Quid Refert, Dummodo non Desinas, Tardius

Ire

 
GENERAL   SPLEEN  

SURGERY   DR.  WENCESLAO  

 
 
ANATOMY   o Red   pulp:   75%   of   total   volume.   Comprised   of   venous  
Since  this  is  not  discussed  by  doc,  I  included  the  book  as  part  of  the  trans  J   sinuses.Separated   by   reticulum.Contains  
  macrophages.Serves   as   filter,   removes   microrganisms,  
v Adult  spleen  weighs  between  100  to  150  grams.  7-­‐11cm-­‐length   cellular   debris,antigen-­‐antibody   complexes   and   old  
v It   is   located   in   the   left   upper   quadrant   and   protected   by   the   lower   erythrocytes  
portion  of  the  rib  cage   o White  pulp-­‐consists  of  lymphoid  follicles  
v Its  position  is  maintained  by  several  ligaments:   • Serves  the  immunologic  function  of  the  spleen  
o Splenophrenic   -­‐-­‐     Marginal   zone   -­‐   poorly   defined   vascular   space   between   pulps.   It   contains  
o Splenorenal     sequestered  foreign  materials  and  plasma  as  well  as  abnormal  cellular  elements  
o Splenocolic      
o Gastrosplenic  (contains  the  short  gastric  vessels)   BOOK  
v It   is   supplied   by   the   Splenic   Artery   (branch   of   the   celiac   artery);    
venous  drainage  course  through  the  splenic  vein   v Largest  reticuloendothelial  organ  in  the  body  
v Accessory   spleens   have   been   reported   in   14   to   30   %   of   patients   v Arising   from   the   primitive   mesoderm   as   an   outgrowth   of   the   left  
usually   seen   at   the   hilum   of   the   spleen,   gastrosplenic,   splenocolic,   side  of  the  dorsal  mesogastrium.  
gastrocolic,  splenorenal  ligaments  and  greater  omentum     v By    the    fifth    week    of    gestation    the    spleen    is    evident    in    an    
  embryo  8  mm  long  
v MC  anomaly  of  splenic  embryology-­‐  accessory  spleen  
v Over    80%    of    accessory    spleens    are    found    in    the    region    of    the  
splenic  hilum  and  vascular  pedicle.  
v  Other     locations     for     accessory     spleens     in     descending     order     of  
frequency  are:  the  gastrocolic  ligament,  the  tail  of  the  pancreas,  the    
greater     omentum,     the     greater     curve     of     the     stomach,     the  
splenocolic   ligament,   the   small   and   large   bowel   mesentery,   the   left    
broad    ligament    in    women,    and    the    left    spermatic    cord    in  men.    
v The    abdominal    surface    of    the    diaphragm    separates    the    spleen  
from     the     lower     left     lung     and     pleura     and     the     ninth     to     eleventh  
ribs.    
v The   visceral   surface   of   the   organ   faces   the   abdominal   cavity   and  
contains  gastric,  colic,  renal,  and  pancreatic  impressions.    
v Spleen   size   and   weight   vary   with   age   and   underlying   pathologic  
conditions,  but  spleen  size  and  weight  diminish  in  the  elderly.  
v 7  to  11  cm    in  length  and    weighs  150  g  (range   70   to   250   g)-­‐average  
adult  spleen  
 
• Accessory   spleen:   The   accessory   spleens   seen   here   at   the   hilum   of   v Most     authors     would     agree     that     "splenomegaly"     would     apply     to  
the  normal-­‐sized  spleen  are  not  uncommon  and  by  themselves  have   organs    weighing  500  mg  or  more  and/or  15  cm  or  more  in  length.    
no  significance     v To   be   palpable   below   the   left   costal   margin,   a   spleen   must   be   at  
least  double  normal  size.  
v The     superior     border     of     the     spleen,     which     separates     the  
diaphragmatic    surface    from    the    gastric    impression    of    the  visceral    
surface,     often     contains     one     or     two     notches,     which     can   be  
pronounced  when  the  spleen  is  greatly  enlarged.  
v Of     particular     clinical     relevance,     the     spleen     is     suspended     in  
position    by    several    ligaments    and    peritoneal    folds    to    the    colon  
(splenocolic    ligament);    the    stomach    (gastrosplenic  ligament);  the  
diaphragm     (phrenosplenic   ligament);   and   the   kidney,     adrenal    
gland,    and    tail    of    the    pancreas    (splenorenal  ligament)  
 
Blood  supply:  
v The    spleen    derives    most    of    its    blood    from    the    splenic    arterythe  
longest    and  most  tortuous  of  the  three  main  branches  of  the  celiac  
  artery.    
Accessory  Spleen   v The    splenic    artery    can    be    characterized    by    the    pattern    of    its  
  terminal  branches.  
v Splenosis     v The  distributed  type  of  splenic  artery  is  the  most  common  (70%)    
o Autotransplantation  of  splnenic  fragments  after  trauma   and  is  distinguished  by  a  short  trunk  with  many  long  branches    
o Capable  of  performing  some  reticuloendothelial  function   entering  over  three  fourths  of  the  medial  surface  of  the  spleen.  
v The  pulp  is  consist  of  three  (3)  zones:    
 
I PUSH MO LANG YAN! J
¬ istepania¬
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 v The    less    common    (30%)    magistral    type    of    splenic    artery    has    a     DIAGNOSTICS  
long    main    trunk    dividing    near    the    hilum    into    short    terminal      
branches,  which  enter  over  25  to  30%  of  the  medial  surface  of     1.  Evaluation  of  size  
the  spleen.        a.  Physical  Examination  
v The  spleen  also  receives  some  of  its  blood  supply  from  the  short     • Normally  not  palpable  
gastric    vessels,    which    are    branches    of    the    left    gastroepiploic     • Felt  in  about  2  %  of  healthy  adults  
artery  running  within  the  gastrosplenic  ligament.     • No   significant   dullness   elicited   by   percussion  
v The    splenic    vein    accommodates    the    major    venous    drainage    of     over  spleen  either  anteriorly  or  laterally  
the  spleen.     • As   organ   enlarges,   dullness   is   detected   at   the  
th
v  It  joins  the  superior  mesenteric  vein  to  form  the  portal  vein   level   of   the   9   ICS   in   the   left   anterior   axillary  
  line  
     b.  MRI  –  depicts  the  spleen  and  define    
             abnormalities  in  size,  shape  and  parenchymal    
PHYSIOLOGY              pathology,  e.g.  cyst,  abscess  or  tumor  
     c.  99Tc  sulfur  colloid  –  defines  the  organ  
v Filtering  Functions   2.  Evaluation  of  Function  
o Most  important  function   – Functional  abnormality  of  hypersplenism  is  manifested  by  
o Splenic  blood  flow  is  approximately  350  L/day   reduction  in  number  of  RBC,  neutrophils,  platelets  in  the  
o Removal   of   abnormal   red   blood   cells-­‐Howell-­‐jolly   peripheral  blood  
bodies,Heinz   bodies,   Pappenheimer   bodies.     – Increase   in   the   rate   of   red   cell   destruction   will   always  
Approximately  20  ml  of  aged  RBC  are  removed   during  the   result   in   compensatory   rise   in   the   rate   of   production  
course  of  the  day   unless  disease  in  bone  marrow  coexists  –  the  hallmark  of  
o Removal   of   abnormal   white   cells,   normal   and   abnormal   reticulocytosis  in  the  absence  of  blood  loss  
platelets  and  cellular  debris    
o It   can   clear   organisms   contained   w/in   the   erythrocytes(   SPLENECTOMY  
malaria  &  Bartonella)  
o Can   clear   unopsonized   bacteria   and   microrganisms   for  
w/c  the  body  has  no  anti-­‐bodies  

 
PRE  OPERATIVE  AND  POST  OPERATIVE  
 
indications  
– Splenic  rupture  (trauma)-­‐the  most  common  indication  
  – Platelet   disorders   (ITP)-­‐the   most   common   indication   for  
  elective  splenectomy    
v Open  circulation-­‐(90%)   – Red  bld  cell  disorders(HS)  
o Blood    goes  first  to  reticular  space  and  cords   – Cyts  and  tumors  
o Comes  in  contact  with  macrophages   – Infections  and  abscesses  
v Closed  circulation-­‐(10%)    
o Goes  directly  to  the  arteriovenous  anastomosis     BOOK  
v Host  Defense    
o Opsonins-­‐makes   organism   attractive   to   ULTRASOUND  
phagocytes   v Ultrasound  is  the  least  invasive  mode  of  splenic  imaging;  rapid,    
o Tuftsin-­‐enhances   the   phagocytic   activity   of   easy    to    perform,    and    does    not    expose    the    patient    to    ionizing    
leukocytes   radiation  
o Properdin-­‐stimulates   the   alternative   pathway   v It  is  often  the  first  imaging  modality  applied  to  the  spleen  during    
of  complement  fixation   evaluation  and  resuscitation  of  the  trauma  patient  
o Circulating   monocytes   are   converted   to   fixed   v Percutaneous  ultrasound-­‐guided  procedures  for  splenic  disease    
macrophages  –at  the  red  pulp   (e.g.,  cyst  aspiration,  biopsy),  historically  avoided  due  to  the  risk    
o Produces  !immunoglobulin  (IgM)   of    hemorrhage    and    other    complications,    are    becoming    more    
o T   and   B   lymphocytes-­‐from   the   lymphatic   common  as  the  safety  of  these  procedures  is  being  increasingly    
sheath  surrounding  the  central  arteries   demonstrated  
   
v Storage-­‐  30%  of  platelets  are  stored  in  the  spleen   CT  SCAN  
v Cytopoeisis-­‐   v Computed  tomography  (CT)  affords  a  high  degree  of  resolution    
o Contributes  to  the  process  of  RBC  maturation   and  detail  of  the  splenic  parenchyma  
th
o Minor  role  in  hematopoeisis  in  the  4  mo.   v In  the  nontrauma  setting,  CT  is  extremely  useful  for  assessment    
o Can  be  reactivated  in  childhood  if  the  bone  marrow  fails   of    splenomegaly,    identification    of    solid    and    cystic    lesions,    and    
to  meet  the  hematologic  need.   guidance  of  percutaneous  procedures  
o Myeloid  metaplasia-­‐abnormal  RBC  are  produced   v The  use  of  iodinated  contrast  material  adds  diagnostic  clarity  to    
  CT    imaging    of    the    spleen,    although    at    the    cost    of    the    small    but    
 

 
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 real  risks  of  renal  impairment  or  allergic  reaction.   • Related   to   temporary   tamponade   of   a   minor  
  lacerations   or   presence   of   a   slowly   enlarging  
PLAIN  RADIOGRAPHY   subcapsular   hematoma   that   eventually  
v    Rarely  is  plain  radiography  used  for  primary  splenic  imaging.   ruptures  
v Plain  films  can    indirectly  provide  an  outline  of  the  spleen  in  the       3.  Occult  splenic  rupture  
left    upper    quadrant    or    suggest    splenomegaly    by    revealing     • Term  applied  to  pseudocyst  of  the  spleen  (1%)  
displacement  of  adjacent  air-­‐filled  structures  (e.g.,  the  stomach      
or  splenic  flexure  of  the  colon)   Clinical  Manifestations:  
v Plain    films    may    also    demonstrate    splenic    calcifications    –    often       1.  Signs  and  symptoms  vary  according  severity  and  
are  found  in  association  with  splenomegaly  but  are  otherwise  a              rapidity  of  infra-­‐abdominal  hemorrhage  and  the  
nonspecific  finding            interval  between  injury  and  examinations  
v Splenic    calcifications    can    indicate    a    number    of    benign,       2.  Hypovolemia  and  tachycardia  
neoplastic,  or  infectious  processes,  including  phlebolith,  splenic       3.  Slight  reduction  in  blood  pressure  
artery  aneurysm,  sickle  cell  changes,  tumors  (e.g.,  hemangioma,       4.  Generalized  abdominal  pain  while  ½  of  patients  complain  of    
v hemangiosarcoma,  lymphoma),  echinococcosis,  or  tuberculosis   localized  left  upper  quadrant  pain  
    5.  Kehr’s  sign  –  at  the  tip  of  the  shoulder  indicative  
MRI            of  diaphragmatic  irritation  
v Although    magnetic    resonance    imaging    offers    excellent    detail       6.  Balance’s  sign  (mass  or  fixed  dullness  at  the  left    
and  versatility  in  abdominal  imaging,  it  is  more  expensive  than        quadrant  secondary  to  subcapsular    hematoma)  
CT  scanning  or    ultrasound  and  offers  no  obvious  advantage  for      
primary  imaging  of  the  spleen   DIAGNOSTICS  
v Magnetic    resonance    imaging    can    be    a    valuable    adjunct    to    the     1.  CBC  –  serial  Hct  determination;  WBC          count   frequently  
more  commonly  used  imaging  techniques  when  splenic  disease     greater  than  15,000/cu  ml  
is  suspected  but  not  definitively  diagnosed   2.  X-­‐ray  of  the  abdomen  –  fractured  ribs                  should   arouse  
  suspicion  of  injury  to  the  spleen;  more  specific  findings:  
ANGIOGRAPHY   • Elevated  immobile  diaphragm  
v Angiography    of    the    spleen    most    commonly    refers    to    invasive     • Enlarged  splenic  shadow  
arterial    imaging,    and    when    it    is    combined    with    therapeutic     • Medial  displacement  of  gastric  shadow  
splenic  arterial  embolization  (SAE)   • Widening   of   space   between   splenic   flexures  
v localization    and    treatment    of    hemorrhage    in    select    trauma   and  preperitoneal  fat  
  3.  CT  scan  
NUCLEAR  IMAGING   4.  Angiography  
v  Radioscintigraphy    with    technetium    Tc    99m    sulfur    colloid      
demonstrates  splenic  location  and  size  
v It    may    be    especially    helpful    in    locating    accessory    spleens    after    
unsuccessful    splenectomy    for    ITP    and    has    recently    proven    
useful  in  diagnosing  splenosis  
 
 
 
 
 
SPLENIC  RUPTURE  
American   Association   for   the   Surgery   of   Trauma   Organ   Injury:   Scale   for   the  
Spleen  

 
 
TREATMENT  
 
v Surgery  
• Children  –  delayed  surgical  treatment  
• Adult   –   require   surgical   intervention;  
splenectomy   as   a   life-­‐saving   procedure   is   still  
standard  treatment  
• Old   age   –   contraindication     for   non   operative  
treatment  
  v Non-­‐operative:  
  – Grades  1-­‐3  splenic  injuries  
Pathology     – 70-­‐90%-­‐success  in  children  
  1.  Acute  splenic  rupture   – 40-­‐50%-­‐success  in  adults  
• Immediate  intraperitoneal  bleeding   • Criteria:  
• Occurs   in   about   90   %   of   the   cases   in   blunt   • Hemodynamic  stability  
trauma  to  the  spleen   • Absence  of  other  abdominal  injury  
  2.  Delayed  rupture   • CT-­‐scan  documentation  of  grade  of  
• Interval   in   days   or   weeks   between   injury   and   injury  
intraperitoneal   bleeding   in   10   –   15   %   of   • Tranfusion   requirement   of   <   2   units  
patients   of  blood  
• The  quiescent  period  (latent  period  of  Baudet)    
-­‐less  than  2  weeks  in  75%  of  cases   PLATELET  DISORDERS  

 
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 IDIOPATHIC  THROMBOCYTOMPENIC  PURPURA  
 
v Idiopathic  thrombocytopenic  purpura    
– Low   platelet   count   with   mucocutaneous   and   petechial  
bleeding  
– Premature  removal  of  platelets  opsonized  by  antiplatelet  
IgG  autoantibodies  produced  by  the  spleen  
• 30,000-­‐50,000/cu.mm-­‐easy  bruising  
• 10,000-­‐30,000/cu.mm-­‐spontaneous   petechiae  
and   eccymosis   <10,000/cu.mm-­‐internal  
bleeding  
Treatment:  
– Oral  prednisone-­‐first  line  of  tx.  
 
• Most  respond  after  3  weeks  (50-­‐75%)    
– IV  immunoglobulin-­‐for  those  w/  internal  bleeding.   BOOK  
• competes   for   binding   to   macrophage    
receptors.given  for  2-­‐3  days   Hereditary  Spherocytosis  
– SPLENECTOMY   v  Hereditary    spherocytosis    (HS)    results    from    an    inherited    
• Failure  of  medical  tx     dysfunction    or    deficiency    in    one    of    the    erythrocyte    membrane    
• Prolonged   use   of   steroids   w/   undesirable   proteins  (spectrin,  ankyrin,  band  3  protein,  or  protein  4.2)  
effects   (>   10-­‐20mg/d   for   3-­‐6   months   to   v The    resulting    destabilization    of    the    membrane    lipid    bilayer    
maintain  platelet  ct  of  >30,000/cu.mm   allows  a  pathologic  release  of  membrane  lipids  
• Provides   permanent   response   (75-­‐85%)   in   1   v  The    red    blood    cell    assumes    a    more    spherical,    less    deformable    
week     shape,    and    the    spherocytic    erythrocytes    are    sequestered    and    
destroyed  in  the  spleen  
v Hemolytic    anemia    ensues;    HS    is    the    most    common    hemolytic  
anemia  for  which  splenectomy  is  indicated  
v HS    is    inherited    primarily    in    an    autosomal    dominant;    the    
v Patients  with  typical  HS  forms  may  have  mild  jaundice.  
v Splenomegaly  usually  is  present  on  physical  examination.  
v Laboratory     examination     reveals     varying     degrees     of     anemia     -­‐
patients    with    mild    forms    of    the    disease    may    have    no    anemia;    
patients  with  severe  forms  may  have  hemoglobin  levels  as  low    
as  4  to  6  g/dL  
v The    mean    corpuscular    volume    is    typically    low    to    normal    or    
slightly  decreased  
v For    screening,    a    combined    elevated    mean    corpuscular    
hemoglobin    concentration    and    an    elevated    erythrocyte    
distribution  width  are  an  excellent  predictor  
v Dramatic    clinical    improvement—even    despite    persistent    
hemolysis—often    occurs    after    splenectomy    in    patients    with    
severe  disease  
v children    can    be    affected    with    HS,    the    timing    of    splenectomy    is    
important  and  is  aimed  at  reducing  the  quite  small  possibility  of    
overwhelming  postsplenectomy  sepsis  
v Delaying  such  an  operation  until  the  patient  is  between  the  ages    
 
  of    4    and    6—unless    the    anemia    and    hemolysis    accelerate—is    
THROMBOTIC  THROMBOCYTOPENIC  PURPURA   recommended  
v Gallstones    are    more    likely    to    develop    in    patients    with    HS,    and    
v Thrombotic  throbocytopenic  purpura  (TTP)  
– Occlusion   of   arterioles   and   caplillaries   with   hyalin   over  half  of  patients  between  the  ages  of  10  and  30  with  HS  have    
deposits  composed  of  aggregated  platelets  and  fibrin.   cholelithiasis  
v For  children  with  cholelithiasis  prophylactic  cholecystectomy  is    
– Pentad:  
recommended  at  the  time  of  splenectomy  
• Fever  
• Purpura     v Hereditary    elliptocytosis    (HE)    merits    brief    discussion    to    
distinguish  it  from  HS  
• Anemia  
v Both  HS  and  HE  are  conditions  of  the  red  blood  cell  membrane    
• Neurological  manifestation  
• Renal  dysfunction   that  result  from  genetic  defects  in  skeletal  membrane  proteins.  
v With  the  HE  defect,  the  red  blood  cell  elongates  as  it  circulates,    
 
so    that    far    fewer    red    blood    cells    are    sequestered    or    destroyed    
RED  BLOOD  CELL  DISORDERS  
when  transiting  the  splenic  parenchyma  
HEREDITARY  SPHEROCYTOSIS  
 
 
 
• Hereditary  spherocytosis    
 
– Deficiency   in   one   of   the   membrane   proteins  
CONGENITAL  HEMOLYTIC  ANEMIA  
(spectrin,ankyrin,band3  protein)  
– Spherical   less   deformable   shape,   they   sequestered   and    
destroyed  in  the  spleen  
– The  only  type  of  hemolytic  anemia  which  splenectomy  is  
the  primary  treatment  
– In  children-­‐delayed  until  ages  of  4-­‐6  years  
 
 

 
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 coexisting    immune    deficiency,    in    large    part    brought    about    by    
iron    overload,    which    may    be    associated    both    with    the    
thalassemia  itself  and  with  transfusions  
 
 
SICKLE  CELL  ANEMIA  
 

 
 
BOOK  
   
 
v Sickle  cell  disease  is  an  inherited  chronic  hemolytic  anemia  that    
BOOK  
results  from  the  mutant    sickle  cell  hemoglobin  (HbS)  within  the    
  red    blood    cell    and    is    inherited    in    an    autosomal    codominant    
THALASSEMIA   fashion  
v Thalassemia    is    the    term    for    a    group    of    inherited    disorders    of    
v Persons    who    inherit    an    HbS    gene    from    one    parent    
hemoglobin    synthesis    prevalent    among    people    of     (heterozygous)    are    carriers;    those    who    inherit    an    HbS    gene    
Mediterranean    extraction    and    classified    according    to    the     from  both  parents  (homozygous)  have  sickle  cell  anemia.  
hemoglobin  chain  (alpha,  beta,  or  gamma)  affected   v In  sickle  cell  disease  the  underlying  abnormality  is  the  mutation    
v As    a    group    the    thalassemias    are    the    most    common    genetic    
of    adenine    to    thymine    in    the    sixth    codon    of    the    B-­‐globin    gene,    
diseases  known  to  arise  from  a  single  gene  defect   which    results    in    the    substitution    of    valine    for    glutamic    acid    as    
v    Most  forms  of  this  disorder  are  inherited  in  Mendelian  recessive     the  sixth  amino  acid  of  the  B-­‐globin  chain  
fashion  from  asymptomatic  carrier  parents  
v Mutant    B-­‐chains    included    in    the    hemoglobin    tetramer    create    
v  In    all    forms    of    thalassemia    the    primary    defect    is    absent    or     HbS.    Deoxygenated    HbS    is    insoluble    and    becomes    polymerized    
reduced    production    of    hemoglobin    chains.    From    this     and  sickled  
abnormality    two    significant    consequences    arise:    (1)    reduced    
v The    subsequent    lack    of    deformability    of    the    red    blood    cell,    in    
functioning  of  hemoglobin  tetramers,  yielding  hypochromia  and     addition  to  other  processes,  results  in  microvascular  congestion,    
microcytosis;  and  (2)  unbalanced  biosynthesis  of  individual  and     which  may  lead  to  thrombosis,  ischemia,  and  tissue  necrosis  
subunits,  which  results  in  insoluble  red  blood  cells  that  cannot    
v  The  disorder  is  characterized  by  painful  intermittent  episodes.  
release  oxygen  normally  and  may  precipitate  with  cell  aging.   v Sequestration  occurs  in  the  spleen,  with  splenomegaly  resulting    
v Both  underproduction  of  hemoglobin  and  excess  production  of     early    in    the    disease    course.    In    most    patients    subsequent    
unpaired     hemoglobin     subunits     contribute     to     infarction    of    the    spleen    and    autosplenectomy    occur    at    some    
thalassemiaassociated  morbidity  and  mortality.   later  time  
v A  diagnosis  of  thalassemia  major  (homozygous  form)  is  made  by     v The    most    frequent    indications    for    splenectomy    in    sickle    cell    
demonstrating  hypochromic  microcytic  anemia  associated  with     disease  are  recurrent  acute  sequestration  crises,  hypersplenism,    
randomly    distorted    red    blood    cells    and    nucleated    erythrocytes    
and  splenic  abscess  
(target  cells)  on  peripheral  blood  smear   v The    occurrence    of    one    major    acute    sequestration    crisis,    
v Elevated    reticulocyte    count    and    white    blood    cell    count    are     characterized    by    rapid    painful    enlargement    of    the    spleen    and    
among    the    associated    findings.    Because    chains    are    needed    to    
circulatory    collapse,    generally    is    considered    sufficient    grounds    
form    both    fetal    hemoglobin    and    adult    hemoglobin,    Athalassemia     for  splenectomy  
becomes    symptomatic    in    utero    or    at    birth.    By     v Preoperative    preparation    should    include    special    attention    to    
contrast,  B-­‐thalassemia  becomes  symptomatic  at  4  to  6  months,    
adequate  hydration  and  avoidance  of  hypothermia.  
because    B-­‐    chains    are    involved    only    in    adult    hemoglobin     v Splenectomy    does    not    affect    the    sickling    process,    and    therapy    
synthesis.   for  sickle  cell  anemia  remains  largely  palliative  
v Heterozygous  carriers  of  the  disease  are  usually  asymptomatic.    
v Transfusions    are    indicated    for    anemia,    for    moderately    severe    
v Homozygous    individuals,    on    the    other    hand,    typically    present     episodes  of  acute  chest  syndrome  (i.e.,  a  new  infiltrate  on  chest    
before  2  years  of  age  with  pallor,  growth  retardation,  jaundice,     radiograph  associated  with  new  symptoms,  such  as  fever,  cough,    
and  abdominal  swelling  due  to  liver  and  spleen  enlargement.   sputum    production,    or    hypoxia)    and    preoperatively    before    
v Among    other    characteristics    of    thalassemia    major    are    
splenectomy  
intractable    leg    ulcers,    head    enlargement,    frequent    infections,     v Patients    experiencing    stroke    or    a    severe    crisis    may    require    
and  the  need  for  periodic  blood  transfusions   hydration    and    an    exchange    transfusion,    which    may    be    
v Untreated    individuals    usually    die    in    late    infancy    or    early    
performed  manually  or  with  automated  apheresis  equipment  
childhood  from  severe  anemia   v Hydroxyurea    is    an    oral    chemotherapeutic    agent    that    
v Treatment    for    thalassemia    involves    red    blood    cell    transfusions     v upregulates    fetal    hemoglobin,    which    interferes    with    
to    maintain    a    hemoglobin    level    of    >9    mg/dL,    along    with    
v polymerization  of  HbS  and  thus  reduces  the  sickling  process  
intensive  parenteral  chelation  therapy  with  deferoxamine  
 
v Splenectomy  is  indicated  for  patients  with  excessive  transfusion    
SPLENIC  CYST  
requirements    (>200    mL/kg    per    year),    discomfort    due    to    
 
splenomegaly,  or  painful  splenic  infarction.  
v True  cyst-­‐  
v  Thalassemia    patients    are    at    high    risk    for    pulmonary    
• Small    (<8cm)  asymptomatic.  Can  observed.  
hypertension    after    splenectomy;    the    precise    etiology    of    this    
•  >8cm  w/  symptoms-­‐partial  
sequela  is  under  investigation    
splenectomy,unroofing    
v The  increase  in  infectious  complications  is  likely  to  be  due  to  a    
v Parasitic  cyst  

 
Page  5  of  7  
 
 Secondary  to  Echinococcus    
• v  cirrhosis-­‐most  common  cause  
Tx.   by   splenectomy   after   sterilization   by   NaCL  
• v Splenic  vein  thrombosis  
or  silver  nitrate   o Secondary  to  pancreatitis  
v Pseudocyst     o w/  bleeding  gastric  varices    
• Secondary  to  trauma   o w/  massive  splenomegaly    
• Most   are   small   (<4cm)   and   undergo   o Splenectomy  is  curative  
spontaneous  resolution  
• Can  be  observed  safely  
 
BOOK  
v Splenic    cysts    can    be    categorized    according    to    a    number    of    
criteria;  one  clinically  relevant  scheme  is  to  characterize  splenic    
cysts  as  either  parasitic  or  nonparasitic.  
v Parasitic    infection    is    the    most    common    cause    of    splenic    cysts    
worldwide,  and  the  majority  are  due  to  Echinococcus  species.    
v Such    cysts    are    more    commonly    found    in    areas    where    the    
 
pathogen  is  endemic.     • One   of   the   most   common   findings   with   portal   hypertension   is  
v Symptoms,  when  present,  generally  are  related  to  the    presence     splenomegaly,   as   seen   here.   The   spleen   is   enlarged   from   the  
of    a    mass    lesion    in    the    left    upper    quadrant    or    a    lesion    that     normal  300  grams  or  less  to  between  500  and  1000  gm.    
impinges  on  the  stomach.    
v Ultrasound    can    establish    the    presence    of    a    cystic    lesion    and      
occasionally  incidentally  detect  asymptomatic  lesions  as  well.      
v Serologic    testing    for    echinococcal    antibodies    can    confirm    or     SPLENIC  VEIN  THOMBOSIS  
exclude    the    cystic    lesion    as    parasitic,    an    important    piece    of      
information  when  planning  operative  therapy.      
v Symptomatic  parasitic  cysts  are  best  treated  with  splenectomy.    
v Avoidance    of    spillage    of    parasitic    cyst    contents    into    the    
peritoneal  cavity  to  avoid  the  possibility  of  anaphylactic  shock  is    
an  important  principle  in  surgical  management.  
v Cysts  resulting  from  trauma  are  termed  pseudocysts    due  to  their    
lack  of  cellular  lining.    
v Less    common    examples    of    nonparasitic    cysts    are    dermoid,    
epidermoid,  and  epithelial  cysts.    
 
v The  treatment  of  nonparasitic  cysts  depends  on  whether  or  not    
BOOK  
they  produce  symptoms.    
 
v Asymptomatic    nonparasitic    cysts    may    be    observed    with    close    
v the  most  common  visceral  artery  aneurysm  
follow-­‐up  by  ultrasound  to  exclude  significant  expansion.    
v Women  are  four  times  more  likely  to  be  affected  than  men.    
v Patients  should  be  advised  of  the  risk  of  cyst  rupture  with  even    
v The    aneurysm    usually    arises    in    the    middle    to    distal    portion    of    
minor    abdominal    trauma    if    they    elect    nonoperative    
the  splenic  artery.    
management  for  large  cysts.    
v Indications  for  treatment:  
v Small    symptomatic    nonparasitic    cysts    may    be    excised    with    
o    presence    of    symptoms,    pregnancy,    intention    to    become    
splenic    preservation,  and    large    symptomatic    nonparasitic    cysts    
pregnant,    and    presence    of    pseudoaneurysms    associated    
may  be  unroofed.    
with  inflammatory  processes.    
v Both  of  these  operations  may  be  performed  laparoscopically.  
v Aneurysm  resection  or  ligation  alone  is  acceptable  for  amenable    
 
lesions    in    the    midsplenic    artery,    but    distal    lesions    in    close    
 
proximity    to    the    splenic    hilum    should    be    treated    with    
 
concomitant  splenectomy.    
SPLENIC  ABCESS   v Splenic  artery  embolization  has  been  used  to  treat  splenic  artery    
  aneurysm,  but  painful  splenic  infarction  and  abscess  may  follow  
v Hematogenous  spread-­‐most  common  cause    
v Most  are  solitary  and  unilocular    
ESOPHAGEAL  VARICES  
v Treatment:  
 
– Solitary   and   unilocular-­‐-­‐image   guided   percutaneaous  
ESOPHAGEAL  VARICES  
drainge+antibiotics    
– Multilocular-­‐-­‐-­‐splenectomy+  antibiotics  
 
SARCOIDOSIS  
v Non-­‐caseating  inflammatory  granulomatous  lesion  
v Second  organ  most  commonly  affected  
v Indications  for  splenectomy    
– Massive  splenomegaly  (>1kg)  
• Thrombocytopenia  
• Anemia  
• Compression  of  adjacent  organ  
• Pain  
 
MISCELLANOUS  DISORDERS  
SECONDARY  HYPERSLENISM  
 
Portal  Hypertension    
v Leads  to  splenic  congestion,sequestration  and  destruction  of    
circulationg  cells   BOOK  

 
Page  6  of  7  
 
   NON  HODGKIN’S  LYMPHOMA  
v Patients    with    bleeding    from    isolated    gastric    varices    who    have   BOOK  
normal   liver   function   test   results,   especially   those   with   a   history   of      
pancreatic     disease,     should     be     examined     for     splenic     vein   v Non-­‐Hodgkin's  lymphoma  (NHL)  encompasses  all  malignancies    
thrombosis    and    treated    with    splenectomy    if    findings    are  positive.   derived  from  the  lymphoid  system  except  classic  HD.    
  v A  proliferation  of  any  one  of  the  three  predominant  lymph  cell    
  types—natural    killer    cells,    T    cells,    or    B    cells—may    be    included    
HODGKIN’S  DISEASE   in  the  category  of  NHL.  
  v Because  of  the  wide  net  cast  by  NHL,  the  clinical  presentations    
of  the  disorders  under  its  umbrella  vary.  
v The  subentities  of  NHL  may  be  clinically  classified  into  nodal  or    
extranodal,  as  well  as  indolent,  aggressive,  and  very  aggressive    
groups.    
v Patients  with  indolent  lymphomas  may  present  with  mild  or  no    
symptoms  and  seek  medical  attention  for  a  swollen  lymph  node,    
whereas    the    aggressive    and    very    aggressive    lymphomas    create    
easily    noticeable    symptoms,    such    as    pain,    swelling    due    to    
obstruction  of  vessels,  fever,  and  night  sweats.    
v Surgical    staging    is    no    longer    indicated    for    NHL,    because    the    
combination    of    history    and    physical    examination,    chest    
  radiograph    and    abdominal/pelvic    CT    scan,    biopsy    of    involved    
  lymph    nodes    (including    laparoscopically    directed    nodal    and    
BOOK   liver  biopsies),  and  bone  marrow  biopsy  is  sufficient.  
  v Splenomegaly    exists    in    some,    but    not    all,    forms    of    NHL,    and    
v Hodgkin's    disease    (HD)    is    a    disorder    of    the    lymphoid    system     splenectomy  is  indicated  for  management  of  symptoms  related    
characterized    by    the    presence    of    Reed-­‐Sternberg    cells    (which     to  an  enlarged  spleen  as  well  as  for  improvement  of  cytopenias  
actually  form  the  minority  of  the  Hodgkin's  tumor).      
v More    than    90%    of    patients    with    HD    present    with     SPLENECTOMY  OUTCOME  
lymphadenopathy  above  the  diaphragm.      
v Lymph    nodes    can    become    particularly    bulky    in    the     v Increase  in  circulating  abnormal  RBC  
mediastinum,  which  may  result  in  shortness  of  breath,  cough,  or     v Pulmonary:   left   lobe   atelectasis-­‐the   most   common  
obstructive  pneumonia.     complication  
v Lymphadenopathy  below  the  diaphragm  is  rare  on  presentation     v Thromboembolic   phenomena-­‐   esp   those   w   /   hemolytic  
but  can  arise  with  disease  progression.     dsordrs,   myeloprolifertive   ,   splenomegaly-­‐portal   vein  
v The    spleen    is    often    an    occult    site    of    spread,    but    massive     thrombosis  
splenomegaly  is  not  common.     v Overwhelming  postsplectomy  infection:  
v In    addition,    large    spleens    do    not    necessarily    signify     o More   common   if   splenectomy   is   done   for  
involvement.   hematologic  problem  
v Four    major    histologic    types    exist:    lymphocyte    predominance     o Strep.  Pneumoniae-­‐most  common  org.(50-­‐90%)  
type,    nodular    sclerosis    type,    mixed    cellularity    type,    and     o Most  occur  2  years  after  splenectomy    
lymphocyte  depletion  type   o children,<5  years  and  adults  >50years  old  
v The    histologic    type,    along    with    location    of    disease    and      
symptomatology,  influence  survival  for  patients  with  HD.     Preventive  measures  against  OPSI  
v Stage  I  disease  is  limited  to  one  anatomic  region;  stage  II  disease     v Elective   splenectomy   in   children   should   postponed   if   possible   until  
is    defined    by    the    presence    of    two    or    more    contiguous    or     child  is  4-­‐10  years  old  
noncontiguous  regions  on  the  same  side  of  the  diaphragm;  stage     v Prophylactic  antibiotics  
III  disease  involves  disease  on  both  sides  of  the  diaphragm,  but     o  <  10  years  old.  For  5  years  after  splenectomy    
limited  to  lymph  nodes,  spleen,  and  Waldeyer's  ring  (the  ring  of     v Role  of  vaccination:  
lymphoid    tissue    formed    by    the    lingual,    palatine,    and     o Elective  splenectomy-­‐2  weeks  before  surgery  
nasopharyngeal  tonsils);  stage    IV  disease  includes  involvement     o Emergency  splenectomy-­‐should  be  given  ASAP  or  w/in  7-­‐
of    the    bone    marrow,    lung,    liver,    skin,    GI    tract,    or    any    organ    or     10  days  after  
tissue  other  than  the  lymph  nodes  or  Waldeyer's  ring.   o Annual  vaccination  
v Staging    laparotomy    for    HD    is    less    commonly    performed    in    the      
current  era  of  minimally  invasive  surgery  and     advanced  imaging    
techniques.      
v Current  indications  for  surgical  staging  include  clinical  stage  I  or    
II    disease    of    the    nodular    sclerosing    type    and    no    symptoms    
referable  to  HD.  
v The    surgical    staging    procedure    for    HD    includes  a    biopsy    of    the    
liver,    splenectomy,    and    the    removal    of    representative    nodes    in    
the  retroperitoneum,  mesentery,  and  hepatoduodenal  ligament.    
v An     iliac     marrow     biopsy     generally     is     included.     Unlike     in    
nonHodgkin's    lymphoma,    studies    have    concluded    that    surgical    
staging    has    altered    clinical    staging    in    as    many    as    42%  of    cases    
(26  to  37%  upgraded,  7  to  15%  downgraded).  
v Staging    information    affects    treatment,    because    patients    with    
early-­‐stage    disease    who    have    no    splenic    involvement    may    be    
candidates  for  radiotherapy  alone.    
v Those    with    splenic    involvement    generally    require    
chemotherapy  or  multimodality  therapy  
 
 

 
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