PEDIATRIC

SURGERY

Neonate
-
Air distends GIT, splinting diaphragm
-
Gastric fluid refluxes into lungs
-
Saliva pools in pouch & aspirated

Recognition

Prenatal
-
Ultrasound

Polyhydraminos

No gastric bubble

Dr. Alfredo Igama

NEONATAL RESPIRATORY DISTRESS

Objectives

To be able to recognize 3 surgical conditions as possible

causes of respiratory distress in neonates, namely:
(1) Esophageal Atresia
(2) Diaphragmatic hernia
(3) Eventration of the diaphragm

To be able to explain the pathophysiology involved in the

above conditions

To be able to provide the appropriate initial management

in such cases

To be familiar with the basic surgical therapy for such

cases

Neonate
-
Clinical

Excessive drooling

Choking when fed

Progressive respiratory distress

-

ESOPHAGEAL ATRESIA / TRACHEO-ESOPHAGEAL FISTULA

Etiology:

Intraembryonic pressure

Epithelial closure

Vascular accident

Factors affecting rate and timing of cell proliferation &

differentiation in region of trachea & lung bud before 4
weeks AOG

Radiologic

Tip of catheter confirms level of pouch

Contrast instillation seldom needed

Contrast actually contraindicated:
barium toxic to lungs

Also confirms:
Distal TEF
Duodenal atresia

Incidence:

1 in 2,500 live births

Most common type is esophageal atresia with distal

tracheoesophageal fistula

There is a slight male preponderance

Chromosomal anomalies frequent (6.6% of infants)

trisomy 13 and 18

Types:
80-90%
5-8%
2-4%
1%
1%

Isolated EA

With gas in abdomen

Gas in stomach only or
double bubble
Gasless abdomen

Prediction of Survival (Spitz Classification)

Group Rate (%)
Birth weight > 1,500g w/o major Congenital Heart
1.
Disease
Birth weight < 1,500 g or a major Congenital Heart
2.
Disease
Birth weight < 1,500 g and a major Congenital Heart
3.
Disease

Pre-operative Preparations:

Limit stress

Keep warm

Hydration

NPO, suction upper pouch

Upper body elevated (if with TEF)

Check for other anomalies

EA with distal TEF
Isolated EA
Isolated TEF
EA with proximal TEF
EA with proximal & distal TEF

Survival
97
59
22

Positioning of Patient & EA Pouch Decompression

Associated Anomalies:
1. Cardiovascular anomalies 11-49%
2. GUT 24%
3. GIT 24%
4. Skeletal 13%
5. Neural tube defects 23%
6. Hydrocephalus 5.2%

Pathophysiology:

In utero
-
Upper pouch distends, compressing trachea & causing
tracheomalacia
-
Amniotic fluid in airways drain to GIT (through
fistula), with decreased stenting of alveoli

Determine other associated congenital anomalies

-
VACTERL syndrome

Surgical Options:

Immediate Primary Repair

-
Early correction of all anomalies
-
May not be tolerated by patients in poor medical
condition


Page 1 of 6

DR. ALFREDO IGAMA MAY 18-19, 2015

Delayed Primary Repair

-
Improve patients condition over a few days before
definitive surgery
-
Requires dedicated supportive care

Staged Procedure
-
Several successive surgeries as the patients condition
permits
-
Supportive care not too demanding

Surgical Procedures:

Staging Procedures
-
Gastrostomy
-
Medium- to long-term vascular access
-
Cervical esophagostomy
-
TEF occlusion or ligation (w/o esophageal
anastomosis)
-
Esophageal lengthening
-
Esophageal replacement

Primary Correction
-
Trans0thoracic ligation of TEF with esophageal
anastomosis
-
Throacoscopic approach recent development

Complications:

Anastomotic leak

Stricture

Recurrent TEF

Gastroesophageal reflux

Tracheomalacia

Diagnosis:

Prenatal ultrasound 25 weeks

-
Bowel loops within chest
-
Polyhydramnios
-
Absent or intrathoracic gas bubble
-
Mediastinal
-
Fetal hydrops

Amniocentesis

Chromosomal analysis

Maternal Serum AFP

Chest X-ray air and fluid loops within the left hemithorax
-
Mediastinal shift
-
Minimal gas within abdomen

Management:
1. Prenatal Care
-
Support the fetus and bring to term
-
Refer to the appropriate tertiary perinatal center

2.

Preoperative care
-
Conventional mechanical ventilation

Avoid mask
-
Endotracheal intubation
-
Control pul,onary HPN
-
Neuromuscular blockade
-
NGT
-
Echocardiography
-
Surfactant replacement therapy
-
Nitric oxide
-
Extracorporeal membrane oxygenation (ECMO)

3.

Surgical Management
-
Timing delayed until there is evidence that the
pulmonary hypertension has been resolved
-
Technique repair of defect primarily or with
prosthesis

Types:

CONGENITAL DIAPHRAGMATIC HERNIA

Herniation of abdominal organs into the thoracic cavity
through a defect in the diaphrag
1 in 2,000-5,000 live births
2x in females
Questions on CDH
30-60% overall mortality
will from Dr.
Tecsons lecture

Bochdalek 85-90%
-
Posterolateral
-
Left-sided 80-90%

Morgagni 2.6%
-
Retrosternal

Pentalogy of Cantrell
1. Omphalocoel
2. Inferior sternal cleft
3. Severe cardiac defect ectopia cordis
4. Pericardial defects
5. Hiatal hernia

Defects:

Pulmonary hypoplasia
-
Decreased pulmonary mass and weight
-
Decreased branchial divisions
-
Reduction in the number of alveoli and bronchioles

Increase muscularization of pulmonary arterioles

Persistent pulmonary HPN

Surfactant deficiency

Associated anomalies

Clinical Presentation:

Respiratory distress
-
Tachypnea, grunting respiration, chest retraction,
pallor cyanosis
-
Hypoxia, hypercarbia, acidosis

Scaphoid abdomen
Increased chest diameter
Mediastinal shift
Bowel sounds in the chest
Decreased breath sounds

EVENTRATION OF THE DIAPHRAGM

Abnormal elevation of the diaphragm
Paradoxical motion of respiration
Incomplete development of muscles and central tendon
Mostly left-side

Clinical Manifestations:

Respiratory distress

Tachypnea

Pallor

Decreased breath sounds

Mediastinal shift

DR. ALFREDO IGAMA MAY 18-19, 2015

Chest Radiology:

Elevated hemidiaphragm

Fluoroscopy or Ultrasound:

Paradoxic motion diaphragm

Mediastinal shift

Treatment: Plication of the diaphragm



Page 2 of 6

Vomiting

Normal vomiting

Abnormal vomiting
-
Medical vs. Surgical
-
Color (bilious = surgical)
-
Timing
-
Manner of vomiting

Diagnostics:

Ultrasound
-
Doughnut sign
-
Target bulls eye sign
-
3-4 mm muscle thickness (normal 1-2 mm)

UGIS
-
String sign
-
Shoulder sign

Electrolyte disturbance

Hypochloremic hypokalemic metabolic alkalosis

Management

Correct electrolyte imbalance normal saline

Ramstedts pyloromyotomy

Intestinal Obstruction in the Newborn

Bilious emesis cardinal symptom

Prompt recognition & treatment

Classification
Bilious vomiting w/ minimal
abdominal distention
(a) Proximal
obstruction
Paucity of gas
No distal air
(b) Distal
Bilious emesis and abdominal
obstruction
distention

DUODENAL OBSTRUCTION
Duodenal atresia
Duodenal web
Duodenal stenosis
Annular pancreas
Duodenal duplication
Malrotation w/ midgut volvulus

Duodenal Atresia

Failure of recanalization of the duodenal lumen from its

solid cord stage

Classification of Duodenal Atresia

Description
Mucosal diaphragmatic membrane
Most common
Short fibrous cord connecting the two sides
Complete separation of two ends

Abdominal calcification meconium peritonitis

Pneumatosis / free abdominal air NEC
Plain abdominal x-ray
Ba Enema
-
Microcolon or to identify small vs. large colon

HYPERTROPHIC PYLORIC STENOSIS

Most common cause of gastric outlet obstruction in children

Type
I
II
III

Clinical Presentation and Diagnosis

-
Hx of polyhydramnios (30-50%)
-
Associated Downs syndrome
-
Bilious vomiting
-
Full epigastrium
-
Abdominal distention not observed
-
NGT > 30 mL of bile-stained gastric fluid
-
Double-bubble sign

Management:
-
Fluid and electrolyte correction
-
OGT
-
Duodenoduodenostomy

Associated Anomalies:
-
Annular pancreas
-
Malrotation
-
Anterior portal vein
-
Downs syndrome

Incidence:

1-3 in every 1,000 live births

M-F = 4:1

Associated Anomalies:

Intestinal malrotation

Obstructive uropathy

Esophageal atresia

Etiology:

Gastric hyperacidity leading to muscle spasm and

hypertrophy

Abnormal pyloric innervation

Abnormal motility secondary to pacemaker cells

Clinical Presentation:

Nonbilious, projectile vomiting shortly after feeding

between 3 and 6 weeks old

Blood-streaked or coffee-ground vomitus

Infant constantly hungry

History of frequent formula changes

Palpable olive-shaped mass in the RUQ

Differential Diagnosis:

Overfeeding

GER

Elevated ICP

Pylorospasm with delayed gastric emptying

Antral web

Gastric neoplasm

Gastric duplication

Extrinsic gastric compression

JEJUNOILEAL ATRESIA
Etiology:

Vascular occlusion

Mesenteric vascular accidents

Incidence:

1 of 400 to 1 of 5,000 live births

Type
I
II
IIIa

Classification of Jejunoileal Atresia

Description
Membranous atresia with intact mesentery
19%
Blind ends of the bowel with intact mesentery
31%
Blind with defect in the mesentery
46%
Page 3 of 6

DR. ALFREDO IGAMA MAY 18-19, 2015

GIT: SURGICAL CAUSES OF VOMITING

Topic Guide:
1.
Hypertrophic Pyloric Stenosis
2.
Duodenal Obstruction
3.
Jejunoileal Atresia
4.
Malrotation and Volvulus
5.
Intussusception
6.
Hirschprungs Disease

Diagnosis:

History of maternal polyhydramnios (> 2,000 mL)

Bilious vomiting

Abdominal distention

Failure to pass normal amounts of meconium

st
-
1 24 hours of life
-
250 grams passed rectally

Baby gram supine, upright, lateral

Ladds bands obstructing the duodenum

Narrow mesenteric pedicle
Volvulus obstructing the jejunum at its blood supply

Management:

Fluid and electrolyte correction

Maintain appropriate body temperature

NGT

Antibiotics

Surgical Management
-
Upper abdominal distention
-
Reestablish intestinal continuity
-
Preserve bowel length and normal anatomy
-
End-to-oblique anastomosis

Nutritional support

Morbidity:

Pneumonia

Peritonitis

Sepsis

MALROTATION AND VOLVULUS
Congenital abnormal positioning of midgut

Elongation
Rotation

Fixation

Clinical Presentation:
st

Seen in the 1 few weeks of life

Bile-stained vomitus due to obstruction of the upper third

of the duodenum

Abdominal distention

Pain and irritability

Passage of blood per rectum

Failure to thrive older infants

Erythema and edema of the abdominal wall

Intestinal Development
6 weeks rapid growth of intestines
10-12 weeks midgut returns to abdominal cavity
o
270 counter-clockwise rotation around the superior
mesentery
C-loop duodenum fixed retroperitoneally at the right
upper quadrant
Cecum fixed to the lateral abdominal wall at the right
lower quadrant

Radiologic Diagnosis:

Plain abdominal X-ray

-
Paucity of intestinal gas
-
Few scattered air-fluid levels
-
Distended gastric and duodenum

Normal Intestinal Rotation

Incomplete Rotation:

Results from non-fixation of the ascending and descending

portion of the colon to the retroperitoneum and a
redundant small mesentery

Cecum remains in the epigastrium

UGIS
-
Preferred diagnostic study
-
Corkscrew-like deformity of the duodenum
-
Duodenum and jejunum is in the right upper
(normally wala sa right)
-
Dilated stomach and airless abdomen
Barium enema is only suggestive of

Management:

OGT

IVF resuscitation

Antibiotics

Surgery should be done within 4-8 hours because of the

threat of volvulus
-
Counter-clockwise derotation of the volvulus



Page 4 of 6

DR. ALFREDO IGAMA MAY 18-19, 2015

Paucity of intestinal gas on X-ray

Clinical Presentation:

Well nourished male infant about 8 months of age

suddenly awakens from sleep with colicky abdominal pain.
Pain may pass and the child may go back to sleep, only to
be awakened shortly with a similar picture that does not
remit. Pain is followed by several bouts of vomiting that
may become bilious and then the passage of blood mucus
rectally, which has the appearance of currant jelly.

Robust, healthy infant with abdominal colic

Stools normal then dark redmucoid stools like currant

jelly 95%

Vomitus undigested bilious 80%

Lethargic and somnolent between attacks

Sausage-shaped abdominal mass

Pathology:

Idiopathic intussusception
-
Less than 1-year-old
-
During attacks of upper respiratory tract infections
and diarrhea
-
Adenovirus, rotavirus, rheovirus, echovirus
-
Hypertrophy of Peyers patches

Intussusception in older age group secondary to

anatomical lead point Meckels diverticulum, appendix,
polyp, tumors, foreign bodies

Starts with venous compression from the invagination fo

the bowel and its

Diagnostic Studies:

CBC, serum electrolytes

Plain abdominal x-ray not diagnostic

-
Abdominal mass
-
Mabnormal distribution of gas and fecal contents
-
Sparse large bowel gas
-
Air-fluid levels in the presence of bowel obstruction

Abdominal ultrasound
-
Target lesion
-
Pseudokidney sign

-
-
-
-

Antibiotics
BT
Hydrostatic barium enema or saline reduction
Pneumatic reduction

Surgical
-
When non-operative management fails
-
Manual reduction anastomosis if gangrenous
-
Appendectomy
HIRSCHPRUNGS DISEASE
Congenital absence of parasympathetic ganglia cells in the
wall of the GT tract
Bowel unable to relax and without peristalsis
Involves rectum extending proximally 75% rectosigmoid

Incidence:

1 in 5,000 live births

M-F ratio 4:1

Total aganglionosis reverse

30% of patients have relatives afflicted with disease

Clinical Presentation:

Newborn
-
Non-passage or delayed passage of meconium
-
Abdominal distention
-
Bilious vomiting
-
Visible bowel loops
-
Rectal exam

Rectal vault empty

Increased sphincteric tone

Explosive stools on removal of examining finger

Infants and older children

-
History of obstipation and constipation
-
Failure to thrive
-
History of delayed passage of meconium
-
Abdominal distention
-
Bouts of diarrhea and vomiting (enterocolitis)
-
Foul-smelling stools

Diagnosis:

Plain abdominal X-rays

-
Air-fluid levels
-
Distended bowels
-
No presacral air

Barium enema
-
Spasm and a narrowed lumen
-
Transition zone

Tissue confirmation: Auerbach plexus in muscular layer

-
Biopsy specimens

Seromuscular biopsy of bowel wall

Full thickness transrectal biopsy

-
Suction biopsy Meissners plexus in submucosal
layer

Management:

Non-operative no signs or peritonitis, perforation,

sepsis, and gangrene
-
NGT
-
IVF

Page 5 of 6

DR. ALFREDO IGAMA MAY 18-19, 2015

Malrotation:

Ladds Procedure
-
Straightening of Duodenbum
-
Widening of Mesentery
-
Small bowel at the right hemiabdomen; large bowel
at the left
-
Appendectomy (optional)

INTUSSUSCEPTION

Invagination of a portion
of the intestine into itself,
producing vascular
compromise

Majority are ileocolic and

without lead point

Seen in infants between

5-10 months old

Adenovirus and rotavirus

infection prior to
intussusception

Trans-anal Rectal Biopsy:

Definitive diagnosis for Hirschsprungs disease

-
Open
-
Suction

Differential Diagnosis:

Premature infant

Sepsis

NEC

Meconium plug syndrome

Intestinal dysmotility syndrome

Functional constipation

Preoperative Management

Colonic / rectal decompression with warm saline

Gastric decompression

Treat enterocolitis

Colostomy normal segment

Nutrition

Operative Management:

Usually performed between 4 months and 1 year of age

and weight of 12-20 lbs

Goal:
-
Resection of the involved segment
-
Re-establishment of functional and continent anus

Definitive procedures:
-
Swenson procedure
-
Duhamel procedure
-
Scott Boleys endorectal pullthrough

Pull-Through Procedures:

Soave-Boley technique

Swensons technique

AJB J Medicine Batch 2016

Page 6 of 6

DR. ALFREDO IGAMA MAY 18-19, 2015