3. 3
Case presentation
Presenting compliant
Watery discharge through the umbilicus of one year duration
History of present illness
A 4 year old male child presentingwith drainingof clear fluid
sometimes mixed with pusof one year duration and intermittent pain
but has no fever, difficulty of urinating, urgency, frequency.
No history of bowel habit change
Immunization history: immunized according to EPI schedule
Family history: his father, mother and rest of family membersare healthy
Physical examination
Generalappearance: well nourished
V/S: PR- 98 bpm RR-24 To - 36.2
LGS: no LAP
RS: clear chest with good air entry
CVS: s1 and s2 well heard
Abdomen : there is sinustract drainingclear fluid at the center of the
umbilicus, no palpablemass
GUS: well formed externalgenitalia
IS: no pallor
CNS: alart
Provisional diagnosis
Urachal fistula
4. 4
Differential diagnosis
Urachal fistula
Urachal sinus
Umbilical granuloma
Omphalomesentricduct sinus
Investigations
WBC: 9900,HGB: 11.6g/dl Plt: 287,000u/l
Neu%-34%
Lym%-44.9%
BG and RH-Otve
Abdominal U/S-thereis well defined walled hypoechoic lesion
measuring1.7X1.6X1.1cm withthick content in underneaththe anterior
at the umbilical level, there is cord like structurecontinuingin to
bladder but no sinus tract with the bladder
Comment: super infected urachal cyst
VCUG: normalposterior urethraloutline with no outpouching,
stricture , filling defect or fistuloustract
Comment: normal voiding cystourethrography
Final diagnosis : urachal sinus R/O fistula
Management: waiting for surgical resection
6. 6
Umbilical Abnormalities
1. ACQUIRED
A. Umbilical Granuloma
Introduction
Granulomasa is small mass of moist, fleshy and pink granulation tissue at
the center of umbilicus
Consist of true granulation tissue with fibroblasts and abundant capillaries
Apparent after separation of umbilical cord
Occurin 1:5000 newborns
Causes:
Not known but proposed hypothesis are
1. Inflammatory process:inflammation result in both overgrowth of endothelial
cells and inadequate epithelization
2. Delay in umbilical cord separation: may occurdue to bacterial infection ,
some immune disorders (leukocyte adhesion deficiency, neutrophil motility
disorders etc.) and imbalance between umbilical skin flora and saprophytic
organism facilitating invasion with pathologic microorganisms which
preclude epithelization
Clinical presentation
Pinkish /yellowish Umbilical discharge and small swelling typically seen in
neonatal period and separation of the cord after one to two weeks
Does not cause pain and irritability unless complicated with infection
because it doesn’tconsistof nerve fibers
Small (1 mm to 1 cm in size), soft, nontender, pale/pink colored lesion at
base of umbilicus with normal surrounding skin.
Diagnosis
Carefulhistory and physicalexamination
Contraststudy: to rule outsmall vitelline or urachal fistula
Ultrasound: to identify associated abdominalanomalies
7. 7
Treatment
1. Silver nitrate
Conventionaltreatmentof umbilical granuloma world wide
Although antiseptic effect are remarkable it has caustic effect as well
75% of silver nitrate and 25% potassium nitrate should be
preferred for chemical cauterization
Umbilical area should be cleaned with antiseptic solution and dried
with sterile spongebefore application and Care must be taken with
application becauseburns and skin injury may occur
If the granulomapersistsafter 3 or 4 applicationswhich are
performed an intervalof 3-4 days, other potential disease or
alternative treatment should be considered.
2. Salt application
Umbilicus cleaned with wet cotton pad and then pinch of crystal
salt is sprinkled on granuloma
Followingthis the granulomais closed with an adhesive drape
The drapeis opened 30 minuteafter the procedureand application
terminated
This processis repeated 3 times a day for 3 consecutivedayswith
success rate of 100% in the study population
3. Surgical excision
Treatment of choice in large granulomaor recurrent cases
Excision and chemical cauterization of the umbilical base may be
combined in relatively big granulomaswith wideroot
If granulomais excised , it should be histopathologically examined
to rule outany embryologic remnant.
Table 1.1 treatmentoptions of umbilical granuloma
Family based options (at Home) Physician based options (in medical center)
Topical home salt Silver nitrate
Topical antiseptic solution Ligation
Topical antibiotics Excision with or without hemostatic materials
Topical steroids Electrocautary
Observation (dry care) Cryotherapy
9. 9
Figure 2.1 Various omphalomesenteric duct remnants. A, Umbilical cyst containing
intestinal tissue. B, Umbilical sinus with a band. C, Umbilical polyp
covered with intestinal mucosa. D, Fibrous band containing a cyst. E, Meckel diverticulum.
F, Patent omphalomesenteric duct.
10. 10
If the omphalomesenteric ductis patent from the terminal ileum to the
umbilicus, fecal umbilical drainage will be noted
Anatomically unusual conditions suchas an unexpected origin of the
omphalomesenteric duct from the appendix will be recognized at the time of
operation
11. 11
Treatment
Unless another, more serious medical condition exists, a patent
omphalomesenteric duct should be excised promptly.
A mechanical intestinal preparation is not necessary, although we
customarily stop formula feeding; perioperative intravenous antibiotics are
also given
Full exploration and identification of all umbilical structures including one
vein, two arteries, and the urachal remnant are indicated.
The omphalomesenteric ductis traced to the ileum and divided. The ileumis
closed, and care must be taken to control any dominant vitelline vessels that
may be present. After the fascia is closed, umbilicoplasty is performed
If a Meckel diverticulum is attached to an omphalomesenteric band
discovered at explo-ration, it is excised.
Cystic remnants of the omphalomesenteric duct may become infected. If an
abscess has formed, it may require surgical drainage; excision of any
remnant may be accomplished at a later time
Rarely, spontaneous regression of a patent omphalomesenteric duct may
occur
A
B
12. 12
Meckel Diverticulum
Embryology
The yolk sac nourishes the embryo via the vitelline circulation.
The intracoelomic yolk sac forms the gut, and the extraembryonic yolk sac
begins to regress as it is replaced by the placenta as the primary sourceof
nourishment for the developing fetus
With growth, the fetal intestine becomes separated from the yolk sac,
leaving only a ductal communication (vitelline/omphalomesenteric duct),
which obliterates between the fifth and seventh weeks of fetal life
Avariety of anomalies can result from failure of involution of the
omphalomesenteric duct: The classic Meckel diverticulum accounts for 90%
of cases
Epidemiology
The most common omphalomesenteric duct remnant and congenital
anomaly of the gastrointestinal tract
Most cases of Meckel diverticula are sporadic and less than 5% of patients
will have any associated abnormalities
Found in 1.2% to 3% of the population
Among symptomatic patients it is twice as frequent in males.
The “rule of 2” is often cited with regard to the diverticulum:
2 feet from the ileocecal valve
2 inches long
2 cm in diameter
2 types of heterotopic mucosa
Usually discovered before 2 years of age
2 times as common in males, and it is found in 2% of the
population.
Pathology
True diverticulum, containing all the normal bowel wall layers.
Almost always on the antimesenteric border.
Three fourths of Meckel diverticula is free-floating within the peritoneal
cavity
Variable location but mostly located within 100 cm of the ileocecal valve
At least 5 feet of distal small bowel should be thoroughly examined to rule
out the presence of a diverticulum.
13. 13
Mean diverticular length is 2 to 3 cm
The incidence of heterotopic mucosa in asymptomatic patients is about 15%
but ranges between 15%to 50% both in incidental and symptomatic cases
Symptomatic patients are far more likely (40%to 50%)to have heterotopic
mucosa
Gastric is the most common type, followed by pancreatic.
Other types of heterotopia (colonic, endometrial, pancreatic islets) are rare
The incidence of gastric mucosain bleeding Meckel diverticula has been
estimated to be as high as 80%
Gastric acid production causes ulceration, usually at the baseof the
diverticulum at the junction of ectopic gastric mucosa and normal ileal
mucosa and rarely at ectopic gastric mucosaitself or on the mesenteric side
of the ileum oppositethe diverticulum
A Littre hernia is defined as one containing a Meckel diverticulum, The
usual sites are inguinal (50%), umbilical (20%), and femoral (20%); the
most common location in children is umbilical
Clinical presentation
Mostly are clinically silent.
It has been called the “great imitator” becauseof its relative infrequency and
protean manifestations.
Symptomatic patients are usually younger
Up to half of symptomatic Meckel diverticula will present in the first 2 years
of life, and most complications will develop before 10 years of age.
Can present as hemorrhage , obstruction, diverticulitis or neoplastic
complication
Complications
A. Bleeding
Accounts for nearly 50% of all lower gastrointestinal bleeding in
children, usually occurring in infants and toddlers
Often diagnose preoperatively unlike inflammatory or obstructive
symptoms
Episodic painless bleeding is the most common scenario
Unexplained isolated anemia is the sole presentation in a small
fraction of children
Color of the stools-bright red (35%), maroon or dark red (40%), or
less commonly, tarry (7%)
14. 14
Helicobacterpylori is rarely found in the heterotopic gastric
mucosa of a Meckel diverticulum: the presence of bile is the
postulated mechanism
B. Obstruction
Intussusception and volvulus are the most common
Intussusception is slightly more common in children, and volvulus in
adults
Mechanisms of intestinal obstruction due to a Meckel diverticulum.
Intussusception with the diverticulum acting as a pathologic
lead point.
Loop of intestine “kinked” around a fibrous vitelline remnant.
Volvulus around a fixed, tethered vitelline remnant attached to
the abdominal wall.
Volvulus of a diverticulum around its own base.
Self-knotting” Meckel diverticulum.
Internal herniation of ileum under a mesodiverticular
band/vessel.
Littre hernia
C. Inflammation
Usually occurs in older children and is commonly misdiagnosed as
appendicitis
Are usually due to the presence of gastric or pancreatic tissue in the
diverticulum
Other mechanisms include stasis of contents in the diverticulum
(kinking, narrow neck), enterolith, foreign body, and parasitic or other
infection
Correct preoperative diagnosis is uncommon
Inflammatory complications are treated by resection, either of the
diverticulum alone or including the involved bowel. Primary
reanastomosis is usually possible
D. Neoplasia
Found in 0.5% to 4% of Meckel diverticula, and malignant tumors
predominate.
Benign tumors:-leiomyomas, lipomas, angiomas, and neurofibromas.
Malignant tumors :-leiomyosarcoma, carcinoids, adenocarcinoma,
villous adenoma, gastrointestinal stromal tumors, andothers.
Overall, carcinoid is the most common tumor arising in the
diverticulum.
15. 15
A mean age of diagnosis is 56.6 years and a male predominance
Less than 5% of patients were younger than 20 years of age.
Approximately three fourths of the tumors were found at the tip of the
diverticulum.
Tumors larger than 5 mm were more likely to metastasize, but only
24% had metastasized at the time of diagnosis, with a significantly
higher incidence of metastases in women.
Slightly more than half were asymptomatic.
The classic carcinoid syndrome was present in 8% at diagnosis
Investigation
Technetium-99mpertechnetate scintigraphy(meckel’sscan)
Is the investigation of choice
It is used to detect heterotopic gastric mucosa
Pentagastrin, histamine blockers and glucagon may enhance the accuracy of
diagnosis
Sensitivity of only 60%to 84%in children, with a high false-negative
predictive value, especially in anemic patients
False negatives may be due to rapid dilution of the radiotracer from a high
bleeding rate, poorblood supply to the diverticulum, or an inadequate
amount of gastric mucosa.
Less accurate in the diagnosis of a bleeding diverticulum in adults, where the
accuracy ranges from 15% to 50%
Mesentric angiography
In patients with severe active bleeding
Usually negative unless the bleeding rate is greater than 0.5 mL/min.
computed tomography(CT)scan
Wireless capsuleendoscopy
Treatment
The treatment of a symptomatic Meckel diverticulum is resection.
This can be done with open or laparoscopic techniques.
Either simple resection of the diverticulum and transverse closure across the
base, or resection of a short segment of ileum containing the diverticulum
with reanastomosis
In patients with bleeding or inflammatory symptom resection of the ileal
segment with the diverticulum is the safest option.
The feeding (diverticular) artery should be clearly identified and ligated.
16. 16
Incidental Diverticulectomy indications
Patient younger than 40 years
Diverticula longer than 2 cm
Diverticula with narrow neck
Diverticula with fibrous band
Suspected gastric ectopic tissue
Inflamed, thickened diverticula
17. 17
B. Urachal Remnants
Urachus is a fibrous remnantof allantosis , a channelbetween the
bladder and umbilicus
In fetus, urineinitially drainsfrom urachusduringfirsttrimester of
pregnancy
The channelof urachususually seals off and eradicates around the
12th week of gestation and remain as median umbilical ligament
Abnormal epithelium including colonic, small intestine, and squamous
Pain plus retraction of the umbilicus during micturition : sign of a urachal
anomaly
Patent urachus
Is associated with drainage of urine from the umbilicus.
Frank drainage of urine from the umbilicus requires an investigation
of the urinary tract to look for bladder outlet obstructionin which the
urachus is functioning as a relief valve
May be approached either through the umbilicus or through an
infraumbilical incision..
18. 18
The patent urachus is ligated and transected at the level of the bladder;
broad-based connections are closed in two layers with absorbable
sutures.
A, Patent urachus with communication between the bladder and umbilicus. B, Urachal sinus. C, Urachal cyst
Urachal sinuses
May give rise to umbilical drainage or be discovered on examination.
Urachal cysts
Most often cause an infection manifested as a painful mass localized
between the umbilicus and the suprapubic area
When a urachal cyst becomes infected and develops into an abscess,
drainage of the acute process is required.
It is unknown whether subsequent operation to remove any residual cyst
remnants is necessary.
A partial list of tumors in adults arising from the urachus
19. 19
Diagnostic imaging occasionally be helpful in diagnosing and treating
umbilical abnormalities including ultrasound, contrast injections, computed
tomography, and magnetic resonance imaging.
Umbilical polyp
Typically domeshaped or pedunculated proliferation of moderately
cellular fibroustissue
Diagnosed duringthe first 2 yearsof life
Male preponderance
Etiology unclear
They do not result in any problem but are excised dueto their unsightly
appearance
Umbilical Hernia
Anatomy
At birth the umbilicus is surrounded by a dense fascial ring that represents a
defect in the linea alba
Reinforced by strongly attached remnants of the umbilical arteries and
urachus in an inferior direction and the more weakly attached umbilical vein
in a superior direction
The peritoneum forms an intact undersurface of the umbilical ring, and skin
overlies the umbilicus after the cord has separated. When the supporting
fascia of the umbilical defect is weak or absent, a direct hernia results
The umbilical ring continues to close over time and the fascia of the
umbilical defect strengthens, which accounts for the spontaneous resolution
of this defect in most children
Direct umbilical hernia : supporting fascia of the umbilicus is weak or absent
An indirect umbilical hernia:-the peritoneal contents herniate from a point
immediately superior to the umbilical ring.
The umbilical hernia of childhood is distinguished from a“hernia of the
umbilical cord,”in which there is a defect in the peritoneum, as well as an
open fascial defect at the umbilicus
Incidence
Occurwith equal frequency in boys and girls.
High incidence in African and African American infants
20. 20
Natural history
The umbilical ring is open throughout most of gestation but becomes
progressively smaller as gestation progresses.
Most umbilical hernias in infants are recognized after cord separation in the
first few weeks of life, and almost all are noted by 6 months of age.
Most undergo spontaneous closure during the first 3 years of life and some
at 5 years of age
Commonly found in low-birth-weight infants (75% of infants weighing <
1500 g), most will resolve but occurin 20 % of full-term neonates
The diameter of the umbilical defect is prognostically important than the
length of the protrusion with a small ring diameter (<1cm) are more likely to
close spontaneously and close sooner than those with a large ring diameter
(>1.5 cm).
Commonly observed in patients with Down syndrome, trisomy 18, trisomy
13, mucopolysaccharidoses, and congenital hypothyroidism
Incarceration is rare, but the small bowel is most commonly affected when it
does occur.
May also becomesymptomatic during pregnancy, and if incarceration
occurs, surgery is required.
Unusual contents of umbilical hernias include uterine fibroids and
endometrial elements
Surgical indications
Typical umbilical hernias should be observed at least until age 2 unless it is
incarceration requiring reduction, strangulation, perforation, and evisceration
If there is no improvement in the size of the umbilical fascial ring, consider
repair.
Ample evidence supports the decision to postponerepair until later in
childhood.
Large defects (>1.5 cm) that persist past the age of 5 should be repaired.
In an infant with an inguinal hernia and a concomitant umbilical hernia, the
umbilical hernia should generally be left alone because it will probably close
spontaneously.
If the child has a tender umbilical mass, the hernia may be reduced by
milking the air out of the incarcerated loop of intestine and applying firm,
steady pressure on the incarcerated mass.
Admitting a patient for observation to rule out peritonitis and performing the
operation the next day are appropriate.
21. 21
Surgical technique, results,
And complications
Appropriate skin preparation and application of sterile linen drapes, a curved
(‘smile’) incision is made in a natural skin crease immediately below the
umbilicus
A supraumbilical incision is also acceptable, especially if a supraumbilical
defect is encountered.
The curved incision should typically not extend beyond 180°
The neck of the hernia sac is circumferentially incised and opened
Omentum is ligated and excised if necessary, and the sac is trimmed
back to strong fa scia
The fascial defect is closed with buried, inte rrupted stitc hes in a transverse
fashion utilizing long-lasting absorbable suture
Skin is closed with subcuticular suture, a liquid adhesive and steri -strips are
placed, and the wound is dressed with a pressure bandage
22. 22
Reference
1. Coran pediatric surgery 7th edition
2. Ashcraft’s Pediatric Surgery7th edition
3. Operative Pediatric Surgery 7th edition