Paediatric Surgery 2

Congenital hypertrophic
pyloric stenosis
(CHPS)
⁕ Incidence:
• It is the commonest cause in surgical practice for persistent
vomiting in infancy.
• 1 in every 400 live births, more 1st. born infant , more in males
(4:1) & may be familial.
⁕ Aetiology: Immaturity of the ganglion cells → failure or delayed
relaxation of pyloric sphincter.
⁕ Pthology:
1. Pylorus: Hypertrophy of the circular muscle layer of the pyloric
canal which stops abruptly at the duodenum and tapers towards the
body. The pylorus projects into the duodenum forming a duodenal
fornix around it.
2. Stomach: first there is thickening & hypertrophy of gastric wall but
later on the stomach is markedly dilated.
3. Intestine is empty and collapsed.

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* Complications: Repeated vomiting leads to:

1. Loss of acid → metabolic alkalosis → tetany.
2. Hyponatraemia , hypokalaemia & hypocalcaemia due to Na , K &
calcium loss respectively.
3. Dehydration.
4. Anaemia & loss of weight.
5. Respiratory obstruction & infection due to inhalation of vomitus.
* Clinical Picture:
1. The condition usually manifests itself 2-4 weeks after birth.
2. The infant is constantly crying due to constant colic and hunger.
3. Vomiting: is most important prominent feature, repeated,
projectile, never bile stained, contains food of previous meals or
days, foul odour (Fermentation).
4. Progressive constipation with small hard rabit stool and
decreasing urination.
5. Picture of complications , dehydration ,weight loss & anaemia .
6. Thick pyloric canal is felt in 90% of cases as olive, oval, firm
mass at the level of L1 vertebra just to right side of middle line is
diagnostic.
6. Epigastric fullness with visible peristaltic waves in the
epigastrium propagating from left to right.
7. In 10% of cases haematemesis may occur due to gastritis &
oesophagitis.

❖ N.B: Any neonate presenting with repeated projectile, non

bile stained vomiting, associated hunger & firm stools
should by considered to have CHPS until proved otherwise.

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⁕ D.D.: other causes of repeated vomiting in newly born.

• Gastroenteritis, over feeding, congenital duodenal atresia or
stenosis, pylorospasm , antral web , volvulus neonatorum,
intussusceptions and intracranial haemorrhage.
⁕ Investigations:
1. Blood chimestry: hypochloremic hyponatraemic hypokalaemic
alkalosis.
2. Gastric suction shows large amount of fluid.
3. Imaging studies are only needed if pyloric olive mass is not
felt:

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a) Ultrasound: The most important imaging modality. CHPS is

diagnosed if the length of the pyloric canal is more the 16 mm &
thickness is more than 4 mm.
b) Plain x-ray or Barium meal are rarely used.
➢ The stomach is dilated .
➢ pyloric canal is elongated & narrow (string sign).
➢ Shoulder sign: Pyloric mass bulges into pyloric antrum.
➢ Double or triple track sign due to passage of barium
between mucosal folds.
➢ Mashroom sign
➢ Caterpillar sign: due to hyperperistalsis .

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Double or triple track sign

Caterpillar sign

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* Treatment: Only surgical.

• Rapid preoperative preparation :
➢ I.V fluids to correct fluid and electrolyte imbalance.
➢ Nasogastric tube for gastric suction and gastric wash.
➢ Treatment of respiratory infections.
• Exposure :
➢ Laparoscopic approach.
➢ Ramstedt’s Operation: Right upper quadrant transverse
incision.
➢ Method :
 Pyloromyotomy: The thick circular muscle layer in pyloric
region is incised longitudinal down to the submucosa , stopping
short of prepyloric vein → pyloric mucosa bulges.
Ramstedt’s Pyloromyotomy

Laparoscopic Pyloromyotomy

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Paediatric Surgery 2

Congenital Duodenal Obstruction

 Incidence:
• It is twice common congenital jejunum or ileal obstruction .
• In 75 % of case, the obstruction is distal to ampula of Vater.
• 1-3 % have associated distal stenosis or atresia.
 Aetiology :
1- It is usually due to failure of canalization of the duodenum in early
gestation.
2- Less commonly, mesenteric vascular abnormality in late gestation
 Pathology:
• Congenital duodenal obstruction may be due to atresia, stenosis,
mucosal web (complete or perforated), annular pancreas,
preduodenal portal vein, peritoneal bands from malrotation (Ladd’s
bands)

A: Stenosis

B: Mucosal web

C: Atresia

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Annular pancreas

Ladd’s band

Preduodenal portal vein

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 Complications: (as usual in any I.O).

 Clinical picture:
1) Prenatal U/S may show polyhydramnios in any fetal I.O.
2) The main presentation is repeated bile stained vomiting
occurs shortly after birth and during attempt of feeding.
3) Absolute constipation in case of complete obstruction with
failure to pass meconium.
4) Rarely mild upper abdominal distension.
5) The baby show attacks of crying due to pain.
6) Associated congenital anomalies (VACTERL) are common
especially Down syndrome which present in 1/3 of cases.
 Investigations:
1) Prenatal U/S reveals polyhydramnios and fluid filled dilated
stomach and duodenum.
2) Genetic amniocentesis to detect Down syndrome.
3) Plain x-ray: show the followings
• Double bubble sign: air appears in distended stomach and
duodenum.
• Absence of air in distal part of intestine indicate complete
obstruction.
• Presence of air in distal part of intestine indicate incomplete
obstruction.

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4) In chronic obstruction, contrast upper GIT study

(gastographin or barium) to exclude intestinal malrotation which
is surgical emergency.
 D.D: Other causes of neonatal GIT obstruction.
 Treatment: (Not surgical emergency)
• Pre-operative preparation: Nothing is taken orally, nasogastic
suction, IV fluid, IV antibiotic.
• Surgery: Laparoscopic or open surgery.
▪ In open surgery: exploration through upper right transverse
incision.
▪ Kocher maneuver with mobilization of the duodenum.
▪ Relieve of obstruction according to the cause:
➢ Duodenoduodenostomy to by-pass the obstruction in
case of duodenal atresia or annular pancreas.
➢ Ladd’s band requires simple division and correction of
malrotation.
➢ Excision of mucosal web.
▪ Irrigation of distal bowel to exclude distal intestinal atresia.

Duodeno-

duodenostomy

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Paediatric Surgery 2

Congenital jejunum, ileum and colon obstruction

 Incidence: Less common than congenital duodenal obstruction.

 Aetiology: Mesenteric vascular accident in utero → aseptic necrosis
and resorption of necrotic bowel.
 Pathology: This condition shows the following types:
• Type I: Stenosis or membranous web occlude the lumen.
• Type II: Fibrous cord between 2 bowel loops.
• Type IIIA: Complete separation of bowel ends with a V-shaped
defect in the mesentery.
• Type IIIB: (apple peel atresia) A blind end proximal jejunum,
absence of long bowel segment and coiled terminal ileum with
absence of distal part of superior mesenteric artery.
• Type IV: Multiple atesia.

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Apple peel

 Complications:( as any I.O).

 Clinical picture:(as congenital duodenal obstruction).
• Repeated bile stained vomiting is the main presentation.
• Moderate to severe abdominal distension.
 Investigations:
1&2) (as congenital duodenal obstruction)
3) Plain X-ray show gas with fluid level and show the level of
obstruction.
4) In incomplete obstruction, contrast upper GIT study
(gastographin or barium) detect the level of obstruction.
 D.D: Other causes of neonatal GIT obstruction
 Treatment:
• Preoperative preparation: (as any I.O)
• Surgery: The main goals of the operations are the followings:
1) Restore continuity of the bowel.
2) Preserve as much intestinal length as possible.
3) Preserve ileocecal valve if possible.

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Disorders of intestinal rotation

 Incidence: rare condition occurs twice in males than females.

 Aetiology & classification:
• Normally, fetal intestine grows faster than the fetal abdominal
cavity → the intestine herniate into the umbilical cord at 4-6
weeks of gestation.
• At 10-12 weeks of gestation, the intestine return to the
abdominal cavity, rotates and fixed in its normal anatomical
position.

• Failure of normal rotation and fixation of the intestine can be

classified into the followings:
1) Non rotation: The midgut is suspended from superior
mesenteric vessels, the small intestine is located in the right

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side of the abdomen and the large intestine in the left side of
the abdomen.
2) Incomplete rotation: Adhesive bands (Ladd’s bands) are
dense peritoneal bands extend from the right flank to the
cecum and obstruct the 2nd. Part of the duodenum.

3) Reversed rotation
4) Abnormal mesenteric fixation: This predispose to internal
hernia (paraduodenal,paracecal& transmesenteric hernias),
mobile cecum and volvulus of cecum.

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 Complications:
• Acute intestinal obstruction & strangulation: due to
▪ Ladd’s bands
▪ Midgut volvulus around the superior mesenteric axis.
▪ Strangulated paraduodenal hernia.
 Clinical picture:
1) Manifestations of acute intestinal obstruction(Mention).
▪ Vomiting of bile is the earliest manifestation, in duodenal
obstruction by Ladd’s band, with mild abdominal distention.
▪ In midgut volvulus, abdominal distention is marked.
▪ Bloody stool & manifestations of peritonitis are manifestations of
strangulation.
2) Partial or intermittent intestinal obstruction.
3) In older patients, chronic nonspecific abdominal symptoms
as attacks of pain ,peptic ulceration and malabsorption .
4) Rarely , the condition may be asymptomatic and discovered
accidentaly.
5) Associated congenital anomalis (VACTERL) are common.
 D.D: Other causes of I.O in newly born.
 Investigations:
1) Plain x-ray abdomen:
• Ladd’s bands with duodenal obstruction show double bubble
sign.

• Midgut volvulus show distension of the proximal bowel.

2) In chronic obstruction, contrast upper GIT study

(gastographin or barium) show distention of the duodenum ,
abdnormal position of the duodenum , narrowing at the site of

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obstruction , small intestine is visualized in the right side of the

abdomen and the colon on the left side of the abdomen.
3) Contrast enema shows abnormal position of cecum.

Dilated loops of intestine Loops of small intestine in right side

The colon on the left side of abdomen with abnormal position of

ileocecal valve

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 Treatment: This condition is surgical emergency treated by

1) Resuscitation: Nothing is taken orally, nasogastric suction, IV
fluid, antibiotics …etc.
2) Ladd’s procedure: Through laparoscopic or transverse upper
abdominal incision, the following steps are performed:
1) Explore the abdomen and evisceration of the intestine.
2) Counterclockwise detorsion of the intestine.
3) Division of Ladd’s bands.
4) Broadening of the root of mesentery of small intestine.
5) Appendectomy.
6) Placement of small intestine in the right lateral gutter and
colon along the left lateral gutter.

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Paediatric Surgery 2

Meconium Ileus

 Incidence: Affect 20% of infants born with cystic fibrosis.

 Aetiology :
• Intestinal secretions are abnormally thick and stick to the intestinal
lining producing inspissated meconium, in the terminal ileum.

 Pathology and complications:

• Meconium ileus may be one of the followings:
1) Simple meconium ileus which can be treated by conservative
measures.
2) Complicated meconium ileus which should be treated
surgery. These complications include the followings:
▪ Volvulus.
▪ Associated intestinal atresia.
▪ Intestinal necrosis.
▪ Intestinal perforation.
▪ Peritonitis.

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 Clinical picture:
1) The newly born has a distended abdomen.
2) No passage of meconium within first 24-48 hours.
3)Bilious vomiting.
4) Loops of thick, distended intestine may be seen and palpated.
 Investigations:
1) Plain x-ray abdomen:
• Distended loops of small intestine proximal to the obstruction.
• Thick calcified meconium in the terminal ileum gives ground-
glass appearance.
• Air mixed with meconium produces soap bubble appearance.
2) Contrast enema:(never use barium)
• It is diagnostic and therapeutic in simple meconium ileus. It
shows:
▪ Microcolon (disuse) with rare meconium.
▪ Reflux of contrast medium through ileocecal valve reveals
narrow terminal ileum containing meconium and
distention of proximal part of intestine.

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Paediatric Surgery 2

 D.D: Other causes of neonatal intestinal obstruction.

 Treatment:

• Resuscitation: Nothing is taken orally, nasogastric suction, IV

fluid, antibiotics …etc.
1) Simple meconium ileus:
▪ Non operative treatment is successful in 70% of cases.
▪ A contrast enema (diagnostic & therapeutic) is performed ,
under fluoroscopic control up to the dilated ileum, with a slightly
hypertonic water-soluble contrast agent (gastroraffin, never
barium) with addition of acetylcysteine (mucolytic) to dissolve the
meconium. The enema is repeated every 12 hours for 4-5 days
until meconium disappear.
2) Complicated meconium ileus or failure of non operative
treatment, operative treatment is needed as follows:
▪ Laparotomy.
▪ Compromised intestine is resected.
▪ The viable ileum is opened, irrigated and cleaned then one of the
followings is performed:
➢ Intestinal anenastomosis.

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➢ Bouble-barrel ileostomy: One channel for intestinal

decompression and the other channel for post-operative
irrigations.
➢ Tube enterostomy .
▪ Appedectomy is performed due to high incidence of appendicitis is
patients with cystic fibrosis:

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Paediatric Surgery 2

Hirschsprung’s Disease
(Aganglionosis of Colon or Aganglionic megacolon)
 Incidence:
3) It affects one in each 5000 live births.
4) It affects males 4 times more than females.
5) Long segment affection has a gender ratio 1:1.
6) It is familial in 10% of cases and Down’s syndrome occurs in
10% of cases.
7) Short segment involving terminal rectum occurs in 10% of cases,
the disease extends to the sigmoid colon in 75% of cases ,
affection of more proximal part of colon in 10% and affection of
entire colon (Total colonic aganglionosis) in 5% of cases.

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 Aetiology:
• It's not clear what causes Hirschsprung's disease. It
sometimes occurs in families.
• It is usually associated with mutations in the RET gene .
• Normally parasympathetic ganglionic nerve cells are derived from
the neural crest and migrate caudally in wall of the gut.
• These ganglion cells arrive the rectum and anal canal by 12 weeks
of gestation.
• Arrest in this migration leads to an aganglionic bowel segment.

 Pathology:
• In Hirschsprung’s disease, there is congenital absence of
parasympathetic ganglion cells in the myenteric
(Auerbach's) plexus located between the longitudinal and
circular muscle layers and Meissner's plexus, situated in the
submucosa of the gut (aganglionic bowel segment).
• It always begins in the anal canal and extends poximally for a
variable distance.

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• This leads to functional obstruction due to failure of relaxation

of aganglionic distal bowel segment in response to proximal bowel
distention.
• There is progressive dilatation of bowel proximal to the
obstructed aganglionic segment.

 Complications:
1) Chronic or acute on top of chronic large intestinal obstruction.
2) Enterocolitis is the most dangerous complication as it is life
threatening →Toxic megacolon → perforation.
3) Chronic toxemia, anaemia and poor development.
 Clinical picture:
• The severity and age of presentation varies greatly
according to the length of the bowel affected.
• This condition usually appears science birth, but may present
in older children and rarely in adults.
• The patient may present by acute intestinal obstruction since
birth or gradual development of the following symptoms.
1) The infant pass little or no meconium in the first 48 hours.

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2) Chronic constipation since birth is the main presentation.

3) Stools are small in caliber.
4) Abdominal pain
5) Progressive marked peripheral abdominal distension.
6) Bile stained vomiting.
7) Reluctance to feed, poor growth and development.
8) P-R exam. Show constricted anal canal , dilated rectum and
followed by passage of stool and flatus with decompression of
abdominal distention.
• Enterocolitis: There are fever, toxaemia, diarrhea and bleeding
per rectum.

 Investigations:
1) Plain x-ray: show dilated loops of bowel.
2) Contrast enema: show the followings
• Narrow contracted (aganglionic) segment.
• Transition zone between the narrow and dilated bowel.
• Dilated proximal bowel.
• Normally, the neonatal rectum is wider than the rest of the
colon and when the rectum is seen to be narrower than the
proximal colon , Hirschsprung’s disease is suspected.

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• Delayed film after 48 hours show retained contrast agent for

prolonged periods and saline enema may be needed to evacuate
it.
3) Rectal biopsy is the sure diagnosis. Mucosal and
submucosal biopsies with a suction biopsy capsule. In doubtful
case, full thickness biopsy from mucosa and musculosa is
needed to detect absence of ganglion cells in Meissner’s plexus and
Auerbach’s plexus.

4) Anal manometry: Rarely needed in older children and adults. A

balloon is inflated inside the rectum → The internal anal sphincter
should relax → If it doesn't relax, Hirschsprung's disease could be
the cause.

Transition zone
(Arrow)

 D.D:
• Other causes of neonatal intestinal obstruction.
• Other causes of chronic constipation in older childrens.

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 Treatment:
• Resuscitation: Nothing is taken orally, nasogastric suction, IV
fluid, antibiotics to patient with enterocolitis …etc
• Definitive treatment is only surgical treatment.
• The goal of the operation is to remove the aganglionic bowel and
anastomose normally innervated bowel just above the anal
sphincter, preserving sphincter function so that the child will
remain fecally continent
• The surgical options vary according to the patient’s age, length of
the aganglionic segment, degree of colonic dilation, and presence
of enterocolitis.

I) In poor risk patient with poor general condition or severe

enterocolitis, 2 stage operation:
a) Proximal colostomy at the level of normal ganglionic colon
(guided by frozen sections during operation).
b) After few months, resection of aganglionic bowel and the
transitional zone between the ganglionic and aganglionic

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bowel segments with performance of a pull-through

procedure.
II) In good risk patient: Single-stage pull-through procedure

• Preoperative preparation: Bowel decompression and cleaning

by placement of a rectal tube and repeated warm saline
irrigation 3 times/day.
• The single-stage pull-through procedure is performed
recently nowadays with laparoscopic or transanal techniques
or rarely nowadays by open surgery.
• The three most commonly performed operations for
Hirschsprung disease are:
a) Soave procedure: In this operation, the mucosa is
stripped from the underlying muscle and the normally
innervated bowel is brought through the rectal muscular
“cuff” and anastomosed just above the anal sphincter. This
operation was designed to prevent injury to pelvic nerves
and blood vessels.
b) Swenson procedure. In this operation, the full
thickness of rectum is removed and the normally innervated
bowel is anastomosed just above the anal sphincter.
c) Duhamel procedure. In this operation the bloodless plane
behind the rectum is developed and the normally innervated
bowel is brought down and anastomosed to the back of the
aganglionic rectum just above the anal sphincter.

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Anorectal Anomalies
(Imperforate anus)

 Incidence:
• 1 in every 5000 babies is born with anorectal malformations and
more in males.
 Aetiology:
• The distal part of hindgut (endoderm) is called cloaca which is
divided by a urorectal septum into urogenital sinus and anorectal
canal. The anorectal canal forms the rectum and upper ½ of anal
canal.
• An ectodermal depression occurs on the surface called
proctondeum which forms the lower ½ of anal canal.
• The anorectal canal is separated from proctodeum by anal
membrane which normally rupture to connect the upper ½ with
the lower ½ of anal canal.
• Anorectal anomalies are due to failure of fusion of the anorectal
canal & proctodeum due to failure of development of anorectal
canal , failure of development of cloaca or failure of rupture of
anal membrane.

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 Pathology: Anorectal anomalies are 2 types.

1) Low anomalies:
• The commonest, the bowel terminates below the level of
pelvic floor muscles or pubococcygeal line.
• In this type one of the followings may be present:
➢ The anus is ectopically placed anterior to its normal position.
➢ Narrow anus due to stenosis or presence of anal membrane.
➢ No anal opening in the perineum because the skin completely
cover the anal opening which is marked by anal dimple.
➢ A fistula usually extends from the anal canal anteriorly to
open in the followings:
▪ In male: in the perineum, scrotum or penis.
▪ In female: in the vagina or vulva.

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• The patient usually has well developed perineal and gluteal

musculature.

• Associated sacral and vertebral anomalies are rare.

2) High anomalies:
• The bowel terminates above the level of the pelvic floor or
pubococcygeal line.
• It is usually associated with a fistula into:
➢ In male: to the urethra or urinary bladder.
➢ In female: to the vagina.
• The most severe anomaly is persistence of cloaca with failure of
development urorectal septum i.e there is a common channel for
the urinary tract and GIT with a single perineal opening.
• The patient usually has deficient perineal and gluteal innervation
and musculature.
• Associated sacral and vertebral anomalies are common.

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 Complications:
1) Pyelonephritis due to reflux of colonic contents into urethra or
urinary bladder.
2) Acidosis due to reflux of urine into the colon and absorption of
ammonium chloride.
3) Associated congenital anomalies.
 Clinical Picture:
1) The condition is usually discovered during routine exam. of
newborn.
2) Examination for ectopic anal opening, anal dimple & associated
local or distal anomalies.

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3) Fistula can be detected in the perineum or presence of meconium

at the urethral meatus, in urine or in the vagina.
4) In high anomaly, there is usually absence of external sphincter
contraction with cutaneous stimulation of the anus.
5) The infant fails to pass meconium in the first 48 hours.
6) If undiagnosed, manifestations of I.O appears (except if there is a
fistula).
7) Associated anomalies occur in 70% of patients with high anomalies
(VACRERL) especially sacral and genitourinary anomalies.
 Investigations:
1) Coin test:
• It is plain x-ray of the pelvis in lateral view invertogram
(the baby is inverted) is usually used or cross table lateral film.
• 24 hours after birth, the infant is held upside down (invertogram)
and a coin is fixed at the anal dimple then take lateral view X-ray.
• Draw pubo-coccygeal line from tip of coccyx to the lower border
of symphysis pubis, if gas in the rectum ends above that line it is
a high anomaly.
• Measure the bowel-skin distance which is the distance between
the coin & distal gas shadow in the bowel. If the distance is more
than 15 mm → high anomalies.
2) Pelvic and perineal U/S, CT and MRI are used to define pelvic
anatomy and vertebral anomalies.
3) Urine analysis & I.V.U: to detect any associated fistula or
anomalies of U.T.
4) Investigations to detect associated anomalies.

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Invertogram(Low anomalies) Cross table lateral film.

A patient with (a) dilated intestinal loops and (b) Invertogram

and PC line show high imperforate anus.

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 Treatment:
A) Resuscitation.
B) Surgery:
• The aim of surgery is to relief intestinal obstruction, closure of
fistula and place the rectal pouch in the perineum in the normal
position with preservation of anal sphincters.
• Methods:
I) If low imperforate anus:
➢ Reconstruction by immediate local perineal approach
without colostomy with very good prognosis .
▪ A muscle stimulator is used to localize the sphincter complex.
▪ The anal opening is mobilized and transferred to the normal
position.
▪ After healing, the anal opening is dilated daily for 3-5 months
to avoid stricture.

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II) If high imperforate anus:

a- 3 stages repair consisting of:
1- Temporary colostomy.
2- After 4-6 week, posterior sagittal Anorectoplasty:
• Median sagittal incision that runs from the sacrum to the
anal dimple.
• Closure of fistula.
• Anoplasty: creating an anal opening.
• Dissection of rectal pouch and pull thorough operation
(putting the rectal pouch into the anal opening)
• Reconstruction of sphincter complex.
3- Closure of colostomy after 2-3 months.
b- Recently one stage repair is performed by both posterior
sagittal perineal and laparoscopic approaches.

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posterior sagittal Anorectoplasty

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