Pediatric surgery by uVLerY

VIEWS: 43 PAGES: 48

									Pediatric surgery

          By
 Pr. Dr. Ayman Reda
                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
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.
   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
  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
           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).
                         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
               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)
Hypertrophic Pyloric Stenosis
• It is one of the most common gastrointestinal disorders
  during early infancy, with an incidence of 1:3000 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 .
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.
                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.
        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.
      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.
         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
  Hirschsprung’s 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.
                  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 Meckel’s 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.
                        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.
                    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.
Air enema reduction of an
     intussusception
               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
       Hirschsprung’s Disease
• Hirschsprung’s Disease is characterized pathologically by absent
  ganglion cells in the myenteric (Auerbach’s) and submucosal
  (Meissner’s) 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 Hirschsprung’s disease is greater if there is a positive
  family history and in patients with Down syndrome.
 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 Hirschsprung’s 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 Hirschsprung’s disease is relatively frequent.
• Enterocolitis is the most common cause of death in patients with
  uncorrected Hirschsprung’s disease and may present with diarrhea
  alternating with periods of obstipation, abdominal distention, fevers,
  hematochezia, and peritonitis.
                         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 Hirschsprung’s 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 Hirschsprung’s disease and may provide
    additional diagnostic yield.
•   Anorectal manometry may also suggest the diagnosis of Hirschsprung’s
    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 Hirschsprung’s
    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.
•
                         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.
         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.
 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.
         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.
                           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.
• 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.
             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.
      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.
• 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.
                 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.
                     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 α1 -
  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%.
                        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.
                   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.
    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.
                            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
                 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.
                       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 Horner’s 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.
                      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.
                       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.
         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 40–90% 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
  Bochdalek’s hernia and distinguished from the
  congenital hernia of the anteromedial, retrosternal
• diaphragm, which is known as Morgagni’s hernia.
• Primary repair or mesh repair once respiratory status
  has been optimized
                                   Hypospadias
Failure of complete urethral tubularisation in the male fetus results in
     hypospadias, a common congenital anomaly affecting about one in
     every 200–300 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.

								
To top