neonatal intestinal obstruction

1. INTESTINAL ATRESIAS
AND MECONIUM ILEUS
DR T SASIDHAR
MODERATOR: DR NARAYANA MS MCh

2. Duodenal atresia and stenosis
• Duodenum is one of the most common sites
of neonatal intestinal obstruction
• Can be intrinsic or extrinsic

3. • Hidtorical aspects:
• 1733- calder – 1st reported case
• 1901 – cordes- described typical clinical findings
• 1905 – vidal from france & 1914 – ernst from
netherlands- credited with the first successful
surgical repairs
• Historically duodenal atresia was encountered in
association with trisomy 21 (Down syndrome), a
finding in nearly one third of patients

4. Incidence:
• 1:6000 to 1:10000
• no specific genetic abnormality is known to
cause duodenal atresia
• reports of the anomaly occurring among
siblings and among several generations of a
family, as well as its frequent association with
trisomy 21, suggests that one may be present.

5. Embryology
• During the third week of embryonic
development, gastrulation occurs
• The cellular surface of the embryo facing the
yolk sac becomes the endoderm, the surface
facing the amniotic sac becomes the
ectoderm, and the middle layer becomes the
mesoderm

6. Theories
• Tandler:
– In the sixth week, the gut epithelium proliferates
rapidly, resulting in obliteration of the intestinal
lumen. The intestine is then gradually recanalized
over the next several weeks of development.
Errors in recanalization are thought to be the
primary cause of duodenal atresia and stenosis

7. • Boyden et al- described D2 as embryological
traffic jam. He suggested a relation between
duodenal atresia and the associated
anomalies of the bile ducts.
• He suggeted that a delayed coalescence of
vacuoles to form 2 orifices of bile duct took
predomenence over the recanalization of the
solid cord portion of the adjescent region of
the duodenum.

8. classification
• Several varieties of intrinsic and extrinsic congenital
lesions can cause complete (81%) or partial (19%)
obstruction
• The spectrum of abnormalities causing intrinsic
obstruction includes imperforate and perforate webs of
variable thickness within continuous bowel as well as
complete or almost complete bowel discontinuity
• Abnormalities causing extrinsic obstruction include
annular pancreas, preduodenal portal vein, Ladd
bands, and volvulus.

9. Gray and Skandalakis
• Type 1 (92% of cases): There is an obstructing septum
(web) formed from mucosa and submucosa with no
defect in the muscularis.
• The mesentery is intact
• A variant of type 1 duodenal atresia, a “windsock
deformity,” can occur if the membrane is thin and
elongated. The base of the membrane usually lies in
the second portion of the duodenum, but balloons out
distally, distending the third and fourth portions.
• Thus, externallythe obstruction appears considerably
more distal than it actually is

11. • Type 2 (1% of cases): A short fibrous cord connects the two
blind ends of the duodenum. The mesentery is intact.
• Type 3 (7% of cases): There is no connection between the
two blind ends of the duodenum. There is a V-shaped
mesenteric defect.
• Associated bile duct anomalies seen in duodenal atresia are
usually noted with type 3 atresia.
• Annular pancreas may be associated with duodenal atresia
• Multiple atresias can be seen in around 15% of cases.

13. • In type 1 atresia, the obstructing septum may
vary in thickness.
• Imperforate septa cause a complete
obstruction, where as those with central
perforations cause incomplete obstruction.
• With perforate septa, the diameter of opening
directly determines the degree of obstruction

14. Typical duodenal web with central perforation

15. • Although intrinsic blockage may occur in
almost any portion of the duodenum, it occurs
near the junction of the first and second
portions in 85% of cases.

16. Duodenal steosis
• Partial obsturction can be caused by a
diaphragm with a central defect or eccentric
opening.
• Stenosis may be associated with extrinsic
obstruction in the form of
• Annular pancreas
• Mesenteric bands
• Preduodenal vein

17. Annular pancreas with
underlying web

18. • The distal portion of the common bile duct often
traverses the medial portion of the septum, with
the ampulla commonly located on the proximal
surface of the obstruction.
• In rare instances, the distal portion of the duct is
bifid, with proximal and distal openings- at first
glance appears to be an impossible situation of
air and bile distal to a complete congenital
obstruction

19. • Fonkalsrad et al – review of 504 patients
– Atresias- 47%
– Diaphragms- 41%
– Stenosis- 10%
• Among the diaphragms
– 20% are preampullary
– 60% are post ampullary
– 14 % are uncertain

20. Associated anomalies
Associated anomaly percent
Down syndrome 28.2
Annular pancreas 23.1
Congenital heart disease 22.6
Malrotation 19.7
EA/TEF 8.5
Genitourinary 8.0
Anorectal 4.4
Other bowel atresia 3.5
Other 10.9
None 45

21. Downs syndrome
• Nearly 30% of neonates with duodenal atresia
are associated with down syndrome
• Bilious vomiting in any infant with down
syndrome mandates evaluation for duodenal
atresia.
• Nixon and Treves- observed high incidence of
preampullary obstruction in their downs patients.
This is not confirmed in other studies.

22. Clinical features
• Prenatal:
– materal polyhydramnios 30-65%
– Fetal usg-
• classic double bubble sign
• Larger of the twin bubbles is stomach and the smaller
one is proximal jejunum.
• Annular pancreas may also be found.

23. Post natal:
• Majority present on 1st or 2nd day of life.
• Repeated bilious emesis is the characteristic clinical
feature of almost all newborns (85%)
(note- 15% of babies may present with nonbilious
vomiting of the obstruction is preampullary and in such
cases the diagnosis is delayed)
• Routine NG tube intubation and aspiration should be
performed in all babies of mothers with a history of
polyhydramnios.
• Continuous vomiting can cause gastritis and blood
statined vomitus.

24. • On examination abdomen shows epigastric
fullness and visible gastric peristalisis.
• Marked epigastric ditension may suggest an
esophageal atresia with distal TEF
• Baby may pass normal meconium
• Jaundice may be present
• Features of downs syndrome may be noted.

25. • Delayed presentation may lead to dehydration
and metabolic disturbances
• In cases of partial obstruction the presentation
may be delayed and they usually present with
• Recurrent vomiting
• Failure to thrive
• Episodes of aspiration
• Baby present most commonly when advancing
the infant to formula or solid feeds

26. • Late recognization in adult life may be with
– Symptoms of peptic ulceration
– Reflux esophagitis
– Obstruction of duodenum proximal to the stenosis
with bezor or a foreign body.

27. diagnosis
Prenatal usg:
• shows maternal polyhydramnios in 30-65% of cases
• Most of the cases are detected at 7th to 8th month of
gestation.
• Duodenal obstruction is suggested by the presence of
dilated fluid filled stomach
• the risk of preterm labour and prematurity can be
decreased by decreasing amniotic fluid by amniocentesis
• Ultrasonography can also detect other associated
anomalies
• A chromosomal analysis is warranted to confirm or rule out
the association with trisomy 21.

29. Plain x-ray abdomen:
• Double bubble sign suggest complete obstruction
with lack of more distal intestinal gas
• Absent double bubble sign suggestive of
duodenal atresia with pure esophageal atresia
• Double bubble sign with digital gas filled bowel
suggestive of partial obstruction (duodenal
obstruction with bifid biliary tract)
• The sensitivity of in X Ray abdomen can be
increased by injecting 20 to 30 ml air via NG tube

30. Plain abdominal film of newborn with
duodenal stenosis

31. Upper gastrointestinal contrast studies
• Rules out malrotation with volvulus- the
twisted bowel is gasless which gives the
impression of complete intrinsic obstruction it
shows cork screwing of deodenum

32. Upper gastrointestinal (UGI) radiographic
contrast study of
duodenal atresia.

33. Ultrasonography
• Shows dilated fluid filled stomach and proximal
deodenum
• Malrotation is suspected when abnormal position
of mesenteric vessels is noted, whirlpool sin is
suggestive of volvulus.
• Annular pancreas can be identified
• Pure esophageal atresia with duodenal atresia
will show dilated stomach and duodenum
suggestive of duodenal obstruction

34. Management
• It consists of pre operative stabilization and Surgical Management
• Preoperative stabilization and workup
– Nil per orally + ryles tube aspiration
– Intravenous fluids
– Intravenous antibiotics
– inj vitamin K
– Warmth care
– Rule out associated anomalies
• Clinical examination to rule out other atresia
• 2D echo
• Ultrasonography of kidney bladder
• Chromosomal studies
– Blood grouping typing and cross matching

35. Surgical principles
• duodenoduodenostomy
• Duodenojejunostomy
• Septectomy
• Tapering duodeno plasty

36. duodenoduodenostomy
• It is the procedure of choice for treatment of
duodenal atresia with or without an associated
annular pancreas
• Principal steps include
– Right supraumbilical transverse incision
– Inspection of abdomen to rule out malrotation and
evaluate for other associated anomalies
– Examine entire intestine to ensure patency
– Adequate kocherization to mobilise duodenum from
hepatic flexure

38. • Ladd's procedure malrotation is noted.
Malrotation identified is psudomalrotation
usually. Absence of ligament of treitz will be
noted in patients with true malrotation.
• Side to side anastomosis of the proximal and
distal pouch using single layer interrupted
sutures of 5-o or 6-0 silk

39. • A diamond shaped anastomosis described by kimura
involved transverse incision over the proximal deodenum
and vertical incision over the distal deodenum. In the apex
of this vertical incision is sutured to midpoint of the incision
on the proximal deodenum.
• The anastomosis itself will maintain in a more wide open
position
• With Type III deformity, the end of distal segment is
spatulated appropriately. Before making incision on the
distal sigment it is useful to inject saline into the lumen
while occluding the bowel distally.

42. Duodenojejunostomy
• It is abandened universally
• It is an alternative in some cases with distal
duodenal obstruction where the
duodenoduodenostomy not feasible
• The problems associated with
duodenojejunostomy are
• 1. slow onset of anastomotic function
• 2. gastric and duodenal ulcer
• 3. high incidence of mega duodenum and associated
problems
• 4. high incidence of blind loop syndrome

43. Tapering duodenoplasty
• It is done if the proximal duodenum is floppy
and dilated- as an initial part of procedure
• It is accomplished either by suture plication or
by a resection using a GIA stapler or a needle
tip electrocautery and suture and closure
• The tapering is positioned along anterior and
Anterolateral surfaces

44. septotomy
• Where there is continuity of the proximal and distal
duodenum, it is best to open the distal bowel near the
Apparent point of obstruction, in a position and
direction suitable for a potential bypass
• The filmy thin webs are often excised rather than
bypass. In this case, the insertion is carried over the
web into the proximal lumen and web is excised with
electrocautery
• The duodenum is closed as a duodenoplasty in
transverse manner (Heinkie-Miculicz) to minimise the
risk of stenosis

45. • Congenital obstruction of distal deodenum at or
near the ligament of treitz presents especially
different problem.
• The long segment of atonic dilated duodenum
difficult to effectively taper, so the reconstructed
Bowel and anastomosis may function poorly
• When annular pancreas is present, bypass is
always the procedure of choice

46. laparoscopic technique
• 3- or 5-mm ports can be used
• laparoscopic duodenoduodenostomy
• technique of repair is the same as the open
approach using a diamond-shaped anastomosis
• Either running sutures or single interrupted
sutures can be used for the anterior and posterior
walls.
• The apical stitches can be tied extracorporeally
and left on tension through the abdominal wall to
help align the enterotomies. Alternatively, U-clips
can be used to perform the anastomosis.

47. • The approach becomes more difficult in
infants weighing less than 2 kg, because there
is little working space.

48. Post operative care
1. Continue NPO + RTA in stools pass and RT becomes
clear or Golden yellow and is less than 1 ml/kg/hour
2. Bile in drainage is not uncommon since there is
duodeno gastric reflex
3. Use prokinetic agents
4. Earth baby in prone position or right side
5. TPN till gastrointestinal function Returns
6. Phototherapy for hyperbilirubinaemia if required
7. Start feeds with elemental diet and low osmolar feeds

49. Use of gastrostomy and trans
anastomotic tubes
Gastrostomy -Very few surgeons advocate now, less
useful.
Trans anastomotic tubes
• Can be used for early postoperative feeding
• Problems associated are
– Difficult insertion
– kinking and displacement of tube back into the stomach is
common
– Non Silicon tubes can cause trauma to the anastomosis
• TPN is an ideal alternative to transanastomotic feeding

50. • Concern-- if relatively normal function has not
been achieved by 3 weeks.
• At that time, and upper GI series is helpful to
search for
– Residual anatomic obstruction
– Anastomotic stenosis
– Previously unrecognised obstruction at different
level
– Poor peristalsis

51. • Late duodenal obstruction may occasionally be noted
in older infants and children when a piece of fibrous
food or a foreign body may become impacted.
Results:
• Survival improved from 68% to 1969 to 90% at 1980
• Mortality was related to associated malformation and
prematurity in 50% of cases
• in present era, complex cardiac defects associated with
duodenal atresia are the main cause of mortality

52. Long-term complications
• Mega Duodenum with or without stasis
• Bile Reflux gastritis, peptic ulcer
• Duodenal diverticula
• Cholelithiasis, cholecystitis
• GERD
• Duodenal polyp
• Adhesive intestinal obstruction

53. • Megaduodenum can occur due to anastomotic
obstruction or impaired motility of the proximal
duodenum.
• symptoms include vomiting, poor weight gain,
abdominal pain
• Tapering duodenoplasty or kimura
duodenoduodenostomy shall reduce the
incidence
• There is high incidence megadeodenum with
duodenojejunostomy.

54. Jejunoileal atresia

55. Jejunoileal atresia
• Most common small intestinal atresias and are
the major cause of intestinal obstruction in
neonates
• Among them atresias- 95% and stenosis- 5%
(partial obstruction)

56. • Historical notes
• 1684- Goeller- first description of ileal atresia
• 1770- Calder And Osiander- later to describe
• 1800- Viosin- first to perform enterotomy
• 1889- Bland And Sutton- proposed
classification system
– Postulated that defects occured at the sides of
obliterative embryological events such as atrophy
of vitalline duct

57. • 1894- Wanitschek- first resection and end to
end anastomosis
• 1900- Tandler- proposal theory of failure of
recanalization and vacuolation
• 1911- Fockens- credited first successful
operation

58. Prevalence
• Vary among different regions of the world
• Highest in African American children
• Factors that increase the risk include
– Use of pseudoephedrine alone or in combination with
acetaminophen
– Receiving ergotamine tartrate and caffeine for the treatment of
migraine headache during pregnancy
• ileojejunal : duodenal = 2:1 by Nixon& Tawes
• Male:female = 1:1
• Mean birth weight equals 2.7kg (0.9-4.8 kg)
• 33% of jejunal, 25% of ileal, and 50% of multiple atresias
have low birth weight

59. Embryology
• Various theories
1. Developmental defects
2. Inflammatory changes
3. Vascular accident

60. Developmental defects:
• Bland& Sutton: Atrisias occurred secondary to
excessive resorption of vitello intestinal duct
• Politzer: Disproportionate growth of gut and
inadequate endodermal proliferation
• Tandler: Failure of recanalization and
vacuolation
• Montsouris- subsequently showed that there
is no solid stage in small bowel except in
duodenum as described by Tandler.

61. Inflammatory changes
• Bernstein: Atrisias can occur due to scar
formation following perforation secondary to
meconium ileus or other causes

62. Fetal vascular accidents
• Chiari: found intussusception as a cause
• Spriggs: Resorption of a segment of intestine due
to vascular compromise after strangulation in the
umbilical ring
• Louw &Bernard: Produced all types of atresias in
an experimental modal after inducing ischemia
– They also noted the presence of bile pigment
squames, implying initial intestinal continuity. They
proposed the theory which stated that late gestational
intrauterine vascular accident cause jejunoileal
atresia.

63. • Volvulus, internal hernia, intussusception and
occlusion of mesentery in a tight abdominal
wall defect are also proposed mechanisms
leading to atresias
• Iatrogenic cause - accidental clamping of
hernia of the cord has been reported

64. • Theories put forth for heriditary multiple interstinal
atresias include
– Intrauterine inflammatary process of unknown origin in
genetically susceptible individuals
– puri et al- suggested that all cases of hereditary multiple
interstinal atresias have resulted secondary to or
malformative process of GIT rather than a late ischemic
event
– Failure of recanalization and vacuolation
– Abnormal epithelial growth due to genetic defect
• Of all the theories, vascular accident theory is the most
accepted theory at present

65. • Genetic contribution
– Unclear
– Numerous other reported HMIA.
– Both autosomal recessive and autosomal dominant inheritance
have been proposed.
Shorted and collegues proposed novel classification system for 5
types of familial atresias
• The basic premise of these authors is that the identification
of familial cases of these various forms indicates the
existence of specific embryologic pathways; when
disrupted, these pathways give rise to intestinal atresias.

66. Classification of Familial Cases of
Gastrointestinal Atresia
Class Description Genetics
1 Pyloric atresia (may include
antral atresia or atresia of the
proximal duodenum)
AR
2 Duodenal atresia—second or
third portion of duodenum, no
other atresias
AR
3 Multiple atresia syndrome,
rotation is usually normal
AR, uniformly lethal
4 Apple-peel atresia, proximal
atresia is
typically at the duodenojejunal
junction, almost always
associated with malrotation
AR
5 Colonic atresia, rarest type,
usually in
the sigmoid colon
X linked recessive

67. Associated anomalies
• Low incidence of associated anomalies - 7%
• Down syndrome 0.5-0.8 %
• Prematurity 35%
• gastroschisis 8 to 13%
• malrotation 10 to 20%
• Meconium ileus must be considered in patients with jejuno
ileal atresia who have signs of prenatal volvulus, meconium
peritonitis or microcolon with pallets of meconium
• There are isolated reports of Jejunoileal atresia associated
with esophageal, gastric, colonic atresia, ARM,HD, spinal
dysraphism, biliary atresia.

68. Classification
• Louw and Bland Sutton classification
• Modification by Martin& Zerella
• Modification by Grossfeld

69. Louw and Bland Sutton classification
• Type 1- membranous atresia
• Type 2- blind ends connected with fibrous
cord with or without mesentric defect
• Type 3- blind gut ends separated by a gap with
associated mesenteric defect

70. Modification by Martin& Zerella
• Type 1- membranous atresia
• Type 2-blind ends connected with fibrous
band or seperated by a gap
• Type 3 – multiple atresia
• Type 4- apple peel atresia.

71. Modification by Grossfeld
• Type 1- membranous atresia
– 20 to 30%
– Variant - windsock deformity
– Bowel length is normal
• Type 2-
– blind ends connected with fibrous cord with intact
mesentery
– 20 to 25%
– Small bowel length is usually a normal

72. • Type 3a-
– blind gut ends separated by a gap with associated
v-shaped mesenteric defect
– 15%
– Small bowel length is subnormal due to
intrauterine resorption of compromised bowel.
– Cystic fibrosis is commonly associated withthis
deformity

73. • Type 3b-
– apple peel atresia
– Less than 5%
– Occurs due to extensive infarction of midgut secondary to
proximal superior mesenteric artery occlusion or a primary
developmental failure of distal vessels.
– Superior mesenteric artery is absent beyond origin of
middle colic artery. distal ileum receives blood supply by a
precarious collateral from right colic artery. ileum coils
around this vessel leading to apple peel/Christmas tree/
may pole atresia.

74. • Radiological features of
– High small bowel obstruction
– Malrotation
– Microcolon
– And coiled spiral like terminal ileum are unique
features
• There may be associated Type 1 and 2 atresia in
the distal Bowel
• Occurrence in families lead to suggestion of
autosomal recessive inheritance

75. • Associated with malrotation in up to 50% of
cases, prematurity in up to 70% of cases
• There is increased morbidity (62-70%) and
mortality (54%)
• Contributing factors include
– Prematurity
– Short gut syndrome
– Abnormal tenacious blood supply

76. • Type 4-
– multiple atresia
– 10 to 25%
– Multiple address of type one or two or three
present, gives morphological appearance of String
of sausages

78. HMIA
Described by Gutman et al in 1973
• Autosomal recessive mode of transmission
• All patients had numerous type 1 atresia and
nearly one third have type 2
• Type 3 atresias have never been observed
• Atresia located anywhere between pylorus
and rectum

79. • Histologically septae have a sieve like
appearance consisting of multiple small cysts
and there is diffuse acute and chronic
inflammation of bowel
• There may be associated cystic dilatation
bowel
• The uniformly fatal outcome of HMIA maybe is
secondary to poor bowel function and primary
cellular and humoral immune deficiency

80. Pathophysiology
• Primary pathology importantly involve proximal
Bowel
• Ischemic insult and dilation resulting secondary
to obstruction impair motility of bowel
• Blind ending proximal bowel is dilated,
hypertrophied and shows abnormal peristalsis
• villi are histological in normal, but brush border
mucosal enzymes are deficient and muscular ATP
levels are low

81. • enteric ganglia at the level of a atresia are a
atrophic and show minimal ACh activity
• These all changes are attributed to local
ischemia at the terminal end of proximal
bowel
• This changes normalise proximally and are
evident up to 20 CM cephalad
• Proximal bowel often looks discoloured and
cyanotic

82. • It is now believed that this vascular compromise is a Postpartum
phenomenon and results secondary to swallowing of air and
progressive distension- tension gangrene of Rutherford
• Peristalsis in the dilated intestine proximal to the atresia was
abnormal. The dilated intestine lacks propulsive function because of
the lower intestinal pressure After relief of obstruction
• The distal bowel has hypertrophied, tortuous and intertwined villi
which often obliterate the lunen. The villi may be hypertrophied 2-3
times normal. It has potential for normal function after bowel
continuity is restored

83. Clinical presentation
Clinical presentation jejunal atresia (%) ileal atresia(%)
Polyhydromnios 38 15
Bilious vomiting 84 81
Abdominal distension 78 98
Failure to pass meconium 65 71
jaundice 32 20

84. Post natal
Vomiting
• Bile coloured gastric fluid or a residual volume of more than 25 ml is
suggestive intestinal obstruction
• Symptoms usually start on first day of life. In less than 20% 2nd or
3rd day of life
• Onset of vomiting and abdominal distension bears inverse relation
• With delay diagnosis there can be preparation with metabolic
derangement, acidosis, respiratory distress, and features of
septicemia
Failure to pass meconium
• In less than 20% with jejunal atresia- evacuate normal meconium
shortly after birth

85. Jaundice
• Unconjugated hyperbilirubinemia
• Nearly 40% of babies with proximal atresia
and 20% in distal atresia
• This is caused by the presence of 4 beta
glucosidase in neonatal intestinal mucosa. This
enzyme unbinds conjugated bilirubin leading
to increased enterohepatic circulation of
unconjugated bilirubin

86. On examination
– Abdomen shows distention
– Visible bowel loops
– Tenderness, rigidity, abdominal wall edema and
periumbilical flare- suggestive of peritonitis or gangrene of
Bowel.
– Although distension usually develops 12-24 hrs after birth,
distension may be noted at birth and this suggests presene
of a gaint cystic meconium peritonitis.
Partial obstruction/ stenosis
– Present weeks or months later with history of poor
feeding, intermittent vomiting and failure to thrive
– High Index of suspicion is required

87. prenatal
• Polyhydramnios
• Positive family history in patients with Type 3b
or 4
• Bile stained liquor with increased bile acid
concentration in amniotic fluid
• Presence of echogenic dilated bowel loops on
maternal ultrasonography after 20 weeks of
gestation is suggestive of obstruction.
• MRI is superior to USG

88. Fetal magnetic resonance imaging (MRI). A, Axial. B, Coronal. Prenatal
ultrasound demonstrated a bowel obstruction. MRI shows multiple
dilated, fluid-filled bowel loops with no meconium. Findings are
suggestive of distal bowel obstruction

89. Imaging
Plain x-ray abdomen
1. Both erect and supine lateral films are taken
2. Thumb sized intestine loops and air fluid levels
are suggestive of neonatal intestinal obstruction
3. Number of air fluid levels may vary depending
on the level of atresia with proximal jejunal
atresia showing three to four levels only
4. Absence of gascash in distal bowel is suggest to
of complete obstruction

90. 5. Calcification suggestive of ? meconium peritonitis
6. Soap bubble appearance is suggestive of
intestinal atresia
7. Free intraperitoneally suggestive of perforation
8. In case of HMIA- findings of high intestinal
obstruction at the level of stomach or
deodenum with characteristic dense
homogenous oval opacity caused by
intraluminal calcification in middle and lower
abdomen (string of pearls appearance)

91. Contrast studies
• Barium enema: is done and useful in
– Differentiating colonic atresia from ileal atresia
– Diagnosing malrotation
– Washing out the firm mucus or meconium for safer
primary anastomosis
• Upper gastrointestinal series: Indicated in
– Evidence of incomplete obstruction
– In differential diagnosis for sepsis or midgut volvulus
associated with malrotation
– Stenosis or other forms of partial obstruction

92. Other routine investigations include
• CBP, TLC, DLC, platelet count, RBS, blood urea,
serum creatinine, serum electrolytes, serum
calcium serum bilirubin, acid base status,
blood culture sensitivity, urine culture
sensitivity
• Low or downward trend of platelet count is
suggestive of sepsis

93. Differential diagnosis
Medical
1. Septicemia
2. Maternal drug addiction
3. Neurogenic ideas secondary to cerebral birth
injury
4. Functional intestinal obstruction of
prematurity and hypothyroidism

94. Surgical
1. Meconium ileus
2. Malrotation, midgut volvulus
3. colonic atresia
4. HD
5. Meconium plug syndrome
6. Diabetic left colon syndrome
7. Mesenteric cyst
8. Duplication cyst
9. NIO/ perforation of other causes
10. Neonatal appendicitis
11. NEC
12. Neonatal intussusception

95. Management
• Includes preoperative preparation and operative treatment
preoperative preparation include
1. Assessment of dehydration and correction
2. Ryles tube aspiration + NPO
3. Warmth care
4. Correction of acid base defects
5. Preoperative antibiotics
6. Injection vitamin K
7. Blood grouping typing with cross match
8. Monitor urine volume, specific gravity and osmolarity
• Then plan for surgery

96. Operative treatment
• has multiple options depending on intra operatively
• Historically, side to side anastomosis was done this resulted in
functional obstruction and also blind loop syndrome
• Nixon noted - proximal atretic segment has significant smooth
muscle hypertrophy and enlargement of bowel diameter. This is
more significant proximal to the obstruction. Studies have
demonstrated the presence of hypoplasia of the enteric intramural
nerves and C-kit positive pacemaker cells. there is remarkable
decrease of interstitial cells of cajal.
• They advised when dealing with a proximal jejunal atresia,
resection of the proximal dilated atretic sigment of bowel up to the
ligament of treitz, followed by end-to-oblique anastomosis

97. 1. Resection + end to end anastomosis in uncomplicated cases
– Patency of distal bowel should be confirmed
• The types of anastomosis include
– End-to-end oblique by Benson
– end to back by Nixon
• This is contraindicated in
– Established meconium peritonitis
– Volvulus with compromised vascularity
– Associated meconium ileus
– Some cases of type 3 atresia e condition

99. • In these cases available options include
1. Double barrel enterostomy (mickulicz)
2. Bishop koop enterostomy (distal chimney
enterostomy)
3. Santulli enterostomy (Proximal chimney
enterostomy)
4. Rehbein tube enterostomy

101. 2.Tapering jejunoplasty :
In the clinical setting of inadequate bowel
length that could potentially lead to
dependence on parenteral nutrition (short-
bowel syndrome), tapering of the dilated
bowel on the antimesenteric border should be
performed with an end-to-end anastomosis

102. 2.Tapering jejunoplasty

103. 2. In case of Type 1 membrane Atresia -Limited
resection and anastomosis is done
3. Multiple atresia
• In multiple atresia with diaphragm and a short intestine
length, a 17 f gum elastic bougie can be used to
perforate the diagram - shahi Kabab technique
• If bowel length is short- multiple anastomosis can be
done
• Other option include small bowel transplantation

104. Repair of type IV jejunoileal atresias. A,
Operative photograph of a neonate with
multiple small bowel atresias.
B, Illustration documents multiple atresias. The
distal atresias are not dilated.
C, Proximal tapering jejunoplasty and multiple
anastomoses for types II and IIIa atresias and a
transverse enteroplasty for type I atresia were
performed to preserve bowel length.

105. 4.Type 3b
• Restricting bands are released
• Proximal tapered is tapered or plicated and
standard anastomosis is performed
• bowel is replaced in position of non rotation

107. 5. Atresia associated with abdominal wall
defects:
– Repair of abdominal wall defect followed by Bowel
decompression and total parenteral nutrition, in
the 2nd stage re exploration and anastomosis is
done in 2 to 3 weeks
– A primary single layer anastomosis can be done
– In presence of well with doubtful viability,
primaryclosure of the defect with proximal
enterostomy lateral to rectus muscle is done

108. Post operative care
1. Warmth care
2. IVF 1/2 DNS + 2-3 meq/kg KCl with the total fluid volume goal of
100ml/kg/day
3. Total parenteral nutrition
4. If gastric output is clear and >30 ml/kg/day, the laws is replaced
with 1/2 NS. If the output is bilious it is replaced with RL
5. Goal is to get urine output of 1.5 - 2 ml/kg/hr with specific gravity
1.005- 1.015

109. 6. When bowel function returns and gastric aspirate
reduce remove RT- start oral intake with low
volume hypoosmolar feeds containing medium
chain fatty acids
7. Initiate feeds ranged 5 to 15 ml per hour. Start
with clear fluid and Later with milk-based feeds
8. Formulas that contain long chain fatty acid
should not be used
9. Overall feeding goal is to get calories of 120 per
kg per day

110. • Etiology of GIT dysfunction in patient with
atresia is multifactorial
1. Length of small Bowel is short
2. Deficiency of mucosal enzymes leading to manage
option and lactose intolerance
3. Ineffective peristalsis of proximal Bowel
4. Bacterial overgrowth syndrome

111. Postoperative complications
1. Anastomotic malfunction and leak
2. Lactose intolerant and malabsorption
3. Necrotising enterocolitis
4. TPS related complications
5. Problem related to extensive intestinal resection
– Rickets/ osteomalasia
– Megaloblastic anaemia
– Gallstones
– Renal calculi
6. Complications of small bowel syndrome

112. Prognosis
• Intestinal obstruction at the site of anastomosis and anastomotic
leak are the two most significant postoperative complication and
are associated with a reported mortality rate of 15%
• Infections related to pneumonia, peritonitis sepsis are the most
common causes of early death in infants with jejunoileal atresia
• Other factors affecting morbidity and mortality include
– Presence of associated anomalies
– Respiratory distress
– Prematurity
– Short bowel syndrome
– postoperative bowel obstruction

113. • Nixon used waterson's classification of risk
stratification
– A:> 2.5 kg, no significant anomaly
– B: 1.5 to 2.5 kg with moderate to severe anomaly
– C: <1.8 kg or with severe anomaly
• High Jejunal atresia- group A and B - 60%, group C 0%
• Mid Jejunal atresia- group A and B 82%, group c 32%
• terminal ileal atresia- group A or B 100 percent, group
C 60%

114. Meconium ileus

115. Meconium ileus
• Commonest cause of prenatal intrauterine intestinal
perforation
History:
• 1905- Landsteiner- first description and related it to the
pathological changes noted in the pancreas.
• 1936 - Fanconi -The term cystic fibrosis of the pancreas was
first coined
• 1938 – Anderson- described, the relationship of the
histologic likeness of pancreatic abnormalities in both
meconium ileus and cystic fibrosis (CF) and described
meconium ileus as an early and more severe manifestation
of the overall lung and pancreatic insufficiency.

116. • 1948- Hiatt and Wilson- described enterotomy
and saline irrigation
• 1953- Boiden- first to attribute the obstruction
to the abnormal thick mucus secretion by
intestine
• 1969- Noblett- introduced gastrograffin
enemas.

117. pathophysiology
Genetics:
• Cystic fibrosis – AR disease
• incidence of 1 per 1150 to 2500 live births in whites
• rare in blacks and it is almost unheard of in Asians and
Africans
• Meconium ileus is the earliest clinical manifestation of
CF. Meconium ileus has been reported to be the
presenting feature of 20.8% of the CF population.

118. • In 1989 the genetic mutation that codes for the cell membrane
protein termed the CF transmembrane regulator was identified by
Francis Collins
• long arm of chromosome 7, band q31
• protein was identified as a cAMP–induced chloride channel that
regulates ion flow across the apical surface of epithelial cells.
• Tubular structures lined by such affected epithelia will be
characterized by desiccation and reduced clearance of their
secretions, and the affected systems include epithelial cells of
respiratory, gastrointestinal, biliary, pancreatic, and reproductive
systems.

119. • The clinical correlate of this pathophysiology has included
pancreatic insufficiency (90%), meconium ileus (10% to
20%), diabetes mellitus (20%), obstructive biliary disease
(15% to 20%), and azoospermia (nearly 100%)
• The ΔF508 mutation is the most common of many CFTR
gene locations – 50% of patients
• A similar higher frequency of meconium ileus is also seen in
ΔF508-expressing patients who also express G542X.
• The association of obstructive lung disease with meconium
ileus suggests other CFTR modifier genes or even
environmental factors that may play a role in this disorder.

120. pathogenesis
• defect of CF is an exocrine-eccrine gland
dysfunction, particularly of the mucus-secreting
and sweat glands
• two simultaneous pathogenetic events appear to
begin in utero and result in an intra luminal
accumulation of a highly viscid and tenacious
meconium
– the development of pancreatic exocrine enzyme
deficiency and
– the secretion of hyperviscous mucus by pathologically
abnormal intestinal glands

121. • Glandular abnormality – less certain
• Various explanations include
– glandular secretion of hyperpermeable mucus
mediated by calcium that influences the
physicochemical properties of mucus to be viscid and
permeable to the loss of water, further concentrating
intraductal secretions. This glandularobstructing
mucus would lead to the pathologic lesions noted
earlier
– impairment of fluid movement to and from the
extravascular space through secretory cells exists that
may prevent the normal dilution

122. • Pathologic data interpreted - suggest that
intestinal glandular disease plays the dominant
role and pancreatic disease a secondary role in
the pathogenesis.
• CFTR was itself a membrane chloride channel and
that it further regulated chloride conductance
through other channels.
• This may be the mechanism that forms the basis
for the increased levels of sodium and chloride in
the eccrine sweat gland secretions that
characterize patients with CF.

123. Meconium itself has a variable composition:
– concentrations of sodium, potassium, and magnesium are
almost twice
– enzyme-catalyzing heavy metals are low
– containing greater amounts of protein nitrogen and lesser
amounts of carbohydrate
– Contain abnormal mucoproteins. The origin of these
proteins is uncertain, but they may arise from either
glandular secretions or swallowed amniotic fluid in utero.
– Because most of this protein is albumin, it is speculated
that the intraluminal proteins are of plasma origin

124. – They react with mucopolysaccharides, and, in the absence
of degrading enzymes, they impart a highly viscid rubbery
character to the involved secretions. The thickened
meconium accumulates and begins in utero to obstruct the
intestine lumen. This accumulation accounts for the
mechanism of the complications of meconium ileus
– The proximal ileum dilates, and its wall thickens as it
becomes filled with the tenacious and tarry meconium.
– Narrowed distal bowel, and, at times, the colon contains
beaded or “boxcar” concretions of gray-white, putty-like
inspissated meconium. The more distal colon is small or
unused, a microcolon.

125. Clinical Features
• Simple or uncomplicated MI
• Complicated MI
– Volvulus
– Perforation with meconium peritonitis
– Necrosis of bowel with atresia
– Meconium pseudo cyst
• In the older child or young adult the presentation
is as a meconium ileus equivalent or distal ileal
obstruction syndrome

126. Simple MI
• typically presents immediately at birth with
the recognition of abdominal distention
• This problem may first have been heralded by
an in utero genetic diagnosis of CF and an
ultrasound suggestive of intestinal obstruction
with echogenic bowel
• bilious vomiting and
• failure to pass meconium.

127. Complicated MI
• will present either in utero or postnatally
• evidence of bowel obstruction complicated by
• evidence of previous intestinal perforation
and/or necrosis may be present, or
• on clinical assessment there may be evidence
of peritonitis including
– an erythematous or edematous abdominal wall
and/or
– demonstrable abdominal tenderness

128. Clinical history
• family history of CF is present in 10% to 33% of
patients
• This history, coupled with in utero amniocentesis
with restriction fragment length polymorphism
analysis, permits the accurate diagnosis
• Maternal polyhydramnios may be a feature of in
utero meconium ileus (20%)
• Although in utero growth retardation is common,
prematurity and other associated anomalies are
rare.

129. PHYSICAL EXAMINATION
• Abdominal distention- In fact, meconium ileus is the only variety of
neonatal intestinal obstruction that produces abdominal distention
at birth before the neonate swallows air.
• Visible peristaltic waves
• palpable, doughy bowel loops
• putty sign
• rectal examination- unremarkable, but characteristically on
withdrawal of the examining finger a spontaneous expulsion of
meconium does not follow

130. • In the presence of an in-utero perforation with
meconium peritonitis and “cyst” formation,
– a palpable abdominal mass,
– discoloration of the abdominal wall, and
– Signs of peritoneal irritation are often observed.
– Physical evidence of hypovolemia may rapidly develop
in infants with peritonitis.
• On passage of a nasogastric tube, a quantity of
bile-stained gastric fluid usually exceeds 20 mL.

131. RADIOLOGIC STUDY
In utero,
– meconium ileus bowel may be distended
– echogenic bowel wall in the third trimester may be
diagnostic
After delivery
uncomplicated meconium ileus
• Plain x ray abdomen:
– great disparity in the size of the intestinal loops because of the
configuration of different segments of the bowel
– no or few air-fluid levels on the erect film because swallowed air
cannot layer above the thickened inspissated meconium; and
– granular, “soap bubble,” or “ground-glass” appearance seen
frequently in the right half of the abdomen,

133. Contrast enema:
– outline a normally positioned colon of appropriate
length but of small caliber
– Colon will be empty or will contain pellets of
inspissated meconium.
– Unused or microcolon
– If reflux of contrast agent into the terminal ileum
occurs, it will outline pellets of inspissated meconium
– If the contrast agent refluxes more proximally, the
transition into dilated loops of small bowel will be
encountered

134. Unused or microcolon

135. Laboratory Testing
• Sweat test-
– sweat is collected from the infant’s forearm, leg,
or back and
– the concentration of sodium and chloride in the
sample is measured
– Minimum amount of sweat to be collected is 100
mg
– a measured concentration of sweat chloride in
excess of 60 mEq/L is diagnostic of CF.

136. • Genetic testing for CF can be done by analyzing cellular
DNA for CFTR- negative results become less meaningful.
• If a family has known CFTR mutations, then amniocentesis
with fetal DNA RFLP analysis may predict fetal diagnosis.
• The pathophysiologic alteration of an increased albumin
concentration in meconium - albumin concentrations in
excess of 20 mg/g of stool. (normal- <5mg/g vs pt with CF -
>80mg/g)
• Stool trypsin and chymotrypsin analysis- A trypsin level less
than 80 mg/g of stool, coupled with operative findings

137. • Operative tissue specimens intestinal (rectal) or
appendiceal pathognomonic changes that include
– goblet cell hyperplasia and
– the accumulation of secretions within the crypts or
within the lumen.
• If operation is done for putative meconium ileus
and an appendicostomy is used as a bowel
intraluminal irrigation site, appendectomy may
be warranted to obtain such a diagnostic
pathologic specimen.

138. DIFFERENTIAL DIAGNOSIS
• Ileal atresia
• Hirschsprung disease
• small left colon syndrome
• Meconium plug syndrome

139. Ileal atresia
• distal bowel obstruction pattern on plain
radiographs with the associated presence of air-
fluid levels
• If a microcolon is demonstrated on contrast
enema, the contrast agent will not reflux into the
proximal dilated “atretic” bowel as it might in
both meconium ileus and Hirschsprung disease

141. Hirschsprung disease (especially total colonic aganglionosis)
– nearly all cases of total intestinal aganglionosis were initially
misdiagnosed as meconium ileus
– definitive diagnosis of Hirschsprung disease may be suggested
both radiographically and by anorectal manometrics, but
ultimately it depends on the histochemical findings
– radiographic features of colonic Hirschsprung disease likely will
include a transition zone
– reflux of contrast medium into the terminal ileum will typically
not demonstrate the filling defects of meconium ileus but,
rather, will show air-fluid levels and a proximally dilated bowel.

142. Neonatal small left colon syndrome
– This abnormality is confined to the left colon
– appears as a funnel-shaped tapering on contrast
enema and
– is often associated with a diagnosis of maternal
diabetes, hyperthyroidism, drug abuse, or
eclampsia.
– Rectal biopsy is required to exclude Hirschsprung
disease

144. Meconium plug syndrome
– is usually confirmed by a contrast enema radiograph
with the finding of “plugs” or “casts” of meconium in
the sigmoid or descending colon.
– Such plugs will often spontaneously pass after
withdrawal of the enema catheter and expulsion of
the enema
– There is a significant association of meconium plug
syndrome with other gastrointestinal anomalies, and
up to 14% of neonates with CF will be seen to have
meconium plug syndrome

145. – pathogenesis of the meconium plug is poorly
understood but may relate to a bowel
hypomotility
– has also been associated with prematurity,
hypotonia, hypermagnesemia, respiratory
distress, sepsis, hypothyroidism, diabetes, and
Hirschsprung disease
– should be studied with both a sweat test to
exclude CF and a rectal biopsy to exclude
Hirschsprung disease after symptoms of
obstruction have been relieved

146. management
• Non operative
• operative

147. Nonoperative Management
Noblett – criteria
1. an initial diagnostic contrast enema should exclude other causes of
distal intestinal obstruction;
2. the complications of volvulus, atresia, perforation, or peritonitis must
be excluded;
3. the enema must be performed with careful fluoroscopic control;
4. intravenous antibiotics should be administered;
5. the patient should be attended by a pediatric surgeon during the
procedure;
6. the patient should have a full fluid resuscitation with fluids given
aggressively (one to three times maintenance) during the procedure;
and
7. the patient should be prepared for imminent operation should
complications develop.

148. • Meglumine
– is a 76% aqueous solution of sodium methyl- glucamine
salt of N1N1-diacetyl-3,5-diamino-2,4,6- triiodobenzoic
acid,
– hypertonic solution has an osmolality of 1900 mOsm/L, a
property that draws fluid into the intestinal lumen and aids
in the release of the inspissated meconium.
– After administration, both a transient osmotic diarrhea
and a putative osmotic diuresis occur, factors that
emphasize the importance of aggressive fluid resuscitation
– In addition, the product is radiopaque, which enables a
safe fluoroscopically monitored administration

149. technique of solubilizing enema
treatment
• fluid resuscitation and nasogastric decompression
• plain abdominal radiographs have excluded the
diagnosis of peritonitis or perforation,
• the diagnostic contrast enema with barium or
water-soluble agent is administered.
• When the preliminary diagnostic study has been
completed, an enema-tip nonballoon catheter is
inserted into the anorectum and the buttocks are
taped together around the catheter.

150. – With fluoroscopic guidance and an initial solution of
50% Gastrografin in water is slowly injected by a
catheter-tipped syringe.
– When contrast medium traverses the colon and
reaches the dilated meconium impacted ileum, the
study is terminated.
– Spontaneous passage of the inspissated meconium
per rectum should follow.
– An abdominal radiograph should be repeated in 8 to
12 hours to determine whether the obstruction has
been relieved.

151. – If instead the evacuation is incomplete and
obstruction persists, the enema may be repeated
– If either no evacuation occurs after a successfully
refluxing enema or if contrast medium cannot be
refluxed into dilated bowel, then this technique
should be abandoned and operative intervention
planned.
– Noblett suggests that after a successful enema, 5
mL of a 10% N-acetylcysteine solution should be
administered every 6 hours through a nasogastric
tube to liquefy upper gastrointestinal secretions.

152. • Advantages
– a reduction in pulmonary morbidity and
– a reduced length of hospital stay.
• Disadvantages
– a delay in operative intervention for those
unsuccessfully treated by the enema,
– the risk of immediate and delayed intestinal injury
or perforation, and
– the induction of hypovolemia

153. Operative Management- simple MI
Enterotomy and saline irrigation:
– Treatment of choice for simple meconium ileus
– More concentrated solutions of N-acetylcysteine or
Gastrografin or the use of hydrogen peroxide and its
attendant risk of air embolism may produce greater risk
than benefit
– After solubilization by the irrigant, injected through an
enterotomy catheter, the meconium is gently milked
distally into the colon or evacuated through the
enterotomy
– The enterotomy and the abdomen may then be closed, an
enterostomy may be created, or the site can be controlled
by insertion of a T tube.

154. – By the seventh to fourteenth postoperative day, the
irrigant should pass freely into the colon, the
obstruction should be relieved, and thereafter the
catheter can be removed
– This avoids the need for reoperation and enterostomy
closure.
• An alternative technique
– appendectomy with appendicostomy, with meconium
evacuation or irrigation through this route.
– A temporary indwelling tube cecostomy may
alternatively be left in place.

155. The most commonly preferred contemporary operative management of
meconium ileus is shown.
An enterotomy is made in the dilated small bowel segment just proximal
to the site of inspissated distal meconium, and an irrigating catheter is
inserted.
After instillation of solubilizing agent, the catheter may be removed and
the enterotomy closed

156. • An alternative operation to enterotomy-irrigation is
placement of a temporary obstruction-relieving stoma with
or without an associated partial resection
• Mikulicz double-barreled enterostomy
– could be performed quickly
– did not require intraoperative meconium evacuation
– After the obstruction is relieved and the infant has recovered, a
spur-crushing Mikulicz clamp can be applied externally at the
stoma to complete a side-to-side anastomosis
– It may not be necessary to close the stoma- the residual
enterocutaneous fistula may spontaneously close.

157. • An alternative operation is resection coupled with
a distal chimney enterostomy, the so-called
Bishop-Koop procedure.
Criteria:
1. limit intraoperative bowel trauma in the neonatal period;
2. resect the disparately enlarged ileal loop filled with
inspissated thickenedmeconium;
3. create an appropriately sized end of proximal to side of
distal ileum anastomosis close to the abdominal wall
exiting the distal ileum, to serve as a functionally
decompressing “proximal stoma” while distal obstruction
persisted;

158. 4. provide access for insertion of a catheter into the distal
bowel containing inspissated meconium pellets so that
solubilizing pancreatic enzymes could be instilled
postoperatively and
5. permit an eventual enterostomy closure by bedside
ligation of the “chimney stoma,” putatively avoiding the
risks of an additional anesthetic in a child with known CF
• If the result of this noninvasive technique is a
persistent enterocutaneous fistula, then a formal
intraperitoneal or extraperitoneal stomal closure can
be performed with the patient under a general
anesthetic.

159. Santulli described a proximal chimney enterostomy:
• The distal ileal end is anastomosed end-to-side to the proximal
ileum and the proximal ileum is exited as an end enterostomy.
• With this stoma arrangement, irrigation and decompression of the
proximal ileum is enhanced.
• As with the Bishop-Koop procedure, an intraoperative catheter
passed through the stoma is positioned into the distal ileum for the
postoperative instillation of solubilizing agent.
• Because a high-output functional end enterostomy has been
created, it is necessary to close such a stoma early to avoid the
complications induced by excessive fluid and electrolyte losses.

161. Complicated MI
• Almost always requires operation, exception- rare
in utero perforation that has left
– a telltale remnant of extraluminal intraperitoneal
calcified meconium,
– a spontaneously sealed perforation, and
– no interruption in intestinal continuity
• Another finding for the latter process is calcified
meconium seen in a patent processus vaginalis
during a hernia operation or by abdominal
radiograph later in life.

162. Postoperative Management
1. support of the infant’s general physiology,
2. evacuation of any residual proximal or distal gastrointestinal
inspissated intraluminal meconium latter can be treated by
instillation of 2% or 4% acetylcysteine (Mucomyst)
3. enteral nutrition may begin with an elemental formula,
supplemental pancreatic enzymes must also be started
4. If postoperative ileus is prolonged or if short-bowel syndrome is a
product of the operative treatment, then total parenteral nutrition
5. Vitamin supplementation including vit.k
6. Short term parenteral antibiotics
7. Pulmonary physiotherapy
8. Stomal care
9. Perhaps the most important feature of postoperative care is to
secure the diagnosis of CF as the cause

163. complications
• Gastrointestinal
• Pulmonary
• Inguino scrotal

164. gastrointestinal
1. meconium ileus equivalent or distal intestinal
obstruction syndrome
2. Appendiceal luminal obstruction
3. Malabsorption
4. Intussusception
5. Rectal prolapse
6. Colonic strictures

165. meconium ileus equivalent
• most common gastrointestinal problem
• Signs and symptoms are heralded by
– crampy abdominal pain,
– distention,
– a palpable right lower quadrant mass;
– and a highgrade obstruction is associated with obstipation,
distention, and vomiting.
• broad differential diagnosis that may include
constipation, intussusception, and even appendicitis
• plain abdominal radiograph supplemented with
contrast body imaging should prove diagnostic

166. • Once the diagnosis is established, the preferred
treatment is solubilizing agents such as acetylcysteine
and Gastrografin, given both orally and per rectum
• Operative intervention is rarely required. Adjustment
of oral enzyme therapy supplemented with better
hydration and the use of stool softeners may be used
to prevent recurrence
• Both histamine H2 blockers and proton pump inhibitors
may prove efficacious by increasing intestinal pH, and
prokinetic agents have also been used.

167. Appendiceal luminal obstruction
• Appendiceal luminal obstruction that produces
signs and symptoms compatible with both acute
and “chronic appendicitis” may also occur
• The diagnosis of appendiceal pathology depends
on both clinical assessment and the potential use
of contrast medium-enhanced computed
tomography.
• Symptoms improved with appropriate enzyme
• replacement therapy

168. Malabsorption
• Malabsorption of carbohydrates, protein, and
fat may all occur, a problem worsened both by
the extent of peritoneal inflammation and the
magnitude of the short-bowel syndrome
• worsened by a disaccharidase deficiency and
by inspissated biliary secretions that produce
both jaundice and impaired fat absorption
• This same pathophysiology may account for
the occurrence of gallstones in about one
fourth of patients

169. Intussusception
• occurs in approximately 1% of older CF
patients,
• likely secondary to inspissated intraluminal
stool serving as the lead point
• typically ileocolic, but small bowel and large
bowel intussusception may also occur
• the diagnosis is difficult to establish and
distinguish from other right lower quadrant CF
pathology,
• and operation with reduction and/or resection

170. Rectal prolapse
• Rectal prolapse may be the first clinical
presentation in a child suffering with CF.
• up to one third to one fifth of children with CF
develop prolapsetypically between the ages of
1 and 3 years of age.
• The preferred treatment is oral enzyme
therapy, and rarely will more aggressive
transanal rectal submucosal sclerotherapy or

171. Colonic strictures
• presumptively secondary to large-dose oral
enzyme therapy
• Most commonly such disease is localized to
the right colon, but segmental colitis and even
pancolonic disease has been reported
• The local signs of obstruction and pain must
be distinguished from the meconium ileus–
equivalent patient
• a contrast enema is typically diagnostic
• Regulation of enzyme therapy may be tried,

172. PULMONARY
• The early morbidity and mortality of meconium
ileus predominantly has a pulmonary origin
• Studies suggest that children with meconium
ileus have worse lung function and more
obstructive lung disease between ages 8 and 12
years than those with CF but without meconium
ileus
• When Pseudomonas, coliforms, or other specific
colonizing or pathogenic organisms are identified,
the early and aggressive use of aminoglycosides
and semisynthetic penicillins has been shown to
improve survival and outcome

173. INGUINOSCROTAL DISEASE
• incidence of inguinal hernias and hydroceles,
as well as an increased incidence of
cryptorchidism
• Two operative findings at the time of inguinal
hernia repair may suggest the underlying
diagnosis of CF
– presence of calcified meconium in the hernia sac
and
– the absence of a vas deferens
• Both findings in the absence of a previous

174. Results of Treatment
• Outcome improved for the last 3 decades for
both complicated and simple MI
• Survival rate for both operative and non
operative treatment was 100% in the past
decade
• No significant overall differences in outcome
were observed with regard to patient gender,
whether complication of meconium ileus was
present, or with regard to the type of
operation performed

175. Thank you