Acute Umbilical Complaints in the Pediatric ER

1. Acute Umbilical Complaints in the Pediatric ER Or “my babies navel looks/smells funny”

2. Issues Umbilical - Case 1 • 9 week girl infant. Presents to PLC-ER • Swelling of the umbilicus for ~5 hours • Erythema and a central Umbilical “lump” noted • No fever • Some poor feeding with no vomiting for less than a day • ~6 wet diapers past 24 hours

3. Issues Umbilical - Case 1 • 5.11 kg, Cap refill <3 sec • T 36.4, R28, P 145, BP 78/49 • Alert, no distress • N H&N • N chest and HS • Soft benign abdomen with no masses • Central, red umbilical bulge within skin cuff (cushion) • Small volume thin purulent drainage? • Slight erythema 4-7o’clock? No induration or demarcation

4. Issues Umbilical • Referred to ACH-ER with: “? Umbilical hernia, R/O Omphalitis” ACH ER exam similar overall • C&S of Umbilical “discharge” • CBC, Lytes • Felt likely to be Omphalitis • Referral to General Surgery • Ancef 25 mg/kg commenced

5. Issues Umbilical • CBC • WBC 9.7, Neuts 2.6 • Hb 106, Platlets 522 • Na 138, Cl 103, K 4.7, HCO3 23 • Cr 17, Urea 2.2 • U/A neg

6. Issues Umbilical • General Surgical Opinion (in the am!) • Likely omphalitis • Consider infected urachal remnant • Admitted • Change to IV clindamycin • U/S booked

7. Issues umbilical • In Hospital course • Always remained afebrile • C&S umbilical discharge “scant skin flora only” • U/S abdomen: • Swollen protruding umbilicus noted to be filled with echogenic material. A sinus tract is identified which extends form the lower umbilicus and connects to the superior and anterior wall of the bladder in the midline. The appearance is consistent with a patent urachus.

8. Issues Umbilical • Day 4 • Discharged home for urgent elective repair to be booked • Clindamycin oral course

9. Objectives of Naval Mission • Discuss omphalitis • Discuss common cord care • Understand the non-infectious abnormalities that can occur in the umbilicus, notably in the infant • Not to discuss • Umbilical hernia management • Case room cord examination and implications

10. Normal Cord care • Policies vary greatly in developing vs developed countries • Marked decrease in incidence of Omphalitis in developed countries • ~0.7% vs up to 6 % • In developed countries: • Cochrane review shows no form of cord cleaning/antiseptic is better than dry cord care • In developing countries antiseptics in cord care markedly decrease death and omphalitis (chlorhexidine, AgSulfadiazine, Triple dye…)

11. Cord Separation • Normal timing of ~1 week or less for separation • Prolonged by certain agents • 70% alcohol: ~17 days • Triple dye: 3-8 weeks • True “delayed” separation (without agent application) is in excess of 3 weeks

12. Umbilical infection • All cords are nearly immediately colonized • Staph and other gm+ves within hours • Enteric organisms shortly thereafter • Devitalized tissue is a good bacterial growth medium • Mild discharge and absent inflammatory change, even with some odor is usually still a normal occurrence. • No proof for or against Rx with Alcohol, Bacitracin or Mupirocin…but many choose this. • When does this constitute early Omphalitis?

13. Omphalitis • Predominately Neonatal • Mean age of onset in term infants is 3.5 days • Infection of umbilicus and/or surrounding tissues • Purulent (+/-bloody) drainage from stump • Surrounding induration, erythema, tenderness • BUT • Lethargy, fever, Irritability, poor feeding suggestmore severe infection/impending sepsis

14. Omphalitis • Complications: • Sepsis / death • Septic umbilical arteritis/portal vein thrombosis • Peritonitis/liver abscess/intestinal gangrene • Small bowel evisceration • Necrotizing fasciitis • Present-day Mortality: 7-15%

15. Case 2 • 14 day infant girl transferred to ACH-ICU for umbilical infection • 41 weeks GA • C/S for fetal distress • APGARs 81 & 95 • GBS+ve • Passed N mec. At 24 hours • No jaundice • Breast fed/BM 8x/day • Cord loss ~1 week of age

16. Case 2 • Day 11 • Some peri-umbilical redness, afebrile • Poor evening feeding • Day 12 • Worsening erythema, wider area • Abdomen appeared “puffy” • T = 38.50C • To local community hospital; blood-streaked stool in ED, and with all serial later BMs • Much worse feeding and lethargy • Sepsis workup/LP/Ampicillin and Cefotaxime and admitted

17. Case 2 • Day 13 • General progression of anorexia, and increasing abdo wall abnormalities. • U/S abdomen, and transferred to ACH overnight • Day 14 ACH - PICU • Change to Flagyl, Meropenum, Clindamycin. And Gentamycin • Surgery/Plastics consult

18. Case 2 • Physical • 88/60, 153,100%RA, 37.5, 40, 4.0Kg • AF flat, no jaundice • CVS N save CRT “2-5 seconds” • No increased WOB • Mottled extremities • Distended abdomen. Black umbilicus, surrounded by an inner purple and outer white halo, both non-blanching. Rt > Lt, ~30% of abdo wall • Whole remainder of abdomen wall is erythematous

19. Case 2 • Lab • WBC of 33.7 • CRP 72.8 • Hb 148, Platlets 501 • To ACH-OR for debridement, and bowel inspection for R/O NEC • Abdo wall biopsy and C&S • Bowel observed to be vital without NEC • Umbilicus and surrounding tissues resected including necrotic skin and abdo. wall to healthy fascia • Frozen section biopsy consistent with Nec Faciitis

20. Case 2: Intra-operative, Post Umbilical Resection

21. Case 2: Intra-operative, Post Umbilical Resection

22. Case 2 • OR visits on PICU-days 1,2,4,6 and 8 for serial lesser debridements and bowel inspection • Wound closure PICU day 8 but subsequent dehisence day 19 • Change to tazocin/vancomycin day 7 • Wound grew • Enterococcus faecalis • Coag neg Staph • Actinomyces

23. Case 2 • Day 12 - extubated • Day 13 - to the ward • Day 19 - Wound dehisced • Day 30 - discharged home • All Abx discontinued • planned delayed closure abdo. wall ~2 weeks later

24. Risk factors LBW Prolonged labor PROM Non-sterile delivery Umb.A. cathetrization Home birth Improper cord care (cow dung, bentonite clay) Immune abnormalities Poorer Prognosis Male Premature “Septic delivery” (including un-planned home delivery) Temperature instability Necrotizing fasciitis (up to 85% mortality) Omphalitis

25. Omphalitis/Any Soft Tissue Infection • There is a continuum of severity: Cellulitis Infection of skin and S.C. fat Necrotizing fasciitis Infection of skin, S.C. fat and superficial and deep fasciae Myositis/myonecrosis Deep muscle infection with muscle death

26. Omphalitis/Any Aggressive Soft Tissue Infection • Should be presumed to be poly-microbial at outset • “the usual suspects” in Omphalitis: • Staph Aureus • Gp A Strep • Coag Neg Staph • Enterococci • Gm Negs: E Coli, Klebsiella P., Proteus Mirabilis… • Anerobes: Bacteroides, Clostridium perfingens/tetani

27. Omphalitis • Pathology of infection is presumed to be polymicrobialfrom the outset • Abx must cover for this, and include: • Anti-stahpylococcal penicilin or vancomycin • Aminoglycoside • Probable Clinamycin or Metronidazole Esp. if maternal chorioamnionitis and/or foul discharge, for anaerobic coverage

28. Omphalitis • Necrotizing Fasciitis • Rare complication of omphalitis • Polymicrobial • Involves skin, subcutis, superficial and deep fasciae • Rapid spread is typical • Bacteremia, systemic toxicity, and shock in high proportion. Death 60-85% • Early aggressive surgical intervention, broad spectrum antibiotics, and supportive ICU care

29. Case 3 • 38 2/7 week boy • 30 yr G1P1 mother, N Vtx Vag delivery • APGARs 81 and 85 • Short ACH transfer Day 1-3 for ?ileal atresia…final Dx Meconium plug • Day 13 • Peri-umbilical redness noted by family

30. Case 3 • Day 14 • Admitted to local hospital • Dx Omphalitis • Ampicillin and Gentamycin • Day 15 • Increasing redness in abrupt fashion: 5cm above and 3cm below umbilicus • Transfer to ACH ICU • Dx Omphalitis, R/O Necrotizing Fasciitis

31. Case 3 • ICU: • Not toxic • Abdo wall is only abnormality of serious note • WBC 16.5, N diff, INR N, Lytes N and Neg AG • Urgent tissue biopsy • No Nec Fasciitis; consistent with cellulitis • Neg gram stain • Neg blood and urine C&S. • Surface Umb C&S from Primary hospital • Coag neg staph, and enterococcus faecalis

32. Case 3 • I.D. Service: Antibiotics changed to • Meropenum, Clindamycin, and Gentamycin • Day 16 • Child improves sufficiently that ward transfer is in process…..then oliguria unresponsive to fluids arises • Scrotal swelling and severe progressive abdominal wall edema • ICU stay maintained

33. Case 3 • Day 17 • 03:00 Resp failure/ETT • 05:00 dobutamine infusion • 05-10:00 progressive metabolic acidosis • 10:00 to OR • Abdominal exploration. Healthy bowel. • Abdo wall : Excision of navel and surrounding tissue. Biopsy now positive for Necrotizing fasciitis • Deterioration: • with coagulopathy, WBC up to 49.5, INR elevated, ARDS / pulmonary hemorrhage

34. Case 3 • Day 17 • Progressive deterioration and difficulty ventilating. Rising Cr up to 180 • 13:30 back to OR • Abdominal compartment syndrome • Bowel “eviscerates” under pressure and ventilatability markedly improves…bowel seems healthy; Abdo Wall Margins still look healthy, and back to ICU with bowel encased in a “silo bag”

35. Case 3 • Severe oliguria • Lines placed and dialysis commenced • Poor tolerance with repeated hypotension and need for fluid bolusing • Day 18 • Several bradycardic arrests • Progressive instablilty and dialysis discontinued • Family agree to discontinue all supportive Rx • 04:20 child pronounced

36. Case 3 • C&S from initial umbilical ACH biopsy • Coag neg staph • Enterococcus faecalis • Clostridium sordellii • Autopsy conclusion • Necrotizing faciitis of poly-microbial nature • Sepsis

37. Conclusion Respect Omphalitis

38. Something is wrong with my babies Navel • Umbilical Granuloma • Omphalo-mesenteric duct remnants • Urachal remnants

39. Case 4 • 12 day infant girl • 41 3/7 weeks, vacuum assisted SVD • GBS -ve • Thriving • Cord dehisced day 7 • Umbilicus raw, oozing with sero-sanguinous discharge since

40. Case 4 • Looks well • P 165, R 26, T 37.1, BP 76/42 • General Exam Normal • No peri-umbilical redness • Moist “nodule” of pinkish-red tissue over stump site. Bleeds easily • ?Umbilical Granuloma (vs some other developmental lesion)…Referred to Surgery Clinic DDR

41. Case 4 • In clinic 1 month later • Major lump had “fallen off” and moist base was cauterized with AgNO3 • Re seen 3 weeks later: • Area dry and fully healed • Diagnosis: Umbilical Granuloma

42. Umbilical Granuloma • Most common cause of umbilical mass and umbilical drainage • Usually post cord separation • Persistent drainage of serous or sero-sanguinous fluid around the umbilicus • A mass of pink granulation tissue at umbilical base • Moist • Pink • Friable • Soft • Often pedunculated • Usually 3-10 mm

43. Umbilical Granuloma • Treatment: • AgNO3 local Rx 1-2 x per week • If it persists post 3-4 Rx sessions • Can be ligated (be sure its not a polyp!) or referred to general surgery for formal excision

44. Omphalo-mesenteric Duct Remnants • Omphalo-mesenteric duct (Viteline duct): • Connects the developing GI tract to yolk sack • Regresses by ~9th week GA • Disruption of this regression causes the list of abnormalities:

45. Vitelline, or Omphalomesenteric Duct Embryology

46. OMD Remnants • Umbilical fistula • Complete patency of OMD with stoma-like connection to the terminal ileum • Partial persistence of OMD • Fibrous band umbilicus to ileum • “Distal” remnant - OMD-enteric cyst • “Proximal” remnant - Meckel’s diverticulum • Umbilical polyp - a mucosal remnant in the umbilical stump

48. OMD remnants • Fibrous band • can cause volvulus; obstruction and/or volvulus are most common infant presentation • Umbilical Polyp • Usually enteric, but occasionally urachal origin. Rarely pancreas, liver • Firmmasses. No response to AgNO3,and must be surgically excised • OMD cyst • often asymptomatic, or may be an umbilical or abdominal mass; occasionally infected

49. Urachal Remnants • Urachus is the embryologic descendant of the allantois. • Allantois is the most distal projection of the primitive gut, projecting into the extra-embryonic cord. Of it’s Intra-embryonic portions: • The bladder = proximal portion. • The urachus = more distal portion.

50. The Allantois