RT Book, Section
A1 Fischer, Anne C.
A1 Griffiths, Eric
A2 Yuh, David D.
A2 Vricella, Luca A.
A2 Yang, Stephen C.
A2 Doty, John R.
SR Print(0)
ID 1104584258
T1 Congenital Pulmonary Disorders
T2 Johns Hopkins Textbook of Cardiothoracic Surgery
YR 2014
FD 2014
PB McGraw-Hill Education
PP New York, NY
SN 978-0-07-166350-2
LK accesssurgery.mhmedical.com/content.aspx?aid=1104584258
RD 2024/04/19
AB EpidemiologyCongenital  pulmonary  anomalies  include  a  multitude  of  clinical  entities  linked  by  a  common  developmental  lineage.  The  prevalence  is  dependent  upon  the  particular  malformation.  These  anomalies  represent  different  variations  of  shared  embryologic  pathways  and  therefore  possess  clinical  similarities  and  occasionally  present  simultaneously.PathophysiologyPhysiologically,  the  impact  of  these  lesions,  which  can  be  mild  or  detrimental,  depends  largely  on  two  factors—pulmonary  hypoplasia  and  fetal  hydrops.  The  mass  effect  of  fetal  intrathoracic  lesions  can  impair  lung  development  and  decrease  cardiac  output  secondary  to  mediastinal  shift,  causing  fetal  demise  or  severe  compromise.  Lesions  that  replace  functional  lung  parenchyma  can  also  cause  pulmonary  insufficiency.Clinical  featuresMany  congenital  pulmonary  anomalies  present  postnatally  with  frequent  pulmonary  infections,  respiratory  distress,  and  hemoptysis,  although  others  are  discovered  incidentally  on  plain  chest  radiography.  Diagnoses  of  congenital  pulmonary  malformations  are  now  typically  detected  with  prenatal  surveillance  imaging.DiagnosticsPrenatal  ultrasound  is  the  primary  diagnostic  modality  whereby  congenital  pulmonary  anomalies  are  identified  in  utero  and  the  degree  of  hydrops  is  assessed.  Postnatally,  computed  tomography  or  magnetic  resonance  imaging  can  be  used  to  differentiate  lesions  based  on  anatomic  characteristics;  but  since  surgical  resection  is  the  treatment  for  most  mass  lesions,  additional  imaging  is  of  value  to  ensure  the  prenatal  lesion  has  not  regressed.TreatmentResection  often  is  curative  for  focal  lesions,  but  outcome  is  ultimately  dependent  on  residual  pulmonary  function  and  associated  medical  conditions.  Fetal  intervention  is  being  offered  as  an  alternative  for  some  congenital  pulmonary  anomalies,  although  outcomes  have  been  mixed.OutcomesOutcomes  depend  on  the  particular  disease  process.  Generally,  excellent  survival  rates  are  noted  with  timely  treatment  of  bronchogenic  cysts,  pulmonary  sequestration,  and  congenital  lobar  emphysema.  A  large  percentage  of  congenital  pulmonary  adenomatoid  malformations  postnatally  do  regress.  The  prognoses  of  other  entities  are  adversely  affected  by  the  presence  of  fetal  hydrops  prenatally  or  the  association  of  recurrent  infections  or  malignant  risk  in  some  of  the  anomalies.