CONGENITAL CYSTIC MALFORMATIONS OF THE LUNG IN CHILDREN

Nabil M Dessouky, Nabhan M T Kaddah, Mohamed G Shouman, Ahmed Hadidi

Pediatric Surgical and Critical Care Departments, The New Children¡¯s Hospital, Cairo University

 

Background / Purpose: Congenital cystic malformations of the lung (CCLM) are rare and vary considerably in their embryology, presentation, management and outcome. The aim of this study was to report the series of these anomalies detected at the New Children¡¯s Hospital of Cairo University, comparing the data with the current literature and clarify the controversies about their diagnosis and management.

Methods: Children who had congenital cystic lung malformations presenting with variable degrees of respiratory distress were diagnosed and managed in the Pediatric Critical Care and Surgical Units over a period of five years, between 1995 and 2000. Radiological studies included both chest x-ray and computed tomographic scan (CT scan). Ultrasonography was performed in some cases. Intensive respiratory management and control of infection- if present- were initiated and once stabilized, surgical management was performed. Extracorporeal membrane oxygenation (ECMO) support was not available in the hospital. Histopathological evaluation was performed for most of the lesions. Pulmonary function tests were only done for two patients to assess them postoperatively. Follow up period ranged between six months and four years.

Results: Thirty- two children consisting of 15 boys (47 %) and 17 girls (53 %) having congenital cystic lung malformation were diagnosed and managed. Their ages ranged from two days to nine years. Four cases (12 %) presented in the neonatal period, twenty-one cases (66 %) during infancy and seven cases (22 %) in childhood. There were 16 patients (50 %) of congenital lobar overinflation (CLO), 13 patients (41 %) of bronchogenic cysts (BC), two cases (6 %) of congenital cystic adenomatoid malformation (CAM) and only one case (3 %) of pulmonary sequestration (PS). Respiratory distress of varying degrees was the presenting symptom in 29 cases (91 %), while recurrent chest infections occurred in three cases of B C (9 %). The right lung was affected in 17 cases (52 %), while the left lung was affected in 16 cases (48 %); these included only one patient with CLO, the lung was affected bilaterally .Generally, the upper lobes were the mostly affected lobes. Lobectomy was performed in 26cases while isolated resection of the cyst was done in one patient where the cyst was attached to the whole lung. Conservative management was done successfully in 2 patients. The postoperative pulmonary function tests revealed 20 % reduction of the functional residual capacity with low compliance. Recurrence of the cyst occurred in one patient. The mortality rate was 25 % (n = 8), three of these died preoperatively.

Conclusions: This review showed that CAM and PS were not common lesions as compared to other series. Respiratory distress of varying degrees is the most common presenting symptom.  C T scan is a diagnostic postnatal imaging modality specially if x-ray is not confirmative, ultrasonography can be used antenatally. Mechanical ventilation was a risk factor and this should be considered in planning for management. Lobectomy is the surgical procedure of choice for most of the lesions. Further studies are needed to assess the late effects of surgical management of these lesions in children.

 

 

 
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