COMMON PEDIATRIC

SURGICAL CONDITIONS

ROCELDA L. MIRASOL, MD, DPBS, DPSPS

PEDIATRIC SURGEON

TOPIC OUTLINE
1) Hirschsprungs Disease
2) Anorectal Malformations
3) Congenital Abdominal Wall Defects
4) Duodenal Obstruction
5) Jejunoileal Atresia
6) Congenital Diaphragmatic Hernia

HIRSCHSPRUNGS
DISEASE

HIRSCHSPRUNGS DISEASE

Incidence: 1 in 4,400 to 1 in 7,000 live births

Male-female ratio: 4:1 in favor of males
Long-segment disease: 1:1 ratio
Mean age of presentation: 3-6 months
Diagnosed in the newborn in more than 90%
of patients

HIRSCHSPRUNGS DISEASE Presentation

Delayed passage of
fecal matter within the
first 48 hours of birth
Constipation,
abdominal distention,
poor feeding, emesis,
failure to thrive
DRE: tight anus

HIRSCHSPRUNGS DISEASE Diagnosis

Radiographic Studies
Plain radiographs:
distended bowel
loops, paucity of air in
the rectum,
pneumoperitoneum

HIRSCHSPRUNGS DISEASE Diagnosis

Radiographic
Studies
Contrast enema: key
finding of a transition
zone (narrow, spastic
distal segment with a
dilated proximal
segment)

HIRSCHSPRUNGS DISEASE Diagnosis

Rectal Biopsy
Gold standard for
the diagnosis
absence of GC
Full-thickness
biopsy, suction
rectal biopsy

HIRSCHSPRUNGS DISEASE Surgical

Treatment
Leveling Colostomy:
severe enterocolitis or
markedly dilated
proximal colon
Endorectal Pull-through
(Soave-Boley)
Other pull-through
procedures: Duhamel,
Swenson

ANORECTAL
MALFORMATION
S

ANORECTAL
MALFORMATIONS
Incidence: 1 in 5,000 live births
Slight male preponderance
IA without fistula: 5% of patients, half
of them also have Downs syndrome

ANORECTAL MALFORMATIONS Classification

MALES
Perineal Fistula
Rectourethral Fistula
Bulbar
Prostatic
Rectovesical Fistula
(bladder neck) 10%
IA Without Fistula 5%
Rectal Atresia

FEMALES
Perineal Fistula
Vestibular Fistula
Persistent Cloaca
< 3 cm common
channel
> 3 cm common
channel
IA Without Fistula 5%
Rectal Atresia

ARM - Clinical Findings & Initial

Management

External appearance of the perineum of female patients with anorectal

malformations. A, Perineal fistula. B, Vestibular fistula. C, Cloaca. D, Very high
cloaca, flat bottom.

ARM Definitive Surgical Management

Distal Colonogram
-before
reconstruction; for
surgical planning
Above: rectobladder neck fistula
Below: rectourethral bulbar
fistula

ARM Definitive Surgical

Management
Anorectal
Reconstruction
(PSARP)
prone position with
pelvis elevated
requires abdominal
approach in
patients with rectobladder neck fistula
and those with
cloaca

CONGENITAL
ABDOMINAL WALL
DEFECTS

CONGENITAL ABDOMINAL WALL

DEFECTS
Omphalocele
lateral, cepahalic,
caudal folds
Umbilical Cord Hernia
Gastroschisis
Ectopia Cordis
Thoracis

CONGENITAL ABDOMINAL WALL

DEFECTS
Omphalocele
Central defect, usually
larger than 4cm in diameter,
covered by a translucent sac
Sac contents: liver, midgut,
spleen, gonad
Incidence: 1-2.5 per 5,000
live births; second after
gastroschisis
male preponderance
Full term

CONGENITAL ABDOMINAL WALL

DEFECTS
Omphalocele Treatment
Initial Care:
NGT decompression, rectal exam to
evacuate meconium
Maintenance of body temperature
Cardiology evaluation
IVF hydration
Painting sac with antiseptic

CONGENITAL ABDOMINAL WALL

DEFECTS
Omphalocele
Treatment
Operative Closure:
Any sac adherent to
the liver may be left
behind
Fascial closure, skin
closure

CONGENITAL ABDOMINAL WALL

DEFECTS
Gastroschisis
Defect usually less
than 4cm, just to
the right of the
cord; no sac
Midgut herniation
Preterm
Incidence: 2-4.9 per
10,000 live births
Male preponderance

CONGENITAL ABDOMINAL WALL

DEFECTS
Gastroschisis
Treatment
Initial Care:
Place the infant
immediately in a plastic
drawstring bag to control
evaporative heat and
fluid loss
Spring-loaded preformed
Silastic pouch placed at
the bedside upon arrival
of the patient

CONGENITAL ABDOMINAL WALL

DEFECTS
Gastroschisis
Treatment
Operative Closure:
The same as that in
omphalocele
Alternative Closure
Technique: use of
transfusion bag for
staged closure

DUODENAL
OBSTRUCTION

DUODENAL ATRESIA
Duodenum: most common site of
neonatal intestinal obstruction
Incidence: 1 in 6,000 to 1 in 10,000
births
Pathophysiology: errors in
recanalization

DUODENAL ATRESIA - Types

Type I (92%) presence of an
obstructing web or septum
Type II (1%) a short fibrous
cord connects the two blind
ends of the duodenum
Type III (7%) no connection
between the two blind ends
of the duodenum; Vshaped mesenteric defect

DUODENAL ATRESIA Clinical

Presentation
Bilious emesis:
characteristic feature
NGT: return of > 30 ml
of fluid is suggestive
Plain radiograph:
double-bubble sign

DUODENAL ATRESIA Treatment

OGT decompression, IVF, TPN, antibiotics
Surgical correction not urgent
Web excision
Duodenoduodenostomy ,
duodenojejunostomy
Survival rate: 45-95%

JEJUNOILEAL
ATRESIA

JEJUNOILEAL ATRESIA
Incidence: 1 in 330 to 1 in 1,500 live
births
Etiology: late intrauterine vascular
insults as volvulus, intussusception,
internal hernia, constriction of the
mesentery in a tight gastroschisis or
omphalocele

JEJUNOILEAL ATRESIA Clinical

Presentation
Prenatal ultrasound:
polyhydramnios, multiple
distended loops of bowel
with vigorous peristalsis
Radiography: thumbsized intestinal loops, airfluid levels, peritoneal
calcification signifying
meconium peritonitis

JEJUNOILEAL ATRESIA Treatment

OGT decompression, IVF, TPN, antibiotics
Resection of dilated atretic segment
followed by an end-to-oblique
anastomosis
Short gut: tapering enteroplasty
Survival rate: 80-90% or greater

CONGENITAL
DIAPHRAGMATI
C HERNIA

CONGENITAL DIAPHRAGMATIC
HERNIA (CDH)

Incidence: 1 in 2,000 to 5,000 births

80% left-sided; 20% right-sided
Likely premature, macrosomic, male
1/3 have associated major defects (1050%)
Cause is unknown; may be due to
environmental factors

CDH - Diagnosis
Prenatal UTZ
40-90% accuracy; mean 24 weeks
AOG
Polyhydramnios 80%
Stomach or fluid-filled bowel loops in
the fetal thorax
Liver or other solid viscera

CDH - Diagnosis
Plain Chest
Radiograph
Confirms the
diagnosis
Loops of bowel in
the chest
Shifting of cardiac
silhouette into the
contralateral chest

CDH - Presentation
Scaphoid abdomen, asymmetrical
distended chest
Breath sounds may or may not be
present
Cyanosis, gasping, sternal
retractions, poor respiratory effort

CDH - Treatment
Preoperative Care
Physiologic, not a surgical emergency
Endotracheal intubation, OGT
decompression
Maintain proper temperature regulation,
glucose homeostasis, volume status
Infant properly sedated
Mechanical ventilation
Sildenafil, surfactant

CDH Surgical Repair

Subcostal incision, thoracotomy, both
Thoracoscopic, laparoscopic
approaches
Viscera gently reduced
Primary repair using nonabsorbable
sutures, reconstruction using
prerenal fascia, rib structures,
muscle flaps

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