Pediatric Umbilical Abnormalities

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
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
• Urachal Abnormalities
• Vitelline Duct Abnormalities
• Umbilical Hernia
• Omphalitis
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
• 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
• 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
 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
• More in girls, premature
• Assoc w/ Down’s Synd, Beckwith-
Wiedemann 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
• Can rapidly progress to Necrotizing
fasciitis (16%)
• Usually polymicrobial
• Rapidly fatal (50%)
• Surgical debridement necessary
Thank You!!!