Pediatric surgery

• Tracheoesophageal fistula & Esophageal Atresia

  • Common (1 in 3,000-3,500 births)
  • Relatively common (1 in 4,000-5,000 births)
  • Relatively rare (1 in 10,000-20,000 births)
  • Rare (1 in 100,000-1,000,000 births)
• Tracheoesophageal fistula & Esophageal Atresia

  • Incidence: Common
  • Incidence: 1 in 4,500 births
  • Congenital anomalies - sporadic or familial
  • Male slightly more than female (1.26:1)
• Etiology


  Many other abnormalities (association) + embryogenesis + environmental
• Classification (Gross-Voyt)


  • Type A: Proximal atresia, no fistula, long gap between proximal and distal esophagus
  • Type B: Proximal fistula and atresia
  • Type C: Atresia with distal fistula (most common type, 85-92%)
  • Type D: Two fistulas, proximal and distal
  • Type E: No atresia, with fistula
• Gastrostomy


  Procedure to create an opening in the stomach
• Esophagostomy


  Procedure to create an opening in the esophagus
• Air in stomach indicate distal fistula
• Clinical Presentation


  • Excessive salivation shortly after birth
  • Choking, gagging, cyanosis during feeding
  • Reflux of gastric contents into airway leading to tachypnea, atelectasis, respiratory distress, pneumonitis
  • Abdominal distension from air entering via fistula, respiratory distress, pulmonary compromise
• Diagnosis


  1. Inability to pass NG tube 11-12cm

  2. Baby gram X-ray to see proximal esophageal coiling

  3. Screening for other anomalies

  4. Echocardiography

  5. Chromosomal analysis
• Treatment


  1. Insert sump catheter/sucker to drain saliva

  2. Position baby with head elevated

  3. Surgical repair of fistula (if Type C, anastomosis; if Type A, esophageal replacement)
• Hirschsprung's Disease


  Congenital megacolon caused by migratory failure of neuronal crest cells
• Hirschsprung's Disease


  • Incidence: relatively common, 1 in 5,000 births
  • Etiology: unknown
  • Mostly affects rectosigmoid region (75%)
  • Females & children with Down syndrome have longer segment aganglionosis (15%)
  • 10% affect entire colon and terminal ileum (total colonic aganglionosis)
• Pathophysiology


  • Aganglionosis leads to functional obstruction, megacolon, and distension
  • Transition zone between normal and aganglionic colon is narrow and cone-shaped
  • Leads to stasis, bacterial overgrowth, enterocolitis
• Clinical Presentation


  • Failure to pass meconium (20%)
  • Abdominal distension
  • Bilious vomiting
  • Possible development of obstructive enterocolitis
  • Chronic constipation if undiagnosed until age 2
• Diagnosis


  1. Barium enema shows dilated colon and transition zone

  2. Anorectal manometry shows abnormal relaxation

  3. Rectal biopsy shows absence of ganglion cells and nerve hypertrophy
• Treatment


  Surgical removal of aganglionic segment and pull-through of healthy ganglionated bowel to anus
• Abdominal Wall Defects


  Omphalocele and gastroschisis are common congenital abdominal defects
• Omphalocele


  • Central defect beneath umbilical ring, greater than 4cm
  • Umbilical cord inserts directly into sac
  • Contains abdominal viscera including liver, bladder, ovary, stomach
  • Associated with other anomalies (81%)
• Gastroschisis


  • Isolated opening in right side of abdomen, 95% of cases
  • Free evisceration of bowel without sac
  • Bowel exposed to amniotic fluid leading to fibrosis, shortening, thickening
  • Uncommon associated anomalies
• Differences between Omphalocele and Gastroschisis

  • Omphalocele: Bowel + liver in sac, umbilical cord insertion
  • Gastroschisis: Bowel only, no sac, right of umbilicus
• Treatment


  1. Omphalocele: Primary closure if small/medium, prosthetic patch if giant

  2. Gastroschisis: Gradual reduction of bowel, staged closure
• Successful outcomes depend on postnatal management (fluids, antibiotics, ventilation)
• Teratoma


  • Incidence: 1 in 20,000-40,000 live births
  • Majority are sacrococcygeal teratomas (45-65% of cases)
• التاريخ


  5.1
• Omphalocele


  Small & Medium Primary closure is Preferred including excision of the Sac
• Treatment for Giant omphalocele

  1. Topical application (Betadin ointment or Silver sulfadiazin)

  2. Epidermal ingrowth & Secondary eschar formation

  3. Repair of Incisional Abdominal hernia
• Treatment for Gastroschisis


  1. Gently return the Content to its place

  2. Stage closure

  3. Flap

  4. Prosthetic Patch
• Successful Outcomes are important and necessary
• After Surgery, nutrition (IV fluid), antibiotics, and ventilator are important
• Teratoma


  Benign tumor with tissues from all three embryonic layers
• Incidence of Teratoma is 1:20,000 - 40,000 live births, with Sacrococcygeal teratoma being the majority of cases (45-65%)
• Sacrococcygeal teratoma


  • Can be well differentiated or non-differentiated
  • Can be discovered prenatally by ultrasound
  • Can include retrorectal or retroperitoneal components
• Nephroblastoma (Wilms tumor)


  Most common solid renal tumor of childhood, accounting for roughly 5% of childhood cancers
• Nephroblastoma


  • Peak age for presentation is during the third year of life
  • Occurs in either kidney with equal frequency, with 5% of cases being bilateral
• Diagnosis of Nephroblastoma


  1. Ultrasound

  2. Physical examination to determine nature of lesion (Solid vs Cystic)

  3. Presence of intra-abdominal component including liver

  4. Tumor markers (AFP & βhCG)
• Treatment of Nephroblastoma


  1. Complete excision of the tumor with the coccyx

  2. Ligation of middle sacral artery
• Wilms Tumor can be associated with various syndromes like Beckwith-Wiedemann, WAGR, and Denys-Drash
• Neuroblastoma


  Malignant tumor originating from sympathetic nervous system cells
• Neuroblastoma


  • Can arise from adrenal medulla, chest, neck, pelvis, and abdomen
  • Can cause symptoms like anemia and bone pain
• Biopsy is contraindicated for Neuroblastoma, excision is preferred