1. Neonatal Surgical Disorders
                                                                        

2. Esophageal Atresia/Tracheo-esophageal Fistula
Esophageal Atresia (EA) represents a failure of the esophagus to develop as a continuous passage. Instead, it ends as a blind pouch.
Tracheoesophageal Fistula (TEF) represents an abnormal opening between the trachea and esophagus.
EA and TEF can occur separately or together.
                                                                        

3. Esophageal Atresia/Tracheo-esophageal Fistula
At the beginning of a pregnancy, the trachea and the esophagus are one single tube, called the primitive foregut.  Between the 23 rd and the 28th day of pregnancy, a time when many women are not even sure they are pregnant, this single tube divides into two tubes – the trachea and the esophagus. When something goes wrong with this division, as it does in approximately one out of every 3,500 – 4,000 births, the baby is born with a medical condition known as esophageal atresia and/or tracheoesophageal fistula. Esophageal atresia with tracheoesophageal fistula occurs in one of 3,000 to 5,000 births.

4. EA/TEF Type A- EA without a TEF
Type B- Has a connection (fistula) between the upper pouch and the trachea
Type C – Has a fistula between the lower esophagus and the Trachea (Most Common)
Type D –Has two TEFs, one between both the upper and lower esophageal segments and the trachea (Very rare)
Type E- has only a TEF and no EA. (H type fistula)

5. In type A atresia, both esophageal segments are blind pouches, and neither connected to the trachea.  It maybe termed as pure esophageal atresia and may be considered either short or long gap, depending on the space between the segments.  An infant born with pure esophageal atresia may appear normal at birth, but as they swallow, secretions fill the esophageal pouch, causing an overflow into the oropharynx, and the infant will drool excessively.  When the infant is fed, vomiting and respiratory distress follow (aspiration).  Continual suctioning temporarily relieves these symptoms.

6. In type E (or H-type tracheoesophageal fistula without atresia) the fistula may occur anywhere between the level of the cricoid cartilage and the mid-esophagus.  However, the fistula is usually higher in the trachea than in the esophagus.  A fistula such as this may be as small as a pinpoint.  Symptoms that may signal this H-type fistula are repeated episodes of lung inflammation (pneumonitis), infection of the lungs or respiratory system (pulmonary infection), and swollen abdomen (abdominal distention). 

7. In types B and D, the upper portion of the esophagus opens into the trachea, a life-threatening condition since an infant with this anomaly can aspirate saliva or food into the trachea.  Both type B (proximal fistula and type D (fistula to both segments) cause immediate inhalation of saliva into the trachea airway and bacterial pneumonitis

8. The most common tracheoesophageal fistula is Type C, which includes esophageal atresia. The upper section of the esophagus ends in a blind pouch, and the lower section ascends from the stomach and connects with the trachea by a short fistulous tract.  A newborn with tracheoesophageal fistula with esophageal atresia appears to swallow normally, but soon after swallowing coughs, struggles, becomes cyanotic (skin turns blue in color), and stops breathing since he is inhaling aspirating) fluid returning from the blind pouch of the esophagus through his nose and mouth. 

9. EA/TEF Clinical Presentation
Maternal History of polyhydramnios
At birth infant usually pink with good apgars then noted to have:
Excessive secretions
Coughing, chocking episodes
Cyanosis with feeding attempts
May have abdominal distention
Additional associated anomalies noted on exam
Unable to pass feeding tube

10. EA/TEF Diagnosis
Attempt placement of a 10 or 12 fr feeding tube
Xray

12. Pre-op care Repogle to constant suction NPO, IV fluids Antibiotics
Echocardiogram to identify arch preference
X-ray to identify skeletal anomalies
Start vater/vacterl work-up

13. EA/TEF Associated anomalies
V – vertebral abnormalities (examples: extra ribs, bifid vertebrae)
A – anal anomalies (examples: imperforate anus, uritogenitary anomalies)
C – cardiac, or heart, abnormalities (examples: Atrial septal defect, ventricular septal defect, holes in the heart, missing arteries such as the pulmonary artery.
TE – tracheo-esophageal anomalies (examples: esophageal atresia, tracheoesophageal fistula, esophagus attaches to the trachea)
R – renal anomalies (examples: missing kidney, horseshoe kidney, multiple kidneys)
L – limb abnormalities (examples: radial bone abnormalities, missing bones in the arm or arms, missing finger, missing bones in the leg and/or toes)

14. Many babies born with this defect have other medical problems – all associated with organs that were being formed during the same period at the beginning of the pregnancy.  At least 25% of these babies are premature, and 25% have additional critical problems such as congential heart disease, Down's Syndrome*, hydronephrosis*, duodenal atresia*, and tracheomalacia*.  Ten percent of the babies have an imperforate anus*, and one-half have vertebral or skeletal anomalies.  Researchers found that it was not uncommon to have vertebral (V), anal (A), tracheo-esophageal (TE), and/or radial limb or renal (kidney) (R) anomalies, which was abbreviated as VATER syndrome (or VATER association) by Quan and Smith in 1973.  This syndrome is also currently being termed as VACTERL, the C referring to cardiovascular problems, and the L to limb defects, because of the high percentage of children with VATER syndrome born with these latter anomalies.

15. Contrast Studies
Water (Isotonic) - useful when to evaluate anastomoses after surgery.
Barium – caution with TEF- pulmonary aspiration
Air

16. Long gap EA

17. Swallow study of H Type Fistula

18. EA/TEF Outcomes Esophageal dysmotility GERD Risk of strictures
H2 blockers
Surgical intervention
Risk of strictures
Barrett’s Esophagus

19. EA/TEF Surgical Repair
Delayed Repair
Bronchoscopy, Thoracotomy, Fistula ligation, G-tube
Await weight gain 6-8 wks
Return for primary repair
Gastric transposition
Colonic interposition
Esophagosotmy
Primary Repair
Bronchoscopy, Thoracotomy, Fistula ligation, primary anastamosis
Chest tube, Post anastamosis feeding tube
Swallow study at day 5

20. Post-Op Care ET Intubation Suctioning Chest tube and Drain
Gastric Decompression—Protect
Enteral Feeds
Water Soluble Contrast Study

21. EA/TEF Outcomes Esophageal dysmotility GERD Risk of strictures
H2 blockers
Surgical intervention
Risk of strictures
Barrett’s Esophagus

22. GASTROINTESTINAL COMPLAINTS

23. Regurgitation and Vomiting
Lose up to 10% of their birthweight in the first week of life
If weight gain is appropriate, then intake is adequate
Observe the caregiver feeding the baby
Small amounts of regurgitation are common

24. Vomiting
Vomiting refers to a forceful ejection of gastric diaphragmatic and abdominal muscle contraction, resulting in expulsion of gastric contents from the mouth
Projectile vomiting
Bilious vomiting (NEVER NORMAL)

25. Regurgitation
Regurgitation is defined as passive, retrograde movement of ingested material. (Spitting)

26. Pyloric Stenosis
Hypertrophic pyloric stenosis (HPS) affects approximately three of every 1,000 live births.
Also known as infantile hypertrophic pyloric stenosis (IHPS) and is the most common cause of intestinal obstruction in infancy.
The hallmark of pyloric stenosis is "projectile“ vomiting that occurs shortly after feeding, and consists of partially digested formula or milk.
More common in whites than Hispanics, blacks, or Asians. The incidence is 2.4 per 1000 live births in whites, 1.8 in Hispanics, 0.7 in blacks, and 0.6 in Asians. It is also less common amongst children of mixed race parents.
Male-to-female predominance of 4:1, with 30% of patients with infantile hypertrophic pyloric stenosis being first-born males.
presentation is approximately 3 weeks of life (1-18 wk). Approximately 95% of infantile hypertrophic pyloric stenosis cases are diagnosed in those aged 3-12 weeks.

27. Pyloric Stenosis 1 in 250 live births Male predominance
Infantile pyloric stenosis is the most common condition requiring surgical intervention in the first year of life.
1 in 250 live births
Male predominance
Caucasians highest incidence
2-4 cases / 1000 live births
Onset: 1st week of life to as late as 5 months
Peak Incidence: 3-5 weeks

29. Pyloric Stenosis Presentation
Nonbilious vomiting
May or may not be projectile
Progressive
Leads to dehydration, weight loss and failure to thrive
Vomiting
Hydrogen ion loss leads to an elevation of serum bicarbonate, followed by a decrease in serum chloride and development of hypochloremic alkalosis

30. Diagnosis History of projectile, non bilious emesis
Palpable mass in epigastric area “olive”
UGI- elongated pyloric channel
Ultrasound – thickness >4mm, length > 16mm

31. Management Correct electrolyte imbalance Repogle to suction
Surgery is the only “cure” (pyloromyotomy)
Progressive feeding post-op
Discharge to home hrs

32. Neonatal Surgical Disorders Associated with Vomiting
Intestinal Obstruction—Atresias
Malrotation and midgut volvulus
Hirschsprung's disease (HD)
Meconium ileus (MI)
Abdominal wall defects

33. Intestinal Obstruction—Atresias
Duodenum, jejunum, ileum, and colon
1 in 2,000
Bilious vomiting is the most common presenting symptom (NEVER NORMAL)
+Abdominal distension
Increased incidence in Down’s Syndrome

34. Duodenal Abnormalities
Duodenal atresia: neonatal presentation “Double Bubble”
Annular pancreas: infants or adults
Duodenal web/diaphragm, Ladd's bands
Duplication cyst

35. Duodenal Atresia/Stenosis
The hallmark of duodenal obstruction is bilious vomiting without abdominal distention
Usually noted on the 1st day of life but stenosis can be as late as 3 weeks
A history of polyhydramnios
Jaundice is present in one third
Double-Bubble sign on KUB
+ Contrast studies

36. Duodenal Atresia
Weight loss, dehydration, and hypochloremic metabolic alkalosis are common.
Duodenal obstruction is commonly associated with Down's syndrome, esophageal atresia, and tracheoesophageal fistula.
Other associated anomalies include lymphangiomatous cysts of the mesentery, vertebral anomalies, club feet, congenital heart disease, mental retardation, and Meckel's diverticulum.

37. Treatment Nasogastric decompression initially
Intravenous fluid replacement
D101/2-NS at 150cc/kg
Call Surgery
+ Antibiotics
Echocardiogram and radiology of the chest and spine
One third of infants with duodenal atresia have associated life-threatening congenital anomalies.

38. Jejunal and ileal anomalies
Jejunal atresia
Ileal atresia
Meconium ileus

39. Small bowel Atresias Bilious emesis suggests mechanical obstruction
Presentation
Abdominal distention
Bilious emesis
Dehydration, electrolyte imbalance
Passage of meconium does not rule out
Films:
Dilated loops of bowel
Unused colon
Bilious emesis suggests mechanical obstruction
Always requires immediate evaluation

40. Pre-op Managment NPO NG to suction IV Fluids
Contrast study from above or below
Evaluate for associated anomalies
T & C
ABX

41. Post-op managment NG to suction NPO IV fluids Stoma/Wound care
In most cases slow refeeding
Monitor for feeding intolerance

42. Malrotation and Volvulus
Intestinal malrotation occurs in 1 out of every 500 live births in the United States.
Symptoms usually occur in the first year of life.
Twenty-five to 40 percent of cases are diagnosed in the first week of life.  
Fifty to 60 percent are diagnosed by the first month of life.  
Seventy-five to 90 percent are diagnosed by one year of age.
The remaining cases (10 to 25 percent) are diagnosed after one year of age.

43. Malrotation and Midgut Volvulus
Malrotation - failure to complete normal rotation and fixation of the bowel
abnormality occurring during the 8th to 12th week of fetal life
Volvulus - twisting of bowel upon itself causing obstruction and ischemia
Usually occurs at sigmoid & ileocecal areas of intestine
Leads to necrosis (tissue death) of twisted portion of the intestine due to obstruction of SMA (superior mesenteric artery)

44. Malrotation and Volvulus
Clinical presentation
vomiting bile  
drawing up the legs  
abdominal pain  
abdominal distention
rapid heart rate  
rapid breathing  
bloody stools

45. Diagnosis
Xray
UGI BE

46. Treatment Surgical Emergency NG to suction IV fluid resuscitation
Antibiotics
Laparotomy/ LADDs procedure
Possible bowel resection
Possible colostomy
Possible open/close

47. Hirschsprung's Disease
1 in 4,000
Male predominance
Present usually in 1st year of life

48. Hirschsprung’s Disease
Chronic intestinal obstruction caused by absence of ganglion cells in the distal bowel
Ganglion cells enable peristalsis of the muscles in the intestine to move food and by-products down to the rectum

49. Hirschsprung’s disease
Clinical presentation
History of constipation
Failure to pass stool within the first 48 hrs of life
Abdominal distention, poor feeding, emesis
Bilious emesis (NEVER NORMAL)

50. Hirschsprung's disease
“megacolon”
Congenital dilatation of the colon
Aganglionosis- absence of ganglion cells in the distal colon
rectosigmoid in 80%
total colonic in 10%
small bowel involvement in 10%

51. Complications Associated w/ HD
Enterocolitis
Bowel Obstruction
Chronic Constipation

52. Hirschsprung's Disease
Barium enema (80% accuracy) shows a transitional zone (funnel-shaped dilatation of bowel) at the junction for the aganglionic and ganglionic intestine.
Rectal suction biopsy (95% accuracy) shows absence of ganglion cells in the myenteric plexus, increased staining of cholinesterase, and the presence of hypertrophied nerve bundles.
There are 2 surgical approaches: performance of a colostomy in the newborn period with a definitive pull-through operation at a later date and a primary pull through operation without a colostomy.

53. Meconium Ileus 1 per 5,000/births
Present is first few hours of life but as far out as Day 2-3
15% of patients with cystic fibrosis (CF) have MI
90% of infant’s with MI have CF
MI is usually identified immediately after birth
Abdominal distention
Bilious vomiting (NEVER NORMAL)

54. Imperforate anus Occurs 1 in 5000 live births Diagnosis by exam
Contrast exam
Treatment based on level of fistula
Colostomy vs anoplasty

55. Management of I.A. Anoplasty in neonatal period
Stabilize life threatening anomalies first
Low-Type:
Anoplasty in neonatal period
High Type:
Diverting Colostomy
Pull-Through (PSARP=Posterior Sagittal Anorectoplasty)
Colostomy Closure
Anal Dilatations—most difficult aspect for parents
Skin Care

56. VACTERL Association V= Vertebral A= Anorectal C= Cardiac
TE= Tracheo-Esophageal
R= Renal
L= Limb

57. Post-op complications
Enterocolitis
Post-op intestinal obstruction
Anorectal stenosis
Fecal incontinence
Constipation

58. Abdominal wall defects
Related to the development of the cord
Omphalocele
Gastroschisis
Bladder & cloacal extrophy
Prune belly syndrome
others

59. Gastroschisis
Gastroschisis is the herniation (protrusion) of abdominal contents through the abdominal wall without involving the umbilical cord

60. Diagnosis Frequently identified by ultrasound SGA, elevated AFP
Controlled delivery is planned
Identified at birth -visual-
Prevention of heat loss/infection
Avoid kinking of bowel
IV fluids

62. Pre-op Cover and protect exposed bowel NG to suction for decompression
IV fluids 1.5-2x maintenance
Monitor bowel for changes in color

63. Associated anomalies Prematurity (55%)
Intra uterine growth retardation (77%)
Cardiac problems
Minor malformations in small bowel, may be a part of the Gastroschisis itself
Intestine shortened to as much as one third of the normal length.

64. Post-op Silo placed Monitor bowel color Monitor resp status
Monitor s/s infection
Pain management/sedation
Following closure
Respiratory status (often intubated)
Urine output
Swelling/compartment syndrome
Infection
Gravity will help reduce intestinal contents.
Gradual reduction by sequential tightening of the silo. Reduce over 3-5 days.

65. Outcomes
Slow to feed
Long term HAL/IL
Failure to thrive
Malabsorption

66. Gastroschisis vs Omphalocele
Incidence: 1 in 3,200-10,000 births
Prenatal diagnosis: Ultrasound, needs echo and amnio
Delivery: vaginal or C-section d
Sac: present, may be torn
Herniated bowel: normal
Prematurity: 10-20%
IUGR: Less common
Malabsorptiom: only if sac is ruptured
Associated anomalies: 45-55%
Gastorschisis
Incidence: 1 in 4,000-10,000 births
Prenatal diagnosis: Ultrasound, elevated AFP
Delivery: C-section debated
Sac: Absent
Herniated bowel: edematous, matted
Prematurity: 50-60%
IUGR: Common
Malabsorptiom: Common
Associated anomalies: 10-15%

67. Omphalocele
Omphalocele is a congenital ventral wall defect that involves herniation of a portion of the abdominal organs into the base of the umbilical cord. This membrane-covered defect can range in size from small, containing only a portion of the bowel, to large, containing most of the abdominal organs
.Approximately 1 in 5,000 babies
1 in 10,000 births have liver involvement

68. Delivery room Vaginal vs c-section Sterile gloves when handling
If giant careful handling to avoid rupturing
antibiotics

72. Pre-op Small Staged reduction if needed Respiratory support IV fluids
ABX
Pain/sedation
NG to decompress
Giant
Long term reduction by gravity (several months)
HAL/IL nutrition eval
Careful dressing changes and handling
Sedation/pain
PT/OT
Special mattress

73. Post-op Respiratory support Pain management
Monitoring of site for breakdown
U/O, swelling of lower extremities, decreased perfusion

74. Sacrococcygeal teratoma
Sacrococcygeal Teratoma (SCT) is the most common tumor of the newborn, which occurs in 1 out of every 35,000 to 40,000 live births

75. Delivery Room C-section Controlled delivery
Neonatology and surgery at delivery
Blood available
Careful handling of mass
dystocia and risk of tumor rupture and hemorrhage

76. Pre-op Echo T &C IV fluids Alphafetoprotein (AFP)
Careful handling of mass

77. Post-op Wound monitoring HUS IV fluids until ready to eat
Pain management
Orthopedic/urology consults