Results of Surgical Treatment of Congenital Cystic Lung Disease

Abstract

Objective: To review the experience with surgical intervention for congenital cystic lung disease (CCLD). Methods: From April 1988 to December 2001, 68 patients were diagnosed and treated surgically for CCLD. There were 27 males and 41 females ranging in age from 2 to 65 years. The majority of the cases were diagnosed by history, clinical manifestations and chest radiography or computerized tomography (CT). There were 25 (36.8 %) right-sided and 43 (63.2 %) left-sided cystic lung lesions. Four patients had massive bleeding and needed urgent or emergency operation. Pneumonectomy, lobectomy, and/or segmentectomy were performed. Results: 29 (42.6 %) patients underwent lobectomy, 25 (36.8 %) had total pneumonectomy, 9 (13.2 %) lobectomy plus segmentectomy, and 5 (7.4 %) bilobectomy. During the operation, left-sided pulmonary sequestration was confirmed in 3 patients. No death occurred in this series. Diagnoses were confirmed by pathology in all cases. Two patients had malignant lesions arising from multicystic lung lesions. Conclusions: A history of recurrent pneumonia, often with hemoptysis and subsequent dyspnea, should raise the suspicion of CCLD. The diagnosis can be confirmed by chest X-ray and CT preoperatively, and early surgical intervention may avoid the development of serious complications. Delayed diagnosis and surgical management usually result in significant morbidity and mortality.

Key words

Congenital cystic lung disease - surgical treatment - pulmonary sequestration - adenocarcinoma

References

• 1 Williams H J, Johnson K J. Imaging of congenital cystic lung lesions.  Paediatr Respir Rev. 2002;  3 120-127
• 2 dell'Agnola C A, Tadini B, Mosca F, Wesley J R. Prenatal ultrasonography and early surgery for congenital cystic disease of the lung.  J Pediatr Surg. 1992;  27 1414-1417
• 3 Albanese C T, Sydorak R M, Tsao K, Lee H. Thoracoscopic lobectomy for prenatally diagnosed lung lesions.  J Pediatr Surg. 2003;  38 553-555
• 4 Wesley J R, Heidelberger K P, DiPietro M A, Cho K J, Coran A G. Diagnosis and management of congenital cystic disease of the lung in children.  J Pediatr Surg. 1986;  21 202-207
• 5 Stocker J T, Drake R M, Madewell J E. Cystic and congenital lung disease in the newborn.  Perspec Pediatr Pathol. 1974;  4 93-154
• 6 Sheffield E A, Addis B J, Corrin B, McCabe M M. Epithelial hyperplasia and malignant change in congenital lung cysts.  J Clin Pathol. 1987;  40 612-614
• 7 Gie R P, Beyers N, Schaaf H S, Nel E D, Smuts N A, van Zyl S, Donald P R. TB or not TB? An evaluation of children with an incorrect initial diagnosis of pulmonary tuberculosis.  S Afr Med J. 1995;  85 658-662
• 8 Ko K S, Lee K S, Kim Y, Kim S J, Kwon O J, Kim J S. Reversible cystic disease associated with pulmonary tuberculosis: radiologic findings.  Radiology. 1997;  204 165-169
• 9 de Perrot M, Pache J C, Spiliopoulos A. Carcinoma arising in congenital lung cysts.  Thorac Cardiov Surg. 2001;  49 184-185
• 10 Granata C, Gambini C, Balducci T, Toma P, Michelazzi A, Conte M, Jasonni V. Bronchoalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation.  Peditar Pulmonol. 1998;  25 62-66
• 11 Chiaki E, Tadashi I, Hideyuki N, Akio E, Mitsuomi K. Bronchioloalveolar carcinoma arising in a bronchogenic cysts.  Ann Thorac Surg. 2000;  69 933-935
• 12 Ozcan C, Celik A, Ural Z, Veral A, Kandiloglu G, Balik E. Primary pulmonary rhabdomyosarcoma arising within cystic adenomatoid malformation: a case report and review of the literature.  J Pediatr Surg. 2001;  36 1062-1065
• 13 Murphy J J, Blair G K, Fraser G C, Ashmore P G, LeBlanc J G, Sett S S, Rogers P, Magee J F, Taylor G P, Dimmick J. Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases.  J Pediatr Surg. 1992;  10 1364-1367
• 14 Ferderici S, Domenichelli V, Tani G, Sciutti R, Burnelli R, Zanetti G, Domini R. Pleuropulmonary blastoma in congenital cystic adenomatoid malformation: report of a case.  Eur J Pediatr Surg. 2001;  11 196-199
• 15 Papagiannopoulos K, Hughes S, Nicholson A G, Goldstraw P. Cystic lung lesions in the pediatric and adult population: surgical experience at the Brompton Hospital.  Ann Thorac Surg. 2002;  73 1594-1598
• 16 Usui Y, Takabe K, Takayama S, Miura S, Kimula Y. Minute squamous cell carcinoma arising in the wall of a congenital lung cyst.  Chest. 1991;  99 235-236
• 17 Al-Bassam A, Al-Rabeeah A, Al-Nassar S, Al-Mobaireek K, Al-Rawaf A, Banjer H, Al-Mogari I. Congenital cystic disease of the lung in infants and children (experience with 57 cases).  Eur J Pediatr Surg. 1999;  9 364-368