Dec.  11,  2012  

Pediatric  GIT,  Abdominal  Wall  and  
Neoplasms    

 

                       Dr.  Abellera  
OUTLINE  

I.  Abdominal  Wall  Defects  
1.
Gastroschisis  
2.
Omphalocoele  
3.
Bowel  Ischemia  
4.
Colonic  Atresia  
5.
Hernia  of  Umbilical  Cord  
6.
Prune  Belly  
7.
Bladder  Exstrophy  
8.
Cloacal  Exstrophy  
9.
Pentalogy  of  Cantrell  
10. Lower  Midline  Syndrome  
11. Beckwith-­‐Widemann  Syndrome  
II.  Neonatal  Intestinal  Obstruction  
1.
Diagnosis  
2.
Five  Clinical  Findings  that  Sugggest  Intestinal  Obstruction  in  the  Neonate  
3.
Other  Findings  
4.
Malrotation  
5.
Hypertrophic  Pyloric  Stenosis  
6.
Intestinal  Atresia  
7.
Duodenal  Atresia  
8.
Colonic  Atresia  
9.
NEC  
10. Meconium  Peritonitis  
11. Meconium  Pseudocyst  
12. Hirschprung’s  Disease  
13. Imperforate  Anus  
III.  Intestinal  Obstruction  in  Older  Children  
1.
Intussusception  
2.
Meckel’s  Diverticulum  
3.
Acute  Appendicitis  
4.
Post-­‐operative  Adhesions  
5.
Inguinal  Hernia  
III.  Pediatric  Neoplasms  
1.
Wilm’s  tumor  
2.
Neuroblastoma  
3.
Hepatoblastoma  
4.
Teratoma  
5.
Hemangioma  
OBJECTIVES  
Pediatric  GIT  and  Abdominal  Wall  
1.
To  diagnose  and  differentiate  congenital  abdominal  wall  defects    
2.
To  know  the  more  common  neonatal  and  general  pediatric  surgical  cases  
3.
To  be  able  to  know  the  basic  principles  of  managing  such  diseases  
Pediatric  Neoplasms  
4.
To  be  able  to  know  the  common  pediatric  neoplasms  that  can  be  managed  surgically  
5.
To  be  able  to  know  the  basic  principles  of  managing  these  pediatric  tumors  
 
NOTE:  Sir  said  that  most  of  his  questions  for  pediatric  GIT  and  abdominal  wall  will  be  
coming  from  his  lecture,  but  those  for  pediatric  neoplasms  will  be  mostly  coming  from  
Schwartz;  hence,  the  abundance  of  book  info  in  the  pediatric  neoplasms  part.  All  book  
info  are  from  Schwartz.  

 

GASTROSCHISIS  

 

ABDOMINAL  WALL  DEFECTS  


   


 
Embryology:  
o Growth  of  the  dorsal  axis  and  longitudinal  infolding  
o From  4  folds  of  the  splanchic  wall  

2  lateral    lateral  walls  

1    cephalic    thoracic  /  epigastric  wall  

1    caudal    hindgut,  bladder,  hypogastric  
o Converge  to  form  the  umbilical  ring  

Daph,  Jaff,  Gill,  Gubs  

 

 

 

Failure  of  closure  results  in  defects  
Imagine  a  piece  of  paper.  If  you  fold  it,  the  top  part  touches  
the  bottom  part,  then  you  bring  together  the  sides.  This  is  
what  happens.  Lateral  folds  become  the  lateral  walls,  the  
cephalic  fold  becomes  the  upper  part  of  the  abdominal  wall,  
the  caudal  wall  becomes  the  lower  part.  They  converge  in  
the  middle  to  form  the  umbilical  vein.  Failure  of  closure  
results  in  defects.  
Mid-­‐abdominal  wall  defects:  
o Incidence  is  approximately  1  in  2000  births  
o Highly  associated  w  ith  prematurity  
o Detection  in  utero  is  important,  with  a  search  for  other  
nd
congenital  anomalies  especially  during  2  trimester  

Brings  to  mind  the  importance  of  prenatal  ultrasound  
Management  is  directed  at  (regardless  of  the  abdominal  wall  
defect:  
o Correcting  hypovolemia  
o Preventing  hypothermia  

Reason  for  the  above  two:  Because  patients  have  
abdominal  wall  defects;  hence,  increased  insensible  
water  loss,  increased  hypothermia  
o Monitoring  for  sepsis/other  complications  

Because  of  the  exposed  bowels,  patients  have  a  
tendency  to  develop  these  
o Nutrional  support  

Patients  cannot  feed  immediately,  so  nutritional  
support  must  be  provided  
o Looking  for  other  associated  anomalies  
o Management  is  always:  Surgical  closure  
Mid-­‐abdominal  wall  defects:  
o Surgery  is  performed  as  soon  as  possible  (primary  vs.  staged  
closure)  to  protect  and  cover  the  bowels/exposed  organs  

Choice  between  primary  and  staged  depends  on  how  
much  of  the  bowels  is  exposed,  or  how  difficult  it  is  to  
bring  everything  back  into  the  abdomen  
o Staged  abdominal  wall  closure:  usually  in  7-­‐10  days  
o Associated  with  growth  delay  in  the  long-­‐term  
o Usual  complications  are  sepsis,  NEC,  prolonged  ileus,  
respiratory  distress  
o
o

Greek  for  “  belly  cleft  “  
Generally  thought  of  as  a  result  of  intrauterine  occlusion  of  the  
right  omphalomesentric  artery  resulting  in  disruption  of  the  
umbilical  ring  and  bowel  herniation  
o Right  omphalomesenteric  artery  does  not  develop,  so  defect  
is  to  the  RIGHT  of  the  umbilicus  
Often  associated  with  intestinal  atresia  
Mortality  is  around  10%  
o In  the  Phils.,  mortality  usually  approaches  50%  because  
patients  arrive  late  in  the  hospital  
Gastroschisis  is  a  congenital  anomaly  characterized  by  a  defect  in  the  
anterior  abdominal  wall  through  which  the  intestinal  contents  freely  
protrude.  Unlike  with  omphalocele,  there  is  no  overlying  sac  and  the  size  
of  the  defect  is  much  smaller  (<4  cm).  The  abdominal  wall  defect  is  
located  at  the  junction  of  the  umbilicus  and  normal  skin  and  is  almost  
always  to  the  right  of  the  umbilicus.  The  umbilicus  becomes  partly  
detached,  which  allows  free  communication  with  the  abdominal  cavity.  

 

       1  of  14  

 This  is  a  case  of  a  newborn  brought  to  the  OR  within  an   hour  after  birth.       Gastroschisis.  mobilize  the  fascia.  look  leathery.  Laying  down  of  sutures     6.  sac  rupture.  associated   anomalies   Non-­‐operative  closure  is  reserved  for  extremely  large  defects  or   high  risk  infants  by  promoting  epithelialization   Mortality  ranges  from  30-­‐60%   o Higher  mortality  mainly  because  it  is  associated  with  other   genetic  abnormalities     There  is  a  covering.     3.  Gubs                    2  of  14     .  It’s  better  to  operate  on  them  earlier  because  there   will  be  less  edema  and  less  infection.  The  defect  is  central.  Intra-­‐op       This  is  a  patient  with  gastroschisis.  The  silo  covers  the  bowel  and  allows  for  graduated   reduction  on  a  daily  basis  as  the  edema  in  the  bowel  wall  decreases     Surgical  Technique   1.  Jaff.       5.  and  the  umbilicus  is  attached  to  this  thin  sac  that   contains  the  bowels.     Gastroschisis  vs.  Temporary  closure/  testing  of  intra-­‐abdominal  pressure     When  everything  is  inside.   4.  and   with  cardiac  anomalies   Survival  depends  on  size.  R   Umbilical.  Omphalocoele  (Very  important!)       Gastroschisis   Omphalocoele   Area   Size  of  defect   Sac   Cord   Bowels   Liver   Atresia   Strangulation   Anomalies   Surgery   Lateral.  prematurity.  The  bowels  are  outside  the   abdomen.  central   Small   Variable  or  larger   Absent   Present   Left  of  defect   On  top  of  defect   Inflamed  (because   they  are  exposed)   Normal-­‐looking   (because  they  are   covered)   May  be  herniated   (because  the  defect   may  be  variable  and   quite  large)   Rare   Not  exposed   Common   Increased  risk   (because  of  a  small   abdominal  defect)   Uncommon   Uncommon   Urgent  (because  the   bowels  are  already   exposed)   Non-­‐urgent  (because   you  still  have  to  look   for  the  other   anomalies)     Stretch  the  skin  to  be  able  to  put  everything  inside.  and  they  exit  at  the  right  side  of  the  umbilical  stump.  Test  if   the  patient  can  tolerate  having  everything  put  inside.  Checking  for  bands/Stretching  the  skin   OMPHALOCOELE   • • • • • Failure  of  migration  and  fusion  of  embryonic  folds  leading  to  a   defect  in  the  middle   Associated  with  several  genetic  syndromes.  The   bowels  are  shortened.   2.  Mobilizing  the  fascia     Make  a  small  incision  upwards.  Gill.  Fascial  closure     Common     Daph.  trisomies.  esp.  the  abdomen  can  be  quite  distended.  then  try  to  put   everything  inside.  and  are  matted  together.     • A  plastic  spring-­‐loaded  silo  can  be  placed  onto  the  bowel  and  secured   beneath  the  fascia.

 you  can   already  close  the  fascia  and  repair  the  ventral  hernia  since  patient  has   already  developed  adequate  abdominal  space  and  fascia)       This  figure  shows  colonic  atresia  in  a  patient  with  gastroschisis.  edema  has  decreased.       COLONIC  ATRESIA       Clockwise  L-­‐R:  1)  Fascial  closure.  since  defect  is  on  top  of   the  umbilicus.  You  insert  it  underneath  the   fascia.     • Closure  using  other  methods     Because  the  defect  is  quite  small.  the  bowels  are   just  underneath  the  skin.  baby  and  abdominal   cavity  have  become  somewhat  bigger.  and  consequently  become   gangrenous.  umbilical  stump  is  there.   You  choose  among  the  available  sizes  depending  on  the  size  of  the   defect  or  the  exposed  bowels.  Allow  the  edema  to  subside.  there  is  a  tendency  for  the  exposed   bowels  to  be  vascularly  compromised.  4)  skin  closure.  and  the  patient  to  be   stabilized.  Gill.       7.  (R)  Preformed  spring-­‐loaded  silo.  and  the   patient  tends  to  have  short  bowel  syndrome  later  on.  After  7-­‐10  days.  D2  (day  2)  post-­‐op   •   Wound  is  healing  nicely.  2)  skin  closure.  you  put  a   blood  bag.     • Silastic  Sheet       8.  hmm  alam  na…)     BOWEL  ISCHEMIA         Spring-­‐loaded  silo     (L)  Looks  like  a  blood  bag  with  a  spring.  The  gangrenous  part  will  have  to  be  removed.  Closure  of  skin   When  the  bowels  are  too  inflamed  and  the  baby  is  too  little.  (So  if  your  gf  or  bf  has   no  navel.  patients  end  up  having  no  navels.  Jaff.  and  you  put  the  bowels  inside.   Patient  still  has  an  umbilicus.  In  omphaloceles.  abdomen  looks  okay.  1  yr  (before  or  around  this  time.  Gubs                  3  of  14     .     Treatment   • Staged  closure  using  blood  bag   HERNIA  OF  THE  UMBILICAL  CORD   • • Usually  <  4  cm  in  diameter  and  the  sac  should  contain  only  a  few   loops  of  intestines   May  be  missed  at  birth  and  injury  to  the  intestine  can  be  caused   by  careless  clamping  of  the  umibilical  cord     Daph.  colostomy  (if  you  are   not  able  to  close  the  fascia  and  you  do  not  want  to  use  a  blood  bag).  so  there  is  a  good  chance  that   you  will  be  able  to  close  it  already.  but  this  is  better  since  the  bowels  are  not   exposed).  3)   skin  closure.  1-­‐2  months  (since  there  is  a  fascial  defect.

    •   Pentalogy  of  Cantrell.  with   the  pubic  bone  splayed                     There  are  2  hemibladders.  urinary  tract  malformations.  anterior  diaphragmatic  hernia.       • • • • •     Gigantism.  imperforate  anus.  an  omphalocele.  HPP)   o External  compression  (ie.  Malrotation)   o Motility  dysfunction  (ie.     There  is  bladder  exstrophy.  (L)  Note  that  omphalocele  is  quite  low.   dilated  urinary  tract  including  the  bladder.   • Upper  midline  omphalocoele.  cardiac  anomalies     Intestinal  obstruction  is  one  of  the  most  common  surgical   emergencies  of  the  newborn   One  (1)  in  500-­‐1500  births   Approximately  half  are  due  to  atresias  or  stenosis   May  be  due  to:   o Luminal  obstruction  (ie.  but  this  patient  wasn’t  operated  on.   (R)  if  you  flip  it  up.   PENTALOGY  OF  CANTRELL     Looks  like  an  omphalocele.  Eagle-­‐Barrett  syndrome   • Prune-­‐belly  syndrome  is  a  disorder  that  is  characterized  by  a  constellation   of  symptoms  including  extremely  lax  lower  abdominal  musculature.  volvulus.  internal  hernia)   o Duodental  atresia  .  This  shows  an  omphalocele  on  the  upper  portion   that  has  already  epithelialized.  Gill.  ectopia  cordis.  an  imperforate  anus.  the  defect  is  smaller.  and  bilateral  undescended   testes.  on  top  of  the   umbilicus.     Note:  Next  3  subtopics  only  show  the  syndromes  in  which  omphalocele   is  a  part  of.  and   no  identifiable  external  genitalia.     LOWER  MIDLINE  SYNDROME   •     BLADDER  EXSTROPHY   Is  a  defect  of  the  anterior  bladder  wall  and  abdominal  wall.  pancreatic  islet   hyperplasia   NEONATAL  INTESTINAL  OBSTRUCTION   • Rare   Increased  survival  in  recent  years   Quite  difficult  to  reconstruct   Similar  to  bladder  exstrophy  in  embryogenesis   Involves  an  anterior  abdominal  wall  defect  in  which  the  two   hemibladders  are  visible  and  separated  by  a  midline  intestinal   plate.  There  is  a  beating  defect  because  of  the   ectopia  cordis.  or  annular  pancreas   o Jejunal  atresia  or  stenosis   o Ileal  atresia  or  stenosis   o Hirschsprung’s  disease   o Simple  meconium  ileus   o Meconium  ileus  with  perforation   o Necrotizing  enterocolitis   o Hypertrophic  pyloric  stenosis   o Imperforate  anus   DIAGNOSIS  (important)   o Clinical  –  history  and  physical  examination   o Radiographic  –  confirmatory    Dilated  bowel  loops      Air-­‐fluid  levels    Air  pattern  may  stop  abruptly    Paucity  of  distal  gas            4  of  14     .  Gubs     BECKWITH-­‐WIDEMANN  SYNDROME   •   CLOACAL  EXSTROPHY   • • • • • Bladder  or  cloacal  exstrophy.  macroglossia.  you  see  the  cloacal  exstrophy     Looks  like  skin  closure.  stenosis.  Note  that  there  is  a  perforate  anus.   vesicointestinal  fistula.  sacral  vertebral  defects.   sternal  cleft.  colonic  atresia.  atresias.  Hirschsprung’s  disease)   Common  causes:   o Malrotation  (duodenal  obstruction.  Jaff.  an  omphalocoele  and  an  imperforate  anus   More  severe  than  bladder  exstrophy     Daph.   cryptorchidism   • Also  known  as  triad  syndrome  (because  of  the  underlined   components  above).  Same  central  location.     PRUNE  BELLY     • Absent/deficient  abdominal  wall  musculature  (such  that  anterior   abdominal  wall  looks  like  a  prune).  However.  omphalocoele.   meningomyelocele     Lower  Midline  Syndrome.

      o Contrast  x-­‐rays  –  specific  diagnosis/may  also  be  therapeutic   FIVE  CLINICAL  FINDINGS  THAT  SUGGEST  INTESTINAL   OBSTRUCTION  IN  THE  NEONATE   o o o o o Maternal  polyhydramnios   Excessive  gastric  aspirate   Abdominal  distension   Bilious  vomiting   Obstipation  or  non-­‐passage  of  meconium       This  figure  supposedly  shows  the  color  of  bilious  vomitus—dark  green.   dark  green.  small  bowel  stenosis  and  atresia.  meconium  ileus.  stenosis.  single  bubble  only  since  gas   cannot  pass  distally   Usually  presents  with  hypokalemic  hypochloremic  metabolic   alkalosis            5  of  14     .   necrotizing  enterocolitis.  distension  occurs  earlier.  gastrointestinal  obstruction  should  be  considered   • Feeding  intolerance  or  non-­‐bilious  vomiting  can  be  the  presenting   symptom     Abdominal  Distension   • Abdominal  distension  may  not  be  apparent  at  birth  but  develops   over  time  as  ingested  air  accumulates  proximal  to  an  obstruction   o If  obstruction  is  proximal.  Gubs         Presence  of  intestinal  patterning  and  peristalsis  that  is  visible   through  the  abdominal  wall   Distended  loops  of  bowel  may  be  palpable   The  rectum  may  feel  tight  on  examination  if  small  and  unused   Abdominal  wall  discoloration  and  ecchymosis  suggest  perforation   and/or  necrosis   Sudden  abdominal  enlargement  may  be  due  to  perforation   o In  neonates.   distention  can  appear  late   • The  time  of  onset.  but  no  problems  in  motility     OTHER  FINDINGS   • • • • •   Areas  of  potential  obstruction  in  the  neonate  (esophageal  atresia.  and  Ladd’s  bands.  abdomen  suddenly  distends  when   there’s  perforation     • • • • • •   INITIAL  TREATMENT  (important)   NPO  and  Placement  of  OGT   o Patient  cannot  eat  and  digest  the  food.  pyloric  stenosi.  Jaff.   arranged  from  proximal  to  distal.  shiny  meconium  usually  within  the  first  12  hours  of   life  and  almost  always  by  24  hours   • Failed  or  delayed  passage  of  meconium  suggests  obstruction   • Passage  of  meconium  does  not  exclude  the  possibility  of   obstruction   o There  might  be  obstruction.   malrotation.  left  colon  syndrome.  Don’t  aggravate  the   situation  further  by  feeding.   Hirschsprung’s  disease.  Place  an  OGT  to  decompress   Fluid  and  resuscitation.  a  palpable  “olive”  at  RUQ   Can  be  diagnosed  by  ultrasound   Presents  with  a  “single  bubble”  on  xray   o Pertains  to  distended  stomach.  duodenal  atresia.  anorectal  atresia     Polyhydramnios   • Proximal  gastrointestinal  obstruction  leads  to  accumulation  of   excess  amniotic  fluid   • Distal  small  bowel  or  colonic  obstructions  do  not  usually  result  in   polyhydramnios   • Prenatal  ultrasound  findings  can  also  be  helpful  or  may  show   signs  of  obstruction     Excessive  Gastric  Output   • Passage  of  an  orogastric  tube  is  often  performed   • If  the  initial  volume  of  gastric  aspirant  is  large  (>50ml)  or  is   bilious.  because  of  the  small  abdominal  cavity  and  the   thin  abdominal  wall.   HYPERTROPHIC  PYLORIC  STENOSIS   • • • • • Presents  with  non-­‐bilious  vomiting   o Since  obstruction  is  proximal   On  PE.     Failure  To  Pass  Meconium   • A  normal  newborn  is  expected  to  pass  a  large  amount  of  thick.  degree  and  characteristic  appearance  of  the   distension  may  suggest  the  level  of  obstruction     Bilious  Emesis   • Bilious  emesis  in  the  neonate  is  a  surgical  emergency  until   proven  otherwise   • Vomiting  begins  soon  after  delivery  if  the  lesion  is  proximal  or   complete  but  may  be  delayed  in  cases  of  distal  or  incomplete   obstruction   Daph.   gastro-­‐esophageal  reflux.  electrolyte  replacement   Empiric  antibiotics   Rule  out  associated  anomalies/problems   Surgery  for  underlying  cause  (referral  to  your  favourite  Pediatric   Surgeon)   Respiratory/cardiac  support  as  needed   OUTCOMES   • • • Vary  with  the  etiology  of  the  obstruction     Influenced  by  prematurity  and  the  associated  anomalies  of  each   condition     Delay  in  diagnosis  and  treatment  should  be  avoided   o Delayelectrolyte  imbalancesepsispoorer  prognosis   WOF  complications  associated  with  treatment   •   Note:  The  following  subtopics  under  neonatal  intestinal  obstruction   pertain  to  its  common  causes  that  were  previously  enumerated.  Gill.  if  distal.

 After  which.  the   more  distended  the  abdomen  and  the  greater  the  number  of  obstructed   loops  on  upright  abdominal  films       MALROTATION   • INTESTINAL  ATRESIA       Different  types  of  intestinal  atresia.  After  resuscitation.  Could  be  a  discontinuity  (type  1.  or  else  the  patient  will  develop  appendicitis  in   the  future.  gap  (type  3A.  a  Fredet-­‐Ramstedt  pyloromyotomy  is   performed.  The  former  route  is  cosmetically  more  appealing.  which  leads  to  a  cycle   of  feeding  and  vomiting  that  invariably  results  in  severe  dehydration  if  the   condition  is  untreated.   Eventually.     • • •   Surgical  treatment  is  pyloromyotomy   Infants  with  HPS  present  with  nonbilious  vomiting  that  becomes   increasingly  projectile  over  the  course  of  several  days  to  weeks.  The  procedure  may  be  performed  using  an  open  or   laparoscopic  approach.  In  type  2  the   atretric  ends  are  connected  by  a  fibrous  band.   Pyloromyotomy   • • • • Usually  presents  with  bilious  vomiting/recurrent  vomiting.  in  which  the   bowel  distal  to  the  atresia  receives  its  blood  supply  in  a  retrograde   fashion  from  the  ileocolic  or  right  colic  artery.  right  middle).  They  can  twist  around  the  narrow   base  and  cause  volvulus.  degree  correlates  roughly  with   level.  failure   to  thrive   Index  of  suspicion  should  be  high   Midgut  volvulus  and  bowel  gangrene  may  develop   Diagnosis  is  best  made  by  an  upper  GI  series   Surgery  is  called  Ladd’s  procedure  (Adhesiolysis  of  Ladd’s  bands  +   appendectomy  +  bowel  arrangement)     (L)  Normal  fixation  of  the  bowel.  fibrotic  whorl  (type  2.  although  the   transverse  incision  provides  easier  access  to  the  antrum  and  pylorus.  lowermost).  the  child  normally  has  a  voracious  appetite.  and  the  proximal  duodenum)                              6  of  14     .  The  more  distal  the  obstruction.  (center  and  R)  malrotation  of  the   bowel  may  look  like  this  (In  malrotation.  because  you   changed  the  orientation  of  everything  in  the  abdomen.  Christmas  tree  deformity  (type  3B.  commonly  in  the  jejunum  and  usually  single  (~90%)   o Could  be  duodenal.  Type  3B  and  type  4  are  the  most  difficult   to  manage  because  they  sometimes  involve  dissecting  a  significant   portion  of  bowel.  string  of   sausages  (type  4.  Type  3B   is  an  "apple-­‐peel"  deformity  or  "Christmas  tree"  deformity.  the  colon  to  the  left.  then  short  bowel   syndrome.  you  need  to   remove  the  appendix.   Fluid  resuscitation  with  correction  of  electrolyte  abnormalities  and   metabolic  alkalosis  is  essential  before  induction  of  general  anesthesia  for   operation.  Despite  the  recurrent   emesis.   left  uppermost).  jejunal.   left  middle).  Jaff.  the  problem  is  the  presence  of   bands  that  can  cause  obstruction.  the  infant  develops  almost  complete  gastric  outlet  obstruction   and  is  no  longer  able  to  tolerate  even  clear  liquids.     .  there  are   multiple  atresias  with  a  "string  of  sausage"  or  "string  of  beads"   appearance       Duodenal  Atresia   • Incomplete  canalization  of  duodenal  lumen   • Usually  presents  with  bilious  vomiting  because  obstruction  is   usually  distal  to  the  ampulla  of  Vater   • Approximately  1/3  associated  with  Down’s  syndrome   • “Double  bubble”  because  obstruction  is  more  distal  (gas  in  the   stomach.  right  uppermost).  Gubs       • • • • • Usually  presents  with  vomiting  and  abdominal  distension   Varying  levels  of  obstruction.  leading  to  gangrene.  In  type  3A  the  two  ends  of   the  atresia  are  separated  by  a  V-­‐shaped  defect  in  the  mesentery.   • In  type  I  there  is  a  mucosal  atresia  with  intact  muscularis.       Ladd’s  procedure:   Lyse  the  bands    change  the  orientation  of  everything—the  small   bowels  to  the  right.  ileal.  In  type  4  atresia.   Daph.  or  colonic   Fetal  mesenteric  vascular  accidents  are  likely  to  be  the  cause  of   most  cases   Treatment  is  surgical:  resection  and  anastomosis   Infants  with  jejunal  or  ileal  atresia  present  with  bilious  vomiting  and   progressive  abdominal  distention.  Gill.  Open  pyloromyotomy  is  performed  through   either  an  umbilical  or  a  right  upper  quadrant  transverse  abdominal   incision.

 but  this  is  for   the  more  common  short-­‐segment  Hirschsprung’s  disease.  Gubs         • • • • • • • •   • Also  called  congenital  aganglionosis   o Absence  of  ganglion  cells  distally    distal  bowel  becomes   spastic    no  peristalsis    proximal  segment  becomes   loaded  with  feces   Failure  to  pass  meconium  in  the  first  24  to  48  hours   Abdominal  distension  and  vomiting  in  the  neonate   Chronic  constipation  in  older  children   Malnutrition.  the  development  of  diffuse  pneumatosis  intestinalis  which  is   associated  with  the  development  of  stage  II  NEC  is  thought  to  be  due  to   the  presence  of  gas  within  the  wall  of  the  intestine  from  enteric  bacteria.  Once  you  feed  these  patients.   where  the  aganglionic  segment  is  just  at  the  rectosigmoid   area.  This  may  progress  to  perforation.   In  all  infants  suspected  of  having  NEC.  It  has  also  been  demonstrated  that  the   premature  intestine  responds  in  an  exaggerated  fashion  to  bacterial   products.  Finally.  tympany   Bilious/fecaloid  vomiting  –  late  sign   Spasm  of  the  rectum  on  digital  examination   Characteristic  forceful  evacuation  of  gray  liquid  stool  at  the  time   of  rectal  examination   o There  is  gush  of  air  when  you  do  rectal  exam.  then  bowel  gangrene.  residuals)  after   initiation  of  feeding   Abdominal  distension  and  hematochezia  eventually  follow   Bowel  ischemia  and  gangrene  are  late  signs   May  lead  to  bowel  perforation  (most  severe  complication)  and   death   Despite  severity  of  illness.   Daph.       Colonic  Atresia   MECONIUM  PERITONITIS   • • • • •     NECROTIZING  ENTEROCOLITIS  (NEC)   • • • • • • • • Disease  of  prematurity  and  LBW  infants   First  sign  is  formula  intolerance  (vomiting.   Plain  radiograph:  air  filled.  Intubation  and  mechanical  ventilation  may   be  required  to  maintain  oxygenation.   which  then  leads  to  peritonitis     Calcifications  can  sometimes  be  seen  on  xrays   Exploratory  laparotomy  may  be  warranted   Usually  associated  with  cystic  fibrosis  that’s  why  it’s  not  that   common  in  the  Philippines   More  commonly  described  as  meconium  ileus   MECONIUM  PSEUDOCYST   • • •       Perforation  occurs  prenatally    peritonitis  meconium   pseudocyst   Calcification  maybe  seen  on  xray   Exploratory  laparotomy  should  be  done   HIRSCHSPRUNG’S  DISEASE   •   Because  of  prematurity.  leading  to  an  inflammatory   cascade.  Complete  aganglionosis  is  rare.  failure  to  thrive   Abdominal  distension.   Furthermore.  the  development  of  pneumoperitoneum  indicates  disease   progression  with  severe  disruption  of  the  intestinal  barrier  (intestinal   perforation).  Gill.  there  will  be  mucosal  injury   coupled  with  bacterial  overgrowth.   leading  to  pneumoperitoneum  (air  in  the  abdominal  cavity).  There  is  bowel  stasis  and  decreased   defenses.  Jaff.     Pneumoperitoneum   • • • In  general.  feedings  are  discontinued.  and  inotropes  are  administered  to   maintain  perfusion  as  needed.  survival  rates  approach  80%  in  most   series   Most  cases  are  managed  conservatively  (only  becomes  surgical   when  bowel  perforates)   Pathogenesis  of  NEC:   •   Meconium  ileus  occurs  when  thick  meconium  becomes  impacted   in  the  distal  intestines  –uncomplicated  meconium  ileus   Complicated  meconium  ileus  is  when  perforation  has  occurred.  The  infant  is  resuscitated.  a   nasogastric  tube  is  placed.  TPN  is  started.  distended  proximal  bowel  that   occupies  most  of  the  abdomen   Barium  enema:  transition  zone  (may  be  unreliable  in  the   newborn  because  the  colon  is  not  dilated  enough  to  show  a   transition  zone)     Transition  zone  seen  in  barium  enema              7  of  14     .   which  suggests  a  causative  role  of  bacteria  in  the  pathogenesis  of  NEC.  host  is  immunologically  vulnerable  and   physiologically  immature.  which  renders  the  host  susceptible  to  barrier  dysfunction  and   the  development  of  NEC.  and  broad-­‐spectrum  parenteral  antibiotics  are   given.  systemic  sepsis  with  diffuse  multisystem  organ   dysfunction  suggests  a  role  for  circulating  proinflammatory  cytokines  in   the  pathogenesis  of  NEC.

 This  is  actually  sloughed-­‐off  mucosa  because  of   the  intussusception       (L)  Intra-­‐op  findings  showing  proximal  bowel  telescoping  into  the  distal   bowel.  surgical  reduction.  If  not   successful.  In  most  cases.”  -­‐_-­‐)   The  rectum  fails  to  descend  through  the  external  sphincter  complex.  the  blind  rectal  pouch  communicates   more  distally  with  the  genitourinary  system  or  with  the  perineum  through   a  fistulous  tract.  by  air  or  by  saline.  No  need  to  do  a   colostomy.  the  infant  may  act  normally.  Bloody  mucus   (currant  jelly  stool)  may  be  passed  per  rectum.  and  performing  an   anastomosis  of  ganglionated  bowel  to  either  the  anus  or  a  cuff  of  rectal   mucosa         (L)  Male  patient  with  imperforate  anus.   you  need  to  do  a  temporizing  colostomy  in  order  to  maximize  the   condition  of  the  patient.     Daph.  you  need  to  do  resection  then  anastomosis.  After  resuscitation  and   administration  of  IV  antibiotics.  if  reduction  is   not  accomplished.  Infants  with  a  high   lesion  require  a  colostomy  in  the  newborn  period.   The  surgical  management  of  infants  with  imperforate  anus  is  determined   by  the  anatomic  defect.   Recurrence  is  3-­‐11%  usually  within  72  hours   Mortality  is  1-­‐3%   Typically.   Although  individual  surgeons  may  advocate  one  procedure  over  another.  the  child  should  undergo  radiographic  reduction.  increasing  lethargy  develops.  center:  female  patient  with   imperforate  anus.  it  is  safer  to   perform  a  colostomy  rather  than  jeopardize  the  infant's  long-­‐term   chances  for  continence  by  an  injudicious  perineal  operation.  Between  attacks.  However.  In  general.  There  are  three  viable   options  for  the  definitive  pull-­‐through  procedure  that  are  currently  used.  an  elongated  mass  is   detected  in  the  right  upper  quadrant  or  epigastrium  with  an  absence  of   bowel  in  the  right  lower  quadrant  (Dance's  sign).  but   as  symptoms  progress.  Gill.  the  child  is  evaluated  for  suitability  to   proceed  with  radiographic  vs.  but  if  not  successful.  gangrene  of  the  intussusceptum  occurs.  check  the   perineum  of  newborns   Treatment  is  surgical  reconstruction  (Having  no  anus  is  not   compatible  with  life.  The   classic  surgical  approach  consisted  of  a  multiple-­‐stage  procedure.  only  a   perineal  operation  is  required  without  a  colostomy.  Gubs         Most  (90%)  cases  are  idiopathic   Incidence  varies  from  1-­‐4  :  1000  births   st 2/3  of  cases  occur  before  patient’s  1  birthday   80%  occur  before  age  24  months   Rare  in  patients  <3  mos  and  less  common  >  36mos  (age  range  is  3   mos  to  3  yrs)     Currant-­‐jelly  stools  occur  in  only  about  50%     Dance  sign  –empty  RLQ   Most  are  reducible  (75%)  by  barium.   treatment  is  surgical.  above  or  below  the   levator  ani  muscle.  In  the  absence  of   peritonitis.  This   included  a  colostomy  in  the  newborn  period.   Patients  with  intussusception  should  be  assessed  for  the  presence  of   peritonitis  and  for  the  severity  of  systemic  illness.  and   perforation  may  ensue.  (R)  manual  reduction  by  pushing  from  the  distal  part  (attempt   this  first.  may  be  staged   • A  diagnosis  of  Hirschsprung's  disease  requires  surgery  in  all  cases.         Currant-­‐jelly  stools.   resecting  the  aganglionic  segment  of  bowel.  the  principles  of   treatment  include  confirming  the  location  in  the  bowel  where  the   transition  zone  between  ganglionic  and  aganglionic  bowel  exists.  “Lahat  tayo  may  butas  ang  pwet.   studies  have  demonstrated  that  the  outcome  after  each  type  of  operation   is  similar.   • • • Increased  staining  of  a  cholinesterase  stain   Presence  of  hypertrophied  nerve  bundles   Treatment  is  surgical  in  all  cases.  the  urinary  tract  needs  to  be  carefully  evaluated  at  the   time  of  colostomy  formation  to  ensure  that  normal  emptying  can  occur   and  to  determine  whether  the  bladder  needs  to  be  drained  by  means  of  a   vesicostomy.     • • • Rectal  manometry:  absence  of  reflex  anal  relaxation   Rectal  biopsy:  definitive  diagnosis  (gold  standard)   o Suction  rectal  biopsy  –  for  neonates   o Full  thickness  biopsy  –  for  older  children   Absence  of  ganglion  cells  in  the  myenteric  and  submucous   plexuses   o Absence  of  ganglion  cells  in  Auerbach's  plexus  and  hypertrophy  of   associated  nerve  trunks.  On  physical  examination.  so….  The  mass  may  be  seen   on  plain  abdominal  radiograph  but  is  more  easily  demonstrated  on  air  or   contrast  enema.  the  rectal  pouch  ends  blindly  in  the  pelvis.  Ultimately.     • IMPERFORATE  ANUS   • • • • • • • • Approximately  1  in  5000  births   Failure  of  descent  of  the  urorectal  septum   Broadly  classified  as  “high”  or  “low”   Patients  with  a  high  type  usually  require  a  preliminary  colostomy   Diagnosis  is  not  difficult  but  it  is  often  missed.  If  there  is  any  doubt  about  the  type  of  lesion.  then  do  surgery)     MECKEL’S  DIVERTICULUM   • “Rule  of  2”   o 2%  of  the  population            8  of  14     .  The  most  common  is  rectosigmoid   Hirschprung’s  where  you  do  a  trans-­‐anal  pullthrough.  (R)  prone  cross-­‐table  field  (x-­‐ray)  showing  how  to   determine  if  it  is  high  or  low  (low  type  in  the  figure)     INTESTINAL  OBSTRUCTION  IN  OLDER  CHILDREN   INTUSSUSCEPTION   • • • • • • • • • • •   Treatment  may  be  staged.  followed  by  a  pull-­‐ through  procedure  at  approximately  2  months  of  age.  followed  by  a  definitive  pull-­‐ through  operation  after  the  child  weighed  >10  kg.  the  infant  develops  paroxysms  of  crampy  abdominal  pain  and   intermittent  vomiting.  if  the  patient  presents  with  a  very  big  abdomen.  For  each  of  the  operations  that  is  performed.  When  a  persistent   cloaca  is  present.  when  a  low  lesion  is  present.   Instead.  Jaff.

 abdominal  distension.  which  requires  a  reconstruction   of  the  inguinal  floor.  episodic  and  massive   • Work-­‐ups:    Meckel’s  Scan   o Pertechnetate  ions  carrying  technetium  99m  secreted  into   lumen  by  gastric  mucosal  cells   o To  improve  accuracy:  histamine  blockers  to  inhibit  secretion   of  pertechnetate.  crampy  pains   • Should  be  considered  in  any  previously  healthy  child  without   history  of  prior  operation  who  develops  a  bowel  obstruction     Bleeding  Secondary  To  Meckel’s  Diverticulum   • Age  –  less  than  5  years  of  age   • Manifestations:  painless  melena.  distension  and  vomiting   Non-­‐operative  management  is  usually  successful  –bowel  rest  and   antibiotics   10-­‐20%  of  the  time  you  have  to  do  adhesiolysis     INGUINAL  HERNIAS   • • • • • • Due  to  a  patent  processus  vaginalis   o When  baby  is  still  in  the  womb.  You  take  out  the  diverticulum.  The  path  of  the  testis  to  the  scrotum  is  the   processus  vaginalis.   70%  are  perforated  before  being  seen  by  a  surgeon   May  differ  in  presentation  from  that  of  adults   Children  may  localize  pain  poorly   Physical  examination  is  also  relatively  more  difficult   • The  definitive  treatment  for  acute  appendicitis  is  appendectomy     • •     Obstruction  Secondary  To  Meckel’s  Diverticulum   • Usually  due  to  intussusception  or  volvulus   o Meckel’s  diverticulum  can  be  the  lead  point  for   intussusception  10%  of  the  time   o Meckel’s  diverticulum  is  an  omphalomesenteric  duct   remnant    there  might  be  an  attachment  to  the  anterior   abdominal  wall    bowel  can  twist  around  it    volvulus   • Symptoms:  vomiting.  inguinal  hernia   develops.  Before  the  baby  is  born.  testis  is  still  inside  the   abdomen.  you  have  to  take  out  the  proximal  part  because   that  is  where  the  ulceration  will  be.  Gill.  Gubs               POST-­‐OPERATIVE  ADHESIONS   • • • • • • Most  common  cause  of  intestinal  obstruction  in  general   Adhesions  are  fibrous  bands  that  form  after  intra-­‐abdominal   inflammation   Obstruction  occurs  when  bowel  is  compressed  or  tethered  due  to   these  fibrous  hands   o Patients  usually  present  with  partial  gut  obstruction   Present  with  colicky  abdominal  pain.  Jaff.  ~80%  in  <5years  old  )   Approximately  52%  of  children  perforate  before  seeing  a  doctor.  just  before  the  baby  is  born.  After  which.   Approximately  1-­‐5%  incidence   Male:female  ratio  is  6-­‐10:1   Right  >  left  >  bilateral   Incarceration  exceeds  60%  in  the  first  6  months  of  life   The  presence  of  an  inguinal  hernia  in  a  child  is  an  indication  for  surgical   repair.  pentagastrin  to  stimulate  uptake.     Fewer  than  2  feet  from  ileocecal  valve   2  types  of  heterotopic  mucosa    Gastric    Pancreatic   o Younger  than  2  years  of  age   o 2  inches  long   Primary  clinical  presentations:   o Obstruction   o Bleeding   o Inflammation   Rare  problems   o Site  of  foreign  body  impaction   o Origin  of  neoplasia   o Site  of  parasitic  infection   o o • •     Wedge  resection.  ther  would  be  ulceration  in  the   part  proximal  to  the  Meckel’s  diverticulum   • Treatment:  segmental  resection  rather  than  diverticulectomy   preferred     Inflammation   • Meckel’s  diverticulitis  rarely  diagnosed  preoperatively   • Differential  diagnosis:  acute  appendicitis   • Treatment:  resection  of  diverticulum  with  or  without  segment  of   intestine     Daph.  you  repair.   glucagon  to  help  restrict  activity  to  stomach   o Accuracy  rate:  90%  (sensitivity  85%.  The  operation  is  termed  a  herniorrhaphy  because  it  involves   closing  off  the  patent  processus  vaginalis.  the  testis  migrates  to  the   scrotum.  specificity  95%)   • Mechanism:  peptic  ulceration  due  to  acid  production  by   heterotopic  gastric  mucosa   o Because  of  acid  secretion.  When  it  does  not  happen.   this  obliterates.     ACUTE  APPENDICITIS   • • • Incidence  of  perforation  rises  as  age  of  patients  decreases  (~25%   in  adolescents.  If  you’re  doing  the   surgery  for  bleeding.  This  is  to  be  contrasted  with  the   hernioplasty  that  is  performed  in  adults.            9  of  14     .  Normally.

 (center)  wide  excision.  WT1.  multidisciplinary  approach.   May  arise  in  1  or  both  kidneys   May  be  associated  with  hemihypertrophy.  and  newer   protocols   Cancer  in  children  (<15y.  adrenal.  the  affected  children  often   present  at  an  earlier  age.  firm  mass  located  within  the  epididymis   (  separate  from  the  testis)   Testicular  cancer  –  a  solid  mass  that  is  part  of  the  testis  may  be   cancerous   • • COMPARISON  OF  CHILDREN  AND  ADULT  HERNIAS   Hernias  in  children   Hernias  in  adults   Congenital   Congenital  &/or  acquired   Indirect   Indirect  &/or  direct   High  ligation  of  sac   High  ligation  of  sac  with  repair  of   floor   • •   Most  common  childhood  renal  malignancy   Clinically.  aniridia  and  other   congenital  anomalies.kidney.3   6.  and   the  adjacent  aniridia  gene.  usually  of  the  genito-­‐urinary  tract   Other  associated  symptoms  include  hematuria.  male  pseudohermaphroditism   Beckwith-­‐Weidemann  –  organomegaly  (liver.0   4.  in  US)   ALL   23.o.  loss   Occasionally  the  mass  is  discovered  following  blunt  abdominal  trauma     Syndromic  or  non-­‐syndromic   o Wilms'  tumor  can  arise  from  both  germline  and  somatic  mutations.   When  a  heritable  risk  factor  is  identified.5   2.   mental  retardation   PEDIATRIC  NEOPLASMS   • The  tumor  usually  develops  in  otherwise  healthy  children.1   16.  a   communicating  hydrocele  is  also  a  hernia)   Epididymal  cyst  –  a  small.o.  non-­‐familial  aniridia   o Hemihypertrophy   o Genitourinary  anomalies   Associated  syndromes:   o WAGR  –  Wilm’s.  genitourinary  abnormalities.  Gill.  distortion  of   collecting  system   Complex  mixed  embryonal  neoplasm  composed  of  3  elements:   blastema.  smooth  and  relatively   firm   Hernia  –  a  mass  that  arises  in  the  abdomen  (cannot  feel  above  it   in  the  scrotum)  and  may  be  tender   Varicocoele-­‐  a  mass  above  th  testis  that  feels  like  a  “bag  of   worms”   Hydrocoele  –  a  cystic  mass  which  surrounds  the  testis  (fluid   accumulation  in  a  processus  vaginalis  that  has  closed.  2000         Full  Scrotum     20.  as  an   asymptomatic  mass  in  the  flank  or  upper  abdomen.  and  are  frequently  bilateral.  Frequently.  exposure  of  femoral  vessels.0   2.   fever.  epithelia.  usually  asymptomatic  except  for  mass  (usually  a  flank   mass)   o • • • • • • Imaging:  solid  renal  tumor  +/-­‐  local  extension.   o   SUMMARY   • • • Discussed  how  to  diagnose  and  differentiate  congenital   abdominal  wall  defect   Discussed  the  more  common  pediatric  surgical  GIT  diseases   Discussed  the  principles  of  managing  congenital  abdominal  wall   defects  and  the  more  common  pediatric  surgical  GIT  diseases       • • •  Tumors  in  children:   o Some  tumors  are  only  or  predominantly  seen  in  children   o Marked  increase  in  survival  because  of  better  imaging.  hypertension.  Nearly   97%  of  Wilms'  tumors  are  sporadic  in  that  they  occur  in  the  absence   of  a  heritable  or  congenital  cause  or  risk  factor.  anorexia.  Jaff.  peak  age  at  3y.1   5.2   Daph.   (R)  muscular  flap  and  split-­‐thickness  graft  placed  over  exposed  areas       • • • • • • WILM’S  TUMOR  (NEPHROBLASTOMA)     Normal  testis  –  testes  are  equal  in  size.  on  chromosome  11p13     Denys-­‐Drash  –  early–onset  renal  failure  with  renal  mesangial   sclerosis.5   locus.2   2.3   6.  the   mass  is  discovered  by  a  parent  while  bathing  or  dressing  the  child.     CNS   Neuroblastoma*   NHL   Wilms  Tumor*   Hodgkins   AML   Rhabdomyosarcoma*   Osteosarcoma   Ewing’s  sarcoma   Others   Haase.  Most  of  these   tumors  are  associated  with  germline  mutations     Non-­‐syndromic  associations:   o Sporadic.  There  is  evidence  to  suggest  that  analysis  of  the            10  of  14     .  new   chemo  agents.  macroglossia.  stroma   Children  between  1-­‐5  years  of  age  .  hemihypertrophy    Beckwith-­‐Weidemann  arises  from  mutations  at  the  11p15.   pancreas).  aniridia.7   7.  wt.  PAX6.  omphalocoele.  Gubs       and  can  occur  in  the  presence  or  absence  of  a  family  history.4     (L)  Testicular  mass.     o o The  WAGR  syndrome  has  been  shown  to  result  from  the   deletion  of  one  copy  each  of  the  Wilms'  tumor  gene.

 Occasionally.   Chemotherapy  pre-­‐op  or  post-­‐op    Stage  I  and  II.  favourable  histology:  vincristine  and   dactinomycin    Stage  III.  lymph  nodes  and  skin  (lung  metastasis  rare)   Less  commonly.      Daph.  and  provide  information  on  the  opposite  kidney.  tumors  with  evidence  of  invasion  of   vessels  in  the  renal  sinus  (without  any  other  reason  to  classify  as  stage  II)  were   classified  as  stage  II  vs.  all  patients  suspected  of  Wilms'  tumor   should  undergo  abdominal  and  chest  CT  scanning.  Tumor  is   not  completely  resectable  because  of  local  infiltration  into  vital  structures.  The  survival  rates  are  worse  in  the   small  percentage  of  patients  considered  to  have  unfavorable   histology     Staging  of  Wilms’  Tumor   Stage  I:  Tumor  limited  to  the  kidney  and  completely  excised.  the  overall  survival  is  less  than  30%.  Importantly.  Lymph  nodes  in  the  renal   hilum.  The  tumor   was  biopsied  or  there  was  local  spillage  of  tumor  confined  to  the  flank.  An  abdominal   ultrasound  should  be  performed  to  detect  the  presence  of   renal  vein  or  vena  caval  extension.  the  periaortic  chains.   Stage  V:  Bilateral  renal  involvement.  The  tumor  originates  most  frequently  in  the   adrenal  glands.   Essentially.   Provided  only  unilateral  disease  is  present.causing  discomfort   o On  plain  radiograph/CT:  (+)  calcification  and  haemorrhage   o 30%  of  cases  (cervical/thoracic/pelvic  ganglia)   o Metastatic  disease    myriad  signs  and  symptoms   (fever.   and  unlike  Wilms'  tumor.  pain.   Biopsy  may  be  required  if  the  patient  does  not  respond  to  the   initial  chemotherapy.  dactinomycin   and  doxorubicin  +  radiation  therapy  to  the  tumor  bed   Following  nephroureterectomy  for  Wilms'  tumor.  limb  overgrowth   Treatment   o Advances  in  the  care  of  patients  with  Wilms'  tumor  have  resulted  in   o • an  overall  cure  rate  of  roughly  90%.  posterior  mediastinum.  Implants  are  found  on  the  peritoneal  surfaces.   which  are  precursor  lesions  to  Wilms'  tumor.  neck.  chemotherapy  is   administered.  the  need  for   chemotherapy  and/or  radiation  therapy  is  determined  by  the   histology  of  the  tumor  and  the  clinical  stage  of  the  patient.     methylation  status  of  several  genes  in  the  11p15  locus  could   predict  the  individual  risk  to  the  development  of  Wilms'   tumor.  followed  by  a  nephron-­‐sparing  procedure.  These   studies  characterize  the  mass.    appreciable  mass/metastatic.  patients  who  have  disease  confined  to  one  kidney   that  is  totally  removed  surgically  receive  a  short  course  of   chemotherapy.  eye  signs.  If  there  is  spread  above  the   hepatic  veins.  and  periorbital   ecchymoses)   • • • • • • • •    The  tumor  may  cross  the  midline.  failure  to  thrive.   Stage  III:  Residual  tumor  confined  to  the  abdomen.  The  vast   majority  of  patients  have  advanced  disease  at  the  time  of  presentation.  orbital  proptosis.  identify  the  presence  of   metastases.  but  can  arise  in   any  sympathetic  ganglion.   children  may  present  with  pain  from  the  tumor  mass  or  to   bone  pain  from  metastases.  ferritin.   The  goal  of  surgery  is  complete  removal  of  the  tumor.  children  present  first  with  neurologic  signs   and  symptoms)            11  of  14     .  If   bilateral  disease  is  encountered.  Patients  with  more   advanced  disease  or  with  unfavorable  histology  receive  more   intensive  chemotherapy  and  radiation.  Rarely.   • Clinically.   • May  be  in  any  sympathetic  site.  No   residual  tumor  is  appparent  at  or  beyond  the  margins  of  excision.  In  the   National  Wilms’  Tumor  Study  5  (NWTS-­‐5).   neuroblastomas  may  invade  through  neural  foramina  and   compress  the  spinal  cord.  a  radical   nephroureterectomy  is  then  performed  with  control  of  the   renal  pedicle  as  an  initial  step.  with   tumor  relapse  rare  after  that  time.  bone  pain.   CT  scanning  also  indicates  the  presence  of  nephrogenic  rests.   bone  marrow.  children  may  have  severe  watery  diarrhea   due  to  the  secretion  of  vasoactive  intestinal  polypeptide  by   the  tumor.   Stage  IV:  Hematogenous  metastases.  bluish   subcutaneous  nodules.  Even  in  stage  IV.  kidney  displaced  &   collecting  sytem  intact  flank   An  embryonal  cancer  of  the  peripheral  sympathetic  nervous   system   The  most  frequently  diagnosed  solid  neoplasm  in  infants   The  median  age  at  diagnosis:  2  yrs   90%  of  cases  diagnosed  before  age  5   Incidence  slightly  higher  in  males  and  in  whites   Clinical  manifestations:   o May  develop  at  any  site  of  sympathetic  nervous  system   tissue.  Diffuse  peritoneal   contamination  by  the  tumor.  such  as  by  spillage  of  tumor  beyond  the  flank   before  or  during  surgery.  and   accounts  for  approximately  10%  of  all  childhood  cancers.  Proptosis  and  periorbital   ecchymosis  may  occur  due  to  the  presence  of  retrobulbar   metastasis.  but  is  completely  excised.  most  commonly  adrenal   • Neuroblastomas  arise  from  the  neural  crest  cells  and  show  different   levels  of  differentiation.  favourable  histology:  vincristine.  most  patients  with  Wilms'  tumor  do  not   have  mutations  at  these  genetic  loci     Klippel-­‐Trenaunay-­‐Weber  –  superficial  and  deep  vascular   anomalies.  Jaff.  or  pelvis.  or  by  tumor  growth  that  has  penetrated  through  the   peritoneal  surface.  even  in  the  presence  of   metastatic  spread.  an  intrathoracic  approach  may  be  required.  Gill.  LDH   Imaging:  Mixed  pattern  with  calcification.  Gubs           Wilm’s  tumor     NEUROBLASTOMA   • Most  common  solid  tumor  of  infancy   • Neuroblastoma  is  the  third  most  common  pediatric  malignancy.  and  can  expect  a  97%  4-­‐year  survival  rate.  Tumor   extends  beyond  the  surgical  margins  either  microscopically  or  grossly.  or  beyond  contain  tumor.  the  stage  I  classification  given  in  NWTS-­‐1  through  4.   o Surgical  extirpation  (radical  nephroureterectomy)    Establish  patency  of  the  IVC  before  the  resection  (check   for  tumor  embolus  to  IVC)    Pre-­‐op  CT  to  check  contralateral  kidney     o Before  operation.  “ill  infant/child”.  causing  muscle  weakness  or  sensory   changes.irritability.  Because  they  originate  in  paraspinal  ganglia.  +/-­‐  IA   bleed.  more  commonly  in  the  adrenal  glands   o Most  arise  in  the  abdomen  (adrenal  gland  or  in   retroperitoneal  sympathetic  ganglia)   o Firm  nodular  mass  usually  palpable  in  the  flank  or   midline.   Most  common  sites  of  metastasis:  long  bones  and  skull.  NSE.  diarrhea   Marker:  VMA.  and  a  majority  of  patients   will  already  show  signs  of  metastatic  disease.   Stage  II:  Tumor  that  extends  beyond  the  kidney.  or  with  paraneoplastic  neurologic  findings  including   cerebellar  ataxia  or  opsoclonus/myoclonus.  cure   rates  of  80%  are  achieved.  It  is   crucial  to  avoid  tumor  rupture  or  injury  to  contiguous  organs.

 a  biopsy  is   performed  and  preoperative  chemotherapy  is  provided  based  on   the  stage  of  the  tumor.  Gill.  which  the  parents  note  while  changing  the   child's  clothes  or  while  bathing  the  child.  These  include  elevated  levels  of   serum  catecholamines  (dopamine  and  norepinephrine)  or   urine  catecholamine  metabolites  (vanillylmandelic  acid  [VMA]   or  homovanillic  acid  [HVA]).  small.  may   have  calcifications   o • • Most  children  with  a  liver  tumor  present  with  an  abdominal  mass   that  is  usually  painless.  bone  marrow  biopsy.  but  only  a  minority  of  patients  have  lesions  amenable  to   complete  resection  at  diagnosis.        Can  present  as  a  paraneoplastic  syndrome  of  autoimmune   origin  (ataxia/opsomyclonus)   Some  produce  catecholamines  (that’s  why  you  measure   VMA).   An  intraspinal  component  may  be  present.  particularly  those  at  the  thoracic  inlet.  electron   microscopy.  without  compromising   major  structures.  The  principal  goal  of   surgery  is  to  obtain  at  least  95%  resection.  but  may  complain  of  anorexia  and  weight  loss.   Can  be  diagnosed  in  a  typical  presentation  without  a  primary   tumor  biopsy   Bone  scan  (cortical  bone  involvement)  to  rule  out  metastasis   Bone  marrow  aspirates  and  biopsies  (marrow  disease)  to   rule  out  metastasis   Clinical  extent  of  disease  and  age.   For  example.  These  patients  may  be  observed  safely   without  surgical  intervention  or  tissue  diagnosis.  However.  those  with   stage  I  have  a  good  outcome.  but  are  increased   much  less  commonly  in  other  liver  malignancies.     o Children  diagnosed  with  stage  I  disease  and  II  hepatoblastoma  have   a  cure  rate  of  greater  than  90%.  Thoracic  tumors  may  be  approached  through  a   posterolateral  thoracotomy  or  through  a  thoracoscopic  approach.   Prognosis   o The  overall  survival  rate  for  children  with  hepatoblastoma  is  70%.  and  in  many  cases.  Gubs         Most  common  childhood  hepatic  malignancy   Clinically.  Under  these  circumstances.  low-­‐stage   neuroblastoma  detected  by  screening  may  have  tumors  that   spontaneously  regress.  others  produce  vasoactive  intestinal  peptide   Children  <1  yr:  4S  stage   o o Spontaneous  regression  of  neuroblastoma  has  been  well  described   in  infants.  often  asymptomatic  except  for  mass  +/-­‐  tumor   rupture/  fractures/precocious  puberty   o • • o Hepatocellular  carcinoma  is  the  next  most  common   Nearly  half  of  all  liver  masses  are  benign.  ptosis.  The  patients  are  rarely   jaundiced.  and  radionuclide   scans  to  search  for  metastases.            12  of  14     .  children  of  any  age  with  localized  neuroblastoma  and   infants  younger  than  1  year  of  age  with  advanced  disease  and   favorable  disease  characteristics  have  a  high  likelihood  of  disease-­‐ free  survival.   Marker:  alpha-­‐fetoprotein   o Pathologic  diagnosis    The  goal  of  surgery  is  complete  resection.  especially  in  those  with  stage  4S  disease.   o o o o o • • The  minimum  criterion  for  a  diagnosis  of  neuroblastoma  is   based  on  one  of  the  following  findings:  (1)  an  unequivocal   pathologic  diagnosis  made  from  tumor  tissue  by  light   microscopy  (with  or  without  immunohistology.  Jaff.   • Diagnosis:   o Generally  discovered  as  a  mass  or  multiple  masses  on  plain   radiographs.  Recent  studies   indicate  that  infants  with  asymptomatic.  the  mitosis-­‐karyorrhexis  index.   Treatment  [Schwartz’s]   Daph.  or  increased  levels  of  serum  catecholamines  or   urinary  catecholamine  metabolites).   o   Disease  is  managed  not  by  pediatric  surgeon  alone.  aggressive  multiagent  chemotherapy  has  resulted  in  a   2-­‐year  survival  rate  of  approximately  20%  in  older  children  with   stage  IV  disease.  surgical  resection  is  performed.  In  all  cases  of   intrathoracic  neuroblastoma.     o By  contrast.     o In  children  diagnosed  with  hepatocellular  carcinoma.     o The  fibrolamellar  variant  of  hepatocellular  carcinoma  may  have  a   better  prognosis.  This  includes   radiograph  of  the  chest.  and  the  presence  or   absence  of  schwannian  stroma.   For  unresectable  tumors   o Chemotherapy  may  be  given  pre-­‐operatively  to  reduce  the   size  and  increase  the  chance  of  resectability    o Liver  transplantation  may  be  offered  to  select  patients    •   Chemotherapy  is  more  successful  for  hepatoblastoma  than  for   hepatocellular  carcinoma.   but  is  only  25%  for  hepatocellular  carcinoma.o.  most  are  hemangiomas   Masses  that  look  malignant  may  require  biopsy   Treatment  requires  complete  surgical  resection  or  hepatectomy   o • Radiographic  evaluation  of  these  children  should  include  an   abdominal  CT  scan  to  identify  the  lesion  and  to  determine  the   degree  of  local  invasiveness   Hepatoblastoma  is  most  often  unifocal.  older  children  with  advanced-­‐stage  disease  have  a   significantly  decreased  chance  for  cure  despite  intensive  therapy.  Measurement  of  VMA  and  HVA  in   serum  and  urine  aids  in  the  diagnosis  and  in  monitoring   adequacy  of  future  treatment  and  recurrence.  After  neoadjuvant  treatment  has  been   administered.  Any  abnormalities  on  chest  x-­‐ray   should  be  followed  up  with  CT  of  the  chest.  whereas  stages  III  and  IV  are  usually   fatal.     o Neuroblastoma  in  the  adolescent  has  a  worse  long-­‐term  prognosis.  CT  or  MRI   The  patient  should  be  evaluated  by  abdominal  CT  scan.     o In  general.  while  hepatocellular   carcinoma  is  often  extensively  invasive  or  multicentric.  Abdominal  tumors  are  approached  through  a   transverse  incision.  the  majority  of  patients   survive.  this  is  often  not   possible  due  to  the  extensive  locoregional  spread  of  the  tumor  at   the  time  of  presentation.  Alpha-­‐fetoprotein  levels  are   elevated  in  90%  of  children  with  hepatoblastomas.  Prior  to  the  institution  of  therapy.     Staging  of  Pediatric  Liver  Cancer   Stage  I:  No  metastases.   HEPATOBLASTOMA   • • Tumor  markers  (HVA  and  VMA)  in  urine  elevated  in  95%  and   help  to  confirm  the  diagnosis    Since  these  tumors  derive  from  the  sympathetic  nervous   system.   it  is  important  to  be  aware  of  the  possibility  that  Horner's  syndrome   (anhidrosis.     The  fibrolamellar  variant  of  hepatocellular  carcinoma  may   have  a  better  outcome  with  liver  transplant  than  other   hepatocellular  carcinomas.   regardless  of  stage  or  site.  together  with  cytogenetic   and  molecular  markers  performed  on  the  tumor  tissue     prognosis  and  determination  of  risk-­‐directed  therapy   Prognostic  Indicators   o The  Shimada  classification  describes  tumors  as  having  either   favorable  or  unfavorable  histology  based  on  the  degree  of   differentiation.   Hepatoblastomas  mostly  diagnosed  before  age  4   HCC  have  peak  incidence  between  10-­‐15  y.  and  (2)  the  combination   of  bone  marrow  aspirate  or  biopsy  containing  unequivocal   tumor  cells  and  increased  levels  of  serum  catecholamines  or   urinary  catecholamine  metabolites.  tumor  completely  resected.  compared  to  60%  for  stage  III  and   approximately  20%  for  stage  IV.   Imaging:  large  hepatic  tumor  often  with  central  necrosis.  meiosis)  may  develop.  which   usually  shows  displacement  and  occasionally  obstruction  of  the   ureter  of  an  intact  kidney.  but  with   the  pediatric  oncologist   If  a  hepatoblastoma  is  completely  removed.  catecholamines  and  their  metabolites  will  be   produced  at  increased  levels.  a  more  prolonged   course.  a   complete  staging  work-­‐up  should  be  performed.   o • • • Most  liver  function  tests  are  normal.

 at   the  earliest  possible  time  because  of  tendency  of  the  mass  to   undergo  malignant  degeneration.   o • • • •   The  rectum  and  genital  structures  are  often  distorted  by  the  tumor   but  usually  can  be  preserved  in  the  course  of  resection.  tumor  grossly  resected  with  microscopic  residual   disease  (i.  The   majority  of  patients  who  develop  recurrent  disease  are  salvageable   with  subsequent  platinum-­‐based  chemotherapy.  mass  effect   Marker:  AFB.  germinoma.  which  can  serve  as   tumor  markers.  or  choriocarcinoma).  bleeding.  In  addition.  with  very  small  to  no  intrapelvic  or   presacral  component.  tumor  unresectable  or  resected  with  gross   residual  tumor.  you  need  to  open  up  the  abdomen.  germinomas  also  can  produce  elevation   of  serum  bHCG.  thus  prenatal  intervention  has  been  advocated  in  such   patients.       TERATOMA   • • Germ  cell  origin   Most  common  sites:  sacrococcygeal  >  ovarian  >  others   o • • • • • • • Thoracic  teratomas  usually  present  as  an  anterior  mediastinal  mass.  bulky  mass.   the  lesion  is  biopsied  and  intensive  chemotherapy  is  administered.  but  not  to  the  levels  associated  with   choriocarcinoma.  while  choriocarcinomas   produce  beta  human  chorionic  gonadotropin  (bHCG).  usually  asymptomatic  except  for  mass.  alveolar  type  is   unfavorable   Management:  resection.  Retroperitoneal   teratomas  may  present  as  a  flank  or  abdominal  mass.   Surgery  is  an  important  component  of  the  staging  strategy.e.   In  surgery  for  types  2  and  3.  Yolk  sac   carcinomas  produce  alpha-­‐fetoprotein.  resulting  in   elevation  of  these  substances  in  the  serum.     Primary  resection  should  be  undertaken  when  complete  excision   can  be  performed  without  causing  disability.   Ovarian  teratomas  present  as  an  abdominal  mass.   In  fetuses  with  evidence  of  hydrops  and  a  large  sacrococcygeal  teratoma.  If  this  is  not  possible.  head  and  neck   >  GU  >  limb   Imaging:  nonspecific  solid  mass   Primitive  soft  tissue  tumor  that  arises  from  mesenchymal  tissues   Can  arise  anywhere  and  manifestation  depends  on  site  of  origin   The  tumor  grows  locally  into  surrounding  structures  and   metastasizes  widely  to  lung.  while  children  with   metastatic  disease  at  diagnosis  have  the  poorest  prognosis.  brain.  Jaff.  based  on  the  amount  of  immature  neuroglial   tissue  present.  Tumors  of  higher  grade  are  more  likely  to  have  foci   of  yolk  sac  tumor.  and  the  likelihood  of  spread  to   coccyx       o Complete  resection  of  the  tumor  as  early  as  possible  is  essential.   Most  common  childhood  soft  tissue  sarcoma   Peak  incidence  in  preschool  and  puberty   Clinically.   Perioperative  complications  of  hypothermia  and  hemorrhage  can   occur  with  massive  tumors  and  may  prove  lethal.   o • • Prognosis  is  related  to  site..  positive  margins).  and   involves  biopsy  of  the  lesion  and  evaluation  of  lymphatics.  This  is  of  particular   concern  in  small  preterm  infants  with  large  tumors.     Stage  IV:  Distant  metastases  regardless  of  the  extent  of  liver  involvement.  CT  scans  of  the  affected  area  and  the  chest.   Diagnosis:   o Important  to  have  this  biopsied  or  sent  to  histopath   o The  diagnosis  is  confirmed  with  incisional  or  excisional  biopsy  after   evaluation  by  MRI.  Gubs       RHABDOMYOSARCOMA   • • • • • • • o •   Classification  according  to  degree  of  intrapelvic  extension   Type  I  –  primarily  external.  or  rupture.  regional  lymph  nodes.     Stage  II:  No  metastases.  or  positive  lymph  nodes.  nonprostate  genitourinary).  and   bone  marrow.   Patients  with  tumors  under  5  cm  in  size  have  improved  survival   compared  to  children  with  larger  tumors.  Immature  teratomas  can  be   graded  from  1  to  3.   Often  benign  but  potentially  malignant   Clinically.  or  tumor  rupture  or  spillage  at  the  time  of   surgery.     The  cure  rate  is  excellent  if  the  tumor  is  excised  completely.  B-­‐HCG   Imaging:  complex  mass  with  calcification   Composed  of  tissue  from  all  3  germ  cell  layers   Arise  anywhere  but  usually  in  the  midline   Mature  teratomas  contain  well-­‐differentiated  tissues  and  are   benign   o Whereas  immature  teratomas  contain  varying  degrees  of  immature   neuroepithelium  or  blastemal  tissues.     Sacrococcygeal  Teratoma  (SCT)   • Large  mass  extending  from  the  sacrum   Diagnosis  may  be  established  by  prenatal  ultrasound   • Classified  according  to  the  degree  of  intrapelvic  extension   • Lesions  with  growth  predominantly  into  the  presacral  space  often  present   later  in  childhood   • • Most  are  benign   • Surgery  requires  excision  of  the  coccyx  together  with  the  mass.   prognosis  is  poor.   o   Daph.  Malignant  germ  cell  tumors  usually  contain   frankly  neoplastic  tissues  of  germ  cell  origin  (i.  and  the  biliary  tract.  yolk  sac  carcinoma.   embryonal  carcinoma.     Stage  III:  No  distant  metastases.  then  CT  scan  to  confirm.  presence  of  metastasis.  often  with   symptoms  of  torsion.  paratestis  and  vagina   (nonbladder.  and   bone  marrow  biopsy.  give  chemotherapy  pre-­‐op  if  mass  is  too   big   Surgery:   o o     nonparameningeal  head  and  neck.  resectability..  Gill.e.   histologic  features   o Primary  sites  with  more  favorable  prognoses  include  the  orbit  and   o • • Embryonal  variant  has  favorable  prognosis.  liver.            13  of  14     .  managed  by  surgery  in  a  prone  position   Type  II  –  primarily  external  with  a  small  internal  component   Type  III  –  primarily  internal  with  a  small  external  component     Type  IV–  primarily  intrapelvic     You  can  palpate  these  with  rectal  exam.

 often  isolated  head/neck  skin  lesion  but  may  be   multiple  in  location   Imaging:  plain  x-­‐ray.             Staging  of  Rhabdomyosarcoma   Stage  I:  Localized  disease  involving  the  orbit  or  head  and  neck  (excluding   parameningeal  sites).     Stage  IV:  Metastatic  disease  at  diagnosis.                                 Daph.  just  control  the  bleeding.  Primary  tumors  must  be   5  cm  in  diameter.   Stage  III:  Localized  disease  of  any  other  primary  site.  Gill.  Jaff.  angiogram.  recording.     o o o After  the  tumor  has  decreased  in  size.  and   there  must  be  no  clinical  regional  lymph  node  involvement  by  tumor.  MRI   Few  evident  at  birth  and  then  most  become  apparent  at  1  month   of  age   Frequently  found  on  the  head  and  neck  area   Undergo  a  rapid  growth  during  the  first  6-­‐8  months  of  life   Stationary  phase  follows.  but  radiation  therapy  benefits   patients  with  stage  1  tumors  with  alveolar  or  undifferentiated   histology.     Stage  II:    Localized  disease  of  any  other  primary  site  not  included  in  the  stage  1   category  (unfavorable  sites).  or  genitourinary  region  (excluding  bladder/prostate   sites).  or  biliary  tract  (favorable  sites).     SUMMARY   • •   Discussed  the  common  pediatric  neoplasms  that  can  be  managed   surgically   Discussed  the  basic  principles  of  managing  pediatric  tumors   -­‐END  OF  LECTURE-­‐     SOURCES:   th th Ppt.  that’s  where  you  do  surgery.     Radiation  therapy  is  effective  in  achieving  local  control  when   microscopic  or  gross  residual  disease  exists  following  initial   treatment.  If  it   bleeds.  after  which  involution  takes  place  at   around  18  months   50%  involute  by  age  5.  These  patients  differ  from   stage  2  patients  by  having  primary  tumors  >5  cm  and/or  regional  node   involvement.     Patients  with  completely  resected  tumors  of  embryonal  histology  do   well  without  radiation  therapy.  70%    involute  by  age  7  and  90%  involute   by  age  9   Management  is  primarily  observation.  resection  of  gross  residual   disease  should  be  performed.  If  it  is  in  the  area  where  it   could  compromise  airway.  Schwartz’s  Principles  of  Surgery  8  and  9  Ed.  Gubs                  14  of  14     .       HEMANGIOMA   • • • • • • • • • Most  common  human  congenital  anomaly:  most  common   tumor  of  infancy   Clinically.