Pediatric Surgery

1
Pediatric Surgery

• Vic V. Vernenkar, D.O.
• St. Barnabas Hospital
• Dept. of Surgery

2
Epiglottitis

• H. Influenza is most common organism
• A lateral xray may show edema of the epiglottis
  (birds beak)
• Orotracheal intubation should be performed in the
  OR so that an open tracheostomy can be done if
  needed.
• Never nasotracheally intubate a child because the
  angle between the superior and inferior glottis
  is too large.

3
Tracheoesophageal Fistula

• A newborn infant has excessive salivation,
  choking, and regurgitation with feeding.
• Results from abnormal ingrowth of ectodermal
  ridges during the 4th week of gestation.
• 25-40 of neonates are premature, low bith
  weight.
• A maternal history of polyhydramnios is common
• 50 of neonates with TEF have an associated
  anomaly (cardiovascular most common)
• GI malformation
• GU anomalies
• Skeletal
• CNS
• Associated with VACTERL (vertebral, anorectal,
  cardiac, TE, renal, Limb)
• 5 types of TEF
• Proximal esophageal atresia with distal TEF (85)
• Isolated esophageal atresia
• H-type TEF without atresia
• Proximal TEF with distal atresia

4
Tracheoesophageal Fistula

• Proximal and distal TEF
• Diagnosis
• Inability to pass nasogastric tube
• CXR to deternime length of esophageal gap
• Abdominal Xray with air in the stomach excludes
  esophageal atresia
• Treatment
• Right thoracotomy thru 4th intercostal space
• Proximal esophagus blood supply is from
  thyrocervical trunk

5
Tracheoesophageal Fistula

• Distally supplied by more tenuous intercostals
• Operation includes TEF ligation, transection, and
  restoration with end-to-end anastamosis.
• POD 5-7 esophagram, if no leak, feed, remove
  drain.

6
Tracheoesophageal Fistula

• Early complications include
• Anastamotic leak, recurrent TEF, tracheomalacia.
• Late Complications include
• Anastamotic stricture (25), reflux (50),
  dysmotility (100).
• Proximal atresia with distal TEF most common.

7
Immunoglobulins

• Which immunoglobulin is secreted in breast milk?
• Which immunoglobulin does not cross blood brain
  barrier?
• IGA is most common antibody in breast milk, the
  gut, saliva, bodily secretions.
• IGM is large and does not cross the placenta.

8
Resuscitation

• An 8 year old boy presents following a bicycle
  crash with a ruptured spleen.What is the best
  indicator of early shock?
• Tachycardia in childhood is defined as a heart
  rate 150 for a neonate, 120 in the first year,
  100 after one year. It is the best indicator of
  shock.
• Fluid resuscitation in children

9
Resuscitation

• 20cc/kg crystalloid bolus for trauma
• If shock persists after a second bolus, give
  blood at 10cc/kg
• Children have a lower GFR in comparison to
  adults.

10
Malrotation

• A healthy infant presents with bilious vomiting,
  abdominal distension, and shock.
• A surgical emergency, bilious vomiting in a
  newborn is malrotation until proven otherwise.

11
Malrotation

• During 6-12 week of gestation, the intestine
  undergoes evisceration, elongation, and eventual
  return to the abdominal cavity in a 270 degree
  counterclockwise rotation with fixation.
• Malrotation is associated with abnormal rotation
  and fixation.

12
Malrotation

• Ladds bands extend from the colon to the
  duodenum, causing duodenal obstruction and
  biliary emesis.
• Midgut volvulus refers to the narrow based
  mesentery twisting around the SMA (usually
  clockwise).
• This results in obstruction and vascular
  compromise.

13
Malrotation

• Most develop symptoms in first month of life.
• If patient stable do UGI series (gold standard).
• See birds beak in third part of duodenum
• Ligament of Treitz is right of midline.

14
Malrotation

• Midgut volvulus is a surgical emergency.
• Volume resuscitation is essential.
• If patient in shock, no studies are warranted.

15
Malrotation

• Immediate exploration to avoid loss of small
  bowel and resultant SBS, death.
• Surgical treatment is the Ladds procedure.
• This consists of division of bands, correction of
  malrotation, restoration of broad based
  mesentery, appendectomy (because it is in the
  wrong place in LUQ).

16
Duodenal Atresia

• A newborn full term neonate with Downs syndrome
  had bilious vomiting during the first day of
  life. The abdominal exam is normal.
• Duodenal atresia. Malrotation can present
  similarly but less common.
• Failure of recanalization during 8-10th week of
  gestation.
• Presents in first 24hrs of life.
• Trisomy 21 is present in about 25
• Characterized by bilious emesis
• Abdominal distension is absent
• 85 distal to ampulla of vater

17
Duodenal Atresia

• Check for patent anus
• Rule out anorectal anomalies
• Abdominal x-ray reveals double bubble sign
• Air in the stomach, and 1st and 2nd portions of
  duodenum.
• If there is no distal air, the diagnosis is
  secure.
• If there is distal air, and urgent UGI needed to
  rule out midgut volvulus.
• Surgical treatment is a duodenoduodenostomy.

18
Jejunoileal Atresia

• A 3 day old infant has bilious vomiting,
  abdominal distension.
• Differential includes
• Duodenal atresia
• Malrotation
• Meconium ileus
• Imperforate anus
• Hirchsprungs

19
Jejunoileal Atresia

• Jejunoileal atresia is caused by an in utero
  vascular accident.
• Presents within first 2-3 days
• Associated with Cystic Fibrosis in 10

20
Jejunoileal Atresia

• Abdominal distension is usually present with
  distal atresia.
• Abd. X-ray demonstrates multiple distended loops
  of bowel with A-F levels
• Contrast enema demonstrates a micro colon and no
  reflux into dilated intestines.
• Multiple areas of involvement in 10.
• Surgical correction involves end-to end
  anastamosis. Preserve length to prevent SBS.

21
Colonic Atresia

• Caused by in utero mesenteric vascular accident.
• Similar to above in presentation
• Abdominal distention present
• X-rays show obstructive picture
• Contrast enema shows microcolon with a cut off in
  proximal colon.
• Surgical correction involves end-to-end
  anastamosis.
• Intestinal atresia can be associated with
  gastrochisis.

22
Meconium Ileus

• A newborn with cystic fibrosis presents with mild
  abdominal distension. An X-ray demonstrates a
  ground glass appearing mass on the right side of
  the abdomen.
• A gastrograffin enema may be all that is needed
  to treat meconium ileus, complicated cases may
  need surgery.
• Caused by obstruction of terminal ileum with
  meconium.

23
Meconium Ileus

• 15 have CF
• Simple MI can be treated with enemas.
• Complex MI requires enterotomy, resection,
  evacuation.
• Mucomyst enemas can be used for this.
  (N-acetylcysteine)

24
Hirchsprungs Disease

• A full-term neonate has bilious emesis during
  first and second days of life. The abdomen is
  distended. X-rays show dilated loops of small
  bowel. A contrast enema reveals a narrow rectum,
  compared to the sigmoid. The baby failed to
  evacuate the contrast the following day.
• A bedside suction rectal biopsy at least 2cm
  above dentate line is the gold standard test.

25
Hirchsprungs Disease

• Failure of the normal migration of neural crest
  cells.
• Absent ganglia in the myenteric and submucosal
  plexus.
• The absence always occurs in the distal rectum
  and extends proximally.
• 80-85 localized to rectosigmoid.

26
Hirchsprungs Disease

• Diagnostic work-up includes
• Contrast enema showing a contracted rectum with
  dilated bowel above.
• Failure to evacuate contrast 24h later can be
  diagnostic.

27
Hirchsprungs Disease

• Rectal biopsy is required to confirm absence of
  ganglion cells and nerve hypertrophy.
• Surgical treatment
• Soave endo-rectal pull through with removal of
  the diseased distal bowel with coloanal
  anastamosis

28
Hirchsprungs Disease

• Children who present acutely ill may need staged
  procedure with colostomy.
• Need to do intraoperative frozen section to help
  determine the anatomic location of transition
  zone.

29
Imperforate Anus

• Anus absent or misplaced
• Usually form anterior fistulous tract
• Associated with coloanal deformity

30
Imperforate Anus

• Divided into high and low malformations with
  respect to the levators.
• High fistula to bladder, vagina, or urethra, are
  treated with colostomy and posterior sagital
  anorectoplasty (PSARP), and genitourinary
  reconstruction if necessary.
• Low PSARP

31
Imperforate Anus

• Preop anal dilatation may be needed to prevent
  stricture.
• A colostomy is generally not needed to treat a
  low (below levator) imperforate anus.

32
NEC

• A 7 day old premature infant has emesis,
  abdominal distension, and bloody stools.
• Differential includes
• NEC
• Malrotation

33
NEC

• More common in premature infants after initiation
  of feeding.
• Abdominal x-ray often reveals pneumotosis
  intestinalis or portal vein air.
• Treatment is conservative NPO, NGT, antibiotics,
  serial x-rays.
• Medical management is successful in 50 of cases.

34
NEC

• Surgical treatment for
• Free air
• Abdominal wall erythema, cellulitis
• Worsening acidosis
• Hyperkalemia
• Palpable mass
• Worsening distension
• Overall deterioration

35
NEC

• Surgery often involves resection of affected
  intestine and creation of and end ileostomy and
  mucous fistula.
• If the neonate survives, reverse around 4-6 weeks
  later after a contrast study rules out
  strictures.
• NEC is the most common cause of SBS in childhood
• Abdominal erythema is an indication for surgery!

36
Hypertrophic Pyloric Stenosis

• A 4 week old infant presents with non-bilious
  vomiting and hypochloremic, hypokalemic,
  metabolic alkalosis.
• Idiopathic thickening and elongation of the
  pylorus causing GOO.
• Age is 3-6 weeks (1 month of age)
• Initially fed normally then projectile vomiting.
• An olive is palpated in 50

37
Hypertrophic Pyloric Stenosis

• Mild jaundice in 5 due to reduced glucoronyl
  transferase activity
• Dx is confirmed by US
• Pyloric diameter 1.4 cm
• Pyloric wall 0.4cm
• Pyloric channel 1.6cm
• Ramstedt pyloromyotomy is treatment of choice
  (open or laparoscopic).
• Surgery is not an emergency (resuscitate).

38
Intussusception

• A healthy 11 month boy presents with sudden
  emesis, crampy abdominal pain, bloody stools.
• Intussusception is most common cause of
  intestinal obstruction in early childhood and
  classically present between ages 3 months and 3
  years.
• Air contrast enema is diagnostic and therapeutic.

39
Intussusception

• Most occur before age of two.
• Etiology is thought to be lymphoid hyperplasia in
  terminal ileum after a viral illness.
• Proximal bowel (intussusceptum) invaginates into
  the distal bowel (intussuscipien) causing
  swelling, obstruction, and possible vascular
  compromise.
• Controlled air contrast enema is successful in 90

40
Intussusception

• Indications for surgery
• Enema not success
• Third episode
• Peritonitis

41
Intussusception

• Surgery involves reduction, appendectomy, and
  bowel resection for pathology.
• Recurrence after radiographic or surgical
  treatment is 5.
• Lead point present in 10 of cases (increases
  with age)
• Meckels diverticulum is most common lead point
• In adults it is malignancy!
• Currant jelly stool!

42
Meckels Diverticulum

• A healthy 2 year old presents with painless
  bloody stools.
• Patent vitelline duct
• True diverticulum
• Located on anti-mesenteric border
• A technetium-99 scan can assist with diagnosis
  and localization.
• Segmental resection is indicated for symptoms.
• Rule of twos

43
Meckels Diverticulum

• 2 of population
• 2 symptomatic
• 2 times more in males
• 2 feet from valve
• 2 years of age or less
• 2 presentations (bleeding or obstruction)
• 2 tissue types (pancreatic, gastric)
• Most common cause of bleeding in children.

44
Biliary Atresia

• A 1 month old infant has acholic stools and
  persistent jaundice.
• Jaundice in the newborn that persists 2 weeks is
  no longer considered physiologic.
• Early diagnosis is critical (before 2 months) to
  prevent progressive liver damage.

45
Biliary Atresia

• Hallmarks
• Bile duct proliferation
• Cholestasis with plugging
• Inflammatory cell infiltrate
• Progression to cirrhosis

46
Biliary Atresia

• Must rule out hepatocellular dysfunction due to
  infectious, metabolic, hematologic, or genetic
  disoders.
• Elevated conjugated bilirubin?
• Elevated unconjugated bilirubin?

47
Biliary Atresia

• Ultrasound helps to determine bile duct size and
  if a gallbladder is present.
• Bile ducts are not enlarged in atresia
• If atresia is suspected do mini RUQ incision for
  local exploration and biopsy.
• Initial goal is to establish diagnosis!

48
Biliary Atresia

• If gallbladder is present do cholangiogram.
• If cholangiogram demonstrates a patent but
  hypoplastic biliary system, the incision is
  closed.

49
Biliary Atresia

• If a patent GB or biliary tract cannot be
  identified, the incision is elongated and a Kasai
  procedure is performed portoenterostomy.
• The fibrotic GB and extrahepatic biliary tree is
  dissected to the porta hepatis and resected.

50
Biliary Atresia

• A Roux-en-Y is created and the roux jejunal limb
  is sutured to the porta hepatis to help
  reestablish bile flow from the minute bile ducts.
• Liver transplant is reserved for progression to
  liver disease, failed Kasai, cases where delayed
  diagnosis.

51
Gastrochisis

• A neonate is born with an abdominal waal defect
  to the right of the umbilicus. The eviscerated
  intestines appear thickened and do not have a
  peritoneal covering.
• Caused by intrauterine rupture of umbilical vein.
  (1 vein 2 arteries)

52
Gastrochisis

• Eviscerated intestines have no covering
• 2-5 cm defect to right of umbilicus
• Intestines are thickened, edematous and
  foreshortened.
• Associated anomalies are uncommon (except
  intestinal atresia in 10-15)
• Perioperative management includes volume, NGT,
  confirmation of bowel viability and protective
  dressings.

53
Gastrochisis

• Treatment
• Attempt primary reduction, if unsuccessful, use a
  silo.
• A silo allows for a bedside staged closure to be
  followed by primary closure.
• During exploration confirm presence or absence of
  atresia.
• Post-op ileus common, TPN life saving.

54
Omphalocele

• A baby is born with an abdominal wall defect. The
  exposed intestines have an intact peritoneal
  covering and appear normal.
• Omphalocele is associated with 40-80 incidence
  of another congenital anomaly!
• Defect thru umbilicus, 4-10cm defect.
• Covered, normal appearing.

55
Omphalocele

• Associated anomalies
• Cardiac most common
• Pericardial, sternal, diaphragmatic
• Musculoskeletal
• GI,GU
• Beckwith-Weidmann syndrome (omphalocele,
  hyperinsulinema, macroglossia)

56
Omphalocele

• Perioperative management includes volume, NGT, ID
  other anomalies.
• Primary reduction is optimal, but if defect too
  large, then staged closure.
• Outcomes related more to associated anomalies
  than to the omphalocele itself.

57
Congenital Diaphragmatic Hernia

• A few hours after birth, a newborn develops
  dyspnea, cyanosis, retractions.
• Two types
• Bochdalek (posterolateral) most common, 80 on
  left
• Morgnani (anteromedial)
• CXR shows loops of intestine or gastric bubble in
  chest. NGT helps in diagnosis.

58
Congenital Diaphragmatic Hernia

• Resuscitate, stabilize first!
• May require ECMO
• Beware of contralateral PTX due to aggressive
  ventilation.
• Surgical repair is done through subcostal
  incision, with reduction of organs, closure of
  defect.
• Primary repair is optimal but may need patch to
  avoid tension.

59
Neuroblastoma

• A 2 year old boy complains of belly pain and lack
  of appetite. Physical reveals a large abdominal
  mass.
• Most common malignant solid tumor in children.
• Derived from neural crest tissue
• May arise anywhere along sympathetic ganglia.
• Most common in adrenal medulla (50)

60
Neuroblastoma

• Age 1-2 years
• Extends across midline
• Ocular involvement may present as raccoon eyes.
• Calcifications on x-ray
• Elevated catecholamines, VMA, metanephrines.
• Due to production of hormones, children may
  present with flushing, HTN, watery diarrhea,
  periorbital ecchymosis.

61
Neuroblastoma

• Age at presentation is major prognostic factor.
• Less than 1 year 70 survival
• Older than 1 year
• Good prognostic features
• Tumors with
• Aneuploid tumors
• Low mitosis index
• Normal LDH and catecholamine levels.

62
Neuroblastoma

• Rarely mets
• If able, surgical excision is treatment of
  choice, chemo may be beneficial.
• The N-myc gene is associated with neuroblastoma!

63
Wilms Tumor (Nephroblastoma)

• Second most common solid tumor in children.
• Mass palpable and HTN, hematuria
• Derived from kidney
• 3-4 years of age
• Rare to extend across midline

64
Wilms Tumor (Nephroblastoma)

• No x-ray calcifications
• Prognosis based on tumor grade
• Mets to bone and lung
• Primary surgical excision very important
• Neoadjuvant chemo for large tumors
• Overall survival is good (85)
• Wilms tumors replace renal parenchyma on CT
  scans whereas Neuroblastoma displaces it.

65
Hepatoblastoma

• A 7 year old boy presents with precocious puberty
  and elevated Alfa fetal protein.
• Most common liver tumor in children.
• Liver cancer variant (better prognosis than
  hepatocellular ca in adults)
• Beta HCG release results in puberty
• AFP elevated
• Surgical resection is treatment of choice.

66
Mediastinal Masses in Children

• A 7 year old girl presents with dysphagia. A CT
  scan reveals a mediastinal mass.
• Most common is T-Cell lymphoma
• Teratoma
• Tumor (neuroblastoma, neurofibroma,
  neuroganglionoma, germ-cell tumors).

67
Most Common Malignancy in Children?

• Leukemia

68
Hernia

• A 14 month old presents with incarcerated right
  inguinal hernia. What do you do?
• Reduce, admit, do semielective repair in future.
• High ligation and division of sac.
• Elective repair to avoid reincarceration (16)

69
Hernia

• 10-30 incidence of contralateral inguinal
  hernia.
• Watch out for a normal ovary in an inguinal
  hernia in females.
• Umbilical hernias occur in 10-30 of live births
• Incarceration very rare
• If less than 2cm, frequently close spontaneously,
  closure and repair should be delayed until age of
  4.
• If larger than 2cm, repair at time of diagnosis.