1. Pediatric Umbilical Abnormalities
Scott Nguyen MD
Mount Sinai School of Medicine
Dept of Surgery

2. 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

3. Embryology - 3rd week

4. Embryology

5. Embrology

6. 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

7. Umbilical Abnormalities
Urachal Abnormalities
Vitelline Duct Abnormalities
Umbilical Hernia
Omphalitis
Delayed Cord Separation

8. Umbilical granuloma

9. 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

10. 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

11. Patent Urachus (50%)
Urachal cyst (30%)
Urachal sinus (15%)
Vesicourachal diverticulum (5%)

12. Patent Urachus

13. Studies
Catherization of tract and injection of dye
Voiding cystourethrogram US

14. Ultrasound

15. CT

16. VCUG

17. Treatment Patent Urachus

18. Patent Urachus

19. Urachal Cyst Usually assx until infected
Rarely become infected in newborn period, usu manifests as young adult

20. 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

21. US

22. CT

23. 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

24. Urachal Sinus Becomes symptomatic when infected
Tx – drainage and resection of urachal tissue

25. Sinogram

26. Urachal Diverticulum
Blind sac at bladder apex
Mostly assx

27. Urachal Diverticulum

28. Vitelline Duct Abnormalities

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

30. Vitelline duct abnormalities

31. Meckel’s Diverticulum

32. 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

33. Presentation
Painless GI Bleeding (50%)
Bowel Obstruction (30%)
Inflammation – diverticulitis (20%)

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

35. Meckel’s Scan – GI bleeding

36. 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

37. Intussusception

38. Intussusseption

39. 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

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

41. Fibrous Vitelline Remnant

42. Fibrous Vitelline Remnant

45. Barium Enema

46. Vitelline Umbilical Fistula

47. Vitelline Umbilical fistula
Umbilical polyp
May drain enteric contents
Fistulogram shows communication w/ bowel

48. Herniation

49. Umbilical Hernia

50. 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, Beckwith-Wiedemann synd, hypothyroidism, mucopolysaccharidosis

51. 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

52. Proboscoid Umbilical Hernias

53. 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

54. Omphalitis

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

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

57. 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

58. 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

59. Thank You!!!