Acute Umbilical Complaints in the Pediatric ER Or “my babies navel looks/smells funny” 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 2 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 3 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 4 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 5 Issues Umbilical • General Surgical Opinion (in the am!) • • • Likely omphalitis Consider infected urachal remnant Admitted • • Change to IV clindamycin U/S booked 6 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. 7 Issues Umbilical • Day 4 • • Discharged home for urgent elective repair to be booked Clindamycin oral course 8 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 9 Normal Cord care • Policies vary greatly in developing vs developed countries Marked decrease in incidence of Omphalitis in developed countries • • • In developed countries: • • ~0.7% vs up to 6 % 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…) 10 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 11 Umbilical infection • All cords are nearly immediately colonized • Staph and other gm+ves within hours • • Devitalized tissue is a good bacterial growth medium Mild discharge and absent inflammatory change, even with some odor is usually still a normal occurrence. • • • Enteric organisms shortly thereafter No proof for or against Rx with Alcohol, Bacitracin or Mupirocin…but many choose this. When does this constitute early Omphalitis? 12 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 suggest more severe infection/impending sepsis 13 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% 14 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 15 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 16 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 17 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 18 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 19 Case 2: Intra-operative, Post Umbilical Resection 20 Case 2: Intra-operative, Post Umbilical Resection 21 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 22 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 23 Omphalitis • Risk factors • • • • • • • LBW Prolonged labor PROM Non-sterile delivery Umb.A. cathetrization Home birth Improper cord care • • (cow dung, bentonite clay) • Poorer Prognosis • • • Male Premature “Septic delivery” • • • (including un-planned home delivery) Temperature instability Necrotizing fasciitis • (up to 85% mortality) Immune abnormalities 24 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 25 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 26 Omphalitis • Pathology of infection is presumed to be polymicrobial from 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 27 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 28 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 29 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 30 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 31 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 32 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 33 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” 34 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 35 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 36 Conclusion Respect Omphalitis 37 Something is wrong with my babies Navel • Umbilical Granuloma • Omphalo-mesenteric duct remnants • Urachal remnants 38 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 39 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 40 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 41 Umbilical Granuloma • Most common cause of umbilical mass and umbilical drainage • • • Usually post cord separation Persistent drainage of serous or serosanguinous fluid around the umbilicus A mass of pink granulation tissue at umbilical base • • • • • • Moist Pink Friable Soft Often pedunculated Usually 3-10 mm 42 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 43 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: 44 Vitelline, or Omphalomesenteric Duct Embryology 45 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 46 47 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 Firm masses. No response to AgNO3,and must be surgically excised OMD cyst • often asymptomatic, or may be an umbilical or abdominal mass; occasionally infected 48 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 Intraembryonic portions: • • The bladder = proximal portion. The urachus = more distal portion. 49 The Allantois 50 Urachal Remnants • • Urachal fistula - complete patency of the urachus Urachal cyst - remnant along tract (usually lower 1/3) • Urachal sinus Blind umbilical tract, unconnected to the bladder • Vesico-urachal diverticulum Antero-superior midline bladder dome • Umbilical (urachal) polyp 51 52 Urachal Remnants • Ultrasound is the ideal investigation for initial definition • Sinogram for patent urachus (“fistula”) or urachal sinus are other options • Renal U/S and VCUG have also been recommended 53 Urachal Remnants • Presentations: • May be subtle with erythema +/- drainage • Umbilical discharge or Omphalitis spectrum • Umbilical pain or retraction on micturition • Umbilical mass or cyst • Peri-umbilical pain 54 Urachal Remnants • All need to be excised • In adults, 50% have malignant (adneocarcinoma) changes at the time of excision (nil in children) • Cuff of normal bladder mucosa is excised during resection 55 Questions? 56 References 1) 2) 3) 4) 5) 6) Vane D.W. et al “Viteline Duct Abnormalities: Experience with 217 Childhood Cases Arch surg122:542, 1987 Pomeranz A. “Anomalies, Abnormalities and Care of the Umbilicus” Pediatric Clinics of N.A. 51:819, 2004 Rescorla F. J. “Hernias and Umbilicus” in Principles and Practice of Pediatric Surgery, volume 2, 2005 Cilento B. G. et al “Urachal Anomalies: Defining the Best Diagnostic Modalitiy” Urology52:120, 1998. Ashley R.A. et al “Urachal Anomalies: a Longitudinal Study of Urachal Remnants in Children and Adults” J Urol 178:1615, 2007 Cushing A.H. “Omphalitis: A Review”Pediatr Infect Dis 2:282, 1985 57 References 7) Sawardekar K.P. “changing Spectrum of Neonatal Omphalitis” Pediatr Infect Dis J 23:22, 2004 8) Mason W.H.et al “Omphalitis in the Newborn Infant”Pediatr Inf Dis J 8:521, 1989 9) Kosloske A.M. “Cellulitis and Necrotizing Fasciitis of the Abdominal Wall in Pediatric Patients”. J Pediatric Surg 16:246-251, 1981 10)Simon N.P. “Changes in Newborn Bathing Practices may Increase the Risk for Omphalitis” Clin Pediatr 43>763-767, 2004 11) Louie J.P. “Essential Diagnosis of Abdominal Emergencies in the First Year of Life”Emer Med. Clinics of N A 25:1009-1040 12) Zupan J. et al “Topical Umbilical Cord Care at Birth(Review)”Cochrane Library 2008, Issue 3 58 References 13) Mullany L.C et al “Development of a Clinical Sign Based Algorithm for Community Based Assessment of Omphalitis” Arch Dis. Child. Fetal Neonatal Ed. 91:F91F104, 2006 14) Mullany, L.C. “Topical Applications of Chlorhexidine to the Umbilical Cord for Prevention of Omphalitis and Neonatal Mortality n Southern Nepal: a Community-based, Clusterrandomized Trial” Lancet 367:910, 2006 15) Hseih, W.S. et al “Neonatal Necrotizing Fasciitis: A report of Three Cases abd Review of the Literature” Pediatrics103:e53, 1999 16) Iacono, G. “Red Umbilicus”:a Diagnostic Sign of Cow’s Milk Protein Intolerance. J. Ped.Gastro. And Nutr. 42:531534, 2006 17) Burd R.S. et al “Evaluation and Initial Management of Miscellaneous Pediatric Surgical Problems”Pediatric Annals30:752-759, 2001 59