Paediatric Surgical

Lectures
Dr S Kasthuri
Consultant Paediatric Surgeon
Teaching Hospital Karapitiya
2023
 Contents

1. Obstructive and GI problems

2. GU Abnormalities, Intersex and gynaecological disorders
3. Pancreatic, Hepatic, Splenic and Billiary disorders,
4. Head and Neck lesions, Hernias, Thoracic and abdominal wall
defects
5. Childhood Tumours
Obstructive and GI problems
1. Oesophagus
1. TOF
2. Achalasia Cardia
3. FB
4. GORD
2. Vomiting
1. Non-Bilious
2. Bilious
3. Intestinal Obstruction
4. Hirschsprungs Disease
5. Imperforate Anus
Tracheo-Oesophageal Fistula
 Oesophageal Atresia and Stenosis

• 1:2500 live births

• Presentation
 Respiratory Distress
 (coughing, choking,
regurgitation with first
feeding)
 Polyhydramnios: TEF 33%. EA
100%.
 Gastric distension
 Desaturation with Breast
feeding
 Inability to pass NGT
 Radiographs show NGT in upper
oesophagus with air in the
stomach except in proximal
fistula.
 Oesophageal Atresia and Stenosis

 Embryology
 Primitive foregut at 4-6 weeks forms a ventral diverticulum to form a trachea
 If posterior deviation of the septum causes incomplete separation and atresia.

 Types
 Atresia with a fistula (88%) -
commonest. The NG tube coiled at
T4-5. Gas beyond.
 Atresia without fistula (8%) – NG
tube at T4-5 and gasless abdomen
 Fistula without atresia (3%) – “H”
type.
 Other varieties about 2%.
 “H” type fistula –
 In fact “N” type with oesophageal
opening very high in the neck (C7-
T1)
 Diagnosis by bronchoscopy and
tube oesophagogram.
 By Right cervical approach.
 Oesophageal Atresia and Stenosis

Preoperatively
Head elevation, NGT suction, Repeated suctioning, H2 blockers

In addition
Echocardiogram – prior to Thoracotomy
For the assessment of for Right sided aortic arch. Found
in 2.5% of children with EA.
PDA
Renal US – usually done after surgery
Bilateral renal agenesis
Multicystic dysplastic kidneys
May need to follow with renal scan
Bronchoscopy – now considered as an essential pre-op evaluation.
Detection of upper pouch fistula
Localization of distal fistula
Detection of aberrant RUL bronchus

We must look for VACTERAL Association

 VACTERAL association – about
30%
• Vertebral anomalies i.e. hemivertebrae,
spina bifida
• Anal malformations i.e. imperforate anus
• Cardiac malformations i.e. VSD, ASD,
Tetralogy of Fallot
• Tracheo-
• Esophageal fistula
• Renal deformities i.e. absent kidney,
hypospadia, etc.
• Limb dysplasia
 Oesophageal Atresia and Stenosis
Repair
Right Thoracotomy via 4th/5th intercostal space
Extra pleural approach
Fistula division close to trachea
Mobilization of proximal oesophagus
Feeding tube across anastomosis
Single layer tension free anastomosis

Complications - Early
Anastomotic leak: 15%.
3 or 4 days post-op. Saliva in chest tube. +/- sepsis
Anastomotic stricture: 50%.
Functionally significant?
Balloon dilatation
For prevention tapering or flap anatomises are routine now
TOF
 Oesophageal Atresia and Stenosis
 Complications - Early
 Anastomotic leak: 15%.
 3 or 4 days post-op. Saliva in chest tube. +/- sepsis
 Anastomotic stricture: 50%.
 Functionally significant?
 Balloon dilatation
 For prevention tapering or flap anatomises are routine now

 Complications Late
 Gastroesophageal Reflux
 Congenital distal dysmotility
 Dysfunction of physiologic antireflux barrier
 Vagal injury/dysfunction leading to gastric
 dysmotility.
 H2 blockers post op for 6months
 Nissen fundoplication
 Oesophageal dysmotility
 domperidone
 Tracheomalacia
 Bronchoscopy reveals trachea that significantly collapses, flattens, or closes on expiration.
Achalasia Cardia

• Due to inability of the lower

Oesophaegeal sphincter to relax
• Food particles collect in the lower
Oesophagus causing
• Vomiting non bilious after meals
• Vomitus has old decomposed foods
• Solid food would be easier to swallow
• Diagnosis
• History
• X-ray – fluid levels in the thoracic
Oesophagus
• Treatment
• Dilatation
• Botulin injection
• Heller’s Cardiomyotomy
Foreign Body ingestion
• Any objects pass through the
cricoid cartilage would pass all
the way through.
• Surgical intervention needed
only
• if it is in the trachea or
bronchus,
• sharp object stuck in the same
place.
• So a chest X-ray is the only
investigation needed
• Few notes of Button battery
ingestion
• we remove either
endoscopically or Lap.
• Oesophagus
• >1.5mm diameter
• Symptomatic
• Concomitant magnet
ingestion
Vomiting (Billious and Non-Billious)

• Alarming symptom at infancy

• Risk of aspiration
• Underlying pathology to be
evaluated immediately
• Non-bilious vomiting
• Commonest is over feeding
• Bilious vomiting
• Intestinal obstruction unless
otherwise proved
Non-bilious vomiting/Pyloric stenosis

• Thickened pyloric musculature causing

obstruction at gastric outlet
• Usually after 6 weeks, first born male,
group ‘O’ blood group.
• By the time they present child is
severely dehydrated and emaciated.
• They have severe electrolyte
imbalances like alkalosis,
hyponatremia and hypokalemia.
• Correction of Na+, K+ and acid-base is
a must. Can delay the surgery until
then.
• Paradoxical aciduria
• Supra umbilical approach.
• Post op wound dehiscence.
Bilious Vomiting

Important ……….

 1. Bilious vomiting is always abnormal.

 2. Abdominal distension (scaphoid abdomen possible).
 3. Delayed, scanty or no passage of meconium.
 4. Polyhydramnios in mother.
 5. Down's syndrome
 6. Family history
 a. Hirschsprung's disease
 b. Diabetic mother
 c. Jejunal atresia
Causes of Billious Vomiting
various categories

• 1. Congenital defects
• Atresia
• Stenosis
• Bands
• Rotational abnormalities
• 2. Other types of defects
• Lack of neurons
• Hirschsprung’s
• Obstructed bowel later in life
• Intussusception
• Obstructed hernia
• internal
Causes of Billious Vomiting
various categories

• 1. Complete Obstruction
• Atresia
• Interstinal Atresia
• Imperforate anus
• 2. Partial obstruction
• Stenosis
• Bands
• Rotational abnormalities
• Malrotation of gut
• Lack of neurons
• Hirschsprung’s
• Obstructed bowel later in life
• Intussusception
Bilious Vomiting
A logical approach to neonatal intestinal obstruction

1. While the infant is being studied, it must be kept in mind that the
problem may be "non-surgical".
a. Sepsis of the new born with associated ileus is the most important
cause of non-surgical bilious vomiting and abdominal distension.
b. Intracranial lesions
i. Hydrocephalus
ii. Subdural haemorrhage
c. Renal disease associated with uraemia.
i. Renal agenesis
ii. Polycystic disease
iii. Other urinary tract anomalies which may be associated with
severe hydronephrosis.
Bilious Vomiting
A logical approach to neonatal intestinal obstruction

2. Plain X-ray of the abdomen.

a. Diagnostic
 in complete high intestinal obstruction (Artesia) -no gas in distal small bowel.
 Double bubble in duodenal obstruction.
 Few gas filled loops beyond duodenum indicates jejunal atresia.
b. Many gas filled loops (requires 24 hours) indicates some form of lower
intestinal obstruction.
I. Ileal atresia
ii. Meconium ileus (an unfortunate misnomer)-obstruction of the distal small intestine by
thick undigested meconium.
iii. Meconium plug syndrome-obstruction of colon by a plug of meconium.
iv. Small left colon syndrome.
v. Hirschsprung's disease-congenital aganglionosis of colon starting with the rectum.
vi. Colonic Atresia.
vii. Imperforate Anus
c. May be nonspecific in instances of Malrotation of the intestines.
This diagnosis must always be considered in neonates with unexplained bilious vomiting .
d. Calcifications-
at some time during fetal life meconium was (is) present in the abdomen.
 Bilious Vomiting
A logical approach to neonatal intestinal obstruction

3. Contrast enema
will differentiate the various types of low intestinal obstruction.
a. Microcolon-complete obstruction of the small bowel.
b. Meconium plug syndrome-colon dilated proximal to an intraluminal mass.
c. Hirschsprung's disease-although it may appear to be diagnostic, not reliable in the newborn.
d. Small left colon syndrome-colon dilated to the splenic flexure, then becomes narrow.
4. Upper G.I.Contrast
• the procedure of choice in diagnosing Malrotation of the intestines. In the past a contrast enema was
thought to be the diagnostic test of choice in instances of Malrotation but the caecum and ascending
colon can be in normal position in an infant or child with Malrotation of the intestines.
5. Rectal biopsy
• a pathologist competent in reading the slides is essential and should not be taken for granted.
 Intestinal Obstruction

Main features
Bilious vomiting
Abdominal distension
Colicky abdominal pain
Absolute constipation

In neonates
Abdominal distension depends on the
The level of obstruction
 More distal more distension
Septicaemia/ NEC
Perforation of viscous
 Antenatal/post natal
Neonates cannot complain of pain.
Instead they are irritable, stops respiration, circulatory collapse but most
importantly grunting and restless specially when we touch
In complete obstruction there is absent of “normal meconium”,
but it is not uncommon to pass some whitish thick slummy material just to
represent what was produced distal to the obstruction
 Partial Intestinal Obstruction

 Cardinal features of partial obstruction are

1. Bilious vomiting
2. Dilated intestine proximal to obstruction
3. Plane X-ray abdomen showing gas shadow beyond the site of obstruction
4. Passage of meconium in neonates

 DD
a. Duodenal membrane

Dilated bowel
b. Annular pancreas
Gas beyond the site of
obstruction
c. Malrotation
 The most important condition to
exclude is the Malrotation

X-ray of a malrotation
Malrotation of the intestine

• Normal abdominal layout

• Bowels are positioned within the peritoneal
cavity to have the longest possible base for
its mesentery (from DJ junction to ileo-
caecal junction) – thus the mesentery
stretches from LHC to RIF in the diagonal of
the abdomen. Normal bowel layout

• In Malrotation
• If intestine are placed in any other way
causing shortening of the mesentery would
prone it to undergo rotation of its own axis
causing ischemia and bowel obstruction or
both.
 Malrotation of the intestine

If the ischemia is long enough

 The compression of the SMA would
leads to loss of the midgut which is
incompatible with life
 So the diagnosis or exclusion should be
prompt with appropriate surgical
correction to give the child best chance
of survival.

 Thus a bilious vomiting neonate should

be considered having Malrotation of
intestine unless proven otherwise.
 Malrotation of the intestine
Diagnosis Bird’s beak appearance

Gas beyond
 High degree of suspicious
 Double bubble appearance with gas
beyond duodenum
 Upper GI Contrast is the gold
standard of diagnosis where it will
be shown the features of
 “Bird’s beak” appearance if the
obstruction is complete
 “Spiral or Corkscrew” appearance if
the obstruction is partial
 If there is no volvulus appearance of
DJ flexure right of the midline Plane X-ray abdomen
Complete obstruction
 US Doppler Scan to assess the SMA

• Correction
• Surgical correction aimed at Spiral or corkscrew
• Correction of the volvulus
• Freeing of duodenal obstructive
bands (Ladd’s bands)
• Making the mesenteric base as long
as possible; by keeping
appendicectomized caecum at RHC
and straightened Duodenum at RIF

Corkscrew appearance of volvulus

 Partial Duodenal Obstructions

• Duodenal membrane
• Mostly at the junction between 2nd
and 3rd parts
• The membrane has a whole through
which gas can go beyond but not food
• The proximal duodenum and the
membrane is usually stretched to cause
a “wind-sock” deformity.
• At surgery this membrane must be
excised/opened up and should be
aware of the possibility of erroneously
anatomising distal to the obstruction .

Windsock deformity
Annular Pancreases

• Failure of the pancreatic buds to develop correctly making it encircle the

duodenum with partial obstruction.
• By-pass procedure needed called Duodenoduodenostomy.
 Complete intestinal Obstruction/Duodenal
Atresia
• When a child presents with absolute constipation, abdominal distension with profuse
bilious vomiting complete obstruction is likely. In newborn atresia is the commonest
reason.

• Duodenal Atresia
• X-ray appearance of “Double bubble” is typical and is enough itself for surgical exploration.
The presence of bile in the vomitus depends on the site of stenosis and mostly, it is distal
to the ampulla of Vater in >2/3rd
• Failure of recanalization. Mostly (65%) beyond Ampulla.
• Again the treatment is a by pass Duodenoduodenostomy (Diamond anastomosis).

Types of Duodenal Atresia Double Bubble appearance

Duodenal Atresia
Duodenal Atresia

Kimura’s diamond shaped anastomosis

•Done between transversely opened proximal pouch and longitudinally
opened distal pouch.
•Less chance of anastomotic complications
 Intestinal (Jejunal/Ileal ) Atresia
Incidents – 1:2000
Pathology
Most of the time a late event
Meaning – after 1st trimester
Since most of the bowel already formed one will see meconium at birth but the
colour and quality is definitely different from the sticky large amount of meconium of
a normal newborn
This is more obvious in the distal bowel atresia
Cause probably a vascular accident
?secondary to a similar phenomenon like Intussusception
Thromboembolic phenomenon
Associated with extravasations of meconium and resultant meconium cysts-Meconium
peritonitis
During laparotomy these cysts have a very thick peritoneal covering which is
intimately adhered to the normal bowel. One should be very careful not to damage
normal bowel in removing these cysts
In X-ray - calcifications
which is a hallmark of intrauterine perforation and extravasation
Beyond the atresia the bowel would not grow normally
with resultant atrophic bowel. Following successful repair they will come back to the
normal calibre and length.
 Intestinal Atresia
Presentation
Intrauterine
Maternal polyhydroamniosis
 Higher the atresia worse the polyhydroamniosis
Serial US Scans would show dilated fluid filled cysts.
At birth
Bilious vomiting
Refusal of feeds
Abdominal distension
 Distal the obstruction more the distension and late vomiting
 Passage of meconium like material
Diagnosis
Clinically
Investigation
Radiology
 Plain X-ray – presence of very few gas shadows are diagnostic of
proximal obstruction. No need of any other studies
 Contrast studies – presence of several gas filled loops would be a
slow transit colon – so a contrast study is needed.
 Lower obstruction – contrast Enema
 Proximal obstruction – upper GI Contrast
Biochemistry – severe dehydration
 Intestinal Atresia
Types – four types
 I – intra luminal diaphragm
 II – cord like segment between
blind ends
 III(a)– Atresia with complete
separation between ends
 III(b) – Atresia with a massive
mesenteric defect
 IV – multiple atresia
 Prognosis depends on
 the type of atresia
 the length of the remaining bowel
 Associated perforation
 Presence of septicaemia
 How soon the remaining bowel comes
back to normalcy
 Thus the presence of parenteral
nutrition in the immediate post op
period is a deciding survival factor,
more so in proximal atresia than
distal
Intestinal Atresia
Intestinal atresia

Prognosis depends on

 the type of atresia

 the length of the remaining bowel
 Associated perforation
 Presence of septicaemia
 How soon the remaining bowel comes back to
normalcy
 Thus the presence of parenteral nutrition in the
immediate post op period is a deciding survival
factor, more so in proximal atresia than distal
 Intestinal Atresia

Surgical Treatment
Only option
Several surgical options
End-to end anastomosis with or without
excision of dilated proximal portion
 End-to-back anastomosis
Faecal diversion
Can occur from DJ Flexure to Ileo-caecal
valve.
Colonic atresia is very rare
Several anal canal atresia also recorded.
Meconium related disorders

1. Meconium ileus
• Associated with cystic fibrosis (10-20%)
• Distal ileum packed with abnormally thick, viscous, inspissated meconium
• Gastrograffin/saline enema
• Surgery only if irrigation failed with surgical stomas
• Prognosis depends on severity of the pulmanory cystic fibrosis
2. Meconium peritonitis
• A chemical peritonitis due to meconium leak to peritoneal cavity in fetal life following bowel
perforation
• Sterile inflammatory reaction
• Simple (healed perforation) or complex (obstruction or pseudocysts)
• Simple – no surgery/ complex – surgical intervention
3. Meconium plug syndrome
• Temporary obstruction of the colon due to a plug of inspissated meconium
• Plug is white and chalky; rarely small bowel.
• Most idiopathic
• associated with prematurity, hypotonia, hypermagnesemia (reduces acetylcholine release with
subsequent myoneural depression), diabetic mother, Hirschsprung’s disease and cystic fibrosis
Imperforate Anus
 without an obvious Anal opening
 Include several forms of anal malformation
 Frequently a fistula present from the distal rectum to the perineum or genitourinary
system
 Diagnosis is usually made at birth or during the newborn assessment
 Clinically
 Absent anal opening
 Abdominal distension
 Vomiting
 Absence of meconium
 Presence of meconium in urine
 Imperforate Anus
• Low imperforate anus:
• the rectum has descended
through the puborectalis sling
and exists as a fistula on the
perineum.
• May see meconium on the
perineum, may be seen in the
rugal folds of scrotum of males
and vagina of females.
• These fistula may be dilated to
temporarily relieve obstruction

• High imperforate anus:

• rectum ends above the
puborectalis sling.
• No perineal fistula, but may
have urinary fistula
• Temporary colostomy is
necessary in all babies with
high imperforate anus.
 ALGORITHM FOR THE MANAGEMENT OF MALE
PATIENTS
PERINEAL INSPECTION

CLINICAL EVIDENCE QUESTIONABLE

PERINEAL FISTULA MECONIUM Lateral shoot through

MEDIAN RAPHE FISTULA IN URINE
BUCKET HANDLE
ANAL STENOSIS
ANAL MEMBRANE
COLOSTOMY >1cm < 1 cm
( NEWBORN) BOWEL- SKIN BOWEL-SKIN
OR QUESTIONABLE

PERINEAL OPERATION PSARP PERINEAL OPERATION

(NEWBORN) (3mo) (NEWBORN)
NO COLOSTOMY NO COLOSTOMY
 Imperforate anus - Female
• By looking at the vestibule
• Three fistula
• Urethra
• Vagina
• Vestibular anus
• Two fistula
• Urethra
• Vagina and recto-vaginal
fistula
• Single fistula
• Cloacal anomaly
 ALGORITHM FOR THE MANAGEMENT OF
FEMALE PATIENTS
PERINEAL INSPECTION

FISTULA NO FISTULA

CLOACA VESTIBULAR PERINEAL Lateral Shoot-through

VAGINAL

< 1cm > 1 cm

BOWEL – SKIN BOWEL – SKIN

COLOSTOMY COLOSTOMY COLOSTOMY

( NEWBORN ) ( NEWBORN ) ( NEWBORN )
± VAGINOSTOMY
± VESICOSTOMY

PSARVUP PSARP MINIMAL PSARP PSARP

( 6 mo ) ( 3 mo ) ( NEWBORN ) ( 3 mo )
NO COLOSTOMY
 Imperforate Anus

• Associated defects
• Urogenital
• Most common
• 20-40%
• Higher the defect higher the
incidents
• Sacrum and spine
• Sacrum frequently abnormal
• Deformed
• Abnormal
• hemisacrum
• Spine frequently showing
hemivertebra
• VACTERAL association
• As described previously
Imperforate Anus

What really matters in the

initial diagnosis is whether
a particular patient needs
a colostomy or not
 Imperforate Anus
 After colostomy
 Clearing of all meconium
from the distal segment
 Distal loopogram to
ascertain the level of
ending of rectum
 The most important
single investigation
 Water soluble contrast to
distal segment
It would show
Sacrum
Height of rectum
Perineum
Fistula location
Bladder, VUR and
urethra
 Imperforate Anus

PSARP
Is the treatment of choice
Principle is to
Maintain a midline position
to prevent damage to nerves
Identifying the sphincter
complex by a nerve
stimulator
Urethral fistula to be
mobilized and divided within
the rectum
Placement of the rectum
within the sphincter complex
Post op dilatation regime up
to 2 years
Hirschsprung’s Disease
• The presence of normal amount
and distribution of ganglion cells
• are important in the process of peristalsis
to propagate the bolus of food. In the
absence of ganglion cells that particular
segment of bowel fails to relax to an on
coming food and remain spastic. This
causes the proximal normal bowel to get
loaded with stool which eventually
distended to massive proportions.

• Absence of ganglion cells

• in the Auerbach’s plexus of the intestine.
• The defect is from caudo-cephalic
direction
• leaving distal segment affects first and
always before the proximal portion
Hirschsprung’s Disease

• Recent finding of genetic

involvement broaden our
understanding of this disease
• Common in Trisomy 21 and HD
patients get more of MEN IIA
• Proximal to aganglionosis portion
may have abnormal or/and
immature ganglion cells (IND) in 20-
70% of cases which may be the
reason of fatal colitis.
• Until neurone become mature (around
4th year) the risk of fatal colitis exist.
 Hirschsprung's disease

 Presentation
 Not passing meconium at birth
 >95% child with HD would not pass meconium
within 48hours of birth
 If present late, on direct inquiry can find that they all did
not have a normal neonatal period;
 Later presents with chronic constipation, huge abdominal
distension with mega colon and colitis.
 Diagnosis
 Clinically
 As above
 Radiologically
 Plain X-ray abdomen
 Lack of gas in the rectum
 Contrast enema
 Reversal of recto-sigmoid ratio
 Histologically
 Rectal biopsy in suspected cases and then follow
up with levelling biopsies
 Hirschsprung’s disease

Treatment
 Initially levelling colostomy proximal to the aganglionic segment to
 Allow normal bowel motion
 Allow distal dilated bowel to get back to normal size
 Allow child to grow before a major procedure needed
 The initial biopsy sites are marked with non-absorbable sutures
 to see them later and identify accurately the exact site.
 The aganglionic bowels are removed and the ganglionic segment is anastomosed to
the anal skin
 Several techniques available
 Swenson’s
 Soave-Boley
 Duhamel’s
 Each has its advantages and disadvantages. Choice depends on the surgeon and
availability of instruments/ patient’s age etc
 Nowadays surgical trend is to perform lesser number of surgeries in simpler form
 Primary pull through with or without colostomy
 Neonatal Pull Through
 Trans Anal Pull Through (with or without Laparoscopic assistance)
 Intussusception

• Intussusception
• The pushing of a proximal bowel through a
distal bowel lumen due to a lead point.
• Common between 6 months to 18 months.
Mainly ileo-colic. Due to ?payer’s patches,
Gastroenteritis
• Outside this age a leading point must be
sorted
• Meckel’s
• tumour
• Most managed with saline reduction and
failing open surgery. Might need stoma