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.