congenital hypertrophic pyloric stenosis

1. CONGENITAL
HYPERTROPHIC PYLORIC STENOSIS

2. • One of most common GI disorders during
early infancy.
• Described by Hirschsprung in 1888.
• Hypertrophy of circular muscles of
pylorus results in constriction and
obstruction of gastric outlet.

3. • Incidence: 1-2/1000 live births
• Epidemiology: more in first born males
M:F - 4-5:1
• Etiology: Unknown
• Genetic- 11q14-22 and Xq23
• F amilial
• Gender
• Ethnic origin- more in whites
EPIDEMIOLOGY AND ETIOLOGY

4. ASSOCIATED ANOMALIES
• Esophageal atresia
• Tracheoesophageal fistula
• Hirschsprung disease
• Exomphalos
• Inguinal hernia
• Hypospadias

5. CLINICAL PRESENTATION
History: 2nd - 8th week of life
Projectile, frequent episodes of non-bilious vomiting
30-60 minutes after feeding
• Weight loss
• Persistent hunger
• Jaundice (2%)- due to decreased
hepatic glucoronosyl transferase
associated with starvation

6. Palpable olive shaped mass
(1.5-2cm) to the right of
epigastric area.
Visible gastric peristalsis from
Lt. upper quadrant to
epigastrium
CLINICAL EXAMINATION

7. • Vomiting - loss of H+ and Cl" -- Hypochloremic hypokalemic
metabolic alkalosis
• Protracted vomiting - ECF volume deficit - urinary excretion
of K+ and H+ to preserve Na+ and water
•Initial alkalotic urine becomes acidotic-Paradoxical aciduria
• Hypochloremic hypokalemic metabolic alkalosis with
paradoxical aciduria with secondary respiratory acidosis
• Hyponatremia may not be evident because of hypovolemia
PATHOPHYSIOLOGY

8. DIAGNOSIS
History and physical examination
Abdominal USG:
Pyloric muscle thickness >3-4mm pyloric length > 15-
18mm in presence of functional gastric outlet
obstruction
Upper GI study when atypical presentation or
negative USG
Diagnostic: narrowed, elongated pyloric
channel with pyloric mass effect on
stomach and duodenum — String/ Double
tract / Beak sign

9. BARIUM SWALLOW
Air filled fundus
Duodenal bulb
Narrowed pyloric channel
Barium filled antrum
Normal stomach
String sign

10. DIFFERENTIAL DIAGNOSIS
° Gastroesophageal reflux, with or without
hiatal hernia.
Differentiated by radiologic studies. Also amount of
vomitus is smaller, and the infant does not usually lose
weight.
° Adrenal insufficiency.
Differentiated by absence of metabolic acidosis,
hyperkalemia, and elevated urinary sodium.
° Viral gastroenteritis.
Unusual in infants less than 6 weeks of age. Associated
with significant diarrhea and sick contacts.

11. • Treatment: medical emergency but NOT surgical
emergency
• Definitive treatment: Ramstedt Pyloromyotomy
• Anaesthetic considerations
• Patient related: Infant age group
Severe dehydration
Electrolyte imbalance
• Surgery related: open/ laparoscopic
• Anaesthesia related: Pulmonary aspiration

12. PREOPERATIVE PREPARATION
• Correction of fluid deficits- over 24-48 hrs
Deficit: isotonic fluid 0.9% saline (20ml/kg
bolus)
Maintenance: 0.45% saline in 5% Dextrose at
1.5 times maintenance rate +10-40 meq/L KCL
added once urine output established
Correction of electrolyte imbalances
Prevention of aspiration: aspiration through NGT
Surgery should only take place when
dehydration corrected

13. • Once resuscitated the infant can undergo the Fredet-
Ramstedt pyloromyotomy, which is the procedure of choice.
• Ramstedt described this operative procedure to alleviate the
condition in 1907
• It consist of incision in to the sphincter muscle of pylorus.
• NG tube is passed and gastric content are aspirated just prior
to surgery.
SURGICAL MANAGEMENT

15. o Complications after pyloromyotomy should be
minimal if performed by experienced surgeons.
o Perforation (In a large series of infants undergoing
open pyloromyotomy, the incidence of perforation
was 2.3%).
o Wound-related complications occurred in 1%.
COMPLICATIONS

16. • Post op pain relief
• Post op concerns:
Respiratory depression and apnea
Hypoglycemia
Hypothermia
POSTOPERATIVE CARE

17. INTUSSUSCEPTION

18. DEFINITION
“INTUSSUSCEPTION IS THE TELESCOPING
OF ONE PORTION OF INTESTINE INTO THE
OTHER, FROM PROXIMAL TO DISTAL, BY
PERISTALSIS, PULLING THE MESENTERY
ALONG WITH IT.”

19. AETIOLOGY
Primary or Idiopathic – usually in children
Secondary to a pathological lead point – incidence
increases with age

20. PATHOLOGICAL LEAD POINTS
- Meckel’s diverticulum
- Benign and malignant neoplasms
- Appendix / appendiceal stump
- Henoch-Schonlein Purpura
- Jejuno-gastric Intussusception
- Post-operative
- Others

21. PRIMARY / IDIOPATHIC INTUSSUSCEPTION

22. Composed of three parts
-Entering inner tube
-Returning or Middle tube
-Sheath or Outer tube
*Apex - advancing part
*Intussusception – mass
*Neck – junction of entering
layer with mass

23. NECK
APEX

24. It is an example of
Strangulating Obstruction as
the blood supply of the inner
layer is impaired
The degree of ischaemia
depends on the tightness of
invagination
Later may lead to Gangrene
and Perforation

27. Age5-10 months
ABDOMINAL PAIN
In children, suspicion should be aroused in case of a
healthy child having sudden, severe, intermittent,
cramping abdominal pain.
Between the episodes of pain, the child is quiet.
Later the child becomes lethargic

28. Ileo colic77%
Ileo-ileo colic12%
Ileo-ileal5%
Colo colic2%
Multiple1%
Retrograde0.2%

29. VOMITINGBilious
RED-CURRANT JELLY STOOLS
On examination,
Palpable abdominal mass, sausage-shaped,
located in the right upper quadrant of abdomen,
right lower region is empty – SIGN DE DANCE
Dehydration,abdominal distension
P/R Bleeding

30. VOMITINGBilious
RED-CURRANT JELLY STOOLS
On examination,
Palpable abdominal mass, sausage-shaped,
located in the right upper quadrant of abdomen,
right lower region is empty – SIGN DE DANCE
Dehydration,abdominal distension
P/R Bleeding

31. • History
• Physical examination
• Investigations
DIAGNOSIS

32. Plain abdominal radiograph
- air-fluid level with a paucity of gas in the
right lower quadrant
- sparse gas within the colon
- soft tissue mass
INVESTIGATIONS

33. MULTIPLE AIR FLUID LEVELS

34. Small bowel
dilatation &
paucity of gas in
the right lower
&upper quadrants
.

35. Ultrasound
• Target’ of intussuscepted layers of bowel on transverse view
• Pseudo-kidney’ sign when seen longitudinally

36. PEUDO KIDNEY SIGN
TARGET SIGN

37. Barium enema – both diagnostic and therapeutic
Pre-requisites;
-intravenous hydration
-nasogastric intubation
-broad-spectrum antibiotics
-sedation
Finding – ‘claw-sign’

38. Contraindications;
1). Peritonitis
2). Haemodynamic instability
3). Intussusception located fully in small intestine

39. CLAW SIGN

40. DESCENDING COLON
INTUSSUSCEPTION

41. TREATMENT
1) Hydrostatic Reduction
Procedure - prepare patient
- contrast material (Barium enema) is elevated 36
inches above the table and reduction monitored
by fluoroscopy
- reduction confirmed by resolution of mass,
reflux of barium into proximal ileum
Complication – perforation (1-3% )
Recurrence – 11%

43. 2) Air reduction
-Column of insufflated air is monitored so as not
to exceed 80mmHg in infants <6 months and
120mmHg in older infants for periods of three
minutes
-Reduction is noted when
caecal mass disappears
and the small bowel
becomes distended with
air

44. AIR REDUCTION IN
INTUSSUSCEPTION

45. Operative Management
Indications;
-Failure with previous methods
-Recurrence
-Peritonitis
-Necrotic bowel

46. Procedure;
-Transverse muscle-cutting incision in right lower quadrant
-Mass identified and manual reduction attempted by
retrograde milking of the intussucepiens proximally,
NEVER pull it out
-

47. REDUCTION
END TO END
ANASTOMOSIS

48. If BOWEL is ischaemic and reduction is not possible –
LIMITED RESECTION with a primary end-to-end
anastomosis
-Perforation and significant fecal soiling - ENTEROSTOMY

49. SECONDARY INTUSSUSCEPTION

50. Diverticulum invaginates into intestine and is
carried forward by peristalsis
May be ileoileal or ileocolic
Clinical features - urge to defaecate
-early vomiting
-red-currant jelly stools
-palpable mass
Treatment – surgical resection

52. 2). NEOPLASMS
Benign – common
Can cause partial or total bowel obstruction
Cramping abdominal pain
Palpable mass
Treatment - Surgery
Colon: Intussusception with
neoplasm - adenocarcinoma

53. 3)APPENDIX
Rare
Difficult to diagnose – symptoms non-specific
Intussusception of appendiceal stump after appendicectomy
–within 2 weeks post-operative
-present as abdominal pain, vomiting, bleeding P/R,
palpable mass
Diagnosis – Barium enema, CT scan
Treatment - Surgery

54. Causes sub-mucosal haematoma
Common in children

55. 5). JEJUNO-GASTRIC
INTUSSUSCEPTION
Rare, long-term complication of Billroth II surgery
Clinical features non-specific
Efferent limb, rather than afferent is the
intussusceptum

56. Diagnosis – water-soluble upper g.i. contrast
study shows ‘coiled-spring’ appearance within
gastric remnant
Endoscopy will show jejunal segments as they
migrate in and out of gastric remnant between
episodes of intussusception
CT Scan