Pediatric surgery

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Pediatric surgery

• By
• Pr. Dr. Ayman Reda

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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

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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.

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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

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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

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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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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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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)

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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 .

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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Air enema reduction of an intussusception
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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

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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.

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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.

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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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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.

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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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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.

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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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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.

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• 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.

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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.

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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.

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• 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.

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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.

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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.

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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.

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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.

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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.

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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

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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.