TY  - CHAP
M1  - Book, Section
TI  - Congenital Chest Wall Anomalies
A1  - Sultan, Ibrahim
A1  - Yang, Stephen C.
A2  - Yuh, David D.
A2  - Vricella, Luca A.
A2  - Yang, Stephen C.
A2  - Doty, John R.
PY  - 2014
T2  - Johns Hopkins Textbook of Cardiothoracic Surgery
AB  - EpidemiologyPectus  excavatum  occurs  in  about  1:400  live  births,  with  a  male  predominance  of  up  to  5:1.  No  genetic  abnormalities  or  chromosomal  aberrations  have  been  associated  with  pectus  excavatum,  although  a  familial  tendency  has  been  described.  Up  to  two-thirds  of  children  with  Marfan’s  syndrome  and  a  large  proportion  of  Ehlers–Danlos  patients  develop  pectus  excavatum  deformities.  The  incidence  of  pectus  carinatum  is  about  one-tenth  that  of  pectus  excavatum,  with  a  similar  male  predominance  and  25  to  30  percent  reported  family  history.  Scoliosis  and  other  spinal  deformities  or  a  family  history  of  these  are  associated  with  pectus  carinatum.PathophysiologyPectus  excavatum  is  a  concave  depression  of  the  sternum  resulting  from  abnormal  growth,  lengthening,  rotation,  or  increased  elasticity  of  the  costal  cartilages.  Reduced  cardiovascular  performance  has  been  linked  to  this  defect.  Pectus  carinatum  results  from  anterior  displacement  of  the  costal  cartilages  due  to  rotation  and  lengthening,  leading  to  sternal  protrusion.  No  significant  cardiopulmonary  abnormalities  have  been  demonstrated  with  this  lesion.Clinical  featuresIn  pectus  excavatum,  the  sternum  is  posteriorly  retracted  and  differential  growth  of  the  costal  cartilages  often  results  in  an  asymmetric  chest  wall  with  a  common  right-sided  rotation  of  the  sternum  and  an  elevated  left  side.  Other  characteristic  findings  include  sloped  ribs,  rounded  shoulders  with  a  classic  stooped  posture,  and  a  protuberant  abdomen.  It  is  most  commonly  discovered  in  the  first  few  years  of  life.  Decreased  stamina  and  endurance  during  exercise  and  excessive  tachycardia/palpitations  are  commonly  observed  with  this  disorder.  Pain  is  occasionally  associated  with  this  defect.  Pectus  carinatum  is  associated  with  pain,  respiratory  symptoms,  ease  of  injury,  and  psychosocial  body  image  concerns.DiagnosticsThe  best  radiographic  imaging  modality  for  pectus  deformities  is  the  computed  tomographic  scan  of  the  chest,  which  provides  precise  information  regarding  cardiac  displacement,  lung  volumes,  the  degree  of  deformity,  and  any  associated  malformations.TreatmentSurgical  correction  of  pectus  excavatum  includes  the  modified  Ravitch  repair  (subperichondrial  resection  of  all  abnormal  costal  cartilages  and  wedge  osteotomy  to  correct  the  posterior  depression  of  the  sternum  by  bringing  it  to  a  more  anterior  and  neutral  position)  and  the  Nuss  repair  (using  a  precurved  substernal  bar  to  bend  the  sternum  and  cartilages  to  a  corrected  position).  Surgical  correction  for  pectus  carinatum  utilizes  the  basic  principles  employed  in  the  open  Ravitch  repair,  namely  excision  of  all  abnormal  cartilages  and  return  of  the  sternum  to  a  neutral  straight  position.Outcomes/prognosisThe  modified  Ravitch  or  Nuss  repairs  represent  the  “gold  standard”  corrective  operation  for  pectus  excavatum.  Hospitalization  rarely  exceeds  3  days,  and  97  percent  of  patients  experience  a  good  result  from  the  operation.  Similar  success  rates  are  reported  for  Ravitch-type  repairs  of  pectus  carinatum.  Complications  include  recurrence,  pneumothorax,  wound  infection,  atelectasis,  and  local  tissue  necrosis.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - accesssurgery.mhmedical.com/content.aspx?aid=1104587320
ER  -