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CPC12-19Hon

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    Clinical Pathological

    Conference2004-12-29

    /

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    Case Presentation

    Present Illness

    This one-year-six-month-old boy came to our

    pediatric emergency department with thechief compliant of bilious vomitingand

    intermittent irritable cryingsince 4hours

    ago.

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    Case Presentation

    Personal and Family History

    Past history:

    Before this presentation,the child was ingood condition without any compliant of

    abdominal symptoms.

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    Case Presentation

    Radiologic & Lab Findings

    Plain film of abdomen : showed a nonspecificlocal ileuspattern over right upper quadrant.

    Laboratory data:

    white blood count:25300/nl (6000-17500/nl)

    with 75% segment(54-62%) and

    18% lymphocyte(25-33%).

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    Case Presentation

    Radiologic & Lab FindingsSerum biochemistry:

    Sodium :145 (139-146)mEq/L

    Potassium :4.5 (3.5-5.0)mEq/L

    Chloride : 130 (98-106)mEq/L

    BUN :19 mg/dl,

    Creatinene :0.4(0.2-0.4)mg/dl

    C reative protein level :0.1mg/dl.

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    Case Presentation

    Radiologic & Lab Findings

    Abdomen sonography :revealed a soft

    tissue mass over right lower quadrant,but noevidence of sign oftarget

    appearance; besides, the relationship of

    superior mesentery artery and superiormesentery vein was in rightposition.

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    Case Presentation

    Hospital Course-IITwelve hours later, heart rate increased to

    150-180 per minutes.

    Meanwhile, decreased urine output anddownhilled blood pressurehappened to him

    despite of intravenous fluid supplement.

    Under the impression of intestinal obstruction

    complicated with shock, our pediatric surgeon

    arranged emergency laparotomy.

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    Case Presentation

    Hospital Course-II

    Post-operative course was relative

    smooth,and started feeding on the4th

    dayof lapatotomy smoothly.

    His condition was stable during the

    follow-up period at our out patientclinics.

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    Major Problems Minor Problems

    Bilious vomiting

    A soft tissue mass

    over right lowerquadrant

    Downhilled blood

    pressure(shock)

    Intermittent irritablecrying

    Decreased urine

    output

    Leukocytosis

    Hyperchloremia

    Local ileus pattern

    over right upperquadrant

    Tachycardia

    Hypertension

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    Questionsabout past, personal, family

    history

    Birth history?

    History of trauma? Child abuse?

    Operation history? foreign body aspiration?

    Drug history? Food history?

    Family history about tumor? About cystic

    fibrosis?

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    Questions- about Physical

    Examination and laboratory dataAnemic conjunctiva? Lymphoadenopathy?

    RLQ soft tissue mass movable or non-

    movable?Bowel sound? Hyperactive to be replacedwith hypoactive bowel sounds?

    Stool rontine examination? occult blood?WBC? pattern?

    Blood smear? blast cell?

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    Questions-about radiologic finding

    Plain film of abdomen :further finding?Foreign body/Bezoars?

    Abdomen Sonography: further finding?Appendix? Ascites?Kidney? Any findingabout tumor?

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    Bilious vomiting

    Vomitus or nasogastric aspirate containing

    bile which in children almost alwaysindicates bowel obstruction distal to the

    sphincter of Oddi.

    By contrast, infants with pyloric stenosishave non-bile-stained vomiting.

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    Intestinal obstruction

    Intraluminal:polyp, mass, parasites,

    and tumor.Intramural:stricture, tumor,

    hematoma.

    Extrinsic:postoperative adhesion,adhesion from peritonitis, hernia,volvulus,and tumor.

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    Clinical Classification of ShockSeptic shock:bacterial,Viral,Fugal..

    Cardiogenic shock : ischemia,cardiomyopathy ,congestive heart failure

    Distributive shock:toxins,anaphylaxis

    Hypovolemic shock:enteritis,hemorrhage,

    Obstructive shock :tension pneumothroax

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    HyperchloremiaPathophysiology

    Metabolic Acidosis with a normal Anion Gap

    Causes

    Artifact (low Anion Gap)

    Metabolic and Endocrine

    Hyperparathyroidism, Renal Tubular Acidosis,Hypernatremia.

    Bromide intoxication

    Nervine, Sominex

    AcetazolamideCarbonic anhydrase inhibition

    Boric acid , Triamterene,Ammonium Chloride

    Excess IV Normal Saline

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    HyperchloremiaGastrointestinal

    Dehydration

    Prolonged DiarrheaLoss of pancreatic secretion

    Ileal loops

    Ureteral colonic anastomosis

    (From: Pediatric

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    Vomiting

    Chronic

    Acute

    History andphysical exam

    Sign or

    symptoms

    suggestive

    of increased ICP?

    Surgery

    Consult

    Malrotation with volvulus

    Appendicitis

    Other causes of intestinal obstrution

    Congenital structural abnormalities

    Postsurgical adhesions

    Foreign body/Bezoars

    Meckels diverticulum

    Incarcerated inguinal hernia

    Meconium ileus

    Intussusception

    Hirschsprungs disease

    Superior mesenteric artery syndrome

    Duodenal hematomaTesticular or ovarian torsion

    Sign or symptoms suggestive

    of increased ICP

    Signs or

    symptoms

    suggestive

    of an

    acute

    abdomen

    No

    Yes(From: PediatricDecision-makingStrategiesaccompanied byNelson)

    No

    Yes

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    Malrotation with volvulus

    Appendicitis

    Congenital structural abnormalities

    Postsurgical adhesions

    Foreign body/Bezoars

    Meckels diverticulum with bleeding

    Incarcerated inguinal hernia

    Meconium ileus

    Intussusception

    Hirschsprungs disease

    Superior mesenteric artery syndrome

    Duodenal hematoma

    Testicular or ovarian torsion

    Ab l

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    Abdomen

    mass

    Yes

    No

    History and

    physical exam

    Abdomen

    US

    Perform

    abdomen USNeonate?

    Perform

    Abdomen CT

    Yes(From: Pediatric Decision-makingStrategies accompanied by Nelson)

    Lower

    abdomen

    mass in

    female Normal result

    Abnormal

    result

    No

    Yes

    NoHepatomegalyor

    splenomegalypresent

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    Not neonate ,

    Not female with lower abdomen mass

    Splenomegaly Wilms tumorAdrenal cortical neoplasms

    Pancreatic masses/cysts

    Neuroblastoma Hydronephrosis

    RhabdomyosarcomaUrinary retention Hepatic lesion

    Teratoma Bezoar

    Appendiceal abscess Intestinal tumor

    Mesenteric cyst Omental cystLymphangioma Lymphoma

    Choledochal cyst Constipation

    Inflammatory bowel disease

    Retroperitoneal hematoma

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    Foreign body/Bezoars

    Meckels diverticulum

    Hirschsprungs disease

    AppendicitisIntestinal tumor

    Neuroblastoma

    Rhabdomyosarcoma

    LymphomaCongenital structural abnormalities

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    Foreign body/Bezoars (bezr)

    An accumulation of exogenous matter inthe stomach or intestine.

    peak incidence between the ages of 6 moand 3 yr

    90% of foreign bodies are opaque.

    vomiting, anorexia, and weight loss.

    An abdominal plain film may suggestthe presence of a bezoar, which can beconfirmed on ultrasound or CTexamination.

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    Gastric trichobezoarPediatric Emergency Care. 19(5):343-7, 2003 Oct.

    On plain abdominal radiographs, thebezoar will appear as a mottled

    heterogenous massthat may be mistakenfor a food-filled stomach.

    The classic sonographic appearance is

    described as a band of increasedechogenicityin the region of the stomachwith complete loss of posterior echoes.

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    Small bowel obstruction and covered

    perforationin childhood caused by bizarre

    bezoars and foreign bodies.

    Small bowel obstruction with

    perforationis an unusual and rarecomplication of bezoars.

    Israel Medical Association Journal: Imaj. 2(2):129-31, 2000 Feb.

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    Hirschsprungs disease

    Abnormal innervation of the bowel .

    Most common cause of lower intestinalobstructionin neonates .

    Usually begin at birthwith the delayed passageof meconium.

    Some infants pass meconium normallybutsubsequently present with a history of chronicconstipation.

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    Hirschsprungs disease

    Failure to thrive, with hypoproteinemia

    from a protein-losing enteropathy, is a

    less common presentation.

    Rectal examinationdemonstrates normal

    anal tone and is usually followed by anexplosive dischargeof foul-smelling

    feces and gas.

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    Hirschsprungs disease

    Rectal manometryand rectal suction biopsyare the easiest and most reliable indicators ofHirschsprung disease.

    Barium enemaexamination is useful indetermining the extent of aganglionosis.

    Sonography may also help in determining thedynamic or adynamic state of fluid-filled orsolid-filled bowel loops.

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    Meckels diverticulum

    Remnant of the embryonic yolk sac

    Arise within the 1st 2 yr of life .

    Intermittentpainless rectal

    bleedingby ulcerationof the

    adjacent normal ileal mucosa.

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    Meckels diverticulum

    Brick colored or currant jelly colored.

    Obstructionoccurs when the diverticulumacts as the lead point of an intussusception.

    A Meckel diverticulum may occasionallybecome inflamed(diverticulitis) and present

    similarly to acute appendicitis.The most sensitive study is a Meckelradionuclide scan

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    Meckel's diverticulum.

    Internal hernia and adhesions without gastrointestinal

    bleeding--ultrasound and scintigraphic findings.

    US study was particularly helpful in this

    case because it shows a nonperistaltic

    region, which is consistent with a

    diverticulumor an internal hernia.

    Clinical Nuclear Medicine. 21(12):938-40, 1996 Dec.

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    Meckel's diverticulummimicking infantile colic:

    sonographic detection.

    Abdominal sonography at 6 months of agedemonstrated an abdominal masswith an

    anechoic center and a double-layered wall,surrounded by bowel loops.

    Histologic examination of the resected massrevealed a Meckel's diverticulumwith a

    perforationsealed off by the neighboring boweland mesenteryto form an inflammatory mass.

    Journal of Clinical Ultrasound. 28(6):314-6, 2000 Jul-Aug

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    Gastrointestinal bleeding in infants and children:

    Meckel's diverticulum and intestinal duplication.

    Seminars in Pediatric Surgery. 8(4):202-9, 1999 Nov.

    Meckel's diverticula and intestinal

    duplications may cause

    gastrointestinalbleedingin almostany age group and require a high

    index of suspicion for diagnosis.

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    AppendicitisThe risk ofperforationis greatest in1- to 4-

    yr-old children (7075%) and is lowest in

    the adolescent age group (3040%) .

    The classic triad consists of pain, nausea

    with vomiting, and fever.

    The progression from onset of symptoms to

    perforation usually occurs over 3648hr.

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    Appendicitis

    History included onset of pain beforevomiting or diarrhea, loss of appetite,

    migration of painfrom periumbilical toright lower quadrant.

    Auscultation may reveal normal or

    hyperactivebowel soundsin earlyappendicitis, to be replaced withhypoactivebowel soundsas it progressesto perforation.

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    Appendicitis

    Findings of appendicitis on abdominal filmsinclude calcified appendicolith, small boweldistention or obstruction, and soft tissue mass

    effect.Graded compression ultrasonographyis anoninvasive study with false-negative and false-

    positive rates of 810% .

    CT is more sensitive and specificthanultrasonographyand more likely to changepatient management.

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    Pediatric appendicitisin "real-time": the value of

    sonographyin diagnosis and treatment.

    Pediatric Emergency Care. 17(5):334-40, 2001 Oct.

    The natural progression in appendicitis

    from initial symptoms to perforation isabout 36 to 48hours . However,perforation may occur more rapidly in theyounger child, sometimes within 6 to 12hours .

    Extensive necrosisof the appendix mayrender it difficult to visualize .

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    Pediatric appendicitisin "real-time": the value of

    sonographyin diagnosis and treatment.Pediatric Emergency Care. 17(5):334-40, 2001 Oct.

    We may have to rely on the otherultrasound features of peri-appendiceal

    inflammation.

    Studies have shown that the presence ofloculated pericecal fluid,prominent

    pericecal fat, atonic bowel loops, thickenedbowel walls,and the circumferential lossof the appendiceal submucosal layer onultrasound were the significant predictivefactors forperforation.

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    Distal Intestinal Obstruction Syndrome

    In the older child or young adultwith CF,

    the distal small bowel may by obstructedby thick stool. This condition was called"meconium ileus equivalent" by Jensen in1962 .

    Palpable mass in the right lowerabdominalquadrant. Bilious vomitingas a result ofthe intestinal obstruction

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    Distal Intestinal Obstruction Syndrome

    Radiographs of the abdomendemonstrate dilated small bowel

    loops and abubbly ileocecal soft-

    tissue mass .

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    Carcinoid tumor

    About 85% of carcinoid tumors develop in thegastrointestinal tract, usually theappendix.

    Carcinoid syndrome:flushing,diarrhea,wheezing.

    Carcinoid crisis:generalized flush, tachycardia,severe diarrhea with abdominal pain,hypotension converting to hypertension, andcentral nervous system changes leading tocoma and then death.

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    Carcinoid tumor

    Approximately 40% of the tumors occurred

    within 2 feet of the ileocecal valve, with very

    few in the proximal small intestine.

    These tumors frequently elicit a mesenteric

    fibrosing reaction, in which the bowel

    becomes shortened and kinked, frequentlycausingpartial small bowel obstruction.

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    Carcinoid tumor

    On CT, the mesenteric extension from

    carcinoid will usually appear as a soft

    tissue-densitymesenteric mass .

    Calcificationcan be seen in up to 70% of

    cases .

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    Lymphoma

    from manual of pediatric hematology and oncology ,3rd

    edition

    Non-Hodgkins lymphoma:

    peak age 5-15years,rick factor includinggenetic and poettransplantationimmunosuppression.

    Clinical feature:Head and neck(13%),

    medicatinum(26%),abdomen(35%).

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    Non-Hodgkins lymphoma

    The ileumis mostly involved due to ahigher number of lymphocytes in the distal

    gut, accounting for about 50% of smallbowel lymphomas

    Present with abdomen pain, vomitingand

    diarrhea, abdominal distension, palpablemass, intussusception,peritonitis, ascites,GI bleeding, hepatosplenomegaly.

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    Multidetector-row computed tomography and 3-dimensional

    computed tomography imaging of

    small bowel neoplasms: current concept in diagnosis.

    Lymphoma can appear as a single mass lesion,which varies in size.These can lead tointussusception, but rarely will result inobstructionbecause the masses are typically

    pliable and soft.

    Again, because the masses are characteristicallysoft, it is rare that the mesenteric vasculature iscompromised.

    Journal of Computer Assisted Tomography. 28(1):106-16, 2004 Jan-Feb.

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    Neuroblastoma

    from manual of pediatric hematology and oncology,3rdeditionGive rise to adrenal medulla and thesympathetic ganglia.

    Most common tumor in infancy ,peakincidence is 2 yearsof age

    Clinical finding related to anatomic site ofabdomen :anorexia ,vomiting,abdomenpain,massive involvement of theliverwithmetastasis (especially in the newborn)

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    Neuroblastoma

    Paraneoplastic manifestations :

    excessive catecholamine secretion

    (sweating,flushing, paller,palpitation,hypertension),VIP secretion (watery

    diarrhea,abdomen distension,

    hypokalemia) ,and acute myoclonicencephalopathy.

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    NeuroblastomaGrainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging,

    4th ed.,

    A neuroblastoma is usually solid with aheterogeneous echotexture.Calcificationis

    evident by the presence of echogenic fociwith posterior acoustic shadowing.

    Anterior displacement and encasement ofthe aorta and inferior vena cava(IVC) bythis retroperitoneal tumour is characteristic.

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    Rhabdomyosarcomafrom manual of pediatric hematology and

    oncology,3rdeditionTwo age peaks:2-6 yearsand 15-19 years.

    Rare primary sites for rhabdomyosarcoma

    include the GI-hepatobiliary tract(3%),

    where in presents with obstructive

    jaundice and a large abdomen mass.

    These tumors arise in the common bileductand may extend into both lobes of the

    liver.

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    Final diagnosis

    1.Meckels diverticulum with

    diverticulitis or congenital structuralabnormalities

    2.Ruptured Appendicitis

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    THANK YOU!!

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    Mesenteric cyst / Omental cyst

    They ranged in age from 1 month to 14years;75% were younger than 5 years.

    The main presenting symptom is abdominalpain, followed by nausea and vomiting.

    Some mesentericcysts may present as anacute abdomen due to a possiblecomplication, such as hemorrhage, rupture,or torsionof the cyst.

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    Mesenteric cyst / Omental cyst

    Mesenteric cysts in children Surgery 1994;115:571-7

    Acute symptoms are related to compression

    of intra-abdominal organsor stretching of

    the mesentery by rapid expansion.

    Among these categories, the cystic

    lymphangiomais differentiated from theothers because it is far more common in

    children.

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    Lymphangioma

    Five pathologic patterns account for mostmesenteric cysts, namely,lymphangioma,enteric duplication cysts, enteric cysts,

    mesothelial, and nonpancreaticpseudocysts.

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    50 mg/dl(6.3 mEq/l)

    164 mgdl (50 mgdl)2004; 54: 189-93

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    2003.12.27

    Boricacid

    205

    http://www.infotimes.com.tw/http://www.infotimes.com.tw/
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    Leiomyoma of the smallbowel with hypercalcaemia:presence of a substance withparathormoneactivity

    Nouvelle Presse Medicale. 8(40):3245-6, 1979 Oct 22.

    A leiomyoma of the smallbowel producedlaboratory features ofhyperparathyroidism which

    disappeared promptly after tumour resection.

    Hypercalcaemia, hypophosphatemia,hyperchloremia, elevated chloride/phosphorusratio, increased urinary cyclic AMP, and blood

    levels of immunoreactive parathormone werepresent.

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    Retroperitoneal hematoma

    In the majority of cases there is an

    associated pathological condition of a

    viscus or vessel due to trauma, aneurysm,

    atherosclerosis, eroding primary or

    secondary tumors, or pancreatitis.

    Furthermore, some have stated thathemophilia and anticoagulanttherapy may

    be the cause in some cases.

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    Retroperitoneal hematoma

    These patients usually present with mild tosevere abdominal pain, nausea and

    vomiting.Physical examination reveals signs ofshock, ileus and flank mass.

    CT scansare used usually to establish thediagnosis of retroperitoneal hematoma.

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    Bilious vomitingintermittent irritable

    crying

    Dehydration

    Hypertension

    Leukocytosis

    Hyperchloremia

    Soft tissue massover RLQ by

    Abdomen sonography

    Decreased urine outputand downhill BP

    in 16 hours

    one-year-six

    month-old boy

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    Wilms tumor

    It may be associated with hemihypertrophy,aniridia,and other congenital anomalies,usually of the genitourinary tract.

    Sign of Wilms tumor:palpable mass inabdomen(60%),hypertension(25%),hematuria(15%),abdomen mass is the mostcommon presenting symptom and

    sign ,occasionally there is abdomenpain,especially whenhemorrhageoccurs inthe tumor following trauma.

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    Wilms tumor

    Some patients may present withabdominal pain and vomitingand,infrequently, hematuria.

    Occasionally, rapid abdominalenlargementand anemia may occurowing tobleeding into the renalparenchymaor pelvis.

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    Wilms tumorGrainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging,

    4th ed.,

    On ultrasound examination the tumourappears as a well-defined, solid massofmixed echogenicity.Areas of haemorrhageand necrosis can produce focal hypoechoic

    lesions within the mass.

    Ultrasound may be used for periodicsurveillance of the opposite kidney.

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    Superior mesenteric artery syndrome

    The classic example is anadolescentwhostarts vomiting after application of a body castfor orthopedic surgery. Other associated

    factors include anorexia, prolonged bed rest,weight loss, abdominal surgery, andexaggerated lumbar lordosis.

    The diagnosis is established radiologicallywith the demonstration of a cutoff of theduodenumjust to the right of the midline.

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    Superior mesenteric artery syndrome

    An extrinsic compression of the duodenumin children after rapid weight lossand in a

    supine position.

    The compression is thought to occur as themesentery loses its fatand allows the

    superior mesenteric artery to collapse on theduodenum, compressing it between thesuperior mesenteric artery anteriorly and theaorta posteriorly.

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    Inflammatory bowel disease

    The most common time of onset of IBD isduring adolescence and young adulthood. A

    bimodaldistribution has been shown with an

    early onset at 1525 yrof age and a secondsmaller peak at 5080 yrof age.

    Nonetheless, IBD may begin as early as the1st yr of life.

    In developed countries, these disorders are themajor causes of chronic intestinalinflammationin children beyond the 1st fewyr of life.

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    Ulcerative colitis

    Bloody stool and diarrheaare the typical

    presentation of ulcerative colitis. Constipation may

    be observed in those with proctitis.

    Fever, severe anemia, hypoalbuminemia,

    leukocytosis, and greater than five bloody stools per

    day for 5 days is what defines fulminant colitis.

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    Ulcerative colitis

    Extraintestinal manifestationsthat

    tend to occur more commonly with

    ulcerative colitis than with Crohn

    disease includepyoderma gangrenosum,

    sclerosing cholangitis, chronic active

    hepatitis, and ankylosing spondylitis.

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    Crohns disease

    Crohn colitis may be associated with

    bloody diarrhea, tenesmus, and urgency.

    Children with Crohn disease often appear

    chronically ill, weight losslinear growthretardation.

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    Crohns disease

    The initial presentation most commonlyinvolves ileum and colon (ileocolitis)but

    may involve the small bowel alone inabout 30% or colon alone in 10%15%.

    Children with ileocolitis typically havecramping, abdominal pain, anddiarrhea,sometimes with blood. Ileitismay present as right lower quadrantabdominal painalone.

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    Crohns disease

    There may be abdominal tenderness that is

    either diffuse or localized to the right

    lower quadrant.

    The diagnosis of Crohn disease depends

    on finding typical clinical features of the

    disorder (history, physical examination,laboratory studies, and endoscopic or

    radiologic findings).

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    Crohns disease

    Plain films of the abdomen may be normalor may demonstrate findings of partial

    small bowel obstructionorthumbprintingof the colon wall.

    An upper gastrointestinal contrast studywith small bowel follow-through may

    show aphthous ulceration and thickened,nodular foldsas well as narrowing of thelumen anywhere in the gastrointestinaltract.

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    Duplication

    Duplications can be classified into threecategories: localized duplications,

    duplications associated with spinal corddefects and vertebral malformations, andduplications of the colon.

    Duplications may cause bowelobstructionby compressing the adjacent

    intestinal lumen, or they may act as thelead point of an intussusception or a sitefor a volvulus.

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    If they are lined by acid-secreting mucosa,

    they may cause ulceration, perforation,

    and hemorrhageof the adjacent bowel.

    Patients may present with abdominal pain,

    vomiting, palpable mass, or acutegastrointestinal hemorrhage.

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    Radiologic studies such as barium studies,ultrasonography, CT, and MRI are helpful but

    usually nonspecific, demonstrating cysticstructuresor mass effects.

    Radioisotope technetiumscanning may localizeectopic gastric mucosa.

    The treatment of duplications is surgicalresectionand management of associated defects.

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    Atypical presentation of an intestinal

    duplicationin a three month old childJournal de Radiologie. 85(6 Pt 1):773-5, 2004 Jun.

    Intestinalduplicationis an uncommoncongenital anomaly that often is diagnosed during

    childhood.Ultrasound diagnosis is based on the presence of acharacteristic double-walled cystic mass.

    We report a case of duplicationin a three Monthold childpresenting with small bowel obstruction.

    I t ti l d li ti ti

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    Intestinalduplicationpresenting as

    spontaneous hemoperitoneum.

    Journal of Pediatric Gastroenterology & Nutrition.31(2):181-2, 2000 Aug.

    In approximately 60% of the cases,

    the condition appears during the firstyear of lifeas a palpable abdominal

    massor as complications such as

    intestinal obstruction due to extrinsic

    compression, volvulus, or

    intussusception.

    I t ti l d li ti ti

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    Intestinalduplicationpresenting as

    spontaneous hemoperitoneum.

    Journal of Pediatric Gastroenterology & Nutrition.31(2):181-2, 2000 Aug.

    It was probably caused by erosion of

    a blood vessel adjacentto theperforation that had been sealed off at

    laparotomy.

    Sudden infant death, large intestinal

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    , g

    volvulus, and a duplicationcyst of the

    terminal ileum.American Journal of Forensic Medicine &

    Pathology. 21(1):62-4, 2000 Mar.

    If an intussusception or volvulus is

    identified, careful search for predisposing

    lesions or conditions such as duplication

    cysts, mesenteric cysts, mesenteric defects,

    Meckel's diverticula, mesenteric lymph

    nodes, polyps, neoplasms, mural

    hematomas, or cystic fibrosis should also

    be undertaken.

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    Jejunal and Ileal Atresia and

    Obstruction

    Jejunoileal atresias have been

    attributed to intrauterine vascularaccidentsleading to ischemic

    necrosis of the sterile boweland

    resorption of the affected segments.

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    Most infants become symptomatic

    during the 1st day of lifewith

    abdominal distention and bile-

    stained emesisor gastric aspirate.

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    Plain radiographs demonstrate many air-

    fluid levels or peritoneal calcification

    associated with meconium peritonitis.

    In meconium ileus, plain films of the

    abdomen show a typical hazy or ground-

    glassappearancein the right lowerquadrant.

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    Pneumoperitoneum is most readily seen

    as free air between the liver and the

    diaphragmon an upright radiograph of theabdomen; if there is a large amount of free

    air, the entire abdomen may look like a

    football from distention with air; the

    ligamentum teres is sometimes clearly

    visible in the midline.

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    Malrotation

    Incomplete rotation of the intestine during fetaldevelopment

    The majority of patients present within the 1st yrof lifewith symptoms of acute or chronicobstruction. Infants often present within the 1stwk of life with bilious emesis and acute bowelobstruction.

    An acute presentation of small bowel obstruction

    in a patient without previous bowel surgery isusually a result of volvulusassociated withmalrotation.

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    The abdominal plain film is usually nonspecific

    but may demonstrate evidence of duodenalobstruction with a double-bubble sign.

    Barium enema usually demonstrates malpositionof the cecumbut may be normal in 10% of

    patients.

    Upper gastrointestinal series demonstratesmalposition of the ligament of Treitz.

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    Ultrasonographydemonstratesinversion

    of the superior mesenteric artery and

    vein.A superior mesenteric vein located tothe left of the superior mesenteric artery is

    suggestive of malrotation.

    Surgical intervention is recommended forany patient with a significant rotational

    abnormality, regardless of age.

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    Intussception

    A portion of the alimentary tract is telescopedinto an adjacent segment.

    The most common cause of intestinalobstructionbetween 3 mo and 6 yr of age.

    Sixty per cent of patients are younger than 1 yr,and 80% of the cases occur before 24 mo; it israre in neonates. The male:female ratio is 4:1.

    Most intussusceptions do not strangulate thebowel within the first 24hrbut may latereventuate in intestinal gangrene and shock.

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    Sudden onset, severe paroxysmal colicky pain

    Vomiting occurs in most cases and is usuallymore frequent early.

    60%of infants pass a stool containing red bloodand mucus, the currant jelly stool.

    Tender sausage-shaped mass,which mayincrease in size and firmness during a paroxysmof pain and is most often in the right upperabdomen,with its long axis cephalocaudal.Plain abdominal radiographs may show a densityin the area of the intussusception.

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    Neonate with abdomen mass

    Neuroblastoma Congenital Hydronephrosis

    Multiple cystic kidney

    Infantile polycystic kindey disease

    Neurogenic bladder Renal vein thrombosis

    Collecting system duplication

    Intestinal duplication Sacrococcygeal teratomaAdrenal hemarrhage Mesoblastic nephroma

    Pancreatic cyst Hepatoblastoma

    Meconium ileus Hematoma(hepatic,splenic)

    Magacolon(obstruction)

    Anterior myelomenihgocele

    Appendiceal abscess Intestinal tumor

    Mesenteric / Omental cyst Choledochal cyst

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    Neuroblastoma

    Congenital Hydronephrosis

    Multiple cystic kidney

    Infantile polycystic kindey disease

    Neurogenic bladderRenal vein thrombosis

    Collecting system duplication

    Intestinal duplication

    Sacrococcygeal teratoma

    Adrenal hemarrhage

    Mesoblastic nephromaPancreatic cyst

    Hepatoblastoma

    Meconium ileus

    Hematoma(hepatic,splenic)

    Magacolon(obstruction)

    Anterior myelomenihgocele

    Appendiceal abscess Intestinal tumor

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    Renal Vein Thrombosis

    In newborns and infants, RVT is commonlyassociated with asphyxia, dehydration,

    shock, sepsis,and infants born to motherswith diabetesmellitus.

    Sudden onset of gross hematuria and

    unilateral or bilateral flank masses,microscopic hematuria, flank pain,hypertension, or oliguria.

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    Renal Vein Thrombosis

    RVT is usually unilateral. Bilateral RVTresults in acute renal failure.Most patients

    also have a microangiopathic hemolyticanemiaand thrombocytopenia.

    Ultrasonographyshows markedenlargement, whereas radionuclide studies

    reveal little or no renal function in theaffected kidney(s).

    Doppler flow studies of the inferior venacava and renal veinconfirm the diagnosis.

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    The advent of technetium (Tc) 99mpertechnetate radionuclide scanning has

    greatly facilitated the diagnosis ofMeckel's diverticula and may also beuseful for intestinalduplications. A

    positive scan requires the presence of

    ectopic gastric mucosa, which may beidentified in both Meckel's diverticula andintestinalduplications.

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    The significance of ectopic gastric mucosais that it contains acid-secreting parietal

    cells, which may cause ulceration andbleeding. Only rarely are intestinalduplications diagnosed preoperatively.After initial fluid resuscitation, bleeding

    from Meckel's diverticula and intestinalduplications require surgical intervention.Resection is the treatment of choice.

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    A barium enema shows a filling defector cuppingin the head of barium where its advance isobstructed by the intussusceptum (coiled-springsign) .

    Ultrasonographyis a sensitive diagnostic toolin the diagnosis of intussusception.

    The diagnostic findings of intussusception

    include a tubular massin longitudinal views anda doughnut or targetappearance in transverseimages .

    P i i l f h i h

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    Posttraumatic retroperitonealrupture of the right

    colon simulating a retroperitonealhematoma.

    Journal of Trauma-Injury Infection & Critical Care. 42(4):741-2, 1997 Apr.This case illustrates the diagnostic

    problems encountered in a patient withposttraumatic retroperitonealabscess

    caused by perforation of the posteriorwall of the cecum,simulating aretroperitonealhematoma.

    Blunt colonic injuries are rare and difficult

    to diagnose. Septic signs are unexpected incase of posttraumatic retroperitonealhematomaand should suggest thediagnosis of retroperitonealcolonic

    perforation.

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    Incarcerated inguinal hernia

    An inguinal hernia appears as a bulge in theinguinal regionthat extends toward and possiblyinto the scrotum.

    The hallmark signs of an inguinal hernia onphysical examination are a smooth, firm massthat emerges through the external inguinal ringlateral to the pubic tubercleand enlarges withincreased intra-abdominal pressure.

    A quiet infant can be made to strain theabdominal muscles by stretching out supine onthe bedwith legs extended and arms held straightabove the head. Most infants struggle to get free,thus increasing the intra-abdominal pressure and

    pushing out the hernia.

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    Incarcerated inguinal hernia

    The infant or child with an

    incarcerated inguinal herniais

    likely to have associated findings

    suggestive of intestinal obstruction

    such as abdominal distention,

    vomiting, and multiple air-fluidlevels evidenton plain radiographs.

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    Wilms tumor

    The incidence is approximately 8cases/million children younger than15 yr of age.

    It usually occurs in children between25 yr of age,although it has also been

    encountered inneonates, adolescents,and adults.

    Neonatal intestinalperforation caused by congenital

    d f t f th ll i t ti l l t t

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    defect of the small intestinal musculature: report

    of one case.

    Acta Paediatrica Taiwanica. 40(4):271-3, 1999 Jul-Aug.

    Congenital defect of the small intestinalmusculature is arare cause of neonatal spontaneous intestinalobstructionor

    perforation.

    Histology examination demonstrates multifocal deficiencyof the inner circular muscle layer three cm around the

    perforation site. The clinical and histological characteristicsare reviewed and discussed. We propose that the muscledefect of small intestine, especially ileum, is secondary toischemic injury rather than an embryological malformation.