Pediatric Umbilical Abnormalities

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Pediatric Umbilical
Abnormalities
Scott Nguyen MD
Mount Sinai School of Medicine
Dept of Surgery
Abnormalities of Umbilical Cord


Umbilical abnormalities result from failure of
umbilical ring to close or persistence of
umbilical structures
Understanding embryology of cord is essential
in understanding the pathophysiology of
umbilical abnormalities
Embryology -
rd
3
week
Embryology
Embrology
Embryology




6th wk – midgut loop elongates and herniates
out through umbilical cord
Midgut rotates 270 degrees
Returns to abdomen by 10th wk
Anterior abdominal wall progressively closes
leaving only umbilical ring
Umbilical Abnormalities
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
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Urachal Abnormalities
Vitelline Duct Abnormalities
Umbilical Hernia
Omphalitis
Delayed Cord Separation
Umbilical granuloma
Urachal formation



Bladder forms from
ventral portion of cloaca
Bladder descends into
pelvis w/ urachus
connecting apex to
umbilicus
Usually urachus involutes
to a fibrous cord –
median umbilical
ligament
Urachal abnormalities
•
•
•
•
failure of obliteration of urachus resulting
complete or partial patency of urachus
< 1/1000 live births
inflammation or drainage from umbilicus
US, CT, contrast studies, or injection of dye into
tract can confirm diagnosis
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•
•
•
Patent Urachus (50%)
Urachal cyst (30%)
Urachal sinus (15%)
Vesicourachal diverticulum (5%)
Patent Urachus
Studies
Catherization
of tract and
injection of dye
Voiding cystourethrogram
US
Ultrasound
CT
VCUG
Treatment Patent Urachus
Patent Urachus
Urachal Cyst


Usually assx until
infected
Rarely become infected
in newborn period, usu
manifests as young adult
Infected Urachal cyst
 Fever,
voiding symptoms, midline
hypogastric tenderness, mass, UTI
 May drain into bladder or umbilicus
 Rarely can rupture into preperitoneal
tissues or peritoneal cavity
 Cultures - Staph Aureus
US
CT
Infected Urachal cyst - treatment
 Incision
and drainage
 Percutaneous drainage
 Complete surgical excision of all
urachal tissue
 30% recurrence if only drainage
 Staged approach limits amount of
bladder resected
Urachal Sinus


Becomes
symptomatic when
infected
Tx – drainage and
resection of
urachal tissue
Sinogram
Urachal Diverticulum
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
Blind sac at
bladder apex
Mostly assx
Urachal Diverticulum
Vitelline Duct
Abnormalities
Vitelline Duct


Vitelline Duct is connection between midgut
and yolk sac
Usually involutes in 7th – 9th weeks
Vitelline duct abnormalities
Meckel’s Diverticulum
Meckel’s Diverticulum
 contains
ectopic gastric or pancreatic
mucosa
 In 2% of population
 2 feet from ileocecal valve,
antimesenteric border
 Majority of symptomatic < 2yrs old
Presentation
 Painless
GI Bleeding (50%)
 Bowel Obstruction (30%)
 Inflammation – diverticulitis
(20%)
GI Bleeding
 Most
common cause of bleeding in
children
 Painless, massive, usually self resolving
 Due to mucosal ulceration from acid
secretion
Meckel’s Scan – GI bleeding
Bowel Obstruction
 Due
to intussusception, diverticulum is
the lead point
 Sudden severe pain out of proportion
to physical exam
 Hydrostatic Barium enema diagnostic,
rarely therapeutic
Intussusception
Intussusseption
Meckel’s Diverticulitis
 Sx
like appendicitis
 Result of lumenal obstruction,
bacterial invasion, progressive
inflammation
 Ectopic gastric mucosa predisposes
 30% incidence of perforations
 Higher risk of peritonitis
Treatment
 Surgical
Resection without removal
of ileum
 V shaped incision at base
 resection of involved segment of
ileum w/ primary anastamosis
Fibrous Vitelline Remnant
Fibrous Vitelline Remnant
Barium Enema
Vitelline Umbilical Fistula
Vitelline Umbilical fistula
 Umbilical
polyp
 May drain enteric
contents
 Fistulogram
shows
communication
w/ bowel
Herniation
Umbilical Hernia
Umbilical hernia
Protrudes
 Rarely incarcerates
 Incidence 10-25% infants
 6-10x higher incidence in Black infants
 More in girls, premature
 Assoc w/ Down’s Synd, BeckwithWiedemann synd, hypothyroidism,
mucopolysaccharidosis

Treatment
 Most
close by 3-4 years age (>90%)
 Defect greater than 1.5 – 2 cm less
likely to close
 Surgical closure indicated in kids >5
years age
Proboscoid Umbilical Hernias
Proboscoid umbilical hernias
 15-20%
of umbilical hernias
 Same sized fascial defect
 Same likelihood of closing
spontaneously
 Excessive redundant umbilical skin
 Surgical repair for social and cosmetic
reasons
Omphalitis
Omphalitis
 erythema
and edema of umbilical area
 excellent medium for bacterial
colonization
 poor hygiene or hospital-acquired
infection
 Staphylococcus, Streptococcus, Gram
(-) rods
Treatment
 IV
Antibiotics
 Local cleaning w/ Etoh
 Can rapidly progress to Necrotizing
fasciitis (16%)
 Usually polymicrobial
 Rapidly fatal (50%)
 Surgical debridement necessary
Delayed Cord Separation
Separation > 3 wks may be associated w/
an immune deficiency
 Normal separation via leukocyte
infiltration, subsequent necrosis
 Inherited malfunction of neutrophil,
monocyte, or natural killer cells
 Susceptible to severe bacterial infections
 Immunologic workup
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Leukocyte Adhesion Deficiency
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Deficiency of phagocyte surface Ag – CR3
Cell surface proteins responsible for phagocyte
adhesion to endothelium
Inability to egress from circulation to areas of
inflammation
Phagocytic activity, degranulaton, and oxidative
metabolism also affected
Thank You!!!
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