Pediatric Umbilical Abnormalities

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Pediatric Umbilical Abnormalities

• Scott Nguyen MD
• Mount Sinai School of Medicine
• Dept of Surgery

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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

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Embryology - 3rd week
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Embryology
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Embrology
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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

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Umbilical Abnormalities

• Urachal Abnormalities
• Vitelline Duct Abnormalities
• Umbilical Hernia
• Omphalitis
• Delayed Cord Separation

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Umbilical granuloma
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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

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Urachal abnormalities

• failure of obliteration of urachus resulting
  complete or partial patency of urachus
• lt 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)

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Patent Urachus
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Studies

• Catherization of tract and injection of dye
• Voiding cystourethrogram
• US

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Ultrasound
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CT
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VCUG
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Treatment Patent Urachus
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Patent Urachus
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Urachal Cyst

• Usually assx until infected
• Rarely become infected in newborn period, usu
  manifests as young adult

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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

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US
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CT
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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

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Urachal Sinus

• Becomes symptomatic when infected
• Tx drainage and resection of urachal tissue

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Sinogram
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Urachal Diverticulum

• Blind sac at bladder apex
• Mostly assx

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Urachal Diverticulum
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Vitelline Duct Abnormalities
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Vitelline Duct

• Vitelline Duct is connection between midgut and
  yolk sac
• Usually involutes in 7th 9th weeks

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Vitelline duct abnormalities
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Meckels Diverticulum
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Meckels Diverticulum

• contains ectopic gastric or pancreatic mucosa
• In 2 of population
• 2 feet from ileocecal valve, antimesenteric
  border
• Majority of symptomatic lt 2yrs old

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Presentation

• Painless GI Bleeding (50)
• Bowel Obstruction (30)
• Inflammation diverticulitis (20)

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GI Bleeding

• Most common cause of bleeding in children
• Painless, massive, usually self resolving
• Due to mucosal ulceration from acid secretion

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Meckels Scan GI bleeding
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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

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Intussusception
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Intussusseption
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Meckels Diverticulitis

• Sx like appendicitis
• Result of lumenal obstruction, bacterial
  invasion, progressive inflammation
• Ectopic gastric mucosa predisposes
• 30 incidence of perforations
• Higher risk of peritonitis

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Treatment

• Surgical Resection without removal of ileum
• V shaped incision at base
• resection of involved segment of ileum w/ primary
  anastamosis

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Fibrous Vitelline Remnant
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Fibrous Vitelline Remnant
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Barium Enema
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Vitelline Umbilical Fistula
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Vitelline Umbilical fistula

• Umbilical polyp
• May drain enteric contents
• Fistulogram shows communication w/ bowel

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Herniation
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Umbilical Hernia
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Umbilical hernia

• Protrudes
• Rarely incarcerates
• Incidence 10-25 infants
• 6-10x higher incidence in Black infants
• More in girls, premature
• Assoc w/ Downs Synd, Beckwith-Wiedemann synd,
  hypothyroidism, mucopolysaccharidosis

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Treatment

• Most close by 3-4 years age (gt90)
• Defect greater than 1.5 2 cm less likely to
  close
• Surgical closure indicated in kids gt5 years age

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Proboscoid Umbilical Hernias
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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

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Omphalitis
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Omphalitis

• erythema and edema of umbilical area
• excellent medium for bacterial colonization
• poor hygiene or hospital-acquired infection
• Staphylococcus, Streptococcus, Gram (-) rods

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Treatment

• IV Antibiotics
• Local cleaning w/ Etoh
• Can rapidly progress to Necrotizing fasciitis
  (16)
• Usually polymicrobial
• Rapidly fatal (50)
• Surgical debridement necessary

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Delayed Cord Separation

• Separation gt 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

• 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

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Thank You!!!