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Pediatric surgery

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Fascial closure may be facilitated by stretching the anterior abdominal wall as well as milking out the contents of the bowel proximally and distally. – PowerPoint PPT presentation

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Title: Pediatric surgery


1
Pediatric surgery
  • By
  • Pr. Dr. Ayman Reda

2
Pediatric surgery
  • Children are not small adults.
  • They suffer from different disorders and their
    physical and psychological responses are
    different.
  • Their capacity for adaptation is greater but they
    must endure any
  • consequences of disease and its management for
    longer.
  • In contrast to adults they rarely have
    comorbidity from degenerative
  • diseases or lifestyle problems but they can
    suffer the unique consequences of congenital
    malformations.
  • Children must be treated within the context of
    their families

3
ANATOMY AND PHYSIOLOGY
  • Anatomical differences between adults and
    children are important in surgery. Infants and
    small children have a wider abdomen, a broader
    costal margin and a shallower pelvis.
  • Thus, the edge of the liver is more easily
    palpable below the costal margin and the urinary
    bladder is an intra-abdominal organ. The ribs are
    more horizontal and respiratory function is more
    dependent on diaphragmatic movement. The
    umbilicus is relatively low lying.
  • In the small child, transverse supraumbilical
    incisions are preferred to vertical midline ones
    for laparotomy.
  • Abdominal scars grow with the child and may
    migrate a gastrostomy sited in the epigastrium
    of the infant may end up as a scar over the
    costal margin.

4
Special features that must be considered in
children when preparing for surgery
  • Problem

    Action
  • Thermoregulation
    Warm fluids, warm theatre, insulate
    child
  • Hypoglycaemia
    Maintain glucose level
  • Clotting
    Give intramuscular vitamin K preoperatively
    to neonates
  • Fluid and electrolyte
    Allow for higher sodium and fluid balance
    needs
  • Less postoperativeCatabolism
    Relatively lower postoperative energy
    requirements
  • Gastro-oesophageal reflux
    Use a nasogastric tube to prevent
    aspiration
  • Atypical presentations of infection
    High index of
    suspicion
  • Psychology
    Trained staff, day surgery for
    minor operations

5
Special features of surgical technique in children
  • Gentle tissue handling
  • Bipolar diathermy is preferred to unipolar
    during dissection
  • Abdominal incisions can be closed with delayed
    absorbable sutures( vic.
  • Bowel can be anastomosed with interrupted
    single-layer
  • extramucosal sutures
  • Skin can be closed with absorbable subcuticular
    sutures

6
Esophageal Atresia and Tracheoesophageal Fistula
  • Esophageal atresia (EA) is a congenital
    interruption or discontinuity of the esophagus
    resulting in esophageal obstruction.
  • Tracheoesophageal fistula (TEF) is an abnormal
    communication (fistula) between the esophagus and
    trachea. EA may be present with or without a
    TEF. Alternatively, a TEF can occur without EA.
  • There is association of anomalies in patients
    with EA/TEF that must be considered under the
    acronym VACTERL (vertebral, anorectal, cardiac,
    tracheal, esophageal, renal,limb).

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8
Diagnosis
  • The diagnosis of EA should be entertained in an
    infant with excessive salivation along with
    coughing or choking during the first oral
    feeding. A maternal history of polyhydramnios is
    often present. The inability to pass a
    nasogastric tube into the stomach of the neonate
    is a cardinal feature for the diagnosis of EA.
    Inability to pass a nasogastric tube in an infant
    with absent radiographic evidence for
    gastrointestinal gas is virtually diagnostic of
    an isolated EA without TEF
  • On the other hand, if gas is present in the
    gastrointestinal tract below the diaphragm, an
    associated TEF is confirmed. These simple rules
    provide the correct diagnosis in most cases.
    Occasionally, a small amount of isotonic contrast
    may be given by mouth to demonstrate the level of
    the proximal EA pouch and/or the presence of a
    TEF, but this is rarely necessary. In fact, the
    risk of aspiration with studies of this type is
    generally high

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10
Management
  • The immediate care of an infant with EA/TEF
    includes IV fluid ,continuous suction of the
    proximal EA pouch and manipulation of the
    endotracheal tube distal to the TEF may minimize
    the leak and permit adequate ventilation.
    Further, placement of an occlusive balloon
    (Fogarty) catheter into the fistula via a
    bronchoscope may be useful.
  • Finally, urgent thoracotomy with direct ligation
    of the fistula after rapid preoperative
    preparation (exclude VACTERL)

11
Hypertrophic Pyloric Stenosis
  • It is one of the most common gastrointestinal
    disorders during early infancy, with an incidence
    of 13000 to 4000 live births.
  • This condition is most common between the ages of
    2 and 8 weeks. In HPS, hypertrophy of the
    circular muscle of the pylorus results in
    constriction and obstruction of the gastric
    outlet.
  • Gastric outlet obstruction leads to nonbilious,
    projectile emesis, loss of hydrochloric acid with
    the development of hypochloremic, metabolic
    alkalosis, and ultimate dehydration. Visible
    gastric peristalsis may be seen as a wave of
    contraction from the left upper quadrant to the
    epigastrium. The infants usually feed vigorously
    between episodes of vomiting. Palpation of the
    pyloric tumor (also called the olive) in the
    epigastrium or right upper quadrant by a skilled
    examiner is pathognomonic.
  • Ultrasound is diagnostic .

12
Hypertrophic Pyloric Stenosis
  • The treatment of this condition is by surgical
    mechanical distraction of the pyloric ring (by a
    pyloromyotomy).
  • Prior to surgery,it is important that the infant
    is hydrated with intravenous fluids to establish
    a normal urine output. It is important that the
    underlying metabolic alkalosis is slowly
    corrected.
  • Postoperatively, infants are usually allowed to
    resume enteral feedings.

13
Duodenal Atresia
  • In contrast with more distal intestinal atresias,
    duodenal atresia (DA) is believed to occur as a
    result of failure of vacuolization of the
    duodenum from it solid cord stage. DA is
    associated with several conditions, including
    prematurity, Down syndrome, maternal
    polyhydramnios, malrotation, annular pancreas,
    and biliary atresia (BA).
  • The classic plain abdominal radiograph of DA is
    termed the double-bubble sign (air-filled stomach
    and duodenal bulb).
  • The treatment of DA is by surgical bypass of the
    duodenal obstruction by duodenoduodenostomy.

14
Jejunoileal Atresia
  • Intrauterine focal mesenteric vascular accident.
    The clinical presentation is typically dependent
    on the level of obstruction.
  • The treatment of JIA is to re-establish
    intestinal continuity.

15
Anomalies of Intestinal Rotation/Fixation
  • Midgut volvulus is a true surgical emergency
    since delay in operative correction is associated
    with a high risk of intestinal necrosis and
    subsequent death. The sudden appearance of
    bilious emesis in a newborn is the classic
    presentation.

16
Meconium Syndromes
  • The meconium syndromes of infancy represent a
    complex group of gastrointestinal conditions
    associated with CF( cystic fibrosis)
  • Meconium Plug is a frequent cause of neonatal
    intestinal obstruction and associated with
    multiple conditions including Hirschsprungs
    disease, maternal diabetes, hypothyroidism, and
    CF. Typically, affected infants are often preterm
    and present with signs and symptoms of distal
    intestinal obstruction. Plain abdominal
    radiographs reveal multiple dilated loops of
    intestine. The diagnostic and therapeutic
    procedure of choice is a water-soluble contrast
    enema. This often results in the passage of a
    plug of meconium and relief of the obstruction.
    The operative management of simple meconium ileus
    is required when the obstruction cannot be
    relieved with contrast enema.
  • Complicated Meconium IleusMeconium ileus is
    considered complicated when perforation of the
    intestine has taken place.

17
Intussusception
  • Intussusception is the telescoping of one portion
    of the intestine into the other and is the most
    common cause of intestinal obstruction in early
    childhood.
  • In most pediatric intussusceptions, the cause is
    unknown, the location is at the ileocecal
    junction, and there is no identifiable pathologic
    lead point. Invariably, there is marked swelling
    of the lymphoid tissue within the region of the
    ileocecal valve.
  • The incidence of a pathologic lead point is up to
    12 in most pediatric series and increases
    directly with age. The most common lead point for
    intussusception is a Meckels diverticulum
    however, other causes must be considered
    including polyps, the appendix, intestinal
    neoplasm, submucosal hemorrhage associated with
    Henoch-Schönlein purpura, foreign body, ectopic
    pancreatic or gastric tissue, and intestinal
    duplication.

18
Clinically
  • Intussusception classically produces severe,
    cramping abdominal pain in an otherwise healthy
    child. The child often draws his or her legs up
    during the pain episodes and is usually quiet
    during the intervening periods. After some time,
    the child becomes lethargic. Vomiting is almost
    universal. Although frequent bowel movements may
    occur with the onset of pain, the progression of
    the obstruction results in bowel ischemia with
    passage of dark blood clots mixed with mucus,
    commonly referred to as currant jelly stool. An
    abdominal mass may be palpated.
  • In about half of cases, the diagnosis of
    intussusception can be suspected on plain
    abdominal radiographs. Suggestive radiographic
    abnormalities include the presence of a mass,
    sparse gas within the colon, or complete distal
    small bowel obstruction. In cases where there is
    a low index of suspicion for intussusception
    based on clinical findings, an abdominal
    ultrasound may be the initial diagnostic test.
    Ultrasonography can be diagnostic.

19
Management
  • When the clinical index of suspicion for
    intussusception is high, hydrostatic reduction by
    contrast agent or air enema is the diagnostic and
    therapeutic procedure of choice.
  • Contraindications to this study include the
    presence of peritonitis or hemodynamic
    instability.
  • Further, an intussusception that is located
    entirely within the small intestine is unlikely
    to be reached by enema and more likely to have an
    associated lead point. Hydrostatic reduction
    using barium has been the mainstay of therapy
    however, more recently, the use of air enema has
    become more widespread.
  • Successful reduction is accomplished in more than
    80 of cases and is confirmed by resolution of
    the mass, along with reflux of air into the
    proximal ileum.
  • To avoid radiation exposure altogether,
    intussusception reduction by saline enema under
    ultrasound surveillance may be employed.
  • Recurrence rates after hydrostatic reduction are
    about 11 and usually occur within the first 24
    hours. Recurrence is usually managed by another
    attempt at hydrostatic reduction. A third
    recurrence is usually an indication for operative
    management.

20
Air enema reduction of an intussusception
21
Acute appendicitis
  • Remember the following
  • Loose stools may be present
  • Tenderness and guarding in the right iliac
    fossa is characteristic
  • Exclude referred pain from right lower lobe
    pneumonia
  • Take special care in diagnosing appendicitis in
    the preschool child
  • Surgery is the treatment but only after fluid
    resuscitation and antibiotics

22
Hirschsprungs Disease
  • Hirschsprungs Disease is characterized
    pathologically by absent ganglion cells in the
    myenteric (Auerbachs) and submucosal
    (Meissners) plexus. This neurogenic abnormality
    is associated with muscular spasm of the distal
    colon and internal anal sphincter resulting in a
    functional obstruction. Hence, the abnormal bowel
    is the contracted, distal segment, whereas the
    normal bowel is the proximal, dilated portion.
  • The area between the dilated and contracted
    segments is referred to as the transition zone.
    In this area, ganglion cells begin to appear, but
    in reduced numbers.
  • The aganglionosis always involves the distal
    rectum and extends proximally for variable
    distances. The rectosigmoid is affected in about
    75 of cases, splenic flexure or transverse colon
    in 17, and the entire colon with variable
    extension into the small bowel in 8.
  • The risk for Hirschsprungs disease is greater if
    there is a positive family history and in
    patients with Down syndrome.

23
Presentation and complication
  • In most, infants are symptomatic within the first
    24 hours of life with progressive abdominal
    distention and bilious emesis. Failure to pass
    meconium in the first 24 hours is highly
    significant and a cardinal feature of this
    condition. In some infants, diarrhea may develop
    due to the presence of enterocolitis.
  • The diagnosis of Hirschsprungs disease may also
    be overlooked for prolonged periods. In these
    cases, older children may present with a history
    of poor feeding, chronic abdominal distention,
    and a history of significant constipation. Since
    constipation is a frequent problem among normal
    children, referral for surgical biopsy to exclude
    Hirschsprungs disease is relatively frequent.
  • Enterocolitis is the most common cause of death
    in patients with uncorrected Hirschsprungs
    disease and may present with diarrhea alternating
    with periods of obstipation, abdominal
    distention, fevers, hematochezia, and
    peritonitis.

24
Investigation
  • The initial diagnostic step in a newborn with
    radiographic evidence for a distal bowel
    obstruction is a barium enema. Prior to this
    study, rectal examination and enemas should be
    avoided so as not to interfere with the
    identification of a transition zone.
  • In a normal barium enema study, the rectum
    is wider than the sigmoid colon.
  • In patients with Hirschsprungs disease,
    spasm of the distal rectum usually results in a
    smaller caliber when compared with the more
    proximal sigmoid colon.
  • Failure to completely evacuate the
    instilled contrast material after 24 hours would
    also be consistent with Hirschsprungs disease
    and may provide additional diagnostic yield.
  • Anorectal manometry may also suggest the
    diagnosis of Hirschsprungs disease. The classic
    finding is failure of the internal sphincter to
    relax when the rectum is distended with a
    balloon.This is more often useful in an older
    patient and is seldom used in neonates.
  • A rectal biopsy is the gold standard for the
    diagnosis of Hirschsprungs disease. In the
    newborn period, this is done at the bedside with
    minimal morbidity using a special suction rectal
    biopsy instrument. It is important to obtain the
    sample at least 2 cm above the dentate line so as
    to avoid sampling the normal transition from
    ganglionated bowel to the paucity or absence of
    ganglia in the region of the internal sphincter.

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26
Treatment
  • Traditionally, in mangement of intestinal
    obstruction caused by this a leveling procedure
    is done, followed by proximal diversion.
  • A definitive procedure is performed later and
    involves variations of pull through procedures
    among three main procedures.
  • In the Swenson procedure, the aganglionic bowel
    is removed down to the level of the internal
    sphincters and a coloanal anastomosis is
    performed on the perineum.
  • In the Duhamel procedure, the aganglionic rectal
    stump is left in place and the ganglionated,
    normal colon is pulled behind this stump. A GIA
    stapler is then inserted through the anus with
    one arm within the normal, ganglionated bowel
    posteriorly and the other in the aganglionic
    rectum anteriorly. Firing of the stapler
    therefore results in formation of a neorectum
    that empties normally, due to the posterior patch
    of ganglionated bowel.
  • Finally, the Soave technique involves an
    endorectal mucosal dissection within the
    aganglionic distal rectum. The normally
    ganglionated colon is then pulled through the
    remnant muscular cuff and a coloanal anastomosis
    is performed.
  • More recently, the Soave procedure has been
    performed in the newborn period as a primary
    procedure and without an initial colostomy.

27
Anorectal malformations
  • The spectrum of anorectal malformations ranges
    from simple anal stenosis to the persistence of a
    cloaca.
  • By 6 weeks gestation, the urorectal septum moves
    caudally to divide the cloaca into the anterior
    urogenital sinus and posterior anorectal canal.
  • Failure of this septum to form results in a
    fistula between the bowel and urinary tract (in
    boys) or the vagina (in girls).
  • Complete or partial failure of the anal membrane
    to resorb results in an anal membrane or
    stenosis.
  • Breakdown of the cloacal membrane anywhere along
    its course results in the external anal opening
    being anterior to the external sphincter (i.e.,
    anteriorly displaced anus).
  • An anatomic classification of anorectal anomalies
    is based on the level at which the blind-ending
    rectal pouch ends in relationship to the levator
    ani musculature . Historically, the level of the
    end of the rectal pouch was determined by
    obtaining a lateral pelvic radiograph (i.e.,
    invertogram) after the infant is held upside down
    for several minutes to allow air to pass into the
    rectal pouch.

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29
Clinical picture and investigation
  • Inspection of the perineum alone determines the
    pouch level in 80 of boys and 90 of girls.
    Clinically, if an anocutaneous fistula is seen
    anywhere on the perineal skin of a boy or
    external to the hymen of a girl, a low lesion can
    be assumed, which allows a primary perineal
    repair procedure to be performed, without the
    need for a stoma.
  • Most all other lesions are high or intermediate,
    and they require proximal diversion by a sigmoid
    colostomy. This is followed by a definitive
    repair procedure at a later date. If required,
    the level of the rectal pouch can be detailed
    more definitively by ultrasonography or MRI.

30
Abdominal Wall Defects
  • During normal development of the human embryo,
    the midgut herniates outward through the
    umbilical ring and continues to grow. By the 11th
    week of gestation, the midgut returns back into
    the abdominal cavity and undergoes normal
    rotation and fixation, along with closure of the
    umbilical ring. If the intestine fails to return,
    the infant is born with abdominal contents
    protruding directly through the umbilical ring
    and is termed an omphalocele . Most commonly, a
    sac is still covering the bowel, thus protecting
    it from the surrounding amniotic fluid.
    Occasionally, the sac may be torn at some point
    in utero, thus creating confusion with the other
    major type of abdominal wall defect termed
    gastroschisis. In contrast with omphalocele, the
    defect seen with gastroschisis is always on the
    right side of the umbilical ring with an intact
    umbilical cord, and there is never a sac covering
    the abdominal contents. The major morbidity and
    mortality with either anomaly are not as much
    with surgical repair of the abdominal defect as
    they are with the associated abnormalities. In
    the absence of other major anomalies, the
    long-term survival is excellent.

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32
Treatment
  • The treatment of an omphalocele consists of a
    nasogastric or orogastric tube decompression for
    prevention of visceral distention due to
    swallowed air. An intravenous line should be
    secured for administration of fluids and
    broad-spectrum antibiotics. The sac should be
    covered with a sterile, moist dressing and the
    infant transported to a tertiary care pediatric
    surgery facility.
  • Prior to operative repair, the infant should be
    evaluated for potential chromosomal and
    developmental anomalies by a careful physical
    examination, plain chest radiograph,
    echocardiography if the physical examination
    suggests underlying congenital heart disease, and
    renal ultrasonography. Since the viscera are
    covered by a sac, operative repair of the defect
    may be delayed so as to allow thorough evaluation
    of the infant.
  • Several options exist for the surgical management
    of an omphalocele and are largely dictated by the
    size of the defect. In most cases, the contents
    within the sac are reduced back into the abdomen,
    the sac is excised with care to individually
    ligate the umbilical vessels, and the fascia and
    skin are closed.
  • Fascial closure may be facilitated by stretching
    the anterior abdominal wall as well as milking
    out the contents of the bowel proximally and
    distally.

33
  • In giant omphaloceles, the degree of
    visceroabdominal disproportion prevents primary
    closure and the operative management becomes more
    challenging. Construction of a Silastic silo
    allows for gradual reduction of the viscera into
    the abdominal cavity over several days.
    Monitoring of intraabdominal pressure during
    reduction may prevent the development of an
    abdominal compartment syndrome. Once the
    abdominal contents are returned to the abdomen,
    the infant is taken back to the operating room
    for formal fascia and/or skin closure.
  • Occasionally, closure of the fascia may be
    impossible. In these cases, the skin is closed
    and a large hernia is accepted. This is repaired
    after 1 or 2 years. When the skin cannot be
    closed over the defect, several options exist,
    including the topical application of an
    antimicrobial solution to the outside of the sac
    such as silver nitrate or silver sulfadiazine.
    Over time, this results in granulation tissue and
    subsequent epithelialization of the sac. A repair
    of the large hernia is then performed a few years
    after this.

34
Inguinal Hernia in the pediatric age group
  • Repair of an inguinal hernia (IH) represents one
    of the most frequent surgical procedures
    performed in the pediatric age group. Virtually
    all IH in children are indirect and congenital in
    origin.
  • Most IH present as a bulge in the region of the
    external ring extending downward for varying
    distances to the scrotum or labia. Often, the
    hernia is detected by a pediatrician during a
    routine physical examination or observed by the
    parents. Inguinal pain may also be a presenting
    complaint.
  • Incarceration and possible strangulation are the
    most feared consequences of IH and occur more
    frequently in premature infants. Because of the
    risk for these complications, all IH in children
    should be repaired.

35
IH repair in premature infants
  • The timing for IH repair in premature infants is
    controversial. Early repair may be associated
    with a higher risk for injury to the cord
    structures, greater recurrence rate, and
    anesthetic-related apnea. These factors must be
    weighed against the higher risk for incarceration
    and strangulation, the potential for losing the
    patient during follow-up, and the development of
    a larger IH with loss of domain in the abdominal
    cavity. Taking these factors into account, most
    pediatric surgeons perform herniorrhaphy before
    the neonate is discharged to home from the
    nursery.
  • If the infant has already been discharged home,
    most pediatric surgeons wait until the infant is
    older than 60 weeks postconception (gestational
    age postnatal age). After this age, the risk
    for postoperative apnea is diminished.

36
  • In patients with incarcerated IH containing
    bowel, attempts should be made to reduce the
    hernia, unless there is clinical evidence of
    peritonitis.
  • This may require intravenous sedation and careful
    monitoring.
  • If the reduction is successful, the child is
    admitted and observed for 24 to 48 hours. The IH
    repair should be done after the period of
    observation to allow for tissue edema to subside.
  • On the other hand, if the IH cannot be reduced,
    the child should be promptly taken to the
    operating room for inguinal exploration.

37
Biliary Atresia BA
  • BA is characterized by progressive (not static)
    obliteration of the extrahepatic and intrahepatic
    bile ducts. The cause is presently unknown.
    Patients who are not offered surgical treatment
    uniformly develop biliary cirrhosis, portal
    hypertension, and death by 2 years of age.
  • Pathologically, the biliary tracts contain
    inflammatory and fibrous cells surrounding
    minuscule ducts that are probably remnants of the
    original ductal system. Bile duct proliferation,
    severe cholestasis with plugging, and
    inflammatory cell infiltrate are the pathologic
    hallmarks of this disease. This histology is
    usually distinct from the giant cell
    transformation and hepatocellular necrosis that
    are characteristic of neonatal hepatitis, the
    other major cause of direct hyperbilirubinemia in
    the newborn.
  • A serum direct bilirubin level higher than 2.0
    mg/dL or greater than 15 of the total bilirubin
    level defines cholestasis and is distinctly
    abnormal, and further evaluation is mandatory.
    Delay in diagnosis of BA is associated with a
    worse prognosis. Thus, the initial opportunity
    for success in the management of this disease
    relies on the early recognition of abnormal
    direct hyperbilirubinemia.

38
Investigation
  • In addition to a careful history and physical
    examination, blood and urine should be obtained
    for bacterial and viral cultures, reducing
    substances in the urine to rule out galactosemia,
    serum IgM titers for syphilis, cytomegalovirus,
    herpes, and hepatitis B, serum a1 -antitrypsin
    level and phenotype, serum thyroxine level, and a
    sweat chloride test done to exclude CF
  • Ultrasonography of the liver and gallbladder is
    important in the evaluation of the infant with
    cholestasis. In BA, the gallbladder is typically
    shrunken or absent, and the extrahepatic bile
    ducts cannot be visualized.
  • The next diagnostic step is to perform a
    percutaneous liver biopsy if the hepatic
    synthetic function is normal. This is well
    tolerated under local anesthesia, and the
    diagnostic accuracy is in the range of 90.

39
Treatment
  • If the needle biopsy and/or the abdominal
    ultrasound are consistent with BA, exploratory
    laparotomy (laparoscopy) is then performed
    expeditiously.
  • The initial goal at surgery is to confirm the
    diagnosis. This requires the demonstration of the
    fibrotic biliary remnant and definition of absent
    proximal and distal bile duct patency by
    cholecystocholangiography.
  • The classic technique for correction of BA is the
    Kasai hepatoportoenterostomy. In this procedure,
    the distal bile duct is transected and dissected
    proximally up to the level of the liver capsule,
    whereby it is excised, along with the gallbladder
    remnant . A Roux-en-Yhepaticojejunostomy is then
    constructed by anastomosis of the jejunal
    Roux-limb to the fibrous plate above the portal
    vein.

40
Choledochal Cyst
  • A cystic enlargement of the common bile duct is
    referred to as a choledochal cyst.
  • Types
  • Type I cysts represent 80 to 90 of cases and
    are simply cystic dilations of the common bile
    duct.
  • Type II cysts are represented as a diverticulum
    arising from the common bile duct.
  • Type III cysts are also referred to as
    choledochoceles and are isolated to the
    intrapancreatic portion of the common bile duct
    and frequently involve the ampulla.
  • Type IV cysts are second in frequency and
    represent dilation of both intrahepatic and
    extrahepatic bile ducts.
  • In type V cysts, only the intrahepatic ducts are
    dilated.
  • The pathophysiology of choledochal cysts remains
    poorly understood.

41
Clinical picture and investigation
  • Although choledochal cysts can produce symptoms
    in any age group, most become clinically evident
    within the 1st decade of life. The triad of a
    right upper quadrant mass, abdominal pain, and
    jaundice is highly suggestive of the diagnosis.
  • In some patients, pancreatitis may bepresent.
  • In older children and adults, the presentation
    may be more insidious and include
    choledocholithiasis, cholangitis, and cirrhosis
    with progression to portal hypertension.
  • Malignant degeneration is also found in up to
    16 of adults with choledochal cysts.
  • In addition to routine measurement of serum
    bilirubin, alkaline phosphatase, and amylase
    levels, the most useful diagnostic test for
    choledochal cysts is ultrasonography. Once
    dilation of the extrahepatic biliary ducts is
    demonstrated, no further testing is usually
    necessary in children.
  • Although seldom necessary, preoperative
    endoscopic retrograde cholecystopancreatography
    may provide additional information regarding the
    pancreaticobiliary ductal anatomy to guide
    intraoperative decision making.

42
Treatment
  • Total cyst excision with Roux-en-Y
    hepaticojejunostomy is the definitive procedure
    for management of types I and II choledochal
    cysts.
  • In cases whereby there is significant
    inflammation, it may be impossible to safely
    dissect the entire cyst way from the anterior
    surface of the portal vein. In these
    circumstances, the internal lining of the cyst
    can be excised, leaving the external portion of
    the cyst wall intact.
  • Type III cysts are typically approached by
    opening the duodenum, resecting the cyst wall
    with care to reconstruct and marsupialize the
    remnant pancreaticobiliary ducts to the duodenal
    mucosa.
  • In type IV cysts, the bile duct excision is
    coupled with a lateral hilar dissection to
    perform a jejunal anastomosis to the lowermost
    intrahepatic cysts.
  • If the intrahepatic cysts are confined to a
    single lobe or segment, hepatic resection may be
    indicated.
  • The treatment of type V cysts involving both
    lobes is usually palliative with transhepatic or
    U tubes until liver transplantation can be
    performed.
  • The postoperative outcomes following excision of
    choledochal cysts are excellent

43
Neuroblastoma
  • Neuroblastoma (NBL), the most common abdominal
    malignancy in children.
  • These tumors are of neural crest origin and, as a
    result, may arise anywhere along the sympathetic
    ganglia or within the adrenal medulla.
  • Although these tumors may occur at any site from
    the brain to the pelvis, 75 originate within the
    abdomen or pelvis, and half of these occur within
    the adrenal medulla. Twenty percent of NBLs
    originate within the posterior mediastinum, and
    5 are within the neck.
  • The median age at diagnosis is 2 years.
  • Approximately 25 of patients present with a
    solitary mass that may be cured by surgical
    therapy, whereas most present with extensive
    locoregional or metastatic disease.

44
Clinical picture
  • The presenting symptoms of NBL are dependent on
    several factors, including the site of the
    primary tumor, the presence of metastatic
    disease, the age of the patient, as well as the
    metabolic activity of the tumor.
  • The most common presentation is a fixed, lobular
    mass extending from the flank toward the midline
    of the abdomen. Although the abdominal mass may
    be noted in an otherwise asymptomatic child,
    patients may complain of abdominal
    pain,distention, weight loss, or anorexia. Bowel
    or bladder dysfunction may arise from direct
    compression of these structures by the tumor.
  • Cervical tumors may be discovered as a palpable
    or visible mass or be associated with stridor or
    dysphagia.
  • Posterior mediastinal masses are usually detected
    by plain chest radiographs in a child with
    Horners syndrome, dyspnea, or pneumonia.
  • Further, the tumor may extend into the neural
    foramina and cause symptoms of spinal cord
    compression.
  • Marrow replacement by tumor may result in anemia
    and weakness.
  • Numerous paraneoplastic syndromes can occur in
    conjunction with NBL. Cerebellar ataxia,
    involuntary movements, and nystagmus are the
    hallmark of the dancing eyes and feet syndrome.
    Excess secretion of vasoactive intestinal
    polypeptide may stimulate an intractable watery
    diarrhea. Hypertension may be significant, owing
    to excessive catecholamine production by the
    tumor.

45
Management
  • A spot urine should be tested for the
    catecholamine metabolites homovanillic and
    vanillylmandelic acid.
  • A serum lactate dehydrogenase level higher than
    1500 IU/mL, serum ferritin level higher than 142
    ng/mL, and neuron-specific enolase levels higher
    than 100 ng/mL correlate with advanced disease
    and reduced survival.
  • CT and/or MRI are the preferred modalities for
    characterizing the location and extent of the
    NBL. This tumor frequently infiltrates through
    vascular structures . As such, many tumors that
    cross the midline are generally not resectable.
  • A CT scan of the chest should be done to exclude
    pulmonary metastasis, and a bone scan should be
    done to identify potential bone metastasis.
  • In addition, radiolabeled metaiodobenzyl
    guanidine (MIBG) is one of the single best
    studies to document the presence of metastatic
    disease.
  • Finally, a bone marrow aspirate .
  • Current therapy for NBL is multimodal
  • incorporating surgery, chemotherapy,
  • radiation, and occasionally immunotherapy.

46
Teratoma
  • Teratomas are tumors that contain elements
    derived from more than one of the three embryonic
    germ layers. In addition, teratomas must contain
    tissue that is foreign to the anatomic site in
    which they occur. Teratomas can occur anywhere in
    the body and present as cystic, solid, or mixed
    lesions. When they occur during infancy and early
    childhood, they are most commonly extragonadal.
    In contrast, in older children teratomas most
    frequently involve the gonads.
  • Teratomas occur most frequently in the neonatal
    period and the sacrococcygeal region is the most
    common site. Sacrococcygeal teratoma (SCT) is
    four times more common in females and is most
    often an obvious external presacral mass.

47
Diaphragmatic Hernia, Congenital
  • 80 are left-sided
  • Symptoms and signs include dyspnea, chest
    retractions, decreased breath sounds on affected
    side
  • Prenatal ultrasound is accurate in 4090 of
    cases, showing herniation of abdominal contents
    in thorax ,chest film, arterial blood gas
    measurements, echocardiogram ultrasound for
    neural tube defects.
  • The posterolateral location of this hernia is
    known as Bochdaleks hernia and distinguished
    from the congenital hernia of the anteromedial,
    retrosternal
  • diaphragm, which is known as Morgagnis hernia.
  • Primary repair or mesh repair once respiratory
    status has been optimized

48
Hypospadias
  • Failure of complete urethral tubularisation in
    the male fetus results in hypospadias, a common
    congenital anomaly affecting about one in every
    200300 boys. In most cases the urethra opens
    just proximal to the glans penis but in severe
    cases the meatus may be on the penile shaft or in
    the perineum.
  • The dorsal foreskin is hooded and there is a
    variable degree of chordee (a ventral curvature
    of the penis most apparent on erection) .
  • Glanular hypospadias may be a solely cosmetic
    concern but more proximal varieties interfere
    with micturition and erection.
  • In severe forms of hypospadias, additional
    genitourinary anomalies and intersex disorders
    should be excluded.
  • Surgical correction of distal hypospadias is
    frequently undertaken before 2 years of age,
    often as a single-stage operation.
  • Proximal varieties may require complex staged
    procedures.
  • Surgery aims to achieve a terminal urethral
    meatus so that the boy can stand to micturate
    with a normal stream, a straight erection and a
    penis that looks normal.
  • Ritual circumcision must be avoided in infants
    with hypospadias because the foreskin is often
    required for later reconstructive surgery.
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