Management of congenital cystic adenomatoid malformation and bronchopulmonary sequestration in newborns.
BACKGROUND
Congenital cystic adenomatoid malformation (CCAM) and bronchopulmonary sequestration (BPS) are major embryonic pulmonary developmental anomalies. Early surgical excision is becoming an increasingly common option. We investigated the clinical features and management of patients with CCAM and BPS at the National Taiwan University Hospital.


METHODS
We conducted a retrospective review of neonates diagnosed with CCAM and/or BPS at the Hospital from July 1995 to January 2008. Prenatal examination, postnatal presentation, management and patient outcome were analyzed. We also propose a concise algorithm for the practical management of these conditions.


RESULTS
Sixteen patients were recruited including eight (50%) with CCAM, five (31%) with BPS and three (19%) with mixed-type lesions (CCAM with BPS). Thirteen (81%) patients were diagnosed antenatally at a median gestational age of 20 weeks. Eleven (69%) patients underwent surgical resection before 6 months of age because of respiratory distress or repeated pulmonary infection. There were no surgery-related complications among the seven patients who underwent early surgery within 1 month of age. Five (31%) patients remained asymptomatic and did not undergo surgery. All patients survived with no limitations to daily activity during follow-up periods of 1-8 years.


CONCLUSIONS
The high proportion of mixed-type lesions suggests that CCAM and BPS may share the same developmental ancestry. Early surgical resection within 1 month of age is safe in symptomatic patients.
Chen
Hung-Wen
HW
Department of Pediatrics, Tzu Chi General Hospital, Taipei Branch, Taipei, Taiwan; Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
Hsu
Wen-Ming
WM
Lu
Frank Leigh
FL
Chen
Pau-Chung
PC
Jeng
Suh-Fang
SF
Peng
Steven Shinn-Forng
SS
Chen
Chien-Yi
CY
Chou
Hung-Chieh
HC
Tsao
Po-Nien
PN
Hsieh
Wu-Shiun
WS
D000203:Activities of Daily Living; D000465:Algorithms; D001998:Bronchopulmonary Sequestration / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D013624:Taiwan; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal