period were grunting, retraction, and cyanosis. Some case presented with acute respiratory decompensation. During childhood and adult period, it is often pneumonia, chronic cough, dyspnea, which directs toward the diagnostic [3, 4]. The presentation of CCAM as a pneumothorax in 8% to 13 % [5]. Developmental of pleuropulmonary blastoma in pre-existing CCAM is well demonstrated by peter C. W. Kim [6].

Treatment was well codified in childhood with resection by lobectomy between 1 and 10 month [7]. Timing for surgery is based on compensatory lung growth, and the possibility to reduce the risk of infection. Lobectomy can be safely performed by thoracoscopy in infancy [8].

Pulmonary vascular malformations were a heterogeneous group. Pulmonary arteriovenous malformations were abnormal communication between pulmonary arteries and pulmonary veins. These were not commonly associated with pulmonary malformation. Association of pulmonary and vascular malformation were found in pulmonary sequestration; hybrid lesions between pulmonary sequestration and cystic adenomatoid malformation [9]. Pulmonary sequestration is associated with anomalous pulmonary venous drainage of the right lung in inferior vena cava in the scimitar syndrome. Our patient presented CCAM associated with pulmonary arteriovenous malformation in the same lobe. His AVM was not symptomatic and probably an incidental diagnosis on CT scan.

Congenital cystic adenomatoid malformation is not classically associated with pulmonary vascular disease. This arterovenous malformation is certainly congenital, because our patient had no other vascular disease, and not reason of secondary aetiology. Surgical treatment is recommended for this both lesions. Resection of these lesions had significantly improved pulmonary function of this young man. Spirometry at 6 months showed significant improvement with an FEV1= 66%, FVC=71%, FEV1/FVC= 76% as expected. As presentation with CCAM in adulthood is rare, no specific treatment guidelines exist, but surgical resection with lobectomy seems appropriate esspecialy if patient was symptomatic.

Classical treatment of PAVM was embolization. This was a definitive treatment and is well-established method [10]. Our case report of an adult with an unusually complex congenital pulmonary malformation associating CCAM and arterio venous malformation within a same lobe treated successfully with lobectomy.



References

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