III-2b Pediatric Surgery Part 2

7/27/2019 III-2b Pediatric Surgery Part 2

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ABDOMINAL WALL DEFECTS

Gastroschisis

Greek Word: Belly Cleft An abdominal wall defect latera l to the umbilicus usually contains

small bowel and has no surrounding membrane

Defect almost always to the right of intact umbilical cord, and in somecases separated from the cord by an intact skin bridge

Anomaly probably results from defect that occurs at the site where thesecond umbilical vein involutes.

Embryonic Origin

Embryology of abdominal wall defect: Previously believed to be a result of failure closure of the

abdominal wall

A result of a vascular accident during embryogenesis (intra-uterine occlusion of right omphalo-mesenteric artery)

Failure of the formation of the umbilical coelom Congenital weakness of the right side of the umbilical cord - - -

RuptureEpidemiology

Most common congenital abdominal wall defect More common than omphalocoele (1.5 to 2:1) Both sexes are equally affected No genetic, geographic or racial predilection Associated with:

o young mothers (


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Omphalocoele

! Abnormality that occurs before b irth as a fetus is forming in its mother'suterus.

! First trimester of pregnancy! Some of the abdominal organs protrude through an opening in the

abdominal wall in the area of the umbilical cord covered by atranslucent membrane (sac)

What causes an omphalocoele?! 6th - 10thweek of pregnancy:

! the intestines actually project into the umbilical cord as theyare growing.

! 11th week of gestation:! the intestines should return to the abdomen! the abdominal muscles should then meet in the middle and

grow together and thus closing off this opening! Omphaloceleoccurs when the abdominal organs do not return to the

abdominal cavity as they should.

Clinical Findings in infants withAbdominal Defects

Factor Omphalocele Gastroschisis

Location Umbilical ring Lateral to cord

Defect Size Large (2-10 cm) Small (2-4 cm)

Cord Inserts in sac Normal insertion

Sac +/- -

Contents Liver and Bowel Bowel and GonadsGI function Normal Prolonged ileus

Associated Anomalies Common (30-70%) Rare

Syndromes Associated with Omphalocoele:! Beckwith-Wiedemann Syndrome

" Macroglossia" Hyperinsulinism" Omphalocoele

! Cantrells Pentalogy" Epigastric omphalocele" Cleft sternum" Anterior diaphragmatic hernia (Morgagni)" Cardiac defects (eg, ectopia cordis,VSD)" Absent pericardium

! Karyotype Anomalies:" 30% of affected infants" More common in those with small defects" Trisomies 13 & 18" Trisomy 21 (less frequently)

! Other associated anomalies:" Cardiac (most common)" Musculo-skeletal" Gastro-intestinal" Genito-urinary

Risk factors!

Maternal illness and infection! Drug use, smoking, and alcohol! Genetic abnormalities! Folic acid deficiency, hypoxia, and salicylates (experimental rats)! 40 years or older

Diagnostics! Prenatal UTZ:

" Inexpensive, safe and noninvasive" Done in real-time and is widely available." Remains the imaging modality of choice for the prenatal

assessment of the fetus." In experienced hands, UTZ is highly accurate in the

diagnosis of most complications associated with pregnancy.

" Can be used as a guide to intervention in pregnancy." Its greatest advantage is that it can be quickly and

frequently repeated as required with minimal risk on thepatient and the fetus

" A definitive diagnosis of omphalocele is possible onlybeyond 12 weeks' gestation (first trimester)

" The mass consists of abdominal contents that haveherniated through a midline central defect at the base ofumbilical cord insertion.

" Mass has a smooth surface and contains abdominalviscera, usually the liver including the bowel and stomach

! Maternal Serum alpha-fetoprotein (MSAFP)" Prenatal diagnosis of abdominal wall defects can be made

by detection of an elevation in MSAFP" MSAFP levels are greater in gastroschisis than in

omphalocele" MSAFP also is increased in spina bifida, which additionally

increases the ratio of acetylcholinesterase andpseudocholinesterase

Surgical Treatment:! SMALL omphalocele ( epithelialization o

the Alloderm patchPrognosis

! Mortality is related directly to the severity of coexisting defects andpresence of chromosomal conditions inconsistent with survival.(Cantrells Pentalogy: 75% mortality)

Groin Hernias


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Inguinal Hernia

An indirect inguinal hernia protrudes through the internal inguinal ringsupero-lateral to the inferior epigastric vessels, within the cremastericfascia and extends down the spermatic cord for varying distances

A direct inguinal hernia protrudes through the posterior wall of theinguinal canal infero-medial to the inferior epigastric vessels,destroying or stretching the transversalis fascia

The typical patient:o Presents with an intermittent lump or bulge in the groin

which may extend towards the scrotum or labiao Most notably noticed at times of increased intra-abdominal

pressure Are almost exclusively indirect type (3-10%) Direct inguinal hernia in children are rare:

o Usually associated with an indirect inguinal herniao Caused by:

! Previous surgery secondary to floor disruption! Connective tissue disease (Marfans, Ehlers-

Danlos and Hunter-Hurler syndromes)! Increased intra-abdominal pressure

Male: Female (8:1 to 10:1); Right sided (2x more often) High risk:

o Prematurityo Low Birth Weight

Management

Elective Herniotomy:o at the earliest convenient timeo simple high ligation of the sac required

Bilateral Exploration (Controversial):o Female (4-6 y.o) and Male (18 hrs of life

Types:! High type (> 1cm from anal dimple) = Colostomy! Low type (< 1cm from anal dimple) = Anoplasty

Associated Anomalies:! VACTERL Syndrome

o V = Vertebral anomalies; ando A = Anal atresia (no hole at the bottom end of the intestineo C = Cardiac defect, most often ventricular septal defect;o TE = TracheoEsophageal fistula (communication between

the esophagus and trachea) with esophageal atresia (part the esophagus is not hollow);

o R = Renal (kidney) abnormalities; ando L = Limb abnormalities, most often radial dysplasia

(abnormal formation of the thumb or the radius bone in theforearm).

Definitive Tx:o Posterior sagital anorectoplasty (PSARP)o Cutbacko Anal transposition

Persistent Cloaca! In females, when the urethra, vagina, and anus all go out

through one opening

Other treatmnents:! Distal colonogram! Colostomy

Hirschsprungs Disease

Absence of ganglion cells in the colon Presentation:

! Acute/chronic obstruction:! Vomiting! Obstipation/Constipation! Abdominal Distention

! No passage of meconium (24-48hrs of life)! Rectal exam shows sudden gush of air and feces

1:5000 live births Absent ganglion cells !ineffective peristalsis!functional bowel

obstruction

Limited to rectosigmoid in majority Failure to pass meconium within 48 hrs Non-specific sxs in older infant and child:

! Episodic abdominal distention, overflow diarrhea, severeconstipation

Diagnosis:

Contrast enema!narrowed rectum with proximal bowel dilation Transition zone may be absent in infants due to underdeveloped

haustra

Retained colonic barium at 24 hrs ispathognomonicDefinitive Diagnosis:

Genetic markers exist as research tools in familial cases; not forgeneral use

Anorectal manometry possib le: "resting pressure, absent anorectalinhibitory reflex

Pathologic confirmation necessary:! Suction mucosal or full thickness rectal biopsy! Absent ganglion cells, thickened nerve trunks, + ACE


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Operative Management:

Traditional:! Temporary colostomy above aganglionic segment at time of

dx

! Second stage pullthrough procedure (6 to 12 mos later) Contemporary:

! Trend to avoid colostomy; Repair within days to weeks ofdx

Key:! Evacuate colon with rectal dilations, tube decompression,saline washouts

Trans-anal pull-through Swensons pull-through Endorectal pull-through

Contemporary Surgical Options

Open neonatal pullthrough via lower midline incision Laparoscopic assisted pullthrough with varied port site incisions Transanal pullthrough... Incision-less procedure (endorectal/full-

thickness)

All procedures:!

reduces need for multiple operations! Reduces morbidity! Reduces operative cost of stoma

Earlier postoperative feeding / hospital dischargeHirschsprungs Enterocolitis

! Majorcause of morbidity/mortality! Commonly misdiagnosed as gastroenteritis! No known etiology or effective prevention! Occurs in 1/3 of patients! Presenting sx: 7.7% Postop: 21%! Higher incidence of enterocolitis associated with:

" early dx (especiallyfirst week of life)" definitive repair


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! Associated with:" Prematurity" polyhydramios

Management! Duodenal:

" Duodenoduodenostomy" Excision of web

! Jejuno-ileal and colonic:" Resection or tapering of bulbous end" Bypass procedure (Duodeno-jejunostomy, jejuno-

jejunostomy)

Necrotizing Enterocolitis

! An ischemic disorder of the intestine in the newborn! Although more common in premature infants, it can also be observed

in term babies! Typically occurs in the second to third week of life in the infant who is

premature and has been formula fed

Etiology! No specific etiology of NEC has been identified! Multiple factors are believed to contribute to its development! The principal factors identified are:

! PREMATURITY! EARLY ENTERAL FEEDING

Clinical Presentation! Abdominal distention! Frank or occult blood in stools! Vomiting! Diarrhea! Feeding intolerance! Visible intestinal loops! Palpable abdominal mass! Erythema in the abdominal wall

Diagnosis! Leukopenia, thrombocytopenia, low hematocrit, low serum sodium

levels, metabolic acidosis and coagulation defects! Abnormal radiographs:

! Distended and edematous intestines; intramural air; portalvein gas; isolated persistent distended loop of bowel; freeperitoneal air suggesting perforation

! Radiograph demonstrates multiple dilated loops in the large bowel andsmall bowel. Note pneumatosis intestinalis with bubbly and linear gascollections in the bowel wall

! Paracentesis may be performed for infants with ascites! A positive finding on paracentesis with the free flow of at

least 0.5 mL of brownish fluid that contains bacteria onGram stain is highly specific for intestinal necrosis.

Objective Staging Criteria(developed by Bell)! Bell stage I: Suspected disease

! Stage IA:! Mild nonspecific systemic signs such as apnea,

bradycardia, and temperature instability arepresent.

! Mild intestinal signs such as increased gastricresiduals and mild abdominal distention arepresent.

! Radiographic findings can be normal or canshow some mild nonspecific distention.

! Treatment is NPO with antibiotics for 3 days.! Stage IB:

! Diagnosis is the same as IA, with the addition ofgrossly bloody stool.

! Treatment is NPO with antibiotics for 3 days.

! Bell stage II: Definite disease! Stage IIA:

! Patient is mildly ill.! Diagnostic signs include the mild systemic sign

present in stage IA.! Intestinal signs include all of the signs present i

stage I, with the addition of absent bowel soundand abdominal tenderness.

! Radiographic findings show ileus and/orpneumatosis intestinalis. This diagnosis issometimes referred to colloquially as medicalNEC.

! Treatment includes NPO and antibiotics for 7-1days.

! Stage IIB:! Patient is moderately ill.! Diagnosis requires all of stage I signs plus the

systemic signs of moderate illness, such as mildmetabolic acidosis and mild thrombocytopenia.

! Abdominal examination reveals definitetenderness, perhaps some erythema or otherdiscoloration, and/or right lower quadrant mass

! Radiographs show portal venous gas with orwithout ascites.

! Treatment is NPO and antibiotics for 14 days.! Bell stage III:Represents Advanced NECwith severe illness that ha

a high likelihood of progressing to surgical intervention! Stage IIIA:

! Patient has severe NEC with an intact bowel.! All of the above conditions with the addition of

hypotension, bradycardia, respiratory failure,severe metabolic acidosis, coagulopathy and/oneutropenia.

! Marked distention with signs of generalizedperitonitis.

! Radiographic examination reveals definitiveevidence of ascites.

! Treatment involves NPO for 14 days, fluidresuscitation, inotropic support, ventilatorsupport, and paracentesis.

! Stage IIIB! This stage is reserved for the severely ill infantwith perforated bowel observed on radiograph.! Free air visible on abdominal radiograph

indicates surgery

Indications for Operative Intervention! The most compelling predictor of intestinal necrosis indicating a need

for operative intervention is pneumoperitoneum! Erythema and sclerema in the abdominal wall, positive paracentesis,

persistent bowel loop on plain film, persistent abdominal mass on aparticular location

! Abdominal tendernessContraindications

! Patients with stage I or stage II disease, for whom nonoperativemedical therapy is the treatment of choice

! Critically ill newborns with a relative contraindication to formal operativexploration may be treated with the placement of a peritoneal drain

! Peritoneal drain placement may be the treatment of choice forextremely small (


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! Diagnosis:" PE findings (KEY Factor)" Differential Diagnosis

! Delay in Dx:" Perforation

! Complications:" Peritonitis & Abcess

! Treatment:" Correction of fluid and electrolyte deficits" Appendectomy

! Acute congestive appendicitis! Acute suppurative appendicitis

Intussusception

Telescopingof a segment of intestine thru itself! Age:

" 3-8 months" well-nourished child

! Diagnosis:" Episodic colicky pain with vomiting" sausaged-shaped mass" currant-jelly stools

! Barium Enema: Diagnostic and Therapeutic! U.S findings: Target or donut hole sign

Treatment:! Barium or Air reduction! Surgery

Meckels Diverticulum

Vitelline (Omphalomesenteric) Duct Anomalies! Meckels Diverticulum! Patent Omphalomesenteric Duct! Cord-like Remnant! Vitelline Cyst! Vitelline Sinus Tract

! Most common form of congenital abnormality of the small intestine! Results from an incomplete obliteration of the vitelline duct! It has been reported in as many as 97% of the vitelline duct anomalie! Meckel diverticulum is a true diverticulumbecause it contains all

layers of the intestinal wall.! Heterotopic mucosa is likely to be gastric in origin in 80% of cases of

Meckel diverticulum

Demography! 1-2% of the Population! No gender predominance (Incidental Diverticula)! 3-4:1 Male:Female (Symptomatic Diverticula)! Associated with:

! Esophageal Atresia (12%)! Imperforate Anus (11%)! Minor Omphalocele (25%)

Clinical Presentation! Rule of 2s:

! 2% of the Population! < 2 feet from the ileocecal valve! 2 types of heterotopic mucosa (Gastric and Pancreatic)! < 2 years old! 2 inches long

! Symptoms:! Variety of clinical signs ranging from no symptoms to acute

abdominal pain

! 3 Most Common Presentations:! gastrointestinal bleeding! intestinal obstruction! inflammation of the diverticulum (diverticulitis)

Complications! Obstruction:

! Most frequently encountered!

Usually present at a younger age! Bleeding:

! Most frequently encountered! Usually present at a younger age

! Inflammation! Others:

! Foreign body impaction, origin of neoplasia, site of parasitiinfection

Bleeding! < 5 years old! Painless Melena (Massive and Episodic)! Radionuclide Scanning (Indication)

! Pertechnetate Ions with Technetium 99m (secreted byGastric Mucosal Cells)


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! 90% Accuracy! 85% Sensitivity! 95% Specificity

! In children, painless gastrointestinal bleeding is the most commonpresenting symptom:

! Prevalence of different types of bleeding:! dark red (maroon): 40%! bright red: 35%! bright red with dark red: 12%! dark red and tarry: 6%! tarry: 7%

! For rapid bleeding, stools are bright red or have anappearance like currant jelly stools

! When slow bleeding occurs, the stools are black and tarryObstruction

! Triad:" Abdominal Distention" Vomiting" Crampy Abdominal Pain

! Secondary to:" Intussusception (More Common)" Volvulus

Inflammation:! Rarely diagnosed pre-operatively!

Clinically similar to Appendicitis! Perforation (Complication 2ndary to Peptic Ulceration)

Complications:

Meckels diverticulitis Intussusceptions on meckels diverticulum Perforated Meckels diverticulitis Torsion of giant meckel Persistent omphalo duct (Mekels diverticuum is attached to the

umbilicus)

Diagnostic Procedures asymptomatic! Blood test:

! to check for anemia! Stool Occult Blood:

! To check for minimal bleeding in stool! Barium enema and/or small bowel series:

! A procedure performed to examine the small and largeintestine for abnormalities

Diagnostic Procedures symptomatic! Meckel's scan:

! A substance called technetium is injected into your child'sbloodstream though an intravenous (IV) line

! Ectopic Pancreatic and Gastric Tissues! Superior Mesenteric Angiography:

! helpful in patients presenting with acute GI bleeding! effective when blood loss exceeds 0.5 mL/min.

Treatment! Symptomatic Diverticulum:

! Surgical (Segmental Resection)! Incidental Diverticulum:

! Controversial! Morbidity/Mortality vs. Chance of becoming symptomatic! Dependent on age, sex and physical characteristics of the

diverticulum! 4-6% lifetime risk of becoming symptomatic

Surgical Approach! Four possible surgical procedures:

! Diverticulectomy with suture closure of the base

! Wedge resection of the intestinal wall containing thediverticulum with suture closure

! Segmental resection of the intestine, including thediverticulum and end-to-end anastomosis

! Division of the fibrous band with or without diverticulectomIncidental Meckels Diverticulum! Resection is recommended for:

! patients younger than 40 years! diverticula longer than 2 cm! diverticula with narrow necks! diverticula with fibrous bands! suspected ectopic gastric tissue! inflamed, thickened diverticula

Pediatric Cancer

United States:

Constitutes less than 1% of adult malignancies 2ndleading cause of death in children


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" Recently, achieves a cure rate of >90%" National Wilms Tumor Study (NWTS) Group and the International

Society of Pediatric Oncology (ISOP)" Developed a multimodal therapeutic regimen

Incidence

" Most common 1omalignant renal tumor in children" 1 to 5 years old (78% of cases)" 3 years old (peak incidence)" Develops in patients with other congenital anomalies (12-15% of

cases)" Hereditary (15-20% of cases)" Bilaterality (5% of cases)

Associated Congenital Disorders

Disorder and Incidence

Hamartomas, Hemangiomas, Multiple Nevi, Caf-au-lait spots7.9%

G-U anomalies, Horseshoe Kidneys, Dysplasia, Cystic disease,Hypospadias, Cryptorchidism, Duplication anomaly 4.4%

Congenital hemihypertrophy - 2.9% Musculoskeletal anomaly, Clubfoot, Rib fusion, Hip dislocation, distal

phocomelia 2.9%

Congenital aniridia - 1.1%Genetic Aspect of Wilms Tumor

" WT1 and WT2: specific Wilms tumor suppressor genes" Located in chromosome 11:

" WT1 (band 11p13)" WT2 (band 11p15)

" A result of mutation and/or deletion" Explains the hereditary and sporadic cases

Clinical Signs and Symptoms

Symptoms and Frequency

Palpable mass (Flank/Upper Abdomen) 60% Hypertension 25% Hematuria 15% Obstipation 4% Weight loss 4% UTI 3% Diarrhea 3% Previous Trauma 3% Others: Bleeding diathesis, Polycythemia, Nausea/Vomiting 8%

Differential Diagnosis

Neuroblastoma Soft-tissue sarcoma Intra-renal Neoplasms:

o Congenital Mesoblastic Nephroma (Benign)o Clear Cell Sarcoma (Malignant)o Rhabdoid Tumor of the Kidney (Malignant)

Diagnostics Ultrasonography:

o Abdominal:! Initial study selected! Localizes tumor, may demonstrate extension

(renal vein and vena cava)

CT-scan:o Abdominal:

! Outlines and localizes tumor! Determines extension as well as regional and

distant spread! May identify nephrogenic rests

(ipsilateral/contralateral kidney)! Plan surgery

o Chest:! Detects metastasis

Chest radiography:! Detects metastasis

MRI:! Expensive but may later find an increasing role

Renal scan:! Function of contra-lateral kidney

Staging of Wilms Tumor" Essential for appropriate treatment"

Determined by:" Extent of the disease" Resectability of the primary tumor" Status of perirenal, capsular and renal involvement" Local invasion of adjacent organs and structures" Metastasis" Contralateral kidney involvement

" Defined at the time of explorationNWTS Group Staging

" Stage 1:" Limited to kidney; completely excised; intact capsule; n

ruptured nor biopsy sampled (except FNAB); no vessinvolvement; no residual tumor at or beyond margin resection

" Stage 2:" Extends beyond kidney but completely excised; may ha

regional extension; may have vessel involvement; biopperformed; tumor spillage confined to flank; no evidence tumor at or beyond margins of resection

" Stage 3:" With residual non-hematogenous tumor confined

abdomen; with anyone of the following: lymph nodes withabdomino-pelvic, penetrated or implants on peritonesurface, gross/microscopic residual tumor, incompleresection; tumor spillage not confined to the flank

" Stage 4:" Hematogenous metastasis or lymph node mestastasis n

confined to abdomino-pelvic area" Stage 5:

" Bilateral involvement" Stage 1 to 5, Anaplasia (Unfavorable Histology)

Local Spread/Extension:

" Renal sinus" Intrarenal blood and lymphatic vessels" Through renal capsule

" Sites of Metastasis:" Lungs" Regional lymph nodes" Liver

Treatment

Surgical:" Primary treatment" Radical or Modified radical nephrectomy" Contralateral Kidney exploration

Chemotherapy:" Pre-operative (Neo-adjuvant):

" Bilateral Wilms tumor" IVC extension (above hepatic veins)" Unresectable on surgical exploration" Bulky or massive tumors" Bleeding diasthesis or clotting abnormality

" Post-operative (Adjuvant):" All stages


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Radiation Therapy:" Stages 3 to 4 (favorable histology)" Stages 2 to 4 (unfavorable histology)" Stage 1 to 4 (clear cell sarcoma and rhabdoid tumor)

Prognosis

" Histologic features (key determinant):" Favorable vs. Unfavorable Histology

" Lymph node involvement:" Important predictor of treatment failure

" Chromosomal markers:" An important predictor of prognosis" Loss of heterozygosity of the 16q and 1p markers

Neuroblastoma

" Most common abdominal malignancy in children" 6%-10% of childhood cancers" 15% pediatric cancer deaths (U.S.)

Pathophysiology:

Results from aberrant migration and differentiation of neural crest cellsdestined to form the sympathetic ganglia

From brain to pelvis Adrenal gland (60% of cases)

Incidence

80% present before 4 years old Majority less than 2 years old Male:Female (1.3:1) Majority have stage 3-4 disease at time of diagnosis (poor prognosis) Overall survival: 30% Favorable prognosis:

o Patients less than 1 year oldo Primary mass and metastasis (liver, bone and skin)o Spontaneous regression and maturation

Clinical Signs and Symptoms

Palpable abdominal mass (75%) Malaise, Fever Abdominal Distention, Anorexia, Weight loss, Abdominal pain Bowel or Bladder dysfunction Respiratory symptoms (dyspnea, wheezes, stridor) Neurologic symptoms (dumbbell lesions or intra-spinal extension) Locoregional spread and metastasis (2/3):

o Bone marrowo Cortical boneo Livero Skin

Diagnostics" Ultrasonography:

" No characteristic diagnostic appearance" CT Scan:

" Greater anatomic detail" Stage disease" Metastasis" Plan surgery

" MRI:" Thoracic or intra-spinal extension

" Biopsy:" Needle" Incision" Excisional

Para-diagnostics" Plain radiography and Bone scan:

" Bone metastasis" Radiolabeled Metaiodobenzyl Guanidine (MIBG):

" Recognizes receptors for peptide somatostatin

" Provide information on tumor viability, disease exterecurrence and prognosis

" Serologic Studies:" Lactate Dehydrogenase (LDH)" Ferritin" Neuron-specific Enolase (NSE)" Vanillymandelic Acid (VMA)

Neuroblastoma Staging (INSS)Stage Definition1 complete gross excision of localized tumo

with/without microscopic residual diseasrepresentative ipsilateral lymph nodes negatifor tumor

2A incomplete gross excision of localized tumorepresentative ipsilateral lymph nodes negativfor tumor

2B complete or incomplete gross excision localized tumor; representative ipsilatelymph nodes positive for tumor;representaticontralateral lymph nodes negative for tumor

3 Unresectable unilateral tumor infiltrating acro

midline with or without lymph node involvemenor localized unilateral tumor with contralateregional lymph node involvement; or midlitumor with bilateral extension

4 With dissemination to distant lymph nodes, bonbone marrow, liver, skin and/or other organs

5 Localized primary tumor with disseminatiolimited to skin, liver, and/or bone marrow; limiteto infant < 1 year old

Treatment" Surgery:

" Early staged (Stage 1 or 2)" Localized" Resectable" Goal: total or near-total excision

" Chemotherapy:" Neo-adjuvant or Adjuvant" Locally advanced (Stage 2b or 3)" Distant metastasis (Stage 4)

" Radiotherapy or Myeloablation (TBI):" Residual disease" Progressive hepatomegaly (Stage 4S)" Prior to bone-marrow transplant

" Immunotherapy:Prognosis

" Shimada Pathologic Classification:Favorable Histology Unfavorable Histology

Stroma rich: well differentiated; intermixed Nodular

Stroma poor:Age 5 years old None All

*MKI, Mitosis karyorrhexis index


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Neuroblastoma Biologic Markers

Poor PrognosisNeuron-specific Enolase (NSE) >15ng/mlLactate Dehydrogenase (LDH) >1,500IU/mlFerritin >142ng/ml

Vanillymandelic Acid/Homovanillic Acid ratio 10 copiesChromosome 1 short arm (p) deleteDNA Ploidy 1CD44 Expression decreasedTyrosine Kinase A (TRKA) Expression increasedMulti-drug Resistance Associated Protein (MDRP) increased

Teratoma

Gonadal:" Ovary: 27%" Testis: 5%

" Extra-gonadal:" Sacrococcygeal (most common): 45% (60%)" Mediastinal: 6%" Central Nervous System (CNS): 5%" Retroperitoneal: 4%" Cervical: 3%" Head: 3%" Gastric, Hepatic, Pericardial, Umbilical cord:


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" Normally high in neonates and normalizes withage

" Post-operative half-life is 6 days" B-human chorionic gonadotropin (B-hcg):

" Choriocarcinoma" May cause precocious puberty

" Carcino-embryonic antigen:" Rare

Treatment" Surgical Excision:

" Complete excision" Incomplete excision carries the risk of malignant

transformation

" Chemotherapy:" Adjuvant or neoadjuvant

DEANNA GUIILLEN

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III-2b Pediatric Surgery Part 2