32 Pediatric Surgery - Bài viết - Bệnh Học
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32 Pediatric Surgery

Cho điểm
Pediatric Surgery
Trudie A. Goers
Patrick A. Dillon
Surgery involving children is predicated on the fundamental facts that infants and children differ from adults in anatomy, physiology, and particularly their reaction to operative trauma and that the necessary adjustments of surgical procedures are not merely matters of scale. Although some disease processes are managed similar to those in adults, this chapter addresses the more pediatric-specific surgical issues.
I. Fluid, Electrolytes, and Nutrition
A. Fluid requirements

  • Children's normal daily fluid requirements are higher than those of adults due to higher insensible losses as a result of the high ratio of body-surface area to volume and the limited ability of immature kidneys to concentrate urine (Table 32-1).
  • Postoperative fluid replacement: daily requirements plus losses (measurable and insensible).
    • Total body water is a higher percentage of body weight (75%) than in adults (60%).
    • Total blood volume in the newborn is approximately 8% of body weight, which decreases to 5% in older infants.
    • The total fluid administered should be adjusted to support urine output between 1 and 2 mL/kg/hour.
    • Output from tubes and drains should be matched by replacement fluid with similar electrolyte composition.


B. Electrolytes

  • Children younger than 6 months old should be given 10% dextrose in 0.25% saline with potassium chloride, 20 mEq/L.
  • Children older than 6 months old should be given 5% dextrose in 0.45% saline with potassium chloride, 20 mEq/L.
  • Daily sodium requirements are 2 to 3 mEq/kg.
  • Daily potassium requirements are 1 to 2 mEq/kg.

C. Nutrition

  • Calories (Table 32-2):

Total daily caloric need (kcal/kg/day) = Resting energy expenditure (REE) + (REE × additional factor),
where the additional factors are as follows:


    • Normal maintenance: 0.2
    • Activity: 0.1 to 0.25
    • Fever: 0.13/(°C >38°C)
    • Simple trauma: 0.2
    • Multiple trauma: 0.4
    • Burns: 0.5 to 1

  • A newborn weight gain of 15 to 30 g/day is expected.
  • Carbohydrates should supply 40% to 45%, lipids 35%, and protein 15% of total calories in the diet.

TABLE 32-1 Normal Fluid Requirements in Children
Weight (kg)
24-hr fluid requirements
<2 (premature)
150 mL/kg
1–10
100 mL/kg
11–20
1,000 mL + 50 mL/kg for each kg >10
>20
1,500 mL + 20 mL/kg for each kg >20
Weight (kg)
Hourly fluid requirements
0–10
4 mL/kg
10–20
2 mL/kg
>20
1 mL/kg

  • Most infant formulas contain 20 kcal/oz; therefore, caloric needs can be calculated by the following formula:

Weight (kg) × 6 oz = Volume of formula necessary to deliver 120 kcal/kg.
  • Enteral nutrition is the preferred method of delivering calories to a patient. However, for patients for whom enteral nutrition in contraindicated, parenteral nutrition should be initiated early.
    • Glucose


Glucose infusion rate (mg/kg/minute) = % dextrose × infusion rate (mL/hour)/weight (kg) × 6,
where dextrose 3.4 kcal/g = % dextrose × 10.
Goal is 13 to 15 mg/kg/minute.


    • Protein

      • <1 year of age: TrophAmine.
      • >1 year of age: Travasol.
      • Goal:

        • Preterm infant: 3.5 to 4 g/kg/day.
        • Term infant: 2 to 3 g/kg/day.
        • Child: 1.5 to 2.5 g/kg/day.




TABLE 32-2 Normal Daily Calorie Needs in Children
Age (yr)
REE (kcal/kg/d)
Average (kcal/kg/d)
<36 wk
 
120
0–0.5
53
108
0.5–1
56
98
1–3
57
102
4–6
48
90
7–10
40
70
11–14
32
55
15–18
27
45
11–14
28
47
15–18
25
40
REE, resting energy expenditure.




        •  




TABLE 32-3 Nothing-by-Mouth Requirements in Children
Age
Clear liquids
Solids/formula/breast milk
<6 mo
3 hr
4 hr
>6 mo
4 hr
6 hr
>12 yr
6 hr
6 hr


    • Lipids

      • 20% intralipid = 2 kcal/mL.
      • Goal: 2 to 3 g/kg/day or 30% to 40% of total calories.



II. Preoperative Preparation

  • History and physical
  • Laboratory tests. Without major comorbidities, usually a hematocrit is sufficient.
  • Nothing-by-mouth (NPO status) (Table 32-3).Studies have indicated that clear liquids ingested 2 to 3 hours before induction of anesthesia do not increase the risk of aspiration (Anesthesiology 1990;72:593). Aspiration risks can also be reduced by administering H2-receptor antagonists, metoclopramide, and buffering agents such as sodium citrate.
  • Preoperative antibiotic prophylaxis indications
    • Patients with cardiac anomalies.
    • Patients with ventriculoperitoneal shunts.
    • Patients with implanted prosthetic material.
    • Most common regimen: ampicillin (50 mg/kg) and gentamicin (1.5 mg/kg) given usually 1 hour before and 6 hours after the procedure.


III. Vascular Access
A. Peripheral venous access
Usual candidates for access: dorsal vein of the hand, antecubital vein of the arm, saphenous vein at the ankle, dorsal vein in the foot, median tributary at the wrist, external jugular vein, and scalp veins.
B. Percutaneous central venous catheter insertion
Central venous access may be needed if peripheral access is exhausted or if drugs or nutrition need to be given centrally or for a prolonged period of time. Access to central veins can be accomplished directly or via peripheral veins [i.e., peripherally inserted central catheter (PICC) placement]. Sites most often chosen are the subclavian vein, internal jugular vein, and femoral vein.
C. Intraosseous (IO) access
Intraosseous (IO) access should be used in an emergency setting when other attempts at obtaining vascular access have failed. A location 1 to 3 cm distal to the tibial tuberosity is the recommended site. The needle should be directed inferiorly during insertion. Alternatively, the femur may be reached by inserting the needle in a cephalad direction 3 cm proximal to the condyles. Either a bone marrow aspiration needle or a 16- to 19-gauge butterfly needle is adequate. Contraindications to IO access include a fracture of the bone or previous IO catheter.
D. Arterial catheters
Arterial catheters are needed in some neonates and infants. The potential sites for placement of an arterial catheter include the umbilical, radial, femoral, posterior tibial, and temporal arteries; however, the radial artery is used most frequently. The typical catheters are 22 to 24 gauge and can be placed percutaneously or by cutdown. In a neonate who is within the first 2 to 4 days of life, the umbilical artery can often be cannulated through the umbilical stump.
IV. Common Neonatal Surgical Problems
It must be remembered that congenital anomalies rarely occur independently. When a defect is identified, laboratory and radiologic investigations must be employed to uncover associated developmental abnormalities (e.g., VACTERL syndromes involving vertebral defects, imperforate anus, cardiac defects, tracheoe sophageal malformations, renal dysplasia, and limb anomalies).
A. Congenital diaphragmatic hernia (CDH)

  • Incomplete diaphragm development at 8 weeks' gestation results in herniation of abdominal organs into the chest, preventing normal lung development.
  • Left leaflet occurs in 90% of cases, and right leaflet in 10%.
  • Bochdalek (posterolateral defect; 85% of cases); Morgagni (anterior, parasternal; fewer pulmonary and systemic complications).
  • A 1:1 male-to-female ratio.


  • Diagnosis

    • Antenatal ultrasound and maternal-fetal magnetic resonance imaging (MRI). Polyhydramnios is detected in up to 80% of cases.
    • Physical exam

      • Cardiorespiratory distress.
      • Asymmetric “funnel” chest.
      • Reduced breath sounds on the affected side.
      • Scaphoid abdomen.

    • Chest x-ray

      • Herniated abdominal viscera within the chest.
      • Mediastinal shift.


  • Mortality

    • Approximately 35%.
    • May be higher in patients with severe pulmonary hypoplasia and hypertension or in the presence of associated congenital anomalies.

  • Management
    • Immediate postnatal care
      • Supplemental oxygen.
      • Endotracheal intubation is indicated if significant respiratory distress is present. Avoid bag-mask ventilation, which exacerbates gastrointestinal (GI) distention and further impedes lung ventilation.
      • Intravenous access is necessary for the administration of fluids to maintain organ perfusion.
      • GI decompression by orogastric or nasogastric intubation reduces distention of the stomach and within the thoracic cavity.

    • Intensive care
      • Conventional ventilation utilizing permissive hypercapnia to minimize barotrauma.
        • Once stabilized, wean fraction of inspired oxygen (FIO2) for preductal oxygen saturation greater than 90%.
        • Arterial carbon dioxide tension of 60 to 70 mm Hg is acceptable.
        • Maintain pH greater than 7.20.
        • Peak airway pressures less than 25 cm H2O.
        • Mean airway pressures less than 12 cm H2O.

      • High-frequency oscillating ventilator (HFOV)

        • May be used if conventional ventilation failed.
        • Possibly limit barotrauma.

      • Inhaled nitric oxide may decrease severity of pulmonary hypertension.
      • Extracorporeal membrane oxygenation (ECMO) is considered for patients with severe preductal hypoxemia or right-to-left shunting due to high pulmonary hypertension. Patients with overwhelming pulmonary hypoplasia may not be not candidates for ECMO.

    • Operative intervention

      • Usually deferred until the patient's pulmonary status is stable.
      • A subcostal incision on the affected side allows the herniated abdominal contents to be replaced in the peritoneal cavity.
      • Repaired primarily or with a synthetic patch, depending on the size of the defect.

    • Complications: long-term respiratory insufficiency, GI reflux, neurologic sequelae from prolonged hypoxia, recurrence of hernia.


B. Tracheoesophageal malformations

  • This is a spectrum of anomalies, including esophageal atresia (EA) and tracheoesophageal fistula (TEF), separately or in combination (Fig. 32-1).
  • Interruption in the normal budding of the trachea from the foregut during embryonic development.
  • A 1:1 male-to-female ratio.

Figure 32-1. Variants of tracheoesophageal fistula. A: Atresia without fistula (5% to 7% of cases). B: Proximal fistula and distal pouch (<1% occurrence). C: Proximal pouch with distal fistula (85% to 90% of cases). D: Atresia with proximal and distal fistulas (<1% of cases). E: Fistula without atresia (H type) (2% to 6% occurrence).
 
  • Diagnosis
    • Physical exam
      • Excessive drooling.
      • Regurgitation of feedings.
      • Choking, coughing, or cyanosis during feeding.

    • Radiology
      • Coiled orogastric tube in the esophageal pouch.
      • Gas in the GI tract implies the presence of a distal TEF.


  • Management

    • Prevent aspiration and pneumonia.

      • Supine position with the head elevated 30 to 45 degrees.
      • Insert an orogastric or nasogastric tube into the proximal esophageal pouch for decompression and to prevent aspiration.
      • Administer ampicillin (50 mg/kg) and gentamicin (2.5 mg/kg) intravenously.

    • Operative intervention

      • Right extrapleural thoracotomy.
      • Ligation of the fistula and repair of the EA with esophagoesophagostomy.

    • Complications

      • Stricture of esophagus, leak of esophageal anastomosis, recurrence of fistula.
      • Gastrointestinal reflux disorder (GERD), esophageal dysmotility.



C. Gastroschisis

  • This abdominal wall defect is believed to arise from an isolated vascular insult in the developing mesenchyme.
  • It usually occurs to the right of a normal umbilical cord with any combination of abdominal organs herniating through the defect.
  • In contrast to omphalocele, there is no membranous sac covering the eviscerated abdominal organs. The incidence of associated anomalies in gastroschisis is low, but approximately 10% may have intestinal atresia.


  • Diagnosis
    • Ultrasound examination

      • Evident after 13 weeks' gestation.
      • Intestine free within the amniotic fluid apart from the umbilical cord.

    • Physical exam

      • Abdominal defect to the right of the umbilical cord.
      • No encompassing sac.
      • Exposed bowel may develop a serositis.


  • Management
    • Prenatal management

      • Serial ultrasound: If bowel dilation and mural thickening of the eviscerated bowel are detected, delivery at the time of lung maturity may be indicated (Surg Obstet Gynecol 1993;81:53).
      • Delivery should be planned at a tertiary care center with both high-risk obstetrics service and pediatric surgical expertise.

    • Postnatal management
      • Intravenous fluids

        • Normal saline, 20-mL/kg bolus.
        • Maintenance intravenous fluid (IVF) to achieve a urine output of 1.5 to 2 mL/kg/hour.

      • Heat and fluid losses can be decreased by covering the exposed bowel with moistened gauze and then wrapping the bowel with Saran/plastic wrap.
      • Position the neonate in a lateral position to prevent kinking of the bowel and vascular compromise.
      • Extension of the fascial defect in the midline for 1 to 2 cm can be done if the bowel mesentery appears to be compressed by a narrow opening.
      • Operative intervention

        • Viscera may be reduced primarily, but if this is not possible, viscera are placed in a spring-loaded or self-made silo, which is secured beneath the fascial edge.
        • Silo and viscera are reduced gradually over time.
        • The abdominal wall defect is then repaired in a primary fashion on an elective basis. Fewer complications may arise with this method of management compared with immediate reduction of viscera and early primary closure (J Pediatr Surg 2000;35:843).

      • Complications: bowel ischemia, short gut, bowel nonrotation, feeding intolerance.



D. Omphalocele

  • This is an abdominal wall defect of the umbilical ring in which the intestines protrude through the base of the umbilical cord and herniate into a sac.
  • High incidence (50%) of related anomalies (cardiac, chromosomal).


  • Diagnosis
    • Antenatal ultrasound

      • Evident after 13 weeks' gestation.
      • Bowel can be seen herniating through the base of the umbilical cord into an echogenic sac.
      • Because of the high incidence of related anomalies, the presence of an omphalocele should direct a thorough search for other birth defects by ultrasonography and amniocentesis.

    • Amniotic fluid α-fetoprotein (AFP) levels are elevated.
    • Physical examination

      • Abdominal wall defect at the base of the umbilical cord. The size of the defect varies from a few centimeters to absence of most of the abdominal wall.
      • The presence of a sac covering the herniated viscera.
      • Rupture of the sac is infrequent, but it is distinguished from gastroschisis by the presence of residual sac in continuity with the umbilical cord.


  • Management. The care of a patient with an omphalocele should be conducted according to the same principles as for gastroschisis (see Section IV.C.2). Rupture of the sac requires that the defect be treated as a gastroschisis.
  • Due to the presence of other anomalies associated with omphalocele, the overall prognosis for infants with omphalocele is significantly worse than that for gastroschisis.

E. Necrotizing enterocolitis (NEC)

  • This acute fulminating, inflammatory disease of the intestine is associated with focal or diffuse ulceration and necrosis of the small bowel, colon, and, rarely, the stomach.
  • Cause is unknown; however, the pathogenesis is thought to be multifactorial, involving an immature gut barrier defense and virulent bacteria.
  • The incidence of NEC is 1 to 3/1,000 live births.
  • It primarily affects premature infants, and occurs in 10% of all babies born weighing less than 1,500 g.


  • Diagnosis
    • High index of suspicion.
    • History and physical exam

      • Lethargy.
      • Skin pallor or mottling.
      • Temperature instability.
      • Apnea.
      • High gastric residuals.
      • Vomiting or bilious nasogastric tube drainage.
      • Hematochezia.
      • Diarrhea.
      • Abdominal distention.
      • Intra-abdominal mass.
      • Edema or erythema of the abdominal wall may indicate peritonitis, local response to inflamed bowel, or perforation of bowel.

    • Laboratory tests

      • Metabolic acidosis.
      • Thrombocytopenia.
      • Leukocytosis or leukopenia.

    • Radiologic studies

      • Dilated bowel loops.
      • Pneumatosis intestinalis.
      • Ascites.
      • Intrahepatic portal vein gas.
      • Pneumoperitoneum.
      • Ultrasonography may reveal portal venous gas, pneumatosis, and the presence of free peritoneal fluid.
      • Contrast imaging is often avoided due to the risk of perforation.


  • Management
    • Nonoperative treatment

      • Bowel rest.
      • Nasogastric decompression.
      • Removal of umbilical catheters.
      • Maintenance of proper fluid balance.
      • Parenteral nutrition.
      • Broad-spectrum antibiotics.
      • Serial abdominal examinations.
      • Serial radiographs.
      • Laboratory studies.

    • Indications for operative treatment

      • Pneumoperitoneum.
      • Portal vein gas.
      • Enteric organisms present in paracentesis fluid.
      • Intestinal obstruction.
      • Intra-abdominal abscess.
      • Sepsis unresponsive to treatment.

    • Choice of procedure

      • Bowel resection of involved bowel with the creation of stomas. When intestinal viability is questionable, reexploration within 24 hours is essential.
      • Open peritoneal drainage.

        • Temporizing measures in critically ill infants until they can tolerate a laparotomy.
        • Primary therapy for extremely low birth weight infants (<1,000 g) (J Pediatr Surg 1990;25:1034).




V. Alimentary Tract Obstruction
A. Congenital causes

  • Intestinal malrotation

    • Intestine fails to undergo its normal rotation and fixation during embryologic development.
    • Incidence 1/500 to 1/6,000 live births.
    • Associated anomalies are present in 30% to 59% of patients.
    • Symptoms most often present in the neonatal period but may present in adulthood.

      • Diagnosis

        • History and physical

          • Bilious emesis
          • Hematemesis
          • Abdominal distention
          • Abdominal tenderness
          • Hematochezia

        • Radiologic studies

          • Plain radiographs. “Double-bubble” is indicative of duodenal obstruction but not diagnostic of volvulus.
          • Contrast imaging

            • Necessary to establish diagnosis.
            • Failure of the duodenum to cross to the right of the midline with right-sided jejunum characterizes intestinal malrotation.
            • Hallmark signs for volvulus include the “bird's beak” sign and a corkscrew appearance of the proximal small intestine.



      • Management
        • Observation for signs of obstruction.
        • Operative intervention. Ladd procedure:

          • Correct volvulus by counterclockwise rotation of bowel and division of peritoneal (Ladd's) bands.
          • Position colon on the left side of the abdomen and the small bowel on the right.
          • Perform appendectomy.





B. Intestinal atresia or stenosis

  • This results from intrauterine vascular insult.


  • Location. Distal ileum > proximal jejunum > duodenum > colon.
  • Diagnosis

    • Antenatal ultrasound

      • Polyhydramnios.
      • Dilated intestinal loops.

    • Neonatal history and physical

      • Symptoms appear shortly after birth for atresia but may take weeks to months for stenosis.
      • Bilious vomiting.
      • Abdominal distention.
      • Failure to pass meconium.
      • Failure to thrive.
      • Poor feeding.

    • Radiology

      • A “double-bubble” sign is diagnostic of duodenal obstruction.
      • Contrast enema may be used to identify a distal intestinal atresia and can also identify obstruction secondary to meconium ileus or meconium plug syndrome.


  • Management

    • Nasogastric decompression.
    • Intravenous fluid.
    • Ampicillin (50 mg/kg) and gentamicin (2.5 mg/kg) should be given preoperatively.
    • Operative intervention

      • Primary anastomosis, which may require resection or tapering of the dilated proximal segment.
      • For duodenal atresia, a duodenoduodenostomy or duodenojejunostomy is created to bypass the obstruction.
      • Saline should be infused into the distal bowel to rule out synchronous intestinal atresias.



C. Hirschsprung disease

  • Variable length of intestinal aganglionosis of the hindgut.
  • Rectosigmoid is the aganglionosis transition point for 80% of patients.
  • Familial or sporadic.
  • Up to 7.8% of cases occur in patients for whom more than one family member is affected.
  • Mutations in the RET protooncogene have been found in both familial and sporadic cases.


  • Diagnosis
    • History
      • Neonates: abdominal distention, infrequent defecation, failure to pass meconium within the first 48 hours of life, or enterocolitis with sepsis.
      • Older infants and children present with chronic constipation or failure to thrive.

    • Radiology
      • Plain abdominal radiographs commonly show a pattern of distal obstruction.
      • Barium enema

        • Usually demonstrates a transition zone between distal nondilated bowel and proximal dilated bowel.
        • Most common in the rectosigmoid, but may be seen anywhere in the colon.
        • Total colonic aganglionosis: no transition zone.


    • Pathology
      • Rectal biopsy is essential for making the diagnosis.
      • Full-thickness specimens are the ideal tissue samples to allow identification of the absence of ganglion cells.
      • In neonates, rectal suction biopsy often is sufficient for diagnosis.


  • Preoperative management
    • Colonic decompression to prevent enterocolitis. Saline enemas may be used to evacuate impacted stool.
    • Nasogastric tube should be placed if the child is vomiting.

  • Operative treatment

    • Goals of surgical management are removal of aganglionic bowel and reconstruction of the intestinal tract by bringing the innervated bowel down to the anus while maintaining normal sphincter function.
    • Primary pull-through

      • Swenson.
      • Duhamel.
      • Soave.
      • Laparoscopic endorectal pull-through (Georgeson).
      • Transanal endorectal pull-through (Langer).



Each of these operations has been modified to improve functional results and may be performed in the newborn period, although surgery may delayed to allow for increased weight gain or resolution of enterocolitis. Advocates of the last two approaches cite decreased recovery time, decreased hospital stay, decreased complications, a potential for decreased adhesions, and decreased cost as some of the advantages of minimal-access approaches in comparison with open transabdominal procedures (Ann Surg 1999;229:678, J Pediatr Surg 2000;35:820).


    • Diverting colostomy

      • Performed proximal to aganglionic segment in patients who are unstable or who have massively dilated bowel.
      • Daily rectal irrigations may obviate the need for a colostomy.



D. Anorectal anomalies
Classification system (Am J Surg 2000;180:370):

  • Males: perineal fistula, rectourethral bulbar fistula, rectourethral prostatic fistula, rectovesical (bladder neck) fistula, imperforate anus without fistula, rectal atresia and stenosis.
  • Females: perineal fistula, vestibular fistula, imperforate anus with no fistula, rectal atresia and stenosis, persistent cloaca.
  • The lesions may also be classified as low, intermediate, or high depending on whether the atresia is below, at the level of, or above the puborectalis sling, respectively.


  • Diagnosis

    • Physical examination.
    • Plain radiographs: obstructive series, invertogram, sacral abnormalities.
    • Contrast studies: mucous fistulogram.
    • Magnetic resonance.

  • Management

    • Define associated defects.

      • VACTERL syndromes.
      • Cardiovascular anomalies occur in conjunction with anorectal malformations 12% to 22% of the time.

    • Inspection of the perineum is required to help distinguish the type of defect. The presence of meconium on the perineum within 24 hours of birth may signify a perineal fistula (low defect), which may be safely repaired without a colostomy.
    • A high defect with probable rectourethral or rectovaginal fistula requires a colostomy and mucous fistula for initial management.
    • If the anatomy is unclear, lateral radiographs of the pelvis with the infant in a prone position can be obtained.

  • Definitive operative repair

The main goal of caring for a baby with an anorectal malformation is bowel and urinary continence. For perineal fistulas, primary repair may be performed without a protective colostomy. For the more complex anorectal anomalies, a three-step methodology is advocated with a diverting colostomy after birth, posterior sagittal anorectoplasty (PSARP), and colostomy closure. Of note, laparoscopic management of anorectal anomalies has been performed.
E. Omphalomesenteric duct abnormalities

  • These represent failure of complete involution of the vitelline duct.
  • They occur in the ileum 60 cm or less from the ileocecal valve.
  • Common presentations:

    • Meckel diverticulum. A true diverticulum on the antimesenteric border.
    • Patent duct between the ileum and the umbilicus.
    • Umbilical cyst lined by intestinal mucosa.
    • Fibrous cord between an ileal diverticulum and the umbilicus or mesentery.



  • Diagnosis
    • Meckel diverticulum

      • More than 50% present before 2 years of age.
      • Painless hemorrhage. Ectopic gastric mucosa that is frequently present results in peptic ulceration and hemorrhage in 22% of cases.
      • Bowel obstruction

        • Internal hernia around a vitelline duct band (13%).
        • Diverticulitis with abdominal pain (2%.)

      • Intussusception (Arch Surg 1987;122:542).
      • Technetium-99m pertechnetate scintiscan. Isotope is taken up by the ectopic gastric mucosa within the diverticulum.

    • Patent duct. Leakage of ileal contents through the child's umbilicus.
    • Umbilical cysts. Secretion of mucoid fluid via the child's umbilicus.
    • Fibrous band. Usually presents as an internal hernia or intestinal volvulus around this cord of tissue, with the accompanying signs of intestinal obstruction.

  • Management
    • Operative intervention

      • Diverticulectomy

        • Open or laparoscopic resection of the diverticulum.
        • Stapling of diverticulum or resection with anastomosis.
        • Dissection with ligation of feeding mesenteric vessel.




When radiographic reduction of an intussusception is unsuccessful:




        • Surgical reduction of invaginated intestine.
        • Diverticulectomy.
        • If gangrenous bowel is present, bowel resection with primary anastomosis should be done.

      • Infants or young children who have a Meckel diverticulum discovered incidentally at laparotomy should usually undergo diverticulectomy.



F. Meconium ileus

  • This is a neonatal intestinal obstruction caused by inspissated meconium that may occur in the setting of cystic fibrosis.


  • Diagnosis
    • Prenatal ultrasound. Polyhydramnios.
    • History and physical exam

      • Abdominal distention.
      • Bilious vomiting.
      • Failure to pass meconium within 24 to 48 hours of life.
      • Pneumoperitoneum.
      • Peritonitis.
      • Abdominal wall inflammation.
      • Hypovolemia.
      • Sepsis.

    • Radiology. Plain film.
      • Dilated loops of small bowel.
      • Ground-glass appearance of air and meconium mixture.
      • Intra-abdominal calcifications suggest prenatal perforation and subsequent meconium peritonitis.
      • Ascites or pneumoperitoneum suggests perforation.
      • Up to 35% of infants with complicated meconium ileus show no radiographic abnormalities.
      • Water-soluble contrast enema. Confirms diagnosis by demonstrating a microcolon and inspissated meconium in the ileum.


  • Management
    • Nonsurgical treatment

      • Intravenous fluid.
      • Broad-spectrum antibiotics.
      • Hyperosmolar, water-soluble contrast enema, which is both diagnostic and therapeutic. This solution draws fluid into the bowel lumen and causes an osmotic diarrhea.

    • Operative intervention

      • Indicated for complicated meconium ileus or when enema therapy fails.
      • Enterotomy followed by irrigation with 1% to 2% acetylcysteine solution. If gentle irrigation does not flush out the meconium, a 14-French ileostomy tube may be placed, and routine irrigations are done beginning on postoperative day 1.
      • If intestinal volvulus, atresia, perforation, or gangrene complicates the illness:

        • Resect nonviable bowel.
        • Create end ileostomy with mucous fistula.
        • Institute routine irrigation.
        • Perform primary anastomosis 2 to 3 weeks later.
        • Advance oral feeds.
        • Institute pancreatic enzyme supplementation.




G. Meconium plug syndrome

  • This refers to altered colonic motility or viscous meconium believed to cause impaired stool transit and obstruction of the colonic lumen.
  • Patients do not have cystic fibrosis.


  • Diagnosis (see meconium ileus, Section V.F).
  • Management (see meconium ileus, Section V.F).
    • Operative intervention is rarely needed to relieve the obstruction.
    • Suction biopsy to rule out Hirschsprung disease.
    • Sweat chloride test to ensure that cystic fibrosis is not the etiology of the disease.


H. Intestinal duplications

  • These are cystic or tubular structures lined by various types of normal GI mucosa.
  • They are located dorsal to the true alimentary tract.
  • They frequently share a common muscular wall and blood supply with the normal GI tract.
  • In 20% of cases, enteric duplications communicate with the true GI tract.
  • They are most commonly located in the ileum but may occur anywhere from the mouth to the anus.


  • Diagnosis

    • Prenatal ultrasound.
    • History and physical exam.

      • Emesis.
      • Abdominal pain.
      • Abdominal distention may occur if the duplication compresses the lumen of the adjacent hollow viscus.
      • Abdominal mass.

    • Abdominal ultrasound.
    • GI contrast studies. External compression or displacement of the normal alimentary tract.
    • Technetium radioisotope scans. Aid in diagnosis if the cyst contains gastric mucosa.

  • Operative intervention
    • Resection.
    • Internal drainage.

      • Minimizes the risk of damage to the biliary system in the case of duodenal duplication.
      • May be indicated if duplication would require extensive bowel resection.
      • When gastric mucosa is found in a cyst, it is stripped, and the cyst lumen is joined to the adjacent intestine.



I. Acquired causes of alimentary tract obstruction

  • Pyloric stenosis

    • This is the most common surgical cause of nonbilious vomiting in infants.
    • It occurs in 1 of 400 live births.
    • The male-to-female ratio is 4:1.
    • Diagnosis

      • History and physical

        • Occurs generally in neonates who are 2 to 5 weeks of age.
        • Vomiting

          • Characteristically forceful or projectile and occurs 30 to 60 minutes after feeding.
          • Formula intolerance initially suspected but does not resolve with change of feeds.

        • Dehydration

          • Lethargy.
          • Absence of tears.
          • Sunken anterior fontanelle.
          • Dry mucous membranes.
          • Decreased urine output.

        • The “olive” mass

          • Mass palpated to the right and above the umbilicus.
          • Approximately 2 cm in diameter, firm, and mobile.

        • Abdominal ultrasonography

          • Pyloric diameter greater than 14 mm, muscular thickness greater than 4 mm, and pyloric length greater than 16 mm are diagnostic





of pyloric stenosis with 91% to 100% sensitivity and 100% specificity (J Pediatr Surg 1987; 22:950).




        • Upper GI contrast study

          • Enlarged stomach.
          • Poor gastric emptying.
          • Elongated, narrow pyloric channel or “string sign.”



    • Management

      • Preoperative fluid resuscitation

        • 20-mL/kg bolus.
        • 5% dextrose in normal saline to achieve urine output of 2 mL/kg/hour.
        • Addition of potassium and changing to 5% dextrose in 0.45% normal saline occurs when urine output is adequate.

      • Correction of the hypochloremic hypokalemic metabolic alkalosis.
      • Operative intervention

        • Indicated only after adequate resuscitation and correction of metabolic alkalosis.
        • Pyloromyotomy

          • Division of the hypertrophied pyloric muscle, leaving the mucosa intact.
          • Open or laparoscopic technique.

        • Postoperative feeding

          • Begin electrolyte solution by mouth 6 hours after pyloromyotomy.
          • Over the next 12 hours, formula or pumped breast milk can be started and should reach goal within 24 hours.
          • Parents should be advised that vomiting may occur postoperatively as a result of swelling at the pyloromyotomy, but this problem is self-limited.
          • If the pyloric mucosa is perforated and repaired during surgery, nasogastric drainage is recommended for 24 hours.




  • Intussusception

    • This refers to an invagination of proximal intestine into adjacent distal bowel, with resultant obstruction of the lumen (most common: ileocolic intussusception). A lead point of the intussusception is identified in only 5% of patients and is most commonly a Meckel diverticulum (see Section V.E.1).
    • This obstruction may compromise the arterial inflow and venous return.
    • Highest incidence is at 5 to 10 months of age.
    • Diagnosis

      • History and physical exam

        • A previously healthy infant who presents with periods of abrupt crying and retraction of the legs up to the abdomen.
        • Attacks usually subside over a few minutes but recur every 10 to 15 minutes.
        • In 30% of cases, a recent viral gastroenteritis or upper respiratory infection may precede onset of symptomatology.
        • “Currant jelly” stool. Dark-red mucoid material, which is sloughed mucosa.
        • Emesis.
        • Hyperperistaltic rushes may be heard during an episode.
        • A sausage-shaped abdominal mass may be palpated, or the tip of the intussusception may be felt on rectal examination.

      • Ultrasound can be used for screening in suspected cases of intussusception.
      • Barium or air-contrast enema confirms the diagnosis by demonstrating a “coiled spring” sign.

    • Management

      • Early surgical consultation.
      • Nasogastric drainage.
      • Intravenous fluid resuscitation.
      • Broad-spectrum antibiotics.
      • Pneumatic or hydrostatic reduction.

        • Air insufflation under radiographic guidance should be attempted if no evidence of peritonitis exists and the patient is stable.
        • This technique has a 90% success rate.
        • The maximum safe intraluminal air pressure for young infants is 80 mm Hg, and that for older children is 110 to 120 mm Hg.
        • Recurrent intussusception in children less than 1 year old can be treated with repeated enema therapy.
        • Postreduction course

          • Observation for 24 hours.
          • Liquid diet with advancement can be started once the child is alert.
          • Recurrent intussusception occurs in 8% to 12% of patients.


      • Operative intervention

        • Indications:

          • Failure of nonoperative reduction.
          • Child presents with peritonitis, sepsis, or shock.
          • Recurrence after enema reduction in an older child is indicated due to small-bowel tumors, which can serve as the lead point more frequently in this age group.

        • A transverse incision can be made to deliver the bowel. Gentle retrograde pressure is applied to the telescoped portion of the intestine in an attempt at manual reduction. Proximal and distal segments should not be pulled apart because of the risk of injury to the bowel.
        • If manual reduction is not possible, resection of the involved segment and primary anastomosis should be done.
        • An incidental appendectomy should also be performed.
        • Recurrence of intussusception after operative treatment is approximately 1%.



  • Distal intestinal obstruction syndrome (DIOS), formerly known as meconium ileus equivalent

    • This intestinal obstruction is caused by impaction of inspissated intestinal contents in older patients with cystic fibrosis.
    • This problem occurs in 10% to 40% of patients with cystic fibrosis who are followed long term.
    • Diagnosis

      • High index of suspicion in a child with cystic fibrosis who presents with chronic or recurrent abdominal pain and distention, vomiting, and constipation.
      • Inciting cause

        • Abrupt cessation of pancreatic enzyme supplementation.
        • Dehydration.
        • Dietary change.
        • Exacerbation of respiratory symptoms.

      • Plain abdominal radiographs
        • Ground-glass appearance of the intestine.
        • Dilated small-bowel.
        • Air-fluid levels.

      • Water-soluble contrast enemas demonstrate the inspissated intestinal contents.

    • Management
      • Water-soluble contrast enemas induce an osmotic diarrhea to flush the intestine and relieve the obstruction.
      • GoLytely solution may be used in selective cases to relieve the obstruction from above.

    • Surgical intervention

      • Indicated when enemas or conservative therapy is unsuccessful.
      • Indicated when intussusception or volvulus complicates DIOS.



VI. Jaundice

  • This is a yellowing of the skin that reflects the presence of hyperbilirubinemia.
  • In jaundiced infants, the total serum bilirubin usually exceeds 7 mg/dL.
  • This elevated bilirubin may reflect a rise in either the conjugated (direct) or unconjugated (indirect) bilirubin, or both. This distinction is integral for establishing a differential diagnosis for jaundice.

A. Unconjugated hyperbilirubinemia

  • This occurs when bilirubin that has not been metabolized in the liver rises above normal serum values.
  • Most common causes:

    • Hemolytic disorders.
    • Breast-feeding.
    • Physiologic jaundice of the newborn.
    • Indirect causes of increased enterohepatic circulation of bilirubin (rare)

      • Meconium ileus.
      • Hirschsprung disease.
      • Pyloric stenosis.


  • Treatment

    • Phototherapy.
    • Exchange transfusion.
    • Correction of primary diseases.


B. Conjugated hyperbilirubinemia

  • This occurs when excess monoglucuronides and diglucuronides in the liver result in elevated levels of bilirubin in the serum. Most common causes:

    • Biliary obstruction
    • Hepatitis (infectious, toxic, or metabolic etiology)
    • TORCH (to xoplasmosis, rubella, cytomegalovirus, and herpes simplex virus) infections

  • Treatment

    • Identify and treat etiologies of hepatitis.
    • Surgical interventions are only possible with the obstructive causes of direct hyperbilirubinemia discussed here.



  • Biliary atresia

    • Most common cause of infantile jaundice that requires surgical correction.
    • Etiology is unknown.
    • Disease characteristics

      • Progressive obliteration and sclerosis of the biliary tree.
      • With age, obliteration of the extrahepatic bile ducts, proliferation of the intrahepatic bile ducts, and liver fibrosis progress at an unpredictable rate.

    • Diagnosis

      • Clinical and laboratory information is often nonspecific.
      • Jaundice.
      • Alcoholic stools.
      • Dark urine.
      • Hepatomegaly.
      • Percutaneous liver biopsy. Biopsy results range from classic biliary tree fibrosis to those unable to be differentiated from α1-antitrypsin deficiency or neonatal hepatitis.
      • Technetium-99m iminodiacetic acid hepatobiliary imaging

        • Aids in differentiation between liver parenchymal disease and biliary obstructive disease.
        • In biliary atresia, the liver readily takes up this tracer molecule, but no excretion into the extrahepatic biliary system or duodenum is seen.

      • Ultrasonography. Shrunken extrahepatic ducts and a noncontractile or absent gallbladder.

    • Management

      • Open liver biopsy and cholangiogram

        • The common bile duct is visualized by cholangiography in only 25% of patients with biliary atresia.
        • Cholangiography in the remaining 75% of patients demonstrates an atretic biliary tree.

      • Operative intervention
        • Kasai procedure

          • “Hepatoportoenterostomy.”
          • Excision of obliterated extrahepatic ducts with hepaticojejunostomy.
          • When the distal common bile duct is patent, a choledochojejunostomy is constructed.
          • The overall results have shown that in children treated with a Kasai procedure, one third show long-term improvement, one third obtain temporary benefit, and one third experience treatment failure. Five-year survival for infants treated with a Kasai procedure before 2.5 months of age was 50% (Ann Surg 1989;210:289).

        • Liver transplantation. Recommended option when liver failure occurs or the Kasai procedure fails.



  • Choledochal cysts

    • This spectrum of diseases is characterized by cystic dilation of the extrahepatic and intrahepatic biliary tree.
    • They are believed to be an embryologic malformation of the pancreaticobiliary system.
    • Type I: fusiform cystic dilation of the common bile duct (most common form).
    • Type II: diverticulum of the extrahepatic bile duct.
    • Type III: choledoochocele.
    • Type IV: cystic disease of the intra- or extrahepatic bile ducts.
    • Type V: single or multiple intrahepatic ducts.
    • Diagnosis

      • Approximately 50% of children present within the first 10 years of life.
      • History and physical exam.

        • Asymptomatic jaundice.
        • Abdominal pain.
        • Jaundice.
        • Abdominal mass.
        • Cholangitis.
        • Pancreatitis.
        • Portal hypertension.
        • Hepatic abscess.
        • Cyst rupture.

      • Ultrasonography.
      • Hepatobiliary scintigraphy.
      • Transhepatic cholangiography.
      • Magnetic resonance cholangiopancreatography (MRCP).
      • Endoscopic retrograde cholangiopancreatography. Superior definition of intrahepatic and extrahepatic ductal disease.

    • Management
      • Cyst excision

        • Entire cyst excision when possible.
        • Shelling out the inner wall and cyst contents.
        • It is important to identify the entrance of the pancreatic duct into the biliary tree before the excision is complete.

      • Choledochojejunostomy.
      • Hepatic resection. Disease is intrahepatic and limited to a lobe or segment of the liver.
      • Liver transplantation. For diffuse intrahepatic disease.



VII. Groin Masses
A. Indirect inguinal hernias

  • Approximately 1% to 5% of children are affected.
  • Boys are affected more than girls, with an 8:1 ratio.
  • Prematurity increases the incidence of inguinal hernia to between 7% and 30%.
  • Incidence of bilateral hernias ranges from 10% to 40%.
  • Bilateral hernias occur more frequently in premature infants and in girls.


  • Diagnosis. History and physical examination:
    • Groin bulge extending toward the scrotum or vulva either by history or observation.
    • Sometimes reproduced only when child laughs, cries, or stands.
    • Thickened spermatic cord.

  • Management

    • Reducible hernias

      • Elective repair with high ligation of sac through a low abdominal incision.
      • Hernia sac is anterior and medial to the spermatic cord in boys and more difficult to locate among the muscle fibers running through the external ring in girls.
      • Sac can contain small bowel, omentum, or ovary.

    • Incarcerated hernias

      • Most can be reduced with gentle direct pressure on the hernia.
      • Simultaneously applying caudal traction on the testicle in addition to direct pressure on the hernia may be necessary in some cases.

    • Strangulated hernias

      • Emergent operative repair is indicated.
      • Even if a severely incarcerated/strangulated hernia is reduced, the child should be admitted and scheduled for urgent herniorrhaphy.
      • If viability of sac contents is in question, the bowel must be examined before abdominal closure.



B. Hydroceles

  • Fluid collections within the processus vaginalis that envelop the testicle.
  • Occur in approximately 6% of full-term male newborns.


  • Communicating

    • Free flow of peritoneal cavity fluid down to scrotum.
    • Processus vaginalis is patent.
    • Must be regarded as a hernia, with elective repair encouraged to prevent subsequent incarceration.

  • Noncommunicating

    • A portion of the processus vaginalis obliterates normally.
    • Fluid remains confined to the scrotum.
    • Usually self-limiting and resolve in 6 to 12 months.


VIII. Abdominal Pain

  • Common complaint in the pediatric age group (see Table 32-4)
  • Differential diagnosis must take the following into consideration:
    • Age.
    • Gender.
    • Duration of symptoms.
    • Circumstances at onset.
    • Modifying factors.

  • History of the present illness

    • This is often difficult to obtain from a child; therefore, parents should be present to corroborate accurate information.


 
TABLE 32-4 Etiologies of Pediatric Abdominal Pain
Very common causes
Less common causes
Rare causes
Acute appendicitis
Intussusception
Henoch-Schönlein purpura
Viral infection
Lower lobe pneumonia
Nephrotic syndromes
Gastroenteritis
Intestinal obstruction
Pancreatitis
Constipation
Urinary tract obstruction
Hepatitis
Genitourinary tract infection
Inguinal hernia
Diabetic ketoacidosis
Trauma
Meckel diverticulum
Lead poisoning
Cholecystitis
Acute porphyria
Intra-abdominal mass
Herpes zoster
Sickle cell anemia


    • Characteristics of pain:

      • Quality (sharp/dull, episodic/constant).
      • Onset.
      • Location.
      • Duration.
      • Presence of exacerbating or relieving factors.

    • Associated symptoms:

      • Emesis (bilious/nonbilious, bloody/foul).
      • Diarrhea/constipation.
      • Melena/hematochezia.
      • Fever.


  • Physical examination

    • General toxic appearance.
    • Patient's ease and comfort are integral to a thorough and accurate physical exam.
    • Elicit presence of peritonitis

      • Palpation.
      • Percussion.
      • Manipulation of the hip.
      • Deep respiratory movements.
      • Rectal examination.

    • Palpate masses.
    • Localize pain.
    • Bimanual pelvic exam in age appropriate patient.


IX. Tumors and Neoplasms
A. Neuroblastoma

  • Neoplasm of the sympathochromaffin system.
  • Incidence of approximately 8 new cases per million children per year.
  • Most common extracranial tumor of childhood.
  • Accounts for 10% of all pediatric malignancies.
  • Median age at diagnosis is 2 years, with 85% of the tumors being diagnosed before age 5 years.


  • Diagnosis

    • Incidental finding on radiographic studies performed for other reasons.
    • Detection of abdominal mass by the parents or pediatrician.
    • Rarely, children present with symptoms of fever, malaise, or abdominal pain.
    • At the time of discovery, up to 75% of neuroblastomas are metastatic.
    • Regional lymph nodes.
    • Liver.
    • Skin.
    • Bone.
    • Orbits.


 
TABLE 32-5 International Neuroblastoma Staging System
Stage
Characteristics
1
Localized tumor confined to the area of origin; complete gross excision with or without microscopic residual disease; identifiable ipsilateral and contralateral lymph nodes negative microscopically
2A
Unilateral tumor with incomplete gross excision; identifiable ipsilateral and contralateral lymph nodes negative microscopically
2B
Unilateral tumor with complete or incomplete gross excision; with positive ipsilateral regional lymph nodes; identifiable contralateral lymph nodes negative microscopically
3
Tumor infiltrating across the midline with or without regional lymph node involvement; or unilateral tumor with contralateral regional lymph node involvement; or midline tumor with bilateral tumor involvement
4
Dissemination of tumor to distant lymph nodes, bone, bone marrow, liver, or other organs (except as defined in stage 4S)
4S
Localized primary tumor as defined for stage 1 or 2 with dissemination limited to liver, skin, or bone marrow

  • International Neuroblastoma Staging System

    • This system places patients in low-, intermediate-, or high-risk groups based on age, surgical staging, and status of N-myc oncogene because these factors significantly predict outcome.
    • Children younger than 12 months at the time of diagnosis have a better prognosis for cure, whereas in older patients with disseminated disease, the prognosis remains poor.
    • Incorporates clinical, radiographic, and surgical information to define the tumor stage (Table 32-5).

      • Plain radiographs of the chest and skull.
      • Bone scan.
      • CT scan.
      • Bone marrow aspirate.
      • 131I-meta-iodobenzylguanidine (MIBG) scan.
      • Operative evaluation may also be necessary for accurate staging.


  • Surgical treatment
    • Local disease

      • Complete excision of the tumor.
      • Lymph node sampling.

    • Bulky or metastatic disease
      • Tumor biopsy.
      • Chemotherapy.
      • Radiotherapy.
      • If tumor shrinkage occurs with chemo and radiotherapy, delayed resection can take place.



B. Wilms tumor

  • This tumor accounts for 6% of all malignancies in children.
  • It is the most common renal malignancy in children.
  • Most children are diagnosed between 1 and 3 years of age.
  • Annual incidence is approximately 5 to 7.8 per 1 million children younger than 15 years.
  • Bilateral in 5% of cases.
  • Gender distribution is equal.


  • Diagnosis
    • History

      • Abdominal pain.
      • Fever.
      • Hematuria.
      • Urinary tract infection.

    • Physical examination

      • 85% of these children have a palpable flank or abdominal mass.
      • Associated anomalies:

        • Hemihypertrophy (2%).
        • Aniridia (1%).
        • Genitourinary anomalies (5%).


    • Abdominal ultrasonography

      • Distinguish an intrarenal mass (Wilms tumor) from an extrarenal mass (neuroblastoma).
      • Rule out tumor extension into the renal vein or vena cava.

    • Abdominal and chest CT scans

      • Necessary for staging.
      • Evaluate contralateral kidney.
      • Screen for pulmonary metastases.

    • Histologic examination confirms the diagnosis.

  • Management
    • Surgery and chemotherapy together result in a better than 90% chance of cure.
    • Surgical intervention.

      • Radical nephrectomy.
      • Sample para-aortic lymph nodes.
      • Isolation of the hilar vessels and examination of the contralateral kidney also are required.
      • If the Wilms tumor is found initially to be unresectable because of size or bilaterality, a second-look operation can be done after chemotherapy.

    • Chemotherapy. Vincristine, doxorubicin, and dactinomycin are used, depending on the stage of the Wilms tumor.
    • Radiotherapy is used for advanced stages of Wilms tumor.


C. Hepatic tumors

  • These make up fewer than 5% of all intra-abdominal malignancies.
  • They are malignant in 70% of cases.
  • Hepatoblastoma

    • 39% of liver tumors.
    • 90% occur before 3 years of age.
    • 60% are diagnosed by 1 year of age.

  • Hepatocellular carcinoma

    • Presents in older children.
    • Approximately one third of these patients have cirrhosis secondary to an inherited metabolic abnormality.



  • Diagnosis
    • History and physical exam

      • Abdominal pain.
      • Enlarging abdominal mass.

    • Elevated serum AFP
    • Radiology
      • CT or MR scan.
      • Angiography.


  • Management

    • Surgical intervention

      • Primary tumor resection and lymph node sampling. Intraoperative histologic analysis of the liver margins is necessary to confirm complete removal of the tumor.
      • Hepatoblastoma that is not initially resectable undergoes chemotherapy and reexploration for curative resection.



D. Teratomas

  • These are composed of tissues from all germ layers (endoderm, ectoderm, and mesoderm).
  • In neonates, sacrococcygeal teratomas are the most common.
  • They are more common in girls (4:1).


  • Diagnosis

    • Prenatal/antenatal ultrasound.
    • CT scan.
    • Rectal exam.

  • Management

    • Antenatal diagnosis may necessitate delivery by cesarean section.
    • Surgical intervention:

      • Resection through a chevron-shaped buttocks incision during the first week of life.
      • Principles important in resection of the tumor include preservation of the rectal sphincter muscles, resection of the coccyx with the tumor, and early control of the midsacral vessels that supply the tumor.
      • May require combined abdominal and perineal approach for large intra-abdominal teratomas.

    • Chemotherapy for malignant teratomas. May shrink the tumor and allow for resection.

  • Complications

    • Hemorrhage.
    • Recurrence. High rate of recurrence if the coccyx incompletely resected.
    • Malignancy

      • Rarely seen in neonates but increases with the age of the child.
      • If the tumor is malignant, a thorough search for metastases is needed.



E. Soft-tissue sarcomas

  • These account for 6% of childhood malignancies.
  • Greater than one half are rhabdomyosarcomas.


  • Diagnosis
    • CT scan.
    • MR scan.
    • Incisional biopsy usually is required to determine the histologic type preoperatively.

  • Management

    • Consultation with a radiotherapist and oncologist before initiating therapy is advised.
    • Nonrhabdomyosarcomas. Wide surgical excision.
    • Rhabdomyosarcoma

      • Treatment is determined by the location of the tumor.
      • Complete resection of head and neck tumors is rarely possible, and they usually are treated with biopsy followed by chemotherapy.
      • Trunk and retroperitoneal tumors are treated with wide excision.
      • Extremity tumors also are treated with wide excision, but resection of muscle groups and the use of radiotherapy or brachytherapy should also be considered (Surg Clin North Am 1992;72:1417).
      • A biopsy of the regional lymph nodes should be included in all procedures.



X. Fetal Surgery
Most disorders diagnosed in the antenatal period are best managed after birth. However, a few disorders with devastating developmental consequences may benefit from fetal treatment. The fundamental conflict in fetal surgery is balancing the risks to both mother and fetus against the potential benefit to only the fetus. Over two decades of rigorous groundwork has resulted in safe surgical intervention for several anomalies.

  • Congenital diaphragmatic hernia

    • To promote growth of the lungs and reverse the effects of potentially lethal pulmonary hypoplasia.
    • Method of repair.

      • Tracheal balloon occlusion with lung distension

        • Eligibility criteria: CDH with liver in chest and a ratio of lung to head of less than 1.4.
        • Pilot study survival: 75% versus 45% to 50% controls repaired after birth.



  • Obstructive uropathy

    • Posterior urethral valves lead to oligohydramnios, pulmonary hypoplasia, and death.
    • Percutaneous vesicoamniotic shunt placement.

  • Congenital cystic adenomatoid malformation

    • Most fetuses diagnosed with a cystic pulmonary lesion either undergo surveillance with spontaneous resolution or require neonatal treatment.
    • A small subset with large lung lesions who become hydropic and deteriorate may benefit from maternal hysterotomy, fetal thoracotomy, and resection of the mass.

  • Sacrococcygeal teratoma

    • High blood flow into the tumor can result in cardiac failure and fetal hydrops.
    • Maternal hysterotomy and resection can be lifesaving.
    • Recently, radiofrequency ablation of tumor's arterial flow has shown promise, but it carries a high risk to the fetus (Am J Obstet Gynecol 2001;184:503).

  • Twin–twin transfusion syndrome

    • Placental vascular connections result in one twin “stealing” the blood supply from the other twin, resulting in death of both twins.
    • Randomized, controlled trials are under way in both Europe and the United States comparing fetoscopic laser ablation of abnormal placental vessels with amnioreduction (Am J Obstet Gynecol 2001;185:708).


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